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a 60-year - old man was referred to our outpatient clinic because of persistent air leakage lasting 17 days from his chest tube , which had been inserted for empyema at another hospital . he had a past history of diabetes mellitus and tuberculous pleuritis treated with antituberculous medicine 30 years ago . he had not been treated previously for empyema by drainage procedures such as closed thoracostomy , percutaneous catheter aspiration , or drainage . a chest computed tomography ( ct ) scan showed loculated pleural fluid collection with air density and pleural wall thickening with enhancement and calcification . further , a 2.2-cm mass - like lesion adjacent to the margin of an empyema cavity was identified retrospectively ( fig . laboratory tests revealed a slightly elevated c - reactive protein level of 1.0 mg / dl , while other parameters were within the normal range . seven days after admission , we planned decortication because of prolonged air leakage and the patient s desire for surgery . the fibrinous contents of the empyema cavity were removed , and the lung was decorticated without difficulty . therefore , the operation was completed after the confirmation of the full expansion of the lung . contrary to our expectations , however , the histopathological diagnosis after the operation was malignant lymphoma . immunohistochemically , the tumor cells were positive for leukocyte common antigen , cd79a , ki-67 ( range , 7080% ) , and cd3 , and negative for cd20 . 2 ) . after the identification of a monoclonal proliferation of b - cells by an immunoglobulin heavy- chain gene rearrangement , the final diagnosis of diffuse large b - cell lymphoma with an aberrant expression of a t - cell marker was made . the postoperative period was uneventful , and the chest tube was removed on postoperative day 12 . we considered the possibility of incomplete resection , and the patient was referred to the hematology department for adjuvant chemotherapy . pyothorax - associated lymphoma ( pal ) is a rare malignant lymphoma developing in the pleural cavity after long - standing pyothorax . according to the current world health organization histological classification published in 2005 , pal is defined as a neoplasm of large b - cells , typically with immunoblastic morphology , usually presenting as a pleural mass. it occurs in patients with a history of long - standing pyothorax resulting from pulmonary tuberculosis or tuberculous pleuritis and is strongly associated with the epstein barr virus ( ebv ) . a majority of these cases have been reported in japan , although some cases have occurred in western countries . it is assumed that the higher prevalence in japan is caused by a higher incidence of ebv infection and lung collapse therapy for tuberculosis in asia , particularly in japan . the etiology of pal is not clearly understood . however , previous reports have suggested that artificial pneumothorax , ebv latent infection , cytokines such as interleukin-6 and -10 , and oxidative stress produced during chronic inflammation might be important factors for pal development . reported a summary of clinical and pathological findings in 106 patients with pal in japan . the median age of the patients was 64 years ( range , 46 to 82 years ) with a male / female ratio of 12.3:1 . the interval between the onset of pleuritis and the initial symptoms of lymphoma was 37 years ( range , 20 to 64 years ) . all of the cases were of non - hodgkin s lymphoma , among which the diffuse large b - cell type was the most common ( 88% ) . the definitive diagnosis of pal can be made by histopathological and immunohistochemical examinations of biopsy or surgically resected specimens . a typical histological examination demonstrates a diffuse destructive proliferation of large cells with a predominant population of immunoblasts . lymphoma cells are mostly positive for cd20 and cd79a in the immunohistochemical examination . according to the pathological findings by aozasa et al . , a majority of the cases were cd20 + and/or mbi+ , cd45ro- , and cd3- , and were of the b - cell lineage . however , there could be an aberrant phenotype with the expression of some t - cell markers , as in our case . ueda et al . analyzed the radiological features of pal to help in the diagnosis of this rare malignant lesion . they reported that a typical radiological finding of pal was a pleural soft - tissue mass adjacent to the margin of a coexistent empyema cavity and the shape of the mass demonstrated on the ct scan was mostly lenticular or crescentic . we were able to find a mass - like lesion on the ct scan retrospectively by reviewing the report of ueda et al . although the optimal treatment is not well - established , most patients with pal have received chemotherapy and/or radiotherapy , as reported in the literature . the prognosis of pal reported in 2002 is poor , with a five - year survival rate of 21.6% , although a better survival rate is observed in patients who are responsive to chemotherapy . however , a recent survey reported an improved overall five - year survival rate of 35% and suggested several prognostic factors for the overall survival . in summary , we have reported a case of pal of a b - cell origin resulting from tuberculous pleuritis . although pal is a relatively rare disease , it should be considered a potential diagnosis in patients with a history of chronic pyothorax .

pyothorax - associated lymphoma is a relatively rare type of lymphoma that occurs in patients who have long histories of tuberculous pleuritis or induced pneumothorax . it is a type of non - hodgkin s lymphoma of mainly the b - cell phenotype and is strongly associated with epstein barr virus infection . a majority of these cases have been reported in japan , although some cases have occurred in western countries . here , we describe a case of pyothorax - associated lymphoma in a patient with a 30-year history of chronic tuberculous empyema . the patient underwent decortication under the impression of chronic empyema with fistula . the histopathologic diagnosis was a diffuse large b - cell lymphoma associated chronic inflammation .

esophagopleural fistula ( epf ) is an uncommon condition despite the anatomical proximity of the trachea and esophagus . causes of epf include pneumonectomy for suppurative or tubercular disease and carcinoma of the lung and malignancy of the esophagus . non - malignant epf is due to trauma or infection . the most common infectious cause of epf is tuberculosis , the others being syphilis , mycotic disease , and crohn 's disease . perforation of the esophagus and subsequent fistula formation can occur as a result of foreign bodies , barrett 's ulcer , and more rarely boerhaave 's syndrome . spontaneous development of a fistula between the esophagus and pleura is rarely described in the literature . a 34-year - old male was admitted with a 1-month history of cough , breathlessness , regurgitation during feeding , and chest discomfort . he was a known case of bipolar mood disorder and was on antipsychotics for the last 7 years . general survey was unremarkable . on chest examination , an enlarged right hemithorax with decreased movement of the right chest and stony dull note over the right hemithorax from the fourth , eighth , and ninth intercostal spaces downward along the midclavicular , midaxillary , and scapular chest x - ray revealed right pleural effusion . on thoracocentesis , there was a dry tap . on ultrasonography of the thorax , there was minimal right pleural collection with internal echoes ( not amenable for aspiration ) . on high - resolution computed tomography ( hrct ) of the thorax , an encysted collection ( 7.9 cm 8.5 cm 10 cm ) was noted in the right pleural space showing air and food particles in it with a linear fistulous communication from the distal esophagus , and on oral contrast administration , the contrast medium was seen to enter into the collection [ figures 1 and 2 ] . finally , the upper gastrointestinal ( gi ) endoscopy confirmed a large fisulous tract measuring 9 mm at the distal esophagus without any mucosal abnormality . with the definite diagnosis of epf , the patient was transferred to the department of cardiothoracic and vascular surgery ( ctvs ) where thoracotomy and closure of the fistula were performed . during the procedure , a number of possible causes for the development of epf following pneumonectomy for suppurative disease of the lung have been suggested . anatomically , the esophagus lies much closer to the right hemithorax than to the left , the left being separated from the pleural cavity by the aorta . the possibility of direct epf and extent of mediastinitis are determined by the anatomic relationship of the esophagus and the pleura , the amount of mediastinal fat , and intervening connective tissue . fistula related to empyema was the most commonly reported case in the preantibiotic era . nonetheless , there continues to be isolated reports of posttuberculous pyopneumothorax fistulas . erosion of an empyema into the esophagus and rupture of caseating lymph nodes into the esophagus are the possible mechanisms . in our case , the cause of epf was unclear . evidence of malignancy was undetectable from radiology and no specific infectious agent was isolated at the time of diagnosis and treatment . esophageal injury should be considered when a patient presents with retrosternal chest pain , fever , dysphagia , and dyspnea , especially when the patient gives antecedent history of instrumentation or surgery . the diagnosis of epf can be suspected clinically ; however , for confirmation , imaging is required . the imaging modalities include chest radiograph , ultrasound , barium swallow , contrast - enhanced computed tomography ( ct ) , and magnetic resonance imaging ( mri ) with each modality having its advantages , and chest ct is a very useful modality . leaks of the esophagus are associated with high mortality and the need to be treated as soon as possible . therapeutic options include surgical repair or resection or conservative management with antibiotic therapy and cessation of oral intake . endoscopic treatment with fibrin glue , clip , suturing , and metallic stents has been described . to conclude , spontaneous development of epf is an unusual condition entity with nonspecific clinical presentation . ct of chest is a very useful modality for early diagnosis and management of epf . it should be performed in patients with pleural effusion presenting with nonspecific clinical symptoms before any intervention or drainage .

esophago - pleural fistula ( epf ) is an uncommon condition , despite of an anatomical proximity of these structures . causes of epf include pneumonectomy for suppurative or tubercular disease of lung and carcinoma lung , malignancy of esophagus . benign epf is rare and may be due to trauma or infection . the most common infectious cause is tuberculosis . spontaneous development of fistula between esophagus and pleura is rarely described in literature . we , hereby present a spontaneous case of such a rare entity in a middle - aged male .

in april 2009 , a 63-year - old woman was admitted to our hospital because of a frontal headache that occurred when she woke up . the thoracic pain was described as continuous , nonconstrictive , not related to breathing , with no irradiation , and which decreased when she laid down . her past medical history was marked by a copd gold ii condition , a chronic left arm oedema of undetermined origin , and an appendicectomy . her current medications included an association of fluticasone and salmeterol as well as tiotropium for the copd . she had been smoking 100 cigarette pack - years , had occasional alcohol consumption , and didnot have any known allergy . the only clinical abnormality was the presence of a murmur on the left supraclavicular area . the first blood test showed an elevation of c - reactive protein at 9.8 mg / dl and a mild normocytic anemia at 11.1 g / dl . the electrocardiogram showed normal sinus rhythm and negative t waves of small amplitude in the second precordial derivation . a computed tomography of the pulmonary artery did not reveal pulmonary embolism but disclosed a circumferential pericardial effusion . the patient was managed as an outpatient and treated with 4 grams of aspirin each day for idiopathic pericarditis . after two weeks of this regimen , the inflammatory syndrome failed to subside , and the controlled transthoracic echocardiogram did not show any improvement of the pericardial effusion . during the course of the following days , the patient developed fatigue and was referred to the consultation of general internal medicine . suspecting giant cell arteritis , fdg - pet was performed because the patient refused the temporal artery biopsy . it revealed striking hypermetabolism of the thoracic and abdominal aortic wall , highly suggestive of large vessel vasculitis ( figure 1 ) . six months after the beginning of the treatment , the fdg - pet didnot show any hypermetabolism in the aortic walls ( figure 1 ) . this case highlights the importance of recognising pericarditis as a possible initial manifestation of giant cell arteritis in patients over 50 years old . it also emphasises the promising role of fdg - pet as a minimally invasive alternative procedure to temporal artery biopsy . fdg - pet can also be used in the followup of patients with giant cell arteritis and seems to identify a subpopulation of patients at risk of developing thoracic aortic dilatation . in the present case , the diagnosis of large vessel vasculitis ( and most probably giant cell arteritis ) has been made based upon clinical grounds , biological markers , and fdg - pet . in spite of the fact that temporal artery biopsy is mandatory to confirm the diagnosis of giant cell arteritis , fdg - pet can be a very useful tool in delineating the diagnostic process .

large vessels vasculitis and more specifically , giant cell arteritis , is characterized by increased inflammatory markers , headaches and altered clinical status . diagnosis is confirmed by biopsy of temporal arteries showing the presence of granuloma and vasculitis . we hereby report the case of a patient presenting initially as pericarditis and revealing large vessel vasculitis using fdg - pet .

woolly hair nevus ( whn ) is characterized by an area with fine , curly and often hypopigmented hair , on a circumscribed area of the scalp . hutchinson et al . classified three variants of woolly hair : hereditary woolly hair ( autosomal dominant ) , familial woolly hair ( autosomal recessive ) and a whn . woolly hair syndrome ( whs ) affects to the whole scalp and it may be associated with other cutaneous anomalies and extracutaneous anomalies , such as noonan syndrome or carvajal disease . recently , it has been found in two cases of whn , somatic hras c. 34 g > a , p.g12s mutation in affected in hair . also , a hras c. 37 g > c , p. gly13arg mutation has been described in nevus of jadassohn and schimmelpenning syndrome . we report the case of a 27-year - old healthy male who presented with an area of curlier and thinner hair than the rest of his scalp localized on the right parietal area . he referred that just at that location , his hair had been different since childhood , curlier than the rest . physical examination revealed a plaque with shorter and curlier hair on an area of 10 cm in diameter on the right parietal area of the scalp . a decreased density of hair was observed . a skin biopsy showed a mild perifollicular lymphocytic infiltrate , telangiectatic capillaries in the superficial dermis and several terminal hair follicles emerged in the same dilated follicular infundibulum . interestingly , an excessive amount of normal apocrine glands were found in the deep reticular dermis . counting of hair follicles was normal [ figure 2a c ] . based on clinical appearance and histopathologic studies , a diagnosis of whn ( a ) a plaque with shorter and curlier hair on an area of 10 cm in diameter on the right parietal area of scalp . skin on the patch was apparently healthy . ( b ) an area of curlier hair than the rest of his scalp localized on the right parietal area since childhood ( a ) several involved terminal hair follicles ending in the same infundibulum , a perifollicular lymphocytic infiltrate and an excessive amount of normal apocrine glands ( h and e , 4 ) . ( b ) several involved sterminal hair follicles ending in the same infundibulum and a perifollicular lymphocytic infiltrate ( h and e , 20 ) . ( c ) an excessive amount of normal apocrine glands ( h and e , 20 ) whn usually appears during the 1 years of life and it remains stable throughout adulthood . it may be associated with pigmented or epidermal nevus , usually on the neck , arm or even coexisting with whn . histopathologic findings of whn are scarcely described in the literature , probably because most cases are diagnosed clinically . a wavy appearance of hair follicles with perifollicular infiltration of inflammatory cells has been described , but normal hairs and cutaneous appendages may also be observed . in our case , we found a different histopathological pattern , with several involved terminal hair follicles ending in the same infundibulum , a perifollicular lymphocytic infiltrate and an excessive amount of normal apocrine glands , findings similar to those of nevus of jadassohn . it may be possible that whn is the clinical manifestation of a wide range of different histopathologic hamartomatous lesions involving the hair follicles , some of them clearly evident , with thinner and curved hair follicles and accompanying epidermal nevus in the overlying epidermis , and subtle histopathologic changes in other cases , as in our case . these findings described indicate that clinicopathologic correlation is necessary to establish a diagnosis of whn with confidence .

woolly hair nevus is a rare disease whose diagnosis is challenging . we present a case of this condition presenting in a 27-year - old healthy male . we describe a histology pattern consisting in the presence of several terminal hair follicles ending in the same dilated follicular infundibulum , a perifollicular lymphocytic infiltrate and an excessive amount of normal apocrine glands in deep reticular dermis , some findings non - previously reported . clinicopathological correlation is very important for making a correct diagnosis .

in 1984 , aitken and minton were the first to document a case of catheter fracture and embolization secondary to a pinching effect between the clavicle and the first rib1 ) . distal embolization of a fractured indwelling venous access catheter is a known , but rare , complication associated with these commonly used devices2 ) . we present a case of catheter fracture as a consequence of the pinch - off syndrome ; this was successfully treated using a transluminal approach , in the pulmonary artery , with a goose neck snare and pigtail catheter . a 39-year - old woman with a 10-year history of liposarcoma had an implantable intravenous access devices ( celsite st 201 , 8.5 fr , b / braun medical inc , france ) placed in the left subclavian area , using the standard technique , to allow the administration of adjuvant chemotherapy . three - months after implantation of the intravenous access device , the patient complained of chest and jaw pain for several days ; she denied palpitations , dyspnea and syncope . cardiac auscultation revealed a regular rate and rhythm without any murmur , gallops or rubs ; a normal s1 and s2 was present . the chest x - ray two months prior to presentation showed a grade two " pinch - off sign " ( figure 1 ) and the chest x - ray on admission showed a fractured and embolized catheter in the pulmonary artery ( figure 2 ) . several days later , transesophageal echocardiography was performed and revealed no thrombus and spontaneous echo contrast in the right side of the heart and pulmonary artery . computed tomography of the chest first a retrieval of the fractured fragment of the catheter was attempted via the right femoral vein with a goose neck snare ( 10 mm loop , er3 , usa ) an h1 catheter ( 5 fr , cook , usa ) . although we could grasp the foreign body using a snare , it was impossible to retrieve it ; the patient continued to complain of severe chest and jaw pain . we , therefore , changed the catheter with a pigtail catheter ( 5 fr , cook , usa ) . with accompanying fluoroscopic monitoring , we were able to successfully grasp the fragmented catheter ( figure 4 ) and to retrieve it ( figure 5 ) . at a three - month follow up the patient was doing well without any complication detected . an implanted central venous access device is frequently used for the administration of chemotherapy or parental nutrition . most of the associated complications can be avoided by a careful approach to implantation and subsequent management4 ) . the complication rate has been reported to decrease as experience with this device increases4 ) ; it has been reported to be 12~14% in studies with large numbers of patients5 , 6 ) . catheter fracture is a rare complication associated with these devices , with an estimated rate of 0.1~1%7 , 13 ) . implantable central venous access devices placed via the subclavian vein may become obstructed by thrombosis , impingement against a vein wall , or a compression between the clavicle and first rib8 ) . chronic intermittent compression of the catheter between the clavicle and the first rib , which can occur when the catheter has been inserted too far medially , can lead to catheter erosion and fracture ( pinch - off syndrome)1 , 8 , 9 ) . at catheter insertion , the angle between the clavicle and the first rib is wide ; the catheter can pass through this area , medial to the vein , before entering the subclavian vein . when a patient is in the upright position , the angle narrows and leads to a mechanical compression of the medially positioned catheter1 ) . when the catheter is inserted , by puncture of the subclavian vein using the seldinger technique , the pinch - off sign is observed in 1.1~5.0% of cases9 - 11 ) . a radiologic scale of catheter distortion was defined by hinke and colleagues9 ) ; a grade zero refers to no compression and distortion , a grade one refers to an abrupt change in direction but no luminal narrowing , grade two indicates some degree of luminal narrowing , and grade three defines complete catheter fracture . in cases of catheter narrowing , patients should be followed carefully by chest radiography every four weeks , and the catheter should be removed within six months following insertion11 ) . the presence of a fragmented central venous catheter should lead to immediate removal to prevent thromboembolic events13 ) . with the goal of avoiding such fractures , insertion of the catheter lateral to the mid - clavicular line this technique ensures that the catheter , when crossing the critical point , is then located within the vein which provides some protection1 ) . denny and frank reviewed 42 cases of fragmented central venous catheters from the surgery , radiology and oncology literature . only two cases ( 5% ) presented with symptoms described as " chest discomfort " in one case and " sudden onset of bilateral chest pain " in the other2 ) . the catheter fracture and embolization , in the asymptomatic patients , were identified on routine chest radiographs . by contrast , our patient presented with chest and jaw pain for several days ; these symptoms were likely secondary to the mechanical irritation of the pulmonary artery by the embolized catheter . percutaneous removal of the catheter , and guide wire fragment , is generally a safe procedure . several devices are available for transcatheter retrieval , including grasping forceps and urinary stone baskets . if the migrated catheter adheres to the myocardium , an open thoracotomy would be required for retrieval ; an alternative approach without retrieval would be a course of warfarin administration8 ) . in conclusion , radiological evaluation of an indwelling catheter , in any case of catheter malfunction ,

vascular catheters are associated with complications such as infection , thrombosis and stenosis . the embolization of a venous catheter fragment is a rare complication . a 39-year - old woman underwent placement of a totally implantable venous access device for chemotherapy to treat a recurrent liposarcoma of the left thigh . the " pinch - off sign " was noted on a routine chest x - ray but that was ignored . three - months after implantation of the intravenous access device , the indwelling central catheter was fractured and embolized to the pulmonary trunk . the catheter in the pulmonary trunk was successfully removed through a percutaneous femoral vein approach using a pigtail catheter and goose neck snare .

fibrodysplasia ossificans progressiva ( fop ) , also known as stone man syndrome , is a severely disabling and catastrophic - inherited disorder of connective tissue characterised by congenital malformation of the great toes , thumbs and vertebrae associated with progressive ossification of striated muscles . in such patients , progressive fusion of axial and appendicular skeleton , temporomandibular joint ( tmj ) ankylosis , associated restrictive lung disease and sensitivity to even trivial oral trauma make airway management challenging . an 11-year - old , 30 kg male child , suspected to be a case of cysticercosis , presented with multiple hard swellings over the nape of neck , paraspinal region , arms , thighs and legs . pre - operative airway evaluation revealed a mallampati class iii , restricted neck movement ( 45 ) and mouth opening ( mo ) of 2.5 cm . cardio - respiratory examination and all routine investigations including chest x - ray and electrocardiogram ( ecg ) were found to be normal . in the operation room , ecg , non - invasive blood pressure , end tidal carbon dioxide and pulse oximeter were attached . the only fibreoptic bronchoscope ( fob ) in the department was not working . hence , truview pcd video laryngoscope ( truview video laryngoscope [ tvl ] , netanya , israel)-guided intubation was planned . anaesthesia was induced with sevoflurane 5%8% ( 1.5 minimum alveolar concentration ) in oxygen and supplemented with propofol 30 mg . during laryngoscopy with tvl , a cormack and lehane ( cl ) grade 3 of glottis was obtained which improved to cl 2b with optimum external laryngeal manipulation . a tube introducer could be inserted into the glottis , and a size 6.0 flexo metallic endotracheal tube was threaded over it . thereafter , muscle relaxation was achieved with injection atracurium 10 mg , and the patient was positioned prone for surgery . adequate padding was applied to eyes , bony prominences , deformities of upper limbs ( shoulder and elbow ) and swellings of lower limbs . during excision biopsy , the surgeon reported bony tissue in the nodules and got suspicious of fop . the surgery was abandoned , the patient was made supine and his trachea was extubated after reversal of neuromuscular blockade . injection diclofenac sodium 50 mg was given as slow iv infusion for post - operative pain . subsequent histopathology of the biopsy tissue revealed mature osseous tissue , audiometry showed conductive hearing loss and skeletal survey revealed ectopic bone formation over the back , neck , upper limbs and lower limbs , thus confirming the diagnosis of fop [ figures 1 and 2 ] . classical signs of fibrodysplasia ossificans progressiva in our patient : great toe malformation ( a ) and extensive heterotopic ossification on the back ( b ) head and neck x - ray with linear ossification of the paraspinal muscles fop ( myositis ossificans progressiva ) is autosomal dominant connective tissue disorder characterised by extra skeletal endochondral ossification . it may be associated with mutation in bone morphogenic protein4 ( bmp4 ) antagonist gene which leads to increased production of bmp . it is characterised by progressive heterotopic bone formation in the connective tissue and skeletal muscle . the ossification usually starts in the neck , spine and shoulder girdle and progressively immobilises all the joints of the axial and appendicular skeleton during the first decade of life . hence , one should avoid deep intramuscular ( i m ) injections , injections into jaws for dental procedures , invasive biopsy , excision procedures for heterotopic masses and manipulations of stiff joints to prevent disease progression . these patients have a median life span of 40 years and usually die due to complications of thoracic insufficiency syndrome or pneumonia . anaesthetic management of these patients is complicated due to anticipated difficulty in airway management ( cervical spine fusion and tmj ankylosis ) , restrictive lung disease and their extreme sensitivity to trauma . the common interventions during anaesthesia such as traumatic iv and arterial cannulation , i m injections , overstretching of joints during positioning and regional blocks can precipitate iatrogenic heterotopic ossification and any such intervention should be avoided . in early stages , patients may not have any symptom , and since it is a rare entity , the diagnosis may be missed . this patient had bony hard swellings with restricted movements and was misdiagnosed as calcified muscular cysticercosis initially . during surgery when hard bone - like tissue was found in the nodule during dissection , fop was suspected . subsequently , a diagnosis of fop was confirmed on the basis of two classical features , namely congenital great toe malformations and heterotopic ossification in specific anatomic patterns [ figures 1 and 2 ] and mature bone tissue on histopathology of the biopsy tissue . difficult airway and can not ventilate can not intubate situation needing emergency tracheostomy have been reported in fop . direct laryngoscopy and manoeuvres - like jaw thrust may cause ossification and ankylosis due to overstretching of tmj . hence , overstretching of the oropharyngeal structures should be prevented by avoiding jaw thrust and ensuring that the mo during laryngoscopy and intubation remains well below the base line values obtained prior to the anaesthetic . awake fibreoptic intubation is considered the gold standard even in patients with adequate mo and jaw movement to avoid any stimulus to tmj . to prepare the airway for awake intubation , nebulisation , spray as you go or direct instillation of local anaesthetic elective tracheostomy should also be avoided as ossification of the incision site may result in airway obstruction on decannulation . our patient had a potential difficult airway ( restricted mo and neck movements ) , was uncooperative and the only fob in our setup was not working . anterior larynx with reduced mo invariably requires excessive force during conventional laryngoscopy , which may be disastrous in a patient with fop . successful use of glidescope has been mentioned in one case for nasotracheal intubation of an adult fop patient ( having a normal mo ) in a case series of 30 patients , but use of vl has never been described for a child . anaesthetic management for fop is extremely challenging , and clinical interventions that may put patient at a risk of new localisation of heterotopic ossification leading to substantial impact on their quality of life need to be diligently avoided . vl assisted with bougie may be considered as an alternative to fob for airway management of these patients .

stone man syndrome or fibrodysplasia ossificans progressiva ( fop ) is an extremely rare ( 1 in 2 million ) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton . surgery and anaesthesia - induced trauma can lead to disease flare - up if due precautions are not taken and disable the patient further . however , rarity of the disease may lead to its common misdiagnosis and anaesthesiologist may be caught unaware . there is relative paucity of literature regarding anaesthetic management of children with fop . videolaryngoscopes ( vls ) provide a non - line - of - sight view and require less anterior force to visualise the glottis , may provide an alternative to fibreoptic intubation for airway management in such cases . use of vl has only been reported once in an adult with fop for nasotracheal intubation . we describe the successful anaesthetic management of an 11-year - old child with fop and anticipated difficult airway .

paget 's disease of bone is a chronic bone remodeling disorder featuring one or more areas of aggressive osteoclast - mediated bone resorption , followed by imperfect osteoblast - mediated bone repair . the disease is frequently recognized because of elevation in serum alkaline phosphatase levels , which reflects both the extent and the activity of the disease . however , alkaline phosphatase levels can be normal when there is only a small focus of paget 's disease or after successful medical treatment . we report one such case that was incidentally detected during evaluation of nephrolithiasis and polyarthralgia and had normal radiographs and normal serum alkaline phosphatase levels . a 46-year - old man , driver by occupation , presented with a history of sudden onset pain abdomen , which was colicky in nature and localized to the right lumbar region . there was no history of dysurea , hematuria , fever , loose stools , or vomiting . he also had a history of multiple joint pains for the last 4 months , aggravated for the past 1 week . there was no history of fever , weight loss , skin rash , loss of appetite , or backache . there was no past history of diabetes , hypertension , tuberculosis , polyarthritis , or any other significant illness . his blood urea , creatinine , lft , electrolytes , and sugar levels were normal . abdominal ultrasound revealed bilateral renal calculi with gross pelvicalyceal dilatation of right kidney and right hydroureter . renal pyelogram showed non - functioning right kidney with multiple left - sided renal calculi . tc99 m diethylene triamine pentaacetic acid ( dtpa ) renogram revealed normal functioning and draining left kidney with a gfr of 55 ml / min and poorly functioning right kidney with a gfr of 10 ml / min . the patient underwent ureteroscopy with intra - corporeal lithotripsy of right kidney and bilateral dj stenting . he had normal esr , negative crp , negative rheumatoid factor , serum ionised calcium 4.4 mg / dl , and serum alkaline phosphatase 86 iu / ml . whole - body bone scan done revealed extremely hot focus involving the right humerus ( head and proximal shaft ) and left hemipelvis - features , suggestive of paget 's disease [ figure 1 ] . x - rays of right humerus and pelvis did not reveal any abnormality [ figure 2 ] . magnetic resonance imaging ( mri ) showed thickened cortex with altered signal intensity involving the right humerus head and proximal shaft and left iliac bone , commensurate with the bone scan findings [ figures 3 and 4 ] . a bone biopsy from left iliac crest was done , which showed irregular bony trabeculae lined by plump osteoblasts , with multinucleate osteoclasts and increase in cement lines [ figure 5 ] . serum calcium was 9 mg / dl and pth level was 12 pg / ml , both normal . hence , a diagnosis of paget 's disease was made and the patient was started on biphosphonate therapy . bone scan done using tc-99 m labeled mdp - showing extremely high osteoblastic activity in the right humerus and left pelvic bone ( as marked ) x - rays of right arm and pelvis showing no abnormality in bones mri right arm showing thickened cortex with altered signal intensity involving the right humerus head and proximal shaft mri pelvis showing thickened cortex with altered signal intensity involving left iliac bone bone biopsy from left iliac crest shows plump osteoblasts ( black arrow ) and increase in cement lines ( white arrow ) bone expansion may also cause various neurological compression syndromes , but most patients with paget 's disease are discovered because of an increased alkaline phosphatase level or an incidental x - ray finding and are asymptomatic . in our case , bone scan was done for polyarthralgia that showed the typical pattern of paget 's later confirmed by bone biopsy . paget 's disease is known to be associated with aortic valve , endocardial , and arterial calcification . the patient was also found to have depression and was started on treatment for the same . skeletal scintigraphy has had a major impact in diagnosis of paget 's disease because of the high sensitivity of the technique for detection of both increased vascularity and increased osteoblastic activity characteristic of the disorder . radiography has much inferior sensitivity and bony pagetic changes may be missed by it , as in our case . lesions become visible on x - rays when more than 30 - 50% of the bone has been resorbed . , in his study found that 73% of patients with positive bone scan and normal radiographs were symptomatic . the scintigraphic appearances are fairly characteristic , showing intense accumulation of radiopharmaceutical throughout the affected part with uniform distribution . pagetic lesions in long bones appear at the articular margin , progressing along the shaft , and producing a sharp v - shaped advancing edge like a flame , as was seen in our case in the humerus . however , scintigraphy is not totally specific , as the differential diagnosis can be metastasis , metabolic bone disease , and even fibrous dysplasia if detected in later age group . preservation or enhancement of normal bony anatomy is typical and differentiates paget 's from other scan abnormalities . also , there is a clear distinction between normal and abnormal bone differentiating it from metabolic bone disease . in fibrous dysplasia , there is a lack of preservation of bony outline unlike paget 's . metastatic prostate cancer and rarely breast cancer can mimic paget 's disease . the pelvis and the spine clinical manifestations of paget 's disease in which pharmacologic therapy is recommended are symptoms resulting from active bone lesions like bone pain , headache with skull involvement , back pain due to pagetic radiculopathy or arthropathy , other neurologic syndromes , and fissure fractures . follow - up of these cases and monitoring the treatment response can be done by serum levels of biochemical markers like alkaline phosphatase . patients with mono - ostotic disease and limited polyostotic disease may have normal levels of these markers . in such patients , scintigraphy is advocated to monitor disease status and treatment response . in our case , despite polyostotic form , the biochemical markers were normal and hence bone scan will be utilized to follow - up the patient . in fact , it has been suggested that scintigraphy may better assess long - term response , since scintigraphic activity may persist after biochemical normalization . this case highlights the role of bone scintigraphy in detecting an unusual presentation of paget 's disease in the form of nephrolithiasis , polyarthralgia , normal radiographs , and normal biochemical markers despite being in the polyostotic form .

paget 's disease of bone is a chronic bone remodeling disorder . although most patients are asymptomatic , a variety of symptoms and complications may develop directly from bone involvement or secondarily due to compression by the expanded bone . it is usually diagnosed from radiological and biochemical abnormalities or in advanced cases it becomes clinically evident due to the expanded bone . we report a case of paget 's disease which was detected incidentally during evaluation of nephrolithiasis and polyarthritis but had normal radiographs and normal biochemical markers .

we report a case of delayed presentation of bilateral trigger thumb along with a brief review of past literature . a six year old girl presented with fixed flexion deformity of interphalangeal joints of both thumbs and notta s nodules . but we found another constricting annular pulley just distal to a1 . only after splitting the distal pulley , we could get complete extension of interphalangeal joints . at two years follow - up splitting of a1 pulley alone may not be sufficient in few cases of trigger thumb which may require distal release too trigger thumb is due to stenosing tenosynovitis of flexor pollicis longus at the level of first annular pulley . the tendon also shows corresponding thickening called notta s nodule just proximal to the stenosis in the sheath . because of these , child develops triggering , pain and later flexion deformity of interphalangeal ( ip ) jointof the thumb . if these are neglected , child may develop metacarpophalngeal joint ( mcp ) laxity with hyperextension deformity . if the joint laxity is not diagnosed and corrected , it will be exacerbated post - operatively . conservative treatment is tried between 0 - 3 yrs , with observation alone or with stretching exercises and splints . surgery is recommended if the child presents after three years of age or if conservative treatment fails . surgery is not preferred in children aged less than one year as rate of recurrence is higher with surgical release at this age . release of a1 pulley alone is sufficient to relieve the trigger thumb in most of the patients . but in some children , another constricting annular pulley ( av ) may be present just distal to a1 . it should be identified and split to attain complete extension of the ip joint . we present a case of bilateral trigger thumb in a child of six years in whom splitting of a1and av pulleys is required to release the constriction . a six year old female child was brought by her parents with flexion deformity of both the thumbs . the deformity was noticed when the child was six months old , but the finger could be extended to the neutral position at that time . since one year , extension has not been possible and the finger becomes painful if it is tried . there was no history of trigger thumb in maternal or paternal families . on examination , a 2 * 2 cm tender nodule was found at the crease of mcp joint . there was no triggering , but minimal extension at ip joint was possible when thumb was flexed at mcp joint . figs 1 , 2 - the child was diagnosed of having bilateral trigger thumb . surgical management was decided upon . under general anaesthesia , with tourniquet applied to the arm fig 3 - complete active extension of ip joint was possible post - operatively , but the joint tended to be in flexed position . a removable extension splint was given and the patient was asked to do active finger movements atleast thrice a day after removing the splint . figs 4,5 - after two months of physiotherapy , the patient had following movements : pre - operative flexion deformity of both thumbs stenosis of the flexor sheath . patient has minimal restriction of ip joint flexion of right thumb and minimal mcp joint hyperextension of left thumb . the child is followed for two years during which she did not develop any complication . the term congenital trigger thumb is thought to be a misnomer by manysurgeons , as the condition is almost never seen at birth . since it develops later , they termed it as developmental trigger thumb. but trigger thumb is reported in twins and siblings , supporting the congenital aetiology to some extent . the stenosed sheath and notta s nodule are biopsied earlier , but no definite pathology can be established .we extension of ip joint beyond 00 is possible.many surgeons , but few advocated longitudinal incision toob - ip joint can not be extended beyond 00.gr.i - the child can actively extend his thumb with triggering , gr.ii - active extension is not possible andtriggering is observed during passive extension of ip joint.gr.iii - ip joint is in fixed flexion deformity . many surgeons , but few advocated longitudinal incision to ob - ip joint can not be extended beyond 00 . gr.i - the child can actively extend his thumb with triggering , gr.ii - active extension is not possible andtriggering is observed during passive extension of ip joint . management of trigger thumb is contrversial . in a study of the treatment of trigger thumb , r.a . lee zl et al showed passive streching exercises and splinting to be effective than observation alone . but some surgeons donot find observaion or streching exercises to be effective and they proceeded to surgery in all their patients . surgery is recommended after the child attains one year if a)conservative management for three months doesnot show any improvement.b)flexion deformity of the finger is presentc)the child is older than three yearsd)the pathology is bilateral . flexion deformity of the finger is present the child is older than three years the pathology is bilateral . since the child in our case is six years old and she has gr.iv deformity , conservative management is not tried . in long standing cases , it is corrected by advancement of mcp volar plate and temporary pinning of mp joint . if it is not identified and corrected , mcp joint laxity will be exacerabated . horizontal incision over mcp joint crease is preferred by many surgeons , but few advocated longitudinal incision to prevent neurovascular injury . but if the neurovascular structures are properly isolated and protected , the incidence of injury is found to be very less , even with the horizontal incision . but occasionally , another annular pulley ( av ) is present between a1 and oblique pulleys . it too may be stenosed and unless it is split , patients will have recurrence of trigger thumb . in our case , splitting of a1 and av pulleys bilaterally is required to get full extension of ip joint . if it is cut along with a1 , bowstringing of flexor pollicis longus will result , causing decreased ip joint flexion . even though complete active and passive extension are possible post - operatively , thumbs tended to be in flexion of approximately 20o because of long standing deformity . percutaneous release of a1 pulley is also advocated , but the risk of damaging digital nerves is high . phalangeal osteotomy may also be required if the child has flexion deformity of ip joint for 10 years or above . recurrence of trigger thumb is noted in 4% of children , especially if the child is aged less than one year . trigger thumb release in older children can be done effectively if complications are anticipated and looked for.release of a1 pulley alone may not be sufficient in every case . if the triggering is not resolved with the transection a1 pulley , other sites of constriction should be explored . trigger thumb release in older children can be done effectively if complications are anticipated and looked for . if the triggering is not resolved with the transection a1 pulley , other sites of constriction should be explored . delineation of anatomy of pulleys is important in acase of trigger thumb to get complete release and to prevent bowstringing of flexor pollicis longus .

introduction : congenital trigger thumb is an uncommon anomaly of children . its management is controversial , ranging from observation to extensive surgical release . we report a case of delayed presentation of bilateral trigger thumb along with a brief review of past literature.case report : a six year old girl presented with fixed flexion deformity of interphalangeal joints of both thumbs and notta s nodules . it is diagnosed as trigger thumb and release of bilateral a1pulleys is done . but we found another constricting annular pulley just distal to a1 . only after splitting the distal pulley , we could get complete extension of interphalangeal joints . at two years follow - up , the child is free of complications.conclusion:splitting of a1 pulley alone may not be sufficient in few cases of trigger thumb which may require distal release too

most case reports are of ingestion of sharp objects that can be retrieved by indirect laryngoscopy or flexible endoscopy in the majority of cases . foreign bodies have been found to migrate to the pancreas from the gastrointestinal ( gi ) tract . presentation varies widely from a 45-year - old man who was asymptomatic by clinical and laboratory data , to a 39-year - old man with pancreatitis , gastric varices , and splenic artery pseudoaneurysm , to a 60-year - old female who presented with what appeared to be locally advanced pancreatic carcinoma . also , reports exist of foreign body migration to the liver as well as into the pancreas . there are isolated reports of complications of fish bone ingestion including perforation to the pharynx , esophagus , stomach , small intestine , meckel 's diverticulum , and colon . there have been published reports of laparoscopic removal of foreign objects , such as sewing needles from a pelvic cul - de - sac , intrauterine devices , and a broken intraperitoneal catheter . however , only one report has been made of laparoscopic removal of fish bone from the head of the pancreas . we report the successful laparoscopic removal of a piece of wire that had perforated the stomach and migrated into the head of the pancreas , resulting in a peripancreatic abscess . a 44-year - old man presented to our hospital on september 2 , 2005 with epigastric pain . his white blood cell ( wbc ) count was 16.1 ; hemoglobin ( hgb ) , 14 ; amylase 119 ; lipase 190 ; his urinalysis ( ua ) was negative . a computed tomographic ( ct ) scan of the abdomen and pelvis was performed , and the patient was given the preliminary diagnosis of diverticulitis . . however , the next day , the formal ct scan read showed that he had a foreign body in the gastric body with a peripancreatic abscess ( figure 1 ) . he was admitted to the hospital and reported continued epigastric pain but did not have nausea , vomiting , melena , hematemesis , or recollection of ingesting a foreign body . a gastroenterology consult was obtained , and an attempted esophagogastroduodenoscopy with endoscopic ultrasound for guidance was performed to remove the foreign body . this was unsuccessful , and the patient agreed to undergo a laparoscopic removal of the pancreatic foreign body . a hyperdense foreign body is present in the posterior wall of the stomach migrating into the head of the pancreas ( black arrow ) . we approached the foreign body and abscess cavity by gaining entrance to the lesser sac through the gastrocolic ligament . the posterior antrum of the stomach was dissected off the head of the pancreas , until a small abscess cavity was found near the head of the pancreas . this abscess was entered , and a small amount of pus was irrigated from the cavity . further dissection revealed a small black wire that was removed with a maryland grasper ( figure 2 ) . we presumed that a gastric perforation was present in this area , so dilute methylene blue solution was instilled into the stomach , which failed to detect a perforation . an omental flap was raised and brought up in a retrogastric manner and secured with a single intracorporeal suture . the patient 's amylase and lipase peaked to 341 and 375 , respectively , on postoperative day 1 , but went down to 81 and 57 on postoperative day 3 . after tolerating a diet , the patient 's drain was removed , and he was discharged home on postoperative day 3 . at 2-week follow - up , he was symptom free . previous reports suggest that if the patient has no symptoms , then they can be observed and followed by repeated clinical examinations and plain abdominal films . however , if the patient becomes symptomatic or has disturbances in the gi tract , then further intervention is warranted . most case reports are of patients who have psychiatric illness , developmental immaturity , altered level of consciousness , or who ingest high - risk foods ; most patients remember what they ingested . when the foreign body has sharp ends at one or both ends , the risk of perforation increases . the site of perforation occurs at points of narrowing or angulation in the gi tract , such as the cricopharyngeal ring , aortic arch , lower esophageal sphincter , pylorus , duodenal curve , ligament of treitz , iliocecal valve , appendix , or the rectosigmoid junction . many case reports are of penetration into the pancreas , which suggests that the narrowing of the pylorus may be the mechanism by which foreign objects penetrate into the pancreas . there have been reports of foreign body ingestion with subsequent removal through open techniques ; however , only one other report exists of laparoscopic removal of a foreign body from the pancreas . the smaller abdominal incisions result in decreased risk of infection and dehiscence , less postoperative pain , and faster recovery time . many abdominal and pelvic surgeries , such as adrenalectomies , colectomies , esophagectomies , and hysterectomies , are now being performed laparoscopically . some argue that visualization for certain procedures , such as the nissen fundoplication , is made easier with the laparoscopic technique . for removal of foreign bodies , the magnified view from the laparoscope aids in visualization of small structures , and the reflected light can aid in differentiation between metallic foreign bodies and the surrounding tissues . in our case , we were able to visualize and enter the lesser sac with careful dissection to locate and remove the wire in the pancreas . since many foreign bodies migrate to the pancreas , a laparoscopic approach may be beneficial over open procedures because it allows the surgeon to approach the lesser sac with minimal manipulation of surrounding tissues while being aided by optimal magnification and illumination . our successful laparoscopic retrieval of a pancreatic foreign body provides a basis for further innovative thinking for complicated surgical problems , and emphasizes the need to incorporate minimally invasive techniques into the practice of all surgeons .

we present a case report of a laparoscopically retrieved foreign body that migrated into the pancreas . the patient is a 44-year - old man who presented with epigastric pain and was subsequently found by computed tomographic scan to have a foreign body in the head of the pancreas . after attempted endoscopic retrieval , we successfully removed the foreign body laparoscopically , thus avoiding laparotomy . laparoscopy is an effective tool for pancreatic exploration .

meigs ' syndrome is characterized by ascites and pleural effusion associated with benign ovarian tumors such as fibroma , thecoma , brenner 's tumor , or granulosa cell tumor [ 1 - 3 ] . pseudo - meigs ' syndrome is defined as a syndrome which shows similar symptoms to meigs ' syndrome in patients with malignant ovarian tumors . however , pseudo - meigs ' syndrome from breast cancer is extremely rare and very poor prognoses after clinical manifestation of pseudo - meigs ' syndrome have been reported . breast cancer often develops pleural metastases , especially in the ipsilateral pleura , resulting in pleural effusion . pleural effusion in the ipsilateral thorax caused by pseudo - meigs ' syndrome can thus mislead physicians and complicate treatment decisions . we report here a very rare case of pseudo - meigs ' syndrome originated from breast cancer , which was treated successfully with oophorectomy . a 54-year - old woman complained of abdominal fullness in february 2009 . she had undergone breast - conserving surgery and axillary lymph node dissection for left breast cancer in october 2000 , and thoracic drainage with intrathoracic administration of ok-432 had failed to control the pleural effusion , which contained no cancer cells . the patient experienced variable amounts of pleural effusion and was followed on an outpatient basis for the next 30 months . the patient then developed abdominal distention with elevated serum levels of carcinoembryonic antigen ( cea ) and carbohydrate antigen ( ca ) 15 - 3 ( figure 2 ) . positron emission tomography / computed tomography ( pet / ct ) showed bilateral ovarian tumors ( figure 3 ) with ascites . systemic chemotherapy ( weekly paclitaxel 80 mg / m ) and intra - abdominal chemotherapy ( carboplatin 100 mg / m ) with repeated aspirations ( 4,000 - 8,500 ml ) failed to control the ascites . bilateral oophorectomies were performed to provide a definitive diagnosis of the ovarian tumors and to establish some therapeutic clues for controlling the ascites . the right and left ovaries were weighed 15 and 250 g ( left tumor ) , respectively and were lobulated . no tumor dissemination was observed in the abdomen and the ascites contained no cancer cells cytologically . pathological examination showed that the tumors were composed of solid and luminal structures ( figure 4a ) and were positive for estrogen receptor ( figure 4b ) , progesterone receptor , cytokeratin ( ck)-7 , and gross cystic disease fluid protein-15 ( figure 4c ) , and negative for her2 overexpression and ck20 . these pathological findings suggested that the bilateral ovarian tumors originated from the patient 's breast cancer . the patient was discharged 9 days after surgery . the left pleural effusion disappeared ( figure 1b ) and serum cea and ca 15 - 3 levels decreased below the normal upper limit ( figure 2 ) . the patient remained well under aromatase inhibitor therapy with no further recurrence for 40 months after the oophorectomies . meigs himself suggested that hard ovarian tumors could irritate the peritoneum and stimulate the production of peritoneal fluid . suggested that inflammatory cytokines such as interleukin ( il)-6 , il-1 , and il-8 play an important role in the formation of ascites and pleural effusion in meigs ' syndrome . the most common etiology of pleural effusion is thought to involve facilitated migration of ascites to the thoracic cavity via the trans - diaphragmatic lymphatic system . however , because the onset of pleural effusion preceded the onset of ascites , we were unable to confirm the etiology of the pleural effusion in the present case . reported overall survival rates in patients with ovarian metastases of 42% , 25% , and 13% at 6 months , 1 year , and 2 years , respectively , but these survival rates were improved to 75% , 63% , and 25% in patients without extraovarian metastases after oophorectomies . patients with ovarian metastases often experience a reduction or complete disappearance of ascites and pleural effusion after oophorectomy , leading to an improved quality of life . several reports have suggested that intra - abdominal injection of anticancer drugs might provide effective treatment for ovarian cancer and peritoneal dissemination . the vast majority of patients with metastatic ovarian cancer develop intra - abdominal dissemination after oophorectomy , which supports the use of intra - abdominal chemotherapy with oophorectomy when possible . however , systemic and intra - abdominal chemotherapy failed to control the ascites before oophorectomy in the present patient , and we were therefore unable to draw any conclusions about the clinical importance of intra - abdominal chemotherapy . breast cancer often develops metastases to the lung , bone , liver , and brain , but the ovary is not a common target organ for breast cancer metastasis ; however , breast cancer is the leading cause of ovarian metastasis from extra - abdominal malignancies . unfortunately , the presence of ipsilateral pleural effusion in this case was misinterpreted as being caused by pleural metastasis from breast cancer , despite the lack of distinct pleural nodules on ct . it seems appropriate to interpret the pleural effusion and ascites in this case caused by pseudo - meigs ' syndrome because of the absence of malignant cells both in the pleural effusion and the ascites . metastatic breast cancers are usually treated according to hortobagyi 's algorithm , which aims for long - term symptom relief with possible elongation of survival , rather than for cure . although the volume of the ovarian metastasis was bulky in this case , surgical intervention to the ovaries seems to be a feasible measure under the concept of oligometastatic breast cancer because no cancer cells were detected in the pleural effusion or the ascites . in conclusion , bilateral oophorectomy led to complete remission for 40 months in a patient with ovarian metastasis from breast cancer and this is the first case of pseud - meigs ' syndrome treated successfully . breast oncologists should thus consider oophorectomy in patients with presumed pseudo - meigs ' syndrome without extraovarian metastatic foci .

a 54-year - old woman with long - lasting pleural effusion developed abdominal distention due to ascites from bilateral ovarian tumors . the patient had undergone breast - conserving surgery and axillary lymph node dissection for left breast cancer in october 2000 , and had developed left pleural effusion in july 2006 . cytological examination of the pleural effusion found no malignant cells . thoracic drainage with intrathoracic administration of ok-432 ( picibanil ) had failed to control the pleural effusion . positron emission tomography taken at the abdominal distention showed bilateral ovarian tumors . after failure to control the ascites with systemic and intra - abdominal chemotherapy , bilateral oophorectomy resulted in normalization of elevated serum tumor - marker levels and the disappearance of both the ascites and pleural effusions ( i.e. , pseudo - meigs ' syndrome ) . pathological examination showed the tumors to be estrogen receptor - positive metastatic ovarian tumors from her breast cancer . the patient remained well with no further recurrence for 40 months under aromatase inhibitor therapy .

we investigated a tbe outbreak , comprising 6 cases , in a mountain region in western austria in july 2008 . the index case occurred in a 43-year - old shepherd who had stayed for 24 days at his alpine pasture ( 1,564 m above sea level ) before he was hospitalized for nonbacterial urethritis and nonspecific influenza - like symptoms ( including pain in the lower abdomen and legs ) , followed by clinical signs of meningitis . tbev infection was confirmed serologically by elisa demonstration of specific immunoglobulin ( ig ) m and igg in serum and cerebrospinal fluid . the patient did not remember a tick bite but had eaten self - made cheese prepared from a mixture of nonpasteurized goat milk and cow milk 811 days before illness onset ; further investigation found 6 additional persons who had eaten the same cheese ( figure ) . for 5 of them , recent tbev infection was serologically proven ( table ) . for 3 of these persons ( 2 men , 44 and 65 years of age ; and 1 woman , 60 years of age ) , similar to the index patient , a typical biphasic course and symptoms of tbe ( nonspecific flu - like symptoms followed by fever , cephalea , meningism , and ataxia after 410 days ) developed and they were hospitalized . the 2 other persons who had eaten the cheese ( female , 37 and 7 years of age ) were clinically asymptomatic . the noninfected person had vomited shortly after eating the cheese because of a gastric banding . time course and series of events of a tick - borne encephalitis ( tbe ) outbreak from cheese made with goat milk . week 0 , transport of goat to high altitude ; , onset of disease ; o i , hospitalization period ; tbev , tick - borne encephalitis virus ; me , meningoencephalitis . * tbev , tick - borne encephalitis virus ; nt , neutralization test ; csf , cerebrospinal fluid ; ig , immunoglobulin ; me , meningioencephalitis ; pos , positive ; bor , borderline ; na , not applicable ; neg , negative . the cheese was prepared from a mixture of fresh milk from 1 goat and 3 cows and was eaten shortly after production . detection of tbev - specific hemagglutination inhibiting ( hi ) and neutralizing antibodies in the goat s serum proved infection in the goat ; the 3 cows were seronegative for tbev . at the time of this investigation ( 1 month after cheese production ) , tbev was already undetectable by pcr in serum and milk of the goat . cheese from the 3 batches produced after the contaminated batch was tbev negative by pcr . the 4 domestic pigs kept at the alpine pasture and fed with the whey and goat milk , however , were seropositive ( tbev hi- and neutralizing antibodies detected ) , which indicated tbev infection , but no clinical signs were observed . serum samples from 105 goats from pastures in the neighborhood also were investigated for tbev - specific antibodies ; all goats were seronegative . our analyses showed that the 6 humans and the 4 pigs were infected through the milk of 1 goat , which had been transported by car from a tbe nonendemic valley to the alp 12 days before production of the tbev - contaminated cheese . experiments have demonstrated that infected domestic animals ( i.e. , goats , sheep , and cows ) can excrete tbev into milk for 37 days , beginning as early as the second or third day postinfection ( 69 ) . in addition , although cheese was produced once or twice each week , only this 1-kg batch of cheese transmitted tbev . therefore , all the evidence indicates that the goat was infected at the alpine pasture at an altitude of 1,564 m. indeed , some ticks were collected from cows that had stayed at this altitude during the entire summer . analyses of these ticks for tbev by pcr , however , yielded only negative results . our findings provide further evidence for the expansion of tbev - endemic regions to higher altitudes in central europe . for example , longitudinal studies in the czech republic , a country with similar climatic and ecologic conditions to those of austria , showed a shift in ixodes ricinus ticks and tbev , from 700 m in 19811983 to 1,100 m altitude in 20012005 ( 10,11 ) . likewise , zeman and benes demonstrated that the maximum altitude at which tbev is found in the czech republic gradually moved upward during 19702000 , corresponding to the rise in temperature during the same period ( 12 ) . in scandinavia , a northward extension of the geographic range of i. ricinus ticks and tbev since the mid-1980s has also been recognized ( 1,1315 ) . climatic changes most likely are the major driving forces for the geographic changes in the distribution of tbev and its main vector , i. ricinus , in europe . this report also emphasizes the efficiency of oral transmission of tbev to humans and to pigs . six of the 7 persons who ate the cheese and all 4 pigs fed residual milk or whey from the same cheese became infected . given the excellent effectiveness of the tbe vaccine ( 2 ) , vaccination probably could have prevented all 6 human cases .

we report transmission of tick - borne encephalitis virus ( tbev ) in july 2008 through nonpasteurized goat milk to 6 humans and 4 domestic pigs in an alpine pasture 1,500 m above sea level . this outbreak indicates the emergence of ticks and tbev at increasing altitudes in central europe and the efficiency of oral transmission of tbev .

dna integrity is a prerequisite , not only for the fertilization of an oocyte , but also for oocyte activation and achievement of an ongoing pregnancy . it has been documented that there is a negative correlation between defective sperm chromatin structure ( dna breaks ) and fertility , in vivo and in vitro . several studies have demonstrated that disturbed dna integrity reduces fertilization and pregnancy rate during in vitro fertilisation ( ivf ) and intracytoplasmic sperm injection ( icsi ) treatment , and also increases the risk of early miscarriage . dna integrity can be assessed by the sperm chromatin structure assay ( scsa ) , by terminal deoxynucleotidyl transferase - mediated dutp nick end labelling ( tunel ) , and more recently , the sperm chromatin dispersion assay . special consideration should be made when comparing these methods , however , because they are each measuring different parameters . it is based on exposing sperm nuclei to acid then staining them with acridine orange ( the acid exposure causes denaturation of double - stranded dna in sperm with impaired chromatin structure ) . the stain will attach to the ends of the broken dna only , enabling evaluation of the proportion of single- and double - stranded dna in the sample . the sperm chromatin dispersion assay also measures the susceptibility of sperm dna to acid denaturation , but in a different way . the scsa is based on the metachromatic property of the stain acridine orange , where intact dna are characterized by a green fluorescence and disturbed dna by orange fluorescence . the evaluation is carried out by flow cytometry ; therefore a high number of cells ( about 10,000 ) can be studied . the sperm chromatin dispersion test is performed using conventional bright field microscopy and analyses dispersed dna loop halos . between 300 and 500 spermatozoa can be evaluated during this test . the tunel assay uses fluorescein - dutp to label single- and double - stranded dna breaks.[5 - 7 ] . in most recent studies , no correlation between dna integrity and conventional semen parameters has been found ; therefore , dna integrity can be considered as a sperm function that is independent from sperm count , motility , and morphology . disturbances in sperm dna integrity have been described in patients with varicoceles , testicular tumours , genital tract infections / inflammations , and patients with idiopathic infertility . disturbances of dna integrity occur predominatly at the post - testicular level , which is why a recent study suggested testicular sperm extraction with subsequent icsi as a possible treatment . research by different groups suggests that extrinsic oxidative stress , including environmental or industrial toxins or smoking , seems to be the major cause of sperm dna damage rather than apoptosis , and a recent study showed that treatment with antioxidants has positive effects on implantation and pregnancy rates after icsi . regarding further treatment options for disturbed dna integrity , it is fortunate that a considerable number of recent studies have focused on the correlation between disturbed dna integrity and genital tract inflammations and infections , respectively . it was shown that dna integrity was impaired in leukocytospermic semen samples , and in patients with less than 1 million leukocytes per ml and high levels of reactive oxygen species . in the last scenario , disturbed dna integrity may still allow oocyte fertilization and formation of the 2-pronuclei stage , but when the paternal genes are switched on at the 4-cell stage , further embryonic development stops because of oxidation and fragmentation of the paternal dna , even after blastocyst formation . another recent advance is the demonstration that dna integrity is improved after antibiotic treatment in patients infected with chlamydia trachomatis . another recent study demonstrated that , compared with men without varicocele , men with varicocele have a higher percentage of sperm cells with dna fragmentation . thus , evaluation of sperm dna fragmentation could be an important factor in deciding treatment options for men with varicocele . this is supported by further studies , including a small one in which 10 of 11 patients with varicocele showed a significant decrease in sperm dna fragmentation after varicocele repair , and an earlier , larger study of 37 patients . however , the different dna integrity tests can often draw different conclusions and sometimes even the same test can yield different results ( most probably due to low study populations ) , therefore , interpretations of test results in clinical practice have to be made with some caution . a greater quantity of larger studies is needed for validation of such tests before they are ready to be used in general clinical practice . despite these limitations , this is an interesting and promising field that may offer new tools for andrological diagnostic work - up and may finally lead to more diagnosis - related instead of symptoms - related treatment of male infertility .

male infertility can be treated by surgical procedures ( e.g. , varicocelectomy ) or by administration of drugs if causal factors ( e.g. , seminal tract infections ) are detected . in more severe cases , methods of assisted fertilization often have to be applied , but even these have only a limited success rate . recent studies have demonstrated that disturbances of sperm dna integrity ( determined by the acridine orange test ) can explain certain cases of fertilization failure and failure to achieve pregnancy following in vitro fertilisation with intracytoplasmic sperm injection . the evaluation of dna integrity should be considered when diagnosing male infertility as it has been shown to be an independent factor and can be used as a supplement to standard semen analysis . analysis of dna integrity may , therefore , provide further information about altered male fertility and lead to administration of more appropriate therapy .

this risk must also be considered for patients who are using these medications for off - label inflammatory skin conditions . we present the first patient with treatment - refractory sweet 's syndrome who developed disseminated nocardia brasilienses whilst on methylprednisolone and infliximab . a 62-year - old white male with a 10-year history of treatment - refractory sweet 's syndrome was admitted to the hospital with purpuric skin lesions . the patient had myelodysplastic disease that was under treatment with intravenous immunoglobulin ( ivig ) infusions , and also diabetes mellitus , peripheral neuropathy , chronic corticosteroid - induced adrenal suppression . as sweet 's syndrome proved refractory to dapsone , methotrexate , etanercept , lenalidomide , rituximab , cyclosporine , mycophenolate mofetil , and cyclophosphamide , methylprednisolone and infliximab infusions were administered . after the second infusion of infliximab , he presented with new purpuric , painful lesions , and subjective fever . on examination , a 6 cm indurated erythematous and purpuric plaque with overlying pustules was noted on the left flank of abdomen , with similar smaller plaques located distally over both lower extremities [ figure 1 ] . subsequently , the patient developed pulmonary involvement and was diagnosed with disseminated nocardia infection . during the course of his prolonged hospitalization , trimethoprim sulfamethoxazole ( tmp - smx ) , imipenem , minocycline , and amikacin were administered for nocardia infection . seven months later , after mistakenly receiving infliximab during a scheduled ivig infusion , the patient experienced a recurrence of nocardia infection with both cutaneous and systemic involvement . the patient was hospitalized for over 5 months in three separate hospitals and was transferred to our care with widely disseminated nocardia . multiple antibiotics were administered during his treatment , but the patient eventually succumbed to respiratory failure . indurated , erythematous , and purpuric plaque on the distal lower extremity branching , filamentous organism on gomori methenamine silver stain consistent with nocardia , 40 as more patients are being treated with immunosuppressive medications such as tnf- inhibitors , the rates of infectious complications , especially with atypical pathogens , are increasing . in the literature , most cases of tnf- inhibitor - related nocardia infections have been reported in patients with psoriasis , rheumatoid arthritis , or inflammatory bowel disease . however , patients being treated with tnf- inhibitors for off - label inflammatory skin disorders are also at risk . to our knowledge , this case represents the only nocardia infection reported in a patient being treated with a tnf- inhibitor for sweet 's syndrome . nocardia asteroides causes most cases of disseminated nocardiosis with skin involvement ; however , most cutaneous nocardiosis is caused by n. brasiliensis . clinically , cutaneous nocardiosis may present as nodules , pustules , ulcerative lesions , bullae , or abscesses . disseminated infection can occur rarely from primary cutaneous infection but more commonly causes secondary skin involvement . mortality rate for patients with central nervous system or disseminated nocardiosis has been estimated to be as high as 50% . culture is the gold - standard for diagnosis of nocardia , but it may also be identified on histologic evaluation as characteristic filamentous organisms . other antimicrobial agents that have been used successfully alone or in combination with tmp - smx include amikacin , imipenem , minocycline , tetracycline , dapsone , streptomycin , linezolid , cycloserine , amoxicillin several tnf- inhibitors , including infliximab , have been reported to be associated with nontuberculosis opportunistic infections , such as nocardia.467 it is hypothesized that infliximab 's high peak drug levels , high - affinity tnf- binding , as well as macrophage and t - cell death lead to more significant immunosuppression when compared with other tnf- inhibitors . a french ratio study demonstrated higher rates of opportunistic infection in patients on monoclonal - type tnf- inhibitors , such as infliximab , versus the soluble - type tnf inhibitor , etanercept . this study also found that one - third of these opportunistic infections were caused by bacterial pathogens , such as nocardia . infliximab binds both soluble and transmembrane tnf- , thereby inducing apoptosis of macrophages and t cells , which are essential for cell - mediated immunity . this impairment of cell - mediated immunity is implicated in the susceptibility to granulomatous infections , such as nocardiosis . in all patients on tnf- inhibitors , including patients being treated for inflammatory skin disorders , such as sweet 's syndrome , the slow - growing types of bacteria should be considered in the differential diagnosis when presenting with signs of cutaneous or systemic infection . cultures must be maintained for at least 23 weeks to detect the presence of such bacteria . it is important for dermatologists to adequately inform patients on tnf- inhibitors about the risk of opportunistic infections and to maintain a higher index of suspicion while culturing such patients .

a 62-year - old white man with a 10-year history of treatment - refractory sweet 's syndrome was admitted to the hospital with the onset of purpuric lesions . methylprednisolone and infliximab were administered . our patient developed disseminated nocardia infection and eventually succumbed . opportunistic infections such as nocardia have been associated with infliximab and other tumour necrosis factor ( tnf)- inhibitors . the astute clinician should be aware of the risk of rare opportunistic infections , particularly in patients on tnf- inhibitors and systemic corticosteroids .

even nowadays , it happens too often that an optic neuritis is misdiagnosed for something else . this is due in part to the misconception that a young woman with acute visual loss in one eye has most probably a disease of the optic nerve ; difficulty in pupil examination , incomplete collection of key points in past history , and lack of knowledge of other entities are other factors . here , we present a case of multiple evanescent white dot syndrome ( mewds ) misdiagnosed as optic neuritis . a 25-year - old female presented to a local hospital for acute onset of a central scotoma in the left visual field . the neurologist collected a visual acuity ( va ) of 20/20 in od and 10/20 in os ; pupil examination as well as optic nerve examination by direct ophthalmoscopy was normal . a retrobulbar optic neuritis was suspected so that a contrast brain magnetic resonance imaging scan was requested . her past ocular history was positive for mild myopia ( 1.75 sf ou ) ; she denied smoke , trauma , or drug consumption , but to specific questioning , the patient reported a flu - like illness 1 month before the onset of her symptoms . pupils were equal a briskly reactive to light with no relative afferent pupillary defect ( rapd ) . ishihara test was full od ( 12/12 ) but abnormal in os ( 7/12 ) ; anterior segment examination and intraocular pressure were within normal limits in ou . on fundus examination , a granular appearance of the macula [ figure 1 ] was evident in os with some residual white spots in the posterior pole [ figure 2 ] . . optical coherence tomography scan of the macula was positive for a disruption of outer retinal layers [ figure 3 ] well matching the granular appearance of retinography . autofluorescence [ figure 4 ] revealed a major number of white spots as compared to fundus examination . granular appearance of the macula is pathognomonic of the condition retinography of the left eye . arrows indicate residual white dots optical coherence tomography scan of the fovea outer segment shows disruption at the inner segment / outer segment junction of photoreceptor layer in the macula posterior pole autofluorescence outer segment shows multiple white dots concentrated in the peripapillary area mewds is an uncommon entity first described in 1984 by jampol et al . as a unilateral va loss notable for white dots of the the retinal pigment epithelium ( rpe ) or deep retina and a granularity of the fovea . an infectious etiology is thought to be involved in the pathogenesis of mewds since frequently , but not always , there is a viral prodrome . the white dot syndromes produce yellow - white retinal lesions located at the rpe or outer retina level and are found primarily in young patients . within this diagnostic group are mewds , acute posterior multifocal placoid pigment epitheliopathy , multifocal choroiditis and panuveitis , punctate inner choroiditis , birdshot retinopathy , acute zonal occult outer retinopathy , and serpiginous choroidopathy . since mewds is unilateral , causes visual loss , and occurs predominantly in young women , it may be confused with optic neuritis . key points in the differential diagnosis are : lack of eye pain exacerbated by eye movements in mewds as compared to optic neuritispredominant color vision and contrast loss as compared to va in optic neuritisan rapd is evident in optic neuritis ( some trace can be seen in mewds but uncommon)chief complaints of patients : blurred vision and color vision impairment in optic neuritis , delineated central / paracentral scotoma in mewdsthe characteristic features of mewds are multiple retinal white dots and foveal granularity on fundus examination . lack of eye pain exacerbated by eye movements in mewds as compared to optic neuritis predominant color vision and contrast loss as compared to va in optic neuritis an rapd is evident in optic neuritis ( some trace can be seen in mewds but uncommon ) chief complaints of patients : blurred vision and color vision impairment in optic neuritis , delineated central / paracentral scotoma in mewds the characteristic features of mewds are multiple retinal white dots and foveal granularity on fundus examination . the condition is usually self - limiting , with excellent recovery . rarely , it recurs or is associated with complications ( e.g. , choroidal neovascularization ) . the neurologist should be aware of this condition , and in doubt , schedule the patient for a detailed ophthalmic examination after pupil dilatation .

a 25-year - old female presented to a local hospital for acute onset of a central scotoma in the left visual field . she was visited by the neurologist , and a diagnosis of left retrobulbar optic neuritis was made . magnetic resonance imaging scan was normal . ophthalmic examination revealed a multiple evanescent white dot syndrome . after a description of the case , a brief differential diagnosis between these two entities is made . the neurologist should be aware of this uncommon condition .

xeroderma pigmentosum ( xp ) is a rare autosomal recessive disorder characterized by sun sensitivity , photophobia , early onset of freckling , and subsequent neoplastic changes on sun - exposed skin . seven xp complementation groups ( xpa to xpg ) , as well as variant ( xpv ) , have been revealed . xpa through xpg group represents defective in ner , which is responsible for the removal of uv - induced damage in genomic dna . the xpv ( omim 278750 ) is caused by defects in the post replication repair machinery while ner is not impaired . xpv patients may display sunlight - induced pigmentation changes and a highly elevated incidence of skin malignancies , but rarely have neurological abnormalities1,2 . the polh gene ( nm_006502.2 ) , located on chromosome 6p21.1 - 6p12.3 , has 11 exons spread over encodes a y - family dna polymerase eta ( pol , np_006493 ) that enables the cells to synthesize the correct daughter strands for translesion synthesis , despite the presence of thymine dimmers , on to the uv - damaged template dna3 . in the present study , we reported a chinese family with xpv phenotype and identified a novel nonsense mutation in polh- that may lead to xpv . a 27-year - old man was admitted due to an unusually increased number of freckle - like pigmentation in sun - exposed areas , occurred when he was 4-month old . however , it was noted that he was born to consanguineous healthy parents and his parents were first cousins ( fig . we collected the data of the patient 's clinical history , clinical findings and laboratory results . a skin biopsy from facial lesion peripheral blood samples were collected from the affected patient and the unaffected family members , including his parents and brother , as well as 100 healthy controls . genomic dna was extracted using dna extraction kit ( omega , madison wi , usa ) . the dna samples were delivered to invitrogen company ( shanghai , china ) , then were amplified by polymerase chain reaction ( pcr ) and sequenced by abi prism 3730 automated sequencer . to confirm whether genetic variants in genes involved in xp may be responsible for the affected family , we sequenced xeroderma pigmentosum , complementation group c ( xpc ) , damage - specific dna binding protein 2 ( ddb2 ) , excision repair cross - complementing rodent repair deficiency , complementation group 4 ( ercc4 ) and polh that lead to xpc , xpd , xpf and xpv , which have no neurological involvement . the study was approved by the ethical committee of the first affiliated hospital , college of medicine , zhejiang university . on examination , the patient showed diffuse cutaneous pigmentation and atrophy on the face and scattered small darken freckles on the nose , zygoma , neck and forearms . no basal cell carcinoma ( bcc ) , squamous cell carcinoma ( scc ) or melanoma lesions were seen in the affected areas ( fig . a skin biopsy of facial lesion showed hyperkeratosis , focal parakeratosis , and atypic keratinocytes with increased melanin granules , abnormally large nuclei and disorder to some degrees . epidermal ridges elongated as bud - like and extended into the dermis . some disseminated melanophages in the superficial layer of dermis and a mild perivascular mononuclear infiltration were observed ( fig . a homozygous c.67c > t mutation in the exon 2 of polh was noted in the affected patient ( fig . 2b and c ) , while the genotype of the unaffected sibling is cc ( fig . in addition , we screened 100 unrelated control subjects and none had this mutation ( data not shown ) , indicating that this mutation was not a common polymorphism but a novel pathological mutation of polh in the affected family . we sequenced other causal genes implicated in other xp groups , including the xeroderma pigmentosum , complementation group c ( xpc / xpc ) , damage - specific dna binding protein 2 ( ddb2/xpd ) and excision repair cross - complementing rodent repair deficiency , complementation group 4 ( ercc4/xpf ) genes . we did not identify genetic variants in these genes that may cause xp in this affected family ( data not shown ) . allelic variants listed in omim ( http://omim.org/entry/603968 ) and reported in previous literatures have revealed some molecular defects in xpv patients from america , europe , africa and asia ( mainly from japan)1 - 10 . of these , ten are missense mutations , nine are nonsense and twenty - one represent frameshift or large deletion . furthermore , four types of possible founder mutations , c.490g > t , c. del1661a , c.916g > t and c.725c > g , are responsible for 87% japanese patients with xpv9 . among the nonsense mutations detected previously , all of them are in exons 4 - 11 and none is from the exon 2 , which are inside the conserved region and catalytic domain of pol ( fig . 3 ) . here , a novel point mutation - c.67c > t in the exon 2 of polh , leading to a nonsense mutation ( p.gln23ter ) -was identified ( fig . 3 ) , which was not reported previously . furthermore , the different predicted protein sizes associated to each nonsense mutation in the present study and the reviewed xpv patients were shown in the fig.3 . one mechanism for the observed large reduction in pol protein would be nonsense - mediated message decay11 . an xpv cell strain with a nonsense mutation in the polh gene had no detectable pol protein levels and showed about 50% of normal polh mrna levels . thus , we inferred that , in the present case , the novel nonsense mutation of polh may result in message decay and further loss of pol protein , responsible for the high frequency and abnormal spectrum of uv - induced mutations , and ultimately their malignant transformation , which needs further in vitro and in vivo functional experiments to confirm . it was ever revealed that mutations outside the catalytic domain of pol is always associated with a very mild phenotype compared with those inside the catalytic domain regardless of the mutation type8,9 . while it was also believed by other researchers that wild variability in clinical features among xpv patients in addition to the localization of the mutation , the incidence of skin cancer in xpv patients might be related to accumulation of sun exposure , life style of a patient and other genetic determinants9 . an actinic keratosis , the most common precancer , had been observed in the present patient till now . therefore , it is important for him to improve his life style , such as sun protection and being away from caffeine . in summary , we have identified a novel nonsense mutation in polh leading to xpv in an affected family and extend the genetic mutation spectrum of xpv .

xeroderma pigmentosum - variant ( xpv ) is one type of xp , a rare autosomal recessive disorder , and caused by defects in the post replication repair machinery while nucleotide - excision repair ( ner ) is not impaired . in the present study , we reported a chinese family with xpv phenotype , which was confirmed by histopathological results . genetic variants were detected by polymerase chain reaction and exon sequencing . furthermore , the reported molecular defects in xpv patients from previous literatures were reviewed . a homozygous c.67c > t mutation in the exon 2 of dna polymerase eta ( polh ) , a novel non - sense mutation in polh , was discovered .

dystonia is a relatively common clinical condition , identified by its characteristic features : sustained muscle contractions , twisting , and abnormal postures . the term primary dystonia is used to describe dystonia where no cause could be identified ( idiopathic ) , while the term secondary is used to describe dystonia which is found to result from an identifiable cause . the clinical features of dystonia are often bizarre and can be varied as blepharospasms , aversive eye movements , trismus , mouth opening , tongue protrusion , grimacing , distortion of lips , displacement / rotation of head and neck , hyperpronation , finger / wrist posturing , opisthotonus , and other axial distortions and gait disturbances . dysarthria , dysphagia , jaw dislocation , tongue amputation , and respiratory cyanosis with stridor may follow . a cramp - like discomfort or tightness of jaw or tongue may occur , making chewing , swallowing , or speaking difficult . it produces substantial disability and from a therapeutic perspective , the available treatments are for the most part unsatisfactory . drug - induced acute dystonia ( did ) is one of the most common forms of secondary dystonia . this complication is seen with varying frequency , with varying rates being reported , based on differences in the population under study , specific drugs prescribed , and indications for the drugs being prescribed . drugs with antidopaminergic properties , such as first and second generation antipsychotics are among the agents most commonly implicated in drug - induced dystonia . less frequently , other drug classes , like anti - emetics such as metoclopramide and prochlorperazine , anticonvulsants such as phenytoin , antidepressants such as nefazodone , citalopram , escitalopram , bupropion and duloxetine , and antivertigo agents such as cinnarizine and flunarizine are involved in this type of complication . the dystonia can occur immediately after ingestion of a single dose , over the course of several days of therapeutic dose administration , after dose increase , or as a manifestation of overdosage . most cases occur in the first 72 h of medication use , often with dramatic symptoms , especially in children . patients at higher risk for did include those with younger age , of male gender , a previous history of similar reactions , recent use of cocaine , comorbidity with mood disorders , and metabolic abnormalities ( hypokalemia , dehydration , hypoparathyroidism , etc . , we discuss the case of an adolescent , who developed dystonia at therapeutic doses of carbamazepine ( cbz ) . a 16-year - old male with moderate mental retardation presented to our clinic with 2 years history of multiple episodes of chewing movements accompanied by a state of altered consciousness , during which he would not respond to his name being called out and would continue to give a vacant stare in one direction . there was no history of unconsciousness , repeated rhythmic movements of the limbs , loss of tone resulting in falls , tongue bite , and urinary incontinence . each episode was abrupt in onset and lasted about 510 min , after which the patient would spontaneously become responsive to the surroundings once again . a clinical impression of complex partial seizure was made based on the semiology , and the patient was started on cbz 200 mg twice daily ( 8 mg / kg / day ) for the same . the patient was brought to the emergency department 4 days later with acute dystonia of the neck which had started approximately 2 h back . his neck was found to be held firmly in an extended position with the head tilted toward the right . he had been fully conscious , oriented , and responsive to external stimuli throughout this period , though he was noticed to be agitated . he was administered promethazine 50 mg intramuscularly with the dual purpose of achieving sedation , as well as using its anticholinergic properties to relieve the dystonia . a serum cbz level estimation was done , and levels of 8 mcg / ml were obtained . since it was within the normal range ( 512 mcg / ml ) , it was considered unlikely that the cbz toxicity could have led to the dystonia . a literature review also confirmed that there was very little likelihood of the dystonia resulting from the use of cbz , especially below toxic levels . it was considered that cbz could be the offending agent , responsible for the episodes of dystonia . thus , it was decided to stop cbz and shift the patient over to another anticonvulsant drug . the patient was shifted over to sodium valproate at 1200 mg / day ( 24 mg / kg / day ) for seizure prophylaxis and was followed up for a 2-week period during which the patient remained seizure free , and no recurrence of dystonia was found . cbz is a drug that is widely used all over the world for epilepsy , bipolar disorder , and trigeminal neuralgia , apart from off - label indications such as restless legs syndrome . while stabilization of sodium channels is believed to be the mechanism behind its antiepileptic properties , researchers have been looking into a possible action at the level of the dopaminergic system too . scientific literature on the connection between cbz and dopamine blockade , however , is far from conclusive at this point . animal studies have shown cbzs ability to increase acetylcholine in striatum and inhibit dopamine transmission , with the latter being observed particularly at subtherapeutic levels , an effect which wanes as the concentration of cbz increases in the blood , thus indicating the possibility of dystonias resulting from the administration of cbz , at subtherapeutic blood concentrations . other literature points toward cbz causing a dyskinetic syndrome only in toxic doses , due to dopamine blockage . other studies , exploring the antipsychotic effects of cbz , have shed some light on its role in dopaminergic transmission , with indications that the antipsychotic effects of cbz might result from reduced sensitivity of presynaptic dopamine receptors . indeed , presynaptic effects after administration of cbz showed reduced synthesis of dopamine and a reduced rate of disappearance of dopamine after tyrosine hydroxylase inhibition , indicating a reduction in turnover . researchers have also used this basis to explain how cbz causes a slight increase in prolactin levels , similar to neuroleptics which act by blocking d2 receptors . rare instances of dystonias associated with cbz have been reported in literature , but this has been largely confined to patients with overdoses / toxic blood levels or drug combinations . literature regarding this happening at therapeutic levels is scant , especially in the case of adolescents . in this case , the patient developed dystonia at therapeutic levels of cbz , with the adverse drug reaction corresponding to a level of definite causality on the naranjo et al . adverse drug reaction probability scale and of certain causality based on the world health organization collaborating centre for international drug monitoring , the uppsala monitoring centre scale . however , we wish to caution the readers that findings from a single case should not be generalized , in spite of the high probability of causality being indicated by the scale used . this case merely serves to indicate the possibility of cbz causing dystonia at blood levels within the therapeutic range , with the aim to usher in further research in this direction .

dystonia is sustained muscle contraction , which may be primary or secondary to other causes . drugs comprise one of the most important causes for the secondary dystonia , the usual mechanism being a dopaminergic blockade . there are very few reports describing dystonia resulting from carbamazepine ( cbz ) administration . in this case report , a 16-year - old male with mental retardation and seizure disorder developed dystonia at therapeutic blood levels of cbz .

although surgery is recommended for ptc , there is no consensus on the therapy when a thyroidectomy is contraindicated because of systemic disease such as heart failure or chronic obstructive pulmonary disease . hypertrophic cardiomyopathy is a common genetic cardiovascular disease with a high risk of sudden death and disability ( 1 ) . given the potential devastating perioperative cardiac complications , hypertrophic cardiomyopathy is generally a contraindication for anesthesia and surgery ( 2 ) . radiofrequency ablation ( rfa ) has been used widely in the treatment of solid cancers ( 3 ) . herein , we presented a case of ptc in a patient with hypertrophic cardiomyopathy treated with rfa . neck ultrasound performed for a 52-year - old female revealed a predominantly solid nodule ( 0.9 0.7 0.5 cm ) with marked hypoechogenicty , microcalcification , moderate vascularity , and spiculated margin in the right thyroid gland . total thyroxine ( tt4 ) and total triiodothyronine ( tt3 ) were 109.60 nmol / l ( 55.47161.25 nmol / l ) and 1.16 nmol / l ( 1.022.96 nmol / l ) ; free thyroxine ( ft4 ) and free triiodothyronine ( ft3 ) were 14.63 pmol / l ( 10.4524.38 pmol / l ) and 4.36 pmol / l ( 2.776.31 pmol / l ) , respectively . thyroid - stimulating hormone ( tsh ) and thyroid peroxidase antibody ( tpo - ab ) were 1.58 miu / l ( 0.3804.340 miu / l ) and 852.0 iu / ml ( 0100 iu / ml ) , respectively . seven years earlier , the patient had been diagnosed with hypertrophic cardiomyopathy and a ddd cardiac pacemaker was implanted . a cardiac ultrasound showed diffuse thickening of the ventricular walls and the left ventricular ejection fraction was 60% . the n - terminal pro - brain natriuretic peptide was 1048 pg / ml ( 0100 pg / ml ) . core needle biopsy ( 18 g needle ) confirmed the diagnosis of ptc ( fig . the patient was considered unable to tolerate a thyroidectomy , so instead rfa was performed . we used valleylab cool - tip rfa system ( boulder , co , usa ) , including monopolar electrode , radiofrequency generator and cool - tip pump . after sterilization and local anesthesia with 2% lidocaine , the ablation electrode was inserted into the nodule under ultrasound guidance . the ablation was started with 5 w and increased to 28 w gradually in a 1 cm active tip . it was terminated when the rfa - induced transient hyperechoic zone covered the entire carcinoma and the needle passage was also ablated when the ablation electrode was retracted . regular follow - up neck ultrasound showed no signs of recurrence or metastases after 1 , 3 , 15 , and 41 months ; and the patient had no complications of rfa ( fig . the size of ablated lesion was reduced and the echogenicity was decreased ; in addition , the tt4 and tt3 were 73.20 nmol / l ( 55.47161.25 nmol / l ) , respectively ; ft4 and ft3 were 14.02 pmol / l ( 10.4524.38 pmol / l ) and 3.76 pmol / l ( 2.776.31 tsh and tpo - ab were 0.97 miu / l ( 0.3804.340 miu / l ) and 670.70 iu / ml ( 0100 iu / ml ) ; respectively . ptc is the most common subtype of thyroid cancer , comprising > 80% malignant thyroid neoplasms . although the prognosis is excellent , the guidelines of the national comprehensive cancer network for thyroid carcinoma and the european thyroid association ( eta ) both recommend a total thyroidectomy or lobectomy plus isthmusectomy as the standard treatment for ptc ( 56 ) . a non - surgical or minimally invasive treatment is required in thyroid cancer patients who can not undergo surgery due to severe disease , such as hypertrophic cardiomyopathy . as minimally invasive therapy , rfa induces irreversible damage to tumor tissue with heat generated by high - frequency alternating electric current ( 7 ) . rfa is an established substitute for surgery for solid cancers such as hepatic cancer ( 3 ) . rfa is also used to treat benign thyroid nodules and metastatic or recurrent well - differentiated thyroid cancer with difficult reoperations ( 89 ) . the residual volume after rfa is reduced significantly in benign thyroid nodules and metastatic well - differentiated thyroid carcinoma ( 89 ) . however , whether rfa is a promising therapeutic option for papillary thyroid microcarcinoma ( ptmc ) , which defined as thyroid cancer 10 mm in diameter , is unclear . ( 10 ) proposed the moving shot technique ( mst ) for ablation of thyroid nodules by moving the electrode during the procedure . compared with fet , mst is more suitable for thyroid gland since it can minimize the risk of heat damage to surrounding critical structures such as recurrent laryngeal nerve , blood vessels and esophagus ( 11 ) . however , at the time , mst was not in use for thyroid nodules in our hospital , and may have been more suitable for this patient . eta and the american association of clinical endocrinologists recommend partial thyroidectomy and lobectomy plus isthmusectomy for ptmc without neck lymph nodes involvement respectively ( 512 ) . in our case , a calcified 0.9 0.7 0.5 cm nodule without enlarged lymph nodes was detected and core needle biopsy confirmed papillary thyroid cancer , so surgery was necessary for the patient . although surgery is the main therapy for ptmc , our case demonstrates that rfa can be a substitute in patients who can not tolerate surgery . however , neck lymph nodes metastases are quite common for thyroid cancer and central node involvement reportedly exist in 64% of ptmc cases ( 13 ) . therefore , for operable thyroid cancer , rfa should be avoided because of undetectable lymph node metastases and patients undergoing rfa as an alternative to surgery should be followed closely . signs of recurrence or metastases have not been observed on periodical follow - up neck ultrasound in our patient . however , further observation and clinical trials are needed to compare the value of rfa with surgery . rfa may be a reliable therapeutic candidate even for low - risk ptc patients without contraindication of surgery . in conclusion , to our knowledge , this is the first reported case of ptc with hypertrophic cardiomyopathy treated with rfa . despite thyroidectomy as prior therapy in our case , rfa is an alternative when surgery is not feasible . however , further long - term observation is needed to confirm the value of rfa in the treatment of thyroid cancer .

standard therapy has not been established for thyroid cancer when a thyroidectomy is contraindicated due to systemic disease . herein , we reported a patient who had hypertrophic cardiomyopathy and papillary thyroid carcinoma treated by radiofrequency ablation because of inability to tolerate a thyroidectomy . radiofrequency ablation can be used to treat thyroid cancer when surgery is not feasible , although the long - term outcome needs further observation .

a 28-year - old male visited our department with complaint of visual disturbance , hyperemia , and epiphora in his right eye , which had developed about four weeks earlier . after symptoms developed , he visited a general ophthalmologist and was prescribed 3% acyclovir ointment ( 5 times / day ) , 1% topical prednisolone acetate ( 8 times / day ) , 0.5% topical levofloxacin ( 8 times / day ) , and oral acyclovir 400 mg ( 1 time / day ) to treat herpetic stromal keratitis . his medical and familial history was unremarkable . on initial examination , his best - corrected visual acuity ( bcva ) was 20/50 in the right eye and 20/20 in the left eye . intraocular pressure was in normal range in both eyes . slit lamp examination of the right eye showed conjunctival hyperemia , localized edema of the central and inferior half of cornea with epithelial microcysts , and moderate anterior chamber ( 2 + ) inflammation . a focal mid - stromal scar at the temporal cornea was observed , which had not been in attention ( fig . 1 ) . suspecting herpetic stromal keratitis , his medication was increased to 1% topical prednisolone acetate every one hour and oral acyclovir 400 mg five times a day while maintaining the other medications . two weeks after the initial visit , his visual acuity decreased to 20/80 and corneal edema with microcysts were still observed . oral prednisolone ( 30 mg once a day ) was prescribed in addition to the other medications . after five days , anterior chamber inflammation was improv ed and his visual acuity recovered to 20/20 , but subtle corneal edema and microcysts still remained ( fig . , he maintained 20/20 vision and his ophthalmic examination showed no significant interval changes . on tapering the medication , after three months , a previously unobserved intraocular foreign body was incidentally identified in the inferior angle of anterior chamber ( fig . thorough history taking revealed that the patient had ocular trauma with a mechanical pencil 12 years earlier . under the operating microscope , the foreign body was surgically removed via corneal incision , which measured about 0.5 1.5 mm in size ( fig . the foreign body was freely movable in the chamber and there was no evidence of fibrosis or granulation reaction at the adjacent tissue . elemental analysis of the foreign body using energy dispersive x - ray spectrometer ( bruker quantax 200 ; bruker axs , berlin , germany ) and scanning electron microscope ( philips xl 30s feg ; philips , eindhoven , the netherlands ) revealed that it consisted mainly of elementary carbon , which is compatible with the component of graphite pencil lead ( fig . two weeks after the surgery , the patient 's bcva was 20/15 , and remaining corneal edema and microcysts were completely resolved ( fig . specular microscopy showed that endothelial cell density was reduced following the event , but the percentage of hexagonal cells increased after removal of the foreign body ( fig . there was no inflammatory reaction during six years of follow - up in one case , while endophthalmitis developed two days after trauma in the other case . the authors mentioned the possibility of infectious endophthalmitis or sterile endophthalmitis caused by elemental aluminum released by the retained graphite fragment . there are also cases of retained graphite in the conjunctiva simulating melanoma , and intracorneal graphite particles , which were well tolerated . in this case , the foreign body which had penetrated 12 years earlier , did not show any cellular adhesion or pigment precipitation on its surface , thus a graphite foreign body seems to be well tolerated in the anterior chamber without inducing any foreign body reactions or inciting ocular inflammation . moreover , given the response to surgical removal of the foreign body , the patient 's manifestations , such as corneal edema and inflammatory response on initial visit , should be attributed to mechanical irritation of the adjacent tissues including the iris and corneal endothelium . based on the absence of ocular symptoms for 12 years , we may assume that the foreign body had remained in a place where it did not contact the corneal endothelium . a certain event such as minor trauma could have dislodged the foreign body from the original place , and resulted in corneal edema by irritating the corneal endothelium . when a young patient presents with unilateral corneal stromal edema and anterior chamber inflammation , differential diagnosis should include glaucomatocyclitic crisis , herpes stromal keratitis , and intraocular foreign body . in this case , although the patient had a history of ocular trauma , it was 12 years earlier , and no evidence of intraocular foreign body except an old focal corneal scar was observed at the first visit . he also revealed normal intraocular pressure , and was therefore treated with anti - virals and anti - inflammatory agents to treat herpetic stromal keratitis . the proper diagnosis and treatment were delayed until the foreign body was detected through the course of follow - up examination . the anti - inflammatory effect of medication or spontaneous interaction with adjacent tissue of the foreign body would explain the moderate response to steroid treatment during follow - up . persistent corneal problems indicate that there had been intermittent mechanical contact of the foreign body with the corneal endothelium . complete resolution of corneal edema and epithelial microcysts was achieved only after surgical removal of the graphite foreign body from the anterior chamber . careful history taking and meticulous slit lamp examination would have led to the proper management earlier . this case showed that localized corneal stromal edema or endothelial dysfunction can be caused by an intraocular foreign body , even with history of trauma that occurred much earlier . a physician should always arouse suspicion and perform a thorough examination , including gonioscopy , in such patients .

we report a case of a retained graphite anterior chamber foreign body that was masquerading as stromal keratitis . a 28-year - old male visited with complaints of visual disturbance and hyperemia in his right eye for four weeks . on initial examination , he presented with a stromal edema involving the inferior half of the cornea , epithelial microcysts , and moderate chamber inflammation . suspecting herpetic stromal keratitis , he was treated with anti - viral and anti - inflammatory agents . one month after the initial visit , anterior chamber inflammation was improved and his visual acuity recovered to 20/20 , but subtle corneal edema still remained . on tapering the medication , after three months , a foreign body was incidentally identified in the inferior chamber angle and was surgically removed resulting in complete resolution of corneal edema . the removed foreign body was a fragment of graphite and he subsequently disclosed a trauma with mechanical pencil 12 years earlier . this case showed that the presence of an anterior chamber foreign body should always be considered in the differential diagnosis of idiopathic localized corneal edema .

mesenteric panniculitis ( mp ) is a rare disease associated with inflammatory and fibrotic changes within the mesentery . it may occur as an incidental finding on ct scanning for other conditions such as nephrolithiasis . alternatively , patients with mp may develop abdominal pain , nausea / vomiting , weight loss , fever , and bowel habit changes . physical examination may reveal the presence of an abdominal mass [ 3 , 4 ] . characteristic ct scan findings in mp include inflammatory mass with hyperattenuation of fat and the appearance of small mesenteric nodules . radiographically , the mesenteric vasculature appears to be spared in mp ( also known as the fat - ring sign ) , and this finding has been suggested to differentiate mp from lymphoma and other neoplasms . nonetheless ct findings such as this are nonspecific and are generally not recommended to rule out the presence of a neoplastic process such as lymphoma . however , a recent study by zissin et al . suggested that positron emission tomography - computerized tomography ( pet - ct ) imaging may be used to differentiate between benign and neoplastic processes of the mesentery . those authors examined 19 patients with known malignancy and incidental findings of mp on ct scan with pet - ct evaluation . the absence of f - fluorodeoxyglucose ( fdg ) uptake within the areas of panniculitis in 11 of the 19 patients was found to be indicative of a nonneoplastic process after clinical evaluation and follow - up . no false - negative results were reported . in this report , we describe a patient with symptoms and ct findings of mp and a negative uptake of fdg on pet - ct scan who was subsequently found to have lymphoma on biopsy of the mesentery . we continue to recommend that a biopsy should be performed in patients presenting with a mesenteric mass . the patient is a 52-year - old caucasian female who was found to have milky - appearing ascites during routine vaginal hysterectomy and repair of an uterovaginal prolapse . 1 ) . this showed the typical features of mp , including strand - like and patchy densities in the mesenteric fat surrounding but not infiltrating the mesenteric vasculature in the mid - abdomen . she described episodes of severe abdominal pain occurring about every 3 months for 23 years . mesenteric panniculitis symptom assessment score ( mpsas ) was 24 , suggestive of moderately symptomatic disease . laboratory examination included : hemoglobin = 14.7 g / dl , hematocrit = 43.7% , white blood cell count = 5,200 , platelet count = 235,000 , erythrocyte sedimentation rate = 13 mm / h , c - reactive protein = 0.2 mg / dl , sodium = 141 mmol / l , chloride = 106 mmol / l , potassium = 4.0 mml / l , bicarbonate = 33 mmol / l , glucose = 85 mg / dl , blood urea nitrate = 11 mg / dl , creatinine = 0.9 mg / dl , calcium = 9.6 mg / dl , albumin = 4.5 g / dl , sgot = 27 u / l , sgpt = 26 u / l , alkaline phosphatase = 75 u / l , bilirubin = 0.8 mg / dl . a pet - ct scan ordered by another practitioner demonstrated no abnormal fdg uptake in the affected mesentery or any surrounding lymph nodes ( fig . biopsies were obtained which demonstrated a b - cell non - hodgkin 's lymphoma of follicular origin ( fig . 4 , 5 ) . these symptoms are frequently encountered in clinical practice and especially when chronic , may not warrant aggressive evaluation . radiographic evaluation with ct scanning was performed in this case due to the incidental finding of milky - appearing ascites during a gynecologic procedure . fourteen percent of the ninety - two patients with mp recently described in the series by akram et al . our group has also described the natural history of chylous ascites in a patient with mp . chylous ascites would more commonly be associated with intraabdominal malignancies , including lymphoma , that interrupt and obstruct flow of lymphatics [ 3 , 8 ] . combination of ct and pet as a single test has been commonly used in clinical practice . this modality allows precise location of fdg uptake in a rapidly performed , well - tolerated procedure . it is most useful for staging of known malignancies . in patients with a known diagnosis of lymphoma , pet - ct can be utilized to find disease within and outside of lymph nodes that would otherwise be missed by conventional ct scanning . in general , recently , zissin et al . have reviewed pet - ct findings in 19 patients with known malignancy that were found to have ct findings consistent with malignancy . five of these had the procedure for staging purposes , while the other 14 patients underwent the procedure to evaluate suspected recurrent tumor . these findings included a well - defined , inhomogeneous fatty mass , preserved perivascular fat , and a pseudotumor capsule . eight patients had increased fdg uptake ( positive pet scan ) , and 11 patients did not have increased fdg uptake ( negative pet scan ) . all 11 patients with negative pet scanning had no change in imaging results for an average follow - up of 10.5 months , suggesting that the mesenteric findings were benign and therefore consistent with mp alone . the authors suggested that pet - ct scanning could have a role in the evaluation of mesenteric masses in oncologic patients and that pet - ct can be used to correctly exclude mesenteric tumor involvement when no fdg uptake is seen within typical ct features of mp . they did not describe any false - negative pet - ct scans in their case series . as our case suggests , in patients presenting de novo with a mesenteric mass , without a history of malignancy biopsy of the mesenteric mass , preferably by laparoscopy , is advisable to confirm the diagnosis of mp and to rule out a neoplastic process .

mesenteric panniculitis ( also known as sclerosing mesenteritis ) is a chronic inflammatory disease of the mesenteric connective tissue . it is known to have a wide spectrum of clinical and radiological presentations . in general , biopsy is recommended for diagnosis ; however , a recent study proposed that a negative positron emission tomography- computerized tomography ( pet - ct ) scan is accurate in differentiating benign and neoplastic mesenteric processes [ br j radiol 2006;79:3743 ] . the following case report questions the accuracy of pet - ct in this setting and confirms the requirement for biopsy to rule out the presence of mesenteric lymphoma .

a 55 year - old man visited our emergency department because of increasing frequency of chest pain . he had undergone off - pump coronary artery bypass grafting ( cabg ) 10 years ago because of unstable angina associated with three vessel coronary artery disease . at the initial operation , the in situ right internal thoracic artery ( ita ) , in situ left ita and in situ right gastroepiploic artery ( rgea ) grafts were used to revascularize the left anterior descending coronary artery , two obtuse marginal coronary branches , and posterior descending coronary artery , respectively . an excess segment of the distal right ita was connected to the side of left ita as a y - composite graft and anastomosed to the first diagonal coronary artery . coronary angiography and myocardial single photon emission computed tomography ( spect ) were performed at 5 years after surgery as a follow - up study . the 5-year angiography showed all patent grafts and the myocardial spect demonstrated no perfusion decrease . exertional chest pain recurred at 7 years after surgery , and a repeated coronary angiography showed patent previous grafts including faint visualization of the in situ rgea graft associated with significant stenosis at the os of the celiac axis . the computed tomographic angiogram also demonstrated a 90% stenosis at the celiac os , which had been without stenosis on abdominal angiography taken before the surgery ( fig . redo off - pump cabg was performed 10 years after the initial surgery because of an increasing frequency of angina and an aggravated finding of the follow - up myocardial spect , which was a newly developed reversible perfusion decrease in the inferior wall ( fig . , the great saphenous vein was harvested from the lower leg and interposed between the middle part of in situ right ita and distal part of in situ rgea grafts used previously , to supply blood flow from the right ita graft to the posterior descending coronary artery . one year after redo surgery , the patient had no symptoms of angina and coronary angiogram was performed and revealed patent grafts , including an interposed saphenous vein graft ( fig . 3a ) . the myocardial spect test was also performed and demonstrated that there was no perfusion decrease including the inferior wall ( fig . reoperations for coronary artery disease have been increased due to the increased number of isolated cabg . the society of thoracic surgeons statistics indicated that nearly 5% of the current cabg procedures done in the us were repeat surgical revascularization . angiographic indications for reoperation are progression of native coronary atherosclerosis , previous graft failure or a combination of both . one previous study demonstrated that 4 out of 400 patients who underwent cabg using the rgea graft needed percutaneous interventions due to the rgea graft failure during postoperative follow - up of 2211 months . one of those 4 patients required an angioplasty for a newly developed stenosis of the celiac trunk . in the present case , an indication for reoperation the patient had been free of angina , and the angiographic and myocardial spect follow - up studies revealed no abnormal findings at postoperative 5 years . when the patient suffered from recurred angina at postoperative 7 years , coronary angiography showed a faint visualization of the in situ rgea graft associated with significant stenosis at the os of the celiac axis . the 10-year follow - up myocardial spect test demonstrated a newly developed reversible perfusion decrease in the inferior wall . the prevalence of celiac axis stenosis was 7.3% in a korean population although it was lower than the previously reported incidence of celiac axis stenosis in western populations ranged from 12.5% to 24% . in the present case , celiac artery stenting could be an alternative option in such a case . however , we performed a redo operation because celiac axis stenting was associated with a high incidence of late restenosis . the aorta or another in situ arterial graft could be chosen as a blood source . alternatively , patent in situ grafts used previously may be re - used as an inflow conduit . with regards to our patient , the 3 in situ arterial grafts had already been used . the saphenous vein graft was interposed between the middle part of right ita and distal part of in situ rgea grafts used previously .

we report a redo coronary artery bypass grafting ( cabg ) in a 55-year - old man . angina recurred 7 years after the initial surgery . coronary angiography showed all patent grafts except a faint visualization of the in situ right gastroepiploic artery ( rgea ) graft , which was anastomosed to the posterior descending coronary artery , associated with celiac axis stenosis . redo - cabg was performed at postoperative 10 years because of aggravated angina and decreased perfusion of the inferior wall in the myocardial single photon emission computed tomography . the saphenous vein graft was interposed between the 2 in situ grafts used previously ; the right internal thoracic artery and rgea grafts . angina was relieved and myocardial perfusion was improved .

many proteins , especially those involved in the signal transduction , contain compact units or multiple domains performing a wide variety of functions ( 1,2 ) . in some cases each involved in a series of steps in a biochemical reaction . in other instances , the functional domains work together in a manner that some domains decide the function and the efficiency of co - existing domains such that the catalytic domains are recruited in a particular pathway selectively . in few other instances , the structurally compact domains may be distinct in their functions at most times but can influence or organize the function of neighbouring domains at the interface through substantial conformational changes . therefore , the compilation of biological information of the protein domains ( 311 ) is an useful step in different areas of the interface between computing and biology , e.g. protein sequence analysis , structure prediction , modelling , rational design of experiments ( such as protein crystallization and site - directed mutagenesis or deletion experiments ) and perception of biological function ( such as signal transduction and allostery ) . several objective methods identify the protein structural domains starting from the atomic coordinates of proteins ( 1218 ) . dial is one such procedure that identifies the structural domains in proteins by clustering substructures on the basis of their spatial distances ( 17 ) . this has been further improvised and compared ( 19 ) with other protein domain resources ( see supplementary data for some examples ) . popular public domain resources often require careful manual examination ( 6 ) or consultation of several algorithms internally ( 20 ) . however , in order that the structural domain boundaries can be identified for newer proteins and for addressing the overall structural domain architecture of proteins with multiple chains , we report the availability of dial web server . dial web server provides additional information such as the presence of secondary structures , conserved residues and functional motifs for the individual domains that are mapped both on sequence and structure . the non - hydrogen atomic coordinates of the protein form the input for dial server . alternately , sequence of the query can be employed as an input to identify the nearest structural homologue for the examination of structural domains . the nearest structural homologue is identified by initiating a psi - blast search against the protein data bank ( pdb ) database at an e - value threshold of 10 . segment of the pdb hit that matches 100% with the entire length of the query protein is considered for the domain delineation . where the protein is reported as crystallographic or physiological multimers and the transformation matrix is provided , the server internally generates the multimer coordinates and offers structural domain architecture solutions for the entire quartenary arrangement . for instance , figure s1 ( in supplementary data ) shows the entire domain architecture of the three protein multimers where extensive interactions between the protomers are evident and the individual domains are composed of multiple chains ; structures of these three protein examples further indicate domain swapping events [ ( 21 ) ; for the structural domains see supplementary data ] . secondary structures and connecting loops are clustered using their structural distances and domains identified as described previously ( 19 ) for both single and multiple chains . subsequent to our previous report ( 19 ) , in our extension to address multiple chain entries , we are also currently considering the short segments ( one or two residues long ) as individual substructures since the interactions between the multiple chains often require small regions of interactions.the best structural domain architectures are projected as convenient bar diagrams ( as shown in figure s1 in supplementary data ) and alternate domain definitions are also provided in a similar manner . alternate domain definitions provide a structural hierarchy of locally compact units and also sometimes permit the user to recognize other structural domain solutions.nearest structural homologues or scop ( 6 ) entries are also reported.sequence and structural files can be downloaded for individual domains and viewed through rasmol and chime ( 22 ) interfaces . static images of the domain definitions are provided using molscript ( 23).additional features such as secondary structural topology and conserved residues are provided . psi - blast ( 25 ) is performed against the structural entries ( 26 ) to identify homologues , sequences aligned using clustalw ( 27 ) and conserved residues identified using motifs ( 28).functionally important residues , by prosite ( 29 ) definitions , are projected for individual domains . domain interface residues are proposed by comparing the solvent accessibility ( 30 ) of individual domains in the free form and the entire protein context . residues that undergo appreciable burial owing to adjacent structural domains are highlighted in the dial server as possible domain interface residues . secondary structures and connecting loops are clustered using their structural distances and domains identified as described previously ( 19 ) for both single and multiple chains . subsequent to our previous report ( 19 ) , in our extension to address multiple chain entries , we are also currently considering the short segments ( one or two residues long ) as individual substructures since the interactions between the multiple chains often require small regions of interactions . the best structural domain architectures are projected as convenient bar diagrams ( as shown in figure s1 in supplementary data ) and alternate domain definitions are also provided in a similar manner . alternate domain definitions provide a structural hierarchy of locally compact units and also sometimes permit the user to recognize other structural domain solutions . sequence and structural files can be downloaded for individual domains and viewed through rasmol and chime ( 22 ) interfaces . psi - blast ( 25 ) is performed against the structural entries ( 26 ) to identify homologues , sequences aligned using clustalw ( 27 ) and conserved residues identified using motifs ( 28 ) . functionally important residues , by prosite ( 29 ) definitions , are projected for individual domains . domain interface residues are proposed by comparing the solvent accessibility ( 30 ) of individual domains in the free form and the entire protein context . residues that undergo appreciable burial owing to adjacent structural domains are highlighted in the dial server as possible domain interface residues . owing to the structural genomics initiative ( 31 ) and the recent high - throughput computational structure prediction of gene products , there will be increasing numbers of proteins whose structures are available and their biological function waiting to be determined . the availability of domain boundaries would be an useful starting point for such analyses . in general , dial domain definitions and boundaries compare very well with crystallographers ' definition and other objective identification methods such as 3dee ( 18 ) with a mean overlap score of 93 and 97% , respectively [ ( 19 ) ; also for details see table s1 in supplementary data ] . in small number of cases , e.g. pdb code 1bia in table s1 ( in supplementary data ) , the domain definitions from pure distance - based methods such as dial can not be compared with the functional domains defined by other resources . the accurate delineation of structural domains is often a non - trivial problem and requires expert opinion where the domains may be heavily interacting or discontinuous in sequence or involve multiple chains . the availability of a web server for the understanding of structural domain architecture of protein structures should be useful for the study of newer proteins . dial server is especially suited for the study of structural domain architecture of multiple chain systems that must give rise to a biologically more meaningful picture of structural domain organization .

dial is a web server for the automatic identification of structural domains given the 3d coordinates of a protein . delineation of the structural domains and their exact boundaries are the starting points for the better realization of distantly related members of the domain families , for the rational design of the experiments and for clearer understanding of the biological function . the current server can examine crystallographic multiple chains and provide structural domain solutions that can also describe domain swapping events . the server can be accessed from . the supplementary data can be accessed from .

dyschromatoses are a group of reticulate pigmentary disorders characterized by the presence of both hyper- and hypo - pigmented macules that are small in size and irregular in shape . it is a spectrum of diseases that includes dyschromatosis universalis hereditaria ( duh ) , dyschromatosis symmetrica hereditaria ( dsh ) or acropigmentation of dohi and a segmental form called unilateral dermatomal pigmentary dermatosis . dsh was the first reported as a clinical entity by toyama in 1929 . in recent years , with the development of genetic analysis , adar1 or dsrad gene mutations have been found in dsh but not in duh , and therefore these two diseases are now regarded as two different entities . adermatoglyphia or immigration delay disease is characterized by congenital absence of epidermal ridges and eventually finger prints . we are presenting a case of duh associated with adermatoglyphia in a young indian male with family history of the disorder . a 31-year - old unmarried male presented with progressively increasing , generalized mottled pigmentation since the age of 3 years . the lesions had started over neck and trunk that spread centrifugally to the extremities over a period of 10 years . there was no history of handling any chemical directly or any significant history of drug intake . on dermatological examination , multiple hyper- and hypo - pigmented macules of varying size ranging from 2 to 30 mm were present in a reticulate pattern without atrophy over the forehead [ figure 1 ] , trunk [ figures 2 and 3 ] , upper and lower extremities [ figure 4 ] . reticulate pigmentation was present also on palms with absent dermatoglyphics distal to the distal creases [ figure 5 ] . dystrophic changes in the fingernails including longitudinal striations , ragged cuticles and pterygium in one fingernail were seen [ figure 6 ] . routine investigations , including complete blood counts , urinalysis , liver function tests , renal function tests , and serum electrolytes were within normal limits . venereal disease research laboratory test and enzyme - linked immunosorbent assay for human immunodeficiency virus were both nonreactive . the basal layer showed pigmentation with some coarse melanocyte granules , predominantly in the lower 23 layers of the epidermis . symmetrically distributed hyper- and hypo - pigmented lesions on forehead generalized symmetrically distributed hyper- and hypo - pigmented lesions on chest generalized symmetrically distributed hyper- and hypo - pigmented lesions on abdomen reticulate pigmentation over legs absent dermatoglyphics on fingertips dystrophic changes in fingernails histopathology from hyperpigmented lesion showed increased melanin deposition in the basal layer of epidermis with prominent pigmentary incontinence ( h and e , 400 ) dyschromatosis universalis hereditaria is a rare genodermatosis that is commonly encountered in japan ; however , rare familial cases have been reported from europe , china , and india . duh is an autosomal dominant ( ad ) disorder with variable penetrance , but a few individuals show an autosomal recessive pattern . although the precise etiology of this disorder is not yet known and the culprit gene still unidentified , duh locus has recently been mapped to chromosome 6q24.2-q25.2 , but has not been confirmed by many . in our patient , there was history of similar lesions in the younger brother . in duh , facial lesions were seen in almost 50% of affected individuals , but involvement of palms and soles is rare . in our case , dyschromatosis universalis hereditaria may be associated with abnormalities of dermal connective tissue , nerve tissue , or be associated with other systemic complications , but no such features existed in our patient . abnormalities of hair and nails have also been reported , but only nail changes were present in our patient . it has been suggested in the past that duh is a disorder of melanocyte number . on the basis of recent electron microscopic study , it has been suggested that duh may be a disorder of melanosome production and distribution in epidermal melanin units rather than a disorder of melanocyte number . adermatoglyphia is caused by a point mutation in the splice - site of a 3 exon of the gene for smarcad1-helicase , which is mapped to chromosome 4q22 . it is an autosomal dominant disorder and has been reported in association with other reticulate pigmentary disorders such as dermatopathia pigmentosa reticularis ( ad ) , naegeli franceschetti jadassohn syndrome ( ad with keratin 14 gene defect ) and benson 's syndrome ( ad ectodermal dysplasia ) . our patient showed adermatoglyphia distal to the distal creases in both the hands , but there were no other cutaneous features suggesting overlapping of adermatoglyphia with these reticulate pigmentary disorders . lesions of duh have to be differentiated from other inherited reticulate pigmentary disorders such as dermatopathia pigmentosa reticularis ( dpr ) , naegeli franceschetti jadassohn syndrome , and dyskeratosis congenita [ table 1 ] . jadassohn syndrome is characterized by complete absence of dermatoglyphics , reticulate hyperpigmentation , palmoplantar keratoderma , abnormal sweating , subtle developmental anomalies of the teeth and hair . differential diagnoses of duh to the best of our knowledge , our report is the first case of duh with adematoglyphia from india . although it is possible that our patient had two distinct genetic disorders by sheer chance , it is important to record any such clinical overlapping , since it may indicate genetic linkage . due to unavailability and cost factor , we could not perform gene mapping , which might have explained their precise association .

dyschromatosis universalis hereditaria ( duh ) is a rare , autosomal dominant genodermatosis with a peculiar reticulate pigmentary change , consisting of hyperpigmented macules mingled with hypopigmented lesions to give an overall impression of mottling . we herein report a case of duh with adermatoglyphia in a young male with family history of the disorder .

ganglioneuromas are benign tumors of the sympathetic nervous system that rarely arise in the adrenal gland . up to the current era of laparoscopic adrenal mass excision , this unusual entity has not been adequately reported in the surgical literature . a 51 year old male with history of hypertension was found to have abdominal bruit during a regular physical examination . a 4 cm right adrenal mass with upper pole calcification and a 6 cm retro - pancreatic mass were subsequently found on a computed tomography scan . preoperative endocrine evaluation showed mildly elevated vanillyl mandelic acid with normal 24-hour cathecolamine , metanephrine and cortisol levels . ganglioneuroma occurs rarely in adrenal gland and preoperative diagnosis is difficult since symptoms are usually nonspecific . due to widespread utilization of abdominal imaging , ganglioneuromas , benign tumors of the sympathetic nervous system , are rare to arise in the adrenal gland . they usually develop from the great sympathetic chains , extending from the base of the skull to the neck , posterior mediastinum , retroperitoneum , and adrenal glands . currently , histopathologic examination is the only tool to diagnose ganglioneuroma and to differentiate it from other neural crest tumors . up to the current era of laparoscopic adrenal mass excision , a 51 year old man was referred to geisinger clinic by his primary care physician for an abdominal bruit that was found during a regular physical examination . abdominal computed tomography ( ct ) scan revealed a 4 cm right adrenal mass with upper pole calcifications and a 6 cm retropancreatic mass ( figures 1 and 2 ) . preoperative endocrine evaluation only showed mildly elevated vanillyl mandelic acid ( vma ) with a normal 24-hour cathecolamine , metanephrine and cortisol levels . intraoperatively , the tumors were able to be separated off the surrounding structures without major difficulty . coronal section of the right - sided adrenal ganglioneuroma axial section of the retropancreatic mass ( 5.8 4.3 cm ) ganglioneuroma ( gn ) originates from cells of the neural crest that include the sympathetic ganglias and the adrenal glands . it is composed of ganglion cells , neurites , schwann cells , and fibrous tissues . this tumor affects children and young people preferentially with three - fifth developed before age 20 . clinical symptoms of gns are non - spesific , mosty hormonally silent and related to their size / locations . despite their generally benign nature , gns may come to attention due to compression of their neighboring structures . approximately up to 30% of patients were found to have elevated plasma and urinary catecholamine but they rarely develop symptoms of vasoactive amines excess . immature element such as neuroblast is not part of mature gn explaining lower rate of metaiodobenzylguanidine ( mibg ) uptake ( 57% ) compared to neuroblastoma ( 92% ) . diagnosis of gn should be suggested when an adrenal tumor harbors the following : ( 1 ) no hormonal hypersecretion , ( 2 ) presence of punctate or discrete calcifications , ( 3 ) absence of vessel involvement , and ( 4 ) a low non - enhanced t1-weighted signal with a late and gradual enhancement on dynamic magnetic resonance imaging ( mri ) . the treatment for this condition is complete surgical resection through either an open or laparoscopic approach . increased level of urinary noradrenaline , dopamine , hva , and vma are frequently encountered in neuroblastoma , while the level of urinary cathecolamine , hva , and vma are usually normal in ganglioneuroma . in the present review of 14 cases displayed in table 1 , an increased urinary catecholamine level and sex hormone were noted in several patients , therefore pharmacological diagnostic tests for such hormonal activities may not necessarily be discriminative in the differential diagnosis of these two related tumors . ganglioneuroma occurs rarely in adrenal gland and preoperative diagnosis is difficult since symptoms vary and oftentimes nonspecific . due to widespread utilization of abdominal ultrasonography , ct scan and mri , detection of such tumor has increased , therefore differential diagnosis of an adrenal / retroperitoneal mass should include ganglioneuroma .

context : ganglioneuromas are benign tumors of the sympathetic nervous system that rarely arise in the adrenal gland . majority of cases are detected incidentally since they are usually asymptomatic . up to the current era of laparoscopic adrenal mass excision , this unusual entity has not been adequately reported in the surgical literature.case report : a 51 year old male with history of hypertension was found to have abdominal bruit during a regular physical examination . a 4 cm right adrenal mass with upper pole calcification and a 6 cm retro - pancreatic mass were subsequently found on a computed tomography scan . endoscopic ultrasound - guided needle biopsy was indeterminate . preoperative endocrine evaluation showed mildly elevated vanillyl mandelic acid with normal 24-hour cathecolamine , metanephrine and cortisol levels . histopathologic examination after an uneventful laparoscopic excision was consistent with ganglioneuroma.conclusions:ganglioneuroma occurs rarely in adrenal gland and preoperative diagnosis is difficult since symptoms are usually nonspecific . due to widespread utilization of abdominal imaging , however , it should be included in differential diagnosis of adrenal or retroperitoneal mass . histopathologic examination is currently the mainstay of diagnosis .

atrichilemmal cyst also known as pilar cyst is a common cyst that forms from a hair follicle . the cysts are smooth filled with keratin , a protein component found in hair , nails , and skin . a 55-year - old female from south india of dravidian race presented with a history of gradually progressive , painless swelling in the left bulbar conjunctiva adjacent to the nasal limbus of 3 years duration . there was no history of trauma or history of any surgery performed in the past . , there was a nodular mass adjacent to the nasal limbus of left eye measuring 5 mm 5 mm , fixed to the underlying tissue , non - tender , lying within the pterygium [ figures 1a and b ] . a fold of subconjunctival tissue extending from the swelling to the caruncle was observed during wide excision biopsy of the lesion . ( a and b ) nodular mass at the limbus histopathology indicated the presence of sebaceous material . microscopic examination showed a cyst lined by stratified squamous epithelium with the absence of granular cell layer , focal basal cell hyperplasia , and flakes of keratin within the cyst [ figures 2a c ] . cyst cavity filled with keratin lacking a granular cell layer in its wall ( h and e , 400 ) focal basal cell hyperplasia in the cyst wall absence of granular cell layer in the cyst wall it poses a diagnostic challenge because most lesions are transitions between inflammation , inflammatory hypertrophies , and true neoplasms . a trichilemmal cyst , also known as wen , pilar cyst or isthmus - catagen cyst forms from a hair follicle . though most often found on the scalp , they can also occur on other parts of the body such as the upper lip , palpebral conjunctiva , caruncle , and pulp of the index finger . the rare location of bulbar conjunctiva in this case could be explained as originating from the caruncle and being pushed toward the nasal limbus . however histologically trichilemmal cysts lack a granular cell layer . approximately , 20% of the epithelial cysts are trichilemmal cysts and other 80% are epidermoid . there are a few reported cases of trichilemmal cyst and malignant trichilemmal tumor of the eyelid . however to the best of our knowledge , no cases of trichilemmal cysts involving the bulbar conjunctiva have been reported . this case report highlights the need for considering trichilemmal cyst as differential diagnosis of the limbal nodule .

we report a rare case of trichilemmal cyst involving the bulbar conjunctiva . a 55-year - old female presented with a history of a painless , progressive swelling in the left bulbar conjunctiva adjacent to the nasal limbus of 3 years duration . wide excision biopsy was performed . histopathologic examination findings were consistent with those of trichilemmal cyst . trichilemmal cyst should be considered as differential diagnosis in a case of limbal nodule .

( 1 ) patients may report dysuria , intermittency , haematuria , frequency , hesitancy , nocturia or sudden termination of voiding . a 62 year old lady presented to the hospital with a swelling in her right thigh . she had a past history of myelomeningocele spina bifida which had been closed at birth . she suffered from urinary incontinence and had been managed with long term suprapubic catheters for 20 years . she had required a vesicolithotomy via a midline incision and a vaginal hysterectomy 13 years previously . initial examination revealed a swollen right thigh and a calcified mass protruding through the skin in the right groin . a strong smell of urine was noted although it was thought that this was secondary to a leakage from around the site of her supra pubic catheter . laboratory data demonstrated normal renal function , a raised c - reactive protein and normal white cell count . due to her swollen right leg a deep vein thrombosis was excluded with an ultrasound scan and negative d dimer and plain radiographs of her right hip and pelvis were requested . a urology opinion was sought , initially , with regard to management of the suprapubic catheter . on closer examination , urine was noted to be leaking not from the suprapubic site but around the calcified mass in the right groin and a diagnosis of vesicocutaneous fistula was made . her radiographs demonstrated two urinary calculi ( fig 1 ) and she went on to have an abdominal computed topography scan ( fig 2 ) which confirmed the diagnosis of a vesicocutaneous fistula . plain radiographs demonstrating two bladder calculi ct demonstrating the two bladder calculi pointing towards the right groin vesicolithotomy was performed removing the calculi by enlarging the fistulous opening . ( fig 3 ) two calculi measuring 40 mm 43 mm 30 mm and 43 mm 50 mm 33 mm were extracted . 4 ) the suprapubic catheter was changed and the fistula was left open and urine leakage continued to decrease in quantity until the fistula tract closed . intraoperative view of the bladder calculi emerging through the vesicocutaneous fistula into the right groin the two bladder calculi following extraction urological complications are often a major source of morbidity for these patients . ( 5 ) abnormal bladder sensation and function combined with sphincter dysnergia predispose the spina bifida patient to urinary stasis , recurrent urinary tract infections , stone formation and renal function impairment . ( 5 ) management of the problem can be difficult and may include the use of anti - cholinergics , intermittent self catheterisation ( isc ) , supra pubic catheterisation or surgical intervention to allow urinary diversion . ( 6 ) increased fluid intake , weekly changes of indwelling catheters if isc is not possible and regular ultrasonic screening , to pick up stone formation at an early stage , may help reduce the burden of these problems . ( 2 - 4 ) predisposing factors to bladder stone formation include urinary stasis , bladder outflow obstruction urinary stasis , recurrent urinary tract infections and foreign bodies within the bladder ( 1 ) , all of which can be seen in spina bifida patients . in our case the added sensory abnormalities secondary to spina bifida left the bladder calculus undiagnosed until it had formed the vesicocutaneous fistula . good control of the predisposing factors can dramatically reduce the risk of developing bladder calculi , but especially in cases where this is difficult , a high index of suspicion , regular urological review and timely uss can prevent the development of both the bladder calculus and its complications .

we present a rare case of a vesicocutaneous fistula secondary to two large bladder calculi in a patient with spina bifida . a 62 year old lady presented with swelling of her right thigh and a calcified mass protruding through the subcutaneous tissues of her right groin . urine was seen to be draining from around the area and plain radiographs and a computed topography ( ct ) scan confirmed the presence of two bladder calculi with a vesicocutaneous fistula . vesicolithotomy was performed and the fistula tract left to close spontaneously . worldwide this is only the fourth case of a bladder calculus causing a vesicocutaneous fistula .

gestational diabetes mellitus ( gdm ) , which is close to type 2 diabetes mellitus , develops in 26% of pregnancies . in addition , a risk of developing autoimmune diseases , such as type 1 diabetes mellitus and autoimmune thyroid disease , increases in the postpartum period . classic type 1a diabetes is classified as autoimmune diabetes , characterized by autoantibodies such as glutamic acid dehydrogenase ( gad ) . postpartum graves disease is known to occur and account for 10% of postpartum autoimmune thyroid disease ( ppats ) . in the present case report , we report a case of a patient with gestational diabetes that is complicated with graves disease and type 1 diabetes mellitus after delivery . the present case was a 28yearold woman with a family history of type 2 diabetes . fasting plasma glucose level was 7.8 mmol / l ( 140 mg / dl ) in the 32nd week of pregnancy . she was then diagnosed with gdm and treated by diet modification . the baby ( 3780 g bodyweight ) was delivered by cesarean section in the 40th week of pregnancy . a month after delivery , her height was 163 cm , bodyweight was 54.0 kg and body mass index was 22.9 . physical examination showed that her thyroid gland was swelling at a degree of iii and a diffuse goiter was detected by ultrasound sonography . laboratory tests showed 9.9 mmol / l ( 178 mg / dl ) fasting plasma glucose level , and 8.0% hemoglobin a1c ( hba1c ) . thyroidstimulating hormone ( tsh ) level was 1.05 u / ml , thyroid microsomal antigen ( mcha ) was positive ( 1:1600 ) . it has been concluded that the patient had developed diabetes after delivery and had been treated by dietary modification . three months after delivery , the patient presented with palpitations and finger tremor . on laboratory examination , the free t4 level was 7.77 ng / dl and the free t3 level was 26.3 pg / ml . tsh level was lower than 0.05 u / ml and tsh receptor antibody ( trab ) was positive ( 30.4% ) . she was diagnosed with postpartum thyroid dysfunction ( graves disease ) and given propylthiouracil . after 6 months from delivery , the patient showed poor glycemic control , and high levels of urine and serum ketones . the patient s plasma glucose level was elevated to 24.6 mmol / l ( 443 mg / dl ) , hba1c level was 12.1% and serum cpeptide level was 0.47 ng / dl . antigad antibody was 144 u / ml and insulin autoantibody was 6.1% . based on these results , after 11 months from delivery , trab became negative and thyroid dysfunction showed remission . the patient gave her written informed consent for publication of the present case report in journal of diabetes investigation . the initiating factor is likely to be increased peripheral insulin resistance of normal pregnancy , but in an attempt to overcome the increased insulin resistance , relative pancreatic insufficiency develops . it is known that women with gdm have a considerable risk of developing type 2 diabetes later in life . this response is known to have a risk of causing autoimmune disease including type 1 diabetes and autoimmune thyroid disease to the mother . type 1 diabetes is thought to arise from an autoimmune attack against the pancreatic cells , which leads to failure to regulate blood glucose levels . usually , antibodies against islets , insulin and gad are found to be positive in patients . the presence of antimicrosomal antibodies , a family history of autoimmune disease and insulin dependent diabetes are known to increase its risk . highrisk mothers for postpartum thyroid dysfunction are well screened by mcha and antithyroid therapy might be a good choice for firstline therapy , as postpartum graves hyperthyroidism is often transient . there was also a report showing the development of both type 1 diabetes and graves disease after delivery . the association of gad with thyroid antibodies might be explained by the fact that gad is not exclusively present in the brain and pancreas , but can also be found in the thyroid gland . it has been reported that in patients with type 1 diabetes and graves disease , hladrb1 * 0405dqb1 * 0401 haplotype was detected and thelper 1 lymphocytes in peripheral blood were reduced . in addition , hyperthyroidism might aggravate glucose intolerance by multiple mechanisms . in the case of our patient , although the pathological mechanism of gdm is considered to be analogous to that of type 2 diabetes , gdm developed into type 1 diabetes with the presence of islet antibodies . in the past , it has been reported that gdm has a risk of developing into type 1 diabetes as well . jrvelet al . reported that 4.6% of gdm developed into type 1 diabetes , whereas 5.3% developed into type 2 diabetes . they also identified that young age ( < 30 years ) , the need for insulin treatment during pregnancy , and positivity for islet cell antibodies ( ica ) and gad conferred a high risk of progression to type 1 diabetes . suggested that cell autoantibodies determined at delivery in gdm were highly predictive for the development of type 1 diabetes in the postpartum period . however , by screening with all three markers ( gad , ica and antibodies to the protein tyrosine phosphataserelated protein 2 molecule ; ia2a ) , sensitivity can be increased up to 82% . they also pointed out that women with one or more pregnancies before the index pregnancy had a higher risk for developing type 1 diabetes after delivery compared with women who were in their first pregnancy . usually , the examination of gad antibodies is used for the screening of type 1 diabetes in japan . the aforementioned findings emphasized the importance of screening and monitoring highrisk gdm patients , not just after delivery , but during pregnancy and at delivery . in addition , the importance of checking all available autoimmune antibodies in diagnosing type 1 diabetes in gdm patients . interestingly in our case , trab became negative and thyroid dysfunction showed remission , whereas still gad remained positive . postpartum graves disease is usually transient . although it is likely that in the case of our patient both graves disease and type 1 diabetes had a common cause , only graves disease was transient whereas type 1 diabetes remained permanent .

abstractpregnancy and the postpartum period are associated with changes of the immune system . these changes might eventually result in autoimmune diseases , such as graves disease and type 1 diabetes mellitus , in the postpartum period . we describe a case of a patient with gestational diabetes who developed both graves disease and type 1 diabetes mellitus in the postpartum period . the pathology of gestational diabetes ( gdm ) is close to that of type 2 diabetes mellitus . however , the present case emphasizes the importance of screening and monitoring highrisk gdm patients for all available autoimmune antibodies throughout pregnancy and the postpartum period , as gdm has a risk of developing into type 1 diabetes and multiple autoimmune diseases . in addition , only graves disease was transient , whereas type 1 diabetes mellitus remained permanent in the present case . thus , the present case shows etiological differences between these two autoimmune diseases . ( j diabetes invest , doi : 10.1111/j.20401124.2010.00089.x,2011 )

twenty - one inpatients ( 15 males/6 females ) with an aver - age age of 23.7 years , who had been diagnosed with fep according to dsm - iv - tr ( diagnostic and statistical manual of mental disorders , fourth edition , text revision ) criteria , were evaluated using the positive and negative syndrome scale ( panss ) . spect was acquired after injection of tc - hmpao ( tc - hexamethylpropyleneamine oxime ) , before drug treatment and again after 3 months of treatment with either risperidone ( mean dose 4.8 mg / day in 7 patients with predominantly negative symptoms ) , olanzapine ( mean dose 11.6 mg / day in 9 patients with predominantly positive symptoms ) , and quetiapine ( mean dose 440 mg / day in 5 patients with predominantly positive symptoms ) . exclusion criteria included the presence of a neurological or other somatic disorder that could modify rcbf , and previous exposure to antipsychotic drug treatment . baseline spect assessments revealed a diffuse low perfusion in the left parietal and temporal cortices , and in the right ventromedial frontal cortex , in 11 out of 14 patients with predominantly positive symptoms ( figure 1 and figure 2 ) . in 6 out of 7 patients with predominantly negative symptoms , we found a reduced perfusion in the frontal cortex ( though bilateral , this reduction was more pronounced on the left side ) , decreased rcbf in the right basal ganglia , and low perfusion in the left orbitofrontal cortex ( figure 3 ) . rcbf was normal in 4 patients ( 3 with predominantly positive symptoms and 1 with predominantly negative symptoms ) . the cortical abnormalities observed in patients at baseline persisted after treatment . after treatment , additional changes in subcortical structures were also observed , notably an increase in rcbf in the left caudate nuclei in 14 patients ( figure 4 ) these results are consistent with the finding of liddle et al on rcbf patterns in schizophrenia , who reported both increases and decreases in rcbf , suggesting a dynamic imbalance rather than a fixed abnormality our data suggest cortical abnormalities in rcbf , at baseline and after treatment , conceivably correlated with the type of symptoms . subcortical changes appearing during treatment ( notably in the left caudate nuclei ) may be the consequence of treatment , but not of any one specific drug in particular .

single photon emission computed tomography ( spect ) is a valuable tool for evaluating regional cerebral blood flow ( rcbf ) in patients with schizophrenia . recently , various neuropharmacological studies using spect in patients with schizophrenia have also characterized dopamine d2 receptors , their correlation with symptoms , as well as receptor occupancy after treatment with atypical antipsychotics.1,2 we studies changes in rcbf in first - episode psychosis ( fep ) before and after pharmacological treatment .

patients on medicare who are hospitalized are allowed six weeks only . after that the patient has to be discharged . how long are patients allowed to remain in hospice before they are discharged ? where do they go after hospice ? actually , an inset in one of the slides shows that when you go home from the hospital you sometimes have a visiting nurse or a nurse aid . that does not continue for long , usually for a couple of weeks in my experience . on the other hand , as long as you meet the criteria , you stay in hospice . you have a nurse aide four hours a day , five days a week ; you have a nurse visiting you regularly ; you have someone available to you 24 hours a day , seven days a week to call if you are in a crisis ; and you have medicines in the house to use if you are in crisis so you can avoid going to the emergency room . you can have morphine , ativan , or whatever else you may need . in general , hospice is for people who have been certified that they have six or months or less to live . the truth is , if you are at six months and they think maybe you have another month or two to live , nobody kicks you out . one situation might be when a patient s name suddenly comes up on the liver transplant list this may be a problem for some people who need a lot of help and to those who can not afford nursing homes much of the time . this is , by the way , a problem with tube feeding because , again , unless you are medicaid eligible , you may have to pay yourself . we think they are going to die within the six months , and they end up being very stable . sometimes those people do have to be discharged from hospice early . it is not like rehabilitation days or physical therapy days ; there is no limit . actually , private insurance is often more generous than medicare because it tends to be oriented towards younger people whom they do not expect will use it as much . people who are of working age and are not on medicare can often go into inpatient hospice for a fairly long period of time , and it will be covered . unfortunately , with medicare you usually have to be at home , but they can bring you into an inpatient setting . in long island we have four places right now in the hospice care network where people can come in , sometimes to die , if they are very sick and it is very hard to take care of them at home . it is a wonderful benefit , and you can see i am a preacher for it . one thing i always say in palliative care is that it is not a one - way conveyor belt , and there is no steel door that slams shut and you can not get out . you can get out . we find in our palliative care units that some people improve considerably because we are doing so much less to them , and we get them out of the icu . if they are on too many drugs , we withdraw some drugs , and they wake up . they usually still go into hospice but often we have restored a level of function that is a gift to them and to their family , at least for a short period of time . what will be the role of the prior care - giving team , to which the patient is so accustomed , once a transition has been made to hospice ? i have to tell you that it is hard for these folks to make house calls , but when i was in general medicine , i almost always remained the supervising physician , and once or twice i made a house call . sometimes dr . i did call in the orders , and i would take messages from the family . sometimes a spouse would insist they wanted antibiotics for the fever , and we would work it out even though we were not sure that they were going to be helpful . therefore , primary physicians can remain involved to the extent that they want to be and they can continue to be the supervising physician and bill for it .

hospice care is ideally suited to meet the psychosocial and spiritual needs of dying patients , providing the opportunity to settle financial , property , and inheritance issues ; to mend lacerations in important lifetime relationships , including forgiving and asking forgiveness ; and to assure a degree of autonomous control over the environment and the social and spiritual processes that attend one s death . physicians are not only imprecise in prognosticating a patient s time to die , they tend to be over - optimistic in their predictions . a no answer to the question , would i be surprised if this patient died in the next year ? is a reasonable starting - point for discussing hospice care as a potential treatment plan , now or in the future . physicians have a duty to present palliative care in hospice as an alternative to the recurrent hospital interventions that are typical in the last six to 12 months of life tor patients who are failing and have declining prospects for one - year survival .

tumors fall into two categories : papillary lesions that tend to recur and invasive cancer . although transurethral resection ( tur ) is often performed as a primary therapy for papillary lesions , 50%75% of patients will yet recur in five years and 10%20% progress to invasive disease.1 for invasive bladder cancer , radical cystectomy is the gold standard procedure.2,3 several cytotoxic and immune modifying agents have been also used intravesically to treat bladder cancer . intravesical administration of bacillus calmette - gurin ( bcg ) , an attenuated strain of mycobacterium bovis , is the most effective immunotherapy for superficial bladder cancer and carcinoma in situ ( cis)4 among currently available therapeutic options . however , its benefits are sometimes outweighed by its severe side effects including cystitis , fever , allergic reactions , sepsis , and even death . these drawbacks thus limit its use in clinical practice , requesting a safer and more effective treatment modality with few side effects . as an alternative approach , many natural agents / substances with low or negligible side effects have been gaining more attention for possible therapeutic modalities for various human malignancies . although scientific studies have not been performed on most of them to demonstrate their biological and medicinal properties , some of them have been well characterized . for example , maitake mushroom ( grifola frondosa ) has been extensively studied for its potential medicinal properties and its immunomodulatory and antitumor activities have been thus far demonstrated in animals and humans.5,6 it is the fact that some cancer patients have been or are still taking a commercially available extract of this mushroom for therapeutic purpose . an 87-year - old retired ob - gyn physician , with a three - month history of intermittent hematuria , required emergent admission for painless gross hematuria in october , 2006 after failure to control the bleeding in an office cystoscopy . he had intermittent painless hematuria for six months prior to admission but had been lost to follow up . he also had an episode of urinary retention following a colon resection in 2002 but was not currently on alpha blocker therapy . the office cystoscopy revealed a large tumor on the right floor of his bladder , and satellite tumors surrounded this base appeared to be papillary tumors . the patient was admitted and immediately underwent tur of bladder tumor as he continued bleeding after the office cystoscopy . tur showed multiple papillary and large ulcer base and the pathology report indicated he had invasive , transitional cell carcinoma ( grade 3 ) , t2b nx mx tumor . when he was discharged home three days after tur , he was referred to a tertiary center to receive his follow - up care such as adjuvant chemotherapy , radical cystectomy , or external pelvic irradiation . he was also warned that he was likely have hematuria in the next six months unless he had surgery . however , the patient has refused all of these conventional treatments and started having an unconventional treatment using two supplements instead . one of the two supplements he took was a bioactive extract of maitake mushroom , maitake d - fraction ( maitake products , inc . , east rutherford , nj ) , following the recommended daily dosage , and the other was vitamin c ( 2,000 mg daily ) . the patient has had no recurrent hematuria , pain , or urinary symptoms since then . in fact , office examination showed that his urine was clear with negative urine cytology , negative fish ( fluorescence in situ hybridization ) test , and negative nmp-22 ( nuclear matrix protein-22 ) test . the natural history of bladder cancer is one of recurrence and progression , with recurrence most common in the first 1224 months.7 our patient was a high risk for disease recurrence , having multiple lesions with a large focus that was poorly differentiated carcinoma invading the deep muscle ( ie , t2b nx mx ) . however , the only interventions our patient underwent were tur of bladder tumor and adjuvant d - fraction regimen , because he refused radical cystectomy and other conventional interventions . particularly , since radical cystectomy is known as the gold standard procedure for such invasive cancer,1,2 performing tur alone is unlikely to remove the whole tumor completely . the patient was then warned for disease recurrence unless appropriate post - tur care was given to him . yet , without any follow - up care , he had no recurrent hematuria with all negative results of cytology , fish , and nmp-22 tests , which have exceptional sensitivity and specificity with a negative predictive value of 90.4%98%.8,9 the patient indeed remains in good pathophysiological condition without any symptoms of his initial disease , indicating possible disease remission . maitake d - fraction is not just another mushroom extract found in the market but has been scientifically investigated for its potential medicinal effects / properties for the past 20 years . besides its confirmed immunostimulatory activity , d - fraction was found to have antitumor activity in tumor - bearing mice , reducing cancer proliferation.6 an early nonrandomized clinical study on patients with various cancers also showed certain improvements in those cancer patients.10 in addition , d - fraction was capable of inducing apoptosis ( programmed cell death ) in human prostate cancer cells11 as well as > 90% growth reduction in human bladder cancer cells.12 thus , these studies support the notion that d - fraction has potent anticancer activity on animal and human malignancies . above all , the most important issue of d - fraction would be its safety . it is supported by the fact that the us food and drug administration ( fda ) had exempted d - fraction from a phase i toxicology study . the fda also approved d - fraction for an investigational new drug ( ind ) application to conduct a phase ii pilot study on patients with advanced prostate and breast cancer , further granting its use in human cases.13 this case study describes an invasive bladder cancer patient at a high risk for disease recurrence who only followed a d - fraction regimen ( with vitamin c ) refusing other medical interventions . the two - year follow - up yet indicated no clinical evidence of progression of residual disease or recurrence with possible disease remission . however , we are recommending him to have additional viable options such as cystoscopy , computed tomography scan , or another tur for further assessment and confirmation of his latest disease status . in addition , a correlation between cancer suppression and d - fraction needs to be fully established and also this is only one case with merely two - year follow - up , thereby requiring more case studies with long terms to draw any affirmative conclusions . meanwhile , it is yet plausible that d - fraction is a natural , safe , and promising agent that may have clinical implications in patients with superficial bladder cancer .

superficial bladder tumors are the most prevalent form of bladder cancers and transurethral resection is the primary surgical modality for those tumors . however , nearly 65% of patients will have tumor recurrence in five years while about 15% will have progression to muscle invasion . thus , the primary therapeutic aim is to prevent multiple recurrences and progression to a more advanced , invasive disease . we here report an 87-year - old white male patient with invasive bladder cancer who received an unconventional oral regimen of d - fraction , the bioactive extract of maitake mushroom ( grifola frondosa ) , following endoscopic transurethral resection of bladder tumor . despite a high risk for disease recurrence , follow - up yet indicated no clinical evidence of progression of residual disease or recurrence of invasive cancer . it has been nearly two years but the patient remains remarkably well and appears to be in remission . to our knowledge , this is the first and only case report of possible disease remission in a bladder cancer patient after the two - year follow - up of d - fraction regimen , so that further studies with long terms are required for drawing a relevant conclusion . nevertheless , it is conceivable that d - fraction is a natural agent that may have clinical implications in patients with superficial bladder tumors .

febrile neutropenia is a life - threatening condition characterized by fever in addition to chemotherapy - induced neutropenia . the longer therefore , granulocyte - colony stimulating factor ( g - csf ) is often used to manage chemotherapy - induced neutropenia . furthermore , prophylactic administration of g - csf is recommended for patients at high risk of febrile neutropenia by several guidelines ( 1,2 ) . pegfilgrastim is a long - acting granulocyte - colony stimulating factor ( g - csf ) formulation . a phase iii randomized trial demonstrated that pegfilgrastim significantly reduced the incidence of febrile neutropenia in breast cancer patients ( 3 ) . according to the data , pegfilgrastim has been approved in japan since 2014 and several associated adverse effects , including rash , joint pain , diarrhea and fever , have been reported . however , interstitial lung disease ( ild ) during pegfilgrastim treatment has rarely been reported . an asymptomatic 64-year - old man was referred to another hospital due to abnormal nodular shadows in the left upper lung on chest computed tomography ( ct ) scan . thoracoscopic pleural biopsy was performed and the patient was diagnosed with small - cell lung cancer ( sclc ) stage iv ( t4n2m1a ) . the patient was treated every 4 weeks with four cycles of first - line chemotherapy , including cisplatin ( 60 mg / m ) and irinotecan ( 60 mg / m ) on day 1 and irinotecan ( 60 mg / m ) alone on day 8 . however , 1 month after the fourth cycle of chemotherapy , the infiltrative shadows progressed . sclc progression was hypothesized and the patient was treated with amrubicin monotherapy ( 40 mg / m on days 1 , 2 and 3 ) as second - line chemotherapy every 3 weeks . due to certain circumstances of the patient , he was transferred to our hospital . after the fourth cycle of chemotherapy , the sclc continued to progress . the patient received combination chemotherapy with carboplatin ( area under the curve=5 ) and etoposide ( 80 mg / m on days 1 , 2 and 3 ) as third - line chemotherapy every 3 weeks . the adverse events of the first course were grade 4 neutropenia and grade 3 thrombocytopenia . prior to the second cycle of chemotherapy , the performance status ( ps ) of the patient was 3 . however , we decided to administer a second cycle of chemotherapy , as this combination therapy was considered to be effective ( fig . one day after pegfilgrastim administration , the patient experienced sudden deterioration of his respiratory status . on physical examination there were fine crackles on the right side of the chest , and the ct scan revealed diffuse infiltrative shadows ( fig . subsequent tests for infectious diseases , such as sputum , blood and urine cultures , were all normal . from these results , we considered that the patient 's clinical course was due to drug - induced lung injury . the patient was treated with methylprednisolone ( 1,000 mg / day for 3 days ) . the interstitial pneumonia improved after 10 days of pulse steroid therapy ( fig . however , the patient succumbed to cancer progression 1 month after the occurrence of interstitial pneumonia . our patient was administered pegfilgrastim for the prevention of febrile neutropenia , but ild developed on the next day . apart from pegfilgrastim , carboplatin and etoposide were suspected as the other possible offending drugs . moreover , the disease did not manifest in the patient after the first course of combination chemotherapy with carboplatin and etoposide . therefore , in this case , we considered that the drug most likely responsible for ild was pegfilgrastim . to the best of our knowledge , interstitial pneumonia has not been reported in clinical trials on pegfilgrastim ( 3,58 ) . however , several reports indicated that the administration of g - csf may be associated with lung injury . matthews reported that pneumotoxicity occurred in 3 out of 5 patients with hodgkin 's lymphoma who received g - csf with doxorubicin , bleomycin , vinblastine and dacarbazin ( abvd ) therapy ( 9 ) . yokose et al reported that pulmonary toxicity had occurred in 6 out of 52 patients with non - hodgkin lymphoma who received g - csf with cyclophosphamide , doxorubicin , vincristine and prednisolone ( chop ) therapy ( 10 ) . the study demonstrated that the mean peak leukocyte count with each therapy cycle had been associated with development of pulmonary toxicity , and concluded that lowering the g - csf dose appeared to be useful in the prevention of this toxicity . furthermore , ruiz - argelles et al reported a case of pulmonary toxicity after the administration of g - csf without any chemotherapy ( 11 ) . adachi et al reported that the mechanism of ild due to g - csf was enhancement of the infiltration of the alveoli by alkaline phosphatase - positive neutrophils ( 12 ) . were reported to be risk factors of drug - induced lung injury ( 13 ) . furthermore , niitsu et al reviewed 20 cases of interstitial pneumonia secondary to treatment with g - csf , and reported that it occurred predominantly in patients aged 60 years ( 14 ) . our patient was 64 years old , had a history of heavy smoking and his ps was 3 at the start of pegfilgrastim administration , placing him at risk to develop ild . in conclusion ,

pegfilgrastim is a long - acting granulocyte colony - stimulating factor formulation that has been approved for the prevention of febrile neutropenia . we herein report a case of interstitial pneumonia following administration of pegfilgrastim . a 65-year - old man with stage iv small - cell lung cancer was treated with carboplatin and etoposide as third - line chemotherapy . pegfilgrastim was administered during the second cycle of chemotherapy . on the day after the administration of pegfilgrastim , interstitial pneumonia developed . the respiratory condition improved with pulse steroid therapy ; however , the patient eventually succumbed to cancer progression . in conclusion , interstitial pneumonia due to pegfilgrastim is rare ; however , physicians should be aware of the possibility of this adverse effect .

crazy - paving pattern is a nonspecific radiologic sign on computed tomography of the lungs . this pattern consists of scattered or diffuse ground - glass attenuation with superimposed interlobular septal thickening and intralobular lines . the classic radiographic finding of the crazy - paving pattern is bilateral , symmetric alveolar consolidation or ground - glass appearance . we report a case of a 44-year - old man in whom congestive heart failure presented with a crazy - paving appearance and enlarged lymph nodes of the lungs . a 44-year - old man presented to our department with a 1-month history of dyspnea and cough . there was no history of sputum , fever , loss of weight , and chest pain . blood pressure was 100/70 mmhg , heart rate was 116 beats / min , body temperature was 36.7c . laboratory findings revealed were normal except na : 121 mmol / l , k : 4.8 mmol / l , aspartate aminotransferase ( ast ) : 204 u / l , alanine aminotransferase ( alt ) : 203 u / l , lactate dehydrogenase ( ldh ) : 808 u / l . thorax computed tomography ( ct ) scans revealed multiple mediastinal lymphadenopathies up to 22 mm [ figure 1 ] . in both upper lobes ; there were diffuse , multifocal , patchy , ground - glass opacities resulting in a crazy - paving appearance [ figure 2 ] . thorax computed tomography scans reveals multiple mediastinal lymphadenopathies up to 22 mm ( arrow ) . ct of the thorax shows diffuse , multifocal , patchy , ground - glass opacities ( arrow ) . there was no pathological finding of 18-fluorodeoxyglucose ( 18-fdg ) uptake on positron emission tomography ( pet ) scan . there were biatrial dilatation , prosthetic mitral valve , and second - degree tricuspid insufficiency on his echocardiography . pulmonary artery pressure was 45 - 50 mmhg , ejection fraction of the left ventricle was 60% , and so confirmed diastolic dysfunction . the patient underwent endobronchial ultrasound ( ebus ) with bronchoalveolar lavage and lymph node transbronchial fine needle aspiration biopsies ( tbna ) were performed to mediastinal lymph nodes bigger than 2 cm [ figure 3 ] . the lavage fluid was evaluated for routine cytology , gram stain and bacterial culture , acid fast stain and mycobacterial culture ; all tests were negative . periodic acid schiff ( pas ) stain was performed for differential diagnosis of pulmonary alveolar proteinosis , but did not stain . the largest lymphadenopathy size decreased to 9 mm and the ground - glass opacities disappeared on ct scan obtained 1 month after the diagnosis [ figure 4 ] . 44-year - old man with dyspnea and cough diagnosed as having congestive heart failure after treatment with furosemide . computed tomography of the thorax shows both lymphadenopathies had decreased and the ground glass opacities had disappeared ( arrow ) . left - sided congestive heart failure is a very common clinical situation . although , pulmonary edema or pleural effusion are the most common radiologic and clinical findings , crazy - paving pattern and lymph node enlargement in congestive heart failure remain unrecognized in daily practice and have been little studied . however , at least 50% of patients with congestive heart failure are likely to develop enlarged mediastinal lymph nodes without evidence of an infectious , tumor - related , or specific inflammatory cause . this pattern consists of scattered or diffuse ground - glass attenuation with superimposed interlobular septal thickening and intralobular lines . it was initially described in cases of pulmonary alveolar proteinosis ( pap ) . in alveolar proteinosis , the ground - glass attenuation reflects the low density intra - alveolar material ( glycoprotein ) , whereas the superimposed reticular attenuation is due to infiltration of the interstitium by inflammatory cells . pulmonary alveolar proteinosis manifests as filling of the alveoli by a proteinaceous material that is positive at pas staining and rich in lipid , in association with an inflammatory response in the adjacent interstitium . the classic radiographic finding is bilateral , symmetric alveolar consolidation or ground - glass appearance . the differential diagnosis of crazy - paving sign includes pneumocystis jirovecii ( carinii ) pneumonia , mucinous bronchioloalveolar carcinoma , sarcoidosis , lipoid pneumonia , adult respiratory distress syndrome , pulmonary hemorrhage syndromes , usual interstitial pneumonia , pulmonary hemorrhage , acute radiation pneumonitis , drug - induced pneumonitis , and cardiogenic edema . among them , p. jirovecii pneumonia is the most common one . it has been reported rarely in congestive heart failure . histologically , three processes lead to the crazy - paving pattern : an alveolar filling process , interstitial fibrosis , and a combination of these . on multislice ct , the most common manifestation of cardiogenic pulmonary edema is ground - glass opacity . the ground - glass opacity associated with hydrostatic edema often will have a central , perihilar distribution , and be associated with enlarged pulmonary vessels and an enlarged heart . lymphadenopathy in such cases is the expression of diffuse intrathoracic edema affecting the pulmonary parenchyma and neighboring structures , including the mediastinum and associated lymph nodes . ngom et al . , reported benign lymphadenopathies caused by congestive heart failure which short - axis diameter was between 10 and 17 mm . in our case , there were diffuse lymphadenopathies up to 22 mm in aortopulmonary window and subcarina and they disappeared after furosemide treatment . although congestive heart failure and cardiogenic pulmonary edema are common situations , they have been reported as a cause for crazy - paving appearance in only four cases previously . crazy - paving appearance in congestive heart failure was thought to be an uncommon radiological finding by clinicians and radiologists . we think this finding is reported in very few cases because most congestive heart failure patients do not undergo chest ct scan before treatment . although , crazy - paving is a nonspecific finding and it may be seen in various other diseases , and in combination with other radiological findings , history , and clinical presentation can often lead to appropriate diagnosis . in chest ct scans that show crazy - paving appearance of lungs with mediastinal lymphadenopathies in patients with heart problems , heart failure should be considered in differential diagnosis . invasive diagnostic procedures can be applied if lymphadenopathies and crazy - paving appearance do not improve after the treatment of heart failure .

crazy - paving sign is a pattern seen on multislice computed tomography images of the lungs . it is characterized by a reticular pattern superimposed on ground - glass opacity . it was first described in the late 1980s in patients with pulmonary alveolar proteinosis , but has now been described in some other diseases of the lung . enlarged mediastinal lymph nodes can be seen in infectious and specific inflammatory diseases and malignancies . the present report describes a case of a 44-year - old man in whom congestive heart failure presented with a crazy - paving appearance and enlarged lymph nodes of the lungs on the chest computed tomography scan .

failures of osseointegrated implants have been associated with many factors , such as poor bone quality , lack of initial instability , overloading and fractures of implants and abutment screws10 . fracture of these elements may be related with a poor fit of the framework , which leads to material fatigue , occlusal overload and intrinsic material failures10,16 . the increasing use of implants in the rehabilitation of partially edentulous patients has widened the possibilities in prosthodontics with the introduction of new concepts , such as the placement of implant - supported prostheses , which can be either retained by screws or cemented . metal fractures may be related to microstructures that influence the material properties , which are related with the localized identification of chemical phases and segregations , frequently associated with failures at interfaces or components , which , in turn , can cause the fractures5 . these aspects may be investigated by scanning electron microscopy ( sem ) and may play a key role on the failure of prosthetic components . this article reports a case of simultaneous fracture of two ucla abutments screws occurring in an implant - supported prosthesis placed in the mandibular molar region . the fractured structures were examined by sem to investigate the probable causes of the failure . a 40-year - old non - smoker female patient in good general health conditions attended a private dental clinic with two branemark implants ( unknown trademark ) in function for about 2 years . the implants were placed in the posterior left side of the mandible , corresponding to the mandibular left second premolar ( 3.75 x 10 mm ) and first molar ( 3.75 x 7.5 mm ) , joined by the structure of a metal - ceramic fixed partial denture . the crows were cemented to the abutments and exhibited visible misfit , mainly on the distal implant . after several unsuccessful attempts to remove the fractured screw fragments , it was decided to remove the implants . the fractured components were further analyzed with a scanning electron microscope ( fei philips xl-30 , eindhoven , the netherlands ) to assess the structural failures . failure analysis by sem demonstrated the occurrence of two moments of rupture in the first fractured implant ( mesial ) , corresponding to the mandibular left second premolar , with evidence of ductile fracture due to shear strength , as demonstrated by the first line , followed by compression ( figures 1 to 3 ) . in the second implant , the fracture was characterized by a strong torsional component , with fewer compressive evidences and characteristics of final fracture due to the presence of " dimples " , also called alveolar - like structures . there were no signs of failure in the material or screw fabrication ( figures 4 and 5 ) . studies on the biomechanics of implants have historically concerned about screw - retained prostheses , with limited scientific analysis of cemented prostheses , in spite of their frequent use . the efficacy of cemented dentures has been questioned because it is different from the protocols described by branemark . the ucla abutment has been used to support cemented prostheses in single implants or fixed dentures . there are currently several types of titanium and ceramic abutments for use in prostheses cemented over single implants4 . screwed prostheses have been designed to promote integrity of the connection between prosthesis , abutment and implant . rieder13 ( 1990 ) and later hebel and gajar8 ( 1997 ) stated that esthetics and occlusal function would be the reasons for use of cemented prostheses . it has also been advocated that passive fit between the prosthetic crown and the abutment is more easily obtained in cemented dentures because the cement would fill the interfacial gaps , thus improving the load transmission to the prosthesis / implant / bone system11 . the passivity of restorations is essential for a successful implant - prosthetic rehabilitation48,11,1416 . according to jemt and book9 ( 1996 ) , prosthetic misfit might not be much significant because the average distortion in their study was 100 m and no passivity was observed in the analyzed restorations . in addition , no significant correlation was found between the lack of passivity and bone loss . these authors suggested that misfit is more related to prosthetic failures due to the ankylotic nature of osseointegration . it has been reported that a misfit equal to or higher than 30 m can be considered dubious or unacceptable2 . restorations with 26 m or less of misfit are considered as " passive fit " . guinchet6 compared the integrity and load transmission of cemented and screwed prostheses and concluded that , after function , the marginal gap of screwed dentures was significantly smaller than that of cemented prostheses . the load transmission of screwed prostheses was higher yet transmitted to the apical portion of the implant , while in cemented prostheses it was lower yet concentrated at the coronal portion of the implant . the smaller marginal gap of screwed prostheses is related to a higher load transmission , and the greater marginal gap of cemented prostheses is related to a lower load transmission . there are no research - based evidence of the effect of misfit on osseointegration failure and the risk it poses to implant success . however , there are evidences that misfit increases the occurrence of mechanic failures of components and/or fracture . several studies have addressed the effect of occlusal overload on implants , although no scientifically based data are available . the tolerance between mechanic components allows short movements at the interfaces , and flexural fatigue plays an important role on denture long - term success . the potential of non - axial strengths to cause plastic deformations , wear or failures due to fatigue of prosthetic components is clearly dependent on the design and material used . the placement of implants on posterior mandibular regions , close to the temporomandibular joint , yields an unfavorable condition due to the magnitude of load transmission in this area . guichet , et al.7 ( 2000 ) compared the relationship between marginal discrepancies and the passive fit of screwed and cemented prostheses in fixed partial prostheses by microscopic analysis and did not find differences after final screw torque and cementation . sendyk and sendyk16 ( 1998 ) demonstrated the load distribution of osseointegrated implants using finite element analysis and concluded that load transmission in screwed prostheses is often concentrated at the gold screw attaching the crown to abutment , which acts as a safety device . in cemented prostheses , the cement and abutment screw receive this load , mainly shear forces . in the same study , these authors did not find significant differences between the use of resin or ceramic in the occlusal platform . a previous study3 on the registration of force development with ceramic and acrylic resin occlusal materials on implant - supported prostheses , both in vivo and in vitro , found differences only when load transmission was assessed in vitro . the review of literature on implantology and prosthodontics is not conclusive with regard to the causes of failures and complications associated with prosthetic treatments with osseointegrated implants . the lack of passive fit and generation of shear forces are the most frequently reported causes of biomechanical failures of implants . in the case reported in this article , the causes of fractures were not related to failures on materials or fabrication of the screws , but rather were due to shear forces . the misfit in cemented prostheses may be the most likely cause of shear force generation .

one of the causes of implant failures in cemented implant - retained prostheses is the fracture of abutment screw or ucla abutment . this article reports a case of simultaneous fracture of two ucla abutments screws occurring in an implant - supported prosthesis placed in the mandibular molar region . the fractured structures were examined under scanning electron microscopy to investigate the probable causes of the failure , which were not related to failures on materials or fabrication of the screws , but rather were due to shear forces . the misfit in cemented prostheses may be the most likely cause of shear force generation .

labetalol , a combined alfa and beta - adrenergic receptor antagonist , is an antihypertensive drug frequently used to treat systemic hypertension in acute care settings , including in obstetrics . we present an unusual case characterized by a rise in blood pressure ( bp ) , rather than hypotension , and temporary neural dysfunction following inadvertent intrathecal administration of labetalol during obstetric anesthesia . a 25-year - old woman , parity status p1 + 0 , weighing 50 kg , presented in early labor at term with a history of loss of fetal movement and cesarean section in the earlier pregnancy . on admission , she was fully oriented with a pulse rate of 80/min and bp of 130/70 mmhg , and the fetal heart sounds were equivocal . she was put up for emergency lower segment cesarean section under spinal anesthesia and premedicated with ranitidine 50 mg by intramuscular ( i m ) route and metoclopramide 10 mg i m in the labor room before being brought into the operation theater ( ot ) . in the ot , her vitals were taken which showed pulse 76/min , bp 140/72 mmhg , and oxygen saturation 98% in room air . intravenous ( iv ) cannulation was done with an 18 g cannula and ringer lactate infusion started . spinal anesthesia was administered , in the sitting posture , at the l2-l3 interspace with a 25 g quincke 's spinal needle by the anesthesia junior resident . the intended drug was hyperbaric bupivacaine 0.5% w / v , and the volume administered was 2.6 ml . immediately upon assuming the supine position , the patient started complaining of acute pain in both lower limbs along with intense burning sensation . the situation was rescued by converting to general anesthesia by propofol 100 mg iv , intubating with the help of succinylcholine 50 mg iv and maintaining with nitrous oxide 60% and oxygen 40% inhalation . muscle relaxation was continued with atracurium 25 mg iv in two divided doses ( 20 mg + 5 mg ) . the cesarean section was completed without problems and a 2.4 kg live baby was delivered . relaxation was reversed by neostigmine 2.5 mg iv with glycopyrrolate 400 mcg iv , and nitrous oxide was withdrawn after recovery of spontaneous respiration . the patient was extubated uneventfully and did not complain of any further leg pain or burning sensation after recovery from anesthesia . vital signs remained normal postoperatively , and the patient was shifted to the postnatal ward . the case was not reported to the hospital administration as there is no system of medication error reporting in the institution . the mix - up had occurred in picking up the vial from the anesthetic injection tray on which multiple ampoules and vials were kept . an intern had picked up the ampoule , broken the top and held it while the resident drew up the injection in the syringe without reading the label . the ampoules [ figure 1 ] were similar in size and amber colored although the labeling was distinct . the ampoule of labetalol from which the injection was drawn shown side by side with an ampoule of spinal bupivacaine from which it should have been drawn . they are similar in appearance though labeling is distinct a medication error is any failure in the medicine selection , prescription , dispensing , handling , and use process that may harm the patient . like adverse drug reactions ( adrs ) , medication errors are an inevitable consequence of pharmacotherapy , although , unlike adrs , they are often preventable through individual and institutional measures . though recognized as an important cause of patient mortality and morbidity globally , particularly in operative , emergency , critical care , and other such high pill burden settings , medication errors remain a neglected phenomenon in india , particularly in public hospital context . it may be related to professional practice , products , procedures , environment or systems ; may involve prescribing and ordering ; dispensing and distribution ; preparation and administration ; labeling , packaging , and nomenclature ; communications and education ; or use and monitoring of treatment . commonly implicated are medication factors ( e.g. , similar sounding names , low therapeutic index , polypharmacy ) , patient factors ( e.g. , impaired cognition , vision problems and other physical disabilities , poor renal or hepatic function ) or health system - related factors ( e.g. , use of inappropriate abbreviations and decimal points on prescriptions , failure to read drug labels , overburdening , and fatigue of healthcare providers ) . in our case , the reasons were keeping similar looking ampoules together , failure on the part of the intern to read the label and failure on the part of the resident to cross - check that the correct medication was being injected . labetalol is a dual alfa - and beta - adrenergic antagonist that is often used to treat systemic hypertension in acute care settings . iv overdose of labetalol has been reported to cause hypotension and bradycardia as would be expected from the receptor profile of its activity . therefore , the acute rise of bp , tachycardia , and the intense pain and burning are difficult to explain . theoretically , the unopposed beta - adrenergic blockade can lead to rise in bp by peripheral vasoconstriction mediated through alfa1-adrenergic receptors on vascular smooth muscle . however , this is not expected of labetalol since it simultaneously blocks alfa - receptors . nevertheless , the network of neurotransmission in the central neuraxis is complex and it is possible that a large dose of labetalol may have unexpected receptor activity ( for instance blockade of central alfa2-autoreceptors will increase noradrenaline release from adrenergic synapses ) that transiently caused these events . finally , it is to be noted that a system of medication error reporting does not exist in this hospital , or for that matter , most hospitals in india , and this may lead to such events recurring in future . introducing such a system can be worthwhile and would be an important step toward assessing and reducing the medication error burden . unfortunately , we anticipate that any attempt at the introduction of such a system would be met by resistance out of fear of being identified and subsequently penalized for negligence . two approaches to human error have been described : ( 1 ) the person approach and ( 2 ) the systems approach and they are also applicable to the issue of medication errors . the systems approach accepts that humans are fallible , and therefore , errors may occur regardless of the competence of individuals working within the system . rather than focusing on the individual , it focuses on the conditions under which individuals work and how those conditions can predispose to errors . it is now widely acknowledged that although professionals must take responsibility for their actions , blaming doctors , pharmacists , or nurses for errors does not encourage a culture of reporting or learning . serious errors are often caused by wider defects in the system that lie dormant ( latent conditions ) until they combine with human error to precipitate a serious incident . in order to reduce risk , a system needs to understand its defects so that it can minimize them by building appropriate defenses and safeguards . defects can only be identified if there is a commitment to an open culture of reporting throughout the system .

labetalol , a combined alfa and beta - adrenergic receptor antagonist , is used as an antihypertensive drug . we report a case of an acute rise in blood pressure and lower limb pain due to the inadvertent intrathecal administration of labetalol , mistaking it for bupivacaine , during obstetric anesthesia . the situation was rescued by converting to general anesthesia . the cesarean delivery was uneventful , and mother as well as newborn child showed no ill - effect . this particular medication error was attributable to a failure on the part of the doctors administering the injection to read and cross - check medication labels and the practice of keeping multiple injections together . in the absence of an organized medication error reporting system and action on that basis , such events may recur in future .

a 50-year - old female presented with nonunion of right subtrochanteric fracture and broken lag screw of long cephalomedullary nail ( 30 long it / st nail , zimmer , warsaw , in , usa ) ( fig . gentle traction was applied to the effected leg by using a foot strap attached to the operating table while the other leg was placed on a leg holder in hemilithotomy position . after removal of the distal screw , lateral end of the lag screw , and the nail respectively by a routine technique , the bonescrew interface was reamed manually by using a trephine reamer ( zimmer femoral trephine set , zimmer ) in a corresponding size with the lag screw diameter about a half way of the retained screw length . the customized removal tool was then applied gently following the tract of the trephine reamer until gripping the broken screw firmly . the broken lag screw was then finally removed by anticlockwise turning the removal tool as a screwdriver . nail exchange was carried out sequentially by using another long cephalomedullary nail ( 125 long gamma-3 nail , stryker , schnkirchen , germany ) . with the difference in angle of the lag screw , a healthy subchondral bone was purchased . as a result of multiple previous revision surgery , partially weight bearing was strictly advocated on the injured leg for the first 3 months . a specific socket screwdriver was created by modifying a used 12 40 mm arbeitsgemeinschaft fr osteosynthesefragen ( ao ) femoral nail and another identical lag screw as a template ( fig . this particular device was then tested on an experimental setting in order to confirm its function prior to surgery . a specific socket screwdriver was created by modifying a used 12 40 mm arbeitsgemeinschaft fr osteosynthesefragen ( ao ) femoral nail and another identical lag screw as a template ( fig . this particular device was then tested on an experimental setting in order to confirm its function prior to surgery . the overall incidence of gamma nail breakage is 0.2%-5.7%.4,5,6,7 ) the most common site of implant failure occurred at the lag screw hole , and the rest occurs along the nail shaft and the distal screw hole , respectively . to our knowledge , no report regarding breakage of the lag screw has been identified . therefore , this might be the first report of lag screw breakage in gamma nailing . varus reduction and poor biology of healing from multiple revision surgery in this patient may contribute to a failure of construct over times.8 ) avoidance of using nail cap , which prevents rotation and excessive sliding of lag screw , probably is another factor of inadequate stability and leads to breakage of implant in this patient.9 ) therefore , the planning for another revision surgery is to preserve and enhance the biology of bone healing as much as possible . normally , there is no specific instrument to remove a broken lag screw from the femoral head . using a hooked instrument as an extractor might not be strong enough to overcome the friction at the bone - implant interface . therefore , the principle to solve this particular problem is to create a socket screwdriver that can grip the broken lag screw firmly enough for removal . by preservation of the femoral head bone stock , the bone - implant interface between the shaft of the lag screw was reamed by using a trephine reamer . the trephine reamer was then replaced with the customized socket screwdriver . after griping the lag screw firmly ,

a broken lag screw of the cephalomedullary nail is a rare condition . removal of the retained lag screw from the femoral head is also very challenging . this article describes a surgical technique and the modified instrument that was available in the operating room for removing the broken implant by closed technique .

he had an unremarkable medical history and no family history of epilepsy or sudden cardiac death . brain magnetic resonance imaging , echocardiography ( ecg ) , and 24-hour holter ecg monitoring revealed no abnormal findings . an electroencephalogram led us to suspect a partial seizure disorder originating from the frontal area . subsequently , the patient experienced recurrent syncopal episodes despite taking antiepileptic medication for four years and the absence of epileptic discharges on follow - up electroencephalograms . at 12 years of age , his cardiac function was re - evaluated because he reported chest discomfort before syncopal episodes . echocardiography , 24-hour holter ecg monitoring , and head - up tilt testing revealed normal findings . these findings were consistent with catecholaminergic polymorphic ventricular tachycardia ( cpvt ) , and the patient was started on a beta - blocker . at the age of 15 years , while on the beta - blocker therapy ( metoprolol , 200 mg / day ) , the patient went into cardiac arrest with initial ventricular fibrillation and underwent defibrillation with an automated external defibrillator . an implantable cardioverter defibrillator ( icd ) was inserted , and flecainide was added to his medication regimen . however , after icd insertion , the patient developed recurrent hemodynamically stable ventricular tachycardia ( vt ) , and six shocks were delivered over the course of six months . these episodes of vt were not terminated by shocks from the icd , but instead persisted for some time , eventually terminating spontaneously ( fig . he was referred to the general thoracic department for left cardiac sympathetic denervation ( lcsd ) . intubation was carried out using a dual - lumen endotracheal tube for one - lung ventilation . a 12-mm trocar was inserted on the sixth midaxillary line for the camera , and two 5-mm trocars were inserted on the third and the fifth anterior axillary lines for the endoscopic instruments . the sympathetic chain was identified from t1 to t5 and resected , including the lateral nerve branches , using a harmonic scalpel with sparing of the stellate ganglion ( fig . a 20-fr chest tube was placed in the left pleural cavity at the end of the operation and was removed on the first postoperative day . the patient was discharged on the second postoperative day without complications and maintained on metoprolol ( 300 mg / day ) and flecainide ( 100 mg / day ) . as of one month after the operation , no shocks had been administered by the icd , and an exercise stress test did not induce vt ( fig . cpvt was first described in 1975 , and is characterized by bidirectional or polymorphic ventricular tachycardia induced by increased sympathetic activity secondary to physical exercise or emotional stress . genetic studies in the past decade have revealed mutations in two genes ( ryr2 , casq2 ) that are known to be associated with abnormal intracellular calcium handling and cause adrenergically mediated polymorphic ventricular tachyarrhythmias by delayed after - depolarizations and triggered activity . cpvt presents clinically with syncope , aborted cardiac arrest , or sudden cardiac death , all of which are triggered by physical or emotional stress . therefore , the accurate and early diagnosis of cpvt is critical for achieving the best possible clinical results in these patients . reproducibility of the typical arrhythmia by provocative testing , such as an exercise stress test or an isoproterenol infusion test , is important in establishing the diagnosis . however , cpvt - related syncopal episodes may include seizures involving urinary or fecal incontinence , and usually present with normal ecg findings . therefore , the average delay in diagnosis has been reported to be as high as 2.00.8 years after the manifestation of the first symptoms . our patient s condition was initially mis - diagnosed as epilepsy , and antiepileptic medication was administered for four years , which resulted in a delayed diagnosis of cpvt . beta - blockers are the first - line therapy for cpvt , and they are known to provide most patients with sufficient protection . since fatal ventricular storms can be initiated by shocks from an icd that trigger subsequent catecholamine release , icd placement should only be considered in patients who have been resuscitated after cardiac arrest and who have experienced recurring syncope or sustained vt despite beta - blocker therapy . other pharmacologic treatments ( sodium channel blockers , amiodarone , verapamil ) have been shown to have an additive benefit in combination with beta - blockers . lcsd can be considered when ventricular arrhythmias remain uncontrolled by beta - blockers and other additive drug therapies . . first reported that lcsd is an effective therapy for cpvt , several other researchers have confirmed that lcsd is highly effective and safe in severely affected cpvt patients . moreover , hofferberth et al . presented the results of thoracoscopic lcsd in 24 patients with life - threatening ventricular arrhythmias , including nine patients with cpvt . in that study , 16 of the 22 patients who underwent long - term follow - up experienced a marked reduction in arrhythmic events , and 12 patients were completely free of arrhythmia . moreover , no major complications and only three minor complications ( two cases of pneumothorax and one case of harlequin syndrome ) were reported . the author had previously reported that left stellectomy in dogs , which is the equivalent of lcsd in humans , raised the ventricular fibrillation threshold , whereas right stellectomy lowered the ventricular fibrillation threshold . other studies have reported the following findings : ( 1 ) left ventricular contractility is not reduced after left stellectomy ; ( 2 ) no evidence indicates that lcsd induces post - denervation supersensitivity ; ( 3 ) left stellectomy increases reactive hyperemia via the coronary artery ; ( 4 ) after lcsd , the heart rate increases more than usual ; and ( 5 ) lcsd increases the activity of the cardiac vagal efferent nerve . clinical evidence has indicated that lcsd may have a role in treating patients with channelopathies such as long qt syndrome , previous myocardial infarction , and cpvt . in summary , cpvt is a genetic disorder involving intracellular calcium handling that leads to adrenergically - mediated life threatening ventricular tachyarrhythmias . although beta - blockers are the gold standard of therapy in patients with cpvt , thoracoscopic lcsd is safe and can be an effective alternative treatment option . lcsd is a particularly important option for patients for whom beta - blockers are contraindicated and in patients who experience recurrent icd shocks despite optimal treatment with medication .

a patient presented with loss of consciousness and conversion . during an exercise test , catecholaminergic polymorphic ventricular tachycardia ( cpvt ) resulted in cardiac arrest . he started taking medication ( a beta - blocker and flecainide ) and an implantable cardioverter defibrillator ( icd ) was inserted , but the ventricular tachycardia did not resolve . left cardiac sympathetic denervation ( lcsd ) was then performed under general anesthesia , and the patient was discharged on the second postoperative day without complications . one month after the operation , no shock had been administered by the icd , and an exercise stress test did not induce ventricular tachycardia . although beta - blockers are the gold standard of therapy in patients with cpvt , thoracoscopic lcsd is safe and can be an effective alternative treatment option for patients with intractable cpvt .

subconjunctival emphysema , which has rarely been reported in the literature because of its uneventful course,1 is associated mostly with orbital emphysema in cases of medial orbital wall fractures due to a newly created sino - orbital communication.2,3 while orbital emphysema may cause compressive optic neuropathy4 in severe cases , subconjunctival emphysema may cause exposure keratopathy when the ability to close the lids is compromised . the patient described here developed simultaneous orbital , subconjunctival , and subcutaneous emphysema after sustaining a small fracture of the orbital floor due to a sudden elevation in intranasal pressure . a 16-year - old boy sustained a trauma to the left orbit as the result of a fistfight . a few hours later , he developed sudden left periorbital pain , swelling , and proptosis after an episode of sneezing . he presented to the emergency department where an ophthalmologic examination showed left subcutaneous and subconjunctival emphysema ( figure 1 ) . the uncorrected visual acuity was 20/30 ( 0.67 ) , and the pupillary responses were normal . there were a mild limitation in left eye levoduction and supraduction with an inability to close the lids , but no paresthesia of the cheek or teeth . there was 3 mm of proptosis in the left eye compared with the right eye . an orbital computed tomography scan showed a left linear , nondisplaced fracture of the orbital floor associated with peribulbar and retrobulbar emphysema without muscle entrapment or fat herniation . the emphysema caused medial displacement of the left lateral and inferior rectus muscles with mild proptosis ( figures 2 and 3 ) . uncorrected visual acuity in the right eye was 20/20 ( 1.0 ) and was normal upon examination and radiologically . the patient was treated with prophylactic broad - spectrum oral antibiotics , a topical antiglaucoma drug ( timolol 0.5% twice daily ) , and lubricating eye drops ( four times daily ) , and was instructed not to blow his nose and to return for a follow - up examination the next morning . after daily follow - up , there was dramatic improvement in the subcutaneous and subconjunctival emphysema and vision , with total resolution of the subconjunctival emphysema on the fifth day with no sequelae . orbital emphysema most often results from a fracture of the orbital wall that allows air to enter the orbit from the paranasal sinuses.5 the presence of orbital , subconjunctival , and subcutaneous emphysema may be due to air from the nasal cavity entering between the loose tissue planes of the orbit after fractures of the orbital walls.3 subconjunctival emphysema can be explained by probable communication between the subcutaneous and subconjunctival planes6 or the probable spread from orbital emphysema through the orbital septum when the intraorbital pressure is high . subconjunctival emphysema has been reported in patients on mechanical ventilation6 and after direct injury to the conjunctiva through macroscopic or microscopic conjunctival lacerations associated with injury due to compressed air.710 subcutaneous emphysema usually occurs during a brief period after trauma when air can be forced into the tissues . after this , the escape routes are sealed by blood clots or herniated tissues in the sinuses at the fracture sites.3 the current case was unusual in that it had all three types of emphysema ( figure 2 ) with a small fracture of the orbital floor ( figure 3 ) . weber et al reported the occurrence of orbital and subconjunctival emphysema after orbital floor fracture in a patient after blowing his nose , but without subcutaneous emphysema.11 to the best of the author s knowledge , this is the first reported case of simultaneous subcutaneous , subconjunctival , and orbital emphysema associated with orbital floor fracture . because of the location of the orbital floor fracture , the initial air was more likely in the orbit , and with the subsequent sneezing and increased intraorbital pressure , direct spread occurred from the orbit into the subconjunctival and later into the subcutaneous planes , although the orbital septum usually forms a barrier between these potential spaces,6 which might be torn by blunt trauma . a prolonged increase in intraocular pressure can lead to optic nerve damage from ischemia similar to compartment syndromes in 12 hours if the intraocular pressure approaches ophthalmic artery perfusion pressure.12 previous reports have described increases in intraocular pressure to 28 mmhg.8 in the current patient , the intraocular pressure increased to 26 mmhg and he was treated with topical timolol 0.5% twice daily , with return of the intraocular pressure to 13 mmhg with treatment and on resolution of the emphysema . cartwright et al4 also reported that an elevation in intraocular pressure after orbital trauma with orbital emphysema is usually not severe or long enough to result in visual compromise but requires continuous monitoring . most orbital emphysema requires no specific treatment , since the process is self - limiting , with an approximate duration of 2 weeks.3,13 attention must be paid to prevent compressive optic neuropathy and exposure keratopathy . although a good visual outcome is common , serious vision - threatening sequelae have been reported.10 increases in intraorbital pressure may cause damage to the blood supply of the optic nerve , leading to optic atrophy and poor vision14 or central retinal artery occlusion.15 the surgical treatment of orbital emphysema includes lateral canthotomy or cantholysis , orbital decompression by needle aspiration , and bone decompression.11,16,17 therefore , the presence of any type of periocular emphysema after blunt trauma should raise suspicion for orbital blowout fracture and mandates orbital computed tomography with careful follow - up of the patient .

a 16-year - old boy presented to the emergency department with the complaint of a sudden , painful left eye and proptosis after an episode of sneezing . a few hours earlier , he had sustained a blunt trauma to the left orbit as the result of a fistfight . the initial examination showed subcutaneous and subconjunctival emphysema . visual acuity in the left eye was 20/30 ( 0.67 ) , the pupils were reactive with no relative afferent pupillary defect , and there were mild limitations in levoduction and supraduction . a slit - lamp examination showed normal anterior and posterior segments with an intraocular pressure of 26 mmhg . an orbital computed tomography scan showed orbital , subconjunctival , and subcutaneous emphysema associated with a small fracture of the orbital floor . following conservative management with broad - spectrum oral antibiotics , a topical antiglaucoma drug , and lubricating eye drops , the patient improved dramatically within one week .

in the field of dentistry , gingival hypertrophy ( gh ) is a common presenting complaint , especially among patients belonging to pediatric age group . gingivitis is a commonly occurring phenomenon since the oral cavity is a potential site for active infection as it is constantly exposed to pathogenic microorganisms . the prevalence of gh among school going children is nearly 14% , inflammatory origin being the most common cause followed by chronic drug intake . hence , there is a tendency among us to consider gh as a mere inflammatory response to local infection rather than considering systemic illnesses ; this can eventually lead to delay in diagnosis and initiating appropriate treatment . gh due to acute myeloid leukemia ( aml ) in a child is extremely rare . here , we report a case of aml ( subtype m5 of fab classification ) in a 3-year - old male child wherein the disease primarily presented as gh . a 3-year - old male child presented to us with fever and body pain for 2 weeks , gum swelling for 1-month duration , initially presented to a pediatrician and a dentist , for which symptomatic treatment was given . the child was again reviewed with the pediatrician after 1 week with persisting symptoms . no history of halitosis or dental caries . on general examination , he had pallor ; there was no icterus , cyanosis , clubbing , pedal edema , or generalized lymphadenopathy . oral examination revealed the enlargement of maxillary and mandibular gingiva covering two - third of the crown structure in buccal , lingual , and palatal aspects . gingiva was reddish with the loss of stippling , soft , spongy , bleeding on touch but was nontender and not warm [ figure 1 ] . on abdominal examination , the liver was palpable 2.5 cm below the right costal margin , and the spleen was palpable 2 cm beyond the left costal margin . g / l , total white blood cell count was 5.6 10/l with 90% blast cells and 10% lymphocytes , and platelet count was 0.9 10/l . his renal and liver function tests were within the normal range ; uric acid and lactate dehydrogenase levels were also normal . bone marrow aspirate smear showed blasts which are large with moderate amount of agranular to granular cytoplasm and round to irregular nucleus with fine chromatin and 13 nucleoli . few of the blasts showed auer rods [ figure 2 ] . in flow cytometric analysis , the blasts were positive for cd33 , cd117 , human leukocyte antigen - dr , and cd13 . parents were counseled about his condition , treatment , and outcome of the disease . due to long distance commuting issues , the parents opted for the child to receive treatment elsewhere ; the child was lost to follow - up . most common causes of gh in children are poor dental hygiene and chronic drug intakes such as phenytoin , cyclosporine , and calcium channel blockers . systemic illnesses such as wegener 's granulomatosis , sarcoidosis , crohn 's disease , infantile systemic hyalinosis , tuberculosis , and hereditary gingival hyperplasia should also be considered in the differential diagnosis . acute leukemia , which is the most common malignant disorder in children , is characterized by neoplastic proliferation of blast cells causing accumulation of > 20% blast cells in the bone marrow , interfering hematopoiesis resulting in fatigability , bleeding tendency , and infections . acute leukemia is subdivided into acute lymphoblastic leukemia and acute myeloid leukemia ( aml ) , based on the phenotype of the blast . aml in a child generally presents with fatigue , pallor , abnormal bleeding and infections with or without splenomegaly and lymphadenopathy ; however rarely , it can present with infiltration of blasts cells in tissues such as skin , mucosa , and gums . literature review has shown only few studies that have reported cases of aml presenting with oral lesion as a primary manifestation . oral manifestations in leukemia are gh , oral ulcers , pale mucosa , herpes , and candida infections . gingival infiltration in aml is rare , usually associated with subtypes m4 and m5 ( fab classification ) . it is due to infiltration of tissues with neoplastic precursors of myeloid cells or secondary to thrombocytopenia , neutropenia , or impaired granulocyte function . hence , gingival tissue , although unusual , is one of the sites that should be monitored for relapse . although confirmatory diagnosis and treatment of aml is primarily done by the pediatric hemato - oncologist , it is almost always a dentist or a pediatrician who first attend to cases of gh , and hence increasing awareness among them will aid in early detection and prompt referral and drastically improve the outcome of the disease . finally , any gh , particularly when associated with hepatosplenomegaly and abnormal blood counts should raise the suspicion of possible underlying hematological malignancy in the child .

acute leukemia is the most common malignant disorder of childhood . acute leukemia is characterized by marrow failure due to the replacement of marrow elements by leukemic blasts . gingival hypertrophy ( gh ) due to acute myeloid leukemia ( aml ) in a child is extremely rare . here , we report a case of aml ( subtype m5 of fab classification ) in a 3-year - old male child wherein the disease primarily presented as gh .

a 33-year - old male was born with transposition of the great arteries ( tga ) . at the age of 1 month , he underwent rashkind septostomy . there was also unroofing of the coronary sinus and persistent draining of the left superior caval vein directly to the left atrium between the appendage and the left pulmonary veins . there was also a large opening at the expected site of the mouth of the coronary sinus . taking into account this anatomic variation , a modified senning operation was performed by using the left atrial appendage for the construction of the new atrial pathway so that the blood of both the left superior caval vein and the coronary sinus would be drained to the newly created venous atrium ( fig . his postoperative course was uncomplicated , and he was discharged from the hospital on postoperative day 10 . during his regular follow - up , 33 years after the operation , he remains without symptoms in new york heart association ( nyha ) class i. the quality of his life is excellent , and he is attaining a normal level of education and employment . he is in sinus rhythm , and the cardiac echocardiography ( including transesophageal echo ) and cardiac magnetic resonance imaging show mild distention of the right arterial atrium with trivial regurgitation of the tricuspid valve ( systemic atrioventricular valve ) . the left ( subpulmonary ) ventricle has normal function with an ejection fraction of 65% , while the right ( systemic ) ventricle shows mild distention and an ejection fraction of 68% . the aortic and pulmonary valves are competent , and the baffle is completely patent without leaks . the caval and the pulmonary venous system showed no signs of stenosis or thrombosis ( figs . 2 , 3 ) . the senning procedure was the first operation performed in patients with tga and changed the survival of these patients . later , it was replaced by the arterial switch operation , which provides anatomic and physiologic repair . despite the overall good outcomes after atrial level repairs , arrhythmias , systemic right ventricular dysfunction , systemic atrioventricular valve regurgitation , subpulmonary stenosis , and baffle problems are well - described complications of post - atrial switch techniques . the use of the left atrial appendage for the construction of the new atrial pathway in the senning procedure has rarely been described in the past [ 46 ] . in particular , to the best of our knowledge , only two papers have been published in english that report on the senning procedure with the use of only autogenous tissue ( that is , the left atrial appendage ) . several studies have described the long - term outcomes of patients with mustard and senning repairs . late survival is 84% to 95% at 10 years , 76% to 89% at 15 to 20 years , and 79.3% at 30 years . most of these studies report that 90% to 95% of the patients are in nyha class i or ii and almost all survivors are able to work and function normally in their social life . despite the overall good outcomes after atrial - level repairs sinus node dysfunction , atrial flutter , sudden death , systemic right ventricular dysfunction , systemic atrioventricular valve regurgitation , subpulmonary stenosis , pulmonary hypertension , and baffle problems are well - described complications after these types of operations . moons et al . , in a retrospective review , reported that patients in the senning cohort had a slightly better survival rate than in the mustard cohort . baffle obstruction occurred more often in the mustard group , and arrhythmia - free survival did not differ between these two cohorts , although this might be determined by the complexity of the transposition . in addition , survivors after senning repair had better functional status and tended to engage in more athletic activities . senning repair was the procedure of choice in our institution , with similar long - term results . our patient , 33 years after the modified senning repair , in an echocardiographic study , presents trivial systemic atrioventricular valve regurgitation and a mild distention of the right arterial atrium . although severe tricuspid valve regurgitation was more prevalent in the senning cohort and was positively correlated with the systemic ventricular function , the systemic right ventricular function is excellent and the patient remains in sinus rhythm . it seems that sub - optimum myocardial protection during cardiopulmonary bypass may be a contributing factor to right ventricular dysfunction . several modifications of the senning procedure using various materials ( autogenous or not ) for the construction of the new atrial pathway have been described in the literature [ 46 ] . the left atrial appendage is an alternative in some cases where the septal defect is large enough or anatomic variations are present , such as an unroofed coronary sinus . the inverted left atrial appendage could be incised and can be used as a flap for creating the atrial pathway [ 46 ] . in our technique , the base of the left appendage was invaginated and was used to create the septal flap in order to isolate the pathway of the pulmonary veins from the overhead space . however , thus far , we have never performed this technique , as we have never encountered this anatomic variation in the tga setting . this technique possibly has another advantage of the potential growth of the autologous tissue , and the created flap seems to participate in atrial contractility . throughout the years , this modification of the senning procedure has been shown to be effective , and the long - term outcome of this patient is excellent . this modified technique could possibly generate renewed interest in atrial baffle procedures as part of a contemporary repair strategy for hearts with congenitally corrected tga .

numerous technical modifications and various complications of the senning procedure have been described in the literature . we describe the excellent clinical status and anatomic result of a 33-year - old patient who underwent a modified senning operation using the left atrial appendage for reconstruction more than 30 years prior to presentation .

in children , acute myeloid leukemia ( aml ) accounts for 15% of all leukemias . extramedullary leukemia deposits or granulocytic sarcoma ( gs ) is a rare manifestation in about 3% of cases and 9.3% of gs manifested as orbit deposit causing proptosis in one or both eyes . moreover , especially in childhood aml , the simultaneous presence of both proptosis and facial palsy have not been previously reported anywhere to the best of our knowledge . in this report , such unusual presentation is being reported . a 3-year old boy presented to our outpatient department ( opd ) with progressive proptosis of left eye for the last 3 months and left facial palsy for last 20 days . on examination , he was found to be moderately anemic without petechial patches and had loss of appetite and weight . his hemoglobin was 7.0gm% and the total leukocyte count was 8000/mm3 , differentiated leukocyte count showed 28% neutrophils , 48% lymphocytes , 12% blast and platelet count was 20,000/mm3 . the contrast ct scan / mri of orbits showed polypoidal soft tissue mass in the left maxillary antrum showing pressure erosions of smooth thinning of the medial , posterolateral walls and boxy orbital floor at places extending into the inferior extraconal space of the left orbit with mild proptosis . the bone marrow aspiration showed hypercellular marrow smears ; 15% blast , 45% promyelocytes with increased myeloid erythroid ( m : e ) ratio conforming to the diagnosis of acute promyelocytic leukemia ( aml , m3 ) . incisional biopsy of the orbital swelling was avoided in view of the low platelet count and imaging report correlating with leukaemic proptosis . thus , the patient was diagnosed as aml , m3 with extra- medullary disease involving left orbit and left facial nerve palsy . after second cycle of chemotherapy induction cycle comprising of injection daunorubicin 45g / m2 and injection cytarabine 200mg granulocytic sarcoma even though a rare presentation can present from infancy to old age affecting commonly children and young adults . these tumors can present prior to or concomitantly or even during remission of systemic leukemia showing its variable natural history [ 2 , 3 ] . in cases with head and neck involvement , they commonly affect the orbit or epidural space and proptosis being the most common presenting feature . murthy et al has reported 12 cases of extra medullary leukaemia in children who presented with only proptosis . in our case the patient was noted to have orbital disease , proptosis , facial palsy and systemic involvement concurrently . most of the reported cases have decreased visual acuity and restricted extra - ocular movements unlike our case . early detection of the leukemia as well as limited orbital extension of the leukemia infiltration may be the reason . when there are no signs of systemic leukemia , the diagnosis of this tumor can be challenging . this tumor can also be confused with rhabdomyosarcoma , neuroblastoma and lymphoma . in case where the orbital tumor is the initial manifestation , peripheral blood and bone marrow involvement usually occurs within a year of the occurrence of orbital disease [ 1 , 3 ] . cranial neuropathies , especially of the seventh nerve , occur in children with central nervous systemic leukemia caused by leukemic infiltration ; however , they are extremely rare as the presenting sign of the disease . other than cns infiltration , acute otomastoiditis subsequent to leukemic infiltration of the temporal bone may be implicated with facial and acaustic nerve paralysis . our patient had both rare clinical presentations of proptosis and facial palsy without systemic sign and symptom of aml . even though peripheral smear is an invaluable tool in diagnosing the systemic form of aml , smear may not always be associated with a leukocytosis or immature cells in the leukaemic proptosis . therefore , advising for bone marrow aspirate or biopsy in all patients manifesting with proptosis in the pediatric age is very much justifiable . previous reports have highlighted the role of non invasive test to diagnose such cases of childhood proptosis [ 8 , 9 ] , complete hemogram and bone marrow examination were advised before any local invasive test to confirm the case of proptosis in our case . the extra- medullary leukemia on ct scan may appear as well defined lesion , isodense to muscles within the orbits and rarely extension to the paranasal sinuses as seen in our patient . chemotherapy is the mainstay of treatment but allogenic bone marrow transplantation from a matched family donor still remains the best long - term option that provides remission - free survival for most patients . in conclusion , simultaneous presence of both proptosis and facial palsy in childhood aml , is very rare clinical presentation and possibility of granulocytic sarcoma as one of the causes should be kept in mind . non- invasive investigations like complete hemogram along with bone marrow aspirate even if the complete hemogram is within normal limits should be performed for early detection and early treatment of this disease .

simultaneous proptosis and facial palsy as the clinical presentation of childhoodacute myeloid leukaemia ( aml ) is very rare . to date , no case have beenreported anywhere to the best of our knowledge . extra medullary leukemic deposits or granulocytic sarcoma ( gs ) is a rare manifestation in about 3% of childhood aml , 9.3% of gs manifested as orbit deposits causing proptosis in one or both eyes . cns infiltration or acute otomastoiditis subsequent to leukemic infiltration of the temporal bone may be implicated with facial paralysis . we are here with reporting the case in a 3-year - old boy who presented with proptosis and facial palsy in a case of aml . the purpose of reporting this case is to emphasize the need of examining the peripheral blood and bone marrow in children presenting as proptosis and facial palsy for early diagnosis of childhood aml .

the exact etiology of the syndrome is an enigma even today , since its first description in 1955 when frank gardner and louis diamond described four adult women with painful ecchymosed lesions , reproducible after an intradermal injection of the patient 's own red blood cell ( rbc ) , and suggested a theory of hypersensitivity to erythrocyte as the cause of its occurrence . , this theory has never been proven , and the usefulness of the test itself remains controversial . later , agle and ratnoff noted the psychological factors underlying the disorder and renamed the syndrome psychogenic purpura ; however , the mechanism of this syndrome still remains unknown . autoerythrocyte sensitization syndrome usually affects women , although occasional reports in men and children are present in the literature.[14 ] in this syndrome , bruises can develop anywhere on the body but are usually located on the extremities . there is usually a prodrome of warmth and pain at the bruising site or systemic symptoms such as headache , nausea , or vomiting . the typical skin lesion is a painful , erythematous bruise that starts after minor trauma or surgery and often involves an area away from the injury site . we report a patient with gardner diamond syndrome who was also having depressive features with amelioration of both depressive symptoms and the ecchymosed lesions with antidepressant treatment . a 25-year - old young , married woman was referred to the psychiatry outpatient department by the dermatologist . her predominant symptoms included sadness of mood , helplessness , hopelessness , feeling of lethargy , and inability to do her routine household work . this created interpersonal problems with her in - laws who considered her to be slow in her work and would repeatedly taunt her . the patient was married young and she still had difficulty in adjusting in her in - laws ' home . she had also lost her mother at a young age and was unable to share her marital problems with any of her relatives including her siblings . the ongoing continuous interpersonal stressors had now affected her sleep and appetite , and since a year , she had also become very irritable . it was during one of her visits to her dermatologist that she voiced her grievances due to which she was referred to psychiatry . the detailed psychiatry evaluation revealed the patient to be suffering from a mild depressive disorder without somatic symptoms as per the icd10 criteria . she had recently developed symptoms of bruising associated with pain over arms and face , occurring recurrently after a fall from the stairs for which she was under treatment from the dermatologist and was diagnosed as having gardner diamond syndrome . these symptoms of bruising and associated pain would occur nearly on a daily basis on either her arms , forearms , or predominantly over her cheeks [ figure 1 ] . they would resolve over a week and new lesions would crop up . as per the dermatologist , there was no known treatment for this syndrome , and the patient was prescribed analgesics for the symptoms of pain . in view of her depressive symptoms , escitalopram 5 mg at night , which was gradually increased to 10 mg over the next fortnight . what was surprising was that as her depression improved and the patient started feeling better , her symptoms of recurrent bruising and pain completely subsided and in her follow - up of 2 months , the patient is currently symptom free , maintained on the antidepressant . ecchymosed bruising on arm gardner and diamond believed that the syndrome was due to auto - sensitization , as has been proposed for lupus erythematosus . other causes include a number of hematologic and immunologic abnormalities . among the various postulated etiologic factors , it is now well established that the syndrome occurs in patients with various psychiatric disorders such as depression , anxiety , difficulties in handling aggression , or obsessive compulsive disorder , among others . agle and ratnoff described prominent features of conversion disorder and histrionic personality disorder , as well as sadomasochistic relationships , with frank abuse by significant others . abnormal fibrin degradation and platelet dysfunction have been proposed as the underlying stress - related bruising tendencies . the associated complaints in such patients also include neurological and ocular symptoms , hemorrhagic manifestations , menorrhagia , and a varied symptomatology . although our patient had only depressive features there were no neurological or haemorrhagic abnormalities were observed . the dramatic response to antidepressants which also helped in the resolution of the bruising episodes shows the association between the psyche and skin . researchers have tried to establish the pathways by which stress and emotion affect the nervous system , immune system , and hormonal system and their subsequent effects on inflammation and autonomic functioning and on the skin . stress can induce or exacerbate anxiety disorders or depression in susceptible individuals . around 30% of patients with skin disorders the rapidly developing science of psychoimmunology has revealed the intricacies of the neuroimmunocutaneous endocrine network , which stands as the latest rigid etiological hypothesis and therefore could explain the response of anxiolytics and antidepressant medication in these patients . selective serotonin reuptake inhibitors exert their effect through antihistaminic , anticholinergic , and serotonin blocking properties and this could have led to improvement in our patient 's symptoms . an increased awareness about psychocutaneous disorders and a team approach to treatment often lead to improved patient outcomes as seen in our case . however , psychodermatology clinics , training opportunities for physicians and residents in psychiatry and dermatology residency programs , and family education are some of the important methods to improve better understanding and management of psychocutaneous disorders .

we describe the clinical presentation of a 25-year - old female patient who presented in dermatology with recurrent episodes of painful ecchymotic bruising over the anterior aspect of both arms and face . on enquiry , these episodes were precipitated by emotional stress and were preceded with a history of fall from the stairs . the patient also had multiple stressors in her day - to - day life and symptoms of depression . a diagnosis of mild depressive disorder without somatic complaints and gardner diamond syndrome was made . the patient was started on antidepressants , which not only improved her mood symptoms but also caused a remission of her painful bruises .

a 54-year - old man presented with a 3-month history of cough and shortness of breath during exercise . on physical examination , he had an audible expiratory stridor . computed tomography ( ct ) of the neck and chest revealed a 1.5 cm polypoid tumor arising from the posterior wall of the upper thoracic trachea ( fig . 1 ) . a flexible bronchoscopy showed a double cone shaped , polypoid tumor arising from the posterior wall of the trachea , 5 cm distal to the vocal cords , and 7 cm proximal to the carina ( fig . the mass was diagnosed as a glomus tumor . to check for an extension of the tumor into the surrounding tissue , an endoscopic ultrasonic exam was performed , and it showed that the tumor was confined to the trachea . since the tumor was located in the upper trachea , we decided to approach the tumor through a cervical collar incision . after exposure of the trachea , the location of the tumor was identified between the 5th and 6th tracheal rings using flexible bronchoscopy . we divided the trachea one cartilage ring below the tumor and a sterile laryngectomy tube was inserted into the distal trachea . anastomotic sutures were placed using interrupted 4 - 0 vicryl sutures ( ethicon , somerville , nj ) . the surgical specimen was 3 cm in length with a luminal diameter of 2.3 cm . there was a 1.31.2 cm blue - red protruding nodule on the mucosal surface of the trachea ( fig . 3 ) . the tumor did not show an infiltrative growth into the tracheal cartilage . at the microscopic level , the tumor showed glomus cells , vascular structures , and smooth muscle tissue ( fig . the glomus cells had undergone a transition to elongated , mature smooth muscle cells in some foci ( fig . the tumor cells showed positive reactions for smooth muscle actin and cd34 , but negative reactions for s-100 and cytokeratin on immunohistochemical staining . glomus tumors are uncommon neoplasms , which are typically seen in the deep dermis or subcutis of the extremities , most commonly in the subungual region of the finger . however , these tumors also occur at sites where normal glomus bodies may be sparse or even absent , such as the patella , chest wall , bone , stomach , colon , nerve , eyelid , nose , mediastinum , and trachea . histologically and ultrastructurally , the tumor cells have varying proportions of glomus cells , vascular structures , and smooth muscle tissue . according to the relative proportions , they have been divided into three subtypes : glomus tumor proper , glomangioma , and glomangiomyoma . glomangiomyomas account for less than 10% of all glomus tumors and are the least frequent type . the overall pattern of glomangiomyomas may be identical to that of an ordinary glomus tumor or a glomangioma . however , there is a gradual transition from glomus cells to elongated , mature smooth muscle cells . for the tumor presented here , the initial bronchoscopic biopsy specimen diagnosed the tumor as a glomus tumor . however , the resected tumor had features of a glomangiomyoma such as glomus cells , vascular structures , and smooth muscle tissue , and the glomus cells undergo gradual transition to smooth muscle cells in some foci . these findings are supported by various immunohistochemical studies in which glomus tumors show positive reactions for smooth muscle actin and cd34 , and negative reactions for s-100 and cytokeratin . since masson first described this tumor in 1924 , sporadic reports of glomus tumors of varying types and locations have been reported in the literature . while the trachea is a very rare site for this tumor , segmental resection of the trachea with primary reconstruction is the treatment of choice for these tumors . for some patients , bronchoscopic resection of the tumor or tracheal stenting was useful in relieving airway obstruction before operation . bronchoscopic resection and laser surgery should be reserved for high - risk patients , as local recurrence may occur .

a glomus tumor is an uncommon soft tissue tumor that is most commonly found in the subungual area and a glomus originating in the trachea is extremely rare . histologically and ultrastructurally , these tumors have been divided into three subtypes : classic glomus tumors , glomangiomas , and glomangiomyomas . glomangiomyomas account for less than 10% of all glomus tumors and are the least common type . we report a case of a 54-year - old man with glomangiomyoma of the trachea who presented with stridor . we treated the tumor by segmental resection and primary repair via a transcervical approach .

aplasia cutis congenita ( acc ) , also known as congenital absence of skin , is a group of disorders characterized by the absence of a skin fragment at birth . the first case was reported by cordon in 1767 , who described a lesion located on the upper limb of a child . trichoscopy revealed hair bulbs , visible through the semitranslucent epidermis , and a vascular network . ( a ) arrow indicating the location of the hypopigmented plaque ; ( b ) dermoscopy 20 magnification : translucent appearance of the epidermis , visible hair bulbs , lack of skin appendages , hair roots in the peripheral area and vessels hair loss is a frequent complaint in children , and the most common causes are tinea capitis , alopecia areata , telogen effluvium , and trichotillomania . furthermore , uncommon causes include atopic dermatitis , folliculitis decalvans , congenital ichthyosis , nevus sebaceous , and acc . it can affect the epidermis and upper dermis , with minimal alopecic scarring ; but it can likewise extend to deep dermis , to subcutaneous tissue or even to the periosteum , the skull , and the dura . most lesions occur on the scalp vertex laterally to the midline . generally , it is a solitary lesion , despite the possibility of multiple lesions and even symmetrical presentation . frieden proposed a classification system for acc based on the number and location of the lesions and the presence or absence of associated malformations . histological examination of acc shows a thin layer of dermal collagen without overlying epithelium or adnexal structures . imaging studies are recommended for atypical or very large scalp defects , to evaluate possible underlying bone , vascular , or soft tissue defects . other conditions can have a similar presentation , such as nevus sebaceous , herpes simplex , epidermolysis bullosa , and trauma . if there is a pattern of findings suggesting a genetic disorder , chromosome analysis may be carried out . recently , trichoscopy has gained importance for the diagnosis of both melanocytic and nonmelanocytic lesions . rakowska et al . described a radial arrangement of hair shafts , visible elongated hair bulbs with darkly pigmented proximal ends through the semitranslucent epidermis , prominent vessels which correlate with skin atrophy and no follicular openings at the center of the lesion . have compared trichoscopy aspects of sebaceous nevus and acc to help in the differential diagnosis : while acc shows a complete lack of skin appendages and a translucent appearance , sebaceous nevus shows sebaceous gland not associated with hair follicles . these findings indicate that the diagnosis of acc can be carried out easily and promptly using trichoscopy .

aplasia cutis congenita ( acc ) is a rare disorder characterized by localized absence of skin that most commonly affects the scalp . we present a case of acc in a 45-day - old girl and the dermoscopic findings . dermoscopy has shown to be an easy , fast and useful method for the diagnosis of this condition .

lichen striatus ( ls ) is an uncommon , asymptomatic , self - limiting dermatosis which lasts an average of nine months and usually occurs in children , although it is also rarely seen in adults . it often arises without a clear trigger , but some cases would appear to be related to several possible precipitating events such as infections and , more rarely , cutaneous injury , trauma , hypersensitivity , or other unspecified factors . ls typically presents as a continuous or interrupted linear band of pink , tan , red , or skin - colored papules that follow blaschko 's lines , which represent the pathways of cutaneous cell migration during embryonic development . the lesions are generally solitary and unilateral , but unusual cases with multiple and bilateral lesions have been rarely reported . however , this therapy is not always effective and its prolonged use may be associated with certain adverse effects including cutaneous atrophy . other anecdotal therapies include oral corticosteroid , photodynamic therapy , topical calcineurin inhibitors , and oral acitretin . we report a case of ls unresponsive to topical steroid therapy associated with psoriasis vulgaris , successfully treated with oral acitretin . a 61-year - old woman was admitted for an asymptomatic linear skin eruption of the right lower limb that had started about four weeks earlier . during this period of time , the lesions had been treated with a topical corticosteroid ( mometasone furoate cream 0.1% once a day ) without any significant improvement . skin examination revealed linearly arranged erythematous papules on and along the right lower limb following the line of blaschko [ figure 1a ] . histopathologic examination of a papule revealed parakeratosis , acanthosis , piecemeal disappearance of the stratum granulosum , lymphocytic exocytosis , mild spongiosis , and a superficial and deep perivascular , lichenoid , perifollicular , and perieccrine infiltrate of lymphocytes and histiocytes [ figure 1b ] . on the basis of the clinical and histological data , a diagnosis of ls was made . in addition to ls , she had chronic plaque psoriasis of 15 years duration mainly localized to the elbows and scalp that had progressively extended to her arms and trunk [ figure 2a ] over the past three months . for this psoriatic exacerbation , the patient had been applying an ointment containing calcipotriol 50 g / g and betamethasone dipropionate 0.5 mg / g ( once a day ) with only slight improvement . since the woman had already used methotrexate and cyclosporine in the past with poor tolerance , we decided to start oral acitretin ( 0.5 mg / kg / day ) . interestingly , we found that ls regressed completely and rapidly after only four weeks [ figure 2b ] . the patient continued therapy with acitretin at the same dosage for other two weeks ; thereafter , the drug was tapered over the subsequent four weeks due to resolution of psoriatic lesions . no recurrence of ls and psoriasis was observed during the subsequent four - month follow - up period . linearly arranged erythematous papules on and along the right lower limb following the line of blaschko ( a ) parakeratosis , acanthosis , piecewise disappearance of the stratum granulosum and spongiosis . inflammatory chronic infiltrates is also observed in the epidermis and around the vessels and skin appendages in the superficial dermis ( h and e , 20 ) ( b ) several psoriatic plaques of the back ( a ) complete regression of lichen striatus after 4 weeks of oral acitretin therapy ( b ) the association between ls and psoriasis is a rare event , since , to the best of our knowledge , there are only two well - documented published reports in the literature . the first case was a 2-year - old boy with a ls localized to his left half of the body who developed an unusual form of unilateral eruptive psoriasis limited to the right half of the body 2 weeks later . the authors stressed that a common , but unknown , triggering factor may have been involved . the second report described a 58-year - old man suffering from plaque psoriasis who developed ls at the third session of narrow - band ultraviolet b phototherapy instituted for a psoriatic exacerbation that had manifested 5 months earlier . in this case , the authors hypothesized that the association between two dermatoses was a mere coincidence . we believe that also in our case the association between the two dermatoses is casual , since a wide time gap between the appearance of each . although ls is an asymptomatic and self - limited dermatosis , it may cause a significant psychological distress in some patients , thus requiring an appropriate therapy . several treatments have been reported with various degrees of success including oral and topical corticosteroid , photodynamic therapy , topical calcineurin inhibitors , and oral acitretin . regarding this last therapy , there is only one report describing an extensive case of ls markedly improved over the course of a few weeks with an attack dose of 0.6 mg / kg / die . in our case of ls , the patient was found to be resistant to topical steroid treatment while she had a complete and rapid response to oral acitretin . ls has been considered to be the consequence of an acquired stimulus that induces a loss of immune tolerance to embryologically abnormal clones , resulting in a t - cell - mediated inflammatory reaction , which causing the typical blaschko linear lesions histopathologically characterized by lichenoid , lymphocytic infiltrate with overlying epidermal acanthosis , dyskeratosis , hyperkeratosis , occasional parakeratosis , and lymphocytic exocytosis . therefore , it can be assumed that therapeutic efficacy of acitretin is attributable to its effect on cutaneous immunomodulation as well as epithelial cell proliferation and differentiation . in conclusion , the clinical improvements observed in this report tend to further support the efficacy of oral acitretin in the treatment of ls and suggest its use not only in extensive and unaesthetic forms but also in cases unresponsive to steroid therapy .

lichen striatus ( ls ) is an uncommon dermatosis of unknown etiology that presents as a continuous or interrupted linear band of pink , tan , red or skin - colored papules in a blaschkoid distribution . the lesions are generally solitary and unilateral , but unusual extensive cases with multiple and bilateral lesions have been also described . albeit ls is typically an asymptomatic and self - limited dermatosis , it may cause a significant psychological distress in some patients , thus requiring an appropriate therapy . topical steroid is the most commonly used treatment but it is not always effective . we report a case of ls unresponsive to topical steroid therapy associated with psoriasis vulgaris successfully treated with oral acitretin .

ever since the term primitive neuroectodermal tumor ( pnet ) has been proposed by hart and earle to define a group of malignant neoplasms of presumed neural crest origin , a number of cases of the same have been described at various locations . primary pnet of the spine is unusual with very few cases reported in the literature.[24 ] occurrence of primary spinal pnet in an intramedullary location is further uncommon ; more so in children . very few case reports of primary pediatric spinal intramedullary pnet are reported in the literature.[310 ] we describe a case of an 18-year - old female with conus intramedullary tumor diagnosed to be a spinal primary pnet following surgery the diagnosis was not suspected until the same was confirmed following final histopathological examination . the importance of recognizing the same lies in the different management strategy and therapeutic outcome compared to the usual intramedullary spinal tumors for which surgery forms the cornerstone of treatment and are associated with a better clinical outcome compared to spinal pnet . an 18-year - old female presented with complaints of low back ache and weakness involving bilateral lower extremities for 3 months along with bowel bladder involvement for 1 week . examination revealed tenderness in lower back with hypotonic and paraplegia ( 0/5 medical research council grade ) in bilateral lower limbs . ninety percent sensory loss to all modalities of sensation was present with no evidence of sacral sparing . magnetic resonance imaging ( mri ) of the spine revealed an intramedullary lesion , predominantly isointense on tiw mri with homogenous contrast enhancement and interspersed cystic / necrotic areas from d11-l2 [ figures 1 and 2 ] . a differential diagnosis of common intramedullary tumors namely ependymoma ( myxopapillary variant ) , paraganglioma , other glial neoplasms , metastases or any inflammatory pathology was considered . histopathology revealed a highly cellular tumor , consisting mainly of small round to oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of homer - wright pseudorosettes ; the tumor cells being immunopositive for cd 99 [ figure 3 ] . bone scan and an intracranial mri were done which did not reveal any evidence of tumor elsewhere and a diagnosis of primary intramedullary pnet was made . post - operative course was uneventful with minimal improvement in power and the patient is awaiting adjuvant chemoradiotherapy . t1w magnetic resonance imaging showing a predominantly isointense intramedullary tumor sagittal ( a ) and axial ( b ) contrast enhanced magnetic resonance imaging showing the homogenously enhancing intramedullary tumor with interspersed cystic / necrotic areas microphotograph showing a highly cellular tumor , consisting mainly of small round to oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of homer - wright pseudorosettes ; the tumor cells being immunopositive for cd 99 ( inset ) pnets are malignant tumors composed of highly undifferentiated neuroepithelial cells comprising of various lesions that are histologically similar and usually occur in the pediatric age . these are located mainly intracranially with management being surgical excision followed by craniospinal radiotherapy and a radiation boost to the primary tumor with or without chemotherapy . it has been postulated that pnets arise from neoplastic transformation of primitive neuroepithelial cells in subependymal zones which are present in all areas of the central nervous system and may explain the presence of pnets at locations other than the cerebellum . due to unknown reasons , primary pnet involving the spine is rare and can be intramedullary , intradural extramedullary , or epidural , with the majority arising from the cauda equina.[241218 ] drop metastasis from primary intracranial tumors disseminating via cerebrospinal fluid accounts for majority of spinal pnet . though , these tumors differ from central pnet in their immunohistochemical profile , genetic backgrounds and potential to metastasize outside cns ; the overall prognosis and survival is equally dismal.[24 ] intramedullary primary pnet can present in both children and adults with no sex predilection across the whole spinal column with preferential involvement of the thoracic region . intramedullary location of a primary pnet is uncommon especially in children,[310 ] where glial neoplasms account for majority of intramedullary tumors . only eight cases of clearly documented purely intramedullary spinal pnets have been reported in the pediatric age group.[310 ] though spinal pnet behaves differently from that of spinal cord astrocytoma or ependymoma , the common tumors at this location , the imaging features does not differentiate between them . this precludes the preoperative diagnosis of spinal pnet which usually is based on the histopathological finding with confirmation by immunohistochemical analysis . however , due to rarity of spinal cord pnet , the possibility of metastasis from a central or peripheral pnet should be ruled out before arriving at the diagnosis of primary spinal pnet as was done in the described case . magnetic resonance imaging of the whole brain and spine or a fdg pet can be performed to exclude these possibilities . the management and prognosis of this highly uncommon lesion in this location is different from the more common tumors involving this region and is uniformly poor . in view of the good clinical response to chemoradiation in medulloblastoma or supratentorial pnet the same should be considered even in spinal pnet , not withstanding the fact that complete excision is difficult to achieve in this eloquent location . to conclude , pnet forms a very unusual differential for intramedullary spinal tumors . recognition of this entity at this unusual location is important for prognostication and management as these tumors involve multidisciplinary treatment with poor overall outcome compared to other intramedullary tumors of the spinal cord which it can resemble radiologically .

though primitive neuroectodermal tumor has been considered to arise from a neoplastic transformation of primitive neuroepithelial cells with propensity to involve any part of the central nervous system , a primary intramedullary spinal primitive neuroectodermal tumor is very unusual . the authors describe a case of an 18-year - old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery . the diagnosis of such a tumor is very crucial as the management strategies for these are relatively unclear and are associated with a poorer outcome compared to the other common intramedullary spinal tumors .

a 66-year - old male visited our hospital with painless gross hematuria with blood clots for the previous 1 month . a cystoscopic examination revealed a huge bladder neck mass and a transurethral resection of the bladder tumor ( turbt ) was performed . the histology showed an adenocarcinoma of the prostate , with a gleason score of 9 ( 5 + 4 ) . the patient 's initial prostate - specific antigen ( psa ) was 2.0 ng / ml . magnetic resonance imaging ( mri ) , computed tomography ( ct ) , and bone scans did not show metastatic lesions . the clinical stage of prostate cancer was pt4a cn0 cm0 at the time of diagnosis . the patient underwent a course of prostatic intensity - modulated radiation therapy ( imrt ) to 7,800 cgy in 39 fractions 1 month after the diagnosis . an adjuvant treatment of total androgen blockade ( tab ) with a luteinizing hormone - releasing hormone ( lhrh ) agonist and an anti - androgen was administered 3 months after the diagnosis . the patient 's psa level rose to 2.5 ng / ml 7 months after radiation treatment and then rose to 17.9 ng / ml after 9 months . a follow - up bone scan showed multiple bone metastases in the thoracic spine , both iliac bones , and the left femur 9 months after radiation treatment . . the patient complained of abdominal discomfort , nausea , and vomiting 18 months after the diagnosis . multiple small , elevated mucosal ulcerations on the anterior wall of the gastric antrum were observed by use of esophagogastroduodenoscopy ( fig . the histopathology of the gastric biopsy showed infiltration of neoplastic cells with marked atypical nucleoli and abundant eosinophilic cytoplasm ( fig . he was given an intravenous dose of 120 mg ( 75 mg / m ) docetaxel with prednisolone every 3 weeks for three cycles . the bone and lymph node disease remained stable for several months with an acceptable performance status and no significant treatment - induced toxicity . the patient complained of facial numbness on the right side 21 months after the diagnosis . a mri brain scan confirmed an intracerebral mass measuring 21.6 cm in the right cerebellum ( fig . pulmonary edema was noted and unfortunately the patient died of cardiac arrest 5 months after the diagnosis of stomach metastasis . prostate cancer commonly metastasizes to the bones and lymph nodes ; its spread to the gastrointestinal ( gi ) tract is rare . a search of published reports revealed only three cases of prostate cancer metastatic to the stomach [ 1 - 3 ] . however , there have been reports of prostate cancer metastatic to other sites of the gi tract [ 4 - 6 ] . two postmortem studies of between 67 and 347 patients with metastatic prostate cancer revealed an incidence of gastric metastasis in 1% to 4% of cases . the initial psa of the patient in the first case reported by onitilo et al was 1,565 ng / ml . our patient had hormone - refractory prostate cancer with systemic tumor disease involving the lymph nodes and bone metastasis . the patient in the first case reported by onitilo et al had indolent disease before presenting with gastric metastasis . the patient responded rapidly and dramatically to treatment and his gi bleeding and other symptoms were resolved . our patient died of cardiac arrest 23 months after the initial diagnosis of prostate cancer . the patient in the first case reported by onitilo et al died 19 months after the initial diagnosis of prostate cancer . hematogenous , lymphatic , and direct infiltrations are the typical routes of spread . because the prostate is richly supplied with lymphatic channels , most gi spread to the abdomen the most common symptoms associated with stomach metastases from prostate cancer were nausea and vomiting [ 1 - 3 ] . we emphasize that in patients with a history of prostate cancer and new - onset gi manifestations with nausea and vomiting , the elevation of intracranial pressure with brain metastasis and stomach metastasis should be considered in the differential diagnosis .

prostate cancer commonly manifests with bony metastases . visceral metastasis can also occur in the lungs and liver . however , stomach metastasis related to prostate cancer is rare . here , we report a case of prostate cancer metastatic to the stomach . a 66-year - old male was diagnosed with prostate adenocarcinoma . he was noted as having abdominal discomfort , nausea , and vomiting 18 months after the diagnosis . a histopathologic examination and an esophagogastroduodenoscopic gastric biopsy revealed stomach - metastatic adenocarcinoma . he was also noted as having cerebellar metastatic lesions , which were identified by using a brain magnetic resonance imaging ( mri ) scan . the patient died of cardiovascular complications 5 months after the diagnosis of stomach metastasis .

primary focal hyperhidrosis is a disorder that involves excessive , bilateral , and relatively symmetric sweating in the axillae , palms , soles , or craniofacial region . the condition , particularly in palmoplantar hyperhidrosis , can result in psychological and physical impairment and can also result in social stigmatization . at present , the pathophysiology of primary palmoplantar hyperhidrosis ( pph ) remains unknown . treatment options include topical aluminum chloride ( ac ) , tap water iontophoresis ( twi ) , botulinum toxin a , oral anticholinergics , and endoscopic sympathectomy . among them , twi and ac are routinely performed and are often effective [ 1 , 2 ] . the accumulation of h within the sweat ducts as a result of the anodal current is suggested to be the mechanism of the twi - induced inhibition of sweating . in contrast , topical ac is considered to induce an aluminum salt blockage of the distal acrosyringium and structural changes of the eccrine glands . aquaporin ( aqp ) is a water - transporting protein that has a variety of physiological functions in the skin [ 5 , 6 , 7 ] . aqp-5 is expressed in mammalian sweat glands and has been reported to play a role in sweating in mice , rats , and horses . although the role of aqp-5 remains unclear in humans [ 8 , 9 ] , aqp members may regulate sweating by controlling the water transport . of note , it has been suggested that the water permeability of aqp is regulated by ph . therefore , we evaluated the skin ph before and after antihidrotic therapy , based on the hypothesis that the skin ph might be related to sweating . a 45-year - old woman ( case 1 ) and a 27-year - old woman ( case 2 ) suffered from pph since childhood . three weeks later , she underwent weekly twi with a direct current at 12 ma for 20 min . the sweating was slightly reduced in 4 weeks and was markedly reduced in 8 weeks . the sweating was slightly reduced in 2 weeks and was markedly reduced in 4 weeks . skin surface ph was measured using a skin ph meter ( d-51 , horiba , ltd . , the experiment was performed in a room with a temperature of 24c and humidity below 65% . the patients were kept at rest and the measurement was conducted after the skin was slightly wiped with tissues . the mean ph values were calculated in four sites on both the palms and soles . twelve healthy volunteers ( 7 males and 5 females , mean sd age 33.6 5.5 years , range 2446 ) were also enrolled in the study . the ph of patients with pph before treatment tended to be high compared to that of healthy subjects ( ph of healthy subjects , 5.23 0.22 ; ph of pph , 6.17 in case 1 and 6.65 in case 2 ) . the comparison of ph before and after the antihidrotic therapy was analyzed by the paired t test ( fig . the skin ph was significantly decreased following the treatment and was accompanied by reduced sweating . the first - line therapy for pph is the topical application of ac solution or twi . the widely accepted underlying mechanism of these treatments is the blockage of the distal acrosyringium and structural changes of the eccrine glands by ac salts as well as the impaired perspiration caused by h movement into sweat ducts by twi [ 3 , 4 ] . in this report , however , we observed a reduction in skin ph with both ac and twi treatment . at present , it remains unclear if the reduction in skin ph after ac and twi treatment is the cause or the result of decreased sweating . our finding calls the role of the anatomical changes observed in ac - treated skin into question and focuses on the importance of reduced ph in the inhibition of sweating . as such , it is intriguing to examine the functions of aqp-5 before and after treatment for pph . this work was supported in part by grants - in - aid for scientific research from the ministries of education , culture , sports , science and technology , and health , labor and welfare of japan .

primary palmoplantar hyperhidrosis ( pph ) is a disorder that involves excessive sweating on the palms and soles . although the pathophysiology of pph remains unknown , some treatments , including topical aluminum chloride ( ac ) and tap water iontophoresis ( twi ) , are effective at suppressing the perspiration . herein , we report the kinetics of the skin ph of two cases of pph treated with ac and twi . we found that the skin ph decreased in accordance with the reduction in sweating . this finding indicates that the reduction in sweating may be attributed to the reduction of skin ph in ac and twi . whether or not the ph - regulated function of aquaporin can explain this finding remains unknown .

metastatic castration - resistant prostate cancer ( mcrpc ) is uniformly fatal : in 2008 , more than a quarter of a million deaths worldwide were attributed to this disease . nevertheless , progress has been made in extending the survival and improving the quality of life of men with mcrpc as improved therapies have become available [ 2 , 3 ] . these improvements have been guided by a limited number of tools ( imaging , serum tumor markers , and clinical signs and symptoms ) for ongoing assessment of mcrpc . additional tools that can contribute to accurate and timely measurement of disease status would improve patient care , quality of life , and potentially survival . circulating tumor cells ( ctcs ) are rare cells that can be found in the peripheral blood of patients with many different types of cancer , but not in healthy controls . patients with 5 ctcs/7.5 ml of peripheral blood have been shown to have a shorter time to relapse and a shorter survival time than patients with < 5 ctcs/7.5 ml [ 5 , 6 ] . in addition , certain properties of ctcs , such as high specificity for malignant disease and prompt changes in concentration with changes in disease state , may lend themselves to accurate evaluation of metastatic disease status . an analytically validated test cleared by the united states food and drug administration is commercially available for enumeration of ctcs in metastatic prostate cancer ( cellsearch , janssen diagnostics , llc ; raritan , n.j . one challenge to integrating any new assessment into patient care is determining the optimum use of the information provided by that assessment . here , we discuss the application of ctc enumeration to assist in the monitoring of the disease status of a patient with metastatic prostate cancer in conjunction with preexisting tests to illustrate how ctc information might be useful in the clinical setting . the patient was a 54-year - old male with a 6-month history of hip discomfort . imaging was ordered and lumbosacral films demonstrated multiple rounded and sclerotic foci over the visualized bony pelvis , which were considered suspicious for metastatic prostate cancer . the patient 's prostate - specific antigen ( psa ) level was 34.2 ng / ml . based on these findings , the patient was referred for a prostate biopsy . the biopsy revealed prostate adenocarcinoma with a gleason score of 7 ( 3 + 4 ) involving all cores from the right side . his psa level reached a nadir of 3.6 ng / ml after approximately 3 months of therapy , after which it began to rise . by the sixth month of therapy , 1 ) . an initial ctc measurement taken at this time returned a value of 36 ctcs/7.5 ml of peripheral blood using the cellsearch system . based on the elevated psa and ctc levels as well as progression of pain due to osseous metastatic disease , the patient was placed in a clinical trial of docetaxel plus bevacizumab therapy . after 3 cycles of treatment ( approximately 8 months after initial diagnosis ) the patient 's ctc level had risen to 145 ctcs/7.5 ml , while the psa level dropped to 2.3 ng / ml . computed tomography ( ct ) imaging demonstrated stable disease . at approximately 11 months after diagnosis , the patient 's psa level had dropped to 1.0 ng / ml and his ctc level had dropped to 13 ctcs/7.5 ml . clinically , the patient reported worsening pain , which was attributed to diffuse osteoblastic metastases . the patient was switched to oral cyclophosphamide . within 1 month of initiating the new therapy , the ctc level had reached zero , while the psa level increased to 3.4 ng / ml . after 16 months of therapy , the ctc level was 2.0 ctcs/7.5 ml and his circulating psa level had fallen to 1.8 ng / ml ; ct imaging showed a partial response . the patient continued on cyclophosphamide treatment for an additional 4 months , during which time both ctc and psa levels slowly increased . by 19 months after diagnosis , his ctc level had risen to 5 ctcs/7.5 ml , while his psa level was 2.4 ng / ml . although ct imaging at this time showed stable disease , the patient reported an increase in pain . considering the increasing pain and elevated ctc concentration as evidence of progression , cyclophosphamide treatment was discontinued and alternative treatment through clinical trials was pursued as a possible avenue for this patient . two assessments commonly used in mcrpc , imaging and psa concentration , are known to have properties that in some situations may reduce confidence in disease assessment . bony lesions frequently occur in mcrpc ; however , ct imaging , radionuclide imaging , or other imaging modes often do not reveal responses to treatment in a timely fashion . frequently , 2 rounds of imaging and 3 or more months of observation are needed to determine if there is progression or response to therapy . psa levels are frequently unrelated to disease status due to factors such as previous therapy and tumor grade . in addition , both of these assessments are subject to flare phenomena that can mask a response to therapy [ 10 , 11 ] . when few assessment options are available , discordance between them can diminish confidence in treatment decision making . ctcs have been shown in multiple prospective studies to be prognostic of both disease - free survival and overall survival in mcrpc . in a manner similar to that described by others , this case illustrates how the prognostic value of the measured ctc concentration in the peripheral circulation can help resolve assessment discrepancies and increase confidence in treatment decisions . in the first instance , after 6 cycles of docetaxel plus bevacizumab , this patient 's psa level had dropped from 16.1 to 1.0 ng / ml , suggesting a therapeutic response . his ctc level was measured at 13 ctcs/7.5 ml , well above the 5 ctcs/7.5 ml cutoff for poor prognosis mcrpc . the increasing pain and elevated ctc level prompted repeat imaging , which confirmed new nodal disease and progression . on this basis subsequently , the patient 's ctc level diminished to zero , his pain resolved , and imaging 5 months later demonstrated a partial response . the decision was ultimately based on the specificity of ctcs for malignant disease and the high prognostic value of ctcs in mcrpc . subsequently , 19 months into treatment the patient again reported worsening pain , although imaging suggested stable disease . the patient 's psa concentration had stayed consistently low , ranging from 1.0 to 3.4 ng / ml . the patient 's ctc level , however , was measured at 5 ctcs/7.5 ml , which supported the conclusion that the patient 's disease was progressing on cyclophosphamide therapy despite his low psa concentration . the decision was made to discontinue cyclophosphamide and evaluate clinical trial options for the patient . the two most frequently used evaluation methods , imaging and psa level , in some cases do not yield a clear answer on disease status and prognosis and are often discordant . this case illustrates how ctc enumeration , with information properties that complement other assessments , can compensate for some of the inconsistencies of other evaluation methods , thereby improving confidence in treatment decisions .

optimal management of therapy can improve quality of life , and potentially survival , of patients with metastatic prostate cancer . circulating tumor cell ( ctc ) enumeration in the peripheral blood holds promise for facilitating optimal therapy management based on high cancer specificity and prompt response to changes in disease status . we present the case of a 54-year - old male diagnosed with metastatic prostate cancer . the patient 's disease status was monitored radiographically and by serial determinations of serum prostate - specific antigen concentration , performance status , and ctc enumeration . at multiple visits , the disease status was not clear due to discordance between standard assessments . the additional information provided by ctc enumeration helped clarify disease status and provided support for treatment decisions .

nonunion of a fracture can pose a significant challenge in terms of skeletal stability , soft tissue biomechanics and potential limb loss . they most commonly occur as a consequence of infection , mal - alignment , metabolic and endocrine abnormalities . however , in otherwise healthy young individuals , such etiologies are rare and alternative pathological factors should be considered . herein , we present the first reported and successfully treated case of tibal fracture nonunion as the result of posterior tibial artery ( pta ) pseudoaneurysm . a 20-year - old man with no past medical history presented to the emergency department with non - displaced and closed transverse fracture of the left mid - tibial shaft and an intact fibula induced by a tackle during a football match . on examination , he had no neurovascular deficit and all crural pulses were palpable . his fracture was treated conservatively with a non - weight bearing above knee plaster cast later replaced by a below knee cast for a total period of 6 months . upon examination during his attendance to the facture clinic , the subsequent magnetic resonance imagining ( mri ) confirmed a nonunion of the fracture along with an ovoid 2.7 cm posterior compartment vascularized mass causing bowing of the interosseous septum with no evidence of avascularity of the bony margins ( fig . 1 ) . an endovascular approach was adopted , and the pseudoaneurysm was injected with 2 ml of thrombin , filled with multiple coils and deployment of an angiography balloon ( in the sac ) ( 4 mm 6 cm ) for 7 min ( fig . 2 ) . this resulted in complete cessation of the pseudoaneurysm with normal flow in the pta ( fig . follow - up x - ray ( 6 months ) demonstrated complete union of the fracture site with no associated complications . figure 1:mri images demonstrating the site , size and the origin of the pta pseudoaneurysm . figure 2:angiographic image , demonstrating the site of the nonunion , coil insertion to the pseudoaneurysm and extravasated contrast . mri images demonstrating the site , size and the origin of the pta pseudoaneurysm . angiographic image , demonstrating the site of the nonunion , coil insertion to the pseudoaneurysm and extravasated contrast . pseudoaneurysms of the pta are rare , and they mainly occur following sudden acceleration and/or deceleration type injury , open fractures and , in few cases , can be iatrogenic ( manipulation of fracture , surgery and ilizarov k - wire ) [ 2 , 3 ] . currently , no report in the literature suggests a single isolated and close fracture of tibial bone to be responsible for pseudoaneurysms of pta . they are mainly asymptomatic and present as a pulsatile lump but in their deeper anatomical course could result in tibial nerve compression causing paresthesia , neuralgia and weakness of the foot and toe muscles . in 39% of reported series , they present with signs and symptoms of lower limb ischemia following pseudoaneurysms thrombosis and/or distal embolization [ 4 , 5 ] . , the patient did not exhibit any neurovascular deficit , and it was an incidental finding following investigations for the tibial fracture nonunion . furthermore , fracture nonunion in a healthy young individual without any risk factor is rare and should raise suspicions about alternative and infrequent pathologies . the management of pseudoaneurysms of pta is varied , and they depend on the individual 's symptomatology and the subsequent course of the pseudoaneurysm . currently , there is no consensus on their overall management ; therefore , indications for their open versus endovascular repair are subjected to various debates . open repair ( ligation and/or excision and interposition vein grafting ) in deep anatomical areas may subject the patient to extensive dissection , longer operative time and higher incidences of morbidity ( motor and sensory nerve damage , prolonged recovery , deep vein thrombosis ) . endovascular repair is another acceptable option , but this approach has its own limitations . the deployment of any type stent for exclusion of pseudoaneurysm from the main artery in the infra popliteal region is known to possess low patency and thrombotic incidences . the use of thrombin , coils and glue remains another alternative , but this mainly depends on the pseudoaneurysm neck size , as wider necks fail to contain the above and can result in failure of the procedure and thrombosis of the remaining artery . overall , the approach should be tailored according to the patient 's arterial status and features of the pseudoaneurysm . to the best of our knowledge , deployment of angiography balloon ( as an occlusive technique ) in addition to thrombin and coil for the exclusion of pta pseudoaneurysm has not been reported in the literature . the use of this novel technique could be beneficial in exclusion of wider neck pseudoaneurysms and limitations of the damage to the main vessel as reported in the current case report . in the reported case , the individual did not exhibit any signs and symptoms of pseudoaneurysms and similarly did not raise any suspicion of such pathology till nonunion was detected . once suspected , the best investigative modality is computed tomography angiography ( cta ) followed by magnetic resonance imaging ( mri ) . both of these modalities have sensitivity and specificity of more than 90% for detection of isolated entities . in conclusion , nonunion of fractures in young healthy individuals should raise the suspicion of pseudoaneurysms , despite the absence of clinical signs and symptoms of vascular disruptions . clinicians should have low threshold for cta and/or mri in such circumstances , and the surgical method of repair ( open versus endovascular ) should be in accordance with the patient 's best interest . written informed consent was obtained from the patient for publication of this case report and accompanying images .

pseudoaneurysm of the posterior tibial artery ( pta ) is uncommon , and they mainly occur following high - velocity trauma , open fractures and can be iatrogenic in nature . to the best of our knowledge , this is the first reported and successfully treated case of pta pseudoaneurysm identified as a consequence of tibia fracture nonunion in an otherwise healthy young individual 6 months following the original incident with a novel intraoperative technique .

first , a person must have symptoms severe enough for medical care ( multiplier 1 ) . second , the physician must collect patients specimens ( multiplier 2 ) and forward them for testing by bacterial culture ( multiplier 3 ) . third , the sample test result must be positive ( multiplier 4 ) , and the confirmed case must be reported ( multiplier 5 ) ( 2,7,8 ) . to obtain multiplier 1 , we conducted a 12-month population - based household survey during march 1 , 2012february 28 , 2013 ( approved by the ethics committee of guangdong cdc ) . respondents were randomly selected from 4 districts in western , eastern , and central guangdong province . we used a standard questionnaire to collect information about diarrhea in the previous 4 weeks . the incidence rate of diarrhea was 0.1081 ( 95% ci 0.10040.1158 ) episodes / person - year ; 38.6% of the household survey respondents with diarrhea sought medical care . multipliers 2 and 3 were based on data from sentinel hospitals and comprised the overall number of diarrhea cases , samples collected , and samples submitted for culture during the year . a total of 75,583 ( 45.3% ) samples of 166,729 registered diarrhea cases in the sentinel hospitals were collected , of which 22,577 ( 29.9% ) were tested . laboratories of sentinel hospitals cultured samples for salmonella in accordance with standard protocol provided by the national reference laboratory by using macconkey agar as plating medium . according to a proficiency testing program , the numbers of salmonella isolates identified and reported to nndrs as nts infectious diarrhea by all sentinel hospitals yielded the proportion of cases reported ( 648/1,061 , 61.1% ) ( multiplier 5 ) . based passive surveillance system , multiplier 1 was the same as for active surveillance . according to a comparison with samples from the submission proportion in a survey of physician - diagnosed diarrhea in guangdong province during 2009 ( mann - whitney test , p = 0.246 ) ( 11 ) , and a comparison between medical institutions that charged and did not charge for testing ( kolmogorov - smirnov test , p = 0.837 ) , the proportion of samples submitted and tested from active surveillance were also used as estimates of passive surveillance . the average test sensitivity of sentinel laboratories before active surveillance began was used as an estimate of all medical institutions ( i.e. , the sensitivity of passive surveillance [ 48.2% ] ) . using numbers of salmonella isolates in guangdong province from laboratory data , and number of reported nts cases by all medical institutions , we determined the proportion of reported nts was 9.6% ( 991/10,360 ) . thus , for each reported nts case under passive surveillance , 414.8 cases actually occurred . multipliers of 5 age groups also were presented ( table 1 ) . to generate a more robust estimate , we conducted uncertainty and sensitivity analyses ( online technical appendix , http://wwwnc.cdc.gov/eid/article/22/4/15-1372-techapp1.pdf ) on passive surveillance data using monte carlo simulation ( @risk 6.0 ; palisade , ithaca , ny , usa ) ( 12 ) . we used distribution to describe the uncertainty of proportions and negative binominal distribution to estimate the number of cases . the uncertainty analysis model predicted a 411.9 ( 95% ci 308.4592.7 ) overall multiplier and estimated that 408,499 ( 95% ci 302,899591,901 ) salmonella cases occurred per year when the overall multiplier was applied to the 991 reported nts cases , resulting in 391.6 ( 95% ci 290.3567.4 ) cases/100,000 persons in 2012 . the rank correlation of various factors in the model showed that patients seeking medical care provided the highest uncertainty in the overall estimate ( influence rate 96% ) ( figure ) . rank correlations for the total number of nontyphoidal salmonella cases in the population ( tornado diagram ) , guangdong province , china , 2012 . our estimated nts incidence was lower than the incidence in china as determined from a literature review ( 626.5 cases/100,000 persons ) ( 13 ) but close to that in the united states ( 352.1 cases/100,000 persons ) ( 3 ) . however , incidences for persons < 5 years of age and 524 years of age in our study were higher than those for persons in china and the united states , highlighting that salmonella represents a major health problem in guangdong province , especially among younger persons . our estimated active surveillance rate ( 35.8 ) of nts infections per reported case is similar to estimates in the united states ( 38.6 and 39 ) ( 2,10 ) but different from those for england ( 3.2 ) , jordan ( 278 ) , and japan ( 63 ) ( 7,8,14 ) . such differences might be due to differences in methods used and to actual differences in salmonella infections . with fewer missing cases and less underestimation , active surveillance has lower overall multipliers than passive surveillance , indicating smaller surveillance artifacts and more accurate incidence estimate and presents remarkable advantages over passive surveillance . the estimate for active surveillance also showed that if we seek to reduce uncertainty in the overall estimate , we should first focus on encouraging patients to seek medical care . our study provides policymakers in china with a reference for the importance of salmonella incidence and calls for balanced surveillance on both foodborne infections and foods and enlarging active surveillance scales . more surveillance guidelines need to be developed to help physicians identify timing of sampling , tests , and performance . laws requiring reporting of foodborne diseases and pathogens need to be enacted to increase quantity and quality of reporting . the result suggests that to increase care seeking and sample submission , government health insurance schemes should be further developed to cover diagnostic tests and treatments of diseases of public health significance . uncertainty and sensitivity analysis and study limitations for a study of nontyphoidal salmonella infection , guangdong province , china , 2012 .

we used active and passive surveillance to estimate nontyphoidal salmonella ( nts ) infection during 2012 in guangdong province , china . under passive surveillance , for every reported nts infection , an estimated 414.8 cases occurred annually . under active surveillance , an estimated 35.8 cases occurred . active surveillance provides remarkable advantages in incidence estimate .

giant cell tumors of foot are uncommon and have been reported to have an incidence of less than 4% of all the locations and typically have a varied natural history ranging from entirely benign to highly malignant behavior with distant metastases . however , after curative local treatment giant cell tumors ( gcts ) of foot and have high incidence of recurrence ranging from 10 to 47% . detection of recurrence with conventional imaging like ct and magnetic resonance imaging ( mri ) can be difficult because of distorted anatomy and implants in situ following surgery . , we report a case of recurrent gct of foot detected on f18-fdg pet / ct . a 48-year - old female patient diagnosed to have gct of right foot was subjected to excision , curettage and arthrodesis of ankle joint . six months post - surgery she presented with increasing pain in right foot and recurrence was suspected . total body f-18 fdg pet / ct acquired 60 min after intravenous injection of 10.2 mci of f18-fdg was performed for detection of recurrence . it showed intense uptake of fdg in soft - tissues in right hind foot along the anterior and medial surface of the right ankle joint . fdg uptake was also seen to involve the inferior surface of talus , cuboid , lateral and intermediate cuneiform , navicular , talus and calcaneum bones of the foot [ figure 1 ] . however , exact soft - tissue delineation was not possible due to surgical implant in situ leading to artefacts on ct . histopathologically the amputated specimen showed giant cell tumor of intermediate grade with involvement of inferior surface of talus , cuboid , lateral and intermediate cuneiform bones , navicular , and calcaneum correlating exactly with the f18-fdg pet / ct findings . fluorine-18 fluorodeoxyglucose ( fdg ) positron emission tomography/ computed tomography ( a ) maximum intensity projection image of lower limbs showing intense tracer uptake in right hindfoot around the ankle joint . fdg uptake is seen to extend into the lower one third of the leg on medial side ( b ) transaxial images through the feet show intense fdg uptake in soft tissue mass in right foot ( c ) transaxial images showing intense fdg uptake in lower surface of talus foot and ankle giant cell tumors have increased rate of local recurrence . early and accurate detection of recurrence is important to decide upon accurate management . conventional imaging modalities are known to be of limited utility in detecting recurrence due to distortion of anatomy post - surgery and also by the presence of metallic implants in many tumors . one study by shimizu et al . highlighted the better performance of functional imaging with gallium scintigraphy over anatomical imaging modalities in prediction of recurrence . f-18 fdg uptake in gcts has been described and gcts are known to be highly f-18 fdg avid . few case reports have demonstrated the utility of fdg pet in evaluation of multi - centric gcts . however , to best of our knowledge there is no literature addressing the utility of f-18 fdg pet - ct in detection of recurrent gcts of foot . fusion with ct has always helped in improving the accuracy of interpretation of functional imaging in the foot . stand - alone ct might have limitations in accurately detecting the recurrence but in conjunction with f-18 fdg pet it definitely helps in accurate localization of uptake to specific bone , which may have implications in appropriate management . detection of involvement of talus is important in case of foot as it favors an amputation against conservative approach limiting to excision of tumor . in our case , giant cell tumors of foot though rare can recur early post - treatment . combined anatomical and functional information obtained from ct and f-18 fdg pet in one investigation fdg pet / ct aids in accurate detection of recurrence and thereby directing appropriate management of recurrent giant cell tumors . we also propose that f-18 fdg pet / ct can be used as primary modality in evaluating the patients with suspicion of recurrent gct of foot .

detection of recurrence of tumors with conventional imaging like computed tomography ( ct ) and magnetic resonance imaging ( mri ) can be difficult because of distorted anatomy and implants in situ . fluorine-18 fluorodeoxyglucose positron emission tomography / computed tomography ( f-18 fdg pet / ct has been shown to be very useful in detection of recurrent tumors with higher accuracy than conventional imaging method . giant cell tumors of foot though rare have high recurrence potential after initial curative treatment . however , currently there is no literature addressing the role of f-18 fdg pet / ct in evaluation of these tumors . we report a case of post excisional recurrent giant cell tumor of foot diagnosed on f-18 fdg pet / ct . in addition , to detection of recurrence f-18 fdg pet / ct also aided in accurate management of the patient .

the majority of swallowed indigestible foreign bodies pass through the gastrointestinal tract without complications [ 1 , 2 , 3 , 4 ] . however , there are three physiological narrowings involving the pylorus , duodenal c - loop and ileocecal valve . foreign bodies longer than 10 cm , such as a toothbrush , can not negotiate the duodenal c - loop due to its fixed retroperitoneal position . these objects should be endoscopically removed as soon as possible to avoid pressure necrosis and gastrointestinal perforation [ 5 , 6 , 7 ] . if endoscopic removal fails or there is evidence of obstruction or perforation , laparoscopic gastrotomy should be performed . an 18-year - old caucasian woman with no previous history of related medical problems was admitted to the department of internal medicine , division of gastroenterology , clinical hospital split because she had accidentally swallowed a toothbrush . the patient admitted she had been using the toothbrush to induce emesis . on presentation , 2 h after ingestion , she was asymptomatic and her vital signs were within normal limits . a plain abdominal x - ray study confirmed the presence of the foreign body in the left upper abdominal quadrant ( fig . informed written consent for upper gastrointestinal endoscopy was obtained from the patient and her parents . esophagogastroduodenoscopy revealed the toothbrush in the stomach with its head positioned against the gastric fundus . the extracted toothbrush was 20 cm long . repeated upper gastrointestinal endoscopy was performed 4 h later and showed no evidence of mucosal lesion to the stomach or the esophagus . the patient was discharged home in excellent clinical condition after being observed for 6 hours . foreign bodies in the stomach will pass uneventfully through the gastrointestinal tract in 8090% of cases [ 1 , 2 , 3 ] . however , foreign objects longer than 10 cm , such as a toothbrush , can not negotiate the duodenal c - loop due to its fixed retroperitoneal position . in such cases , these objects should be removed as soon as possible to avoid pressure necrosis and gastric perforation [ 2 , 3 , 4 , 5 ] . removal of long foreign bodies from the stomach is influenced by the patient 's clinical condition and technical abilities of the endoscopist [ 2 , 3 , 4 , 5 ] . if endoscopic removal fails or there is evidence of obstruction or perforation , surgical gastrotomy should be performed . we had no need for conscious sedation since the patient was actively participating during the procedure . special attention has to be paid during the extraction of the toothbrush to its alongside alignment with the esophagus . otherwise , this most critical and demanding part of the extraction procedure may easily result in mucosal damage or foreign body impaction . the second important phase of the extraction procedure is when the foreign body reaches the oropharynx . the patient has to extend his head backwards and the endoscopist has to reach for the toothbrush with his hand and pull it out . this case report describes a rare case in whom a toothbrush was safely extracted from the stomach endoscopically by snare extraction . the procedure is brief , does not require conscious sedation , and the patient can be discharged from hospital after a few hours . early endoscopic retrieval of the toothbrush is critical for reducing morbidity and mortality . in cases when endoscopic removal fails

most ingested foreign bodies will pass uneventfully through the gastrointestinal tract . nevertheless , long and rigid foreign bodies are associated with an increased risk of gastrointestinal impaction , perforation and bleeding . moreover , there has been no case of spontaneous passage of a toothbrush reported . therefore , the prompt removal of such ingested foreign objects is recommended before complications develop . this case report describes a case of an 18-year - old woman who accidentally swallowed her toothbrush . the toothbrush was successfully removed via flexible endoscopy using a polypectomy snare . a swallowed toothbrush is a special clinical challenge . early endoscopic retrieval of the toothbrush is critical for reducing morbidity and mortality . in cases when endoscopic removal fails , a laparoscopic surgical approach may be an alternative .

vitamin b6 is part of the vitamin b group complex and can serve as a cofactor in the metabolism of glucose , amino acids , and lipids . since it is essential for neurotransmitter synthesis , its deficiency causes various neurological disorders.1 seizures associated with vitamin b6 deficiency are usually observed in pediatric patients and these are characterized by refractoriness to antiepileptic drugs and a positive response to pyridoxine administration.2 here , we report a case of adult - onset seizures associated with vitamin b6 deficiency and discuss the pathophysiological mechanism . a previously healthy 36-year - old man visited our hospital because of recurrent loss of consciousness and convulsions . he had had poor nutritional intake , nausea , and vomiting for about 6 weeks . he was a heavy drinker with a reported alcohol intake of more than two bottles per day of soju ( korean whiskey ) for 10 years . complete blood count revealed that his hemoglobin level was as low as 9.4 g / dl . mean cell volume was slightly elevated ( 104.7 fl , normal value 80100 fl ) , but the mean corpuscular hemoglobin concentration was normal ( 33.6 g / dl , normal value 3236 g / dl ) . liver function tests revealed aspartate aminotransferase , alanine aminotransferase , and alkaline phosphatase levels of 59 iu / l(normal value 040 the patient had a generalized tonic - clonic seizure in the emergency room prior to admission . the seizure ceased after administration of 4 mg of lorazepam and 1 g of valproate . after one day , another seizure developed and 800 mg of controlled - release carbamazepine was administered . vitamin b6 levels were measured to be 4.4 nm / l ( normal value : 20202 nm / l ) , serum homocysteine levels were 65 m / l ( normal value 5.0 13.9 m / l ) and vitamin b1 level was normal ( 79.8 ng / ml ) . pyridoxine ( 12 mg / day ) and carbamazepine were administered for 2 months . since discharge follow - up vitamin b6 levels were also normalized ( 59.3 nm / l ) . vitamin b6 is a water - soluble vitamin with the active form being pyridoxal 5-phosphate ( plp).3 plp is essential for the metabolism and synthesis of amino acids , gluconeogenesis , hematopoiesis , hormone regulation , immunologic functions , and synthesis of neurotransmitters.4 importantly , plp is a crucial cofactor in the synthesis of -aminobutyric acid ( gaba ) from glutamate because of its involvement in the mechanism of decarboxylation . therefore , plp deficiency results in the lowering of seizure thresholds by impairing the synthesis of gaba.5 in this case , wernicke s encephalopathy and hepatic encephalopathy must be differentiated . our patient showed no physical or laboratory findings compatible with those disorder , i.e. profound alteration of mentality before and after seizures , no stigmata of chronic liver disease , no significant abnormalities on liver function test and platelet counts , and normal thiamin level . our patient had seizures without abstinence period , which was not compatible with alcohol withdrawal seizures . this finding suggests that the seizures of our patient were probably associated with vitamin b6 deficiency , not with an epileptic brain disorder . the most common cause of seizures associated with vitamin b6 deficiency in adults is isoniazid ( inh ) toxicity.5 inh hyrozone , a metabolite of inh , inhibits pyridoxine phosphokinase , which is involved in the process of converting pyridoxine to pyridoxine phosphate . this induces the reduction of plp and gaba and thereby increases the excitability of nerve cells.5 vitamin b6 deficiency - associated seizure caused by mechanisms other than inh toxicity are rarely reported in adults.6,7 gerlach et al.6 reported three patients with status epilepticus who showed refractoriness to antiepileptic drugs and low levels of plp . all of them were admitted to an intensive care unit in critical condition . in that report , all three patients were chronic alcohol drinkers with alcohol - associated liver disease . low concentrations of plp in patients with chronic alcoholic liver disease may result from a dietary deficiency coupled with an intact plp metabolizing enzyme , aldehyde oxidase.8 hyperhomocysteinemia may also induce seizures , but its mechanism is not well understood.9 our patient , who previously had recurrent seizures despite treatment with antiepileptic drugs , became seizure - free after normalization of vitamin b6 levels . we propose that a differential diagnosis for vitamin b6 deficiency needs to be considered in patients with chronic alcoholism and nutritional deficiencies who show refractoriness to antiepileptic drugs .

vitamin b6 is closely associated with functions of the nervous , immune , and endocrine systems . its deficiency may result in neurological disorders including convulsions and epileptic encephalopathy . until today , this has only been reported in infants , children , and critically ill adult patients . we report a case of a 36year - old man with chronic alcoholism who presented with seizures after gastrointestinal disturbance . his seizures persisted even after treatment with antiepileptic drugs , but eventually disappeared after administration of pyridoxine . hence , vitamin b6 deficiency may cause seizures in adult patients with chronic alcoholism .

most cases of intrathoracic goiter are asymptomatic and are discovered at chest radiography by chance . it is difficult to gain a definite diagnosis for malignancy by magnetic resonance imaging ( mri ) and iodine-123-radioisotope ( i - ri ) . then fluorine-18-fluorodeoxyglucose positron emission tomography ( f - fdg - pet ) is expected to be useful in this issue but the specificity is not always clear . we experienced a case of intrathoracic benign goiter which showed high uptake of f - fdg - pet . a 55-year - old woman was referred to our hospital because of a suspicion of mediastinal tumor incidentally found through a medical - checkup plain x - ray photography ( x - p ) ( figure 1a ) . chest mri revealed a 3 cm diameter tumor which seemed to connect to the right lobe of thyroid and projected into the mediastinum ( figure 1b ) . by i - ri , no unusual accumulation was detected ( figure 1c ) . a fine needle aspiration biopsy , which is viewed as the gold standard for diagnosis in most cases , was tried but could not reach a conclusive diagnosis . thereby , f - fdg - pet was performed and a high accumulation was revealed with standardized uptake value ( suv ) of 3.8 ( figure 1d ) . a 3 cm diameter tumor which seemed to connect to the right lobe by mri ( panel b ) . no unusual accumulation by i - ri ( panel c ) . a high accumulation with suv of 3.8 by f - fdg - pet ( panel d ) . thus , as a possibility of malignancy could not be excluded , the right lobe excision procedure for thyroid gland was enforced . the surface was flat and smooth and the exfoliation from the circumference organization was easy ( figure 2a ) . microscopically , the encapsulated tumor consisted of atypical large - sized follicles without malignant characteristics , the background thyroid tissue showing no remarkable change ( figure 2b ) . flat and smooth surface of the tumor connected to the right thyroid lobe ( panel a ) . atypical large - sized follicles without malignant characteristics and the background thyroid tissue showing no remarkable change ( panel b ) . ethical approval was not thought to be necessary because all the clinical course of the case was completely within usual medical cares . informed consent was given from the case on each occasion of diagnostic examinations and therapeutic procedures . especially , intrathoracic goiter is a rare condition . in the early years of applying f - fdg - pet for the differentiation of thyroid nodule malignancy , high sensitivity , and selectivity however , recent reports did not confirm the high selectivity by suv ( 4060% ) . thus , follicular adenoma of thyroid could present negative i - ri uptake and positive f - fdg - pet accumulation .

abstracta 55-year - old woman was referred for a suspicion of mediastinal tumor through plain x - ray photography ( x - p ) . magnetic resonance imaging ( mri ) revealed a 3 cm diameter tumor which seemed to connect to the thyroid and projected into the mediastinum . a fine needle aspiration biopsy was tried but could not reach a conclusive diagnosis . thereby , fluorine-18-fluorodeoxyglucose positron emission tomography ( 18f - fdg - pet ) was performed and a high accumulation was revealed with standardized uptake value ( suv ) of 3.8 . thus , the right lobe excision procedure was enforced . the obtained tumor was continuous to the right lobe as expected . microscopically , the encapsulated tumor consisted of atypical large - sized follicles without malignant characteristics . thus , histological diagnosis was follicular thyroid adenoma.thus , follicular adenoma of thyroid could present negative iodine-123-radioisotope ( 123i - ri ) uptake and positive 18f - fdg - pet accumulation .

the incidence of vesicoenteric fistulas has been reported to occur in 0.01% to 0.05% of surgical admissions1 ) . in one study of 56 instances of vesicoenteric fistulas , hepatocellular carcinomas ( hccs ) account for 90% of primary liver cancers ; up to 80% of hccs occur in korea occur in patients with cirrhotic livers . according to our literature review , several malignancies have been reported as causes of ileovesical fistula , though such complications have not yet been reported to be caused by hcc . here , we report a case of ileovesical fistula caused by hcc arising from the noncirrhotic liver . a 27-year - old man was referred to our hospital for the evaluation of hepatic masses . he had consumed 30.0 g alcohol twice weekly for 8 years . on presentation , his vital signs were stable and the physical examination was normal . viral marker study showed that hbs antigen was negative , anti - hbs antibody positive , and anti - hcv negative . -fetoprotein ( fp ) was over 50,000 iu / ml and carcinoembryonic antigen ( cea ) was 4.94 ng / ml . abdominal computed tomography ( ct ) with enhancement revealed two heterogeneous hypodense masses in the right hepatic lobe of about 2.4 2 cm and 3.4 4 cm . no cirrhotic change was observed in the liver . liver magnetic resonance imaging ( mri ) revealed two relatively well - defined masses in the right hepatic lobe ( figure 1 ) . these lesions were homogeneous low signal intensity on t1-weighted image and slightly inhomogeneous high signal change on t2-weighted image . immunohistochemical stains were positive for cytokeratin ( ck ) , epithelial membrane antigen ( ema ) , fp , and hepatocyte - specific antigen ( hsa ) . however , evidence of cirrhosis and chronic injury were absent . under the diagnosis of hcc , two months later , follow - up ct scan showed a newly developed mass in the caudate lobe about 4.5 4.0 cm in size , with several daughter nodules . follow - up ct scan after 3 weeks showed defective lipiodol uptake masses in both the right hepatic lobe and the caudate lobe with remnant tumoral enhancement . several days later , the patient complained of dysuria , fecaluria , and intermittent lower abdominal pain . pelvic ct scan showed a lobulated soft tissue mass about 6 cm in size ( figure 3 ) . barium study of the small bowel showed a fistula between the small bowel loop and the urinary bladder ( figure 4 ) . there were soft tissue masses between the ileum and the bladder and multiple lymphadenopathies at the mesenteric root . adhesion and fistula were found between the ileum at 10 cm proximal to the ileocecal valve and the dome of the bladder . the distal 65 cm of ileum was segmentally resected and anastomosed end - to - end . several malignancies have been reported to be causes of ileovesical fistula . according to our literature review , these include bladder cancer , lymphoid malignancies , and others1 , 3 - 5 ) . although other various malignancies themselves were not direct causes of ileovesical fistula , radiation enteritis complicated by radiation therapy could also cause the ileovesical fistula6 ) . in korea , about 80% of hccs occur in patients with cirrhotic livers . in our case , hcc occurred in the noncirrhotic liver , and the cause remains uncertain . in one study of 403 patients with hcc , 148 patients ( 37% ) with extrahepatic metastases were identified . the lung , abdominal lymph nodes , and bone are the most common sites of extrahepatic metastatic hcc . peritoneal metastases were illustrated at ct scan in 16 ( 11% ) of 148 patients with extrahepatic metastatic hcc7 ) . ho et al . reported that 3% of patients with hccs in noncirrhotic liver involved the peritoneum8 ) . fecaluria , abdominal pain , and pneumaturia were the most common presenting symptoms of vesicoenteric fistulas . other symptoms were dysuria , gross hematuria , fever and chills , diarrhea , and urine per rectum . the diagnosis of vesicoenteric fistula can be highly indicated by the above mentioned symptoms , using various diagnostic tests to establish diagnosis . in our particular case , cystoscopic and cystographic examinations showed inflammatory mucosal changes on the dome of the urinary bladder , but could not detect a fistula . cystoscopy revealed either the fistula itself or highly suggestive , localized mucosal abnormalities ( " herald lesions " ) in 86% . ct scan revealed abnormalities , such as pelvic masses or bladder or intestinal wall thickening ( 87% ) , and revealed a fistula ( 24% ) . it should be possible to establish the diagnosis of a vesicoenteric fistula through a combination of cystoscopy , cystography , and barium study in almost all patients . other diagnostic tests for vesicoenteric fistulas include sigmoidoscopy or colonoscopy , intravenous urography , radioisotope studies , and so on . forms of treatment for vesicoenteric fistula include diverting enterostomy , single stage repair , and multi - staged repair . when secondary to radiation necrosis and recurrent tumor , fistulas have an extremely poor outlook with some palliation afforded by a diverting colostomy2 ) . multi - staged repair is limited to those patients with evidence of intestinal obstruction , uncontrolled local sepsis , and questions of intestinal viability9 ) . in summary , we experienced one case of ileovesical fistula , which was caused by hcc arising from noncirrhotic liver . the combination of cystoscopy , cystography , and barium study of the small bowel confirmed the diagnosis of ileovesical fistula , and single stage repair was performed successfully .

ileovesical fistula is a very rare clinical entity , the most frequent cause of which is crohn 's disease . furthermore , it is an exceptionally rare complication of malignancies . we experienced one case of ileovesical fistula which had been caused by hepatocellular carcinoma ( hcc ) arising from the noncirrhotic liver.a 27-year - old man was diagnosed with hcc in a noncirrhotic liver . despite treatment with transarterial chemoembolization ( tace ) , the disease status became more aggravated . the patient complained of dysuria , fecaluria , and intermittent lower abdominal pain . pelvic ct scan showed a soft tissue mass of 6 cm abutting on the distal ileum which was downwardly displaced . barium study of the small bowel showed a fistula between the small bowel loop and the urinary bladder . upon operation , adhesion and fistula were found between the ileum and the urinary bladder . the microscopic findings of the surgical specimen were compatible with metastatic hcc . we confirmed that ileovesical fistula had been caused by metastatic hcc .

hypoparathyroidism - retardation - dysmorphism ( hrd ) syndrome also known as sanjad - sakati syndrome is characterized by permanent parathyroid hormone ( pth ) deficiency , hypocalcemia , hyperphosphatemia , facial anomalies , and psychomotor retardation . most of the cases reported have been associated with parental consanguinity and have come from the arabian peninsula . we report hypoparathyroidism , tetany , short stature , facial dysmorphism and growth retardation suggestive of hrd in a hindu child born to consanguineous couple . a 13-year - old hindu male child 4 born to a consanguineously married couple with uneventful perinatal event , having delayed social and personal development was brought with spasms of the upper and lower limbs . he has been suffering from similar episodes of spasms frequently since the age of 5 years . he also had developmental bilateral cataract for which he was operated at the age of 6 years . at admission , he had carpopedal spasm and his vitals were normal . his weight was 29 kg ( < 5 centile ) , height 132 cm ( < 5 centile ) and head circumference 49 cm ( <3 centile ) . on examination , he had facial dysmorphism features like long face , prominent forehead , bilateral dropping of eyelids , preauricular tag on the right side , bilateral pseudophakia with posterior capsular opacification ( secondary ) , beaked nose , depressed nasal bridge , long philtrum , high arched palate , maloccluded teeth , micrognathia and microcephaly [ figure 1 ] . one of his sisters also had operated for developmental cataract at the age of 4 years , having delayed social and personal development , similar facial dysmorphism [ figure 1 ] but no tetany or latent tetany signs . facial dysmorphism features like long face , prominent forehead , bilateral dropping of eyelids , preauricular tag on the right side , beaked nose , depressed nasal bridge , long philtrum , maloccluded teeth , micrognathia and microcephaly his investigations revealed normal blood counts and peripheral smear . his serum calcium was 6.8 mg / dl , serum phosphorus 7.1 mg / dl , serum magnesium 2.2 mg / dl , serum albumin 3.2 g / dl , alkaline phosphatase 124 u / l and intact pth was 5.46 pg / ml ( n = 15 - 65 pg / ml ) . audiogram showed mild hearing loss on the left side with normal hearing on the right side . he was started on intravenous calcium gluconate slow infusion for 2 days followed by calcium tablets . his facial dysmorphic sister serum calcium , phosphorus , magnesium , albumin , alkaline phosphatase , intact pth and ct head were normal . hypoparathyroidism is an uncommon metabolic disorder characterized by hypocalcemia , hyperphosphatemia with absent or low levels of pth . familial occurrence of hypoparathyroidism with autosomal dominance , autosomal recessive or x - linked recessive have been well - established . hrd or sanjad - sakati syndrome ( online mendelian inheritance in man [ omim ] # 241410 ) is a rare autosomal recessive syndrome predominantly seen patients of arab origin . it is characterized by hypoparathyroidism , hypocalcemia , hyperphosphatemia , facial anomalies , and psychomotor retardation . clinically , the syndrome has typical facial dysmorphism features like long narrow face , deep set eyes , microcephaly , micrognathia , thin lips , long philtrum , beaked nose , depressed nasal bridge , ear anomalies . microcephaly with various degrees of mental retardation ranging from mild to severe degree is usual . the molecular pathology of this syndrome was shown to be due to mutations in the tubulin - specific chaperone e ( tbce ) gene in chromosomal area 1q42-q43 and encoding a tbce . tbce mutations also cause kenny - caffey syndrome ( kcs ; omim 244460 ) similar to hrd syndrome with permanent hypoparathyroidism . they share the same gene locus , but clinically kenny - caffey is characterized by normal intelligence , late closure of anterior fontanel , microcephaly , postnatal growth retardation along with cortical thickening and medullary stenosis of the bones . ophthalmic examination help to differentiate the two syndromes . nanophthalmos and corneal opacity have been documented in kcs patients , but ocular disease has not been well - described in hrd apart from the external ophthalmic features . parvari et al . demonstrated that both maps to the same region and suggested that these were likely to be allelic disorders , if not the same condition . usually they manifest in the new born period with complications of hypocalcaemia as seizures or tetany . however , the age at the diagnosis of the previously reported cases ranged from 4 months to 12 years . our child also had symptoms of hypoparathyroidism as tetany at the age of 5 years along with cataract . affected siblings , mainly as products of phenotypically normal consanguineous parents , have been reported . until now more than 26 patients have been reported , of whom more than half were familial and affected siblings were reported in five non - consanguineous families also . however , prevention could be achieved through pre - implantation genetic diagnosis and carrier detection . to conclude , it is better to do molecular study for tbce gene , which confirm our clinical evaluation and help much in genetic counseling for the family .

congenital hypoparathyroidism , growth retardation and facial dysmorphism is a rare autosomal recessive disorder seen among children born to consanguineous couple of arab ethnicity . this syndrome is commonly known as sanjad - sakati or hypoparathyroidism - retardation - dysmorphism syndrome ( hrd ) . we report 13-year - old hindu boy with hypoparathyroidism , tetany , facial dysmorphism and developmental delay , compatible with hrd syndrome .

urethral diverticular adenocarcinoma is a rare cancer found in female patients , presenting only 5% of female urothelial cancers . we report a case of a urethral diverticular adenocarcinoma after the fenestration of the anterior vaginal wall for pelvic floor abscess . a 61-year - old woman presented to the gynecology department with complaints of atypical genital bleeding . magnetic resonance imaging ( mri ) revealed a localized urethral tumor extended to vagina ( fig . 1 ) . computed tomography did not reveal any evidence of metastasis . under the diagnosis of urethral tumor , the patient was referred to our department . because the fenestration of the anterior vaginal wall was performed under the diagnosis of the abscess in the pelvic floor for the patient 15 years ago and the histological test demonstrates the adenocarcinoma , we suspected a urethral diverticular adenocarcinoma . figure 1:sagittal t2-weighted mri of the pelvis revealed a localized urethral tumor extended to vagina . sagittal t2-weighted mri of the pelvis revealed a localized urethral tumor extended to vagina . on admission , we performed standard open total cystectomy with lymph node excision and ileal conduit . the resected specimen showed the mass on the anterior vaginal wall ( fig . 2 ) . histopathological examination of the specimen showed adenocarcinoma tumor that continues to anterior vaginal wall , but urethral diverticular wall could not be identified ( fig . we could not establish a diagnosis of urethral diverticulum ; however , we clinically diagnosed as urethral diverticular adenocarcinoma . the proximal margin was negative , and all lymph nodes were negative . because the post - surgical course was uneventful , the patient was discharged from our hospital on day 27 post - surgery . figure 3:histopathological examination of the specimen showed adenocarcinoma tumor which continues to anterior vaginal wall , but urethral diverticular wall could not be identified . histopathological examination of the specimen showed adenocarcinoma tumor which continues to anterior vaginal wall , but urethral diverticular wall could not be identified . , there are less than 60 reported cases of urethral diverticula carcinoma , including adenocarcinoma and squamous cell carcinoma . urethral bleeding is the most common presenting symptom , occurring in 51% patients . a painless growth , found initially as either an anterior vaginal mass or a urothelial obstruction , was present in 24% patients therapy for urethral diverticular carcinoma consists of local excision such as diverticulectomy or more aggressive therapy such as definitive radiation therapy or radical cystourectomy with pelvic lymph node dissection . in our case , it is possible that drainage treatment was performed for urothelial diverticulum 15 years ago . patients with urethral diverticula often present with a variety of nonspecific lower urinary symptoms such as frequency , urgency , localized pain or dysuria . approximately 4% cases of female urethral diverticulum cause urinary retention . an asymptomatic patient with urethral diverticulum can be managed by observation . on the other hand , the first and most common choice of treatment for symptomatic urethral diverticulum is complete vaginal excision . however , because of several risks and the high recurrence rate of complete vaginal excision , we have advocated that the management by transvaginal drainage could be the appropriate treatment for urethral diverticulum on the past published case report . this procedure is effective and conservative with few complications , but we should not forget very rare cases of carcinoma arising in urethral diverticula have been reported . in this case , if the urethral diverticulum had removed completely 15 years ago , the generation of malignant tumors might have been avoidable . anyway , for the patient with urethral diverticula , close long - term follow - up for the recurrence or generation of malignant tumors by genitourinary examinations or images is necessary .

a 61-year - old woman presented to the gynecology department with complaints of atypical genital bleeding . magnetic resonance imaging revealed a localized urethral tumor extended to vagina . histological test of the biopsy tissue of the mass suggested the adenocarcinoma . the patient was performed the fenestration of the anterior vaginal wall 15 years ago . under the diagnosis of urethral diverticular adenocarcinoma , we performed standard open total cystectomy with lymph node excision and ileal conduit . we could not establish a diagnosis of urethral diverticulum from the histological test ; however , we clinically diagnosed as urethral diverticular adenocarcinoma . because carcinoma arising from urethral diverticula is reported , a close long - term follow - up for the recurrence or generation of malignant tumors by genitourinary examinations or images is necessary , for the patient with urethral diverticula .

the incidence is about 1 in 60,000 to 1 in 100,000 deliveries . in a large multicenter worldwide research that studied the largest sample of conjoined twins , conjoined twins recognized late in labor is an unfortunate malady associated with very high perinatal mortality and maternal morbidity . such a malady can be easily prevented if a checklist is followed while performing an ultrasound for a multiple pregnancy . a 32-year - old , nulliparous woman with one previous abortion was admitted at 40 weeks of pregnancy with labor pains . she had had a non - consanguineous marriage two years earlier . she was admitted to the labor room at term with pains and draining for half hour . she had been already diagnosed to have a twin pregnancy at 30 weeks of gestation by a general practitioner . the cervix was 3 cm dilated , fully effaced and vertex was at -3 station . her cervical dialation and descent of the first vertex progressed satisfactorily with the partograph line to the left of the alert line . traction on the delivered part did not cause any further descent . neither could the limits of the stalk be made out from the vaginal approach . unfortunately , by this time none of the fetal heart sounds could be clinically auscultated . there was a cartilaginous stalk connecting the twins from the xiphi - sternum to the mid - abdomen . the conjoined twins were carefully delivered intact from the uterus after reverse manipulation from the vagina aided by traction on the second twin from above . both the fetuses were born dead . xiphopagus twins , both female , with a combined weight of 5.4 kg were still born ( figure 1 ) . there was a single placenta and single umbilical cord attached to the middle of the stalk . the cord revealed two arteries and one vein with a velamentous insertion ( figure 2 ) . postnatal skeletal survey showed a cartilaginous connection , normal thoraces and normal skeletal morphology ( figure 3 ) . the wound was re - sutured after controlling infection with antibiotics as per the culture report . figure 2the velamentous insertion of the single umbilical cord . the velamentous insertion of the single umbilical cord . figure 3x - ray of the twin showing normal skeletal survey and separate thoracic cages . xiphopagus twins are joined at the xiphoid process ( part of the sternum ) and usually linked only by cartilage and soft tissue . chang and eng bunker ( 18111874 ) were the first known xiphopagus twins with conjoined livers . their autopsy had revealed a band of liver along with the xiphoid with a median bursa like sac . the synchondrosis had been stretched by these active twins to enable them to stand side by side . they observed that 69% were female , 10% were associated with polyhydramnios , 50% had cephalic - breech presentation , 36% had dystocia and the still birth rate was 19% . they further reported that 6% had only a skin bridge , 81% had conjoined liver , 56% had conjoined sterna cartilage , 17% had conjoined diaphragm , 3% had conjoined genitor - urinary system and 33% had associated other malformations . twenty - seven of these thirty - six pairs had been operated upon of which 18 sets and 7 singles survived . prognosis of these twins depends on the nature and extent of organ sharing and presence of other malformations . hoyle ( 1990 ) analyzed all attempts at surgical separation of conjoined twins reported in the literature until 1987 . the highest mortality occurred among thoracopagus ( 51% ) , craniopagus ( 48% ) , and omphalopagus ( 32% ) . he also noted a significant increase in mortality for emergency separation ( 70% ) compared with elective procedures ( 20% ) . in a recent study , 36 twin pregnancies with conjoined twins seen over a period of 12 years in a single tertiary hospital were analyzed . of these , 69.4% were thoracopagus and 5.6% were omphlaopagus . twelve pregnancies with poor prognosis were terminated and the remaining cases were delivered by cesarean section . xiphopagus twins with only soft tissue bridge , as in our case , carry a very good prognosis after separation , with the earliest survivors operated way back in 1952 . shukla and colleagues reported the case of a pair who was delivered by cesarean for prolonged labor . in this case , also the diagnosis had been missed in the previous scans performed during pregnancy . emergency separation had been undertaken as it was threatening the survival of the live fetus . various techniques have been used to delineate the hepatobiliary and vascular sharing amongst the xiphopagus twins before planning separation surgery . sequential scintiangiography technique using tc-99 m sulfur colloid was used by margouleff et al . for the delineation of the size , configuration , anatomic point of fusion , and quantity of cross circulation in xiphopagus conjoined twins . in our case , it was extremely unfortunate that , in spite of two scans performed on two different occasions by two different observers in the second trimester , this problem was missed . diamniotic placentation almost always rules out conjoined twins with the exception of 3 cases reported in literature . detailed , patient and repeated scans may be necessary to rule out fixed relationship of fetus to each other and hence the possibility of conjoined twins . the canadian task force on preventive health care has made recommendations for the best use of ultrasound in twin pregnancies based on the study by morin and lim . . early diagnosis with scans keeping the checklist in mind can avert unwarranted challenge and dilemma to the obstetrician in the labor room , and morbidity and extreme pain and psychological anguish to the mother . planned delivery in appropriate centers with expertise for separation and post - operative support once the extent of organ sharing is known can reduce the perinatal mortality and maternal morbidity .

conjoined twins are a very rare entity . it is associated with poor survival rate in the presence of vital organ sharing . the entity can be diagnosed as early as the first trimester . a conjoined twin diagnosed late in labor is a malady with high perinatal mortality and maternal morbidity . we present one such case of xiphopagus twins . the management of a case diagnosed late in labor can be very challenging . such obstetric challenges can be avoided by a meticulous early scan with a high index of suspicion , especially in the absence of separating membrane while scanning multiple pregnancies .

a 35-year - old healthy lady presented with high grade fever , severe abdominal pain , nausea , vomiting and profuse watery diarrhea , sometimes green in color . there was no history of animal contact , recent travel or camping . on exam , the patient was hypotensive and was looking acutely ill . she also had hypokalemia of 3.3 mmol / l , acute kidney injury with elevated creatinine of 1.6 mg / dl from a baseline of 0.6 secondary to dehydration . murphy 's sign was positive which prompted us to do a right upper quadrant ultrasound which showed thickened gall bladder wall of upto 1 cm consistent with cholecystitis . patient started feeling better after the surgery and was discharged home . during the post - hospitalization follow - up after 2 weeks campylobacter is a small , slender , gram - negative curved rod , which is one of the most common causes of enteritis in humans . campylobacter fetus may have some attraction towards the gallbladder as in a survey , 20% of slaughtered 700 cattle and sheep harbored this bug in their gallbladder.1 campylobacter can cause cholecystitis without diarrhea unlike the case that we report here . the diagnosis of campylobacter cholecystitis is usually missed because culture of campylobacter is not routinely requested after cholecystectomy . however , even if the bile is cultured , campylobacter appears to be a less common cause of cholecystitis . darling et al cultured about 280 bile samples post cholecystectomy for campylobacter . but none of them grew campylobacter.2 hence routine ordering of bile culture under microaerophilic condition is not recommended unless the gram stain shows gram negative curved rods . 3 resistance of campylobacter fetus to cephalosporins and penicillins was reported as early as 1986.4 majority of the reported cases including our patient had good outcome with cholecystectomy and antibiotics especially erythromycin ( see table 1 ) . only one of the reported cases died , however she had advanced hepatocellular carcinoma.3 there is one case report of relapse of campylobacter bacteremia in a aids patient in about 8 months after the first episode of campylobacter cholecystitis.1 in conclusion , campylobacter cholecystitis is rare but should be kept in the back of the mind while treating a patient with campylobacter enteritis .

there are 13 cases of campylobacter cholecystitis reported so far in the medical literature . among them , only 4 patients had diarrhea . we report another case of acalculous cholecystitis in a setting of campylobacter enteritis . the case report is followed by a literature review regarding this rare condition .

the first case is a 29-year - old female who presented with a 2-year history of multiple bluish nodules particularly on her trunk and limbs , ranging from 0.52.5 cm in size . although some of the lesions had been present since she was 5 years old , their accelerating growth over the past year had caused concern . her mother also reported 23 similar lesions , which had developed during adolescence , on her upper limbs but no investigation had been conducted in the past . no other family member was affected . several soft , partially compressible nontender blue , nodular and multifocal lesions ranging from 0.5 to 2.5 cm in diameter were noted on the back , flanks , thighs , upper arms , palms and soles ( fig.1a , b ) . the second case is a 14-year - old female who presented with multiple subcutaneous nodules on the leg and the trunk , some of which were painful . her younger sister , aged 6 years old , was noted to have similar lesions on her feet ( fig.1c ) . her mother , aged 49 years old , had similar lesions on her trunk , especially on the back and across the shoulder . excision biopsy taken from both case 1 ( nodules in her lower back ) and case 2 ( right leg ) showed a nonencapsulated intradermal tumour composed of thin - walled vascular channels lined by endothelial cells . these were surrounded by irregular layers of cuboidal cells , in keeping with glomus - like cells ( fig.2a , b ) , which stained positively for smooth muscle actin on immunohistochemistry ( fig.2c ) . staining for s-100 was negative . glomus tumours are benign neoplastic proliferations of the glomus body , which is made up of perivascular smooth muscle cells and forms arteriovenous anastomoses , which play an important role in vascular regulation of skin temperature . they present in young individuals as small papules or nodules and are blue or bluish - purple in appearance . the most commonly affected sites are the distal extremities , such as the nail bed or the palm . glomus tumours are typically painful and tender to touch , especially in response to changes in temperature or pressure . histologically , there are solid sheets of glomus cells around small blood vessels [ 3 , 4 ] . glomangiomas differ clinically from glomus tumours in that they occur in childhood and adolescence , do not have a predilection for the subungal region , and often are multifocal . they are usually multiple , soft , red - to - blue nodules but may appear as pink - to - deep - blue plaque - like lesions . the histopathology of glomangiomas differs from glomus tumours in that there are dilated venous channels , resembling venous malformations which stain positively for -smooth muscle actin . most glomangiomas manifest sporadically ; however , there have been reports showing autosomal - dominant inheritance patterns . our cases , both having a strong familiarity with many members affected on different generations , all seem to be of familial type , with dominant inheritance . the chromosome 1p21p22 is thought to be involved , and it has been suggested that this results in loss of function of the protein glomulin , which is considered important for smooth muscle cell differentiation [ 6 , 7 ] . due to the benign , indolent course , often no treatment is required . excision may be more difficult for multiple glomus tumours because of their poor circumscription and the large number of lesions . for these cases , there is a range of possible treatments , including sclerotherapy , electron - beam radiation , argon and co2 lasers or observation of asymptomatic lesions . the prognosis for excised glomus tumours is very good , with a low recurrence rate .

glomangiomas are a variant of benign glomus tumours ; glomus tumours are benign neoplastic proliferations of the glomus body , which is made up of perivascular smooth muscle cells . this forms arteriovenous anastomoses which play an important role in vascular regulation of skin temperature . the aim of this article is to describe 2 cases of hereditary glomangiomas .

the centers for disease control and prevention ( cdc ; atlanta , ga , usa ) has been performing passive surveillance for c. gattii in the united states since 2009 . isolates were collected as part of routine surveillance for c. gattii or submitted to the fungal reference laboratory at the cdc for identification . we identified all isolates to the species level as described ( 8) and used the consensus protocol for multilocus sequence typing ( mlst ) ( 9 ) . in the course of this surveillance , several isolates were received from states in the southeast ; genotypic analysis revealed that these isolates consisted of 2 molecular types , vgi and vgiii ( 8) . of > 400 isolates received from throughout the united states , 42 were molecular type vgi . mlst analysis of vgi isolates showed a cluster of isolates with a single mlst sequence type originating in the southeastern states ( figure 1 ) . five isolates from georgia and 1 each from florida , tennessee , and michigan were indistinguishable by mlst ( sequence type 162 ) . a second isolate from georgia differed from this group by 1 single - nucleotide polymorphism ( snp ) in the intergenic spacer gene locus . maximum - parsimony tree of multilocus sequence typing analysis of vgi isolates of cryptococcus gattii from the southeastern united states . in the predominant clade , 1 isolate was from michigan ; all remaining isolates were from the southeastern united states . nearest neighbor isolates were included for comparison , and an environmental vgi isolate from australia was used as an outgroup . scale bar indicates 1 single - nucleotide polymorphism . because the population of vgi isolates from the southeastern states ( vgi - se ) was only recently identified , it might be a newly emerging population similar to the independent emergences of vgiia and vgiic in the pnw ( 68 ) . to further characterize this cluster , we used whole - genome sequence typing ( wgst ) of isolates from this cluster , other closely related isolates , and unrelated control isolates ( table 1 ; figure 2 ) . , san diego , ca , usa ) , used gatk unified genotyper version 2.4 ( broad institute , cambridge , ma , usa ) for snp detection , and constructed maximum - parsimony snp trees using paup * ( sinauer associates , inc . , sunderland , ma , usa ) , as previously described ( 6 ) . maximum - parsimony tree of whole - genome sequence data of isolates of cryptococcus gattii from the southeastern united states . nearest neighbor isolates were included for comparison , and an environmental vgi isolate from australia was used as an outgroup . although the overall topology of the tree we generated remained almost identical to that seen by mlst , we were able to separate each of the isolates within the cluster using wgst ( figure 2 ) . a total of 41,024 snps were within the vgi - se cluster , which had an average branch length of 4,558 snp differences between any 2 isolates . the isolate from georgia that differed in mlst by 1 snp was placed well within the cluster by wgst . outside the cluster , the nearest neighbor , an isolate collected from a resident of oregon with a history of living near the southeastern united states in texas ( table 1 ) , differed by > 29,000 snps . the other cluster of vgi isolates was separated from vgi - se by 49,992 snps . previously , whole - genome sequencing was performed on isolates of c. gattii molecular type vgii exclusively from the pnw . results showed that the vgii isolates consisted of 3 different highly clonal and recently emerged populations with only 107 , 132 , and 137 snps identified within the vgiia , vgiib , and vgiic populations , respectively ( 10 ) ( table 2 ) . the average branch length between any 2 isolates was < 18 snps for the vgiia and vgiic populations and only slightly higher for the vgiib population . on the basis of snp diversity , the vgi - se subtype is substantially older than the clonal vgii populations in the pnw and has likely been in the united states for a much longer time . * these isolates , accessed from the same national reference laboratory , were submitted without clinical history ; therefore , we can not discern whether they represent the diversity of 2 isolates from 1 patient over time or whether they are part of a local clonal population . all of the patients for whom disease manifestation was known had meningitis ( table 1 ) . this finding differs notably from c. gattii vgii patients in the pnw , among whom a substantial proportion ( 59% ) had a primary pulmonary infection ( 8) . the average age of the patients with isolates in the vgi - se cluster was 46 ( range 1870 ) years . for most patients , the source of infection was unknown , although 2 of the patients had been exposed to rotting wooden structures ( 1 demolished a wooden structure , 1 power - washed old houses ) . our data indicate that the clonal diversity of the c. gattii vgi - se clade in the southeastern united states is an order of magnitude greater than that seen in isolates from the pnw . hypothesized that the emergence in the pnw began in the mid-1990s in british columbia , canada , and spread to the united states in the early 2000s ( 12 ) . comparison of the average number of snps detected among the 3 vgii clonal groups in the pnw with the average number of snps detected between the vgi - se isolates indicates that the vgi - se isolates have been in place substantially longer . the limitation to this conclusion is that we do not know the rate of mutation for the vgi or vgii molecular types ; however , there is no reason to believe that they would be substantially different . there may also be higher rate of recombination within the vgi - se clade related to opposite - sex or same - gender sexual recombination . the isolates from this clade for which the mating type is known are all , but that does not preclude the possibility that some mating type a isolates exist in the environment in the southeastern united states . the first identified isolate of c. gattii in the united states was reported from west virginia in 1924 ( 11 ) , although the isolate was not recognized as c. gattii vgi until decades later ( 12 ) . two other historical reports of c. gattii isolates from the southeastern united states exist : the first , from 1968 , describes clinical and environmental isolates from savannah , georgia ; the other , from 1982 , describes isolates from alabama , tennessee , and louisiana from patients with no travel history to a c. gattii endemic region ( 13,14 ) . although both of these reports use the previous name for c. gattii , c. neoformans serotypes b and c , their results clearly indicate that c. gattii has existed in the southeastern united states for > 40 years , as opposed to the recent emergence in the pnw during the past 2 decades . a more recent report describes a c. gattii vgi isolate from an hiv - positive patient in north carolina ( 15 ) ; those authors surmised that the isolate was related to travel to san francisco , but that assumption may have to be revised . no current molecular clock for c. gattii indicates the time required to generate the level of diversity seen in the vgi isolates however , our findings lend credence to the hypothesis that c. gattii has circulated in the southeastern united states long enough to be considered endemic .

cryptococcus gattii is a recognized pathogenic fungus along the pacific coast of the united states from california to washington . here we report that c. gattii may also be endemic to the southeastern united states and has probably been present there longer than in the pacific northwest .

the world health organization ( who ) panel of experts on odontogenic tumors has defined dentinogenic ghost cell tumor ( dgct ) as locally invasive neoplasm characterized by ameloblastoma , like islands of epithelial cells in a mature connective tissue stroma . aberrant keratinization may be found in the form of ghost cells in association with varying amounts of dysplastic dentin . it occurs in an average age of 50 years with a slight male predilection and equal frequency of involvement of maxilla and mandible in canine to first molar region as a predominant site . the etiology of this rare lesion is still unknown , but it has been suggested that missense mutation in -catenin in wingless integrated ( wnt ) pathway plays a crucial role in the development of dgct . the treatment is conservative enucleation , but local reoccurrence was noted . in this article , the authors present a case of an 80-year - old female diagnosed with dgct , a neoplastic form of calcifying odontogenic cyst ( coc ) , due to its characteristic histologic features , numerous ghost cells and dentinoid material . a female patient aged 80 years had a chief complaint of slow - growing swelling in the right side of the face since 1 year . intraorally , the swelling was hard , nontendered and nonulcerated , involving the right maxillary alveolar ridge . intraoral photograph showing large nonulcerated lesion with obliteration of buccal vestibule radiographic examination revealed the presence of a radiolucent resorptive lesion involving right maxillary residual ridge and maxillary antrum admixed with areas of radio - opacities [ figure 2 ] . occipitomental radiograph showing mixed radiopaque and radiolucent lesion in the right maxilla microscopically , sheets and rounded islands of odontogenic epithelium lined with tall columnar ameloblast - like cells and loose stellate reticulum type cells with mature connective tissue were observed . active formation of dysplastic dentin in connective tissue stroma and prominent eosinophilic transformation of anucleated epithelial cell called ghost cell in epithelium and connective tissue resulting in multinucleated foreign body giant cell reaction were also present [ figure 3 ] . van gieson stain showed positivity for ghost cell and dysplastic dentin , whereas pan - cytokeratin positivity was observed in the odontogenic epithelial islands [ figures 47 ] . histopathology showing odontogenic epithelium and ghost cells in the epithelium and in the connective tissue with foreign body reaction ( 10 ) van gieson stain showing positivity toward dentinoid material immunostain showing strong positivity of odontogenic epithelial islands with pan - cytokeratin ghost cell ( h & e , 40 ) foreign body giant cell reaction adjacent to ghost cell ( h & e , 100 ) coc constitutes 1%2% of all odontogenic lesions occurring in oral cavity . out of this , 88% of coc shows cystic nature , whereas 12% are solid in nature . in 2005 , the who has renamed coc based on its proliferative qualities as calcifying cystic odontogenic tumor ( ccot ) to the cystic type of coc and neoplastic variant as dgct to the solid form of coc . dgct is a neoplastic counterpart of coc described by praetorius et al . in the year 1981 . clinically , the lesion is asymptomatic but causes noticeable swelling with asymmetry of face , which in turn depends on the size of the lesion . mixed features of radiolucent destruction and radiopaque flecks were observed with the wide incidence of 27 decade of life . the histopathology shows a cystic lining of odontogenic epithelium surrounded by numerous ghost cells and formation of dentinoid - like material juxtaposed to epithelial lining . the ghost cells are altered cells undergoing aberrant keratinization arranged singly or in sheets with defined cell outline but devoid of nucleus . it is believed to arise from the different layers of epithelium with a tendency to infiltrate into connective tissue where they evoke foreign body reactions with the formation of giant cells . the classical feature is the presence of dentinoid - like material which is thought to be an inductive phenomenon , whereas howel in 1968 suggested that dentinoid - like material is due to the inflammatory response of the body to the ghost cells . the histopathology of the present case showed the nests of odontogenic epithelium with tall columnar and stellate reticulum - like cells with no inflammatory response in or around the dentinoid - like area . the pathogenesis behind dgct is still controversial and has created a way for researchers to intrude into the molecular level of genes . the wnt and sonic hedgehog play a crucial role in tooth initiation , morphogenesis and differentiation . the plethora of molecules and their intricacy of interaction during tooth formation may lead to homeostatic errors which in turn result in abnormal or no formation of the tooth and other related disorders . it has been suggested that wnt/-catenin signaling promotes tooth neogenesis , but mutation in wnt/-catenin pathway can result in the formation of smaller tooth or misshapen tooth and ectopic eruption of tooth . -catenin is a coactivator of lymphoid enhancer factor ( lef ) , which is a cell type - specific transcription factor that mediates wnt signaling during odontogenesis . therefore , loss or alteration of these factors can cause arrested tooth development at late bud stage . the molecular mechanism / genetic aberration confirmed the potent role of -catenin in the formation of abnormal dysplastic dentin in dgct . [ flow chart 1 ] . molecular pathogenesis of occurrence of dentinogenic ghost cell tumor the immunohistochemistry assessment by piattelli et al . showed positivity for cytokeratin characterizing the presence of an odontogenic epithelium whereas the calcified bodies and ghost cells were devoid of immunoreactivity . the negative immunoreaction represented their derivation either from metaplastic transformation of odontogenic epithelium or a product of a coagulative necrosis of the odontogenic epithelium . the present case also showed strong positivity of odontogenic epithelium with pan - cytokeratin whereas ghost cells and the dentinoid - like material showed bright red / magenta color with van gieson stain . a more radical approach is accepted considering the biological behavior and neoplastic nature of dgct . the patient is under follow - up since 6 months , and no recurrence is observed till date . dgct remains rare and controversial counterpart of coc due to the characteristic ghost cell , dentinoid , and its common resemblance to the benign ameloblastoma . here , we presented a case of dgct which is unique due to multivariate age and histological presentation .

calcifying odontogenic cyst ( coc ) is a term used broadly to define lesions which were either cystic / solid in nature . however , a new term defining dentinogenic ghost cell tumor ( dgct ) , as its neoplastic counterpart , histopathologically showed the presence of dentinoid - like areas , ghost cells and ameloblastomatous - like odontogenic epithelium . this possesses a great challenge to an oral pathologist in diagnosing and differentiating it from solid multicystic ameloblastoma or cocs so as to ensure the biological behavior and pathogenesis behind its multifaceted nature . the author presents an exceptional case of dgct , with special emphasis on its pathogenesis , occurring in an 80-year - old female with facial asymmetry and unique histopathology .

a 31-year - old man who had no significant medical history presented with status epilepticus . he had headache , fever of 39 and generalized tonic clonic seizure without recovery of consciousness . his vital sign was stable except fever . on neurological examination , there was no abnormality except stuporous mental status between frequent generalized tonic clonic seizures . initial blood tests showed no leukocytosis and normal level of inflammatory markers ( c - reactive protein and erythrocyte sedimentation rate ) . cerebrospinal fluid ( csf ) was clean and contained 108 leukocytes / mm ( 83% polymor - phonuclear leukocytes and 17% lymphocytes ) with normal glucose ( 78 mg / dl ) and protein ( 42 mg / dl ) . initial brain mri showed t2 hyperintensity and swelling of isolated bilateral hippocampus , especially ca1 region without any abnormal lesion in other areas ( fig . 1a ) . on the day of admission , we assumed that the patient had herpes encephalitis , and started maximum dosage of acyclovir ( 15 mg / kg three times a day ) which is the hospital protocol for treatment of herpes encephalitis . electroencephalography ( eeg ) showed frequent spike and waves on bilateral fronto - temporal areas dominantly on temporal areas , so we started antiepileptic drugs ( initial phenytoin loading with subsequent add - on of levetiracetam , oxcarbazepine and topiramate ) and midazolam - coma therapy . further serologic tests including polymerase chain reaction ( pcr ) for herpes species were negative and no bacteria and viruses were grown . he recovered from status epilepticus three weeks later , but afterwards he developed kbs which is characterized by hyperphagia ( he ate a large amount of food six to seven times a day ) , hypersexuality ( he wanted to have a sex with the nurses , his ex - girlfriend and his mother ) , hypermetamorphosis , global amnesia ( memory registration was normal , but recall was impaired ) and dysosmia . on the 46th day of hospitalization , the score of his korean - version of mini - mental status examination was 20/30 ( orientation to time- 3/5 , orientation to place- 2/5 , immediate recall- 3/3 , delayed recall- 0/3 , attention and calculation- 3/5 ) . since we considered that his symptoms occurred due to secondary temporal lobe epilepsy , we performed an eeg , but his eeg showed no definite epileptiform discharges . forty - five days later , follow - up brain mri showed isolated bilateral hippocampal atrophy ( fig . finally , we concluded that he had a kbs , and the patient was discharged on the 58th day of hospitalization and his cognitive dysfunction is persistent with minimal improvement for six months follow - up . this report describes a patient presenting as kbs after status epilepticus associated with mri - documented structural lesions exclusively located in bilateral hippocampus . after recovery from status epilepticus , many of the patients show postictal psychiatric phenomenon for a while . typically , the delayed postictal psychosis lasts between 12 hours and 7 days , but occasionally psychiatric symptoms may persist for up to 3 months . however , the constellation of symptoms including hypersexuality , global amnesia , dysosmia , and hypermetamorphosis in this patient was compatible with kbs , and it lasts for a long time . the lesions associated with kbs are assumed to include to affect not only the anterior temporal structures,13 such as amygdala,4 uncus and hippocampus , but also extratemporal structures such as cingulate or orbitofrontal cortex.5 kbs may occur due to various etiology such as of dementia,6 large territorial infarction,7 uncal herniation,8 or herpes encephalitis.9 lee et al.10 reported that the fdg - pet may represent functional anatomy through reduced glucose metabolism over the temporal lobe in the patient with herpes simplex encephalitis . we wanted to observe the glucose metabolism of the temporal lobe and amygdala , but we could not perform the fdg - pet because of his low economic status . this case suggests the possibility that the lesion limited to bilateral hippocampal area may result in the kbs . however , we can not exclude the microscopic or functional involvement of other areas beyond hippocampus such as amygdala that could not documented by mri . the functional neuroimagings such as fdg - pet or spect may give us additional information of the relationship between human behavior and limbic system .

klver - bucy syndrome may result from affection of various location of brain . we report a case of klver - bucy syndrome associated with isolated bilateral hippocampal atrophy without any abnormal lesion in other areas following status epilepticus . a 31-year - old man who had no significant medical history presented with status epilepticus after encephalitis of unknown etiology . he had been recovered from status epilepticus three weeks later , but afterwards he developed klver - bucy syndrome : hyperphagia , hypersexuality , hypermetamorphosis , anterograde amnesia and dysosmia . initial brain mri showed t2 hyperintensity and swelling of isolated bilateral hippocampus , especially ca1 region without any abnormal lesion in other areas . one month later , follow - up brain mri showed isolated bilateral hippocampal atrophy . this is a meaningful case report because this case differs from other reports of klver - bucy syndrome in humans in that the anatomic abnormalities revealed by mri were very selective . we report this case because this case is very educative for above reason . moreover , this report would give us additional information of the relationship between human behavior and limbic system .

female genital mutilation ( fgm ) refers to all procedures involving partial or total removal of the external genitalia or other injury to the genital organs , for non - medical reasons . according to the who classification , there are four types of fgm : type i , partial or total removal of the clitoris and/or the prepuce ( clitoridectomy ) ; type ii , partial or total removal of the clitoris and the labia minora , with or without excision of the labia majora ( excision ) ; type iii , narrowing of the vaginal orifice with the creation of a covering seal by cutting and positioning the labia minora and/or the labia majora , with or without excision of the clitoris ( infibulations ) ; and type iv , an unclassified category including all other harmful procedures to the female genitalia for non - medical purposes , for example , pricking , piercing , incising , scraping and cauterization . fgm is performed on 89% of sudanese women , with the most common procedure being type iii . physical short - term and long - term complications have been well documented . in spite of this , medicalization of fgm has increased in areas of high prevalence , which reinforces and legitimizes this practice and hinders the efforts towards its elimination . we report a case of an inclusion cyst resulting from female genital mutilation which enlarged to the degree of restricting the patient 's movement . this report aims to raise the awareness of the medical community to the dangers that arise from common remote complications of fgm . a 40-year old grand - multiparous woman presented to kassala new hospital ( al - saudi ) with painful genital swelling . three years ago , she noticed a painless swelling in her vulva which was gradually increasing in size . her movement became restricted , and after being unable to walk due to severe pain for a few days , she decided to seek treatment . she was afebrile , with pulse 80 beats per min , blood pressure ( bp ) 130/70 mmhg . local examination showed a large cystic swelling originating in the circumcision line and covering the introitus ( figure 1 ) . her hemoglobin concentration was 10.7 g / dl and her urine analysis was clear . after inserting an indwelling foley catheter , the cyst was excised under spinal anesthesia through a vertical incision in the skin . the cyst was easily removed by dissection along the lines of cleavage . the cyst measured 1110.6 cm and weighed 1.9 kg . the redundant skin was excised and the edges were approximated . there were no complications following surgery and the patient was discharged on day 2 after surgery . the development of inclusion cyst along the line of the scar has been reported as a remote complication of fgm . these result from the embedding and proliferation of keratinized epidermal cells of hair follicles and sebaceous glands within a circumscribed space of the dermis in the stitched area . this is likely to occur during reinfibulation , which is a secondary form of fgm performed on women who have undergone deinfibulation ( being cut open ) to allow childbirth . reinfibulation is usually performed by almost all birth attendants in northern sudan , as fgm is practiced by more than 90% of the female population . the complications after reinfibulation might be severe because the process exposes the woman to a repeated risk of tissue damage . multiparous women usually have heavily scarred and deformed perineums due to repeated episiotomies , deinfibulations and reinfibulations and more frequently present with inclusion cyst formation . these cysts grow slowly , usually without symptoms , may become infected causing pain and discomfort . they may grow to a size that causes coital difficulty but rarely reach a size that restricts movement , as in this case . kroll and miller reported a large inclusion cyst in a somali woman that required the use of both hands so it could be carried while walking . apart from this inconvenience , inclusion cysts cause disfigurement , anxiety , shame and fear of cancer . it is important that health professionals become aware of the complications of fgm and recognize its seriousness . it is an easy operation that health professionals must know how to perform . in conclusion , this is a neglected inclusion cyst that had been left to reach a serious stage . we recommend encourageing patients with fgm complications to seek medical advice and discourage birth attendants carrying out reinfibulation after childbirth .

we report a case of an inclusion cyst , resulting from female genital mutilation ( fgm ) , which enlarged to such a degree that it restricted the patient 's movement . this report aims to raise the awareness of the medical community to the dangers that arise from a common remote complication of fgm .

paraneoplastic neurological disorders associated with anti - ri - antibodies , which are typically present with opsoclonus - myoclonus - ataxia . most cases with anti - ri - antibodyassociated paraneoplastic syndrome due to breast cancer occur in women - its occurrence in men is extremely rare . we present herein the case of a male patient with breast cancer who had atypical anti - ri - antibody - associated paraneoplastic syndrome presenting as complete horizontal ophthalmoplegia , left trigeminal sensory symptoms , and truncal ataxia . following the diagnosis of paraneoplastic syndrome , chemotherapy and immunomodulating treatment including intravenous immunoglobulin and oral prednisolone although the patient was negative for serum anti - ri - antibodies 14 weeks later , his symptoms persisted . to our knowledge , this is the first case report of ophthalmoplegia without opsoclonus - myoclonus in a male anti - ri - antibody - positive patient with breast cancer . paraneoplastic neurological disorders associated with anti - ri - antibodies ( also known as anti - neuronal nuclear antibodies type ii ) mainly present with opsoclonus - myoclonus - ataxia.1 ophthalmoplegia without opsoclonus is very rare.2,3 although anti - ri - antibodies have been reported in patients with gynecological tumors and small - cell lung cancer,1,4 they are mostly identified in patients with breast cancer . its occurrence in men is extremely rare ; there is thus far only one reported case , in which paraneoplastic opsoclonus - myoclonus was found in a man with breast cancer and anti - ri - antibodies.5 we present herein the case of a male patient with breast cancer who had atypical anti - ri - antibody paraneoplastic syndrome presenting as complete horizontal ophthalmoplegia , left trigeminal sensory symptoms , and truncal ataxia . a 52-year - old man was referred to a hospital with subacute onset of binocular diplopia , left facial numbness , and gait disturbance . he was diagnosed with breast cancer 1 month after the onset of symptoms and then underwent a right modified radical mastectomy to remove an invasive ductal carcinoma . his cerebrospinal fluid showed normal cell counts and protein values , and negative cytology and viral markers . immunofluorescence analysis revealed that the patient 's serum contained anti - ri - antibodies ( serial dilutions revealed a specific antibody titer of 1 : 60 ) , and was negative for anti - hu , anti - yo , and neuromyelitis optica antibodies . a brain magnetic resonance imaging ( mri ) scan revealed a high - signal - intensity lesion in the pontine tegmentum that was not enhanced with gadolinium ( fig . 1 ) . a brain magnetic resonance angiogram showed no significant steno - occlusive lesion . initial infusion of intravenous immunoglobulins ( 400 mg / kg / day for 3 days ) was administered 4 months after symptom onset , followed by a maintenance dose ( 400 mg/ kg / day ) every 2 months for 1 year . after four cycles of chemotherapy , the patient 's serum tested negative for anti - ri - antibodies , and a follow - up brain mri showed neither abnormal signal change nor atrophy in the brainstem . further extensive searching , including whole - body positron - emission tomography , produced no evidence of tumor recurrence . however , this patient 's neurological deficit has persisted during the subsequent 3-year follow - up , in spite of intensive chemotherapy and immunomodulating treatment . whilst opsoclonus - myoclonus ataxia syndrome is often used to describe anti - ri - antibody - associated paraneoplastic disorder , it has a wide spectrum of neurological manifestations.6,7 the present case emphasizes the clinical heterogeneity of this disorder . however , even in the absence of opsoclonus , oculomotor dysfunction is usually prominent,8,9 and the brainstem is a major site of autoimmunity . the main clinical features in our patient were brainstem syndrome involving the structures of the dorsal pons : the parapontine reticular formation , abducens nucleus , medial longitudinal fasciculus , and trigeminal nucleus of the spinal tract . in anti - ri - antibody - positive patients , there are reports of an abnormal signal in the dorsal midbrain,10 and atrophy of the cerebellar vermis1 on brain mri , associated with anti - ri - antibodies . in our patient , mri disclosed symmetric hyperintense lesions in the dorsal pons that were reversible with treatment . however , his symptoms persisted even after the lesion had disappeared . tumor removal and chemotherapy did not improve the paraneoplastic abnormalities and the effect of immunoglobulin was also disappointing . thus , it appears that irreversible , rather than functional damage to the neurons causes the symptoms of anti - ri - antibody - associated paraneoplastic syndrome . the underlying tumor in our case was an invasive ductal cell carcinoma of the breast . the incidence of breast caner in man is low in the literature , representing approximately 1% of all breast cancer diagnoses.11 to our knowledge , this is the first case report of ophthalmoplegia without opsoclonus - myoclonus in a male , anti - ri - antibody - positive patient with breast cancer . the findings of this case support the differentiation of anti - ri - antibody - associated paraneoplastic syndrome even in the absence of opsoclonus , and that it can also occur in male patients with breast cancer .

backgroundparaneoplastic neurological disorders associated with anti - ri - antibodies , which are typically present with opsoclonus - myoclonus - ataxia . most cases with anti - ri - antibodyassociated paraneoplastic syndrome due to breast cancer occur in women - its occurrence in men is extremely rare.case reportwe present herein the case of a male patient with breast cancer who had atypical anti - ri - antibody - associated paraneoplastic syndrome presenting as complete horizontal ophthalmoplegia , left trigeminal sensory symptoms , and truncal ataxia . following the diagnosis of paraneoplastic syndrome , chemotherapy and immunomodulating treatment including intravenous immunoglobulin and oral prednisolone were administered . although the patient was negative for serum anti - ri - antibodies 14 weeks later , his symptoms persisted.conclusionsto our knowledge , this is the first case report of ophthalmoplegia without opsoclonus - myoclonus in a male anti - ri - antibody - positive patient with breast cancer .

a 3,240-g male neonate born at the 39th week of gestation was transferred to seoul national university hospital due to severe cyanosis and cardiac arrest . pregnancy and delivery had been uneventful , and the prenatal ultrasound had been normal . there was no history of perinatal distress . although ventilator support was provided , he remained profoundly hypoxemic , and cardiac arrest occurred . after restoration of spontaneous circulation with cardiopulmonary resuscitation ( cpr ) , he was transferred to seoul national university hospital 3 hours after birth . an infantogram taken immediately after admission revealed a pneumothorax on the right side ( fig . 1a ) ; thus , we performed a closed thoracostomy . although the lung was fully expanded after the closed thoracostomy ( fig . transthoracic echocardiogram ( tte ) showed severe tricuspid regurgitation ( tr ) , with a flail anterior leaflet having a thickened , echogenic tip ( fig . the pulmonary valve had a normal appearance , but the antegrade pulmonary flow was nearly absent , consistent with functional pulmonary atresia . a right - to - left shunt was detected through the patent foramen ovale , and patent ductus arteriosus was not present . the right atrium and ventricle were not markedly dilated , favoring the acute occurrence of secondary tr due to a papillary muscle rupture . despite maximum conventional ventilator support , the neonate continued to be severely hypoxemic , with an oxygen saturation of less than 60% . continuous inhalation of nitric oxide , aimed at lowering the pulmonary vascular resistance was attempted , but this produced a minimal increase in oxygenation . other adjunctive measures , including infusion of sodium bicarbonate , inotropic agents , and prostaglandin e5 , all failed to improve either oxygenation or the state of low cardiac output . the patient was placed on venoarterial extracorporeal membrane oxygenation ( ecmo ) at 36 hours of life , and tricuspid repair was undertaken . ascending aortic and bicaval cannulation was implanted , and mild hypothermic cardiopulmonary bypass was maintained . the papillary muscle of the anterior tricuspid valve ( tv ) leaflet was ruptured , showing organized and ischemic change ( fig . we reimplanted the ruptured papillary muscle head into the healthy endocardium ( 7 - 0 polytetrafluoroethylene [ ptfe ] pledget - supported suture ) and created artificial chordae from 7/0 ptfe in the case of papillary muscle re - rupture ( fig . 3b ) . the ligamentum arteriosum was divided , and the patent foramen ovale was preserved . ventilation with nitric oxide was necessary for the next few days , which was followed by extubation on the 11th postoperative day . tte on the 17th postoperative day showed negligible tr and good systolic function of both ventricles ( fig . after 6 months of postoperative follow - up , the patient remained very well and his tte showed normal cardiac function with trivial tr . papillary muscle rupture in a neonate is a rare event that leads to severe mitral or tricuspid insufficiency and is associated with high perinatal mortality . there are few reports in the english literature regarding cases of newborns with critical atrioventricular valve insufficiency secondary to ruptured papillary muscle or chordae tendinae . hypoxia in the newborn is a possible cause of papillary muscle dysfunction , pulmonary hypertension , and tricuspid insufficiency . myocardial ischemia , which can result from antepartum or peripartum asphyxia , congenital heart disease , viral infections , rhesus isoimmunization , thromboembolism , or other more obscure causes , can induce a papillary muscle rupture . furthermore , permanent antenatal ductal closure is an interesting cause of the papillary muscle rupture of the tricuspid valve . if ductal closure occurs suddenly , a tremendous increase in the right ventricular afterload would result during severe fetal stress , and the combined hemodynamic and metabolic insult might provoke an ischemic rupture of the tricuspid papillary muscle . gerry et al . reported two cases of a ruptured papillary muscle of the tv as a complication of cpr . however , tricuspid insufficiency is thought to be a rare complication of blunt , non - penetrating chest trauma . found 24 cases of papillary muscle ruptures in an autopsy study of 546 cases of trauma , but there was only one instance of an isolated rupture of the tricuspid papillary muscle . almost all of these cases had coexisting myocardial lesions such as myocardial ruptures . in our case , the etiology of the papillary muscle rupture is unclear because tr was not diagnosed before cpr . however , soon after birth and prior to cpr , the patient had already developed severe cyanosis , which might have been related to severe tr with functional pulmonary atresia . this supports the echocardiographic and intraoperative findings that suggest that the rupture of the papillary muscle was induced by myocardial ischemia . tr secondary to a papillary muscle rupture may be sufficiently severe to preclude an antegrade pulmonary flow , with a resultant massive right - to - left atrial shunt . medical treatment , which is aimed at reducing the high pulmonary vascular resistance , allows for an increase in the antegrade flow from the right ventricle and may also improve the systemic perfusion . however , the inability to open the ductus limits the therapeutic options , with the exception of ecmo . in our case , severe tr without ductal patency did not provide appropriate vital signs ; thus , the repair of tv was performed after initial stabilization by using ecmo . a number of similar cases have been reported for the use of ecmo as a bridge to tv repair . long - term follow - up studies on ptfe artificial chordae in tv repair in children are lacking . however , promising results have been achieved with the use of the artificial chordae of the mitral valve . freedom from reoperation with chordal placement in the mitral valve position was 94.7% at 5 years and 89.5% at 8 years . considering that ptfe sutures lack the possibility of growth , further studies on the tv position in neonates is warranted .

severe tricuspid regurgitation resulting from a flail leaflet is a rare cause of neonatal cyanosis . we report a neonate with profound cyanosis and severe tricuspid regurgitation caused by a rupture of the papillary muscle supporting the anterior leaflet , without other structural heart defects . ductal patency could not be established . the repair of the tricuspid valve was performed after initial stabilization by using extracorporeal membrane oxygenation .

aspergilloma is a fungal ball which is composed of hyphal structure fungus , fibrin , mucus and cellular debris and settled in a pre - existing pulmonary cavity or an ectatic bronchial . although chest radiography provides valuable information , it can be scanned more effectively by computed tomography ( ct ) . however , in this case report , we presented a case with operated laryngeal carcinoma whom we first had considered to have metastasis and who had received a diagnosis of aspergilloma in ct and positron emission tomography ( pet ) . aspergilloma has the appearance of a solid mass with round or oval tissue density within the cavitary lung tissue in conventional computed tomography ( ct ) ( 1 , 2 ) . it is typical to observe air - values in different forms between the mass appearance and the cavity wall ( 1 ) . the primary cancer or metastases of lung can be monitored in many different forms on ct , such as solitary pulmonary nodules , appearance of spicules - contoured apparent mass , increased local density or ground - glass appearance . early diagnosis and positron emission tomography ( pet ) is very effective in the diagnosis , staging and treatment planning of lung cancer . however , an increased fludeoxyglucose ( fdg ) uptake can be monitored also in various non - malignant lesions , such as granulomatous infections and inflammations ( 5 ) . to the best of our knowledge , there is a small number of patients with aspergilloma who were mistakenly considered to have lung cancer reported in the literature . a 61-year - old male patient who was operated due to laryngeal carcinoma six months ago . first , mdct and then , whole body pet - ct scan ( 643 mbq f-18-fdg i.v . ) was performed for the patient due to the lesion monitored on the right apical in pa radiography of the patient , who was followed up stably . a lesion of 15x10 mm with radially spiculated extensions , in irregular appearance and containing a few microcalcifications was monitored in the apical region of the right lung in thoracic ct ( figure 1 , 2 ) . it was observed during the pet - ct that the lesion had a moderate f-18fdg uptake ( 2.40 suvmax ) ( figure 3 ) . in addition , no focus was detected during the pet - ct scan outside the thorax . the patient was first considered to have metastasis in the light of the existing findings . microscopic examination of the surgical material showed a group of fungal hyphae , and the patient was diagnosed aspergilloma . the patient was not given any antifungal treatment because the entire region was cleaned within the surgical borders and there was not any other focus . thorax ct , it is observed that the lesion shows spiculation into the lung parenchyma in the coronal reformatted images and parenchymal window torax ct , lesion with irregular borders and containing milimetric calcified focuses in the axial mediastinal window in the right apical region of the lung . appearance of the lesion with a moderate fdg uptake in fusion images in pet - ct . red - looking lung tissue in the lesion and its surrounding area monitored off - white yellow in colour in total . aspergilloma , also known as mycetoma and fungal ball , is an entity formed within the cavity and associated with colonized fungi ( type of aspergillus , as the main source ) . aspergilloma is settled within cavities which develop after pathologies , such as tuberculosis , sarcoidosis or blistering emphysema , and preexists ( 3 , 5 , 6 ) . in addition , the occurrence of immune response in fully healthy individuals is extremely rare ( 1 ) . besides the typical aspergilloma , there is also a wide spectrum of aspergilloma - associated pulmonary diseases that can mimic malignancies with their symptoms , findings and radiological characteristics ( 5 , 6 ) . it appears in the form of mass - like appearance with round or oval soft tissue density within the parenchymal cavity without demonstrating any radiologically significant contrast enhancement ( 1 , 2 ) . it usually involves the upper lobes and lower lobe superior segments ( 3 ) . in our case however , its appearance in ct scan was in the form of a lesion with relatively low density which involved parenchyma and had radially spiculated extensions with irregular borders . also , there was no air crescent sign monitored . al . reported that aspergilloma was in the form of solid lesion in most of the patients in their study in which they compared surgical and ct characteristics of aspergilloma ( 7 ) . also , they monitored the lesions in the upper lobes and with low density in half of the patients . in our case , aspergilloma had the characteristic of solid lesion causing shrinkage and spiculation surrounding the lesion parenchyma . pet - ct is an imaging method that facilitates the separation of benign and malignant and provides guiding ( 1 ) . however , low activity tumors and lesions smaller than 1 cm are one of the major causes of false negative results in pet - ct ( 8) , such as carcinoid tumors and metastases of mucinous component . in addition , pet - ct can sometimes show uptake in granulomatous inflammatory conditions such as aspergilloma . in a study conducted with patients with aspergilloma who had been mistakenly diagnosed with lung cancer , although there were not that high suv values in our case ( suvmax 2.2 ) , there was a moderate fdg - pet uptake . due to the existing imaging findings and larynx ca history of the patient , surgical option was preferred giving particular importance to metastasis . although there is a small number of patients diagnosed with aspergilloma in contemplation of lung cancer ( 1 , 3 , 5 ) in the literature , there is only one patient who has been suspected of metastasis and diagnosed with aspergilloma ( 9 ) . it should be kept in mind that aspergilloma may bear characteristics which can mimic malignancy besides the conventional ct appearances and cause false positive pet - ct scans .

introduction : aspergilloma is a fungal ball which is composed of hyphal structure fungus , fibrin , mucus and cellular debris and settled in a pre - existing pulmonary cavity or an ectatic bronchial . it may cause colonization in patients with an immunosuppressive and underlying lung disease . although chest radiography provides valuable information , it can be scanned more effectively by computed tomography ( ct ) . monitoring fungal ball within the cavity in ct provides establishing the diagnosis.case report : however , in this case report , we presented a case with operated laryngeal carcinoma whom we first had considered to have metastasis and who had received a diagnosis of aspergilloma in ct and positron emission tomography ( pet).conclusion : imaging findings may remain limited in definitive diagnosis of aspergilloma . therefore , surgical resection will allow for both pathological diagnosis and treatment .

in a case of acidosis induced by the administration of a mineral acid , such as hcl or nh4cl , acidemia itself increases serum k concentration . it is well known that hyperkalemia is caused by the release of intracellular k into extracellular fluid . in contrast , in animal experiments involving the administration of nonmineral acids , such as lactic acid and methylmalonic acid there was no increase in serum k concetration by a nonmineral acid clinically except in diabetic ketoacidosis . we report 2 cases of hyperkalemia which developed after -fluoroethylacetate was ingested in which serum k was increased to 5.57 meq / l and 6.7 meq / l , respectively , with severe metabolic acidosis . a 59-year - old woman was admitted to the hospital via the emergency room in a semicoma with vomiting and intermittent general convulsions , following the ingestion of 6.0 gm of -fluoroethylacetate ( kilatol f ) in a suicide attempt . the laboratory findings on admission were as follows : hemoglobin 13.6 gm% , hematocrit 39.2% , white cell count 10,000 , platelet count 40,200 , total protein 6.2 gm , and albumin 3.9 gm% . she was in metabolic acidosis ( ph 7.26 , hco3 17 meq / l , pco2 38 mmhg ) . therefore , 24 meq / l of hco3 was given every 2 hours for 24 hours along with 10% glucose solution and 5% dextrose in saline . forty milligrams of lasix was also prescribed every 8 hours for 24 hours . on the second hospital day the ph was 7.34 , hco3 19 meq / l , pco236 mmhg and serum k 4.9 meq / l . bicarbonate and hypertonic glucose solution were given . on the 5th hospital day there was a marked improvement in the ecg , and lidocaine was withdrawn . on the 6th hospital day the results of the laboratory examinations were ph 7.36 , hco3 24 meq / l , pco2 43 mmhg and serum k 3.6 meq / l . a 38-year - old woman brought to the emergency room with convulsions , following the ingestion of 20 ml of -fluoroethylacetate solution 12 hours before in a suicide attempt . the laboratory findings on admission were as follows : hemoglobin 13.2 gm% , hematocrit 37.2% , white blood cell count 6,900 , platelet count 26,200 , total protein 6.5 gm% , and albumin 3.5 gm% . the findings on an arterial blood gas analysis were ph 6.83 , pco2 90 mmhg , hco3 15 mmol / l and po2 55 mmhg ( table 1 ) . in acidosis associated with mineral acids , acidemia , in which there is a predictable increase in serum k concentration occurs while in uncomplicated cases of acidosis associated with organic acids , the serum k concentration usually remains within the normal range . this difference is thought to be caused by the difference of membrane permeabilities between mineral acid and organic acid . normally , there exist some mechanisms to maintain the h balances between intracellular and extracellular fluid and some mechanisms to maintain the balanced k ratio . recently , alder et al . reported that there may be an undetermined relationship between intra - and extracellular h / k ratio , but the definite relationship between serum ph and k concentration remains unclarified . in 1958 he reported that there was a significant increase in serum k concentration in a hcl , nh4cl group after 2 hours and no change in that of the lactic acid and acetic acid groups . in 1980 they reported an increase in serum k concentration in the hcl and nh4cl group but a decrease in it in the initial period in the lactic acid group . mentioned in his review of a physiological phenomenon of lactate transport through cellular membrane , that lactic acid enters into cells in 2 forms , as ionized lactate and as unionized lactic acid . in 1983 oh et al . reported the development of hyperkalemia in dogs in a severe acidosis state ( ph 6.9 , hco3 6.0 he reported that the serum k concentration decreased below the baseline for the first 2 hours , before it started increasing . he suggested that hyperkalemia might develop if severe acidosis induced by nonmineral acid were to have been prolonged further . there have been a few reports to the effect that hyperkalemia has developed in humans with organic acidosis , especially diabetic ketoacidosis or lactic acidosis . the fluoroacetate which the patients had taken is a highly toxic , nonmineral acid which comes from the south african plant , dichapetalum cymosum . when administered to living subjects it interrupts the cellular metabolic process by blocking the citric acid cycle . fluoroacetate itself is nontoxic , but once transformed into fluorocitrate acid it is highly toxic , causing generalized convulsions and severe ventricular arrhythmia .

when acidemia occurs associated with mineral acids , there is a predictable increase in the serum k+ concentration . in uncomplicated cases of acidosis associated with nonmineral organic acids , the serum k+ concentration usually remains within the normal range . the mechanism of this different effect of mineral and organic acidemias on the transmembrane movement of k+ remains undefined.we observed increases of serum k+ concentration in 2 patients who ingested -fluoroethylacetate solution for the purpose of committing suicide

however it is considered to have a high mortality through cardiac tamponade or exsanguination and concomitant chest or abdominal cavity injury . we present a 39-year - old schizophrenic woman who attempted suicide with the aid of a 6.35 mm caliber handgun , after self - discontinuing of antipsychotic treatment . lower third of sternum , right heart atrium and ventricle and inferior caval vein were hit by the bullet which consequently got lodged in the right paravertebral muscle mass at the lower thoracic vertebral level . as she was hemodynamically unstable due to hemopericardium and a huge right hemothorax , she underwent emergent surgery . the postoperative course was uneventful and she was transferred to a psychiatric facility on the 7th postoperative day . one year after the surgery she is well , compliant to antipsychotic medications and on periodic follow - up by psychiatrists . this case represents management of complex self - inflicted gunshot cardiac injury in a schizophrenic patient who discontinued antipsychotic medication . liaison between themedical rescue service and high level trauma center essentially reduced injury - to - surgery time . unlike other penetrating chest trauma , the hyperkinetic projectile gunshot injuries are prone to cause complex heart injury or concomitant chest / abdominal injury . therestrictive firearm laws reflect low rate of self - inflicted gunshot trauma in western europe countries . up to 40% of schizophrenic patients a 39-year - old schizophrenic lady attempted suicide when she discounted psychiatric treatment for two weeks . she was immediately intubated at the scene , volume resuscitated and catecholamine commenced to maintain organ perfusion pressure and were quickly transferred by helicopter to the emergency room ( er ) of our hospital . injury - to - gate time was 78 min ( 87 km transfer distance ) and cardiothoracic team was alerted and on standby . on admission to er she was stabilized on low - to - medium catecholamine support ( norepinephrine 0.15 ug / kg / min ) , sedated and mechanically ventilated . a gunshot wound was evident in the lower third of the sternum ( fig . 1 ) and surprisingly the projectile was felt on deep palpation at the level of the right 9th rib paravertebrally . chest and abdominal ct scan revealed a moderate volume hemopericardium ( 13 mm ) , a huge right hemothorax and a metallic projectile located in thebody of the right paravertebral muscles at the level of the fractured the 9th rib ( fig . 3 ) . emergent surgery through median sternotomy was performed ; 300 ml of blood was evacuated from pericardium and 800 ml from the right pleural cavity . the projectile trajectory fractured lower third of the chest bone , punctured the anterior walls ofthe right ventricle and right atrium and perforated inferior vena cava . the bullet was finally lodged inthe paravertebral muscle at the level of the right 9th rib . due to the complex nature of the cardiac injury and profuse bleeding from inferior vena cava , the injuries were repaired on extracorporeal circulation with bicaval cannulation without cardiac arrest ( fig . 4 ) . wounded free wall of the right atrium and ventricle were sutured with four interrupted 4/0 polypropylene stitches buttressed by pericardial strips , and consequently the defect in the inferior caval vein was closed with continuous 5/0 polypropylene stitch ( prolene , ethicon , uk ) . the postoperative course was uneventful and she was transferred to the psychiatric facilityon the 7th postoperative day . one year after the surgery she is well , compliant to antipsychotic medications and on periodic follow - up by psychiatrists . chest or upper abdomen gunshot wounds need to be suspected along with the cardiac injury in these instances , , . there is a considerable risk of concomitant chest or abdominal cavity injuries due to high kinetic energy of the projectile or secondary injuries due to high velocity bone fragments . furthermore , gunshot injuries are prone to be complex : they create septal shunts , disrupt valves or papillary muscle apparatus , or might lacerate coronary arteries , particularly the left anterior descending artery ( 39% of all cases ) , . if the gunshot is located at the front of the body , the right ventricle injury dominates followed by the injury of left ventricle , right atrium , and great vessels , . scoop and run approach including prompt diagnosis , speedy transportation and emergent surgery are paramount factors for a favorable outcome of penetrating cardiac injury victims . pericardial effusion ( > 5 mm ) confirmed by sonography and ct - angiography in thoracic penetrating trauma are indications for surgical heart exploration , . in case of refractory hemodynamic instability in penetrating chest trauma , emergency department thoracotomy is justifiable in an attempt to relieve cardiac tamponade or to control bleeding , . hemodynamic instability , cardiac arrest and prolonged injury - to - gate time were identified to be independent risk factors for death . on the other hand , midline sternotomy , routine heart surgery experience , and availability of extracorporeal circulation increase the chance to survive gunshot heart injury , . nevertheless , it requires immediate transfer of victims to high level trauma center with cardiothoracic surgery background , , . suicide is the major cause of premature death in patients with schizophrenia . among these patients , 40% report suicidal thoughts , young age , depression , alcohol or drug misuse , history of prior suicide attempt and discontinuation of antipsychotic medications are the major risk factors for suicide among individuals with schizophrenia . more than 85% of schizophrenic patients end their life by jumping from a height or by jumping in front of a moving vehicle . however , the use of firearms in suicidal intent is rare , particularly pointed at the heart . this case represents successful management of complex gunshot cardiac injury in a schizophrenic patient who attempted suicide after self - discontinuing of antipsychotic treatment . tight cooperation between rescue medical service and high level trauma center played important part in shortening injury - to - surgery time . petr santavy managed the patient as attending and indicating consultan , performed the surgery all authors read and approved the final manuscript .

highlightsself- inflicted gunshot injury to the heart is uncommon in western europe.up to 40% of schizophrenic patients have history of suicide attempt.discontinuation of antipsychotic medications is one of the major risk factor for suicide among individuals with schizophrenia.midline sternotomy provides superior access to the heart and large vessels , pulmonary hilum as well as for access for extracorporeal circulation .

a 30-year - old female patient presented with a history of weakness and breathlessness . along with these complaints she also had pain in gums on both sides . patient underwent f-18-fluorodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( ct ) for initial evaluation . it demonstrated a necrotic mass in right atrium with multiple fdg avid lesions in both upper and lower alveolus , liver , multiple bones , and bilateral lungs [ figures 1 and 2 ] . further , patient underwent cardiac magnetic resonance , which showed a lobulated heterogeneous mass lesion on t1/t2 arising from right atrium with few hyperintense foci within the lesion suggesting hemorrhage / necrosis [ figure 3 ] . whole body f-18 fdg pet / ct : ( a ) maximum intensity projection demonstrated fdg uptake in the region of heart ( thick arrow ) and multiple foci of increased tracer uptake involving liver ( thin arrow ) and skeletal sites ( outlined arrow ) . ( b , c ) transaxial fused and ct images at the level of thorax showed large necrotic mass lesion in the region of right atrium with increased radiotracer uptake in the periphery ( thick arrow ) . ( d , e ) head and neck transaxial fused and ct images showed irregular soft tissue density lesion with increased fdg uptake involving bilateral alveolar region with subtle necrosis . f-18 fdg pet / ct transaxial fused and ct images : ( a , b ) transaxial images at the level of liver showed multiple foci of increased tracer uptake and large hyperdense mass lesion in segment v / vi of right lobe with mildly increased tracer uptake in the periphery ( thin white arrow ) . ( c - f ) transaxial images showed lytic lesions in multiple vertebrae ( thick arrow ) with increased radiotracer uptake and multiple nodules in bilateral lungs ( thin black arrow ) suggestive of metastases . cardiac magnetic resonance images : ( a ) large lobulated intracavitary right atrial ( ra ) mass lesion with ra free wall appearing heterogenous on t2wi- 4ch trufi ( four chamber true fast imaging with steady - state free precession ) . ( b ) perfusion images showed nodular uptake of contrast predominantly in the periphery ( thin arrow ) with no significant enhancement in rest of the mass ( thick arrow ) . ( c , d ) post gad / psir images at 5 min demonstrated patchy nodular intense enhancement ( thin arrow ) . primary cardiac tumors are rare and comprise only 0.001 - 0.0028% of autopsy report . among these , approximately 20 - 25% are malignant of which angiosarcoma is the most common . surgical removal is the definitive treatment for localized angiosarcoma and so preoperative evaluation to accurately localize the primary tumour and detect metastasis is important . although f18 fdg pet / ct has been used in staging of various tumor , its use in staging of cardiac tumor is not well defined . limited data are available in use of f18 fdg pet / ct in primary cardiac tumor . in present case , there was unusual presentation of gum swelling and along with diagnostic dilemma , the extent of disease was also not clear . f-18 fdg pet / ct in this patient not only localized probable primary site but also helped in accurately staging the disease by detecting metastatic sites .

primary cardiac tumors are rare with angiosarcoma being the most common among malignant cardiac tumor . we present a case of 30-year - old female patient in whom f-18-fluorodeoxyglucose positron emission tomography / computed tomography demonstrated a necrotic mass in right atrium with multiple fluorodeoxyglucose avid lesions in both upper and lower alveolus , liver , multiple bones , and bilateral lungs . patient underwent biopsy from gum swelling which revealed metastatic angiosarcoma .

we measured drug inhibition of hiv-1 infection using a single round infectivity assay as previously described . we chose a single round assay because complex factors such as growth and death of target cells distort m in multiround assays . to accurately mimic infection events in vivo , we carried out infections in primary cd4 t lymphoblasts obtained by pha activation of peripheral blood mononuclear cells from health donors as previously described . all blood donors provided informed consent according to a protocol approved by the johns hopkins institutional review board . we infected cd4 t lymphoblasts with recombinant hiv-1 pseudoviruses generated by transfection of hek 293 t cells with a previously described hiv-1 vector ( pnl43-e - egfp ) containing a gfp - tagged , defective envelope . the envelope was provided in trans by co - transfection with an envelope expression vector . in most cases , we used a cxcr4-tropic envelope ( hxb2 ) because it gave higher level infection of primary cd4 t lymphoblasts , thus affording a wider dynamic range . however , ic50 and m values obtained in infections with ccr5-tropic pseudoviruses were not significantly different from those obtained with cxcr4-tropic viruses ( supplementary table s8 ) , and modes of drug interaction were also similar ( supplementary table s7 ) . to account for individual variation in cellular uptake and metabolism of drugs , we performed replicate assays in lymphoblasts from 1060 donors for each drug and computed average levels of inhibition . we used high serum concentrations ( 55% ) to account for protein binding . for protein bound drugs , titrations of drug effect vs. serum concentration demonstrated that inclusion of 50% human serum and 5% fetal calf serum provided a close approximation for in vivo drug binding . drugs were added to lymphoblast targets , and cells were incubated at 37c for 16 hours before infection to allow sufficient time for triphosphorylation of nrtis . after spin infection in 96 well plates , targets incubated for 3 days at 37c . cells were then fixed in 2% formaldehyde , and gfp expression was analyzed by flow cytometry . after gating for viable cells , the fraction of infection events unaffected by drug ( fu ) was determined as the % gfp cells in the presence of drug divided by the % gfp cells in control wells without drug . calculation of iip from fu is described in supplementary material , methods . to determine whether the combined effect of two antiretroviral drugs followed the predictions of the bliss or loewe models , we tested pairs of drugs at constant molar ratios chosen to maximize the difference between the levels of inhibition predicted by the two models . drug combinations are often evaluated in surface experiments in which all possible combinations of several different concentrations of each drug are tested . however , the difference between the inhibition predicted by the two models increases with drug concentration ( fig . 2b ) and is maximal when both drugs contribute equally to the observed suppression ( r. siliciano , unpublished observation ) . therefore , we chose drug concentrations such that at the highest concentrations used , the total inhibition predicted by the bliss model would be within the dynamic range of the assay ( iip < 3 ) , with each drug contributing equally . experimental analysis was carried out in cells from 515 donors / combination as described above .

highly active antiretroviral therapy ( haart)13 has dramatically decreased mortality from hiv-1 infection4 and is a major achievement of modern medicine . however , there is no fundamental theory of haart . elegant models describe the dynamics of viral replication3,59 , but a metric for the antiviral activity of drug combinations relative to a target value needed for control of replication is lacking . treatment guidelines10,11 are based on empirical results of clinical trials in which other factors like regimen tolerability also affect outcome . why only certain drug combinations control viral replication remains unclear.here we quantify the intrinsic antiviral activity of antiretroviral drug combinations . we show that most single antiretrovirals exhibit previously unappreciated complex non - linear pharmacodynamics that determine their inhibitory potential at clinical concentrations . we demonstrate that neither of the major theories for drug combinations accurately predicts the combined effects of multiple antiretrovirals . however , combined effects can be understood with a novel approach that considers the degree of independence of drug effects.this analysis allows a direct comparison of the inhibitory potential of different drug combinations under clinical concentrations , reconciles the results of clinical trials , defines a target level of inhibition associated with treatment success , and provides a rational basis for treatment simplification and optimization .

in obstetrics and gynecology angiographic embolization has been successfully used in patients with life - threatening intractable pelvic hemorrhage when routine medical and conservative methods of bleeding control have been tried and not found effective . management options available to treat intractable pelvic hemorrhage include : hysterectomy , bilateral internal artery ligation and uterine artery embolization ( uae ) . since embolization preserves the uterus and avoids the hazards associated with its removal , it is emerging as a preferred technique to arrest intractable obstetrical hemorrhage . we studied the clinical profile and outcome in 35 patients requiring uae for intractable obstetrical hemorrhage over a period of five years ( from august 2005 to august 2010 ) . all the procedures were performed under local anesthesia , percutaneous catheterization was done through the femoral artery , arteriogram taken to visualize the arterial arcade and catheter advanced in to uterine artery and artery occluded with polyvinyl alcohol particles 500 - 700 m and/or gel foam pledgets / coils . there were seven post lower segment cesarean section ( lscs ) cases out of which six patients were with secondary postpartum hemorrhage ( pph ) including one case of placenta accreta and one patient with primary pph ; post vaginal delivery nine patients presented with primary pph and four with secondary pph , of these seven had atonic pph , three were with coagulation abnormalities of which one was with puerperal sepsis , two had undergone manual removal of the placenta and one case with one traumatic pph [ table 1 ] . in 13 patients , eight patients underwent uae to control bouts of bleeding following repeated dilatation and curettage ( d and cs ) for first trimester abortions , three patients had second trimester abortions and two patients with gestational trophoblastic disease ( gtd ) on chemotherapy with heavy bleeding underwent uae . all patients were hemodynamically unstable , but with normal coagulation profile except three which was simultaneously corrected , hcg was sent in all patients with postabortal hemorrhage at admission to rule out gtd . uae was done successfully in all patients followed by d and c in next 24 h if required . there was one patient with cervical ectopic pregnancy who underwent uae followed by ultrasonography ( usg ) guided evacuation within 24 h of uae with an uneventful post evacuation period . day 15 post embolization no definite arterial flow was appreciated on usg and patient remained asymptomatic on follow - up . causes of obstetrical haemorrhage requiring uae maximum number of women 42.86% were in the age group of 26 - 30 years [ table 2 ] . distribution according to age distribution according to gravidity we had 100% of success rate with no post procedure hysterectomy and there was no mortality . the requirement of blood products was significantly less post uae ( p - 0.006917 ) in post vaginal delivery and ( p - 0.000231 ) in postabortal patients on paired t - test [ table 4 ] . bt requirement pre- and post - uae a total of 17 patients had post embolization syndrome , which was managed with analgesics . pph is a potentially serious obstetric complication and its management represents an issue of critical concern to an obstetrician . after failure of conservative local measures , disadvantages of surgical treatment include significant failure rates for hypogastric artery ligation , the need for general anesthesia and surgical complications including infection , bleeding and ureteric injury . transcatheter arterial embolization has recently emerged as a highly effective percutaneous technique for controlling acute and chronic uterine / pelvic hemorrhage in a wide variety of obstetrical and gynecological conditions . benefits for the patient and health - care providers have included low complication rates , avoidance of surgical risks , fertility preservation and shorter hospital stay . one drawback of the procedure , though highly effective as demonstrated by the literature as well as confirmed by our experience is the risk of radiation as the procedure takes about 30 min with experienced hands at our center . the amount of radiation to ovaries during the procedure has been found to be : 22 - 66 cgy . this is 30 - 100 times higher than those during conventional diagnostic radiographic examinations ( hysterosalpingogram ) yet it is much lower than the doses required for irradiation of malignancy . our clinical experience suggests that embolization should be used before surgical treatment of pelvic bleeding in many clinical settings , including postpartum , post - cesarean and postabortal bleeding . some studies using embolization in obstetrics and gynecology practice have already been conducted [ table 5 ] .

aims and objectives : the objective of this study is to review the clinical profile and outcome in patients requiring the angiographic embolization in obstetrics.methods:the retrospective data of patients requiring uterine artery embolization for control of obstetrical hemorrhage was studied during the period from august , 2005 to august , 2010.results:a total of 35 patients with obstetrical hemorrhage ( seven post lower segment caesarean section , 13 post vaginal delivery , 13 postabortal , one with cervical pregnancy and one patient post laparotomy for abdominal pregnancy ) underwent angiographic embolization for control of bleeding . in all patients , hemorrhage was successfully controlled ; none required post procedure hysterectomy and one patient subsequently became pregnant with in a year.conclusion:our clinical experience suggests that embolization is superior first - line alternatives to surgery for control of obstetric hemorrhage including postpartum , post - cesarean and postabortal bleeding and prevents hysterectomy .

to report a rare case of encephalocraniocutaneous lipomatosis ( eccl ) presented with characteristic multiple organ involvement . a 7-day - old white iranian girl was referred with ocular , skin and brain abnormalities . the findings of nevus psiloliparus , eyelid choristoma and intracranial lipoma were consistent with eccl . since the skin and ocular manifestations can be easily observed at birth examination , pediatricians and ophthalmologists should be aware of this condition . encephalocraniocutaneous lipomatosis ( eccl ) , also known as fishman syndrome , is a rare congenital neurocutaneous disease that commonly involves ectomesodermal tissues , such as eye , skin , and central nervous system.1 , 2 nevus psiloliparus ( np ) , a rare skin anomaly characterized by alopecia and an excessive amount of fat tissue is the hallmark of eccl . eyelid choristoma is the predominant ocular feature , while intracranial lipoma is the major central nervous syndrome ( cns ) manifestation . we present a 7-day - old girl with ocular , skin , and cns malformations consistent with eccl . a 7-day - old white iranian girl was referred to the oculoplasty clinic for ophthalmic evaluation . she was a full - term infant from healthy , non - consanguineous parents after an uncomplicated pregnancy . she was born with cesarean - section ( c / s ) due to previous c / s . general physical examination showed a length of 49 cm , weight of 3.2 kg and head circumference of 35 cm , which were in normal ranges . maternal screening tests for trisomy 13 , trisomy 18 , and down syndrome were negative . ophthalmic examination showed multiple skin - colored papules and pedunculated lesions located on her right and left eyelids , and lateral canthus , right upper eyelid coloboma , right bulbar conjunctival hypertrophy , right limbal dermoid , corneal haziness and corneal peripheral vascularization extending from 2 to 6 o'clock in the left eye ( fig . 1 ) . fundus examination of the right eye was not possible due to corneal opacity , but left fundus showed a peripapillary hypopigmented creamy - white irregular choroidal lesion ( 67 disc diameters ) . ultrasonographic scan showed high intensity echo spikes and highly reflective choroidal mass with posterior acoustic shadowing in favor of posterior globe calcification ( fig . dermatological examination showed a soft , elevated area of patchy hair loss on the right scalp extending to the forehead without signs of inflammation or scarring , clinically compatible with np ( fig . 1 ) . routine laboratory examination including complete blood count , erythrocyte sedimentation rate , and c - reactive protein were normal . axial non - contrast computed tomography ( ct ) scanning of the brain and orbit revealed fat density lesions in subcutaneous tissues of the right temporal region and ipsilateral suprasellar , and cerebellopontine angle hypodense lesion suggestive of lipomas . focal calcifications were seen in the posterior globe of both eyes as well as extraconal area of the right eye . the eccl or fishman syndrome is presented with congenital skin , eye and brain lesions . the etiology is most likely due to dysgenesis of the anterior neural tube and cephalic neural crest . a nonhereditary , autosomal mutation that may survive only in a mosaic state may be a cause of the clinical picture of eccl . the first description of this syndrome as encephalocraniocutaneous lipomatosis was by haberland and perou in 1970 . afterwards , fishman et al in 1987 reported more cases of eccl . in a literature search on pubmed database , 77 patients with fishman syndrome were found ( accessed on september 2013 ) . there is no clear gender , racial , or geographical predilection . the diagnosis of eccl is mainly based on history , clinical examination , and imaging studies . maclaren et al in 1995 and hunter in 2006 have laid down diagnostic criteria , but moog in 2009 proposed revised diagnostic criteria for eccl . ocular lesions are always present and consist of conjunctival choristoma , with or without associated anterior chamber anomalies . persistent hyaloid vessels , lens dislocation , iris dysplasia , aniridia , colobomas , microphthalmia , ocular calcifications , and optic disc pallor have also been reported.9 , 10 the most characteristic scalp lesion in eccl is np , a soft , subcutaneous mass with demarcated area of alopecia . brain abnormalities include cerebral and spinal lipomas , intracranial calcifications , abnormal intracranial vessels , ventricular and subarachnoid enlargement , agenesis of the corpus callosum , intracranial cysts , cortical dysplasia , and brain atrophy.7 , 8 , 10 there is no clinical correlation between the clinical manifestations of eccl and severity of brain malformations . differential diagnosis with other neuroectodermal syndromes such as oculocerebrocutaneous syndrome ( occs or delleman syndrome ) , focal dermal dysplasia , ( or goltz syndrome ) , sebaceous nevus syndrome ( epidermal nevus syndrome ) , oculo - auriculo - vertebral syndrome ( goldenhar syndrome ) should be considered . in occs syndrome , brain malformations are not rare and often show a consistent pattern of forebrain anomalies and a very characteristic mid - hindbrain anomaly , both of which are unknown in eccl or any other syndrome . in goltz syndrome , brain anomalies are rare and much less frequent than in eccl , occs , or sebaceous nevus syndrome . sebaceous nevus syndrome is a mosaic condition with various linear nevi , epilepsy , and mental retardation . these nevi are composed of hyperplastic sebaceous glands , atypical apocrine glands , and immature hair follicles . in goldenhar syndrome , epibulbar choristomas are characteristic features , but in this syndrome , there is a variety of abnormalities including preauricular appendages , hemifacial microsomia , and vertebral , digital , urogenital , or heart anomalies not present in eccl . the treatment of ocular lesions includes the excision of the conjunctival tumors and lamellar or penetrating keratoplasties . eccl patients may have a normal development or be severely retarded . in conclusion , this is the first case of fishman syndrome in the iranian population who presented with characteristic multiple organ involvement . since the skin and ocular manifestations are always present in eccl and can easily be observed at birth examination , pediatricians and ophthalmologists should be aware of this condition . neuroimaging studies must be carried out in all patients suggestive for eccl to rule out brain abnormalities . parents should be reassured that this genetic syndrome will not be transmitted to the offspring.application of the criteria to the diagnosis of encephalocraniocutaneous lipomatosisdefinite casethree systems involved ( major criteria ) orthree systems involved , proven nevus psiloliparus ( np ) or possible np + > 1 of minor skin criteriatwo systems involved with major criteria , one of which is proven np or possible np > 1 of minor skin criteriaprobable casetwo systems involved , major criteria in bothtwo systems involved , proven or possible np

purposeto report a rare case of encephalocraniocutaneous lipomatosis ( eccl ) presented with characteristic multiple organ involvement.methodsa 7-day - old white iranian girl was referred with ocular , skin and brain abnormalities.resultsthe findings of nevus psiloliparus , eyelid choristoma and intracranial lipoma were consistent with eccl.conclusionsince the skin and ocular manifestations can be easily observed at birth examination , pediatricians and ophthalmologists should be aware of this condition .

however , not every orthopaedic surgeon has had the opportunity to observe a case of simultaneous shoulder dislocation with ipsilateral humeral shaft fracture . the literature related to this rare injury is limited to case reports often with inadequate follow ups . herein we present a case of a 15-year - old boy with an anterior shoulder dislocation and ipsilateral fracture of humerus , and discuss his non - invasive treatment . the patient was a 15-year - old , boy who had been working at a bakery , and his right upper extremity had been caught in an electrical mixer used to mix wheat dough . he had sustained a combined anterior dislocation of shoulder with ipsilateral fracture of humeral shaft and greater tuberosity ( figure 1 ) . the patient underwent an urgent attempt for closed reduction of both shoulder and humeral shaft under general anesthesia . counter traction method by grasping the proximal fragment and traction against the counter traction through a rolled sheet in the axilla of the patient ( figure 2 ) . the successful closed reduction , achieved at first attempt , was followed by coaptation plaster splint for four weeks followed by sarmiento splint for an additional four weeks ( figure 3 ) . anteroposterior radiograph showing an anterior dislocation of the right glenohumeral joint with ipsilateral fracture of humerus . the neurologic exam for axillary and radial nerve was normal before and after the procedure . despite the somehow common occurrence of the shoulder dislocation and humeral shaft fracture , simultaneous occurrence of them is rare such that all the reports in the literature are restricted to case reports by various authors . in 1977 chen et al . reported two cases of this injury , and their attempt at reviewing the literature only revealed only 14 case reports . they first fixed the humeral fracture with a plate , and then closed the reduction of shoulder dislocation in both cases . . also reported one case treated by humeral shaft internal fixation with plating and closing the reduction of shoulder dislocation . review of various cases presented in the literature shows that the main problem for surgeons has been the lack of adequate lever arm to do the closed reduction of the joint . this forces surgeons to first fix the shaft by a plate or external fixator , and then to attempt closed reduction of the shoulder joint . there is , however , a report , similar to our experience , of successful closed treatment of both problems . the other problem with this rare combination is the possibility that shoulder dislocation is missed , especially if it is posterior , and the x ray is of poor quality and does not clearly show the shoulder joint position . the present case indicates that closed reduction of both injuries under general anesthesia was accopanied by good clinical results 15 years later .

simultaneous dislocation of shoulder and humeral shaft fracture is a rare injury , and there is no clear protocol for its treatment . herein we present a case of a 15-year - old boy , who suffered from a job - related accident and sustained fracture of humeral shaft associated with ipsilateral anterior shoulder dislocation and fracture of greater tuberosity 15 years ago . he received closed reduction of both injuries and coaptation plaster splint for four weeks , followed by sarmiento splint at that time . fifteen years after the injury , he has no problem related to the previous injury , and does not experience any episode of shoulder instability .

acquired deficiency of the inhibitor of the first component of human complement ( c1-inh ) is an extraordinarily rare cause of intermittent episodes of angioedema , with approximately 100 cases reported in published literature.1 underlying low grade lymphoid hemopathies , in which immediate chemotherapy is typically unwarranted , may be associated with acquired c1-inh deficiency . in such patients , treatment with rituximab ( genentech , inc . , san francisco , ca , usa ) can ameliorate symptoms of angioedema.2,3 a 51-year - old male with a past medical history of chronic obstructive pulmonary disease and depression described a 2-year history of intermittent edema of the hands , feet , genitals , and tongue occurring approximately once per month . he had been managed acutely for these symptoms with epinephrine injection , corticosteroids , and antihistamines with no improvement . he was seen in consult in the critical care unit of wake forest baptist medical center , winston - salem , nc , usa after an episode of laryngeal edema requiring intubation . an undetectable c4 of < 5 mg / dl ( reference range [ ref ] 1570 mg / dl ) prompted administration of 30 mg of subcutaneous ecallantide ( dyax corp , burlington , ma , usa ) , which led to rapid resolution of swelling . further complement assays were consistent with acquired c1-inh deficiency : c1-inh of 5 mg / dl ( ref : 2139 mg / dl ) , a c1-inh function of 16% ( ref : > 68% ) , and undetectable c1q of < 3.6 mg / dl ( ref : 58.6 mg / dl ) . evaluation to ascertain an etiology of acquired angioedema led to the discovery of an increased total number and percentage of b - cells ( 1,240 cells/l [ ref : 50870 cells/l ] and 34% [ ref : 5%17% ] ) . in addition , the percentage of t - lymphocytes was low ( 62.9% [ ref : 66%90% ] ) ; however , the total t - lymphocyte count was normal ( 2.391,000 cells/l [ ref : 0.664.591,000 cells/l ] ) . peripheral flow cytometry identified a b - cell population with excess kappa expression that was cd5/10 negative and cd19/20 positive , suggesting a monoclonal b - cell population . three months after hospitalization , the patient was evaluated by the oncology team at wake forest baptist medical center . at this time a positron emission tomography - computed tomography ( pet - ct ) demonstrated no hypermetabolic activity or evidence of lymphoma , but did reveal a spiculated 1 cm nodule in the right apical lung . the lung nodule was an incidental finding , not believed to be associated with the monoclonal b - cell population nor acquired angioedema . though the watch and wait approach was considered for treatment of the patient s low grade b - cell lymphoproliferative disease , the frequency and severity of angioedema was concerning . our preference was to treat his lymphoproliferative disease in order to prevent future angioedema , as opposed to acute management with ecallantide or prophylactic management with c1-esterase inhibitor concentrate indefinitely . ecallantide is not a disease modifying drug ; therefore , the decision was made to start rituximab 375 mg / m weekly for 4 weeks . since his first dose of rituximab 9 months ago , he has had no further episodes of angioedema . acquired c1-inh deficiency is characterized by consumption of c1-inh ; however , the pathophysiologic mechanisms are not clearly defined . type i acquired c1-inh deficiency describes patients with lymphoproliferation and excessive c1-inh consumption.4 type ii acquired c1-inh deficiency describes anti - c1-inh autoantibody production in the setting of monoclonal gammopathy of unknown significance ( mgus ) or b - cell lymphoid hemopathy.2 consumption of c1-inh leads to hyperactivation of the classical complement pathway and fibrinolytic system with resultant low plasma c4 , low plasma c1q , normal plasma c3 , and elevated bradykinin levels.5,6 clinical manifestations include intermittent angioedema of the subcutaneous tissues in the absence of urticaria and pruritus , and appears to be largely mediated by bradykinin . pharmacologic treatment options for acute attacks include c1-inh concentrate , kallikrein inhibitor concentrate , and b2 bradykinin receptor blockade agents . chemotherapy for mgus or b - cell lymphoproliferative disease is not always indicated ; however , patients with concomitant acquired ci - inh deficiency may benefit from such treatment . in a patient with acquired angioedema and b - cell lymphoma , levi et al described cessation of symptoms after treatment with rituximab.3 a review by branellec et al describes outcomes of rituximab therapy in seven patients with acquired ci - inh deficiency , five of which had an underlying b - cell lymphoproliferative disease or mgus.2 two of these five patients had no subsequent attacks after treatment . certainly the prevalence of acquired angioedema in the setting of low grade b - cell lymphoproliferative disease is low , with only a few case reports published in literature . trials in which such patients are randomized to a rituximab group or alternative therapy group are impractical . therefore , case reports are paramount so that practitioners can make management decisions based on a collective experience . in the above patient with acquired c1-inh deficiency in the setting of low grade b - cell lymphoma , rituximab eradicated symptoms of life threatening angioedema . therefore , rituximab warrants consideration for patients with recurrent angioedema in the setting of acquired c1-inh deficiency and low grade b - cell lymphoproliferative disease .

acquired angioedema is often associated with significant morbidity . an underlying lymphatic malignancy , autoimmune disorder , adenocarcinoma , or other malignancy may be present . screening for these disorders should occur in all patients with acquired angioedema as treatment may result in resolution of angioedema .

herpes simplex virus ( hsv ) is the most common cause of infectious esophagitis after candidiasis . herpes simplex esophagitis ( hse ) in immunocompromised hosts is well documented in the literature , particularly among those diagnosed with human immunodeficiency virus ( hiv ) infection . however , rare cases have been reported in immunocompetent individuals . in a search of medline until october 2012 we present the first case of hse in a healthy 36-year - old female at 27 weeks gestation who recovered without antiviral therapy . a healthy 36-year - old ( gravida 3 , para 3 ) female presented to the infectious diseases department at 27 weeks gestation with a six day history of fever , epigastric pain and dysphagia . physical examination revealed gingivostomatitis , ulcerations and erythema of the gingiva , buccal mucosa and tongue . upper endoscopy revealed several white patches and exudates throughout the esophagus with an edematous friable mucosa suggestive of severe candidal esophagitis . except for mild gastric mucosal erythema , histological examination of the esophageal biopsies revealed multinucleated giant cells with intranuclear inclusion bodies , typical for hse ( figure 1 ) . her symptoms resolved completely within five days following the administration of intravenous hydration and a high dose proton - pump inhibitor . she delivered a healthy daughter that weighed 3100 g , whose apgar scores were 9 ( one minute ) and 10 ( five minutes ) . after 14 months of follow - up , the patient and her baby remained in good health . histologic section of an esophageal biopsy obtained from a 36-year - old pregnant female showing multinucleated giant cells with intranuclear inclusion bodies , typical for herpes simplex esophagitis ( hse ) . it has been initially described as an incidental observation during post - mortem examination with an incidence of 1.8% . hse is an opportunistic infection in immunosuppressed patients with hiv , in cases of underlying malignancies , organ transplantations , inflammatory bowel diseases , and in patients who are prescribed corticosteroids , other immunosuppressive therapy , and radiation therapy . the reasons for not considering this diagnosis in healthy adults may be due to its spontaneous remission and the inability to perform an esophagoscopy due to the presence of severe dysphagia . hse may represent the reactivation of a latent infection , however it is more often due to a primary infection , with local spread of the virus from an orolabial or pharyngeal focus . prior exposure to a family member with possible hsv lesions has been reported in about 20% of the cases . trauma resulting from gastroesophageal reflux , esophageal instrumentation , nasogastric drainage or ingestion of caustics may predispose an immunocompetent individual to hse.10- 12 our patient had no history suggestive of gastroesophageal reflux or other predisposing factors . the most common mode of transmission of an hsv infection is via direct contact of the fetus with infected vaginal secretions during delivery . hse usually affects young males and is typically manifested by the acute onset of odynophagia , dysphagia , retrosternal pain , and fever . gingivostomatitis with bilateral oropharyngeal lesions , ulcerations and erythema of the gingiva , buccal mucosa and tongue , lymphadenopathy and fever are usual manifestations of primary infection , while unilateral oropharyngeal lesions and cold sores suggest secondary infection . the disease predominantly affects the distal or mid - esophagus , but can affect the entire esophagus , and even the stomach . the gross appearance may vary depending on the timing of endoscopy . in the early stage , vesicles are seen , which then slough to form discrete , circumscribed ulcers with raised edges ; the mucosa is friable.9 , 16 these lesions may be punched - out and volcano - like in appearance , or may coalesce to exhibit a cobblestone or shaggy ulcerative appearance . exudate is present in the majority of cases16 and mucosal necrosis may be seen later . specimens that have been obtained from the base of the ulcer are frequently devoid of epithelial cells . the most effective diagnostic method for hse is histology . the characteristic histologic appearance is the presence of multinucleated giant cells with eosinophilic intranuclear inclusions , called cowdry type a intranuclear inclusions and nuclear chromatin with a groundglass appearance . pcr techniques for the detection of viral genome have demonstrated very high sensitivity and specificity in the diagnosis of a herpetic infection , even greater than those of the viral culture . serologic tests have limited value , because of the high prevalence of hsv antibodies in individuals , but may be useful in diagnosing primary infections . in contrast to immunocompromised patients , hse is usually a self - limited disease that has a favorable outcome in immunocompetent individuals . in these cases , hse resolves spontaneously within 1 to 2 weeks , and exceptionally may be complicated by gastrointestinal bleeding or esophageal perforation.18- 20 acyclovir is a well established treatment for hse in the immunocompromised host but its efficacy in immunocompetent adults and adolescents is controversial . therapy with acyclovir in immunocompetent patients may shorten the duration of symptoms although a controlled study has not been performed and may not be feasible because of the rarity of the disease . acyclovir is safe in pregnant women and infants , with no congenital malformations or infant toxicity.23 , 24 pregnant women are relatively immunocompromised ; thus antiviral therapy may be indicated in cases of hsv esophagitis . our patient recovered promptly without antiviral therapy . in conclusion , hse in pregnant woman hse should be suspected even in healthy patients who present with the triad of symptoms of odynophagia , fever and retrosternal pain . an upper endoscopy with biopsy for histopathology and viral culture should be performed to confirm the diagnosis .

herpes simplex esophagitis ( hse ) has rarely been reported in immunocompetent individuals . in a search of medline until october 2012 , we found only one case of hse in a pregnant female . we present the first case of hse in a healthy 36-year - old female at 27 weeks gestation who recovered without antiviral therapy .

a 67-year - old male patient presented to the hospital with a right lower lobe nodule and a paraesophageal cystic mass , which were discovered by regular chest computed tomography ( ct ) . the 4-cm paraesophageal cystic mass was located on the left side of the esophagus ( fig . the right lower lobe nodule was proven to be adenocarcinoma by a frozen biopsy , and a right lower lobectomy and mediastinal lymph node dissection were carried out . after further dissection through the mediastinum , the mediastinal mass was located on the left side of the esophagus . however , rather than originating from the esophagus , it seemed that the mass was connected to the thoracic duct . on the basis of this finding , we suspected that the mass was a lymphangioma originating from the thoracic duct . to prevent chylothorax , there was no sign of chylothorax , and the chest tube was removed on the 4th postoperative day . the mass was confirmed to be a lymphangioma by a pathologic examination and by immunohistochemistry , on which the mass was positive for cd31 , cd34 , factor viii , and d2 - 40 . a 48-year - old female patient presented to the hospital for dysphagia that had begun 1 month prior . chest ct showed a 5.5-cm mediastinal cystic mass , which was located posterior to the lower esophagus ( fig . endoscopic ultrasonography indicated that it may be a bronchogenic tumor or an esophageal duplication cyst . an operation was performed by right - side vats with the patient in a semi - prone position . the mass seemed to originate from the thoracic duct ; therefore , the thoracic duct was clipped , and the mass was excised ( fig . 3 ) . when the mass was dissected on a table , a milky fluid was found ( fig . the patient was discharged from the hospital on the 5th postoperative day . in the pathologic examination , , the mass was demonstrated to be positive for cd31 and negative for d2 - 40 ( figs . a 67-year - old male patient presented to the hospital with a right lower lobe nodule and a paraesophageal cystic mass , which were discovered by regular chest computed tomography ( ct ) . the 4-cm paraesophageal cystic mass was located on the left side of the esophagus ( fig . the right lower lobe nodule was proven to be adenocarcinoma by a frozen biopsy , and a right lower lobectomy and mediastinal lymph node dissection were carried out . after further dissection through the mediastinum , the mediastinal mass was located on the left side of the esophagus . however , rather than originating from the esophagus , it seemed that the mass was connected to the thoracic duct . on the basis of this finding , we suspected that the mass was a lymphangioma originating from the thoracic duct . to prevent chylothorax , there was no sign of chylothorax , and the chest tube was removed on the 4th postoperative day . the mass was confirmed to be a lymphangioma by a pathologic examination and by immunohistochemistry , on which the mass was positive for cd31 , cd34 , factor viii , and d2 - 40 . a 48-year - old female patient presented to the hospital for dysphagia that had begun 1 month prior . chest ct showed a 5.5-cm mediastinal cystic mass , which was located posterior to the lower esophagus ( fig . endoscopic ultrasonography indicated that it may be a bronchogenic tumor or an esophageal duplication cyst . an operation was performed by right - side vats with the patient in a semi - prone position . the mass seemed to originate from the thoracic duct ; therefore , the thoracic duct was clipped , and the mass was excised ( fig . 3 ) . when the mass was dissected on a table , a milky fluid was found ( fig . the patient was discharged from the hospital on the 5th postoperative day . in the pathologic examination , , the mass was demonstrated to be positive for cd31 and negative for d2 - 40 ( figs . the thoracic duct originates in the abdomen at the level of the diaphragm , penetrates through the posterior and superior mediastinum , and exits the thorax through the thoracic aperture , which is usually posterior to the left innominate vein and left of the common carotid artery . the cysts are often symptomatic because of the pressure they exert on the adjacent structures . furthermore , acute respiratory insufficiency after the ingestion of a fatty meal is also observed in some patients . in case 2 , the patient experienced dysphagia , which may caused by the cystic mass . bronchogenic and esophageal duplication cysts account for a majority of the mediastinal cysts , and thoracic duct cysts are rarely included in the differential diagnosis . magnetic resonance imaging is not routinely required for the management of mediastinal cysts but may be able to demonstrate the communication of the cyst with the thoracic duct or the cisterna chyli . the high signal intensity of the t2-weighted images is attributed to the high lipid and protein content in the cyst . surgical resection of mediastinal cysts , including thoracic duct cysts , is indicated once they are discovered . these cysts are removed to relieve the symptoms and to prevent further growth , infection , and possible malignant transformation . cd-31 and d2 - 40 are the known markers of lymphangioma . in both cases , the mass was positive for cd-31 , which is also called pecam-1 and is an adhesion molecule that is expressed in the monocytes , granulocytes , and t - cell subsets . d2 - 40 , a monoclonal antibody , which binds lymphatic epithelial cells , stained positive only in the mass from case 1 . surgical treatment for lymphangioma consists of the removal of the cyst and the ligation of all the lymphatics connected to it . kwak and bae reported that they ligated the thoracic duct and that the chylothorax was not observed thereafter . in the case of a mediastinal cystic mass that is adjacent to the esophagus , as such , when removing the mass , the operator should note its relationship with the thoracic duct . if the mass seems to originate from the thoracic duct , it should be ligated to prevent chylothorax . here , we report two cases of thoracic duct lymphangioma . initially , these cases were thought to be esophageal duplication cysts ; however , they were found to be thoracic duct lymphangiomas . to prevent postoperative chylothorax , it is important to suspect thoracic duct lymphangioma if a cystic mass is present along the course of the thoracic duct .

a 67-year - old male patient came to the hospital due to lung cancer and mediastinal cystic mass which was suspected to be esophageal duplication cyst . video - assisted thoracoscopic surgery ( vats ) was performed and intra - operative finding suggested it as a cystic mass along the thoracic duct . thoracic duct was ligated and the cyst was completely resected . a 48-year - old female patient visited the hospital for dysphagia . mediastinal cystic mass was suspected to be an esophageal duplication cyst . intraoperative finding suggest a thoracic duct lymphangioma . after thoracic duct ligation , the mass was completely resected with vats . postoperative chylothorax did not develop in both cases .

anisakiasis , a well - known disease occurring in humans , is caused by ingestion of anisakid larvae in dishes with raw marine fish and squids . post - consumption , the third stage larvae can penetrate the stomach and intestinal walls , leading to such symptoms as stomach pain , fever , diarrhea , vomiting , or even , in cases of multiple infection , mental stupor . also , recently anisakid larvae have been implicated as a possible causative food allergen in adult patients presenting with urticaria , angioedema , anaphylaxis , and other maladies . most cases of human anisakiasis have been traced to the genus anisakis , and the genus pseudoterranova is the second most common anisakid found in humans . the republic of korea has a longstanding tradition of raw fish consumption , and so it is not surprising that numerous korean patients have been diagnosed with anisakiasis , most of those cases being anisakis simplex . as for pseudoterranova , however , there have been only 12 confirmed cases in korea . in this paper , we report a case of human infection by p. decipiens larva , discovered in a routine physical examination . in june 2011 , an anisakid larva was discovered in a 61-year - old woman during the course of endoscopy executed at dankook university hospital ( fig . the laboratory findings were within the normal limits , except for a mild elevation of the eosinophil count ( 5.3% ) . she was living in cheonan city , chungcheongnam - do , and on an evening about 13 hr prior to her endoscopy , ate a rockfish ( sebastes sp . ) . the larva was immediately fixed in 10% formalin , and transferred to the department of parasitology , college of medicine , dankook university . it was cleaned in glycerin - alcohol , mounted with glycerin - jelly and observed under a light microscope . the esophagus was 2.4 mm in length , and was composed of long muscular and short ventricular parts . the intestinal cecum , confirmed by section of the anterior part of the worm , was 1.1 mm in length , stretching to the middle level of the ventriculus . the presence of an intestinal cecum without ventricular appendage was easily observed in the transverse section of the worm ( fig . all of these findings readily identified the specimen as a third stage larva of pseudoterranova decipiens . as already noted , this case of p. decipiens infection is the 13th discovered in korea . since anisakis type i larvae have been implicated in most cases of human anisakiasis in korea , other anisakid larvae such as pseudoterranova and contracaecum frequently have been overlooked . in fact , human infections with pseudoterranova spp . is far from rare in northern japan , amounting to 769 cases by the mid-1990s and 7 cases of p. decipiens have occurred in chile . recently , p. decipiens was classified , by the molecular method , into 5 sibling species , i.e. , p. decipiens sensu stricto , p. azarasi , p. cattani , p. krabbei , and p. bulbosa . unfortunately , whereas multiple infections of anisakid larvae are possible , most patients have been singly infected , which fact has hindered application of the molecular technique , as in the present case . although molecular identification often is unavailable , comprehensive and detailed understanding of anisakid fauna can be attained through morphology - based genus - level identification . the most important structure of anisakid larvae in species identification is their esophagointestinal morphologies with the corresponding measurements . larvae have a simple and straight arranged digestive tract , i.e. , muscular esophagus , ventriculus , and intestine . on the other hand , in pseudoterranova spp . larvae , the presence of an intestinal cecum without ventricular appendage is the key to distinguish from contracecum spp . the present worm could be diagnosed as a third stage larva in consideration of the fact that the cecum reached beyond the one - third level of the ventriculus anteriorly , along with the presence of a mucron at the posterior end . curiously , the patient whose case prompted this study did not complain of any symptoms arising from p. had acute or subacute abdominal pain , and korean patients have complained of epigastic pain . however , p. decipiens larvae , as seen in a chilean case , can be expelled from the mouth in the absence of gastric symptoms . the sensation of a foreign body between the teeth has been the chief complaint of some infected patients . finally , it has been suggested that p. decipiens larvae are less invasive than a. simplex , though it should be noted that the varied symptoms of p. decipiens may result from different responses of individual hosts or different pathogenicity of pseudoterranova spp . although pseudoterranova spp . larvae exist in very small numbers compared with other anisakis spp . , they have been traced to more than 30 marine fish species such as cod , pollock , halibut , and flatfish worldwide . for example , the larvae recovered from the yellow corvina ( pseudosciaena manchurica ) , have been anisakis simplex ( 80.4% ) , contracecum spp . nonetheless , total 13 cases of p. decipiens infection , including the present case have been diagnosed in korea . this reflects the fact that p. decipiens larvae must be distributed in fish hosts caught in korea . certainly in korea , where raw seafood , as already noted , is a popular feature of the cuishine , human infection resulting from ingestion of p. decipiens larvae , which reside primarily in fish muscle , could be discovered much more . infection , its distribution and epidemiology , along with molecular examinations of pseudoterranova larva , are required .

pseudoterranova decipiens larva is a rare cause of anisakiasis . indeed , prior to the present study , there had been only 12 reports of larval p. decipiens infection in the republic of korea . in june 2011 , an anisakid larva , 32.1 mm in length and 0.88 mm in width , and finally identified as the third stage larva of p. decipiens owing to the presence of an intestinal cecum but lacking ventricular appendage , was discovered in a 61-year - old woman during the course of endoscopy executed as a part of routine physical examinations . the patient had eaten raw a rockfish 13 hr prior to the endoscopy , but showed no symptoms of anisakiasis . this paper is the 13th report of p. decipiens infection in korea .

adequate treatment planning is one of the most important factors in successful conventional implant prosthesis , requiring clear communication between the prosthetic dentist and surgeon . it becomes more critical when the patient has unfavorable conditions such as a severely resorbed alveolar ridge . treatment planning must be based on a thorough dental diagnosis and a prognosis assessment for the remaining dentition . improper planning may result in a range of prosthetic complications and a poor prognosis due to unfavorable mechanical stress and poor oral hygiene . partial edentulism can be restored with implants by several methods.1 in considering implant placement , the numbers and position of implants should always contribute to the convenience and longevity of a definitive prosthesis fulfilling esthetic and functional demands . implants in partial edentulous patients involve either fixed type implant prosthesis or removable type implant prosthesis . there are several reports that removable partial dentures with posteriorly - placed implants showed a favorable outcome and counted as an adequate treatment option.2,3 this clinical report concerns the rescuing procedure for inadequately planned prosthodontic case for a partially edentulous patient with severely resorbed alveolar ridge . predetermined impractical fixed type prosthesis to restore posterior teeth was properly re - evaluated and restored with a removable partial denture with milled bars and magnetic attachments . a 26-year - old male patient presented to the department of prosthodontics at yonsei university dental hospital to restore the posterior edentulous area . radiographic examination showed that implants were placed in the area of teeth number # 17 , 16 , 22 , 25 and 27 and clinical examination revealed 3 degree of hypermobility on teeth number # 15 and # 24 ( fig . 1 ) . teeth # 15 and 24 were diagnosed as hopeless and were extracted . impressions were made with alginate and casts were poured and mounted in centric relation for prosthodontic evaluation . a provisional denture was fabricated on the articulator and placed in the patient 's mouth . an impression was made with implant level impression coping and casts were mounted for prosthetic treatment planning . it was determined that implant supported fixed partial denture was not indicated due to severe bone resorption resulting in a poor crown - to - root ratio and buccal placement of implants . so , the treatment option of removable partial denture was presented to the patient which involved fabrication of a milled bar with a magnetic attachment . a customized abutment and a crown for tooth number # 22 were fabricated on the articulator and delivered to the patient . the milled bar was fabricated accounting available interocclusal space , as measured from the vinyl index on mounted casts of provisional denture . a two - degree tapered milled bar wax pattern was fabricated on the master cast and magnet keepers were embedded in the pattern , which was then invested and casted ( figs the fit of the milled bar and framework were checked in patient 's mouth , and the fit between them was adjusted with a tungsten carbide bur ( bredent medical , senden , germany ) , and the maxillomandibular relationship was recorded for denture fabrication using an occlusal rim . the prosthesis was remounted on the articulator after polymerization and the occlusion was adjusted on the articulator . the prosthesis was inserted ; phonetics , esthetics , function , and comfort were clinically verified . the magnet was embedded in the denture with gc pattern resin at 2 weeks after initial placement of the final prosthesis . postoperative instructions were given to the patient , and no complications have arisen since insertion of the prosthesis five years ago . as number of implants and their position depend on prosthesis type , making presurgical prosthetic treatment planning critical for the success of the prosthesis . in our case , implant surgery had been performed without proper evaluation of remaining teeth or adequate prosthodontic treatment planning . as a result , two hopeless teeth had to be extracted at six months after initial implant surgery . because of additional extraction followed implant surgery , supplementary implants were necessary for the fixed - type prosthesis especially due to poor crown to root ratio . however , the patient rejected additional implants placement but accepted a removable prosthesis to reduce treatment time and cost . removable prosthesis is more advantageous as restore prosthesis with a poor crown - to - root ratio caused by severe resorption of the alveolar ridge . though guidelines for the crown - to - root ratio in natural teeth are not applicable to implants4 , the ratio is still a prognostic indicator . when treating partial edentulism with implants , an implant - supported , fixed - type prosthesis is usually the first option . it is reported that implant supported removable partial denture is suitable and has advantages to restore kennedy class i posterior edentulous patients.5 those advantages include enhancing retentive or supportive elements of removable partial dentures.6 - 7 strategically - placed dental implants in conjunction with the remaining natural teeth can also establish a favorable removable partial denture design by significantly reducing the effect of the reciprocal arm and improving the fulcrum line position.7 in this case two milled bars with magnet attachments connected to upper posterior implants were used to provide enhanced support for the denture . this report illustrates how improper planning may prolong treatment and give rise to complicated treatment procedure with poor prognosis . countermeasures in this case consisted of correcting to an implant supported removable partial denture in conjunction with careful evaluation of the oral condition during treatment . dental treatment should be based on an adequate treatment plan , and procedures must be guided by proper planning achieved by cooperation between implant surgeon and restorative dentist . implant - supported removable - type prosthesis with milled bar and attachment is a viable and useful treatment option in patients with improperly placed implants .

presurgical prosthetic treatment planning is critical for the success of the implant prosthesis . inadequate treatment plan , due to insufficient discussion between prosthodontist , and surgeon , may result in poor prognosis . a 26-year - old male patient was referred for prosthodontic treatment after implant was placed in the area of teeth # 17 , 16 , 22 , 25 and 27 , without adequate discussion nor the treatment planning between oral surgeon and prosthodontist . it was found that the patient had two hopeless teeth , and a severely resorbed alveolar ridge . additional tooth extraction was needed and the type of definitive prosthesis was shifted from fixed type to removable one . proper pre - surgical treatment planning is essential for the good prognosis . implant - supported removable prosthesis on milled bars may be a useful treatment option in patients with incorrect angled placement on severely resorbed alveolar ridge .

an 18-year - old male patient presented with pneumothorax on postoperative day four after the minimally invasive repair of pectus carinatum . the pectus carinatum repair was performed using the sandwich technique , which involves a 33.02-cm press - molded internal bar and a 30.48-cm press- molded external bar ( fig . a small- bore central venous access catheter was inserted to relieve the pneumothorax in a less invasive manner . a chest computed tomography scan indicated the presence of multiple bullae at the apex of the upper lobe of the right lung . on postoperative day seven after the pectus repair , three days after the development of pneumothorax , we decided to remove the bullae through video - assisted thoracoscopic surgery ( vats ) because the lung collapse was persistent . under general anesthesia , using double - lumen endotracheal intubation with the patient in the left lateral decubitus position , a 10-mm thoracoscopic port was placed at the eighth intercostal space of the middle axillary line , below the pectus bar insertion level in order to avoid bar interruption . a 2-cm working incision was made at the fourth intercostal space of the anterior axillary line and a 5-mm working port incision was made at the subscapular tip area of the posterior axillary line for a three - port vats approach . vats exploration using a 30 thoracoscope ( karl storz endoscope ; karl storz , tuttlingen , germany ) revealed minimal hemothorax and multiple apical multiple bullae as indicated by the preoperative computed tomography scan . a finding that we did not anticipate was that the middle lobe of the right lung was entrapped between the chest wall and the pectus bar . adhesion was present around the pectus bar and the entrapped lung , but we did not observe air leakage at that moment ( fig . the entrapped lung was separated from the adhesion and carefully pulled out of the pectus bar . however , a portion was injured , and it had to be resected with endostaplers ( tristapler ; covidien , norwalk , ct , usa ) . an air leak test confirmed that both resected areas of the apex and the entrapped middle lobe were airtight . bio - absorbable felt ( neoveil ; gunze , osaka , japan ) was used to cover the lesions , followed by glue injection on the resected lesions . pectus deformity repair using pectus bars is a standard operation to correct pectus excavatum that has been widely performed since its development in 1998 by donald nuss . pneumothorax is one of the early postoperative complications of this procedure , and is caused by an incidental lung injury or air that was retained during the surgical procedure . in order to prevent this early complication , we routinely insert hemovac catheters into the bilateral pleural cavities in cases of the parallel insertion of two bars , intrapleural procedures , such as suturing for the placement of hinge plates , or in adult cases . however , primary spontaneous pneumothorax , caused by the rupture of subpleural blebs or bullae , may occasionally occur in combination with pectus repair . in such instances , it is difficult to determine the etiology of the pneumothorax and the subsequent treatment strategy . initially , pneumothorax can be conservatively managed by chest tube or catheter insertion , but prolonged air leakage or recurrent pneumothorax must be treated by thoracoscopic wedge resection . lung entrapment by surgical hardware is sharply distinct from usual cases of trapped lung , in which a part of the lung is unexpandable due to pleural inflammation , the development of adhesion after a surgical procedure , such as coronary artery bypass surgery , or pleural diseases including empyema , hemothorax , tuberculosis , and pleuritic . the entrapment of part of the lung can occur in pectus repair using pectus bars , and it may be difficult to recognize without surgical exploration . our vats finding , although it was incidentally discovered during a surgical procedure for pneumothorax , clearly demonstrated that the lung was caught between the pectus bar and the chest wall in our patient . in our case , a sizable portion of the parenchyma of the right middle lobe was caught between the pectus bar and the anterior chest wall , with adhesions formed around it . the cause of pneumothorax was uncertain in this case because no definitively identifiable ruptured bullae were leaking air at the time of surgery . although the air leak points in the entrapped lung were also uncertain , we thought that the damaged lung may have been leaking air , which may have been the cause of the pneumothorax . our surgical approach , therefore , combined resecting all bullae that we encountered and relieving the lung entrapment , which was ultimately accompanied by the removal of some damaged lung tissue . we report a case of spontaneous pneumothorax involving lung entrapment in a pectus bar and multiple bullae in the lung . this case demonstrates that pectus repair may be complicated by lung entrapment in the pectus bar .

we report a case of an entrapped lung after the pectus bar repair of a pectus deformity . the entrapped lung was found incidentally during video - assisted thoracoscopic surgery ( vats ) for pneumothorax . based on vats exploration , multiple bullae seemed to be the cause of the pneumothorax , but the entrapped lung was suspected to have been a cause of the air leakage .

the normal hemoglobin level in the healthy individual is greater than 130 g / l . in the ambulatory patient levels below this are considered to represent anemia and trigger a search for potentially correctable causes , including sources of occult blood loss . however , the hemoglobin level of most hospitalized patients and , in particular , patients in an intensive care unit ( icu ) falls substantially below this normal range . multiple factors are responsible , including acute blood loss , dilution secondary to fluid retention and depressed hematopoiesis . at a certain level of anemia , the surgeon becomes concerned that reduced oxygen - carrying capacity may be detrimental to the patient 's welfare and considers transfusion . oxygen delivery to the tissues ( do2 ) depends upon the concentration of hemoglobin ( hb ) , the percent saturation of that hemoglobin ( sao2 ) , and the cardiac output ( co ) : do2 = hb % sao2 co a reduction in oxygen delivery below a critical level deprives tissues of the oxygen necessary for oxidative metabolism and results in a shift to anaerobic metabolism . because oxygen requirement by tissues may be increased during acute stresses , it is intuitive that maintaining adequate oxygen delivery will result in improved clinical outcome . indeed , the concept that supranormal oxygen delivery was desirable led intensivists to devise strategies to increase oxygen delivery in critical illness to supraphysiologic levels . the benefits of such an approach , however , have not been borne out by randomized controlled trials . in theory , however , hemoglobin is normally almost fully saturated with oxygen , and increasing cardiac output in the face of adequate filling pressures requires the use of inotropic agents . clinical studies , however , demonstrate the fallacy in such an approach ; isovolemic hemodilution evokes reflex increases in cardiac output , with the result that oxygen uptake at the tissue level remains constant . transfusion is in essence a transplant of allogeneic cells , and its risks , although modest , are not negligible . transfusion carries a small risk for transmission of viruses such as hiv and hepatitis , although with application of adequate screening methods this risk is low . transfusion is also known to be immunosuppressive , and transfusion is an independent risk factor for nosocomial infection or for recurrence of malignancy . perhaps most importantly , blood is an increasingly scarce resource that must be used responsibly . if transfusion to supranormal levels is not intrinsically helpful , then what is the minimum level of hemoglobin that can be tolerated by a healthy individual ? the answer is unknown , but studies in human volunteers have demonstrated that isovolemic hemodilution to a hemoglobin of 50 g / l or less does not result in biochemical evidence of anaerobic metabolism . anecdotal reports documenting the course of jehovah 's witness patients who have experienced major bleeding episodes confirm the ability to tolerate hemoglobin levels well below those conventionally accepted by physicians . until quite recently , physicians accepted the maxim that patients should be transfused so that the hemoglobin was greater than 100 g / l , and that transfusion should be given 2 units at a time . a recent canadian initiative was undertaken to define patterns of blood transfusion in critical illness and to determine optimal transfusion strategies in critically ill patients . using a scenario - based questionnaire , it was determined that transfusion triggers vary widely and are strongly influenced by the geographic locale of the individual clinician 's practice . in general , clinicians selected a higher transfusion trigger for patients with underlying cardiac disease or for patients with sepsis in whom supply dependency might be present . substantial variability was documented in a retrospective survey of transfusion practices in four canadian icus . the transfusion requirements in critical care ( tricc ) trial , a multicenter , randomized , controlled trial , was conducted between 1994 and 1997 . a total of 838 patients from 25 centers were randomly assigned to a liberal transfusion strategy ( maintenance of the hemoglobin > 100 g / l ) or a restrictive transfusion strategy ( maintenance of hemoglobin > 70 patients enrolled in the two study groups had comparable baseline demographic features , and compliance to the protocol was excellent . patients in the restrictive arm received half the volume of transfused blood that patients in the liberal arm did . surprisingly , when outcome data were analyzed , patients in the restrictive arm exhibited a strong trend toward improved 30-day survival and a significant improvement in hospital survival . the development of new organ dysfunction in the icu was significantly less in patients randomly assigned to the restrictive arm ( table 1 ) . there was a trend toward decreased 30-day mortality among patients who were treated according to the restrictive transfusion strategy . the significant differences in mortality rates during hospitalization , rates of cardiac complications , and rates of organ dysfunction all favored the restrictive strategy . a review of the adverse events in both groups revealed that the major morbidity in the liberal group was the sequelae of transfusion acute respiratory distress syndrome , congestive heart failure , and volume overload . subsequent review of patients with a cardiac diagnosis showed that , even in this high - risk population , a restrictive transfusion policy resulted in improved clinical outcomes , although the differences were not statistically significant . outcomes following transfusion : the tricc trial mod , multiple organ dysfunction . improved techniques of screening the donor pool for infecting organisms have reduced rates of infection . although hard data are not available , it is likely that leukodepletion has reduced the frequency of symptomatic adverse events associated with transfusion . marik and sibbald showed that transfusion of old blood ( > 12 days ) was associated with worsening oxygen delivery because stored red blood cells lose their deformability and hence their ability to pass through the microvasculature and unload oxygen . the best evidence currently available suggests that transfusion can safely be withheld as long as the hemoglobin remains above 70 such a policy appears not only to be safe but also perhaps even preferable to a more liberal strategy , even in patients with underlying cardiac disease . whether a lower transfusion trigger is superior is unknown . ultimately , however , the decision to transfuse should be based on expectation of individual physiologic benefit rather than on adherence to an arbitrary numeric transfusion trigger .

the decision to transfuse a hospitalized patient must balance the known risks of transfusion with the need to provide adequate tissue oxygenation and the appropriate utilization of blood as a scarce resource . the minimum tolerated hemoglobin level is not well established , and considerable variation exists in intensivists ' transfusion practices . conventional transfusion triggers of 100 g / l have been challenged by reports indicating that aerobic metabolism is supported by hemoglobin levels of 50 g / l or less . evidence from randomized trials also indicates that withholding transfusions may result in improved outcomes . arbitrary numeric hemoglobin triggers , however , can not supercede intervention based on individual physiologic need and clinical circumstances .

glaucoma drainage implants ( gdi ) are a useful modality in the management of intractable glaucoma cases . late exposure of the implant tube through the overlying conjunctiva is a known complication of the surgical procedure . untreated tube exposure seems to be a risk factor for endophthalmitis.1 gedde et al . recommended prophylactic surgical revision with patch graft in all cases of tube exposure.1 we describe the novel use of fibrin sealant for the surgical revision of tube exposure . a 42-year - old male had retinal detachment repair with placement of an episcleral encircling band in right eye . an ahmed glaucoma valve ( agv ; model fp7 , new world medical , rancho cucamonga , la ) was placed to treat his intractable secondary open - angle glaucoma . the plate of the agv was placed above the encircling band in the supero - temporal quadrant and the donor scleral patch graft covering the limbal portion of the tube was sutured with non - absorbable sutures to the underlying sclera . the scleral patch graft overlying the agv tube had retracted posteriorly by about 3 mm . the dashed line outlines the scleral patch graft , ( b ) schematic demonstrating layering of scleral and conjunctival grafts during revision surgery . the arrow heads denote sticking of lateral edges of the conjunctival auto - graft to the edges of the reflected conjunctiva and the underlying episcleral surface , ( c ) slit - lamp photograph at 4-month post - revision surgery shows well - integrated patch grafts we performed revision surgery of the exposed tube under peribulbar anesthesia . the conjunctiva was incised in a parallel fashion on either side of the exposed tube . a lateral flap of the conjunctiva was undermined in the episcleral plane on either side of the exposed tube . a 5 3-mm human donor scleral patch graft was stuck on the episcleral bed over the exposed segment of the tube using fibrin sealant ( tisseel kit ; baxter ag , vienna , austria ) . adequate care was taken to avoid capture of the conjunctiva under the scleral patch graft to reduce the chances of epithelial entrapment and epithelial inclusion cyst formation.2 a 7 4-mm - free conjunctival autograft was obtained from the inferior fornix and was stuck over the scleral patch graft . the lateral edges of the conjunctival graft were stuck to the edges of the conjunctival flap as well as to the episcleral surface beyond the scleral graft using the fibrin sealant [ figure 1b ] . amniotic membrane was fixed with the fibrin sealant to cover the denuded conjunctival surface in the inferior fornix . the grafts were well integrated and iop was in the target range at the 4-month post - revision follow - up visit [ figure 1c ] . tube exposure occurs in patients with drainage devices despite the use of patch grafts that are covered with the patient 's conjunctiva . an inflammatory and/or immune mediated melting of the patch graft and mechanical breakdown of the overlying conjunctiva appears to be a prominent mechanism.3 in our case , the scleral patch graft , though intact , was retracted posteriorly . conjunctival scarring and retraction following agv implantation above the pre - existing episcleral encircling element may be responsible for retraction of the scleral patch graft , and thereby , might have predisposed to exposure of the tube . the surgical repair of exposed tube involves replacing a patch and covering this patch by conjunctiva . a graft of collagenous human tissue is necessary as conjunctiva does not reliably remain closed over immediately underlying synthetic material e.g. silicone as in this case.2 in a study comparing donor sclera , pericardium , and dura as materials for gdi tube coverage , no material was found to be associated with a reduced rate of tube exposure.4 we chose human donor sclera as it is readily available and most affordable . some methods to cover the patch by conjunctiva include conjunctival advancement,3 vascularized flap5 or grafting . our patient had limited mobility of the superior conjunctiva and either conjunctival advancement , or vascularized conjunctival flap was not possible . buccal mucous membrane graft6 and amniotic membrane transplantation7 have been described as conjunctival substitutes to cover exposed gdi tubes . moreover , the suture material may act as a source of inflammation and localized tissue melt . this was reported to be the cause of failure of repaired tube exposure.7 on the other hand , fibrin sealant has been shown to be safe and effective suture substitute for conjunctival and scleral wound closure in glaucoma surgery8 and for placement of a human donor scleral patch graft covering the sub - conjunctival portion of agv tube.910 in addition to saving suturing time , the fibrin sealant did allow sticking of the conjunctival graft to the edges of the conjunctiva on either side as well as to the underlying episcleral surface beyond the extent of the revision scleral patch graft in our case . since our patient was relatively young and had a thick tenon 's capsule , the adhesion was expected to be strong . the intention of this surgical modification was to gain maximum possible blood supply to the grafted conjunctiva . in our opinion , this novel use of the fibrin sealant and the technique used help in better integration of the patch grafts in the revision of an often difficult problem of exposed agv tube .

tube exposure is a known complication of ahmed glaucoma valve ( agv ) implantation . repair of the exposed tube is not easy . a 42-year - old monocular aphakic male had undergone retinal detachment repair with placement of an episcleral - encircling band followed by implantation of agv . he presented to the clinic on routine review with exposure of the shunt tube . the complication was managed by placing scleral and conjunctival grafts over the exposed tube using a fibrin adhesive ( tisseel kit ; baxter ag , vienna , austria ) . the novel use of the fibrin sealant in the repair of agv tube exposure was for better graft - integration .

it has been well accepted that hypercholesterolemia is a major risk factor for coronary heart disease . on the other hand , some previous studies [ 25 ] observed that hypocholesterolemia was associated with a significantly increased risk of lung cancer . to our knowledge , there is only one previous study that investigated the relationship between preoperative total serum cholesterol ( tsc ) and length of survival after nonsmall cell lung cancer ( nsclc ) resection . they reported that preoperative tsc might be an important prognostic factor for overall survival after nsclc resection using the median value for tsc as the cutoff for dividing patients into low and high tsc groups . however , they did not show the number of patients with tsc below normal range ( hypocholesterolemia ) . we believe that hypocholesterolemia group should be defined as patients with tsc below normal range , not median value . therefore , in the present study , we investigated the prognostic significance of preoperative tsc , using the comparison between nsclc patients with hypocholesterolemia and others . the present retrospective study was conducted from 2001 through 2006 and included 262 patients with nsclc who had underwent complete resection , which consisted of either a lobectomy or a pneumonectomy , together with regional lymph node dissection . patients who did not have a preoperative dosage of cholesterol and who had a followup period of less than 5 years were excluded . preoperative tsc was determined as part of routine preoperative examination ; the normal range was 129220 mg / ml ( 3.345.69 mmol / l ) . ( p ) tumor - node - metastasis ( tnm ) staging was recorded in all patients based on the 7th edition of the american joint committee on cancer ( ajcc)/international union against cancer ( uicc ) classification . followup information , including cause of death , was ascertained through a review of clinic notes and direct or family contact . comparisons of categorical data between the two groups were made using fisher 's exact test with yates ' correction . cary , nc , usa ) , and values of p less than 0.05 were accepted as being significant . although the number of patients with hypocholesterolemia was small , there were no significant differences between hypocholesterolemia group and others ( table 1 ) . in addition , all 13 patients with hypocholesterolemia had not received any cholesterol - lowering therapies . the survival curve based on preoperative total serum cholesterol level is shown in figure 1 . the 5-year survival rate was 38.46% among patients with preoperative hypocholesterolemia and 64.26% among others ( p = 0.0289 ) . univariate cox proportional hazard regression analysis revealed that the gender ( male versus female ) , the histological subtype ( adenocarcinoma versus others ) , pt status ( pt1 versus pt2 - 3 ) , pn status ( pn0 versus pn1 - 2 ) , and serum cea level ( normal versus high ) were related to patients ' prognoses ( table 2 ) . however , the prognostic significance of preoperative hypocholesterolemia did not reach statistical significance ( p = 0.0557 ) . besides prognostic analysis on overall survival of preoperative hypocholesterolemia , we compared the 5-year survival rate according to various potentially prognostic variables . as shown in table 3 , the five - year survivor group was characterized by significantly higher proportions of females , adenocarcinomas , pt1 status , pn0 status , and normal serum cea level in comparison with those who did not survive 5 years . among the patients with hypocholesterolemia , the number of 5-year survivors was smaller than that of nonsurvivors ( 5 versus 8) . in addition , sok et al . reported that preoperative tsc is a prognostic factor after nsclc resection . the association between hypocholesterolemia and cancer risk and poor prognosis is probably complex and largely unclear ; however , some possible explanations exist . muldoon et al . reported that hypocholesterolemic men had significantly fewer circulating lymphocytes , fewer total t cells , and fewer cd8 + cells than those with hypercholesterolemia . therefore , hypocholesterolemia might act by impairing the function of the immune system , thereby making defense mechanisms against tumor spread inadequate . further , several pathways that are important in carcinogenesis , such as the sonic hedgehog and akt pathways , are cholesterol sensitive . moreover , signal transducer and activator of transcription-6 ( stat6 ) is a member of the stat family of latent transcription factor , and stat6 knockdown is associated with inhibiting proliferation and enhancing apoptosis . found an inverse relationship of cholesterol biosynthesis and stat6 in lung cancer cell lines . our data indicates a trend toward an association between preoperative hypocholesterolemia and patient survival in nsclc ; however , it did not reach statistical significance by univariate analysis and the comparison between 5-year survivors and the others . one of the most plausible reason for not reaching statistical significance is the small number of patients with hypocholesterolemia ( 4.96% ) . used the median value for tsc as the cutoff for dividing patients into low and high tsc groups . however , we believe that hypocholesterolemia should be defined as patients with tsc below normal range , not median value . since high intake of saturated fat or meat is known to elevate the tsc concentration , hypocholesterolemia might reflect the low nutritional status of patients . all patients in our study are surgical cases ; therefore , it is possible that patients with low nutritional level might be excluded before surgery . we showed a trend toward an association between preoperative hypocholesterolemia and poorer survival in nsclc ; however , it did not reach statistical significance .

the association between hypocholesterolemia and lung cancer risk has been confirmed in some studies . the purpose of the study was to determine whether preoperative hypocholesterolemia ( below normal range ) is a prognostic factor for survival after nonsmall cell lung cancer ( nsclc ) resection . two hundred and sixty - two consecutive cases of resected nsclc with a followup period for more than 5 years were reviewed retrospectively . in our results , there were only 13/262 patients having hypocholesterolemia . a significant association was observed between preoperative hypocholesterolemia and patients ' survival . however , we failed to find the prognostic significance of preoperative hypocholesterolemia by univariate analysis . no statistical differences were also found by the comparison between 5-year survivors and the others . our data indicates a trend toward an association between preoperative hypocholesterolemia and poorer survival in nsclc ; however , it did not reach statistical significance .

we collected data on confirmed human q fever cases in taiwan that occurred during 20042012 from the notifiable infectious diseases statistics system established by the centers for disease control and prevention of taiwan ( taiwan cdc ) , which is an open and public website ( http://nidss.cdc.gov.tw/ch/singledisease.aspx?dc=1&dt=4&disease=0830 ) . the data included the number of confirmed cases , patient sex , age groups ( 5-year groups ) , and the geographic locations of cases ( county and district ) in taiwan every month from 2004 through 2012 . reported cases included suspected cases of q fever reported to the taiwan cdc by clinicians . generally , paired blood specimens ( acute or convalescent phase ) from reported case - patients are collected and sent to the contracted laboratories of the taiwan cdc for laboratory testing of q fever . confirmed cases are reported cases that are confirmed positive for q fever by laboratory tests . q fever was confirmed either by serologic detection of a > 4-fold increase in specific antibodies against c. burnetii phase ii antigen by using an indirect immunofluorescence antibody assay or by a molecular method consisting of positive detection of c. burnetii dna in blood using pcr . we collected husbandry data on goats and cattle from 2004 through 2012 from the open and publicly available data released by the council of agriculture , executive yuan , taiwan ( http://agrstat.coa.gov.tw/sdweb/public/official/officialinformation.aspx ) . the maps of the geographic distributions of q fever cases and animals were created by using supergis desktop software ( supergeo technologies inc . , we identified 879 ( 6.3% ) confirmed cases of q fever among the 13,962 cases reported during 20042012 . the number of confirmed cases increased dramatically starting in 2004 and peaked in 2007 but declined in 2008 and 2011 ( figure 1 , panel a ) . additionally , the annual incidence increased from 0.44 cases per 100,000 population in 2004 to 0.68 in 2007 and decreased from 0.40 in 2008 to 0.15 in 2011 . cases occurred mainly in southern taiwan ( 674 [ 76.7% ] of 879 ) and particularly in the southern ( 17.4% ) and kaohsiung - pingtung ( 59.3% ) regions ( figure 1 , panels a , b ) , and were most prevalent from march through september ( 669 [ 76.1% ] cases ) ( figure 1 , panel c ) . most case - patients were 3069 years old ( 727 [ 82.7% ] ) and male ( 793 [ 90.2% ] ) ( figure 1 , panel d ) . d ) age and sex distributions of patients with confirmed q fever . during 20042012 , cattle and goats were distributed primarily in the southern and kaohsiung - pingtung regions ( figure 2 , panels a , c ) . the trend of human q fever cases was significantly correlated with the number of goats , rather than with the number of cattle ( figure 2 , panels b , d ) . q fever in cattle and goats and comparison with number of human q fever cases , taiwan , 20042012 . a ) average number and distribution of cattle during 20042012 ; b ) comparison of human q fever cases and number of cattle showing no correlation ( p = 0.123 ) . c ) average number and distribution of goats during 20042012 ; d ) comparison of human q fever cases and the number of goats showing a significant correlation ( p = 0.003 ) . the correlation between human q fever , cattle , and goat was analyzed by pearson s correlation . during 20042012 , the average incidence of q fever in taiwan was 0.43 cases per 100,000 population , which was higher than the incidence in the united states ( 0.04 ) ( 10 ) but lower than that in france ( 2.5 ) ( 11 ) . regardless of these differences in incidence , q fever cases increased after q fever became notifiable in the united states ( 5,10 ) and france ( 11 ) . this increase might be attributed to improved recognition and increased reporting of notifiable infectious diseases to the authorities . in taiwan , we have previously illustrated that reported and confirmed q fever cases dramatically increased beginning in 2004 , 3 years before it became notifiable in october 2007 ( 6 ) . however , confirmed cases of q fever decreased in 2008 , even though the number of reported cases remained steady during 20072010 ( figure 1 , panel a ) . accordingly , the changing number of confirmed cases could not be explained by a change in the number of reported cases or by the advent of q fever as a notifiable disease in taiwan . most cases occurred in southern taiwan , particularly in the kaohsiung - pingtung region , despite fluctuations in the yearly number of cases ( figure 1 , panels a , b ) . geographic distribution was correlated with the distribution of cattle and goat husbandry , which was predominant in southern taiwan ( figure 2 , panels a , c ) . a serologic study in the kaohsiung - pingtung region found a high seroprevalence of q fever in animals ( 12 ) . seroprevalence rates in the overall herd and in individual animals were , respectively , 73.6% and 48.3% in goats and 66.7% and 19.5% in cattle . in addition , the 26.3% seroprevalence in persons engaging in veterinary and animal - related work was higher than in the reference population ( 2.7% ) . thus , we suspected that the decrease in the number of human cases might have been associated with animal reservoirs , particularly goats . we illustrated that the increase and decrease in human q fever cases was correlated with variation in the number of goats , rather than cattle ( figure 2 , panel b ) . after the number of goats began to decrease in 2007 , human q fever cases dramatically decreased in 2008 and 2011 . the decrease in the number of goats was possibly associated with 2 episodes of goat pox epidemics in july 2008 and april 2010 ( figure 2 , panel d ) and the culling of 210 and > 20,000 goats in the 2008 and 2010 epidemics , respectively ( 13,14 ) . to control the goat pox epidemics , several measures were enacted , including animal and vehicle movement control within infected areas , cleaning and disinfection of infected farms and equipment , culling of infected animals , and vaccination . although these measures were applied to control goat pox , collateral effects that diminished the spread of c. burnetii from infected goats or a contaminated environment to humans also might have existed . the association between livestock numbers and human q fever might be ecologic because data on individual exposures and on q fever in goats and cattle over time were not available for analysis . in conclusion human cases increased beginning in 2004 and decreased in 2008 and 2011 , which was correlated with the number of goats and possibly was associated with the collateral effects of measures taken to control goat pox in 2008 and 2010 .

in taiwan , q fever cases in humans began increasing in 2004 and peaked in 2007 but dramatically declined in 2008 and 2011 . cases were significantly correlated with the number of goats . the decline might be associated with the collateral effects of measures to control goat pox in 2008 and 2010 .

his family noticed sudden inward deviation of his left eye 9 months ago , and as time passed , this deviation became increasingly noticeable . on first presentation , the angle of esodeviation was 50 prism diopters ( pd ) at distant and near fixation . the patient 's visual acuity and sensory status were not checked , but he demonstrated alternate fixation . no difference in the size of the deviation was found with fixation of the right versus the left eye . no nystagmus was noticed , and there was no family history of strabismus or amblyopia . review of old photographs demonstrated that at 1 year of age , the patient 's corneal light reflexes were centered . he was referred to a pediatric neurologist , but the neurological examination did not reveal any additional pathological findings . a brain mri revealed a 5 cm mass with an enhanced peripheral rim at the midline of the cerebellum as well as hydrocephalus ( fig . three months after neurosurgery the patient 's visual acuity was 20/125 in each eye and the angle of esodeviaton increased up to 60pd at distance and near fixation without any deficit of abduction . eight months after the initiation of occlusion therapy , the patient 's visual acuity had improved to 20/50 in the right eye and 20/40 in the left eye . the angle of esodeviation was reduced to 50pd at distance and near fixation , and the esotropic angle did not show any sign of decrease . one year after neurosurgery , a bilateral 6 mm recession of medial rectus muscles was performed . immediately after the strabismus surgery one month later , the angle of esodeviation became 10pd at distance and 6pd at near fixation . occlusion therapy was stopped due to lack of patient compliance . at the last follow - up examination , 2 years after eye muscle surgery , the patient 's angle of esodeviation remained 8pd at distant and near fixation ( fig . 3 ) , and the corrected visual acuity was 20/40 with a glasses prescription of + 0.75 diopters in both eyes . at this time , ocular motor fusion had not been reestablished following strabismus surgery . in most cases , the onset of acute acquired comitant esotropia occurs during infancy or childhood . although comitancy is often thought to not be associated with neurologic disease,2 brain tumors and other intracranial processes in pediatric patients may in fact present with comitant esotropia.3 - 7 william and hoyt5 described six children with acute onset of comitant esotropia who were found to have tumors of the brain stem or cerebellum . several reports have established that brain tumors such as cerebellar astrocytomas,5 - 7 medulloblastomas,5 pontine gliomas,5 astrocytoma of the corpous callosum ( with hydrocephalus),3 and arnold chiari malformation4,8 were associated with acute acquired comitant esotropia in childhood . liu et al.9 reported 30 children with acquired esotropia resulting from known neurologic insults such as a brain tumor , meningitis , or pseudotumor cerebri . of the 30 children reported , twelve ( 40% ) of them had comitant esodeviation whereas the other 18 ( 60% ) had incomitant esotropia . the authors concluded that comitant esodeviation can be common in children with identifiable neurologic insults . the exact mechanism responsible for acute comitant esotropia in patients with brain tumors is not clear . lennerstrand,10 hoyt and good11 reasoned that comitant strabismus might result from involvement of supranuclear mesencephalic structures , which control vergence eye movement . jampolsky12 and others13,14 have ascribed acquired comitant esotropia to infranuclear insults , such as varying degrees of bilateral sixth nerve paresis . spread of comitance is another suggested mechanism.15 the presenting clinical symptoms of patients with brain tumors are diverse , depending on the tumor 's location and size , and sometimes , the age of the patient . unlike various symptoms such as headache , vomiting , or more specific focal signs , acute comitant esotropia is a very unusual first sign of a brain tumor . nevertheless , some authors have noted acute comitant esotropia as the first sign of a brain tumor.16 - 18 for example , dikici et al.16 reported a 3-year - old boy presenting with acute comitant esotropia as the first sign of cerebellar astrocytoma , and the patient had no other neurologic signs or symptoms except moderate bilateral papilledema . our case also presented with acute comitant esotropia as the first sign of cerebellar tumor , except our patient only had very mild bilateral papilledema . zweifach17 presented the case of a 9-year - old boy with acute comitant esotropia in the absence of any neurologic symptoms or signs when first examined . twenty eight months later , when he developed signs of posterior fossae dysfunction , he was found to have a cerebellar medulloblastoma . musazadeh et al.18 also reported a 3-year - old boy with late onset esotropia without any neurologic signs and symptoms on first presentation . eight months after first presentation , a brain mri was performed to work - up the patient 's intense headache , and imaging and biopsy revealed a cerebellar astrocytoma . it is practically impossible to perform a radiologic examination of all children with isolated , acute comitant esotropia . no single clinical sign can reliably reveal the rare patient with acute onset of comitant esotropia secondary to a brain tumor . lyons et al.19 have emphasized that a high index of clinical suspicion should be maintained , and a neuro - imaging study should be considered in the absence of expected findings associated with acute comitant esotropia such as hypermetropia , fusion potential , atypical features , or neurologic signs . in conclusion , it is important to remember that acute comitant esotropia in the absence of other neurologic signs and symptoms in infancy or childhood may be the first sign of an underlying brain tumor .

we report a case of acute comitant esotropia in a child with a cerebellar tumor . a 3-year - old boy was referred for management of a 9 month history of acute acquired comitant esotropia . on first presentation , the patient 's angle of esodeviation was 50 prism - diopters ( pd ) at distance and near fixation without any lateral incomitance . the cycloplegic refraction revealed + 0.75 diopters in both eyes . very mild bilateral papilledema was found on the fundus examination , but the neurological examination did not reveal any other pathological findings . brain mri showed a 5 cm mass located in the midline of the cerebellum as well as hydrocephalus . the mass was completely excised and histological examination confirmed the diagnosis of pilocytic astrocytoma . despite neurosurgery , the patient 's strabismus remained unresolved . one year after neurosurgery , both medial rectus muscles were surgically recessed by 6 mm , resulting in esotropia of 8pd at distant and near fixation without restoration of bifoveal fusion at follow - up 2 years after the eye muscle surgery . therefore , acute onset comitant esotropia in a child can be the first sign of a cerebellar tumor , even without any other neurological signs and symptoms .

the association between non - ketotic hyperglycemia ( nkg ) and epilepsia partialis continua ( epc ) is rare but well known . we report a patient who presented with nkg and epc who was admitted to the medical intensive care unit ( micu ) in hamad medical corporation in qatar . it is vital to recognize the association between those conditions as correcting the metabolic abnormalities is the main step to treat the seizures . a 52 years old pakistani right handed woman presented to emergency department with polyuria and polydepsia for four days . also she had recurrent abnormal movements in the left hand with progressive involvement of the upper limb that started abruptly three days earlier . the blood sugar was found to be more than 24 mmol / l when tested by glucometer in the emergency department . the vital signs , heart , lung and abdominal examination were normal apart from sinus tachycardia . however , her head was intermittently turning to the left side with abduction and external rotation of the left arm . laboratory results showed that blood sugar of 96 mmol / l ( 1728 mg ) , serum sodium of 123 meq / l , potassium level of l 5.6 meq / l and serum osmolarity of 375 mosm / l . there were negative ketons in blood and urine with bicarbonate level of 24 mmol / l . cerebrospinal fluid analysis showed normal cells , protein and high glucose level ( 22 mmol / l ) . computerized tomography ( ct ) and magnetic resonance imaging and ( mri ) of brain were normal . electroencephalogram ( eeg ) showed frequent epileptiform discharges involving the right frontocentral area which is consistent with epc ( figure 1 ) . correction of metabolic disorder ( nonketotic hyperglycemia ) with proper fluid replacement and insulin intravenous drip resulted in a gradual reduction in the number of seizures until they stopped three days later . seizures may result from any metabolic disorders but are not commonly associated with nonketotic hyperglycemia [ 1 , 3 ] . hyperglycemia may precipitate epc by reducing gamma - aminobutyric acid levels leading to a lower seizure threshold [ 3 , 4 ] . on the other hand , seizures are not usually associated with diabetic ketoacidos as ketosis and intracellular acidosis probably increase the seizure threshold . hyperglycemia may also precipitate epc if there is asymptomatic structural cerebral lesion . however , this is not the case in our patient as both ct and mri brain were normal . epc is defined as a clonic muscular twitching repeated at fairly short intervals in one part of the body for a period of minutes , hours , days or weeks . it is a form of focal status epilepticus manifesting as a continuous focal motor seizures . it is usually associated with a wide range of eeg abnormalities including focal spikes and focal slow waves . many conditions have been reported as a cause of epc including structural abnormalities such as central nervous system tumors , trauma , cerebral infarction , intracerebral hemorrhage , cerebral abscess , and vascular malformation . however , in up to 50% of cases conventional brain imaging may be normal . metabolic abnormalities especially hyperglycemia , hyponatremia , and hepatic encephalopathy are a well known causes of epc . the majority of cases with epc are precipitated by hyperglycemia which occurs before impairment of consciousness ; therefore it is important to recognize this association . this could lead to the diagnosis of previously undiagnosed diabetes mellitus and early reversal of the neurological condition . our patient was treated with insulin , intravenous fluids and a carbamazepine 400 mg daily .

we report a patient with epilepsia partialis continua ( epc ) associated with non ketotic hyperosmolar hyperglycemia . epc is an uncommon presentation of nonketotic hyperglycemia . this case was reported in the medical intensive care unit in hamad medical corporation , doha , qatar .

a 31-year - old female presented with a severely protruding upper sternum combined with depressed lower sternum ( fig . 1a , fig . she did not complain of any cardio - respiratory symptoms , such as dyspnea , palpitation , chest discomfort , or exercise intolerance . her father also had a chest wall deformity with the same features , which had never been treated . on preoperative chest computed tomography scan , the right ventricle was compressed by the sternum and the pulmonary artery was two times as large as the aorta in diameter . we planned a modified ravitch - type operation , instead of a minimally invasive repair using a pectus bar , due to the protruding upper sternum . a vertical mid - sternal skin incision was made . after dissecting under the bilateral pectoralis major muscles , we performed the first horizontal osteotomy in a wedge shape on the most protruding point , the angle of louis . in order to unbend the anteriorly concaved lower sternum the bone segments , harvested from the wedge - shape osteotomy , were minced into small pieces and implanted into the unbent lower osteotomy site . after unbending , the remnant protruding part was ground with a bur grinder . the osteotomized sternum was fixed with multiple vertical wires to prevent horizontal displacement ( fig . external morphology of the chest wall has been stabilized and more satisfactory after a 6-month remodeling period ( fig . 1b , fig . pectus arcuatum , which is also called pouter pigeon breast, is a rare complex chest wall deformity . it involves a wave - like deformity , a mixed form of excavatum and carinatum features , either along a longitudinal or along a transverse axis . this very rare form of chest wall deformity is not frequently dealt with in the literature , particularly not in korea . utilization of the ravitch operation has decreased , while the treatment of pectus excavatum has been substituted with minimally invasive repair using the nuss technique . however , the ravitch operation still has a critical role in the correction of chest wall deformity in cases of pectus carinatum or in asymmetric pectus excavatum . pectus arcuatum is a rare mixed form of chest wall deformity combining pectus carinatum and excavatum . the basic steps in the surgical correction described by ravitch are as follows : bilateral parasternal and subperichondrial resection of the deformed costal cartilages , detachment of the xiphoid process , transverse wedge osteotomy at the upper edge of the sternal depression , and bending of the sternum to straighten its course , securing the corrected position of the sternum . based on these steps , in 1987 , shamberger and welch reported their experience of 14 pectus arcuatum cases among 152 pectus carinatum cases , with satisfactory overall results of 98% . in 2009 , wurtz et al . reported their surgical outcomes of chest wall deformity including 5 pectus arcuatum cases out of a total 205 cases . they proposed their technique as a less invasive and simplified one , characterized by small skin incisions and the discontinuation of both xiphoid process resection and extensive retrosternal dissection . although they did not detail specific results with pectus arcuatum , satisfactory results were acquired in 97.5% of the whole patient group . the ravitch technique has a risk of growth limitation to the thoracic cage due to wide resection of the rib cartilages . however , in an adult with rigid skeletal structure , this conventional chest wall repair technique and its modification can be a useful surgical option , especially for complex chest wall deformity including pectus arcuatum . in this case we achieved a satisfying clinical result with minimal mid - sternal skin incisions , double horizontal osteotomies , subperichondrial chondrectomy , and bone graft reimplantation to the osteotomy defect .

pectus arcuatum is a rare complex chest wall deformity . a 31-year - old female presented with a severely protruding upper sternum combined with a concave lower sternum . we planned a modified ravitch - type operation . through vertical mid - sternal incision , chondrectomies were performed from the second to fifth costal cartilages , saving the perichondrium . horizontal osteotomy was performed in a wedge shape on the most protruding point , and followed by an additional partial osteotomy at the most concaved point . the harvested wedge - shape bone fragments were minced and re - implanted to the latter osteotomy site . the osteotomized sternum was fixed with multiple wirings . with chondrosternoplasty , a complex chest wall deformity can be corrected successfully .

lingonberry ( vaccinium vitis - idaea l. ) , small cranberry ( vaccinium microcarpum ) and bigger cranberry ( vaccinium oxycoccos l. , ) are popular berries in nordic countries and russia , which are used in gravies , dressing and pantry products . furthermore , past few years cranberry / lingonberry products have been increasedly marketed as a natural solution to avoid recurrent urinary infections [ 1 - 4 ] . cranberry / lingonberry juice or cranberry - concentrate tablets , which appear to inhibit the attachment of pathogens to uroepithelium , have been incorporated to the prophylaxis of chronic urinary tract infections in elderly as well as the prophylaxis of recurrent acute uncomplicated urinary tract infection in sexually active women . we herein describe a case of allergy to lingonberry in a 25-year - old woman with a history of mite allergic rhinitis , but no food allergies . she entered a restaurant and then ate meatballs , baked potatoes and lingonberry jam . during the meal , itching wheals developed around her mouth . skin prick testing by prick - prick method with fresh lingonberry revealed mean wheal diameter of 5 mm and mean flare diameter of 20 mm ( figure 1a ) . skin tests either with the foodstuffs involved in the episode or other berries showed no reaction . on her responsibility and against medical advice she immediately noticed more intense symptoms with intense itching on mouth , tongue and throat and wheals over her mouth . skin prick test with a positive response to lingonberry fruit right : immunoblotting of lingonberry extract . autoradiograph of specific ige immunoblot of the control ( lane 1 ) and the patient ( lane 2 ) . we prepared a protein extract of 0.6 mg / ml in phosphate buffered saline using frozen lingonberries in the presence of protease inhibitors . two independent lingonberry allergen extract - coated ige elisas using goat anti - human ige - labelled - peroxidase ( sigma - aldrich , st louis , mo , usa ) showed positive results . ( od 495 ) : 0.343 ( patient ) vs 0.017 ( control ) ( mean values from 2 different assays ) . sds - page was performed with a 12% polyacrylamide gel and a stacking gel of 4% . it was applied 19.2 g of lingonberry extract to every lane and electrophoresis was performed ( mini protean ii system , bio - rad laboratories , richmond , usa ) . then , proteins were electrophoretically transferred from the separating gel to immobilon - p ( pvdf , millipore corporation , billerica , ma , usa ) membranes in a transfer buffer . after blocking with a solution of gelatine 3% for 1 hour , next day , membranes were washed and incubated with goat anti - human ige - labelled - peroxidase as mentioned above . detection was performed with a chemiluminescence substrate ( pierce chemical company , rockford , illinois ) . the western - blot revealed ige in the patient 's serum that bound to some medium / high - molecular - weight protein bands ( figure 1b ) . the nordic countries ' people pick and use about 50 million kg of lingonberries per year , which they use in jams , jellies , preserves , concentrates , and liquors ; the berries are also sold fresh . interest of lingonberry in the possible health benefits is not only due to the inhibition of the attachment of pathogens to uroepithelium , but also to the total content of flavonoids , which is higher than that in the commonly consumed fruits or vegetables . some flavonoids , such as quercetin , in which lingonberries are an excellent source , have potent antioxidant and free - radical scavenging activities observed in vitro . some studies support a protective effect of flavonoid consumption in cardiovascular disease and cancer . because there are many biological activities attributed to the flavonoids further studies in both the laboratory and clinic are running . to the best of our knowledge , it is tempting to speculate that previous exposure by means of lingonberry products was sensitizing . the positive skin test , ige - elisa , western - blot and the symptoms and timing of the episode strongly implicate lingonberry .

past few years cranberry / lingonberry products have been incorporated as healthy products to the us and european market as prophylaxis of recurrent urinary tract infections in young women as well as in chronic infections in elderly which because of there are many biological activities attributed to the that fruit is a very popular additive to the new diets . to the best of our knowledge , this is the first case of allergy to lingonberry . we speculate that previous exposure to lingonberry products could be sensitising . the symptoms , timing of the episode , positive skin test , ige - elisa and western - blot strongly support the role of lingonberry as the causative agent .

tuberculosis ( tb ) is a major global health problem , and can be a multisystemic disease . tuberculosis of the ribs usually presents with rib destruction and soft tissue mass of the chest wall , which is called tubercular abscess of the chest wall ( tacw ) or cold abscess of the chest wall . the isolated involvement of caries rib without caries spine has rarely been described in previous literature . cases of parenchymal tuberculosis and tubercular lymphadenitis with simultaneous caries rib and tacw have not been reported so far to the best of our knowledge . an 18-year - old female student referred to jss hospital , city of mysore , state of karnataka , south india with chief complaints of cough with white mucoid sputum since two months , swelling over the back on the left side since one and a half months , and weight loss since one month . there was no history of fever , breathlessness , chest pain or hemoptysis and no history of contact with a case of tuberculosis . on examination , the patient was moderately built and nourished with weight of 46 kg , height of 153 cm and body mass index ( bmi ) of 19.5 , pulse rate of 110/min , blood pressure of 110/80 mmhg . she had right posterior cervical lymphadenopathy with a node , which was single , non tender , mobile , firm in consistency , and measuring 32 cm . local examination revealed a swelling in the left side of the chest on the posterior aspect in infrascapular region ( figure 1 ) . the swelling , measuring about 1010 cm in size , was non tender , soft in consistency with no local rise of temperature . the swelling was also fluctuant and irreducible , had no cough impulse , and was situated in the subcutaneous plane . respiratory system examination revealed dullness in the left infra - axillary area with reduced intensity of breath sounds . a swelling in left infrascapular region the patient s hemoglobin was 9.5 gm% , and the smear of her peripheral blood showed normocytic normochromic anemia . plain chest radiograph showed non - homogenous opacity in the left lower zone with blunting of left costo - phrenic angle ( figure 2 ) . it also showed asymmetry of the soft tissue shadow on the left side of the chest with mediastinal lymphadenopathy . initial chest x - ray showing non - homogenous opacity in the left lower zone with blunting of left costo - phrenic angle . computed tomography ( ct ) of the thorax showed features suggestive of pulmonary tuberculosis in the left lower lobe ( figures 3 and 4 ) . the ct also showed destruction and pathological fracture of the left sixth rib with a collection of low density fluid in the left pleural cavity , left side of the chest wall and the region inferior to left breast , which were suggestive of cold abscess . a few paratracheal and retrocaval lymph nodes were also seen . computed tomography of the thorax . fine needle aspiration cytology ( fnac ) from the chest wall swelling showed smears positive for acid - fast bacilli . sputum analysis revealed acid fast bacilli in all three samples . patient was scheduled to receive anti tubercular therapy ( category i ) with four drugs including isoniazid , rifampicin , ethambutol and pyrazinamide on the fifth day after admission . repeat plain radiograph of the chest after two weeks of treatment showed clearance of the shadows and mediastinal lymphadenopathy ( figure 5 ) . tuberculosis is a major global health problem , and any organ system can be involved . tuberculosis of the chest wall accounts for 1 - 5% of all cases of musculoskeletal tb . the disease may also involve lymph nodes including mediastinal , mesenteric or cervical , and visceral lesions . computed tomography of the thorax . it also shows features suggestive of destruction and pathological fracture of the left sixth rib with low density fluid collection in the left pleural cavity , left side of the chest wall and the region inferior to left breast suggestive of cold abscess chest x - ray after two weeks of treatment with antituberclosis drugs . it shows clearance of the shadows and mediastinal lymphadenopathy depending on the predominant clinical or pathologic feature , there are many terminologies such as caries rib or cold abscess , etc for tacw . but all the terms come under the same disease entity i.e , tuberculous abscess of the chest wall with or without rib involvement . it has been postulated that tb of bone results from lymphatic or hematogenous dissemination of bacilli from a source of primary infection in lungs . combined effects of pressure necrosis by granulation tissue and the direct action of invading bacilli result in bone erosion in tb . it has been hypothesized that infection of lymph nodes in the chest wall is a result of pleuritis due to invasion of the tubercle bacilli . cold abscesses of chest wall are extraparenchymal collections consisting of caseous material from the necrosed lymph nodes . these can track through the chest wall to form visible swellings on the surface without redness or tenderness . sternum , costochondral junctions , rib shafts , costovertebral joints and vertebrae can be involved in tuberculous abscesses of the chest wall . involvement of intrathoracic lymph nodes with relatively lesser involvement of lung parenchyma and pleura can also occur . computed tomography is necessary for assessing tuberculous chest wall lesions , as it elucidates the nature and extent of soft tissue collections , intrathoracic adenopathy and bone erosion . our patient , a young immunocompetent lady , had a posterior chest wall tuberculous abscess / cold abscess , which was due to caries rib . fine needle aspiration cytology from the abscess showed smears positive for acid - fast bacilli . our patient also had sputum positive pulmonary tuberculosis and tubercular lymphadenitis of neck and mediastinum . therefore , an optimal treatment strategy is controversial . though anti tubercular therapy ( extended course ) is the cornerstone of the treatment of tuberculous abscess of the chest wall the occurrence of caries rib and cold abscess of the chest wall with concomitant pulmonary tuberculosis and tubercular lymphadenitis of neck and mediastinum has rarely been described in an immunocompetent individual . the rarity of our case lies in the fact that the patient was immunocompetent with cold abscess due to caries rib and rare association of pulmonary tuberculosis and tubercular lymphadenitis of neck and mediastinum . parietal chest wall tb is rare , and tb of the rib still rarer . computed tomography is necessary for assessing tuberculous chest wall lesions . anti tubercular therapy ( extended course ) is the cornerstone of the treatment of tuberculous abscess of the chest wall and surgical treatment also plays a vital role .

musculoskeletal tuberculosis accounts for 1 - 2% of all types of tuberculosis . tubercular abscess of the chest wall accounts for 1 - 5% of all cases of musculoskeletal tuberculosis . herein , we report a case of tubercular abscess of the chest wall . the occurrence of caries rib and cold abscess of the chest wall with concomitant pulmonary tuberculosis , and tubercular lymphadenitis of neck and mediastinum has rarely been described in an immunocompetent individual . the rarity of our case lies in the fact that the patient was immunocompetent with cold abscess due to caries rib , with rare association of pulmonary tuberculosis and tubercular lymphadenitis of neck and mediastinum .

non - use osteoporosis is well known in the literature ; it is caused by a decrease in osteoblastic activity arising from an absence of weight bearing and motion . chemotherapy in patients who have undergone bone tumor surgery causes osteoblast inhibition and consequently a decrease in bone apposition and body mass density [ 16 ] . methotrexate directly locks onto osteoblastic proliferation in the epiphyseal plate , inducing apoptosis and a drop off in type - ii collagen synthesis ; in spongy bone , it appears to act directly on osteoclast cell differentiation ; osteoporotic fracture is a possible complication . the other three drugs used in our therapeutic protocols , are doxorubicin , cisplatin and ifosfamide , and they can also diminish bone apposition ; their effects on bone resistance become more evident with advanced age . osteoporotic fractures are common complications in patients who have undergone chemotherapy [ 9 , 10 ] . a 14-year - old caucasian girl showed up at our department for the onset of a tumefaction located in the posteromedial aspect of the distal right femur . examination showed a mass seven centimeters in apparent size that was painful , hard , mobile on the overlying layers and fixed in the underlying layers . distal femur x - rays indicated an osteolytic lesion with cortical erosion and minimal periosteal reaction ( codman s triangle ) . 1a c preoperative images showing a mass in the medial aspect of the distal femur . d postoperative x - rays showing the prosthesis at the site a c preoperative images showing a mass in the medial aspect of the distal femur . d postoperative x - rays showing the prosthesis at the site mri ( fig . 1b ) showed neoformation with various aspects that was more evident on stir - weighted images and hypointense on t1-weighted images . it extended throughout the whole distal femur eroding medial cortex and periosteum without exceeding the epiphyseal plate ; this condition was confirmed by tc ( fig . bone scans revealed intense local uptake and no bone metastasis ; this condition was also evident in total - body tc . the patient was placed in an oncology ward for neoadjuvant chemotherapy with methotrexate , cisplatin , adriamycin and ifosfamide ( italian sarcoma group protocol ) [ 11 , 12 ] . a tumor resection and reconstruction with global modular replacement system arthroplasty ( gmrs , stryker ) was performed ( fig . postoperative days were normal , and when the wound had healed completely the patient was treated with adjuvant chemotherapy . seven months after the surgery , a nondisplaced asymptomatic neck fracture of the right femur was diagnosed by routine x - ray and ct scans ( fig . mri with contrast clearly showed that it was not a metastasis , so biopsy was considered unhelpful ( fig . 2c ) . a percutaneous fixation with cannulated screws was performed in the period between the last chemotherapy and the next , when immunodeficiency was less important.fig . 2a x - rays showing a nondisplaced fracture ; b ct scan showing correct fragment apposition ; c t1-weighted mri image showing no metastasis on the proximal right femur a x - rays showing a nondisplaced fracture ; b ct scan showing correct fragment apposition ; c t1-weighted mri image showing no metastasis on the proximal right femur postoperative days were normal , and weight - bearing was not allowed for 40 days . x - rays taken 60 days after surgery showed the healing process ( fig . 3 ) . the patient and her parents gave their consent to the publication of the clinical case.fig . 4x - rays showing that the fracture had healed at one year of follow up postoperative check showing that the fracture had been stabilized by cannulated screws x - rays showing that the fracture had healed at one year of follow up neoadjuvant and adjuvant chemotherapy caused a revolution in the treatment of musculoskeletal sarcoma , increasing the percentage of treatment success . the most popular treatment protocol is based on the administration of four chemotherapy drugs : methotrexate , cisplatin , adriamycin , and ifosfamide . above all , methotrexate is known to be related to a drop in bone mass density , a decrease in osteoblastic activity , and a stimulation of osteoclast cell differentiation over the short term , as well as a decrease in peak adolescent bone mass and a facilitation of future osteoporosis over the long term [ 1416 ] ; moreover , methotrexate osteopathy , characterized by osteoporosis and joint pain , has been described . pre- and postsurgery immobilization and a decline in normal activity are other important causes of worsening osteoporosis . osteopenia was first described in long - term survivors of osteosarcoma in 1988 , and remains evident many years after chemotherapy . although osteoporosis is well known in patients undergoing chemotherapy for osteosarcoma , there are no cases of spontaneous fracture in adolescents and preadolescents . in our case , the fracture occurred about 20 days before , and in a healthy young girl this is a sufficient length of time for the fracture to heal . however , in these patients , chemotherapy retards the healing process , so this fracture was probably destined to evolve into a pseudarthrosis . the other problem was to choose the best time for the operation , because chemotherapy causes immunodeficiency , which can increase the risk of infections and wound closure problems . the patient had been administered high - dose ifosfamide , and growth factors were administered to decrease the risk of myelotoxicity , meaning that surgery was possible about one week after chemotherapy without the need to postdate the next cycle . bisphosphonates derive from the union of two phosphonic acids joined to one carbon atom ; particular formulations are able to interfere with osteoclast precursor activation , preventing bone linkage and slowing bone resorption . as it is already done for osteoporosis associated with other pathologies ( like lymphoblastic leukemia ) , their assumed use is rational , but new clinical trials are needed to evaluate a possible decrease in bone growth and retarded prosthesis integration .

non - weight - bearing , pre- and postsurgical immobilization , neoadjuvant and adjuvant chemotherapy are known to act on bone turnover , causing osteoporosis over short and long time periods . treatment of fracture insurgence is very difficult because it really depends on being able to choose the right time ( i.e. , when immunodeficiency is less important ) . we report a case of spontaneous neck femur fracture during adjuvant chemotherapy in a young girl treated with resection and prosthesis reconstruction for distal femur osteosarcoma . possible prevention and the correct approach and surgical timing are emphasized considering immunodeficiency following chemotherapy .

stroke patients have a two - fold higher risk of falling than other patients of the same age or gender1 . in particular , 30% to 50% of the elderly those over 65 years old , experience falls every year2 . falls may result in fractures , brain damage , or other secondary complications and may thus lead to decreased physiological functions and weakened muscle strength3 . falls are affected by various factors , including personal , social , and environmental factors4 . however , these studies were conducted by using different or unstandardized methods , and focused on physical functioning even though fall efficacy is one of the most significant factors of falls8 . self - efficacy is the self - belief and capability to create a purposeful plan and implement it9 . the oep is an evidence - based fall prevention program developed and designed for performance in the home by physical therapists11 . the oep is composed of three domains , namely muscle strengthening , balance training , and walking , which suggests a program method in detail10 . in a previous study , elderly people who performed the oep showed an increase in balance , muscle strength , and a decrease in both fall percentage and fall frequency12 . however , the research was conducted with healthy elderly people and did not estimate activities of daily living ( adls ) and quality of life ( qol ) . for the elderly , falls may restrict daily activities because of long - term pain13 , and strong adls dependency was associated with high fall efficacy14 . therefore the purpose of this study was to investigate the effects of the oep on fall efficacy , adls , and qol , and to suggest guidelines for implementing the oep in elderly stroke patients . the study was conducted from april 2015 to june 2015 , and the participants were recruited at the j city health center . all of the subjects were required to meet the following inclusion criteria : ( a ) diagnosis of stroke by a medical doctor , ( b ) age 65 years and older , ( c ) ability to walk with or without a caregiver , and ( d ) no cognitive dysfunction that would hinder understanding of the instructions . we excluded patients if they ( a ) had other neurological diseases , ( b ) were not able to read and understand the informed consent form , or ( c ) had participated in an exercise program for fall prevention . all the subjects were informed of the purpose and procedure of this study and agreed to participate in this study . the oep was performed for 50 minutes , three times per week for 8 weeks ( 2 group and 22 home programs ) by two trained occupational therapists and four exercise assistants . all of the participants received the oep protocol pamphlet and answered the telephone monitoring questions for checking the frequency of falls and oep performance at the end of each week . the oep starts with head movements , neck movements , back extension , trunk movements , and ankle movements . the muscle strengthening exercises include front knee strengthening , back knee strengthening , side hip strengthening , calf raises , and toe raises . balance training includes knee bends , backwards walk , walk and turn , sideways walk , heel toe stand , heel toe walk , one leg stand , heel walk , toe walk , heel toe walk backwards , sit to stand , and stair walk . adls were measured by using the modified barthel index ( mbi)18 , which assesses the independent performance of basic adls . the eq-5d was a multiple choice questionnaire that measures individual health status according to five domains , namely mobility , self - care , usual activities , pain , and anxiety / depression19 . the mean differences between the pre - intervention and post - intervention values were analyzed by using the wilcoxon signed rank test . the significance level was set at p < 0.05 for all the measurements in this study . the proportion of paretic side was the same both right and left hemisphere , and the disease duration was 7.6 4.0 years . the general characteristics of the subjects are shown in table 1table 1.general characteristics of subjectscharacteristicsn ( % ) gendermale3 ( 37.5)female5 ( 62.5)age ( years)65745 ( 62.5)75842 ( 25.0)85 and above1 ( 12.5)lesion typehemorrhagic3 ( 37.5)infarction5 ( 62.5)paretic sideright4 ( 50.0)left4 ( 50.0)disease duration ( years)7.6 4.0**mean sd . after the oep , the fes score significantly improved from 36.1 11.7 to 50.6 9.0 ( p < 0.05 ) . the mbi showed an improvement from 60.3 21.5 to 68.0 23.4 , and the eq-5d however , both the mbi and eq-5d score did not show a statistically significant improvement ( p < 0.05 ) . the results are shown in detail in table 2table 2.comparison of pre and post - intervention scores of fes , mbi and eq-5dpre - interventionpost - interventionfes36.1 11.750.6 9.0*mbi60.3 21.568.0 23.4eq-5d0.3 0.30.4 0.2fes : fall efficacy scale , mbi : modified barthel index , mean sd , * p < 0.05 . fes : fall efficacy scale , mbi : modified barthel index , mean sd , * p < 0.05 this study aimed to identify the effect of oep on fall efficacy , adls and qol in elderly stroke patients . participants were recruited subjects who had visited j city health center . at the beginning of this study , 12 subjects participated in the oep , of whom 8 completed the study . four elderly participants did not finish the oep because of personal reason : relocation outside the city for a short term , decondition state , or poor motivation . the fes was used to assess fall efficacy , the mbi was used to estimate adls , and eq-5d was used to assess qol . standardized instruments that are often used in stoke patients were used in all of the measurements in this study20,21,22 . similar findings were reported in a previous study for the effects of oep on fall efficacy9 and falls risk23 , 24 . our results can not be compared with those of previous studies because this study was the first to study the influence of the oep on adls and qol with oep . therefore , our results maybe attributable to the fact that chronic stroke patients seldom show improvement and that the intervention program was shorter than previous study . the limitation of this study is that its results could not be generalized to elderly stroke patients because of the small number of participants . in this study we recommend that further studies be conducted with a larger sample size and follow - up tests .

[ purpose ] the purpose of this study was to determine the effects of the otago exercise program on fall efficacy , activities of daily living , and quality of life in elderly stroke patients . [ subjects and methods ] eight subjects performed the otago exercise program three times per week , for 8 weeks . the outcome measures were the fall efficacy scale score for fall efficacy , modified barthel index for activities of daily living , and eq-5d for quality of life . [ results ] in our comparison of the results before and after the intervention , we found that the otago exercise program improved fall efficacy significantly as well as the score for activities of daily living and quality of life , though not significantly . [ conclusion ] we consider that the otago exercise program is an effective method for improving fall efficacy in elderly stroke patients .

patients having anisometropic amblyopia were enrolled in a prospective cohort study , conducted in an urban private - practice setup , from august 1992 to august 2000 . the patients , after a detailed history - taking underwent a meticulous examination as follows : assessment of visual acuity , pupillary reflexes , orthoptic check - up for fixation , alignment and motility , an external and a fundoscopic examination . patients having any other associated ocular , systemic pathology or congenital anomaly , were excluded . baseline characteristics recorded were gender , initial visual acuity , interocular visual acuity difference and the type of refractive error . a standard treatment protocol , as follows , was used for patient management : all patients underwent a cycloplegic refraction . the best refractive error correction , closest to the cycloplegic refraction , accepted by the patient was prescribed . refractions were repeated at every follow - up and the prescriptions changed until the full cycloplegic correction was accepted . the patients , after the initial refractive adaptation period , which varied from one month to three months , were put on effective occlusion , defined as part - time occlusion of the dominant eye for three hours , with active use of the amblyopic eye . occlusion of the dominant eye was done with a ground glass in the spectacle ( or an initial opticlude patch , till they became cooperative ) , for a continuous period of three hours . during this period of occlusion , active use of the amblyopic eye was ensured by giving the patients near visual tasks . they were asked to trace alphabet or picture charts and encouraged to do their study / office work during the time of occlusion . compliance was ensured by asking the patients to submit these charts as proof of homework done , at the time of the next follow - up . the refractions were repeated and the best corrected visual acuity ( bcva ) was recorded . once the full cycloplegic refraction was accepted , the follow - up was done three - monthly . the patients were classified according to their age into two groups : group a : patients less than 12 years of age . criterion for success was defined as the bcva of snellens visual acuity of 20/40 ( 0.5 logmar equivalent ) or more . the treatment was considered as unsuccessful when the bcva was less than snellens 20/40 ( 0.5 logmar equivalent ) , with no further improvement after three consecutive follow - ups at three - monthly intervals . statistical analysis was done using epiinfo statistical program ( version 6 , world health organization , geneva ) . the chi - square test was used to compare the baseline characteristics and the success rates group a consisted of 144 ( 72% ) patients , the average age being 7.77 years ( 2.34 , range 1 to 12 ) and the median being eight . group b comprised 56 ( 28% ) patients , the average age being 19.8 years ( 5.47 , range 12 to 30 ) and the median being 20 . interocular visual acuity differences ranged from less than two lines to more than four lines in both groups . there were 56 ( 28% ) myopes , 104 ( 52% ) hypermetropes and 40 ( 20% ) patients with mixed astigmatism . there were no significant differences in the baseline characteristics between the two groups ( p = 0.07 ) . follow - ups ranged from nine months to 24 months , with an average of 15 months , the rate of improvement and the duration of treatment being similar in both groups . the treatment was successful in 108 ( 75% ) in group a and in 34 ( 60.7% ) in group b. there was no statistically significant difference ( p = 0.07 ) between the two groups . the study shows that patients of anisometropic amblyopia responded to treatment irrespective of age . as far back as in 1982 , sen2 had suggested that every effort should be made to treat patients even after 12 years of age . hence , in this study the patients were classified into two groups namely , those more than and less than 12 years of age . previous amblyopia studies3,4,5 which concluded that the success rate did not vary with the age of starting treatment were conducted in patients in a smaller age group , the mean age being 4.58 to 5.42 years . success of amblyopia treatment in older children up to 15 years of age , with fulltime occlusion is reported.6 all patients in this study have been treated with part - time occlusion with equivalent success . improvement of two or more lines with part - time occlusion is reported in 27% of patients from 10 to less than 18 years of age.1 this study also differs in having a large part ( 28% ) of the sample size , with the highest number of patients ( 56 ) , in an older age group varying from 12 to 30 , with a median of 20 years . a literature survey showed no documentation of a comparison of success rates between patients of anisometropic amblyopia , presenting in the pediatric group ( before 12 ) and in the older , adult group ( after 12 years of age ) . the findings of this study suggest that there may be no cut - off age beyond which a patient of anisometropic amblyopia will not respond to treatment . the limitations of our study were as follows : a ) " compliance " was not monitored . if such patients were considered as an exclusion criterion , the success rate would have been higher . despite these limitations , a success rate of 60.7% was achieved in group b- pubmed search revealed no other documented proof of successful treatment in the largest number ( 56 ) of older and adult patients . these findings are significant , as these 60.7% of older patients who can be successfully treated , may not even get enrolled for treatment due to long - standing false beliefs . it is important that ophthalmologists classify their amblyopic patients and treat patients of anisometropic amblyopia irrespective of age at presentation . the primary health workers , orthoptists , family physicians and pediatricians should also be made aware that the age of the patient need not be a bar for the treatment of anisometropic amblyopia . in conclusion , there is no statistically significant change in the success rate of treatment of anisometropic amblyopia , even in patients older than 12 years of age .

a prospective cohort study of 200 anisometropic amblyopes was conducted . the patients were classified into two groups . group a : patients less than 12 years of age . this consisted of 144 ( 72% ) patients , the average age being 7.77 years ( 2.34 , range 1 to 12 ) . group b : patients more than 12 years of age . this comprised 56 ( 28% ) patients , the average age being 19.8 years ( 5.47 , range 12 to 30 ) . criterion for success was defined as best corrected visual acuity of 20/40 ( 0.5 logmar equivalent ) or better . the chi - square test was used to compare baseline characteristics and success rates . there were no significant differences in the baseline characteristics between the two groups ( p = 0.07 ) . the treatment was successful in 108 ( 75% ) in group a and in 34 ( 60.7% ) in group b ( p = 0.07 ) . there was no statistically significant change in the success rate of treatment of anisometropic amblyopia , even beyond 12 years of age .

physicians submitted $ 9.4 billion in charges for surgical services under medicare in 1985 ( table 1 ) , which was 2.5 times the amount submitted in 1980 ( $ 3.8 billion ) . in 1985 surgery charges comprised : almost one - third of all physician medicare charges , about the same as in 1980 . almost one - sixth of all physician charges for office services , compared with one - eighth in 1980 . nearly one - half of all physician charges for outpatient hospital services , up from less than one - third in 1980 . more than two - fifths of all physician charges for inpatient hospital services , down slightly from 1980 . about one - eighth of all physician charges in other settings , nearly four times the level for 1980 . note that the surgical charges in this article do not include other physician charges associated with surgical services such as assistance at surgery ( $ 545 million in 1985 ) , anesthesia ( $ 1,523 million in 1985 ) , or other medical services and supplies ( an undetermined amount ) . fifteen dominant medical specialties accounted for 94 percent of all surgical charges ( table 2 ) . three specialties ophthalmology , general surgery , and orthopedic surgery accounted for one - half of these charges ( figure 1 ) . the proportion of medicare physician charges accounted for by surgery charges varied by medical specialty ( figure 2 ) . some highlights of these variations are as follows : overall , surgical charges represented about 32 percent of medicare physician charges ( medical and surgical combined ) . ophthalmologists accounted for the largest proportion of total medicare surgical charges , with 18.7 percent ; however , surgical charges accounted for only 64.9 percent of all physician charges generated by that specialty . among the specialties , plastic surgeons had the highest proportion of surgical charges as a percent of total charges , with 87.4 percent ; however , plastic surgeons accounted for only 1.5 percent of all surgical charges . most surgical charges , nearly two - thirds , were derived from inpatient hospital settings ( figure 3 ) . office and outpatient hospital settings each contributed about one - sixth of all surgical charges . a relatively small share , nearly 3 percent , occurred in other settings homes , nursing homes , and ambulatory surgical centers . surgical charges have shifted from inpatient settings to ambulatory settings since 1980 . in that year , nearly 85 percent of surgical charges were derived from inpatient settings , compared with 65 percent in 1985 . although most of the dominant specialties received most of their surgical charges from inpatient hospital services , some notable exceptions did not ( table 3 ) . ophthalmologists generated nearly one - half of their surgical charges in outpatient hospital settings and nearly one - twelfth from ambulatory surgical centers . podiatrists also obtained a significant share of their surgical charges from home and nursing home settings . since 1980 , general surgeons and urologists ' shares of surgical charges have dropped sharply , as ophthalmologists ' shares have increased ( table 2 ) . as can be seen in table 4 and figure 4 , the dominant physician specialties in 1985 varied by place of service : inpatient hospitals : three surgical specialties general , orthopedic , and thoracic accounted for more than one - half of all inpatient hospital surgical charges . office : ophthalmologists , dermatologists , and podiatrists accounted for more than one - half of all office surgical charges . outpatient hospitals : ophthalmologists alone accounted for more than one - half of all outpatient hospital surgical charges . plastic surgeons , gastroenterologists , ophthalmologists , otologists , laryngologists , rhinologists , and internists shifted substantial shares of surgical care from inpatient to ambulatory settings . much smaller shifts to outpatient settings occurred in other surgical specialties general , neurological , orthopedic , thoracic , and urological . data for 1980 in this article were derived from a 5-percent sample of part b carrier bills ( the physician summary record ) , which did not contain information on billings made under : part a by hospitals for physician services ( i.e. , combined billing ) . part b by hospitals on behalf of physicians ( i.e. , on form 1554 ) . neither type of excluded billings for 1980 contained any significant dollar amounts related to surgery . data for 1985 were derived from the part b medicare annual data ( bmad ) procedure file . the bmad procedure file is an annually submitted , 100-percent sample of all procedures processed by part b carriers , including billings formerly made under part a of the medicare program ( i.e. , combined billings ) and under part b for these services of hospital - based physicians whose billings formerly were made by hospitals under the terminated form 1554 procedure .

since 1980 , a number of medicare practice and utilization patterns have changed as a result of payment reform , certification of new types of providers , and changes in technology . the shift in physician surgical charges by specialty and by setting is examined in this article .

nonalcoholic fatty liver disease ( nafl ) is a term applied to the accumulation of fat in the liver in the absence of alcohol consumption . nafl refers to a wide spectrum of liver damage ranging from simple steatosis to non - alcoholic steatohepatitis ( nash ) to advanced fibrosis and finally , cirrhosis . the clinical significance of nafl is derived mostly from its high prevalence in the general population and in its possible progression to liver failure . obesity , type 2 diabetes mellitus and hyperlipidemia are the major risk factors associated with nafl . a bulk of evidence suggests that insulin resistance plays a major role in the pathogenesis of nafl and nash . however with meticulous questioning , more than half would mention fatigue , right upper quadrant pain and occasional discomfort , although the significance of these symptoms is uncertainmost cases initially come to medical attention by the incidental finding of increased liver echogenicity during ultrasonography performed for other reasons . ultrasonography could be used as a non - invasive predictor of liver histology in both moderate and severe steatosis , and advanced fibrosis with acceptable sensitivity and specificity . nevertheless , gradual and sustained weight reduction and exercise are most frequently recommended for overweight nafl patients . weight reduction can not only improve symptoms , but also the risk of progression of nafl to cirrhosis . in the present study we compare the effect of a weight reduction diet on liver ultrasonographic findings in overweight nafl patients . to our knowledge , only one study has thus far researched weight reduction in nafl adults . from july 2003 to december 2005 all patients incidentally found to have fatty liver by ultrasonography who referred to radiology and gastroenterology clinics were included . fasting blood sugar ( fbs ) , and liver transaminase were checked for all patients . waist circumference was measured using a measuring tap placed in a horizontal plane around the abdomen at the level of the iliac creast . patients with body mass index ( bmi ) above 25 and of the ages 16 to 60 were included in the study . patients not consenting to the study or not able to refer for regular follow - up were also excluded . the degree of steatosis was graded by ultrasonography from 0 to 3 according to table 2 . the diet contained 50 percent carbohydrates , 30 percent fat and 20 percent protein given in three meals with a deficit of 500 calories planned to ideally reduce weight by 2 kg per month . patients were followed monthly by a nutritionist . at the end of month three , anthropometric and ultrasonographic measures before and after dieting were compared using student s paired t - test by spss version 14 . the study protocol was approved by the institutional review board and ethics committee of the digestive disease research center ( ddrc ) of tehran university of medical sciences ( tums ) . in this study , 23 patients ( 13 females , 10 males ) were enrolled . the decrease in weight , bmi , waist circumference and hip circumference were statistically significant . the ultrasonographic grade decreased in all patients ; eight had a two grade reduction and 15 patients reduced one grade ( table 4 ) . the decrease in ultrasonographic grade had a significant correlation with decrease in weight and bmi , but not with changes in waist and hip circumferences ( table 5 ) . the amount of decrease in weight and bmi was significantly greater in patients whose ultrasonographic grades improved by two scores when compared to patients who only had a one score improvement . men had a significantly greater weight loss ( p<0.01 ) , but changes in ultrasonographic grade , bmi , waist circumference and hip circumference were not significantly different among the two sexes . the treatment of nafl is controversial and even the necessity of treatment is doubted when there is no indication of liver damage . even in nash , where liver damage is well documented , there is no universally accepted treatment . when treatment is instituted , evaluation of treatment response is usually through transaminase levels or liver histology . in nafl patients , where the transaminase levels are normal to begin with , histology is the only guide to treatment . but liver biopsy is a fairly invasive procedure and is difficult to justify in subjects with normal liver transaminase levels where the prognosis is generally considered to be very good . in such cases another non - invasive method , such as ultrasonography , could be a good substitute . in this study we observed a significant improvement in ultrasonographic features of nafl by a low calorie diet . unfortunately , we did not have histologic confirmation but we believe it is safe to assume that improvement in histology parallels improvement in ultrasonography . ultrasonography has an acceptable sensitivity and specificity in evaluating liver steatosis and provides a non - invasive predictor of liver histology and degree of steatosis in nafl patients . based on the current study , we suggest ultrasonography as a guide to weight loss . we also observed that patients with a higher initial ultrasonographic grade had greater improvement . it can be concluded that more severe cases will respond better to weight loss . in our study , the diet was designed with a modest calorie deficit aiming at a maximum weight loss of 2 kg per month . the gradual weight loss is important as rapid and severe weight loss , starvation , or even total parenteral nutrition , could result in or perpetuate fatty liver disease 3 - 5 . although slightly more rapid weight loss might also be safe , a modest diet would result in better patient compliance . as mentioned above , the necessity of treatment for non - complicated nafl with no indication of liver injury is in doubt . but considering the safety and other obvious benefits of gradual weight loss , we believe it should be offered to all overweight cases of nafl . we conclude that weight loss is effective in improving the ultrasonographinc grade of steatosis and ultrasonography may be used as a non - invasive indicator of improvement of liver steatosis in patients with nafl during weight reduction .

background non - alcoholic fatty liver ( nafl ) includes a spectrum of diseases ranging from simple hepatic steatosis to nonalcoholic steatohepatitis ( nash ) and cirrhosis . nafl is typically seen in association with obesity , diabetes and hypertriglyceridaemia . in order to seek the role of diet therapy in treatment of nafl , we compared the ultrasonographic findings of patients with fatty liver disease before and after standard diet therapy . methods twenty - three overweight or obese subjects with incidental fatty liver discovered during ultrasonography were included . subjects underwent 3 months of diet therapy , and anthropometric data including weight , height , bmi , waist circumference , and hip circumference were measured . ultrasonographic findings were graded from 0 to 3 . changes in ultrasonographic findings and anthropometric data were studied . results after three months of dieting , the ultrasonographic grade of all patients decreased by one or two grades . fifteen patients decreased one grade while 8 others decreased by 2 grades . we observed a significant correlation between the decrease in ultrasonographic grade and the decrease in weight and bmi . conclusion our study indicates that standard diet therapy could be used as an effective treatment for nafl patients .

oligodendroglioma ( odg ) accounts for 1 - 2% of all primary intracranial tumors among children . the majority of odgs are single lesions , located in the cerebral hemispheres , involving subcortical white matter and the overlying cortex . brainstem remains the rarest site of odg occurrence.[36 ] english literature describes six cases of intrinsic brainstem odgs and 10 cases of exophytic brainstem odgs.[16 ] the present report describes a child with brainstem odg who presented with obstructive hydrocephalus , on account of a large pontine mass lesion , infiltrating midbrain , and medulla oblongata . a 13-year - old girl presented with complaints of bifrontal headache , associated with projectile vomiting for 6 months duration . in the last fortnight , she had developed visual obscurations and ataxic gait . she had deviation of angle of mouth to the right side while talking and smiling for 2 months . in the last 5 days , she had developed drooping of both eyelids . on examination , she could count fingers at 2 meter with the right eye , while mere perception of light with the left eye . thus , signs and symptoms were suggestive of bilateral , multiple , asymmetrical cranial nerve palsies with long tract signs consistent with intrinsic brainstem lesion , localized to the pons and midbrain , more towards the left side . computed tomography ( ct ) scan of the head revealed mass lesion in the brainstem , compressing and obscuring the fourth ventricle , leading to obstructive hydrocephalus . after right - sided ventriculo - peritoneal ( vp ) shunt , the patient underwent contrast - enhanced magnetic resonance imaging ( mri ) study , which showed a large pontine mass lesion , protruding from the floor of the fourth ventricle and occluding it . the mass lesion was hypo - intense on t1-weighted scan and iso - intense to hyper - intense on t2 scan . on contrast administration , there was variegated contrast enhancement , with well delineation of cystic regions within the tumor [ figure 1a and b ] . with a clinico - radiological diagnosis of pediatric brainstem glioma , the patient underwent midline sub - occipital craniectomy and near - total excision of tumor . intraoperatively , the median sulcus was split and tumor was entered , which was debulked using cavitron ultrasonic suction aspirator ( cusa ) . the tumor was soft , friable , with intermittent regions of fluid - filled cavities , suggestive of old hemorrhages and necrosis . postoperatively , the patient 's deficits remained static , except for increased right - sided motor weakness . the patient was discharged on eighth postoperative day and was subjected to craniospinal irradiation of 50 gy . histopathology showed moderately cellular neoplasm with a monotonous pattern of uniformly rounded hyperchromatic nuclei separated by delicate branching network of capillaries . overall features were suggestive of oligodendroglioma [ figure 1c ] . at 1 year of follow - up , the child is ambulatory with a karnofsky performance scale ( kps ) of 90% . there is no evidence of recurrence of tumor at the operative site or spinal cord . further , immunohistochemistry showed positive glial fibrillary acidic protein ( gfap ) immunostain in the glial component . ( a ) t1-weighted contrast - enhanced image showing variegated contrast uptake by the tumor . ( c ) photomicrograph showing psammoma body like micro - calcification in tumor ( hematoxylin and eosin , 200 ) . ( d ) photomicrograph showing nuclear ki67 labeling in the tumor cells ( ki67 immunostain , 400 ) . in children , brainstem gliomas account for 10 - 30% of posterior fossa tumors , but only a few of these are odg in nature.[16 ] the incidence of true brainstem odg has not been well studied , likely because of their rarity . in the past era , because of non - availability of high - end imaging tools ( mri scans ) , the true incidence of these brainstem odgs can not be determined . median age for these brainstem odgs is 11.5 years , which is significantly less as compared to patients harboring supratentorial odgs ( median age , 25 - 40 years ) . most brainstem odgs are located in the pons and/or medulla , while one case of odg located in the cervico - medullary region has been reported . invasion of the midbrain was observed only in instances of tumor spread from cerebellar odg . the present case is unique where invasion of the midbrain from pontine tegmentum is described . the well - maintained arachnoid layer over the floor of fourth ventricle indicated the containment of tumor within the brainstem and explained the cranial spread of tumor to invade the midbrain . these odgs have a distinct propensity to infiltrate along white matter tracts of the posterior fossa , which is revealed by the preferential involvement of left - sided dorsal columns of the mid brain . the infiltrative capabilities of odgs and their propensity to disseminate via the cerebrospinal fluid ( csf ) are also now well established . relatively short clinical history for this group of odgs is attributed to limited availability of space in the posterior fossa , combined with dense apposition of vital nuclei and tracts . the degree of cellular atypia , presence of vascular proliferation , necrosis and mitotic figures are better prognostic factors . henceforth , aggressive biology combined with early presentation mark poor prognosis for such patients.[13 ] rare cases with good long - term follow - up and/or survival are on records . brainstem odgs may be considered pathological surprises , as the rarity of these lesions forbids defining clear radiological criteria for them . radical extirpation of the tumor from such an eloquent part of the brain is possible and should be attempted . cranio - spinal irradiation remains the adjuvant treatment of choice and a long - term outcome may not be nihilistic as previously believed .

the authors present the first report of pediatric brainstem oligodendroglioma , infiltrating midbrain , and medulla oblongata . the report details clinical features , radiological findings , and surgical steps . as this entity is exceedingly uncommon , the overall epidemiology , prognosis , and long - term outcome remain far from established .

spine giant cell tumors are rare , and if occurs commonly are located on the sacrum . although they are benign , their prognosis is dominated by their aggressiveness and neuronal structures involvement , recurrence and the risk of malignant transformation . their surgical management should be radical excision whenever possible , accompanied by stabilization of the spine . we report the case of a patient with uncommon spinal location of giant cell tumor . a thirty - nine - year - old patient with no history of trauma or constitutional symptoms who reported upper thoracic back pain lasting for 8 months before seeking medical attention . the pain was dull in nature to begin with , gradually progressive in intensity and not posture related . initially , the pain was responding to conventional non steroidol anti - inflammatory drugs ( nsaids ) . there was a marked increase in pain intensity in last 4 days before admission , and this was accompanied by inability to walk due to lower limb weakness and urinary incontinence . the neurologic examination revealed power grade 3/5 in all lower limb muscle groups , hypesthesia to the level of nipples and locally there was tenderness on d1d2 level . the thoracic spine computed tomography ( ct ) and magnetic resonance imaging ( mri ) showed a tumor involving d1 and d2 vertebral bodies . the lesions are osteolytic eroding the two vertebrae with extension into the spinal canal and enhancing with gadolinium . the patient underwent emergency decompressive laminectomy of d1 and d2 together with stabilization by laminar hooks and rods through c7d3 ( figs 1 and 2 ) . figure 1:preoperative gadolinium - enhanced mri ( a ) and ct ( b ) showing expansile lytic tumor at d1 and d2 and compression of the cord at these levels . figure 2:postoperative sagittal ct showing ( a ) hook and rod stabilization posteriorly , and ( b ) bone graft and plate fixation anteriorly after corporectomy of d1 and d2 . preoperative gadolinium - enhanced mri ( a ) and ct ( b ) showing expansile lytic tumor at d1 and d2 and compression of the cord at these levels . postoperative sagittal ct showing ( a ) hook and rod stabilization posteriorly , and ( b ) bone graft and plate fixation anteriorly after corporectomy of d1 and d2 . given this diagnosis , this was performed by an anterior approach whereby cervicothoracic incision extending from lower cervical ( c6 ) to upper thoracic ( d3 ) was performed . it involved detachment of two heads of sternocleidomastoid muscles on the right side , and medial third of right clavicle was excised . corpectomies of d1d2 carried out , and residual tumor on posterior longitudinal ligament ( pll ) and other surrounding structure was excised . strut graft from the clavicle was placed to bridge the defect and stabilized by anterior plate bridging between c7 and d3 . he fared well postsurgery , at 4 weeks , the neurological deficit improved and the power was grade 4/5 . at 6 weeks , he had fully recovery of sensorimotor functions on lower limbs and trunk . the control ct scan showed a good restoration of the spinal canal dimensions . at 3 months he did not receive radiation therapy as it has been found to be controversial interims of recurrence . giant cell tumors are classified benign , but can be very aggressive locally with a risk of malignant transformation in 10% of cases . their spinal localization is rare outside the sacrum or they are the third most common type of tumor after plasmacytoma and chordoma . they occur between the second and fourth decade of life with a female predominance of up to 70% for spinal locations . these tumors have predilection to vertebral bodies and spares the posterior arch in majority of cases . clinically , the patient experiences constant back pain which increases as tumor expands and destroys the architecture of involved vertebra . the radicular and myelopathic symptoms and signs appear later , as in the case of our patient [ 1 , 2 ] . giant cell tumors are radiologically destructive lesions , lytic and expansile with irregular edges , sometimes condensing with surrounding tissues . a ct scan allows proper assessment of the cortical involvement and extension into the soft tissues . the mri shows the tumor in several planes and the details of soft tissue involvement . the main radiological differential diagnoses are spine aneurysmal bone cyst and osteoblastoma mostly found in the posterior arch . . the histological examination of giant cell tumor can be difficult since giant cells are present in several bone tumors . presence of multiple multinucleated giant cells within a stroma of mononuclear cells , hemorrhagic foci , telangiectasia entholialized and no evidence of ossification points to giant cell tumors . three histological grades of aggressiveness ( i iii ) have been described : mild aggressive ( benign ) , moderate aggressive ( borderline ) and malignant . . the resection should be radical whenever it is possible because of local aggressiveness , frequent recurrence and the risk of malignant transformation [ 14 ] . giant cell tumors are chemoresistant and the place of radiation therapy is controversial . indeed , many authors have questioned its effectiveness , neurological risks and the potential for sarcomatous transformation . it is reserved only for cases where the location or extension makes it impossible surgery . giant cell tumors are often very vascular requiring vertebrobasilar spinal arteriography and preoperative selective embolization to limit bleeding intraoperatively . indeed , dahlin in his study reported 75% of good results at 5 years and above , following local wide resection ( normal clinical findings and no tumor recurrence ) . if tumor involves the posterior arch as well , corpectomy and excision of posterior arch components should be performed ; as in the case of our patient with a posterior arch involvement , the first stage surgery was excision of tumor extension to posterior arch elements together with freeing the cord and stabilization . second - stage surgery was aimed at removal of primary tumor and cord decompression anteriorly , grafting and stabilization with plate . additional treatment was not considered necessary in our patient given the radical nature of our surgery . the prognosis is determined by the risk of recurrence of up to 25% for the spine in a period of 2 years and malignant transformation . our patient 's outcome was favorable with complete recovery of his neurological deficit and the disappearance of pain till the end of first 6-month follow - up . spinal locations of giant cell tumors require radical treatment whenever possible to improve survival and prevent early recurrence . the authors have no personal financial or institutional interest in any of the drugs , materials or devices described in this article .

generally , giant cell tumors are rare and their localization in the spine is even more so . they are locally aggressive leading to spine instability and neurologic deficits . radical excision is highly advocated . a role of radiotherapy in these tumors is controversial . we report the case of a giant cell tumor localized in d1 and d2 on a 39-year - old patient , presented with interscapular back pain , paraparesis grade 3/5 and sphincter dysfunction . thoracic spine computed tomogarphy and magnetic resonance imaging showed a vertebral body tumor in d1 and d2 , compressing the spinal cord at the same level . the patient initially underwent decompressive laminectomy of affected levels and stabilized with laminar hooks and rods . second surgery performed through an anterior approach whereby tumor excision together with corpectomy of d1 and d2 carried out , autograft was placed and plate applied . three weeks postoperatively , the patient 's neurologic deficit recovered fully and back pain subsided .

several studies are available on laparoscopic duhamel pull through for hirschsprung disease.1 2 3 advantages of laparoscopic approach have been already published decreasing the hospital stay and postoperative adhesions . for a clear reason , regarding the laparoscopic approach for duhamel pull through , the longer operative time may be responsible for increasing the infection risk . to our knowledge , we report the first case of postoperative abdominal cellulitis after laparoscopic duhamel pull through . a full - term baby girl presenting at 2 days of age with abdominal distension , tenderness , fever , and severe sepsis was took to the operative room with a preoperative diagnosis of small bowel volvulus . the operative findings were consistent with hirshsprung disease ( hd ) showing a left transverse transition zone . fresh frozen section was done confirming the diagnosis and a stoma diversion was performed just above the transition zone . characteristics clinical findings of mowat wilson syndrome with typical facial features ( square - shaped face , prominent and narrow triangular chin , hypertelorism , large eyes , broad nasal bridge , posteriorly rotated ears , and large uplifted ear lobes with central depression ) were associated and confirmed by genetic analysis . three months later , she was booked for a definitive treatment of hd and a laparoscopic duhamel pull through as previously described.1 preoperative preparation of the colon was not performed . prophylactic antibiotics as per protocol were used ( amoxicillin , metronidazole , and gentamicin ) . to confirm the length of colon involved , a fresh frozen section was done on the left colon just beyond the splenic flexure . this was performed under laparoscopic visualization doing a full - thickness biopsy . during this procedure , a leak of intestinal contents occurred with peritoneal septic inoculation and the biopsy site was then closed . the patient developed a left flank abdominal cellulitis with purulent fluid coming from a left port incision , high fever and clinical signs of sepsis shock requiring an admission in the intensive care unit . appropriate antibiotics were used . despite the treatment , extension of abdominal cellulitis occurred requiring repeated surgical debridement and hyperbaric oxygen ( fig . the infection was controlled and the dress was changed daily in the operative room until complete recovery ( fig . finally , a skin graft on the left flank was done 2 months later for a complete healing . to our knowledge , abdominal necrotizing cellulitis starting from a port incision has never been previously published . three causes may be responsible : intestinal fluid leak during the biopsy , the use of a large peritoneal irrigation that could have spread the fluid through the port incision , and the long operative time . many studies have already reported that colon and rectal surgery can be safely performed without preoperative mechanical bowel preparation.4 thus , we do not really think that it could be responsible for the abdominal cellulitis . because intestinal bacteria was involved in this complication suggesting a peritoneal inoculation during the full - thickness biopsy we think that doing it each time it is possible using an open approach is better . umbilical incision has been reported as a validated option in this indication.5 this could be done also exteriorizing the bowel through the port incision . otherwise , full - thickness biopsy can be done safely using a stapler as previously described by mazziotti and langer.6 furthermore , it seems important to determine preoperatively in this kind of patient what could be the intestinal flora status performing microbiology analysis on fecal samples . indeed , on patient who has been admitted in hospital for many days before and presenting with a stoma diversion ( excluded bowel ) , bacteria flora is certainly modified and can present antibiotic resistance . this can permit to use prophylactic antibiotics during the surgery adapted to the patient 's flora . in our patient , the use of antibiotics adapted to his own intestinal flora during and after the surgery might have limited the infection . furthermore , a large peritoneal irrigation associated with the long operative time is for sure risky and may probably result for a port site 's inoculation . actually , as is shown in case of perforated appendicitis,7 it is probably better not to use a large amount of fluid irrigating the peritoneal cavity as it could be responsible for production of greater bacterial contamination . finally , the operative time is certainly a crucial point . time to conversion to open surgery is still left to the surgeon and remains controversial . if a surgical procedure under laparoscopic approach is quite long but mostly not on going , it is probably time to convert to open approach . in this case , the previous surgery with postoperative adhesion , the deloyer artifice used for pull through which is hard to do to preserve the vascularization of the right colon is certainly responsible for the long operative time . furthermore , this is the part that has to be repeated many times to make the surgery faster and safer . we report here a severe complication following a laparoscopic duhamel pull through . of this case , there are some laparoscopic surgical tricks we can learn : biopsy under direct vision each time it is possible or using a stapler device , the use of moderate peritoneal irrigation and only when it is necessary , the use of adapted prophylactic antibiotics based on preoperative microbiology study on patient with medical history . furthermore , time to conversion to open surgery remains debated and depending of the surgeon and is a part of learning curve .

advantages of laparoscopic approach in hirschsprung disease have been already published decreasing the hospital stay and postoperative adhesions . to our knowledge , we report the first case of postoperative abdominal cellulitis after laparoscopic procedure . a laparoscopic duhamel pull through was done on a 3-month - old child . full - thickness biopsy under laparoscopy was performed with intraperitoneal inoculation . large peritoneal irrigation was used . abdominal necrotizing cellulitis starting from a port site occurred few days after the procedure requiring repeat surgical excision , broad spectrum antibiotics , and hyperbaric oxygen .

neurological complications due to spinal anesthesia are dysesthesia , paresthesia , cauda equina syndrome , and neuropathic joint . however , neurological complications are rare . we report a case of neuropathic joint of knee as a complication of syrinx following postspinal anesthesia . a 33-year - old female came with complaints of pain and instability of her left knee and left foot drop . she had developed weakness of her left lower limb following her cesarean section surgery done under spinal anesthesia . clinically and radiologically , she had features of the neuropathic left knee joint which had occurred as a complication of syrinx following spinal anesthesia . the case is reported for its rarity and to highlight the possible neurological complications of spinal anesthesia . most common anesthesia given in day to day practice is spinal due to its cost - effectiveness , safety , and efficacy , which provides both motor and sensory block with a high success rate . complications such as hypotension , headache , and urinary retention are relatively common [ 1 , 2 ] . however , permanent neurological complications are very rare . neurological complications due to spinal anesthesia are burning sensation over buttocks , dysesthesia , paresthesia , transverse myelitis , anterior spinal artery syndrome , and cauda equina syndrome [ 1 , 3 ] . we report a case of the neuropathic left knee joint which developed because of formation of a syrinx following inadvertent dural penetration during spinal anesthesia [ 2 , 3 ] . a 33-year - old female gravida 2 came with a complaint of weakness of left lower limb and swelling in the left knee . the patient was apparently normal before and underwent lower segment cesarean section under spinal anesthesia in november 2014 . she is unaware of the level of which it was given . immediately , after introduction of the spinal lumbar puncture needle , she experienced severe shooting pain and tingling sensation over left lower limb . postoperatively , the patient was not having any bladder / bowel incontinence , but she had weakness of her left lower limb which showed no recovery and was gradually progressive . 2 months before presentation , the patient had a history of slip and fall over her left knee while walking following which she developed swelling over her left knee joint . on examination , diffuse swelling was noted over left knee . valgus and varus instability tests were also positive . clinical picture of left knee joint and varus and varus instability test . the power of left lower limb was reduced ehl : extensor hallucis longus , edl : extensor digitorum longus , fhl : flexor hallucis longus , fdl : flexor digitorum longus knee , ankle , and plantar reflexes were absent . pain and temperature ( crude sensations ) were absent involving l4 , l5 , and s1 dermatomes . routine blood investigations are done found to be within normal limits except erythrocyte sedimentation rate ( 90 ) . x - ray and computerized tomography of knee joint were done which show marked destructive changes , sclerosis , dislocation , and subchondral fractures of knee joint ( fig . magnetic resonance imaging of the whole spine was done which show syrinx at d7 , d9 , d10 , and d12 and l1 level ( fig . arthrodesis could be attempted but with high failure rates and loss of available knee movements . knee replacement with constrained / hinged custom mega prosthesis ( in view of gross instability ) could be done with high rates of early loosening and infection . the patient was not willing for any surgical procedure , and hence , she was advised and fitted with orthoses . the incidence of permanent neurological injury following spinal anesthesia varies between 0 and 4.2/10000 patients . preexisting spinal pathology or the possible reason for this was indicated as misplacement of needle at the lower end of the spinal cord , misidentification of tuffier s line ( line drawn between the highest points of iliac crest in adults which corresponds to l4 spinous process ) [ 6 , 7 ] , arachnoid membrane attachment to the conus like a web . a traumatic needle insertion can cause severe disturbance in intramedullary microcirculation , and the direct toxicity of anesthetic agent over the injured cord resulted in syrinx [ 8 , 9 ] . neuropathic arthropathy develops in weight bearing joints , and the most common cause is diabetes mellitus , other causes being leprosy , meningomyelocele , syringomyelia , spinal cord injury , tabesdorsalis and syphilis [ 11 , 12 ] . painless abnormal mobility due to the destruction of afferent proprioceptive fibers , loss of sensation of joint , followed by severe degenerative changes , osteophyte formation , and subchondral fractures suggests neuropathic joint . neurological deficit as a complication of syrinx can be disabling and can lead to the neuropathic joints even in larger joints as illustrated in this case report . patients who have severe radicular pain following spinal anesthesia should be carefully followed up for the development of a syrinx and neurological deficit . the neuropathic knee can occur as a complication and is difficult to treat with poor outcome .

introduction : neurological complications due to spinal anesthesia are dysesthesia , paresthesia , cauda equina syndrome , and neuropathic joint . however , neurological complications are rare . we report a case of neuropathic joint of knee as a complication of syrinx following postspinal anesthesia.case report : a 33-year - old female came with complaints of pain and instability of her left knee and left foot drop . she had developed weakness of her left lower limb following her cesarean section surgery done under spinal anesthesia . clinically and radiologically , she had features of the neuropathic left knee joint which had occurred as a complication of syrinx following spinal anesthesia.conclusion:the case is reported for its rarity and to highlight the possible neurological complications of spinal anesthesia .

epigastric pain is a common symptom in the emergency department ( ed ) and is generally not fatal . however , it can be the only symptom of isolated spontaneous gastric artery dissection . here , we report the case of a 51-year - old woman who initially presented to the ed with epigastric pain only and was subsequently diagnosed with isolated spontaneous gastric artery dissection and hepatic artery aneurysm . a 51-year - old woman presented to the ed with epigastric abdominal pain for 2 days . two days before admission , she consumed spicy food and subsequently developed a burning , sharp , intermittent , colicky pain with nausea . she denied vomiting , diarrhea , fever , sweating , dyspnea , or any other symptoms including back and chest pain . her vital signs were as follows : blood pressure , 125/70 mmhg ; pulse rate , 80 beats / min ; respiratory rate , 20 breaths / min ; and body temperature , 36.0c . physical examination revealed a soft and flat abdomen as well as tenderness without rebound tenderness in the epigastric area ; other findings were unremarkable . her laboratory data were normal , including hemoglobin , platelet count , white blood cell count , and blood chemistry . abdominal radiography revealed no abnormal bowel gas pattern , abnormal calcific density in the abdominopelvic cavity , or organomegaly . three hours later , her symptoms ( i.e. , burning , colicky pain , and epigastric discomfort ) improved , and she was discharged with oral medications . two hours later , she returned to the ed with burning , sharp , persistent epigastric pain ; rebound tenderness in the epigastric area with a newly developed murphy s sign . vital signs at her second visit were as follows : blood pressure , 110/60 mmhg ; pulse rate , 95 beats / min ; respiratory rate , 23 breaths / min ; and body temperature , 36.7c . enhanced abdominopelvic computed tomography was performed to exclude acute cholecystitis and showed a left hepatic arterial pseudoaneurysm with an acute hematoma due to its rupture ( fig . 1 ) . selective celiac angiography revealed a left hepatic arterial aneurysm and right gastric artery dissection ( fig . the patient was subsequently managed in the cardiovascular department and discharged without complications 10 days later . they mainly involve the splenic artery ( 60% ) ; other less - frequent visceral sites include the superior mesenteric artery ( 5.5% ) , celiac trunk ( 4% ) , left gastric artery ( 4% ) , and gastroepiploic artery ( 4% ) . the superior mesenteric artery is the most frequent site of dissection in visceral arteries , and celiac artery dissection is rare . isolated spontaneous gastric artery dissection is rare , although there are reports of isolated spontaneous celiac artery dissection . spontaneous visceral artery dissection is five times more common in men than in women , and the average age of patients is approximately 55 years . the risk factors are hypertension , smoking , connective tissue diseases , atherosclerosis , abdominal trauma , medial degeneration , fibrodysplasia , and vasculitis [ 4 - 8 ] . although the clinical presentation varies , almost all cases of celiac artery dissection are characterized by abdominal pain mainly located in the epigastric region accompanied with nausea , vomiting , or diarrhea [ 7 - 9 ] . epigastric pain is a common symptom in the ed and is generally not fatal except in acute myocardial infarction , aortic dissection , and abdominal aneurysm rupture . given its low incidence and unpredictable natural history , hepatic artery aneurysm is rarely considered in the diagnosis of acute abdominal pain . depending on the location of the arterial dissecting lesion , jaundice associated with the hepatic artery dissection and pancreatitis with elevated serum amylase levels associated with the dissection of branches of pancreatic artery may be observed . computed tomography is the best diagnostic modality for this purpose ; in addition to being fast and accurate , it provides the most information for making therapeutic decisions . on angiography , artery dissection is characterized by a double lumen , string sign , intraluminal defects , intimal flaps , tapered occlusions , short segmental narrowing , irregular stenosis , and aneurysms . the disease courses of visceral artery dissection include limited progression with thrombosis of the false lumen , progression of dissection , narrowing or obliteration of the true lumen due to expansion of the false lumen , and rupture . therefore , management aims to limit the progression of the dissecting hematoma , control hypertension , and prevent thromboembolic complications in order to prevent the expansion of the false lumen , which may cause malperfusion , aneurysmal dilatation , or rupture . drug treatments include anticoagulants , anti - inflammatory drugs , steroids , and secondary antiplatelet therapy , which are advisable in patients with stenotic lesions to prevent thrombosis . endovascular or surgical treatment for revascularization should be considered in patients with persistent abdominal pain , bowel ischemia , necrosis , progression of the dissection , and arterial rupture . however , endovascular treatment with a stent or embolization is an attractive option for patients at high surgical risk . although extremely rare , patients presenting with epigastric pain who are at risk of vasculopathy should be considered for isolated spontaneous gastric artery dissection .

epigastric pain is a common symptom in the emergency department . most epigastric pain is not fatal , except in acute myocardial infarction , aortic dissection , and abdominal aneurysm rupture . epigastric pain can also be the only symptom of isolated spontaneous gastric artery dissection . we report the case of a 51-year - old woman diagnosed with neurofibromatosis who initially presented with only epigastric pain . she was initially misdiagnosed with gastritis , treated conservatively , and discharged . two hours later , she returned to the emergency department with persistent epigastric pain and rebound tenderness in the epigastric area . angiography revealed a left hepatic arterial aneurysm and right gastric artery dissection , which were subsequently embolized . although extremely rare , patients presenting with epigastric pain at risk of vasculopathies should be considered for isolated spontaneous gastric artery dissection . early recognition and definitive therapy lead to a favorable outcome .

tracheomegaly has been reported in cases of congenital disease , connective tissue disease , and acquired conditions . the pathogenesis of tracheomegaly in amyotrophic lateral sclerosis ( als ) , however , is unclear . herein , we report a case of tracheomegaly secondary to tracheotomy tube cuff in a patient with als . a 51-year - old man was referred to our hospital for dyspnea and recurrent air leakage around the tracheotomy tube of recent origin . the patient had a tracheotomy 4 years ago for respiratory failure , which required prolonged all - day mechanical ventilation . there were instances of respiratory distress that occurred after either air leakage around the tracheotomy tube or rupture of tracheotomy tube cuff . the cause of these episodes was attributed to a dilated trachea , which led to air leakage around the tracheotomy tube , an increased need for air volume to ensure an adequate cuff seal , and trouble in maintaining mechanical ventilation . after tracheotomy , we performed chest computed tomography ( ct ) scan annually . before this event , the diameter of the trachea was unremarkable . a chest radiograph and ct scan revealed tracheomegaly , mainly at the level of the tracheotomy tube cuff ( figs . 1 and 2 ) . the maximum transverse diameter of the trachea was measured to be 43 mm and 42 mm in the axial and sagittal sections , respectively ( fig . 2 ) , the shape and diameter of tracheal dilatation matched those of tracheotomy tube cuff . ( a ) anteroposterior radiograph of the chest shows marked tracheomegaly ( arrows ) . computed tomography scans in axial ( b ) and sagittal ( c ) planes show marked tracheomegaly at the level of the tracheotomy tube cuff ( arrows ) . three - dimensional computed tomography scan demonstrates that the shape and diameter of tracheal dilatation are exactly similar to those of tracheotomy tube cuff . after the ct scan , considering the patient 's clinical status , we performed conservative treatment instead of surgical correction . we used the adjustable tracheotomy tube , which can pass above the level of tracheomegaly . after using the adjustable tracheotomy tube , air leakage around the tracheotomy tube or rupture of tracheotomy in addition , serial chest radiograph revealed gradual reduction of the initial tracheomegaly ( fig . anteroposterior radiograph of the chest shows decrease in tracheomegaly after using the adjustable tracheotomy tube ( arrows ) . this study was approved by the institutional review board of the chonnam national university hwasun hospital . amyotrophic lateral sclerosis , also known as lou gehrig disease , is a specific disorder that is characterized by stiff muscles , muscle twitching , and gradual loss of muscle power . besides a general lack of mobility , the affected individuals have difficulty in speaking , swallowing , and breathing . recently , home tracheotomy mechanical ventilators have become the device of choice for patients with advanced als as their use can prolong survival , reduce hospitalization , and improve quality of life . in this case , the patient had used the home tracheotomy mechanical ventilator . tracheomegaly in als may result from pressure necrosis , impairment of blood supply , infection , and injury to tracheal mucosa because of mechanical ventilation and prolonged intubation . the unique finding of this report is that the diameter of trachea progressively increased on serial ct scans after tracheotomy . this result suggested that tracheomegaly might have occurred because of mechanical ventilation or pressure from the tracheotomy tube . our three - dimensional ct scans demonstrated a size and shape match between the dimensions of tracheomegaly and the tracheotomy tube cuff . therefore , in this case , the primary cause of tracheomegaly was believed to be the tracheotomy tube cuff . tracheomegaly is diagnosed when the transverse diameter of the trachea exceeds 25 mm and 21 mm in men and women , respectively . in this report , the maximum transverse diameter was measured to be 43 mm on ct scan . a variety of surgical treatments have been suggested , but these are multistaged surgical procedures and each procedure carries serious risks . because this patient had advanced als , we performed conservative treatment instead of opting for surgery . we tried to use the adjustable tracheotomy tube accommodating the enlarged trachea and thus maintaining the use of the home tracheotomy mechanical ventilator in addition to protecting the trachea . in conclusion , tracheomegaly has not been reported in an als case and this is the first report of tracheomegaly associated with tracheotomy tube cuff and a home tracheotomy mechanical ventilator . when a patient with als develops repeat air leakage around the tracheotomy tube or rupture of tracheotomy tube cuff , the clinician should consider the possibility of tracheomegaly in the differential diagnosis .

abstracttracheomegaly has not been reported in amyotrophic lateral sclerosis ( als ) . herein , the authors report a case of tracheomegaly secondary to tracheotomy tube cuff in a patient with als . to our knowledge , this is the first report of an als patient with tracheomegaly and of tracheomegaly being associated with tracheotomy tube cuff and home tracheotomy mechanical ventilator.the clinician should consider the possibility of tracheomegaly in the differential diagnosis , if a patient with als develops repeat air leakage around the tracheotomy tube or rupture of tracheotomy tube cuff .

a 75-year - old right - handed woman was admitted because of the abrupt development of mental confusion , left hemiparesis , and dysarthria . one day before admission , she developed weakness in her left arm and disorientation , followed 20 hours later by inappropriate speech , agitation , and dysarthria . she had a history of hypertension and diabetes , but no history of psychiatric illness . , she showed partially impaired orientation to time , but had well - preserved orientation to place and person . the neurological examination revealed left homonymous hemianopsia , but no obvious motor or sensory deficits . she showed no deficits in the line bisection and cancellation tasks , but was unable to copy an interlocked pentagon . three days later , she developed motor weakness ( grade 4 ) and hypesthesia in her left arm and face . the motor deficits and dysarthria were improved the next day , but the sensory deficit was unchanged . initial brain magnetic resonance imaging ( mri ) with diffusion - weighted images ( dwi ) was performed on admission , which revealed an acute infarction in the right parietal lobe with subtle involvement of the posterior temporal area ( figure 1a ) . magnetic resonance angiography ( mra ) revealed an obstruction of portion m1 of the right middle cerebral artery ( figure 1b ) . repeat mri on the fourth hospital day after the motor deficits developed revealed that the infarction had increased in size ( figure 1c ) . brain 2-deoxy-2-[f]fluoro - d - glucose positron emission tomography ( f - fdg pet ) performed 13 days after admission showed reduced fdg uptake in the right hemisphere , which was most severe in the right parietal lobe and left cerebellum ( figure 2a ) . when she was discharged 2 weeks after admission after 13 months , her neurological deficits and manic symptoms had resolved completely , except for the left - sided visual field defect . repeated brain f - fdg pet showed that the previous reduction in fdg uptake in the right hemisphere had improved ( figure 2b ) . this patient had sudden - onset restlessness with manic symptoms , including agitated mood , irritability , euphoria , talkativeness , pressured speech , and decreased need for sleep . the close temporal relationship between the onset of her symptoms and ischemic stroke , along with the absence of a history of affective disorders , supports the hypothesis that her restlessness with mania was caused by acute infarction of the right parietal lobe . an extensive literature search revealed 17 reports that described 29 patients who had post - stroke manic episodes . of these , eleven patients developed manic symptoms concomitantly with their strokes . in the other cases , the temporal relationship between the stroke and mania was obscure , either with a fairly long latency or due to insufficient information . the reported anatomical locations of the strokes were the temporal and frontal cortex ( 13 patients ) , 1,2,5,6,1517 thalamus ( 9 patients),3,5,8,9,11,13 caudate nucleus ( 2 patients),6 and ventral pontine region ( 2 patients).7 some patients had infarctions in widespread regions involving the frontal , temporal , and parietal lobes . our patient had an infarction that was mainly confined to the right parietal region on the initial brain mri , although there was subtle involvement in the posterior temporal area . post - stroke mania due to a localized infarction in the right parietal lobe has not previously been reported . one report described a case affecting the right parieto - occipital area involving the geniculocalcarine tract with agitation , hallucinations , and delusion , but the computed tomography result was not reported.18 widespread reduction in right hemispheric activity was seen on the brain fdg pet . although there was no structural damage ( i.e. , infarction ) , this may have been responsible for the restlessness and manic symptoms in this patient . however , the persistence of reduced activity in the right hemisphere , as shown on the repeated brain pet , along with a complete resolution of the manic symptoms , supports the possible relationship between the manic symptoms and the right parietal infarction . disinhibition syndrome , including restlessness and manic features , is usually caused by lesions in the orbitofrontal and basotemporal cortices of the right hemisphere.5,19 these areas are thought to inhibit the motor , instinctive , affective , and intellectual behaviors elaborated in the dorsal cortex selectively.19 lesions in these areas or in their connections to the dorsal cortex could produce disinhibition syndrome . this may range from mildly inappropriate social behavior to full - blown mania.19 caplan et al.20 observed that about half of the patients with infarcts of the inferior division of the right middle cerebral artery were in an agitated , confused state , showing hyperactivity , restlessness , and easy distractibility . et al.20 suggested , the inferior parietal lesions in our patient may interrupt the connections between the medial limbic cortex and superior parietal and frontal lobes , altering the emotional tone and affective behavior .

mood disorders following acute stroke are relatively common . however , restlessness with manic episodes has rarely been reported . lesions responsible for post - stroke mania can be located in the thalamus , caudate nucleus , and temporal and frontal lobes . we present a patient who exhibited restlessness with manic episodes after an acute infarction in the right parietal lobe , and summarize the case reports involving post - stroke mania . the right parietal stroke causing mania in our case is a novel observation that may help us to understand the mechanisms underlying restlessness with mania following acute stroke .