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invasive pneumococcal isolates from adults > 18 years of age were obtained from blood ( n = 460 ) and cerebrospinal fluid ( csf ) ( n = 229 ) during 19962003 by 54 laboratories serving 85% of the population in the czech republic . antimicrobial drug susceptibility and mics were determined by the broth microdilution method according to clinical and laboratory standards institute recommendations ( 10 ) . genotyping was performed by using pulsed - field gel electrophoresis ( pfge ) ( 11 ) and multilocus sequence typing ( mlst ) ( 12 ) . mlst results were identical between isolates of the same serotype within a pfge subtype ( 13 ) . cluster analyses were based on allelic data that used the eburst algorithm ( 14 ) . numerical trend was evaluated by regression analysis of the logarithm of the number of isolates by using spss for windows version 14 ( spss , inc . , chicago , il , usa ) . the test for trend ( epi info version 3.4 ; centers for disease control and prevention , atlanta , ga , usa ) was used to evaluate variations in proportions of different serotypes ; p values < 0.05 were considered significant . during 19962003 , a total of 446 ( 64.7% ) isolates were obtained from adults 1864 years of age and 243 ( 35.3% ) isolates were obtained from adults > 65 years of age . a progressive increase in recovery of isolates from blood was observed from 44 during 19961997 to 199 during 20022003 ( p = 0.009 ) ( figure 1 ) . half of all strains belonged to serotypes 3 ( 12.7% ) , 4 ( 8.6% ) , 8 ( 7% ) , 1 ( 5.8% ) , 19f ( 5.5% ) , 14 ( 5.5% ) , and 9v ( 5.2% ) ( figure 2 ) . serotype coverage rates by pcv7 , pcv10 , pcv13 , and 23-valent polysaccharide vaccine were 32.5% , 44.2% , 62.3% , and 82.1% , respectively , among adults 1864 years of age and 35.4% , 42.8% , 61.3% , and 85.6% , respectively , among adults > 65 years of age . a ) invasive pneumococcal isolates from blood and cerebrospinal fluid ( csf ) and b ) frequency of streptococcus pneumoniae serotypes 1 , 3 , and 4 among adults , czech republic , 19962003 . serotype distribution did not vary significantly with age group and type of isolate , except for serotype 1 , which was more common ( p = 0.037 ) among younger adults ( 1864 years ) and more prevalent in blood ( p = 0.004 ) . with the exception of 19981999 , this serotype , which composed11% of all isolates during 19962001 , increased to 15% during 20022003 ( p = 0.289 ) . a change was also observed in the proportions of serotypes 1 and 4 ( figure 1 ) . serotype 1 increased from 6% during 19961997 to 17% during 19981999 ( p = 0.027 ) and then decreased to 3% during 20022003 ( p<0.001 ) . serotype 4 increased from 2% during 19961997 to 13% during 20022003 ( p<0.001 ) ( figure 1 ) . penicillin - resistant pneumococci were uncommon ( range 1%5% ) , and no isolate showed high resistance to penicillin ( mic > 1 to chloramphenicol decreased from 5% during 19961997 to 2% during 20022003 ( p = 0.114 ) . tetracycline resistance also decreased from 8% during 19961997 to 6% during 20022003 ( p = 0.732 ) . resistance to chloramphenicol , usually observed with resistance to tetracycline , was seen most often in serotype 19f isolates . of 347 isolates of the 7 most common serotypes , 335 were characterized by pfge and 130 pfge subtypes were analyzed by mlst ( appendix table ) . forty - six allelic profiles were identified , 18 of which carried new alleles ( n = 11 ) or novel combinations of known alleles ( n = 7 ) . at least 60% of isolates of serotypes 1 , 3 , 9v , 14 , and 19f have been identified within clones in the pneumococcal molecular epidemiology network . in contrast , only 25% of serotype 4 and 8 isolates were identified in this network , and these serotypes consisted of larger clusters unrelated to global clones . when we compared our results with those of a study from the united states ( 5 ) , where 51% of ipd was among elderly persons before introduction of pcv7 and caused by pediatric serotypes ( 6b , 9v , 14 , 19f , and 23f ) ( 5 ) , our data showed a low frequency ( 27.2% ) of pediatric serotypes in older adults . the low frequency of penicillin - nonsusceptible pneumococci isolates of pediatric serotypes explains the observed predominance of drug - susceptible serotypes . local differences in blood culture protocols could also bias the serotype distribution ( 7 ) . although the number of blood cultures performed in the czech republic is increasing , lower numbers of invasive pneumococci have been isolated than in other countries in europe ( www.rivm.nl/earss ) . the observed predominance of serotype 3 , previously reported as less invasive but associated with a high death rate ( 15 ) , could be explained by sample collection primarily from the most severe ipd cases . in 1998 , an increase in serotype 1 invasive disease was reported in sweden . in our study , subsequently , the frequency of this serotype decreased but the frequency of bacteremia increased . the gradual increase in bacteremia and increased frequency of serotype 4 , previously found to be moderately invasive ( 7 ) , could be ascribed to the increase in blood cultures that facilitated detection of moderate disease caused by serotype 4 rather than dissemination of a specific invasive clone because the isolates showed high genotypic diversity . in our collection of invasive strains , the frequency of antimicrobial drug resistant global clones , all previously found in the czech republic ( 13 ) , varied between serotypes . our results support the need for ipd surveillance because differences in detection methods might modify the effect of the vaccine . continued surveillance will be helpful in detecting any shift in the distribution of invasive serotypes and clones circulating in the czech republic and in monitoring the effect of pcv7 on serotype frequencies and the genetic structure of pneumococci . genotypic data of invasive pneumococcal serotypes among adults , czech republic , 1996 - 2003 *

we conducted surveillance on invasive pneumococci isolated from adults in the czech republic during 19962003 . the 7 most prevalent serotypes were characterized . coverage with the 7-valent pneumococcal conjugate vaccine was low . our observations confirm that detection methods may have modified the expected effect of this vaccine .

elbow fractures are among the most common traumatic injuries in the paediatric population , consisting of 15% of all paediatric fractures [ 1 , 2 ] . humeral medial epicondyle fractures , in the paediatric population , account for up to 20% of elbow fractures , 60% of which are associated with an elbow dislocation . the chance of median and ulnar nerve injuries , an incarcerated fragment in the joint , as well as other complications , further increases when a fracture is associated with a dislocation . we present the case of a child who sustained a medial epicondyle fracture of the elbow , with a complete ulnar nerve palsy but without a joint dislocation , who was found intraoperatively to have ulnar nerve entrapment in the ulnohumeral joint . the rare occurrence of complications relating to both acute and late ulnar nerve entrapments have been reported before in literature including following supracondylar fractures , medial epicondyle fractures with elbow dislocations , forearm fractures and galeazzi fracture dislocations . however , there have been no reports describing an ulnar nerve intra - articular entrapment , with a medial epicondyle fracture but without an elbow dislocation . a 9-year - old girl presented to the emergency department with right elbow pain and swelling , after falling from a monkey bar onto her right elbow . she had no history or clinical signs of an elbow dislocation , but she had a complete ulnar nerve palsy [ with the absence of sensation and increased 2-point discrimination , medical research council ( mrc ) sensation grading s0 ; mrc power grading 0/5 of intrinsic muscles of the hand , the little finger flexor digitorum profundus ( fdp ) and flexor carpi ulnaris ( fcu ) ; froment 's sign was positive ) , but there was no evidence of vascular compromise . a plain radiograph showed that the medial epicondylar apophysis had been avulsed into the ulna humeral joint space , but there was no other fracture or dislocation seen ( fig . figure 1:plain radiographs of the injured right elbow : ( a ) anteroposterior view and ( b ) lateral view . plain radiographs of the injured right elbow : ( a ) anteroposterior view and ( b ) lateral view . she was taken directly to the operating theatre for emergency surgery , and an examination under anaesthesia revealed no elbow joint instability . the ulnar nerve was not found in the anatomical position , so further exploration was undertaken . the ulnar nerve was subsequently found to be trapped between the trochlea and proximal ulna , intra - articularly ( fig . 2 ) . the medial epicondyle was also found avulsed from the humerus , with an incarcerated medial epicondylar fragment in the elbow joint . the medial epicondyle was reduced to its anatomical position and fixed with a single cortical screw . figure 2:intraoperative image showing the intra - articular entrapment of the ulnar nerve highlighted by the arrow . intraoperative image showing the intra - articular entrapment of the ulnar nerve highlighted by the arrow . a few hours post - operatively the sensation over the ulnar territory started to improve ( mrc grading s2 ) , fcu and fdp had partially recovered ( power improving to mrc grading 3/5 ) , but there was still mrc grading 1/5 of the intrinsic muscles of the hand . on the first post - operative day , the fcu and fdp were fully recovered with a mrc grading of 5/5 , but the intrinsic muscles of the hand remained weak . at 2 weeks follow - up , the wound had healed well and the plaster was changed to an above elbow cylinder cast . neurological examination of her hand revealed some still reduced sensation over the ulnar nerve territory , normal fcu and fdp function , with some further recovery of the intrinsic muscles of the hand ( mrc grading 3/5 ) . at 5 weeks follow - up , the plaster cast was removed and the patient had a good range of motion of the elbow , with no signs of instability . she had a mrc grading of 5/5 of all the muscles , including the intrinsic muscles of the hand , and froment 's sign was negative , but there was still some reduced sensation ( mrc grading s3 ) . follow - up , the patient had full range of movement of the stable elbow with completely recovered ulna nerve function . figure 3:plain radiographs showing the healed fracture of the right elbow at 5 months : ( a ) anteroposterior view and ( b ) lateral view . plain radiographs showing the healed fracture of the right elbow at 5 months : ( a ) anteroposterior view and ( b ) lateral view . the contribution of the soft tissue structures around the elbow is of great importance in the stabilization of the elbow . knowledge of the anatomy and biomechanics of the elbow , particularly the soft tissue and bony constraints , is of paramount importance in the diagnosis , management and long - term sequelae of injuries . in children , as the bony constraints of the elbow are not well developed , the stability of the elbow depends mainly on the soft tissue structures . therefore , elbow dislocations in children will result in signs of damage of the soft tissue structures of the elbow . however , open reduction with internal fixation of the epicondyle fragment is clearly indicated in cases of intra - articular entrapment of the fragment , on suspicion of entrapment of the ulnar nerve , or in cases of marked instability of the elbow . a medial epicondyle fracture associated with elbow dislocation , in children , may not be easily diagnosed on conventional radiographs because of the small size of the fragment , its hidden position behind the distal humerus and the fact that it can be mistaken for the trochlear ossification centre . in the case presented , complete ulnar nerve palsy was present without any evidence of elbow dislocation in the history and with there being no signs of elbow instability intra - operatively . prompt clinical assessment and surgical treatment confirmed the intra - articular ulna nerve position and avoided that a simple reversible neurapraxia further developed into an irreversible devastating nerve lesion .

elbow fractures are not uncommon in children , and some are associated with neurovascular injuries . having a nerve injury in an elbow fracture without dislocation is rare and was not described in the literature . here , we have reported probably the first case of an ulnar nerve injury in an elbow fracture without dislocation . a 9-year - old female presented to the emergency department after falling off a monkey bar . she had a painful , swollen and tender right elbow with no history or clinical signs of an elbow dislocation but had complete ulnar nerve palsy . she was managed initially with analgesia and plaster application and was taken directly to the operating theatre . examination under anaesthesia revealed no elbow joint instability . the ulnar nerve was found entrapped between the trochlea and proximal ulna , intra - articularly . the medial epicondyle was also found avulsed from the humerus , with an incarcerated medial epicondylar fragment in the elbow joint .

uterine artery anatomic variants have been the subject of detailed study , since the beginning of last century , given fibromyoma role in the pathogenesis of arterial vasculature . according to various studies , there are certain classifications of uterine artery anatomical variants and paper proposes such a classification , the observations selected aspects of examinations performed angiographic fibromyoma embolization . 110 examinations were conducted in women aged between 20 and 47 years , with symptoms and clinical diagnosis of uterine fibromyoma . embolization procedure was performed by unilateral brachial vascular access , using seldinger technique with a sheath pressure mounting and placing a 4f or 5f cobra catheter tip , running through the axillary artery , subclavian artery , ( brachiocephalic arterial trunk ) descending aorta , iliac artery common internal iliac artery , uterine artery tracing is the purpose of selective catheterization of it . retrospective evaluation of imaging uterine artery arteriography allowed classification into four groups , depending on the origin of the uterine artery . 220 were observed in the uterine artery catheterization and were classified number 200 , which represents 90.1% of the total , the remaining 20 were not conclusive arteriogram . analysis arteriogram interpretation allowed a classification into 4 types , given the classifications listed in the literature as follows : - type i - the origin of the uterine artery inferior gluteal artery ram ( fig . 1 ) - type ii - the origin of the uterine artery bifurcation ram inferior gluteal artery ( fig . 2 ) - type iii - the origin of the uterine artery to the internal iliac artery trifurcation ram , with lower and upper gluteal artery ( fig . 3 ) - type iv - uterine artery proximal to the origin of the origin of the gluteal arteries top and bottom ( fig . 4 ) the origin of the uterine artery inferior gluteal artery ram the origin of the uterine artery bifurcation ram inferior gluteal artery the origin of the uterine artery to the internal iliac artery trifurcation ram , with lower and upper gluteal artery uterine artery proximal to the origin of the origin of the gluteal arteries top and bottom anatomy descriptive notes uterine artery origin from the internal iliac artery anterior trunk , with arteries bladder , rectal average obturatory artery , internal pudendal artery , sciatica , however note the variant of origin of uterine artery from a common trunk artery umbilical [ 5 - 8 ] . in fact , the origin of the uterine artery is variable , according to recent studies , which are based on angiographic examinations . currently there is a classification of the uterine artery origin : type i ( the origin of the uterine artery inferior gluteal artery ram ) in 45% , type ii ( the origin of the uterine artery bifurcation ram inferior gluteal artery ) 6% , type iii ( origin uterine artery to the internal iliac artery of the ram trifurcation , with the upper and lower gluteal artery ) 43% and 6% type iv ( proximal to the origin of the uterine artery to the origin of the upper and lower gluteal arteries ) . recent studies , which are based on angiographic examination , note the presence of two trunks of bifurcation of the internal iliac artery , anterior and posterior in 70% of cases , and uterine artery is inferior gluteal artery origin or branch of the internal iliac artery trifurcation . the present study shows different values of such a percentage , such type i is 24% of the total arteriograms classifiable and type ii is in a proportion of 10% . for type iii percentage different values are observed to specialized studies , with a much increased type iv also shows that the type iii and type i are halfway to the classification established values . this again demonstrates the variability of uterine artery origin in different percentages , but major practical importance . in addition to the above aspects of the present and the emergence of uterine artery dissection of the cadavers in department of anatomy , university of medicine craiova . this notice these issues . in fig.5 the origin of the uterine artery uterine artery dissection origin proximal to the superior and inferior gluteal arteries emerging ( arrow ) in fig.6 uterine artery arises from the internal iliac artery , making common core with superior gluteal artery and inferior gluteal . knowledge of emerging variants uterine artery has an important role in the success of fibromyoma embolization , thus avoiding damage to other vascular structures , also correct assessment of uterine artery origin is reflected in the reduction of radiation dose and shortening the time of intervention .

uterine artery embolization as a therapeutic method in fibromyoma requires a good knowledge of the origin of the uterine artery to the success of this procedure involving selective catheterization . this study presents a classification of anatomical variants of uterine artery as a retrospective review of consecutive arteriogram , complete with various aspects of the origin of the uterine artery in cadaver dissection , in the department of anatomy .

brain abscess though less common , continues to constitute one of the most important neurological emergencies with potential poor outcome . thalamic and basal ganglia abscesses are much rarer than abscesses in other locations of the brain . only a few cases of such inflammatory lesions have been reported [ table 1 ] . we report a rare case of basal ganglia and thalamic abscess as a complication of ventriculo - peritoneal ( vp ) shunt . pediatric basal ganglia and an 18-month - old female child presented with fever , vomiting off and on for 2 months and altered sensorium and seizure for 1 day at an outlying hospital . child was operated at 25 days of life for cervical meningomyelocele and vp shunt was placed for hydrocephalus . shunt revision was carried out at 1 month of age for oozing from surgical site . at 6-month of age , vp shunt was seen protruding through anus , for which distal end of shunt was shortened surgically and shunt left in situ at the outlying hospital . child was referred to us with altered sensorium and glasgow coma score of 8/15 . magnetic resonance imaging ( mri ) revealed multiple abscesses in the left basal ganglia and one in thalamus which measured approximately 25 mm 24 mm with midline shift of 11 mm [ figure 1 ] and shunt was transversing through the trigone , quadrigeminal area into the thalamic area on the contra - lateral side . investigation revealed high total leukocyte count ( 25700/mm ) with thrombocytosis ( 469 10 ) , prothrombin time / international normalized ratio-20.1/1.99 , na-123 meq / l . child was managed with mechanical ventilation , 3% saline , intravenous meropenem ( 120 mg / kg / day q 8 h ) and vancomycin ( 60 mg / kg / day q 6 h ) empirically . hyponatremia was corrected gradually over next 48 h. vp shunt was removed and external ventricular drain ( evd ) was placed in right ventricle for immediate decompression followed by stereotactic aspiration of abscess . morganella morgagni , klebsiella pneumonia , and enterococcus faecalis were isolated from the pus and m. morgagni was isolated from blood . intravenous antibiotics were given for a total of 6 weeks and subsequently patient underwent vp shunt revision . magnetic resonance imaging brain showing abscess in left thalamic and basal ganglia ( see arrow ) with midline shift to right thalamic and basal ganglia abscesses are relatively rare , the reported incidence varies from 1.3 - 6% of brain abscesses . thalamic abscesses are metastatic lesions of hematogenous origin , mostly from congenital heart disease , intra - thoracic sepsis , abdominal sepsis , cryptogenic and of local origin , from dental caries , otitis media , and sinusitis . immuno - compromised state is one of the predisposing factors ; however , in many cases source of sepsis or predisposing factor could not be determined . in our case , there was a retrograde spread of shunt infection from the colonic bacteria as the shunt that was protruding through the anus was only repositioned and not completely replaced . most patients present with fever with leukocytosis , signs of raised intracranial pressure such as headache , altered sensorium , and hemiparesis . computed tomography ( ct ) and mri differential diagnosis includes fungal , nocardial or tubercular abscesses , neurocysticercosis , toxoplasmosis , glioblastoma , metastasis , infarction , and resolving hematoma . the diagnosis can be difficult , particularly in the absence of fever and serological signs of infection . treatment options include stereotactic aspiration with or without continuous drainage , free hand aspirations through a burr hole , stereo - endoscopic aspiration , ultrasound guided aspiration , surgical trans - ventricular approach , and medical management . stereotactic aspiration remains the preferred treatment as it drains the contents of the abscess , reduces mass effect , and confirms diagnosis . it is minimally invasive ; carries less morbidity , mortality , and can be performed on compromised patients under local anesthesia . reported incidence of complications with stereotactic surgery is 7.2% and incidence of symptomatic hemorrhage ranges from 0 - 5.3% . since the procedure is blind , there are chances of incomplete evacuation with recurrence of abscess and fulminant ventriculitis by intra - ventricular leakage . after stereotactic aspiration or drainage , vp shunt may be required in 50% of the cases . evacuation of brain abscess by a stereotactic endoscope has been reported by wan and jiang the endoscopic aspiration with antibiotic irrigation of thalamic abscess was successfully reported by gajdhar and yadav advantage of this technique was aspiration of abscess under direct visualization with dissolution of loculi , direct assessment of completeness of aspiration and lesser chances of complications like hemorrhage . the ability to monitor the drainage by using near - real - time imaging information is exceptional with mri . multiplanar capabilities , high spatial resolution , excellent soft - tissue contrast and the absence of ionizing radiation with mri makes it a more attractive alternative ; however , high cost may limit the availability . in our case , due to non - availability of real - time mri at our institution , ascending infection with gram negative enteric organisms in central nervous system is a known complication of vp shunt , may rarely cause abscess in thalamus and basal ganglia requiring neuro - intensive care , early drainage , appropriate antibiotics , and stereotactic neurosurgical intervention .

we report an 18-month - old female child with ventriculo - peritoneal shunt related thalamic abscess treated with stereotactic aspiration . deep seated abscesses are complex due to difficult access and are associated with an increased risk of intra - ventricular rupture as well as antibiotic resistance , a fact which justifies a more aggressive and immediate neurosurgical management .

episodic loss of consciousness presents as a diagnostic challenge to the physicians as well as to the neurologists , many a time . often , such episodes are named epileptic and even treated wrongly , depending on the specific and nonspecific interictal electroencephalography ( eeg ) changes . when symptoms keep recurring , despite treatment , one would consider other organic and nonorganic causes and invariably subject the patients to many diagnostic procedures with very little positive results . changes in cardiac rhythms are known to occur during clinical seizures , but in situations where there is an epileptic discharge in eeg along with bradycardia and syncope , it is often referred as ictal bradycardia syndrome or ictal syncope . seizure - related cardiac arrhythmias are mostly encountered in older children and adults only and very rare in infants . the given case in an infant describes the initial difficulty in arriving at a diagnosis and the subsequent outcome . a 6-month - old girl was referred from a primary physician for recurrent breath holding attacks since the age of 4 months . she was born as term , intrauterine growth retardation , and at 4 months age , she started developing frequent apnea , following prolonged incessant cry with momentary stiffness and rolling up of eyes . these episodes were diagnosed as breath - holding spells , although there were no definite precipitating events . routine electrocardiography ( ecg ) and eeg could not reveal initially any specific abnormality except bradycardia ; she received oral iron syrup for some time with no improvement of symptoms and so parents stopped the drug . at 6 months of age , symptoms became more severe and of prolonged durations ; at one instance she was hospitalized when she developed prolonged apnea , needed oxygen inhalation , and bag and mask ventilation . at that time , ecg revealed , prolonged sinus bradycardia , asystole , junctional escapes preceded by sinus tachycardia . sick sinus syndrome ( sss ) was contemplated on this basis and oral beta - blocker ( propronolol ) treatment was started . , mode vv1 dash rate adaptive pulse generator with rate of 80/ min ) following left thoracotomy with an intramyocardial unipolar lead . with this , her symptoms improved and so she was labeled as sss . after 2 weeks , she had a severe life - threatening event ; she collapsed with bradycardia and lost consciousness suddenly . she was intubated and ventilated for 48 h. pace maker function was checked and found to be normal . phenobarbitone was given as loading dose with the probable diagnosis of autonomic seizures as , interestingly , a prolonged sleep eeg recording with video telemetry , at this time , revealed a left frontotemporal paroxysmal theta burst , which was not documented before ; this confirmed a diagnosis of ictal syncope . carbamazepine was planned ; however the next day , she developed one more prolonged episode of syncope , bradycardia and died as she could not be resuscitated . syncope as an isolated manifestation of seizures in children has a poor localizing value and the term ictal syncope is proposed for transient loss of consciousness and posture that occurs in a seizure before or even without convulsions . seizure - related cardiac dysrhythmias are well recognized and present mostly as tachyarrhythmia and bradycardia ; asystole , on the other hand , is exceptional and causes life - threatening hemodynamic disturbances . they invariably occur along with clinical seizures or as late as 60 min after the seizures . thus , ictal asystole is the most dramatic manifestation of ictal bradycardia and may contribute to seizure - related injury and sudden unexplained death in epilepsy patients ( sudep ) . the present case of ours , presenting as " breath - holding spell " was diagnosed initially as prolonged qt interval syndrome leading to syncope , which is often misinterpreted as the possible etiology . long qt syndromes are associated with genuinely life - threatening syncope , which may be simple or convulsive . there was no history suggestive of typical clinical seizures in our patient , which prompted us to diagnose this as primary cardiac rhythm disorder , as the primary event was apnea . the diagnosis of ictal bradycardia and syncope was contemplated , only , when a prolonged eeg trace was done , after one life - threatening episode , which showed left temporal focus , although routine eeg was normal before . eeg findings in syncopal events are nonspecific with slowing of the background rhythms , followed by high amplitude delta waves and then absolute flattening , depending on the severity of cerebral hypoperfusion , but tend to improve in the reverse sequence as clinical improvement sets in gradually . ictal bradycardia syndrome may occasionally mimic syncope and so unless simultaneous eeg and ecg are not performed , the diagnosis of this unique disorder could be missed . a review of the literature also emphasizes the difficulties in the diagnosis and treatment of this rare condition . a possibility of dominant parasympathetic ictal stimulus following excitation of reticular formation , precipitating ictal bradycardia and sinus arrest can not be ruled out . when autoregulation gets disrupted because of missing sympathetic innervations , especially in the presence of associated cortical dysplasia , sudden cardiac ictal death might occur , which could have been the reason in our patient . ictal sinus arrests with temporal lobe focus and seizures ( tle ) have been reported in the past that became symptom free after epilepsy surgery . this child , in spite of cardiac pacing and specific anticonvulsant treatment , succumbed to the illness probably because of the underlying cortical abnormality controlling autoregulation . mri study of brain was planned to delineate any dysplastic abnormality of the brain but could not be done . the ictal bradycardia / syncope syndrome should be considered in patients with unusual or refractory episodes of syncope or in patients with history of both epilepsy and syncope . correct diagnosis may be attained by simultaneous eeg / ecg at the time of syncope . at times , when the diagnosis could not be achieved , a video eeg recording of the ictal events becomes mandatory , which could throw more light . cardiac pacemaker implantation along with antiepileptic drug therapy may be necessary to minimize the attacks , although permanent cure could be claimed with epilepsy surgery . pacemaker alone may correct the malignant form of bradyarrhythmia in the heart , and thereby decrease seizure occurrence but may not prevent death due to seizures totally .

isolated syncope is a rare manifestation of seizures in children and it is a poorly localizing sign in the evaluation of seizures in children . many times , such episodes are mistaken as primary cardiac dysrhythmias and we present herewith one such case in a child , who succumbed to the illness despite treatment .

although pterygium excision with conjunctival autograft is a widely performed surgical procedure , surgically induced necrotizing scleritis ( sins ) following such surgery is extremely rare . on postoperative day 17 , the conjunctival graft was avascular , with epithelial defect . although topical steroid and antibacterial treatments were continued , the graft and sclera melted , with the ischemic sclera showing gradual thinning . the thinning area spread to the adjoining cornea , and active inflammation with epithelial defect was observed adjacent to the site of thinning . the patient was suspected of having sins , and administration of oral prednisolone was started . although the necrotic area was reduced temporarily , medication was discontinued due to nausea , and the area of thinning increased . sins must be considered in the differential diagnosis of patients with scleritis following pterygium surgery , especially if radiation or mitomycin c has not been used . surgically induced necrotizing scleritis ( sins ) , a local autoimmune reaction occurring near previous surgical wounds , has been reported after cataract surgery , trabeculectomy , retinal detachment , and strabismus surgery.1 we report sins after primary pterygium excision with conjunctival autograft , an extremely rare occurrence , with only two previously reported cases.2,3 a 68-year - old man with no history of systemic or ocular disease was referred to tokyo dental college ichikawa general hospital for treatment of primary pterygium . bestcorrected visual acuity ( bcva ) was 20/20 in both eyes . slit - lamp examination revealed nasal pterygium in his right eye , and pterygium excision and conjunctival autograft transplantation without mitomycin c were performed uneventfully . on postoperative day 1 , slit - lamp examination revealed a well - adapted , epithelialized graft , with corneal epithelial defect at the site of pterygium excision . topical treatment with 0.5% levofloxacin and 0.1% betamethasone on postoperative day 17 , the conjunctival graft was avascular , with epithelial defect ; the initial corneal epithelial defect had diminished with no symptoms . although topical steroid and antibacterial agents were continued , the graft and sclera melted , with the ischemic sclera showing gradual thinning . on postoperative day 29 , although the scleral bed was covered by keratinized conjunctival epithelium , active inflammation with epithelial defect was observed adjacent to the site of thinning ( figure 1 ) . by postoperative day 36 , the thinning area had spread to the adjoining cornea , and active inflammation persisted . a diagnosis of sins was suspected , and administration of oral prednisolone ( 20 mg per day ) was commenced . five days later , the conjunctival and scleral inflammation had decreased and vascularization was observed on the inferior scleral side . however , prednisolone was discontinued due to nausea , and the area of thinning increased in size ( figure 2a and b ) . resection of necrotic tissue and lamellar keratoplasty were planned but not performed , as the patient ceased visiting the hospital . conjunctival flap with tenon s capsule was later performed at another hospital , and the graft was well accepted . in this case , sins occurred approximately 2 weeks after surgery , with ischemia of the conjunctival graft and underlying sclera , as previously reported.2,3 in earlier cases , the extent of the disease has been limited to the sclera . here , however , scleral thinning and inflammation spread to the adjoining cornea . scleral necrosis and melting can occur after pterygium surgery due to the use of adjunctive irradiation , mitomycin c , or excessive cauterization of the sclera.46 these etiologies should be ruled out before diagnosing sins . in this case , no radiation or mitomycin c was used , and cauterization was kept to a minimum during surgery . in fact , hundreds of pterygium surgeries have been performed at our institute , in the same manner , with no other cases developing necrotizing scleritis . the spread of the disease to the cornea eliminated cauterization as a likely primary cause . in addition , reduced inflammation after systemic immunosuppression supported our diagnosis , indicating the utility of immunosuppressive treatment in identifying sins . treatment of sins after pterygium surgery has included immunosuppression with systemic steroids , cyclophosphamide , or tacrolimus,2,3,7,8 and surgical intervention , including resection of necrotic tissue , amniotic membrane transplantation , and scleral or corneal tissue patch grafts.2,7,9,10 in this case , systemic steroids had to be discontinued , and the administration of tacrolimus and further surgical procedures were considered . conjunctival flap with tenon s capsule is an option , especially if obtaining a scleral or corneal graft is difficult . resection of necrotic tissue and conjunctival flap with tenon s capsule were performed , and the graft was well accepted . this case suggests that sins should be considered as a possible diagnosis in patients with scleritis after pterygium surgery , especially if radiation or mitomycin c has not been used . ischemia and melting in the conjunctival graft and underlying sclera indicate sins , and a risk exists of it spreading to the adjoining cornea .

backgroundalthough pterygium excision with conjunctival autograft is a widely performed surgical procedure , surgically induced necrotizing scleritis ( sins ) following such surgery is extremely rare.methodsa 68-year - old man underwent nasal pterygium excision with conjunctival autograft uneventfully . on postoperative day 17 , the conjunctival graft was avascular , with epithelial defect . although topical steroid and antibacterial treatments were continued , the graft and sclera melted , with the ischemic sclera showing gradual thinning . the thinning area spread to the adjoining cornea , and active inflammation with epithelial defect was observed adjacent to the site of thinning.resultssystemic and microbiological examination was noncontributory . the patient was suspected of having sins , and administration of oral prednisolone was started . although the necrotic area was reduced temporarily , medication was discontinued due to nausea , and the area of thinning increased . conjunctival flap surgery was later performed , and the graft was well accepted.conclusionssins must be considered in the differential diagnosis of patients with scleritis following pterygium surgery , especially if radiation or mitomycin c has not been used .

one such tumor , pleuro pulmonary blastoma ( ppb ) , is very rare , highly aggressive and malignant , and originates from either the lungs or pleura . it occurs mainly in children aged less than five or six years [ 1 , 2 ] . ppb was classified into three groups by dehner in 1995 as cystic ( type i ) , mixed ( type ii ) , or solid ( type iii ) . type i occurs in infants ( median diagnosis age , 10 months ) in contrast to types ii and iii ( median diagnosis ages , 34 and 44 months ) , respectively . type ii is similar to wilms tumor morphologically and therefore , it is sometimes incorrectly called the treatment of children with type i ppb has been a surgical resection with or without adjuvant chemotherapy . ppb is very rare in children and there is no defined optimal treatment regimen for it to date . in this report , a case of ppb is presented whose management was carried out successfully with surgical resection followed by neoadjuvant chemotherapy . a 40 month old male child was admitted to the hospital with cough , and wheezing . chest x - ray showed an opacity filling the right lower lobe and leading to mediastinal shift to the opposite side . computerized tomography ( ct ) of thorax revealed a large mass in the right hemithorax ( 7x5x3 cm ) containing solid and cystic components . the patient underwent right thoracotomy , which demonstrated a solid and cystic mass in that partial resection . histological diagnosis of the tumor was ppb type ii because it contained both solid and cystic components . we scheduled adjuvant chemotherapy with vie(vincristine 1.5mg / m2 on day 1 , ifosfomide1 gr / m2 on day1 to3 , etoposide 150mg / m2 on day 1to3 ) alternated with vac ( vincristine 1.5 mg / m2 on day 1 , actinomycin - d 15 gamma / kg on day 1 , and cyclophosphamide 750 mg / m2 on day 1 ] . the ice and vac courses were alternated every three weeks . after the completion of the first course of chemotherapy , dyspnea resolved completely , and the third course of chemotherapy revealed a 90% reduction in mass size and the child was well at the 6 month fallow up . pleuropulmonary blastoma is an aggressive tumor accounting for less than 1% of all primary malignant lung tumors in the pediatric population . manivel and associates coined the term ppb to describe a specific subtype of pulmonary blastoma on the basis of its exclusive clinical presentation in childhood and its pathologic features of variable anatomic location , primitive embryonic - like blastoma and stroma , absence of a carcinomatous component , and potential for sarcomatous differentiation . denher and associates classified ppb into three groups ; type 1 with purely cystic tumors , type 2 as an intermediate type , and type 3 with predominantly solid tumors . a progression from type i to type the histologic appearance is variable - the tumor is characterized by primitive blastoma and a malignant mesenchymal stroma often showing multidirectional differentiation as rhabdomyosarcomatous ( as in our case ) , chondrosarcomatous or liposarcomatous . vargas et al . demonstrated , with cytogenetic analysis , that the polysomy of chromosome 8 is a constant feature of pleuropulmonary blastoma and the clonal proliferation in pleuropulmonary blastoma is restricted to the malignant mesenchymal elements , supporting the notion that the epithelial components are non - neoplastic . this neoplasm occurs not only in the lung , but it may arise from mediastinum , diaphragm and/or pleura . this has raised the possibility that ppb might originate from the splanchnopleural or somatopleural mesoderm . common metastatic sites include the brain , bone , lymph nodes , liver , pancreas , kidney , and adrenal glands . ppb may be associated with cystic pulmonary lesions , which may be evident at the time of diagnosis or predate the appearance of the tumor ; there are contradictory reports about the value of prophylactic resection of the pulmonary cysts in protecting patients from developing ppb . the occurrence of ppb appears to herald a constitutional and heritable predisposition to dysplastic or neoplastic disease in approximately 25% of the cases . associated conditions include ppb , medulloblastoma , malignant germ cell tumor , thyroid neoplasia , and others . the tumor has no characteristic findings on imaging studies ; however , it should be considered in the differential diagnosis of other benign cystic lung lesions on imaging studies . the baby in this case like most reported cases presented with a picture of pulmonary infection and respiratory difficulty . as complete tumor ablation is essential to prevent local recurrence and allow any chance of survival , the main goal of therapy should be radical surgery , followed by chemotherapy . because the response to chemotherapy is poor , some authors suggest that chemotherapy should be given with local radiotherapy in the majority of the patients . types ii and iii ppbs are clearly aggressive malignancies with projected overall survival of 62% at 2 yrs and 42% at 5 yrs , even after multimodality therapy . patients with pleural , mediastinal or extra pulmonary involvement at the time of diagnosis have worse prognosis than those without such involvement [ 4 , 13 ] . ppb is an aggressive tumor having poor outcome and it usually occurs in young children . though radiotherapy is an alternative treatment modality , especially in cases with high risk of recurrence , it causes severe morbidity , especially in younger children . we concluded that despite the presence of poor prognostic factors such as presence of solid tumor or those factors that increase the probability of local recurrence such as chest tube insertion and microscopically residual disease after surgery , chemotherapy and aggressive surgery may provide remission and a long - term disease - free period . complete surgical resection of the tumor at the time of diagnosis is the cornerstone of ppb management , but in the majority of patients , initial surgery is incomplete as a large tumor may involve vital structures . thus , chemo - reduction followed by complete excision may be the satisfactory treatment of this highly aggressive tumor in childhood . ppb is an aggressive tumor having poor outcome and it usually occurs in young children . though radiotherapy is an alternative treatment modality , especially in cases with high risk of recurrence , it causes severe morbidity , especially in younger children . we concluded that despite the presence of poor prognostic factors such as presence of solid tumor or those factors that increase the probability of local recurrence such as chest tube insertion and microscopically residual disease after surgery , chemotherapy and aggressive surgery may provide remission and a long - term disease - free period . complete surgical resection of the tumor at the time of diagnosis is the cornerstone of ppb management , but in the majority of patients , initial surgery is incomplete as a large tumor may involve vital structures . thus , chemo - reduction followed by complete excision may be the satisfactory treatment of this highly aggressive tumor in childhood .

pleuropulmonary blastoma [ ppb ] is a very rare , highly aggressive and malignant tumor that originates from either the lungs or pleura . it occurs mainly in children aged less than five or six years . it has poor prognosis with three different subtypes : cystic [ type i ] , combined cystic and solid [ type ii ] and solid [ type iii ] . ppb is treated with aggressive multimodal therapies including surgery and chemotherapy . we present a case of 3.5 years old boy with ppb type ii successfully treated with complete surgical resection followed by neoadjuvant chemotherapy .

inflammation of the pericardium due to infection or non - infectious etiology is known as pericarditis . the most common forms are idiopathic and viral which are usually self - limiting and benign conditions . predisposing factors include renal disease , previous cardiac and thoracic surgery , endoscopic oesophageal variecal sclerotherapy , laproscopic nissins fundoplication , fiberoptic bronchoscopy and percutaneous coronary angioplasty the portal of entry is usually direct invasion of infection from adjacent pneumonia or empyema and to a lesser extent may be due to hematogenous seeding from a distant infection . we report a fatal case of acute purulent pericarditis due to methicillin - resistant staphylococcus aureus ( mrsa ) with molecular characterization of its resistance mechanisms . a 78-year - old male who was a known case of squamous cell carcinoma of buccal mucosa , which was excised with cervical lymph node dissection two years ago along with systemic hypertension and renal failure ( urea 179 mg / dl , creatinine 5.28 mg / dl ) was hospitalized with complaints of fever since 10 days and dyspnoea on exertion class iii of two days duration . a chest x - ray [ figures 1 and 2 ] showed massive pericardial effusion and an electrocardiogram ( ecg ) [ figure 3 ] revealed low voltage waves , st elevation , atrial fibrillation and a fast ventricular rate . the white cell count was 21 k/l with 88% neutrophils and liver enzymes were also elevated ( sgot 103 iu / l , sgpt 80 emergency pericardiocentesis was done and 800ml of fluid was drained and a pigtail catheter was introduced through a subxiphoid incision and placed under fluoroscopic guidance in the pericardial cavity . pericardial fluid analysis showed ada 15.2 u / l , glucose 97.8 g / dl , protein 5.84 g / dl . chest x - ray on admission showing a massive pericardial effusion with a pig - tail catheter in situ for drainage chest x - ray after pericardiocentesis showing a pig - tail catheter left in situ for continuous drainage electrocardiogram on admission showing low voltage waves in leads ii , iii and avf ; st elevation in leads i , ii , avl and chest leads v1 v6 ; atrial fibrillation and a fast ventricular rate of 134 beats / min blood and pericardial fluid cultures grew gram positive cocci which were identified as mrsa with the vitek 2 ( biomerieux , inc . , both the mrsa isolates ( from blood and pericardial fluid ) were resistant to multiple classes of antibiotics namely oxacillin , penicillin , trimethoprim - sulfamethoxazole , gentamicin and ciprofloxacin while susceptible to vancomycin , clindamycin , tertracyline , linezolid , quinupristin / dalfopristin , nitrofurantoin , and rifampicin as determined by vitek 2 system . vancomycin ( 1 g / ml ) and daptomycin ( 0.94 g / ml ) susceptibility was confirmed by etest ( biomerieux , inc . , the isolate was intermediately sensitive to erythromycin ( 1 g / ml ) and a d - test for inducible clindamycin resistance was found to be negative . the mrsa isolate from blood and pericardial fluid were identical and identification was confirmed by amplifying the 16s rdna gene of the genus staphylococcus and sau gene specific for s. aureus . a multiplex polymerase chain reaction ( pcr ) was done to simultaneously detect genes responsible for resistance to four classes of antibiotics and the isolate was positive for aac a - aph d , erm ( c ) and meca encoding for resistance to aminoglycoside , erythromycin and oxacillin respectively . the organism was susceptible to tetracycline and , therefore , tetk and tetm genes were not amplified . the patient who was empirically put on piperacillin / tazobactum was shifted to vancomycin when cultures identified the isolate as mrsa on the third day . subsequently he developed septic shock and metabolic acidosis and expired on the fourth day of admission due to heart failure . purulent pericarditis historically a disease of children and young adults has been a rare entity in the modern antibiotic era . since 1945 , the median age at the time of diagnosis has increased from 21 to 49 years . the predisposing factors in the post antibiotic era were found to be an underlying non - infectious condition like thoracic surgery or chronic renal disease . pericardial diseases are more common in patients with renal disease and frequency has declined from 20% , which was 35 years ago , to 5% presently . in most of these cases pericardial fluid did not show any evidence of infection , making purulent pericarditis a rare entity . due to the absence of typical pericarditis features diagnosis of purulent pericarditis these patients typically present with an acute illness characterized by fever , chills , and tachycardia . pericardial rub and chest pain are frequently not present and ecg may be normal in 35% of cases . primary site of infection is rarely pericardium and four pathways have been described for its spread to the pericardium namely direct extension of an intrathoracic process , penetrating injury to the chest wall , local extension and hematogenous spread . purulent pericarditis following percutaneous procedures is being increasingly reported and therefore these nosocomial infections are more likely to be caused by multi - drug resistant ( mdr ) pathogens . though the introduction of antibiotics has drastically reduced the incidence of purulent pericarditis in the last few decades , it is slowly reemerging due to the increased prevalence of mdr pathogens . s. aureus is the most common cause of purulent pericarditis , and therefore reports of mrsa purulent pericarditis are on the rise . even though there are potent antibiotics to treat mrsa a delay in diagnosis often leads to high mortality . our case highlights one such case of fatal purulent pericarditis caused by and mdr mrsa where early diagnosis and appropriate empirical antibiotic therapy could have resulted in a favorable outcome . we suggest that acute purulent pericarditis with mdr mrsa should be considered in patients with renal disease .

though pericardial disease is common in patients with renal disease , purulent pericarditis is very rare . we report a fatal case of purulent pericarditis and sepsis due to methicillin - resistant staphylococcus aureus in a 78-year - old male with systemic hypertension and renal disease along with the molecular characterization of its resistant mechanism .

the presence of postoperative seromadesis is common , corresponding to the presence of serum in the subcutaneous tissue post a surgical event . we present a case of a 63-year - old woman having undergone femoral prosthesis surgery and total hip replacement with a subfacial seroma without findings of infection , refractory to standard treatment of compression bandages , massage and cleaning surgery in two oportunities . a literature review was undertaken to obtain the therapeutic alternatives where erythromycin seromadesis is chosen with excellent response . erythromycin sclerotherapy should be considered as an effective and safe option in the treatment of seroma in general surgery and traumatology . we believe that this is the first study of use of erythromycin as sclerotherapy in a traumatology case . a seroma is the collection of fat , liquid , serum and lymph in one area of the body following a traumatic or surgical event . usually , it is formed in a wound in the immediate postoperative period , usually in the first week , although it may be delayed . generally it is benign , but often annoying to the patient and has a moderate risk of infection . the presence of a seroma may be asymptomatic , or may have swelling , pain and exude from the wound . the diagnosis can be by inspection and palpation of the wound , with computed tomography and the ultrasound beign useful for identifying fluid collection . seroma s have been seen in orthopaedic surgery as well , where they are likely to have serious repercussions . this is true especially in prosthetic surgery , with greater chances of infection of prosthetic components and failure of the joint replacement associated with high costs , lengthy hospitalizations , and at times need for a subsequent surgery . treatment options can be categorized as preventive or curative , xonsisting of intraoperative application of sclerosing chemicals as hypertonicsaline and talc in humans and gram positive anaerobe like corynebacterium parvum in rat models , tissue adhesives ( fibrin or platelet concentrates ) , use of negative pressure drainage , repeated percutaneous aspiration and surgical drainage with complete removal of seroma and its capsule . the use of erythromycin as a sclerosing agent was described in animal studies , that was first assessed as a pleural sclerosant , proving effective and with fewer sideeffects than talc , tetracycline and diazepam . this is used safely in humans in the management of breast surgeries , non neoplastic ovaran cysts and pleurodesis . there are no reports of its use in orthopedics , and we believe that this is the first study of this kind . a female patient of 63-year - old presented to our orthopaedic department with prosthetic loosening of the isoelastic stem ( fig . 1 ) , also femur and acetabulum with massive osteolytic defect performed by modular aloprosthesis of proximal femur and acetabulum of tantalum , and metal on polyethylene pair of friction ( fig . patient had good clinical evolution with the drain removed at the first postoperative day with output of 100 cc . five days later , patient was re - admitted to the emergency room for increased volume in relation to wound without infectious signs . 3 ) . cemented total hip arthroplasty left hip , with extensive osteolysis in bone cement interface . . soft tissue ultrasonography , in hypodermic one hypoechoic collection of 10.5 4.0 4.6 cm . to avoid superinfection of a large extent surgery , it was decided to make an early cleaning surgery , in order to drain and take tissue samples and fluid culture . a collection is located under muscle planes adjacent to the aloprotesis , with communication between up fascia lata muscleplane . after the cleaning surgery and drainage , equally the seroma is maintained for 5 days with daily output of 200 cc of serous fluid . it was decided to perform a new scouring , with obliteration of third space , massage and compression bandages . negative cultures were obtained . given the persistence of seroma , a literature review was undertaken to obtain therapeutic alternatives . finally , surgical cleanliness was performed with resection of pseudocapsule seroma with curettage , making crops and application of 2 g. erythromycin diluted in 200 cc.of 0.9% saline solution . customary closing was performed flat leaving a aspiration drainage n 9 undert he fascia , which was measured daily . the next day , the drainage measured serohematic liquid 100cc , and 60cc was measured on the 2nd day . on the 3rd day , 10 cc of liquid was measured , and the drainage was removed on the 4th day . subsequently , the patient was discharged in good condition without signs of infection , tension , edema or collection in the thigh wound . it has been assessed that there are certain factors that could favor the formation of seroma , such as a high bodymass index , use of electrocautery to dissect , early withdrawal of drain ( within 24 hours ) , obliteration of dead space and use of drains under vacuum . in our case , it is highlighted that the drainage was removed on the first postoperative day , which could have favored the formation of seroma . although , maintaining a drainage for more number of days also increases the risk ofinfection . it is important to consider the differential diagnosis of surgical wound infection , hence blood inflammatory parameters were evaluated , and fluid cultures of the wound were taken . if an infection is confirmed , systemic antibiotic treatment should be given . in addition , open the wound , drain the liquid and perform irrigation and debridement folllowed by wound closure . in the search for bibliographical studies , with terms mesh in pubmed for seroma and erythromycin a single study was located , ali - khan et al . 2009 , which refers to the use of erythromycin as sclerotherapy in the management of seroma in series of patients of plastic surgery , showing effectiveness and no serious complications . the search for terms mesh in pubmed for seroma and sclerotherapy , located two investigations , throckmorton et al . in 2008 with povidoneiodine or ethyl alcohol in postmastectomy and moritz et al . in 2013 with polidocanol in varicose vein surgery . the current evidence allows the use of this therapy in this patient , although there is no evidence in trauma surgery . it is necessary to conduct more studies to evaluate the effectiveness and reproducibility of this therapy . it is known that erythromycin as sclerosing product tends to be very effective with fewer adverse effects than other compounds used for the same condition . it has evaluated with satisfactory results in pleurodesis , non - neoplastic adnexalcyst , and plastic surgery . erythromycin use as sclerosing product in orthopedic surgery must be evaluated as a effective and safe therapeutic alternative .

introduction : the presence of postoperative seromadesis is common , corresponding to the presence of serum in the subcutaneous tissue post a surgical event . erythromycin has been reported as sclerosing , although not in orthopedic surgery . we report a case of erythromycin seromadesis in orthopedic surgery.case presentation : we present a case of a 63-year - old woman having undergone femoral prosthesis surgery and total hip replacement with a subfacial seroma without findings of infection , refractory to standard treatment of compression bandages , massage and cleaning surgery in two oportunities . a literature review was undertaken to obtain the therapeutic alternatives where erythromycin seromadesis is chosen with excellent response.conclusion:erythromycin sclerotherapy should be considered as an effective and safe option in the treatment of seroma in general surgery and traumatology . more studies are necessary to get a better evidence . we believe that this is the first study of use of erythromycin as sclerotherapy in a traumatology case .

a previously well 59-year - old lady presented to her gastroenterologist with per rectal bleeding . apart from intermittent inhaled salbutamol for asthma a staging ct of chest / abdomen / pelvis revealed no obvious distal metastatic disease . both lymphatic and venous invasion were noted ; 17 of 18 regional lymph nodes including the apical gland contained metastatic tumour . she received adjuvant folfox-6 ( fluorouracil , oxaliplatin , bolus fluorouracil , and leucovorin ) for six months . repeat ct scan at end of chemotherapy ( january 2007 ) demonstrated two 5 mm lesions in the left lower lobe and right middle lobe of the lung . while metastatic disease could not be ruled out , the lesions were thought to be granulomatous in nature . two low attenuation areas were identified in the liver posteromedially , measuring 8 mm and 15 mm in diameter . a para - aortic lymph node measured 6 mm . repeated 4 months later , a new lobulated 5 mm nodule was noted in periphery of right upper lobe along with more extensive para - aortic and left common iliac lymphadenopathy , suspicious of recurrent disease . pet scanning demonstrated fdg avid disease involving lymph node groups both above and below the diaphragm , and in particular the left para - aortic chain . ct confirmed extensive left para - aortic lymphadenopathy with more focal nodal masses posterior to left renal vein and at common iliac bifurcation . a needle biopsy of the para - aortic lymph node confirmed adenocarcinoma consistent with prior colonic primary . she received a further 6-month course of folfox-6 from 3/3/2008 to 6/8/2008 ; again achieving a good partial response with resolution of the adenopathy and stable disease in the other measurable lesions . in november 2008 the oedema progressed and spread to the thigh , with left iliac fossa tenderness felt at the same time . ct showed no evidence of recurrence at surgical site , as well as decreased size of both right middle lobe lung lesion and left external iliac lymph nodes . the malignant cells were positive for ck20 and negative for ck7 , supporting diagnosis of metastatic colonic carcinoma . p16 was negative and cea / mucin stains were difficult to interpret due to degenerative changes . . per vaginal examination showed the uterus to have a firm and nodular consistency . along with the pv bleeding , the patient reported right upper back pain in a radicular pattern from the lower cervical spine to the right proximal humerus . ct on same day showed a destructive lesion in c7 vertebral body on right side with pathological fracturing . whole body bone scan with spect 19/2/10 showed intense abnormality in body c7 consistent with a metastatic lesion . mri of the spine 19/2/10 showed pathological superior endplate fracture associated with metastatic deposit in the c7 vertebral body . marked right - sided c8/t1 foraminal stenosis was noted with possible right c7 radicular impingement . the patient commenced palliative radiotherapy to the cervical spine in 10 fractions of 30 gy . a dermatitic like eruption on left upper / inner thigh and buttock was noted in april 2010 , which did not respond to treatment with topical steroids ( see figures 2 and 3 ) . she was reviewed by a dermatologist and punch biopsy of left upper thigh was carried out on 28/4/10 . this showed multiple micronodules pleomorphic cells in superficial and mid dermis , showing focal ductular differentiation . she had 3 cycles of folfox , however , this was poorly tolerated due to anaphylactic reactions . colorectal cancer is the second most prevalent malignancy in australia ( excluding nonmelanocytic skin cancers ) . the most common metastatic sites are the regional lymph nodes , liver , lungs , and peritoneum . these include local invasion causing malignant fistula formation into adjacent organs , such as bladder or small bowel . fever of unknown origin , intra - abdominal , retroperitoneal , or abdominal wall abscesses can often be the first manifestation . these presentations are very rare individually , however , there has been no previous case report of a patient with both endometrial and cutaneous features . brand et al . reported a series of 6 patients presenting with per vaginal bleeding in the setting of recurrent colon cancer . only one of the patients in that series received adjuvant chemotherapy prior to per vaginal bleeding . despite aggressive chemo - radiation or surgical management , all patients had recurrence of colonic carcinoma . hu et al . found 124 cutaneous metastases from a series of 12.146 patients with internal malignancies ( 1% ) . 16 of these cases originated from colorectal cancer , equating to a rate of cutaneous metastasis of 0.81% . other authors have found rates of cutaneous metastasis between 2.3 and 6% [ 68 ] . skin metastases from the breast usually occur on the chest , the lung to the chest and upper extremities , and the gastrointestinal tract to the abdomen . these metastases usually manifest as a rapidly growing , mobile nodule . on immunohistochemical staining , ck20 positivity often correlates with colorectal tumours . this case highlights the importance of paying close attention to symptoms and signs in patients with a past history of internal malignancy . it is important to maintain a high index of suspicion to clinical features that may represent a metastatic process . furthermore , tissue diagnosis to confirm the aetiology of a symptom can prove very useful , as shown in this case .

this case highlights two very rare complications of metastatic colorectal carcinoma . it describes a 59 year old female with both cutaneous and endometrial metastases from colorectal carcinoma . while both of these presentations are very unusual , they highlight the need to be vigilant about the detection of metastatic complications during follow up .

according to popular studies , the attempted resuscitation of patients in traumatic cardiac arrest in the prehospital care environment should not be pursued due to undo risk to the provider and has been shown to be ineffective . however , after review of current literature , sherren and colleagues developed and published in a recent edition of critical care a detailed treatment algorithm for their helicopter emergency medical service ( hems ) that encourages aggressive resuscitation for patients in traumatic cardiac arrest ( tca ) . what is known in a review of the quoted literature is that in the prehospital care environment , the survival rate of tca patients is 0 to 3.7% , but in newer published studies survivability has risen to 7.5% . what we already know is that prehospital medical cardiac arrest ( mca ) survival is approximately 9.8% , with in - hospital cardiac arrest survival at 24.2% . in mca , chest compressions , defibrillation , medication , and oxygenation are the mainstay treatment tools . where the tca patient differs is that many do not have extensive co - morbidities or coronary artery disease ; their arrest is primarily due to one of or a combination of factors : hypovolemia , obstruction of blood flow and hypoxia . in the patient with thoracic trauma , these causes are addressed by well described techniques of thoracostomies , endotracheal intubation and blood products . this is followed by a clamshell thoracotomy , allowing for a better chance for hemorrhage control and treatment of hemopericardium if found . sherren and colleagues discuss a very well outlined pathophysiology and rationale for tca survivorship in their article . although mca and tca are not the same diseases , their survival statistics are interesting and possibly some inference can be made when mca data are more closely observed . it appears that the differences between mca survival in the prehospital versus a hospital setting , where admitted patients are older with increased morbidities , might be due to the personnel performing the resuscitation . staffing models for emergency medical services ( ems ) , especially for hems , differ between the us system and the european model . in the us , pre - hospital ems systems ( including most hems ) are staffed by allied health professionals and not by physicians . in the european model ( including australia ) hems are staffed by well trained physician / paramedic / nurse teams and this is possibly where the difference occurs for survival for tca patients . in multiple european studies it was noted that there was a decrease in mortality of trauma patients without a decrease in scene time when a physician was part of the flight crew [ 6 - 9 ] . in the us , less than 5% of hems are staffed by physicians , and most of those involved in such teams are in their first few years out of medical school . thus , no us ems systems have fully trained physicians as part of their standard ambulance crew . a reasonable question to consider is whether staffing is the reason why tca statistics differ between the us and european models ? paramedic training teaches needle thoracostomy for suspected tension pneumothorax , which in many jurisdictions do not need online direct physician permission as it is deemed life saving and with limited risk . however , it is well known that in patients with larger chest walls , almost 50% of the time the needle fails to reach the plural space to relieve the obstruction of blood flow caused by the tension pneumothorax . on the contrary , the placement of a tube or finger thoracostomy are very successful and fairly routine in ems systems staffed with experienced physicians with excellent results . it can not be fully applicable to hems not staffed by a well trained physician , as the proficiency of thoracotomy is an advanced skill that takes practice and years of anatomical training to be able to perform well . as the authors stated , more study is needed to see if it is the depth of training or the protocols that are the reason for the increases in survivorship from tca . i do not believe that this algorithm will be promoted in hems systems staffed only by paramedics and nurses , as their level of training does not allow for advanced procedures such as these . therefore , one should expect that prehospital attempts at the resuscitation of the tca in those systems without physicians on board will remain futile . ems : emergency medical services ; hems : helicopter emergency medical service ; mca : medical cardiac arrest ; tca : traumatic cardiac arrest .

traumatic cardiac arrest resuscitation is considered a heroic and futile endeavor . however , newer articles have more promising statistics and divide between prehospital ground and helicopter transport . here we discuss why there might be a difference in the survivability of this subset of trauma patients .

anesthesiologists can come across difficult airway in patients for elective or emergency surgery in the operating room ( or ) or outside the or . airway and ventilatory procedures outside the or are associated with a higher incidence of adverse events and a higher risk of mortality than similar events in the or . morbidly obese patients are at risk of difficult mask ventilation as well as intubation , and airway management is a major factor underlying morbidity and mortality related to anesthesia in such patients . the intubating laryngeal mask airway ( ilma ) may provide better conditions for achieving effective ventilation and tracheal intubation in such patients . a 36-yr - old male presented to the surgery emergency after blunt trauma chest with chief complaints of chest pain , more on the left side , and difficulty in breathing . there was no history of vomiting , loss of consciousness , pain in neck or any bleeding from the ear , nose or throat . on examination , the patient was morbidly obese ( body mass index 45 kg / m ) , drowsy but arousable on verbal commands with glasgow coma score 13/15 . pulse oximetry showed oxygen saturation of 60% on room air and 80% on ventimask with fio2 0.5 . chest examination revealed bony crepitus on the left side and subcutaneous emphysema all over the chest , extending up to the infraclavicular area . arterial blood gas analysis ( on ventimask ) revealed ph 7.28 , pco2 38 , po2 50 , hco3 18.1 and so2 80% . chest x - ray showed bilateral multiple rib fractures and bilateral pneumothorax with left lower zone collapse . the patient had persistent hypoxemia despite bilateral intercostal drainage being in situ , and was shifted to the intensive care unit for mechanical ventilation . quick airway examination revealed mouth opening of 2.5 cm , large tongue , heavy jaw , short and thick neck , thyromental distance less than three finger breadths and mallampati score 4 . difficulty in face mask ventilation as well as tracheal intubation was anticipated and , therefore , awake intubation was planned . difficult airway cart was prepared , but fiberoptic bronchoscope was not available and tracheostomy was expected to be difficult . after explaining the procedure to the patient , xylometazoline nasal drops were instilled into both nostrils and a prelubricated nasopharyngeal airway was gently inserted , through which 100% oxygen supplementation was carried out by bain circuit and movements of the reservoir bag with respiration observed . bilateral superior laryngeal nerve block and transtracheal block were given with 2 ml of 2% lignocaine each using an 18 g intravenous ( iv ) cannula . the cannula sheath was left in situ to provide translaryngeal jet ventilation if needed and 10% lignocaine was sprayed over the tongue and oropharynx . direct laryngoscopy was attempted , which revealed cormack lehane ( cl ) grade iv and an intubation attempt was unsuccessful . we then attempted blind nasal intubation , but 6.5 mm i d endotracheal tube ( ett ) could not be negotiated through the nasal cavity . subsequently , we gently inserted a size 4 ilma and the patient tolerated it well . the cuff was inflated with 30 ml air and placement confirmed by movements in reservoir bag and capnography . a well - lubricated 8 mm i d ett was passed , through the ilma , without any difficulty . after confirming the placement by auscultation and capnography , ilma was gently removed while pushing the proximal end of the tracheal tube with the help of the stabilizing rod . the patient was sedated with iv midazolam 2 mg , propofol 100 mg and morphine 6 mg . neuromuscular blockade was achieved with vecuronium 7 mg iv and controlled ventilation was carried out . the patient remained on mechanical ventilation for 7 days , following which tracheotomy was performed . he was sedated with midazolam and put on synchronous intermittent mandatory ventilation and was gradually weaned off from the ventilator after 21 days [ figure 1 ] . morbidly obese patient with endotracheal tube in place the use of ilma to achieve adequate oxygenation / ventilation and provide a conduit for intubation in difficult to manage airways has been proven . according to the american society of anesthesiologists 2003 difficult airway guidelines , awake intubation is the gold standard in a difficult airway patient after trauma if the patient is cooperative . . demonstrated , in a study on 118 patients scheduled for bariatric surgery , that ilma is an effective and safe ventilatory device and blind intubation guide in morbidly obese patients . the rate of successful tracheal intubation was 96.3% and the success rate was not different among patients with low - grade ( cl - i , ii ) and high - grade ( cl iii , iv ) views . awake intubation in the emergency department using ilma has earlier been reported in a morbidly obese patient with acute respiratory failure . however , ilma usage in difficult airways is limited in certain situations such as mouth opening less than 1.5 cm , increased risk of aspiration and suspected or known abnormalities in the supraglottic anatomy . position , bullard laryngoscope or awake fiberoptic intubation can be used in obese patients at high risk of difficult tracheal intubation . other options in morbid obesity include the short handle laryngoscope , video laryngoscopic - guided intubations and lma c - trach , but they may not be available in an emergency . ilma is more efficient in the morbidly obese patients than in normal patients , with fewer airway adjustments needed for establishing ventilation . the successful airway management of this morbidly obese patient with chest trauma suggests that use of ilma may provide a viable and safe option for awake endotracheal intubation outside the or . we also suggest that the ilma can be well tolerated in minimally sedated patients who are breathing spontaneously and who have been appropriately prepared for this method of tracheal intubation .

a morbidly obese male who sustained blunt trauma chest with bilateral pneumothorax was referred to the intensive care unit for management of his condition . problems encountered in managing the patient were gradually increasing hypoxemia ( chest trauma with multiple rib fractures with lung contusions ) and difficult mask ventilation and intubation ( morbid obesity , heavy jaw , short and thick neck ) . we performed awake endotracheal intubation using an intubating laryngeal mask airway ( ilma ) size 4 and provided mechanical ventilation to the patient . this report suggests that ilma can be very useful in the management of difficult airway outside the operating room and can help in preventing adverse events in an emergency setting .

in this retrospective , single center study , we reviewed charts from 68 patients with ana comprehensive panels . inclusion criteria were patients 18 years old and had an ana cp billing code between may 2015 and october 2015 . variables included age , sex , specialty of the ordering physician , test indication , and ana result . our institutional review board determined this study was non - human subjects research ; institutional approval was not required . the mean age of the sample was 54.419.4 years old , and 60.3% were female . internal medicine ordered the majority of ana cps ( 83.8% ) followed by family medicine ( 7.4% ) , emergency medicine ( 2.2% ) , and psychiatry ( 2.2% ) . hypercoagulable work up , transaminasemia and skin rash were the most frequent indications for ordering the ana cp ( 8.8% for each indication ) . the remaining indications ( 73.6% ) covered a broad spectrum and combined as an other category . all the ana cps ordered were considered to be inappropriate including the three patients who had previous history of rheumatological disease and did not require re - testing . sixty - three ana comprehensive panels were negative for rheumatological disease ( 92.6% , fig . multiplex immunoassays have led to a paradigm shift in the methodological testing of autoimmune diseases . high throughput multiplex immunoassays have supplanted the use of traditional methods like indirect immunofluorescence ( iif ) and elisa . iif testing is subject to poor specificity , has a high false positive rate , lack of standardization in substrate and dilution protocols , and interobserver variability in pattern interpretation ( 2 ) . enzyme immunoassays ( eia ) testing removes the subjective variations of iif testing ( 3 ) ; however , there exist interlaboratory method variations and heterophile antibody interferences causing false - positive results . the correlation between elisa and mutiplex assays is high , with a 90% concordance ( 4 ) . ana testing with multiplexed microsphere fluorescence allows for rapid quantification and efficient profiling of multiple clinically significant antibodies in a single run of assay ( 5 ) . the multiplex ana screen is a composite screen which tests for 11 specific autoantibodies that are known to be associated with autoimmune diseases . if none of the specific antibodies are present , the ana screen is reported as negative . positive screens are reflexed , and the reflexed antibodies are resulted semi - quantitatively as numeric antibody indices ( ai ) ( 5 ) . the authors identified that the major reason behind inappropriate ana comprehensive panel ordering could be due to physician unawareness regarding test components performed under the order panel . ana testing by mutiplex assays should be performed in a bi - leveled sequence with an initial screen followed by comprehensive antibody testing only if ana screen returns positive . ana comprehensive panel is not a reflex laboratory test and examines directly for specific antibodies without going through an initial screen . comprehensive antibody testing without an initial screen leads to unnecessary cost burden on the patient and a waste of laboratory resources . in our study , net calculated value of incurred losses resulting from comprehensive lab order ( ana cp ) testing amounted to $ 66,000 over a 6-month study period . the ana comprehensive panel does not provide any further utility over the ana screen with reflex. hence , the ideal test of choice should be ana screen with reflex. also , the term ana comprehensive panel gives the physicians a sense of it being a more complete test over the ana screen , thus leading to inappropriate use of the second - level testing without going through the initial screen . over the last few years , ana comprehensive panels have emerged as a very convenient test with fewer false positives and the convenience of being completed in a single run , resulting in the ordering physician choosing it over the appropriate ana screen . it is important to recognize that autoantibody testing is fraught with low specificity and false - positive results , leading to potential over - diagnosis and mistreatment . in fact , false positives occur in a significant proportion of healthy population ( 6 ) . ana testing in the wrong clinical setting , especially in the absence of specific signs and symptoms suggestive of rheumatic diseases can potentially lead to misdiagnosis and unnecessary rheumatology consultations . also , repeat ana testing is not warranted in patients with established rheumatological disease ( as noted in 3 of 68 in our study patients ) except to monitor disease activity , in which case , repeating specific antibody titers may be considered and not the ana comprehensive panel. a retrospective study on patients who had ana testing suggested that a large proportion ( 30% ) of them had non - specific and unrelated signs and symptoms ( 7 ) . incorporation of ana testing into initial lab order bundles in an effort to expedite diagnoses ; overestimation of the pretest probabilities ; failure to consider non - rheumatological factors expected to cause false - positive results contribute to ana testing among physicians . one of the limitations of our study was that it was a single institutional experience . we initially conducted this as a pilot study and based on the results in our center , we intend to conduct the study across all advocate healthcare hospitals . ana testing with multiplex immunoassay systems allows for rapid profiling of multiple analytes in a single run of assay and have few false - positive results compared to conventional lab methods . however , it is important to educate physicians on following a sequenced diagnostic algorithm based on the guidelines laid down by american college of rheumatology to avoid unnecessary higher level testing and increase cost burden on their patients . also , inappropriate ana testing without adequate pretest probability estimation after careful history taking and clinical examination can lead to over - diagnosis with unnecessary consultations and should be avoided . the authors have not received any funding or benefits from industry or elsewhere to conduct this study

healthcare providers use antinuclear antibodies ( anas ) to screen and diagnose patients with autoimmune diseases . in the recent years , commercial multiplex ana kits have emerged as a convenient and fast diagnostic method . diagnostic testing should follow sequenced algorithms : initial screen followed by specific antibody analysis . second - level testing as an initial screen for autoimmune disease is inappropriate . we reviewed 68 patients with ana comprehensive panels over a 6-month period from may 2015 to october 2015 . we assessed appropriateness and estimated incurred losses from inappropriate testing . we found 92.6% ( 63 out of 68 ) of the ana comprehensive panel results to be negative . incurred losses from inappropriate ana comprehensive panel testing were $ 66,000 . physicians should become familiar with ana - sequenced diagnostic algorithms to avoid unnecessary higher level testing .

congenital heart disease encompasses a variety of lesions that may include communication / s between the left and the right side of the heart . such communications may cause volume overload in the short and medium term , possibly resulting in heart failure , or irreversible complications in the long term such as eisenmenger 's syndrome . previously , such defects ( typically atrial ( asd ) and ventricular septal defects ( vsd ) and patent ductus arteriosus ( pda ) ) were closed surgically . over the last decade , a vast variety of devices have been developed to close such defects through the transcatheter route . we report one patient who had self - limiting haemolysis after implantation of an amplatzer perimembranous vsd device . our patient was born via normal vaginal delivery at 38 weeks gestation after an uneventful preganacy . at the routine 6 week visit , she was noted to be failing to thrive and a 3/6 systolic murmur was noted . diuretics were commenced and she throve . at 4 years of age , she was admitted to the paediatric surgical ward with fever , abdominal pain and vomiting . surgical exploration of the abdomen under antibiotic cover was normal , as was an echocardiogram at this time . fever persisted and a repeat echocardiogram showed tricuspid valve endocarditis with a large vegetation that eventually eroded part of the valve resulting in significant regurgitation . the endocarditis was treated with antibiotics and transcatheter closure of the vsd was done successfully using a 14 mm amplatzer perimembranous vsd device at 5 years of age . one week after discharge , she was readmitted due to jaundice and dark coloured urine . haemoglobin was 9.5g / dl , liver function tests were deranged ( bilirubin 80umol / l ; direct bilirubin 12umol / l ; gamma gt 80 u / l ; alt 58 u / l ; alkaline phosphatase 707 u / l , with urobilinogen in the urine . viral studies , inclujding cmv , ebv , hepatitis a and b , were all negative . she was discharged home as after two weeks as her jaundice cleared and her liver function tests improved . haemolysis has been documented after amplatzer device closure of pda,1 asd,2 and vsd.3 the one reported case after vsd closure resulted in transient renal failure.3 hemolysis has also been associated with the use of amplatzer devices to close paravalvar mitral valve leaks after mitral valve replacement.4 conservative treatment is usually sufficient but reintervention of some form may occasionally be necessary , such as intradevice coil deployment in order to completely eliminate any degree of residual left to right shunting.5

over the last few years , a vast variety of devices have been developed to close various septal defects through the transcatheter route . haemolysis has been documented after amplatzer device closure of patent ductus arteriousus , atrial septal defect , and ventricular septal defect . we report one patient with self - limiting haemolysis after implantation of an amplatzer perimembranous vsd device .

canaliculitis is a common encounter in ophthalmic practice but supernumerary puncta and canaliculi ( spc ) are rare congenital disorders . in a large series a 59-year - old gentleman presented with painful swelling of the left lower lid for a week , which was associated with epiphora . the swelling was confined to the nasal aspect of the left lower lid ( 0.50.5 mm ) with inflamed overlying skin ( figure 1a ) . eversion of the lower eyelid revealed two puncta , 0.5 mm apart ( figure 1b ) . the outer punctum was situated at the normal anatomical position ; whereas the inner punctum in the caruncle . gentle pressure did not result in any regurgitation from the both puncta . the patient was treated with oral cloxacillin 500 mg , 6 hourly for 5 days . the outer punctum had a soft stop with regurgitation of fluid from the same punctum . the outer punctum - canaliculus system was a cul - de - sac ( figure 1c ) . c ) dacryocystography showed pooling of dye in the cul - de - sac ( white arrow ) . c ) dacryocystography showed pooling of dye in the cul - de - sac ( white arrow ) . most spcs ( 78% ) present with epiphora . among the 23 patients reported by satchi et al . , none presented with canaliculitis . sequestration of tear and debris in the cul - de - sac served as nidus for infection . the resultant canaliculitis with its surrounding edema caused obstruction of the lacrimal drainage ; hence epiphora . epiphora however , may develop despite patent lacrimal drainage system . the 2-compartment model for lacrimal canalicular drainage of kakizaki et al . , suggested that the muscle of duverney - horner may deviate normal flow within the accessory canaliculus and thence transport tears back to the lacrimal tear lake , leading to epiphora . a solid epithelial cord forms in the region of the medial lower eyelid ( figure 2a ) and sends projections to form the canaliculi and the nasolacrimal duct ( figure 2b ) . spc is due to extra out - budding of the solid epithelial cord ( figure 2c ) . canalization begins at 4 months of gestation with disintegration of the central ectodermal core , forming lacrimal drainage outflow system . in this case , the extra inner canalicular epithelial bud ( nearer to the main epithelial cord ) underwent complete canalization and remained connected to the main epithelial cord . the outer canalicular epithelial bud , although its punctum is located at the normal anatomical position , was separated from the main epithelial cord ; forming a cul - de - sac ( figure 2d ) . c ) extra out - budding of the solid epithelial cord in supernumerary puncta and canaliculi . d ) the outer canalicular epithelial cord was separated from the main epithelial cord , forming a cul - de - sac . c ) extra out - budding of the solid epithelial cord in supernumerary puncta and canaliculi . d ) the outer canalicular epithelial cord was separated from the main epithelial cord , forming a cul - de - sac .

we report the first case of supernumerary puncta and canaliculi presented with canaliculitis . a-59 year - old gentleman presented with painful swelling of the left lower lid for a week , which was associated with epiphora . the swelling was confined to the nasal aspect of the left lower lid ( 0.50.5 mm ) with inflamed overlying skin . two puncta ( 0.5 mm apart ) were noted . the outer punctum at the normal anatomical position was a cul - de - sac while the inner punctum it the caruncle was patent . we described the embryology leading to supernumerary puncta and canaliculi to explain the paradoxical patency of the abnormally located punctum as well as the pathomechanism leading to canaliculitis . the patient was treated with oral cloxacillin 500 mg , 6 hourly for 5 days ; the cellulitis subsided after three days .

they have traditionally been considered benign , but recent evidence indicates that frequent apbs are a strong predictor of atrial fibrillation development and may be associated with increased risk of cardiovascular death in general population . in addition , frequent apbs may cause cardiomyopathy . her heart was structurally normal heart and there was no evidence of any sustained tachyarrhythmias . to our knowledge , this is the first report on successful ablation of frequent apbs in the non - coronary aortic cusp . a 59-year - old female with a long history of highly symptomatic frequent apbs presented to our hospital for catheter ablation . p wave was negative in inferior leads ii , iii , avf , positive in avr and v1v3 , indifferent in lead i , avl and v4v6 ( fig . 1 ) . apb burden in repeated holter recordings was 2040% ( 29000 apbs in the last holter recording ) . neither atrial fibrillation nor any other sustained supraventricular tachyarrhythmia was detected . clinical examination and thyroid function were normal . diagnostic catheters were placed in the coronary sinus and right ventricular apex vie right femoral vein . mapping and ablation was performed retrogradely via right femoral artery with a 3.5 mm tip open irrigated ablation catheter ( biosense webster navistar rmt , diamond bar , ca , usa ) using remote magnetic navigation ( epoch , stereotaxis inc . , st louis , mo , usa ) and 3d electroanatomical mapping system ( carto3 , biosense webster ) . mapping was started from the right atrium because of the p wave morphology and coronary sinus activation sequence . fast anatomical activation mapping demonstrated that the earliest activation in the right atrium was in the vicinity of the his bundle ( fig . 2 ) . no ablation attempt was made although local activation at this site preceded the p wave because of high risk of damage to the atrioventricular conduction system . meticulous mapping at the aortic root demonstrated earliest local activation of the clinical apb in the non - coronary sinus of valsalva anterior and superior to the his bundle . at this site no his potential was detected , and the local electrogram was earlier than in the para - hisian area in right atrium and preceded the p wave by about 50 ms ( fig . 3 ) . radiofrequency energy delivery at the non - coronary aortic cusp resulted in immediate cessation of the apbs . no junctional beats or pr prolongation were observed during ablation ( 120 s with maximum power of 35 w ) . total duration of the procedure was 110 min and fluoroscopy exposure 2 min 35 s ( 3.0 gy / cm2 ) , respectively . at discharge a routine holter recording was scheduled at 3 months and the patient was advised to contact her physician for ecg monitoring if any arrhythmia symptoms recurred . during the follow - up of 12 months the patient reported no recurrence of symptoms and there were no clinically relevant apbs in the holter recording . atrial premature beats are present in 1020% of the general population . in most cases they are benign , but frequent apbs have been associated with development of atrial fibrillation and cardiomyopathy as well as with increased risk of cardiovascular mortality and stroke , , . in our case no sustained atrial tachyarrhythmias were documented , and left ventricular function was normal despite long history of frequent apbs . nevertheless , it is possible that the high apb burden could have caused atrial fibrillation and/or apb - induced cardiomyopathy if not treated . the majority of apbs arise from within and around the pulmonary veins ( pv ) . other sites of origin include left atrial posterior wall , ligament of marshall , coronary sinus , superior and inferior vena cava , crista terminals and tricuspid annulus . catheter ablation has become the treatment of choice in patients with various paroxysmal supraventricular tachycardias and ventricular premature beats , but it is used quite rarely to eliminate isolated apbs . the frequently multiple sites of origin and capricious manifestation have made it difficult to ablate extrapulmonary abps . however , in patients with frequent monomorphic apbs the focus can usually be identified by careful activation mapping and treated by ablation . to the best of our knowledge this is the first report on successful ablation of frequent apbs in the non - coronary aortic cusp . based on our experience the main benefit of using magnetic navigation in cases like this is that mapping with a softer and more flexible magnetic catheter is less likely to cause perforation and to provoke catheter - induced extrasystoles than mapping with a manually steered ablation catheter . in addition , the magnetic technology has proven to reduce personnel and patient radiation exposure . it offers important safety benefit by reducing the risk of perforation but also makes creation of transmural lesions difficult in some areas ( e.g. , cavo - tricuspid isthmus ) . the p wave morphology and right atrial mapping demonstrated that the apbs originated from a site close to the his bundle . we and others have previously shown that sustained atrial tachycardia in this area can often be effectively and safely ablated within the non - coronary aortic cusp which is anatomically in close proximity to the interatrial septum and his bundle , . in the current case , the local atrial activation at the non - coronary aortic cusp was about 20 ms earlier than that recorded at the his region in the right atrium . at the ablation site no his potential was visible , and the catheter was stable and far from the coronary artery ostia . this case demonstrated that in addition to focal atrial tachyarrhythmias and ventricular premature beats aortic root can be a source for frequent apbs . due to the close anatomic relationship between the atrioventricular node and the non - coronary aortic cusp mapping of the aortic root and non - coronary cusp

a 59-year - old female with structurally normal heart was admitted to our hospital for treatment of highly symptomatic , drug refractory atrial premature beats ( apb ) . ecg revealed atrial parasystolic trigeminy . the arrhythmogenic focus was mapped and ablated using magnetic remote navigation and 3d electroanatomical mapping system . to our knowledge , this is the first report on successful ablation of frequent apbs in the non - coronary aortic cusp .

fibrous dysplasia ( fd ) of the bone is a slowly progressive bone disorder on which normal bone is replaced by abnormal fibro - osseous tissue . the true incidence and prevalence rates of fd are difficult to estimate , but the lesions are not rare . the disease may involve single bone ( monostotic-60% ) or multiple bones ( polyostotic-40% ) with a predilection for the craniofacial bones , ribs , pelvis , and long bones . whole body f-18 flourodeoxyglucose pet / ct ( fdg pet / ct ) has been widely used in tumor imaging . several fdg pet / ct reports suggested that the appearance of fd could mimic a malignant process . therefore , this case was presented with the aim of guiding physicians in evaluating bone lesions . a 45-year - old female patient presented with pain in the right leg and x - ray tibia showed sclerotic areas with bony expansion . she was referred for whole body bone scintigraphy , which showed intense uptake in shaft of right femur and tibia [ figure 1 ] . fdg pet / ct was done for detection of other site involvement and rule out primary malignancy . whole body pet / ct showed intense tracer uptake in the right femur and tibia [ figure 2 ] . fused fdg pet / ct and ct showed uptake in expansile , multifocal , endosteal scalloping soft tissue lesions ground glass pattern of the bone and surrounded by a distinct rim of reactive bone in the medullary cavity of right femur and tibia , with an suv max of 8 to 10 [ figure 3 ] , arrows . a diagnosis of polyostotic fd was made and she underwent internal fixation for both femur and tibia to prevent fracture . whole body tc99m - mdp bone scintigraphy image showing intense uptake in the right femur and tibia . also thyroid and gastric muscosa due to free pertechnetate seen whole body f-18 fdg pet / ct showing intense tracer uptake in the right femur and tibia . coronal fused pet / ct ( a and c , green arrows ) and ct ( b and d , red arrows ) showed expansile , multifocal , endosteal scalloping soft tissue ground glass pattern of the bone and surrounded by a distinct rim of reactive bone in the medullary cavity of right femur and tibia , charecteristic of fd fd is a benign disorder of bone - forming mesenchyme , comprising around 10% of all benign bone tumors . the ribs , femur , tibia , calvarium , bones , spine , pelvis , and shoulder girdle are the frequently involved sites . it is caused by an activating somatic mutation in the alpha subunit of the gs protein , resulting in abnormal osteoblastic differentiation and increased bone turnover . it is known to be difficult to make the radiologic differential diagnosis between fd and malignant neoplasm . the mr signal intensity of fd is low to intermediate on the t1 images and it is variable on the t2-weighted sequences . histopathologic examination of the t2 hyperintense cases revealed fewer bony trabeculae , less cellularity and fewer collagen fibers than that of the t2 hypointense cases . fd usually shows increased uptake on bone scintigraphy . in a conventional three - phase bone scan , it shows hyperemia , increased blood pool , and intense tracer uptake in the delayed cortical phase . this is related to the high bone turnover associated with the immature woven bone , a characteristic feature of this disorder . ct is still the best technique for demonstrating the typical radiographic descriptions of fd , which are the ground glass pattern of the bone and the lesion being surrounded by a distinct rim of reactive bone . therefore , thorough interpretation of the ct information from fdg pet / ct study is helpful to make a correct diagnosis . with regard to fdg pet / ct , it is known that there is large variability in the fdg avidity of fd . there are several reports that fd showed increased fdg uptake and this mimicked malignant bone involvement . however , the discrimination between fd and malignant bone tumor is not well - defined because of a large suv max overlap . the suv max values of fd have ranged from 3.8 to 7.2 in the previous case reports . histiocytic and giant cell containing benign bone lesions in particular can show fdg hyper metabolic activity , and the turnover of the remodeling process may have various rates in different patients who have different stages of this disease . in addition , fibroblasts predominately proliferate in fd lesions , and the difference in the amount of proliferating fibroblasts or their metabolic turnover may result in a discrepancy of fdg uptake among fd lesions . this case highlights the potential pitfalls in the interpretation of bone scans and fdg pet / ct when widespread abnormal areas of increased uptake are seen , particularly in the context of suspected malignancy .

fibrous dysplasia ( fd ) of the bone is characterized by the medullary cavity of bones becoming filled with fibrous tissue , and its etiology remains unknown . it is usually asymptomatic and found incidentally on imaging studies that are performed for other purposes . fd may closely mimic the appearance of bony metastatic disease on radiological examinations . we report the case of a 45-year - old female patient , which appeared to have multiple bone lesions on initial workup images . subsequently , the bone lesions that showed increased fdg uptake on pet / ct in right femur and tibia were identified as fd . the present case is a useful addition to the current body of literature of false positive f-18 fdg pet / ct due to a benign skeletal pathology and underscores the importance of high index of suspicion and careful correlation , whenever one comes across such an unusual pet / ct finding .

scientists have known for decades that cancer cells can become invasive and metastatic by undergoing an epithelial - to - mesenchymal transition ( emt ) . more recently , researchers have discovered that the emt also confers resistance to radiation and a wide spectrum of chemotherapy drugs , including dna - damaging agents and targeted inhibitors of specific kinases . moreover , cancer cells that undergo an emt are , in many cases , functionally indistinguishable from cancer stem - like cells . these observations have revealed that , by merely changing their differentiation state , cancer cells can gain the key malignant traits responsible for most cancer - related deaths . in light of this surprising fact , there is significant interest in finding ways to treat tumors by targeting the emt . one major focus has been to delineate the ligands , receptors , and downstream signaling proteins that , when activated , induce cells to undergo an emt ; inhibiting these factors could suppress tumor progression by either preventing cancer cells from undergoing an emt , or by reversing emt in cells that have already undergone the transition . a second area of focus has been to delineate the molecular mechanisms by which the emt causes cells to acquire either invasiveness or drug resistance ; while it would not prevent or reverse emt , inhibiting these mechanisms could provide a therapeutic benefit by suppressing the malignancy of cells that have undergone an emt . a more direct approach to targeting the emt would be to search for agents that are selectively lethal to cells that have undergone an emt . in a high - throughput screen of over 300,000 compounds , we succeeded in identifying a few small molecules with strong emt - selective toxicity . the discovery of these emt - selective compounds was not a foregone conclusion because emt cells were resistant to all test compounds they had been exposed to before the actual screen , and suggested that such agents were exploiting unique vulnerabilities acquired by cells upon emt . at the time , however , we did not know what any of these vulnerabilities actually were . in our recent publication , we show that 2 of the emt - selective compounds identified in the above chemical screen selectively activate endoplasmic reticulum ( er ) stress pathways collectively termed the unfolded protein response ( upr ) . by contrast , closely related structural variants of these compounds that are not toxic to emt cells do not activate er stress signaling . we further show that emt sensitizes cells to 4 molecules that are established perturbagens of er function and to reductions in expression of the er chaperone bip . taken together , our findings identify the first known vulnerability of emt cells : sensitivity to agents that perturb the function of the er ( fig . 1 ) . figure 1.epithelial-to-mesenchymal transition sensitizes cancer cells to endoplasmic reticulum stress . this achilles heel of cells that have undergone emt appears to be a consequence of physiological changes that occur in cells when they migrate and invade . invading cells cells significantly upregulate the synthesis and secretion of pro - migratory ecm components ; this , in turn , significantly increases the protein load within their er . in our recent publication we show that the increased sensitivity of emt cells to er stress is a consequence of this increased er load , since inhibiting ecm synthesis reduces the sensitivity of emt cells to er stress . inhibiting ecm synthesis , however , also prevents emt cells from migrating . because ecm synthesis is essential for invasion , our findings suggest that increased sensitivity to er stress may be a general feature of metastatic cancer cells . because some highly secretory cell types appear to selectively utilize upr pathways , we examined whether there was any evidence of this occurring upon emt . we found that the highly secretory emt cells specifically activate the perk branch of the upr at low levels , even in the absence of any treatment with exogenous er stressors . in contrast , there was no detectable activation of the ire1 or atf6 branches of the upr upon emt . treatment with er stressors greatly increased the level of perk signaling in emt cells , while also strongly activating both ire1 and atf6 signaling . these findings led naturally to the question of the role of perk signaling in emt cell biology . the perk branch of the upr pathway is critical for the function of many secretory cells including osteoblasts and cells . perk loss of function causes reduced secretion in osteoblasts and cell death in cells , manifesting in animal models as decreased bone density and diabetes , respectively . although perk inhibition did not affect the survival or growth of emt cells , we found that inhibiting perk activity increased the sensitivity of emt cells to er stress . moreover , we found that emt cells required perk signaling to form tumorspheres and to migrate . the observation that the emt program leads to selective activation of the perk branch of the upr was supported by analysis of gene expression data from uncultured patient tumors . analysis of expression data from 800 tumors spanning a range of tumor types including breast , colon , gastric , and lung cancer revealed a strong positive correlation between the expression of emt genes and perk pathway genes . in contrast , no significant correlation was observed between emt genes and ire1 pathway genes in the same set of tumor expression data . first , they suggest that agents that promote er malfunction should be explored for their potential to selectively eradicate cancer cells that have undergone an emt . second , they suggest that perk pathway inhibitors may prove useful for suppressing several of the malignant traits associated with emt . this research was supported by grants from the richard and susan smith family foundation and the breast cancer alliance ( to pb gupta ) , and the national science foundation graduate research fellowship ( grant no .

in a recent report published in cancer discovery we identified a novel vulnerability of cancer cells that have undergone an epithelial mesenchymal transition ( emt ) and established that the perk branch of the unfolded protein response is constitutively activated upon emt . in this commentary , we summarize and provide context for our findings .

adrenal incidentaloma is an adrenal mass , generally one cm or more in diameter , that is discovered serendipitously during a radiologic examination performed for indications other than an evaluation for adrenal disease . the incidence of adrenal incidentaloma based on imaging and autopsy data varies from 4 to 6% . the differential diagnoses of unilateral adrenal incidentaloma include cortical adenoma , pheochromocytoma , adrenocortical carcinoma , granulomas ( e.g. tuberculosis and histoplasmosis ) , adrenal cyst , myelolipoma , ganglioneuroma , metastatic deposits and rarely adrenal oncocytoma . . sites of origin for oncocytoma include kidney , salivary glands , parathyroid , lung , pituitary , and ovary . however , the oncocytoma of the adrenal gland is very rare . there are nearly 50 case reports of adrenal oncocytoma in english literature.[47 ] these are usually large and non - functional ; however , rarely functional adrenal oncocytomas presenting as cushing 's syndrome and pheochromocytoma have also been reported . we describe a case of large , non - functioning adrenal oncocytoma in a young woman who presented with abdominal pain . a 28-year - old female presented to the out - patient department with history of ill - defined , non - colicky abdominal pain in the right flank region for the past 2 months . abdominal examination revealed a non - distended , soft abdomen , which was not tender on palpation . ultrasound ( us ) abdomen revealed a large heterogeneous mass between the right kidney and the liver , containing both hyperechoic and hypoechoic areas . computed tomography ( ct ) scan showed well - demarcated 7 5.5 cm homogenous mass arising from the right adrenal gland with few foci of necrosis [ figure 1 ] . the mass enhanced ( 50 hu ) after contrast and there was no evidence of calcification or surrounding tissue invasion and adenopathy . computed tomography adrenal showing well - demarcated , 7 5.5 cm homogenous mass arising from the right adrenal gland with few foci of necrosis ( arrow ) hemogram , electrolytes , renal and liver function tests were normal . nmol / l ( normal 171536 nmol / l ) and was suppressible after overnight 1 mg dexamethasone ( 30 nmol / l ) . 24 hours urinary metanephrine was 111.6 g ( normal up to 131 g/24 hours ) and normetanephrine was 157.1 g ( normal up to 177 g/24 hours ) . histopathology revealed well - encapsulated tumor [ figure 2a ] and the cells were arranged in sheets as well as nests . individual cells had abundant eosinophilic granular cytoplasm with centrally placed nucleus [ figure 2b ] . she continues to remain well till the last follow - up after 1 year of surgery . cells arranged in sheets as well as nests with abundant eosinophilic granular cytoplasm and centrally placed nucleus ( a ) and histopathology showing well - encapsulated tumor ( b ) with the availability and improvement in imaging modalities , adrenal incidentalomas are not uncommon in clinical scenario . adrenal incidentalomas 4 cm in size are likely to be malignant ; however , adrenal oncocytoma , a rare cause of adrenal incidentaloma , despite being larger in size , is usually benign . all the cases of adrenal oncocytoma so far reported in the literature have been diagnosed retrospectively , as there are no clinical and radiological clues to suspect the diagnosis preoperatively.[48 ] most common site of origin of oncocytoma is kidney ; however , the adrenal is affected rarely . adrenal oncocytoma has been reported in the age group from 27 to 72 years with modest female predilection . these tumors vary in size from 3 to 17 cm and are usually larger than 6 cm as seen in our case . moreover , adrenal oncocytoma dose not follow the rule of four : ( a ) 4% diagnosed on ct ; ( b ) 4% of these tumors are pheochromocytoma or adrenocortical carcinoma ; ( c ) 4 cm in size is an indication for surgery and ( d ) 4 years of follow - up is required . they are usually non - functional and benign in nature as was seen in our case . however , few cases of functioning adrenal oncocytomas secreting cortisol and adrenal androgens have been reported . though benign , a few cases of malignant oncocytoma with metastasis to adjacent and distant organs have also been reported . though imaging characteristics for renal oncocytoma are well described , imaging features of adrenal oncocytoma lack clarity . fibrous encapsulation visible on us and ct is a characteristic finding for adrenal oncocytomas , though it was not seen in our case . despite being larger in size , the surrounding tissue invasion , particularly fat plane and vascular structure , the differential diagnoses of other larger but benign adrenal masses include adrenal myelolipoma and adrenal cysts and both can be diagnosed with confidence on imaging . however , the diagnosis of oncocytoma can be secured preoperatively by fine needle aspiration cytology after careful exclusion of functionality of the tumor and particularly in a large mass with preserved tumor outline and without invasion to the surrounding structure . on gross examination , these tumors are round , well circumscribed and encapsulated . histologically , these tumors contain cells arranged in solid , tubular , papillary and trabecular pattern . cells are highly eosinophiilic and granular due to high mitochondrial density . while the majority of oncocytomas are benign and have an uneventful course , malignant oncocytoma , however , are rare and have a poor prognosis . adrenal oncocytoma should be included in the differential diagnosis of a unilateral large non - functioning adrenal mass with preserved tumor outline .

adrenal incidentalomas of more than 4 cm size are usually malignant . we describe a 28-year - old woman with a 7-cm , non - functioning , well - demarcated adrenal mass , which was identified as an oncocytoma on histopathological examination . therefore , a large , non - functioning , unilateral adrenal mass with preserved tumor outline should invoke the suspicion of oncocytoma , which is invariably a benign tumor .

another brick in the wall of knowledge on renal resistive index ( ri ) was provided by dewitte and colleagues in the previous issue of critical care . it is additional evidence that much remains to be done to understand what describes this parameter , as rightly concluded by the authors . the overall messages repeatedly emerging from these studies are that renal injury is associated with ri elevation but that considerable overlap between patients with and without acute kidney injury ( aki ) precludes the use of this index in daily practice [ 1 - 4 ] . additional observations have been made with less reproducibility from one study to another as the number of studies increases : elevated ri may differentiate persistent versus transient aki , ri increases with lower arterial partial pressure of oxygen ( pao2 ) , and ri increases with lower mean arterial pressure ( map ) . this last point is challenged by the study by dewitte and colleagues , in which such a relationship was observed only in patients without aki . ri data in the critical care setting are reminiscent of the story in the field of nephrology , in which ri is very easy to measure but of little help in practice . before ri 's poor utility in clinical practice and progressive abandonment , initial enthusiastic reports claimed that ri could help to assess the impact of renal urinary tract obstruction , diagnose acute rejection and chronic dysfunction of renal transplant , and predict renal function recovery after renal artery stenosis dilatation [ 6 - 8 ] . we know several reasons why ri failed to hold its promise to predict or accurately diagnose aki , but we do not know which of the several determinants that impact ri - systemic pulse pressure , map , pao2 , renal vascular resistance , renal vascular compliance , and renal parenchymatous pressure - relate to the pathophysiology of aki . the term ' renal resistive index ' is undoubtedly misleading , and the less definite term ' renal vascular index ' ( rvi ) may be better chosen to preclude erroneous interpretation . indeed , persistent circulatory abnormalities ( low renal blood flow ) have been observed at the established phase of acute tubular necrosis , and renal vascular damage now emerges as a prominent feature of aki . however , animal data showed that , at the initiation of septic shock , renal blood flow is maintained or even increased provided that cardiac output is maintained . renal hemodynamics probably evolves along the different phases of aki , obscuring what can be inferred by rvi . second , it has been hypothesized that increased rvi may reveal renal vasoconstriction secondary to inadequate renal perfusion eventually leading to renal ischemia . this led some authors to suppose that rvi may guide hemodynamic therapeutic interventions and notably that lowering rvi may be a resuscitation endpoint . however , the observation by dewitte and colleagues that the relationship between a parameter of systemic hemodynamics ( map ) and rvi is present only in patients who do not develop aki may indicate that the use of renal doppler to optimize renal hemodynamics may be possible only in patients who do not need it . furthermore , if we make the assumption that rvi actually permits us to evaluate renal vascular resistance , the relationship between rvi and map ( with lower map associated with higher rvi ) may be difficult to reconcile with the usual concept of physiological regulation of renal circulation . indeed , although this may be debated , it has been demonstrated that , along a certain range of map , renal blood flow remains constant ( ' autoregulation ' phenomenon ) ; that is , in this range , lowering map results in renal vasodilation ( unlike other vascular beds ) ; thus , rvi diminution , not augmentation , should be observed . therefore , rvi does not represent renal vascular resistance , or renal autoregulation is abolished in some or all critically ill patients , or patients have been evaluated below the autoregulation map range . there is an urgent need to establish pathophysiological models allowing an understanding of what exactly rvi describes . routine invasive measurements of several hemodynamic parameters may give the critical care field , unlike the nephrology field , the opportunity to refine our understanding of rvi . aki : acute kidney injury ; map : mean arterial pressure ; pao2 : arterial partial pressure of oxygen ; ri : renal resistive index ; rvi : renal vascular index .

the previous issue of critical care reports new data on renal resistive index in critically ill patients . although high renal resistive index may indeed be associated with acute kidney injury , the existence of several determinants of this index , of which renal resistance is only one among many , obscures the usefulness of this index in clinical practice .

the use of glucocorticoids in the management of patients with sepsis remains controversial . in this issue of critical care forum , the dispute regarding steroid use in sepsis touches on the most basic aspects of glucocorticoid action . trials performed in the 1970s and 1980s indicated that pharmacologic doses were harmful , and glucocorticoid use was abandoned , only to resurface with the seminal article by annane and colleagues in 2002 . these data suggested that glucocorticoids would be of value in septic patients incapable of mounting a sufficient endogenous response ( that is , in patients with relative adrenal insufficiency ) . the work by annane and colleagues was followed by the corticus ( cortico - steroid therapy of septic shock ) trial , the data of which did not confirm the findings of annane and colleagues . however , significant methodological differences between the two studies leave the issue unresolved . to this day , the debate continues . in the discrepant results of these trials , two specific findings in patients with sepsis steroids limit activity in white blood cells and thus function as anti - inflammatory agents . but in the liver and heart , glucocorticoids stimulate reactions that are pro - inflammatory : expression of genes encoding acute - phase reactants and potentiation of the cardio - stimulatory actions of catecholamines . the second key finding relates to serum levels , which are often quite elevated , a finding that may reflect changes in cortisol metabolism induced by critical illness . given these issues , it is not surprising that the role of glucocorticoids in sepsis is confounding . one possible answer becomes apparent when the mechanisms underlying steroid activity are examined . unlike protein hormone pathways , steroid pathways are wholly intracellular and do not involve cell - surface receptors . rather , steroid receptors are intra - cytoplasmic . several years ago , revollo and cidlowski began a series of experiments examining the structure of the glucocorticoid receptor ( gr ) . these studies demonstrated that grs arise from a single gene that is located on chromosome 5rq31 - 32 and that contains nine exons and gives rise to three mrnas ( figure 1 ) , two of which are germane to this discussion . the second , gr , is generated by an alternative splicing site that removes a long segment at the beginning of exon 9 and splices the shorter remaining portion of exon 9 onto exon 8 . gr contains several potential translational start sites , resulting in the formation of at least eight additional isoforms that differ in the length of their n - termini ( figure 1 ) . although the affinity for glucocorticoids is the same in each isoform , their ability to bind co - factors and dna polymerase ii , and thus to act as transcription factors , differs markedly . gross and cidlowski suggest that this differential ability to induce gene expression is responsible for the tissue - specific actions of glucocorticoids . in contrast to the isoforms of gr , gr does not bind glucocorticoids . by means of mechanisms that are unclear , gr acts as a dominant negative inhibitor of glucocorticoid - responsive gene expression . guerrero and colleagues found that the expression of gr in white blood cells of nine patients with septic shock was significantly higher on admission than on discharge . serum from these patients enhanced the in vitro expression of both gr and gr in cultured t and b cells but had a more profound effect on gr. the serum also induced glucocorticoid resistance in mononuclear cells in vitro . these findings suggest that septic shock inhibits the activity of glucocorticoids by enhancing expression of the dominant negative gr. variability in the abundance of gr might explain why exogenous glucocorticoids exert such capricious effects in patients with sepsis . enhanced gr expression might also explain why glucocorticoid levels are so dramatically elevated in patients with sepsis : the presence of a receptor that inhibits glucocorticoid activity will induce increased release of the hormone to compensate for and stimulate the desired response . we have invoked a similar explanation for the markedly increased interleukin-6 ( il-6 ) levels observed in sepsis . our work on il-6 has demonstrated impairment of the gp130/jaks1/stat-3 signal transduction pathway that mediates many of that cytokine 's effects . to overcome this block , increasingly massive amounts of il-6 the implication of these findings may be that the increased abundance of glucocorticoids , il-6 , and perhaps a number of additional mediators and hormones is an adaptive response that should be augmented . however , most trials of cytokine - based therapy attempt exactly the opposite : blocking activity . certainly , the findings of guerrero and colleagues reinforce the notion that sepsis is a complex disorder and mono - therapy is unlikely to work .

glucocorticoid use in sepsis is controversial . in contrast to other extracellular signaling molecules , glucocorticoid receptors ( grs ) are intra - cytoplasmic . several gr isoforms have been identified . a study in critical care forum suggests that sepsis alters the abundance of the dominant negative gr. here we discuss gr isoforms and how they may affect cellular responses to glucocorticoids in sepsis .

attendances to emergency departments due to accidental digital auto - injection of adrenaline seem to be on the rise . for every 50000 epipen units administered correctly , one is accidentally performed into a digit , usually the thumb ( 1 ) . we present the case of a 32 year old gp who unwittingly deployed an epinephrine pen into the palmar aspect of her distal phalanx of her right thumb . she bathed the affected digit in hot water with no relief and attended the accident and emergency department an hour later where the medical staff were unsure how to proceed with treatment . 120 minutes post - incident , she was referred to the vascular surgery unit and after examination , the distal phalanx of the thumb was still ischaemic , lacked sensation and was difficult to move . capillary refill was absent . in the first instance , a gtn patch was applied to the radial artery just below the thenar eminence . there was no benefit after 30 minutes or two hours post incident . following a google search for similar cases , various case reports and a series looking at digital epinephrine injection instructions regarding phentolamine administration were followed and 2 millilitres of 0.5% phentolamine were administered subcutaneously along the same tract taken by the epinephrine pen . as per advice online , the patient was continually monitored for hypotension or cardiac arrhythmias on the ward . within two minutes , blood supply to the digit was fully restored , with normal movement , sensation and capillary refill and the patient was discharged home . epinephrine is indicated in the emergency treatment of severe anaphylactic reactions to insect stings , bites , food , drugs and other allergens as well as idiopathic or exercise - induced anaphylaxis . the epipen ( 1:1000 or 0.3mgs in 0.3mls ) and epipen jr ( 1:2000 or 0.15mgs in 0.3mls ) auto - injectors administer a single dose of epinephrine intended to be delivered intramuscularly in cases of severe anaphylaxis . for stability purposes , approximately 1.7 mls remains in the auto - injector after activation and can not be used . since preliminary work by zucker in the 1950s into adrenaline analogue reversal ( 3 ) and later a case report by jordan in 1969 whereby a dental assistant had self administered epinephrine to a cut finger to reduce bleeding ( 10 ) , phentolamine reversal of epinephrine has been suggested in the literature repeatedly as the most suitable method of reversal for accidental digital epinephrine injection . phentolamine is a competitive -receptor antagonist that has similar affinities for -1 and -2 receptors . it can be used in large doses for the short term control of hypertension in patients with phaeochromocytoma or in the treatment of hypertensive crisis following the abrupt withdrawal of clonidine . warm water immersions , the administration of systemic or topical nitroglycerin preparations and even topical terbutaline infiltration have been used to treat the accompanying vasospasm that occurs in the digit with limited success ( 4,5 ) . despite this , using phentolamine in this manner remains poorly publicised and lacks a license for use in the uk for the reversal of end artery vasospasm caused by epipen administration . in fact it is only available as rogitine ( ciba ) , containing 10 mg of phentolamine in 1 ml of clear solution . since administration of around 2 - 5 mls of 0.1% phentolamine appears in most reported cases to be completely effective in reversing the effects of 0.3mgs of epinephrine , the greatest danger in administering phentolamine surely comes from drug errors in calculating the correct dilution . many case reports in fact report the reversal of epinephrine after 12 hours with no harmful sequelae at all ( 6,7 ) . this raises the possibility that the ischaemia induced is not complete or that the digital arteries are augmented by another blood supply . indeed , the dalhousie project clinical phase trial recorded 1340 fingers electively injected with low dose adrenaline ( 1:100,000 ) without a single case of digital skin , or fat loss , let alone digital infarction ( 1,2,6 - 8 ) . treatment with phentolamine is nevertheless worthwhile due to the distressing ischaemic pain and parasthesia suffered by the patient.(9 ) previously reported cases of digital infarction in the literature , dating from the pre 1950s were all associated with co - administered procaine or cocaine , both well known to cause massive arterial spasm and digital infarction on their own . of note , there have been no case reports of digital gangrene using commercial lidocaine and epinephrine since its introduction in 1948 . multiple studies involving thousands of patients support the premise that the use of lidocaine with epinephrine is safe in the digits ( 1 ) . hinterberger compared various methods of phentolamine dilution and administration , suggested a 0.5% solution administered by direct local infiltration to the initial puncture region is the most effective as this concentration provides sufficient phentolamine for reversal of ischaemia , without the need to present an inordinate amount of fluid to the pulp spaces of the finger , accessing the affected receptors and any residual epinephrine directly . a digital block technique was found to be less effective as it has to diffuse through tissue and a later study suggested that introduction f large amounts of diluted phentolamine itself could cause an iatrogenic hydrostatic ischaemia ( 9 ) . there are no reports of systemic side effects related to the local use of small doses of subcutaneous phentolamine for the treatment of adrenaline induced digital ischaemia ( 4,5 ) nonetheless , due to the small risk of systemic absorption or accidental intravascular administration of phentolamine it has been standard practise to monitor ecg and blood pressure readings although evidence points to this being unnecessary . this case further highlights the importance of instant access to the internet and the need for available phentolamine in a&e , surgical assessment units or any department where minor surgery takes place . despite the lack of previous experience in the management of such cases , the patient was successfully treated after performing a quick search on google search , demonstrating the need for online resources to augment our practice .

after the accidental injection of epinephrine into a digit , various techniques to try and reverse the ensuing ischaemia were unsuccessful . to identify a further treatment strategy and as members of the admitting team were unfamiliar with digital injection of epinephrine a google search was performed . previous cases were described and separate sources indicated appropriate management protocols utilising phentolamine . after administration , an almost immediate reversal of ischaemic symptoms occurred . this highlights the role of the internet as an adjunct in managing unfamiliar situations and practising evidence based medicine .

primary intraosseous odontogenic squamous cell carcinoma ( pioscc ) is a rare and malignant tumor . the etiology seems to be related to the malignant degeneration of embryological remains . in this line , epithelial rests of malassez , dental lamina and epithelium of the dental follicle represent potential suspects . according to the 2005 , the world health organization classification of tumors , three subtypes of pioscc exist : solid tumor that invades the marrow spaces and provokes osseous absorptionsquamous cell carcinoma ( scc ) originated from the lining of an odontogenic cyst . specifically , this subgroup includes two variants : keratocystic odontogenic tumor and odontogenic cystsscc associated with other benign epithelial odontogenic tumors . solid tumor that invades the marrow spaces and provokes osseous absorption squamous cell carcinoma ( scc ) originated from the lining of an odontogenic cyst . specifically , this subgroup includes two variants : keratocystic odontogenic tumor and odontogenic cysts scc associated with other benign epithelial odontogenic tumors . the diagnostic criteria of pioscc included undamaged oral mucosa and absence of distant metastasis during the follow - up . moreover , the switch from the epithelium of the cyst to scc is essential for a proper diagnosis . differential diagnosis includes alveolar carcinomas , jaw metastases from other locations , odontogenic tumors and tumors of the maxillary sinus . in this sense , in fact , due to the rarity of this disease , there are no established treatment protocols . the adequate surgical resection remains the gold standard for the treating this tumor . in this report hence , this case underlines the importance of routine monitoring of patients presenting large inflammatory cyst . the article describes a case of pioscc of the mandible affecting a 77-year - old man . more in detail , the patient was referred at maxillofacial outpatient department of granada university hospital by his general practitioner . in fact , the patient reported a progressive painless swelling in the left side of mandible . oral and extraoral examination evidenced an abnormal mandibular ballooning in the left side . in view of that , an orthopantomography and a computed tomography ( ct ) scan of the cervicofacial area were performed for allowing the proper diagnosis . panoramic radiography evidenced the presence of a large cyst in the left molar region and the lack of several tooth . ct scan evidenced an aggressive lesion invading body and ascendant branch of the jaw [ figures 13 ] . after analyzing this data , anamnesis and physical examination patient referred a tooth extraction of tooth 38 , 3 years ago because of the presence of a pericoronal cyst . after due consideration of all data , we staged cancer as ct3n0m0 . in the oncological committee of our hospital , we decided to treat the case with a wide surgical resection , neck dissection and postoperative radiotherapy . postoperative examination confirmed the diagnosis of pioscc poorly differentiated [ figure 4 ] with resection margins free . three - dimensional computed tomography image showing an intraosseous odontogenic squamous cell carcinoma of the mandible . at the physical examination , the patient presented a bone consistency with swelling of vestibular region in the left side of the jaw axial computed tomography image of mandibular intraosseous odontogenic squamous cell carcinoma . the lesion affects the body and the ascendant branch of the mandible in the left side orthopantomogram of mandibular intraosseous odontogenic squamous cell carcinoma histopathological image of mandibular intraosseous odontogenic squamous cell carcinoma with h&e stain however , the majority ( 50% ) are well - grade malignant tumors . the median age of presentation is between the sixth and eight decade of life and it occurs more often in males . according to the literature , the time diagnosis is often delayed due to the absence of symptoms provoked by pathology . in this line , a review of 30 cases evidenced that pioscc showed the following forms of presentation : asymptomatic ( 26,7% ) , asymptomatic mass ( 26.7% ) , mass ( 16.7% ) , pain ( 13.3% ) , nerve involvement ( 10% ) and expansion ( 6.7% ) . thus , it is possible that the tumor could have been confused with a pericoronal cyst . panoramic x - ray and ct scan are essentials for a proper diagnosis and analysis of the tumor extension . a number of other lesions should be considered in the differential diagnosis such as alveolar carcinomas , jaw metastases , odontogenic tumors and maxillary sinus tumors . moreover , several reports suggest that postoperative radiotherapy could be helpful to improve the overall survival . we treat the case with surgical resection , neck dissection and postoperative radiotherapy . considering the wide surgical margins and lack of evidence about the effectiveness of chemotherapy resuming , this case underlines the importance of routine follow - up of patients presenting large inflammatory cyst .

primary intraosseous odontogenic squamous cell carcinoma ( pioscc ) is a rare tumor . the incidence is low , and approximately 200 cases are reported in literature . the etiology is associated with the malignant degeneration of embryological remains . differential diagnosis includes alveolar carcinomas , jaw metastases from other locations , odontogenic tumors , and tumors of the maxillary sinus . however , the diagnosis could be delayed due to the absence of symptoms in early stages . surgery represents the first choice treatment . whereas , postoperative radiotherapy could be helpful to improve the overall survival . the prognosis is generally poor . in this report , we describe the case of a 77-year - old man accidentally diagnosed pioscc . the patient referred a tooth extraction and cystectomy 3 years before . the pathological examination of the cyst tissue evidenced an inflammatory cyst . however , no patient follow - up was performed . hence , is important to stress that routine monitoring of patients affected by large inflammatory cysts of the jaw bones should be mandatory .

carcinoma cervix is a common malignancy of the female genital tract and radiotherapy is a standard mode of management . although with the recent advances , the complications associated with radiotherapy have reduced , we do continue to come across long - term complications of radiotherapy given decades ago for different malignancies . management of these complications are a challenge to the oncology team and requires case oriented approach . we present a case of vesicocutaneous - vesicovaginal fistula with osteomyelits of the right pubic bone which was a late complication of post - cervical cancer radiotherapy . a 75-year - old lady presented with a non - healing ulcer in her lower abdomen since 1 year . she was treated for carcinoma cervix 30 years ago with radiotherapy treatment and was disease free since then . two years ago , she noticed discoloration of her lower abdominal skin which gradually ended in an ulcer with dribbling of urine . she was treated with surgical debridement and regular dressings . despite this treatment she developed ulcer in lower abdominal which was increasing in size with continuous dribbling of urine and intermittent vaginal dribbling of urine . for the past year she was confined to her home with continuous dribbling of urine which required frequent change of dressings , irritation of the surrounding skin and low self - esteem . for further management , there was an ulcer over the hypogastrium which was dribbling with urine , exposing a part of the posterior wall of the urinary bladder and granulation tissue associated with features of urinary tract infection [ figure 1 ] . she was admitted and her general condition was improved with administration of intravenous antibiotics , control of infection , blood transfusion and improvement nutritional status . radiograph of pelvis was suggestive of osteomyelitis of right pubic bone . computed tomography of pelvis was suggestive of osteomyelitis of right pubic bone [ figure 2 ] . multiple biopsies from the lesion were performed to rule out recurrence of cancer and the histopathological examination was suggestive of chronic infection . after improvement of patient , general condition urinary diversion was performed with bilateral percutaneous nephrostomy . multiple wound debridement 's were performed with removal of the necrotic soft tissue , right pubic bone , remnant bladder wall and ligation of the lower ureteric ends . once the infection settled and the urinary fistula was controlled with diversion , the lower abdominal defect was covered with pedicled anterolateral thigh flap from the left side [ figures 3 and 4 ] . the flap settled well and the patient was able to perform activities of daily living . . clinical picture suggestive of non - healing ulcer over hypogastrium computed tomography of pelvis with 3 d reconstruction suggestive of osteomyelitis of right pubic bone postoperative clinical picture showing anterolateral thigh flap covering the hypogastric ulcer follow - up clinical picture showing well - accepted anterolateral thigh flap carcinoma cervix is a common malignancy of the female genital tract ; radiotherapy , chemotherapy and surgical treatment are the modes of management.1 the complications associated with pelvic radiation include osteomyelitis of pubic bone , secondary osteosarcoma , vesicovaginal fistulas . due to use of the recent advances in radiotherapy treatment the complications associated with radiotherapy have been reduced , but the delayed complications of radiotherapy given decades ago for different malignancies at presented in clinical settings . management of these complications is a challenge to the oncology team and requires case - oriented approach . although a single complication can be managed by concerned specialist , rare occurrences of multiple complications requires a combined team approach . in this case , the inflammation associated with continuous urinary flow was first controlled by bilateral percutaneous nephrostomy , the infection was then treated with higher antibiotics and repeated debridement of necrotic soft tissue and pubic bone . the wound was thus converted from a chronic wound to an acute wound , the vesicovaginal fistula was repaired and the entire wound was covered with pedicle anterolateral thigh flap . osteomyelitis of the pelvic bones is a rare complication after pelvic radiotherapy and occurs a long time after primary radiation.2 in our case , the osteomyelitis of the right pubic bone was a primary complication or secondary to the urinary fistula could not be determined . plain radiograph , computed tomography and magnetic resonance imaging aids for diagnoses of osteomyelitis of pubic bone . the osteomyelitis of the right pubic bone was managed with radical debridement and wound wash . percutaneous nephrostomy is a standard treatment in the management of post - radiotherapy urinary complications.3 percutaneous nephrostomy is used most commonly in obstructive symptoms , in our case it was adopted to control the infection and reduce the inflammation associated with continuous flow of urine in to the pelvis and on to the abdominal skin causing excoriation of the wound margins . the remnant parts of the posterior wall of the urinary bladder along with the mucosa were removed and the vesicovaginal fistula was closed . complex defects in abdominal wall are due to infection , tumour resection and traumatic injuries and the treatment options are use of free flaps or pedicle flaps.456 anterolateral thigh flap is an ideal flap ( pedicled and free ) for soft tissue cover in different regions of the body.789 the entire defect in the lower abdomen was covered with a pedicle anterolateral thigh flap ; an anterolateral thigh musculo - cutaneous flap measuring 23 9 cm was mobilised to cover the defect . the flap settled well by filling in to the cavity and giving a good skin cover . this flap was selected because it was well away from the area of irradiation and the flap donor site was closed primarily enabling good healing and faster recovery . we emphasize role of team - based approach for treatment of complication of radiotherapy in treatment of various malignancies .

vagino - vesico - cutaneous fistula is a rare condition characterised by continuous dribbling of urine and secondary infection of the involved areas with poor self-esteem.osteomyelitis is delayed complication of radiotherapy treatment for cervical cancer.treatment of these conditions is a challenging entity especially after previous surgery and irradiation . we present a case of vesicocutaneous - vesico vaginal fistula with osteomyelits of the right pubic bone which was a late complication of post - cervical cancer radiotherapy .

we , as medical doctors , hardly ever come across rare zoonoses , such as buffalopox . we here report an outbreak in our area that affected buffaloes and humans simultaneously in an attempt to increase this awareness . an owner of a buffalo herd and five of his assistants at the farm had vesicular and pustular lesions on hands , legs , and feet . , 10 new buffaloes were bought from a distant market , all of whom were healthy at the time of purchasing . five of the farm workers , all involved in milking buffaloes , were affected by the disease . here , the history of human illness revealed that affected individuals had one to five lesions on hands , forearms , and legs [ figure 1 ] . lesions in the farm workers appeared within seven days of appearance of illness in the buffaloes . the lesions progressed from macules to papules , vesicles , and pustules over the next three to five days . they had fever ( 39 to 40c ) , malaise , body ache , and loss of appetite . the lesions healed after 8 - 10 days , but scabs fell off only after about three weeks . virological study confirmed that the humans and buffaloes were infected by buffalopox virus ( bpxv ) . the tests carried out included antigen detection , polymerase chain reaction ( pcr ) positivity and isolation of bpxv from the lesions or antibodies in serum . buffalopox is a viral disease of economic importance primarily affecting domestic buffaloes ( bubalus bubalis ) . the bpxv belongs to genus orthopoxvirus a member of family poxviridae and is closely related to vaccinia virus ( vacv ) . such outbreaks have been reported from various parts of the world and india.[35 ] in india , even now small and medium herd owners prefer manual milking . the milkers wash hands with soap and water before milking ; however , the practice of using gloves while milking is almost nonexistent . this is probably responsible for the spread of infection from infected buffalos to the uninfected ones . in conclusion , we believe that clinicians must be aware of the fact that pustular skin lesions that not yield bacteria might be of viral origin , and need to be investigated from that perspective also . with regard to dairy practices , awareness about the use of gloves , at least while milking infected buffalos of paramount importance , and should be emphasized .

an outbreak of febrile illness with vesicular lesions on hands and forearms of six persons was investigated . there was simultaneous outbreak of similar illness in buffaloes that were milked by these persons . buffaloes had lesions on many parts of body including udder and teats . manual milking without gloves exposed the persons to the infection . investigations proved that both the outbreaks were due to buffalopox virus infection . improved dairy practices like wearing gloves while milking might help in prevention of spread of infection to other animals and humans .

ecg abnormalities , especially in the st segments are caused by many reasons such as genetic diseases , cardiac ischemia , and electrolyte disorders 1 . aside these internal reasons , direct mechanical compression to the heart can also induce ecg changes such as brugada - like ecg 2 . as the heart is usually protected in the mediastinum , there are limited case reports for these mechanical compression - induced ecg abnormalities . we describe here a case of a mechanical compression from the right ventricular outflow tract to the right ventricle inducing an abnormal u - wave morphology . a 69-year - old woman was referred to our hospital due to ecg abnormality after retrosternal reconstruction surgery for esophageal cancer . at the first visit , ecg showed a prominent unusual shaped u wave in the right precordial leads ( v1-v3 ) despite least change in other leads ( fig . the plain chest ct demonstrated compression of not only the right ventricular outflow tract ( rvot ) , but also the right ventricle free wall by the reconstructed stomach tube ( fig . transthoracic echocardiogram showed the compression of the right ventricle without any abnormal valvular disease and left ventricular wall motion . the u - wave morphology in the precordial leads changed for every visit ( fig . 1d ) . during the follow - up , the patient had symptomatic sinus bradycardia and a permanent pacemaker was implanted . though the heart rate increased and the symptom improved , the u wave did not shorten ( fig . eso , reconstructed stomach tube , svc , superior vena cava , rvot , right ventricular outflow tract , lvot , left ventricular outflow tract , ao , aorta , rv , right ventricle , ra , right atrium , lv , left ventricle . mechanical compression of the rvot is reported to cause a brugada - like ecg showing an st segment elevation in the right precordial leads , due to loss of the action potential dome at rvot sites 3 . in the present case , the ecg had some st segment elevation in leads v1-v3 though it looks clearly different from brugada - type ecg , and the loss of dome usually does not induce u wave . as hypothermia is reported to induce repolarization abnormalities , ecg was recorded during the patient taking ice - cold water and no change was found 4 . bradycardia is also another cause of u wave , but was negated as the u wave was still present after pacemaker implantation 5 . interpretation of t wave and u wave on ecg is occasionally difficult and there is no absolute definition to distinguish them as in the current case 6 . we therefore defined the unusual wave seen in v1-v3 as a u wave by the assessment proposed by postema et al . briefly , the end of the t wave is the intersection of a tangent to the steepest slope of the last limb of the t wave and the baseline in lead ii or v5 . measured corrected qt interval calculated by bazett 's formula for all ecg was stably around 420 msec . m cell has the longest action potential duration among the three myocardial layers , hence we speculated that the broad mechanical compression to the broad right ventricle caused the electrical dissociation between the epi- and endocardial layer , and the authentic power of the m cell became visible through the right precordial ecg 8 . as the heart is usually protected in the mediastinum , the incidence of mechanical compression to the heart inducing ecg abnormality is rare . however , we must be aware of these causes for differential diagnosis for ecg abnormalities as the therapeutic strategy may change .

key clinical messagethe present case shows that a broad compression of the right ventricle by the reconstructed stomach tube after esophagus cancer surgery induced an abnormal u wave . when facing an abnormal ecg , we should keep in mind of the mechanical compression to the heart as a differential diagnosis .

large clinical trials have shown that sorafenib prolongs median survival and time to disease progression in patients with advanced hepatocellular carcinoma ( hcc ) [ 1 , 2 ] . however , no patients achieved complete response in these studies . even after sorafenib was launched onto the market , cases achieving complete response are extremely rare . we report herein a case of complete response induced by short - term sorafenib treatment against lymph node metastasis of hcc . a 60-year - old man received interferon / ribavirin combination therapy for chronic hepatitis c in 2002 and achieved sustained virological response ( svr ) . in 2008 , a hepatic tumor with a diameter of 60 mm appeared and a dynamic ct scan suggested hcc . the elevated serum des--carboxy prothrombin ( dcp ) level ( 1,097 mau / ml ) normalized after surgery . after surgery , our patient was followed up at a local clinic . in march 2011 , when he was 69 years old , he was referred to our hospital because of portal lymph node swelling . apart from a mild elevation in alkaline phosphatase and glucose levels , the hematological and biochemical laboratory data were also normal . serum levels of dcp ( 65 mau / ml ) and afp ( 25.1 ng / ml ) were slightly elevated ( fig . the lectin - bound afp level was also increased up to 22.8% , indicating recurrence of hcc . abdominal ultrasonography , dynamic ct , and dynamic mri did not show any tumors in the liver . however , these examinations revealed portal lymph node swelling ( 18 11 mm ) ( fig . 2a ) . based upon the diagnosis of hcc metastasis to the portal lymph node , we started sorafenib therapy at a dose of 800 mg / day . after 11 days , we discontinued sorafenib due to grade 3 hand - foot skin reaction and rash ( fig . 3 ) . in spite of treatment termination , portal lymph node swelling disappeared ( fig . the patient maintains remission up to june 2012 , more than 1 year after the discontinuation of sorafenib therapy . although pathological examination had not been performed , we made the diagnosis of hcc metastasis to portal lymph node for the following reasons . first , serum markers specific to hcc including dcp , afp and lectin - bound afp increased , and second , these markers then decreased to the normal range along with the shrinkage of the enlarged lymph node after sorafenib therapy . large clinical trials have demonstrated that sorafenib prolongs median survival and the time to disease progression in patients with advanced hcc [ 1 , 2 ] . however , very few patients ( 23.2% ) achieved a recist - based partial response and no patients at all achieved a complete response in these studies . even after sorafenib was launched onto the market , very few cases with complete response have been reported [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 ] . the patient reported here progressed to hcc 6 years after having achieved svr to interferon / ribavirin treatment . achieving svr the relative risk for hcc is 0.06 in patients with svr compared with untreated controls . although the risk is considerably reduced , hcc can occur even after achieving svr like our case . our patient required discontinuation of sorafenib treatment after 11 days due to grade 3 hand - foot skin reaction and rash . dose reduction due to hand - foot skin reaction was reported in 511.4% of the patients [ 1 , 2 ] . even advanced hcc could rapidly regress after a few weeks of sorafenib treatment . to our knowledge , this is the first case report in which hcc metastasis to a lymph node disappeared with sorafenib treatment .

a 60-year - old man received interferon / ribavirin combination therapy for chronic hepatitis c in 2002 and achieved sustained virological response . in 2008 , a hepatocellular carcinoma ( hcc ) with a diameter of 60 mm appeared and surgical resection was performed . in march 2011 , the patient was referred to our hospital because of portal lymph node swelling . abdominal ultrasonography , dynamic ct and dynamic mri did not show any tumors in the liver , but revealed portal lymph node swelling ( 18 11 mm ) . taking the elevation of serum des--carboxy prothrombin and alpha - fetoprotein levels , including the lectin - bound type , into consideration , we made the diagnosis of hcc metastasis to the portal lymph node . we started sorafenib therapy at a dose of 800 mg / day , but discontinued it after 11 days due to grade 3 hand - foot skin reaction and rash . in spite of treatment termination , portal lymph node swelling disappeared and the serum des--carboxy prothrombin and alpha - fetoprotein levels normalized . we considered that our patient achieved complete response to sorafenib according to the response evaluation criteria in solid tumors ( recist ) . the patient maintains remission up to june 2012 , more than 1 year after the discontinuation of sorafenib therapy . sorafenib could be a good option for unresectable or recurrent hcc .

in this issue of critical care , marin - caballas and coworkers investigate the relationship between cerebral perfusion pressure ( cpp ) and brain tissue oxygen tension ( ptio2 ) . identifying the optimal cpp following traumatic brain injury ( tbi ) is as crucial to best care for this vulnerable patient population as determining what is the best parameter ( with acceptable sensitivity and specificity ) for detecting impending brain ischaemia . marin - caballas and coworkers investigated the relationship between cpp and ptio2 using an observational study design . the patients enrolled had suffered varying degrees of diffuse axonal injury , and six of the 22 patients underwent surgical evacuation of a mass lesion . observation is always a good place to start exploring a research question when little is known about the subject . however , there are many data already available on ptio2 monitoring after tbi , and the relationship between ptio2 and physiological variables has been extensively investigated . a randomized controlled trial could have been performed , because there is equipoise among clinicians and guideline authors as to what is the optimal cpp , and this might have allowed standardization of other patient parameters . it is not possible to address the relationship between cpp and ptio2 in only 22 patients , in whom spontaneous fluctuations in cpp ( but mostly intracranial pressure [ icp ] ) were related to ptio2 measurements . an important confounding factor in this observational study is the patient management protocol , which mandates that when a low ptio2 is detected , all causes of this possible impending ischaemia should be corrected and cpp increased . thus , low ptio2 is likely to be associated with lower cpps , and higher ptio2 will be associated with higher cpps solely because of the management protocol . although an impressive 1672 data points were recorded , these should have been analyzed per patient and summary data subsequently analyzed for all 22 patients combined or with all data presented for each individual participant . many factors can influence ptio2 , and many of them are documented in table 1 of the report by marin - caballas and coworkers . there appears to have been little physiological stability in the patient cohort , making any interpretation of the relationship between cpp and ptio2 impossible ; for instance , the core temperature ranged between 31c and 39c , icp between 0 mmhg and 69 mmhg , and haemoglobin between 6.7 g / dl and 14 g / dl . with large fluctuations in icp documented and relative arterial pressure stability described , the authors might have considered looking at the relationship between icp and ptio2 rather than that between cpp and ptio2 . this commentary may appear somewhat critical , but it is worth emphasizing the potential for successful intervention to prevent critical reductions in ptio2 , and marin - caballas and coworkers describe a protocol with the potential to achieve this aim . mixenberger and colleagues showed that , in patients who had suffered tbi , low brain tissue oxygenation was associated with a worse outcome on neuropsychological testing , especially with respect to executive function and memory . these data suggest that there is possible predictive value of brain tissue oxygen for global functional recovery after head injury . the data generated by clinical indices must be incorporated into treatment protocols , which require development adhering to evidence - based medicine guidelines and then tested in a rigorous way . to date there has been no adequately powered intervention study ( powered for outcome ) in tbi with which to modulate a monitored physiological variable that is closely associated with outcome , with outcome assessed as the primary end - point . current recommendations regarding the management of patients after traumatic brain injury include reduction in brain tissue pressure ( i.e. icp ) and maintenance of an adequate arterial pressure ; these measures combined should result in cpp sufficient to achieve adequate oxygen delivery . after almost 20 years of observational studies comparing cpp and indices of cerebral oxygenation , it is apparent that there is no single value for cpp that , if achieved , will provide adequate cerebral oxygen delivery in all patients . thus , in order to minimize exposure to the risks associated with cpp interventions and to maximize benefit , clinicians must measure or assess cerebral oxygen delivery . ptio2 can provide this information and allows titration of icp and mean arterial pressure interventions to a directly measured endpoint and not a surrogate ( i.e. icp / cpp ) . tbi remains a common problem , and this should encourage us to design better and adequately powered trials of monitors and their associated interventions . cpp = cerebral perfusion pressure ; icp = intracerebral pressure ; ptio2 = brain tissue oxygen tension ; tbi = raumatic brain injury .

current recommendations regarding the management of patients after traumatic brain injury include reduction in brain tissue pressure ( i.e. intracranial pressure ) and maintenance of an adequate arterial pressure ; these measures combined should result in cerebral perfusion pressure sufficient to achieve adequate oxygen delivery . after almost 20 years of observational studies comparing cerebral perfusion pressure and indices of cerebral oxygenation , it is apparent that there is no single value for cerebral perfusion pressure that , if achieved , will provide adequate cerebral oxygen delivery in all patients . traumatic brain injury remains a common problem , and this should encourage researchers and clinicians to design better and adequately powered trials of monitors and associated interventions .

the treatment of orbital floor fractures with silastic sheet was prominent , until the advent of the titanium plate . it was an adequate treatment ; however , it had a tendency to migrate through the skin . diplopia when present for months tends to be difficult to resolve surgically , and what makes this case unique is that diplopia has resolved after > 30 years . a 54-year - old male presented to our department with a right - sided chemosis , periorbital swelling and was systemically unwell . clinically , his visual acuity was 6/12 in his right eye and 6/6 in his left eye . he reported that he had surgery on a fractured right orbit 33 years ago and had had persistent diplopia since . he reported that his diplopia had worsened , but clinically his eye and swelling was much improved . a ct scan ( fig . inferior and medial recti are both trapped by the implant. figure 1:coronal slice of ct scan demonstrating silastic sheet protruding through the right orbital floor . a decision was made to remove the implant surgically via a caldwell luc approach . the patient reported that his diplopia had resolved , for the first time , in 33 years . the treatment of orbital floor fractures with silastic sheet is well documented and there are large case series published . however , there is a high complication rate when using silastic as a material to repair orbital floor fractures . complications include : implant migration to the skin and the maxillary sinus , skin fistula , persistent diplopia , communication between the orbit and the sinus , periorbital cellulitis . the treatment of the fractured orbital floor has advanced over the last 30 years . computerized tomography , custom made implants , resorbable and non - resorbable implants , the use of endoscopic surgery and stereolithography all have an important role in the treatment of the fractured orbital floor . silastic complications may develop late and the latest published resolution of diplopia following surgery is 20 years . we feel that our patient is fairly remarkable , in that his diplopia has resolved at 33 years after initial surgery .

a 54-year - old male sustained a fractured right orbital floor 33 years ago , which was treated with a silastic implant . his diplopia never resolved , and he presented to us with an acute orbital cellulitis . computerized tomography showed a displaced inferior rectus and entrapment of both inferior and medial recti . the implant was removed surgically via a caldwell luc approach and his diplopia completely resolved after 33 years .

it is characterized by the presence of a congenital unilateral port - wine nevus ( flat capillary facial angioma ) affecting the area innervated by the first sensory branch of the trigeminal nerve , ipsilateral leptomeningeal angiomatosis , and calcification in the occipital or frontoparietal region . some authors define sws as a triad of the above two criteria and the third criteria as glaucoma and other vascular eye abnormalities . in type i ( classic ) sws , the individual demonstrates facial angioma , leptomeningeal angioma , and glaucoma ; in type ii sws , the patient has facial angioma and glaucoma , with no evidence of intracranial lesions ; in type iii sws ( rarest variant ) , the patient presents with only leptomeningeal angioma . the incidence of type iii sws is not known , although few cases are reported . in most sws cases without facial angioma , a seven - year - old boy with normal birth and development had presented with a focal complex partial seizure since nine months of age . his seizures were well controlled with valproic acid and clobazam for the initial three years . as his first mri [ figure 1 ] was normal , the antiepileptic medicines were subsequently withdrawn . since then , he has had breakthrough complex partial seizures despite being on medicine . for the last three years , he also had a hemicranial throbbing headache of moderate severity , lasting for few hours , associated with irritability and phonophobia , and intermittent vertiginous sensations , without imbalance when walking . his physical examination and neuropsychological assessment did not reveal any abnormalities ; specifically , no facial port - wine stain was present . an ophthalmological examination did not reveal any abnormality , including glaucoma . for his breakthrough seizues his video electroencephalography ( veeg ) revealed right posterior head region spike and wave discharges . a recent brain computed tomography ( ct ) [ figure 2 ] showed gyral calcification over the right parietal cortex . a recent mri [ figure 3 ] showed focal cortical atrophy over the right parietal region and the gadolinium contrast revealed leptomeningeal angioma over the same region . the ct angiogram [ figure 4 ] did not show any venous or arterial malformation . we put the patient on an oxcarbazepine and clobazam combination , and on the last follow up six months later , the patient was seen to be seizure - free . axial t1 , t2 images of an unenhanced mri do not showany abnormality axial , coronal , and sagittal images of a plain ct head show gyriform calcification in the right parietal cortex . associated volume loss in the parietal lobe is noted axial , coronal , and sagittal images of a contrast - enhanced mri reveal focal enhancement with the right parietal sulci axial ct angiogram source images and reconstructed ct angiogram images do not reveal any arteriovenous malformation however , in sws without facial nevus , the age of the onset is usually variable and marked by manifestation of other clinical features of the classic sws , including epilepsy ( 75 - 90% of patients ) , mental retardation ( 50% ) or hemiplegia / hemiatrophy ( 30% ) . glaucoma is present in almost 30% of classical sws , but in sws without facial nevus , glaucoma may be absent , because the nevus is not present in v1 distribution . recurrent headaches and migraine - like attacks are other common manifestations in sws patients , which were present in our patient . typical neuroradiological features of sws include gyriform calcification in the posterior cerebral areas , focal cortical atrophy , and enlargement of the choroid plexus on the side of the pial angioma . these abnormalities are more evident on a ct scan , whereas , a brain mri commonly reveals leptomeningeal enhancement after contrast infusion of gadolinium , and mr angiography detects abnormal veins draining into the deep venous circulation . in addition , functional neuroimaging investigations in sws may show areas of hypometabolism and hypoperfusion around the vascular malformation . the pathogenesis of sws is probably because of the abnormal persistence of an embryological venous plexus . the close proximity of the ectoderm , destined to form the facial skin , to the portion of the neural tube , destined to form the parieto - occipital area of the brain , may explain the association of a facial nevus and leptomeningeal angioma . we propose that the abnormal persistence of the embryonic venous plexus only in the neural tube destined to form the parieto - occipital region , but not in the ectoderm , destined to form the facial skin , is responsible for this rarest ( type iii ) form of sws . our patient has only an imaging abnormality suggestive of sws , with symptomatic epilepsy , without facial nevus , suggesting he has type iii sws , the rarest variant . the diagnosis of sws in patients without facial nevus , who present with neurological manifestations typical of sws , is made by the demonstration of a typical radiographic finding of calcification and leptomeningeal angioma . disorders known to produce cerebral calcifications , such as , celiac disease , encephalitis , purulent meningitis , ossifying meningoencephalopathy , and leukaemia , should be excluded in such cases . the necessity of recognizing this particular entity is to avoid misdiagnosis , which can often lead to incorrect management of epilepsy , including wrong choice of anti - epileptic medications . we conclude that , in the appropriate clinical scenario , the diagnosis of sws without facial nevus should be considered before labelling it as idiopathic or cryptogenic localization - related epilepsy of childhood , and a gadolinium - enhanced mri should be done in clinically highly suspicious cases .

sturge - weber syndrome ( sws ) , a rare sporadic neurocutaneous disease , is characterized by a congenital unilateral port - wine nevus affecting the area innervated by v1 , ipsilateral leptomeningeal angiomatosis , and calcification in the occipital or frontoparietal region and glaucoma / vascular eye abnormality . three types of sws have been described in literature : type i ( classic ) demonstrates facial and leptomeningeal angioma , often with glaucoma ; type ii has facial angioma and glaucoma , with no evidence of intracranial lesions ; and type iii ( rarest ) presents with only leptomeningeal angioma . only a few cases of type iii sws have been reported . here , we report a case of a seven - year - old boy with focal complex partial seizure , who was diagnosed with sws without facial nevus . recognition of this type of sws is important , as our patient had been misdiagnosed and received inappropriate antiepileptic drugs for six years . we suggest that in the appropriate clinical scenario , the diagnosis of sws without facial nevus should be considered before labelling idiopathic or cryptogenic localization - related epilepsy , and gadolinium - enhanced magnetic resonance imaging ( mri ) should be done in clinically suspicious cases of sws , without facial nevus .

opuntia vulgaris mill ( sappattukkalli in tamil , nagophema in oriya , family : cactaceae ) is a large succulent shrub common throughout india , found widely in the northern india . various parts of this plant were used in tribal medicine as bitter , laxative , stomachic , c native , antipyretic and cures biliousness , tumours , anemia , ulcers and enlargement of spleen . recently , a great deal of interest has been directed towards the bioactivity of natural plants as sources of antioxidant[68 ] . as o. vulgaris posses anti - tumor and anti - inflammatory activities which are related to antioxidant property ; the present communication deals with the in - v it m evaluation of the antioxidant activity of aerial parts of o. vulgaris . evaluation of the antioxidant activity of any drug sample or herbal extract can be carried out either by in vitro or in vivo models . various procedures are available in each model to determine the antioxidant capacity . here , the evaluation was carried out by in vitro non - enzymatic glycosylation of haemoglobin method . since non - enzymatic glycosylation of haemoglobin is an oxidation reaction , an antioxidant is expected to inhibit the reaction . the degree of haemoglycosylation in vitro in the presence of different concentration of extracts can be measured colorimetrically . the fresh stems of o. vulgaris were collected from hill area near the subarnarekha river in the district of mayurbhanj , orissa , india in the month of september . they were authenticated by dr h. j. chowdhury , additional director , central national herbarium , botanical survey of india , howrah , and west bengal , india . the voucher specimens have been preserved in our laboratory for future reference ( dm1 ) . shade - dried , powdered , sieved ( 40 mesh size ) plant materials were exhaustively extracted successively with petroleum ether ( 40 - 60 c ) , chloroform , ethyl acetate , ethanol and distilled water using a soxhlet extractor . non - enzymatic haemoglyeosylation method : the antioxidant activities of different extracts were investigated by estimating degree of non - enzymatic haemoglobin glycosylation measured colorimetrically . haemoglobin , 60 mg/ 100 ml in 0.01 m phosphate buffer ( ph 7.4 ) was incubated in presence of 2 g/ 100 ml concentration of glucose for 72 h in order to find out the best condition for haemoglobin glycosylation . the assay was performed by adding 1 ml of glucose solution , 1 ml of haemoglobin solution and 1 ml of gentamycin ( 20 mg/ 100 ml ) in 0.01 m phosphate buffer ( ph 7.4 ) . the mixture was incubated in dark at room temperature for 72 h. the degree of glycosylation of hemoglobin in the presence of different concentration of extracts and their absence were measured colorimetrically at 520 nm[1114 ] . the fresh stems of o. vulgaris were collected from hill area near the subarnarekha river in the district of mayurbhanj , orissa , india in the month of september . they were authenticated by dr h. j. chowdhury , additional director , central national herbarium , botanical survey of india , howrah , and west bengal , india . the voucher specimens have been preserved in our laboratory for future reference ( dm1 ) . shade - dried , powdered , sieved ( 40 mesh size ) plant materials were exhaustively extracted successively with petroleum ether ( 40 - 60 c ) , chloroform , ethyl acetate , ethanol and distilled water using a soxhlet extractor . non - enzymatic haemoglyeosylation method : the antioxidant activities of different extracts were investigated by estimating degree of non - enzymatic haemoglobin glycosylation measured colorimetrically . haemoglobin , 60 mg/ 100 ml in 0.01 m phosphate buffer ( ph 7.4 ) was incubated in presence of 2 g/ 100 ml concentration of glucose for 72 h in order to find out the best condition for haemoglobin glycosylation . the assay was performed by adding 1 ml of glucose solution , 1 ml of haemoglobin solution and 1 ml of gentamycin ( 20 mg/ 100 ml ) in 0.01 m phosphate buffer ( ph 7.4 ) . the mixture was incubated in dark at room temperature for 72 h. the degree of glycosylation of hemoglobin in the presence of different concentration of extracts and their absence were measured colorimetrically at 520 nm[1114 ] . results of antioxidant activity of stems of o. vulgaris mill extracts are summarized in table-1 and figure 1 , respectively . the results obtained indicated that benzene and chloroform extract of stems of o. vulgaris had better antioxidant activity than petroleum ether , ethyl acetate , ethanol and aqueous extract . benzene extract showed 40.9% , chloroform extract 36.3% , petroleum ether extract 6.2% , ethyl acetate extract 30.4% , ethanol 11.5% and aqueous extract 11.6% inhibition of haemoglobin glycosylation with a concentration of 1.0 mg / ml of each . the activities were compared with d---tocopherol ( vitamin e ) and ascorbic acid ( vitamin c ) that were used as standard antioxidant compounds . antioxidant activity of different extracts of o. vulgaris mill the antioxidant activity of different extracts of bark of o. vulgaris mill . preliminary phytochemical investigations indicated the presence of saponin and alkaloid in the stems of o. vulgaris . the detailed chemical nature of the active principle(s ) responsible for antioxidant activity and their mode of action are under investigation . percent inhibition of haemoglobin glycosylation was measured at two concentrations of petroleum ether extract , benzene extract , chloroform extract , ethyl acetate extract , ethanol extract and aqueous extract . . percent inhibition of haemoglobin glycosylation was measured at two concentrations of petroleum ether extract , benzene extract , chloroform extract , ethyl acetate extract , ethanol extract and aqueous extract . the in vitro antioxidant activity of stems of o. vulgaris mill revealed that benzene and chloroform extract of it had excellent antioxidant property . hence , the present study demonstrates the potential effectiveness of stems of o. vulgaris , which supports the claim by traditional medicine practitioners as an anti - tumor and anti - inflammatory remedy .

the in vitro antioxidant activity of stems of opuntia vulgaris mill had been investigated by estimating degree of non - enzymatic haemoglobin glycosylation measured colorimetrically at 520 urn . it was found that benzene and chloroform extract of o. vulgaris had better antioxidant activity than petroleum ether , ethyl acetate , ethanol and aqueous extract . the antioxidant activity of the extracts was concentration dependent and comparable to that of d-- tocopherol ( vitamin e ) and ascorbic acid ( vitamin c ) , standard antioxidant compounds used .

a 62-year - old male was transferred to the emergency department due to presyncopal attacks developed after taking pilsicainide . the patient has history of paroxysmal atrial fibrillation and stroke and was treated by cibenzoline 300 mg / day in combination with adequate anticoagulation . since his atrial fibrillation could not be suppressed by cibenzoline alone , his home doctor prescribed additional pilsicainide of 100 mg for pill - in - the - pocket treatment . this was a first attempt of the pill - in - the - pocket treatment for this patient . his electrocardiography ( ecg ) on arrival shows 2 to 1 atrial flutter , interventricular conduction disturbance , and coved - type st segment elevation in leads v1 - 2 consistent with type i brugada ecg1 ( figure 1 ) . his previous ecg taken during daily cibenzoline treatment antiarrhythmic drugs were terminated after admission and the ecg returned normal and his clinical course was uneventful . based on the ecg findings on admission one possibility is due to nonsustained ventricular fibrillation which was associated with brugada syndrome , and another possibility is 1 to 1 atrio - ventricular conduction during the atrial flutter . sodium channel blockers are now widely used for rhythm control therapy for paroxysmal atrial fibrillation.2 however , physicians must care for the side effects . the class ic drug can unmask brugada syndrome or induce atrial flutter called ic flutter . the pill - in - the - pocket treatment for atrial fibrillation is recognized as safe and effective therapy for the rhythm control for the atrial fibrillation.3 it is important that this therapy should be applied after confirming the drug safety to the patients .

rhythm control therapy by sodium channel blockers is widely performed for the treatment of paroxysmal atrial fibrillation . here , we present a case of acquired brugada syndrome by pill - in - the - pocket treatment using pilsicainide . it is important that this therapy should be applied only after confirming the drug safety to the patients .

the total study sample consisted of 569 subjects , 280 men and 289 women , with a mean sd age of 69.8 6.4 years . details of the 20002001 follow - up examination from the hoorn study have been described before ( 4 ) . hypertension was defined as a diastolic blood pressure 90 mmhg and/or a systolic blood pressure 140 mmhg and/or taking antihypertensive medication . glucose metabolism status was based on an oral glucose tolerance test using the world health organization 1999 diagnostic criteria ( 5 ) . neuropathy was defined as the presence of impaired foot sensitivity , assessed with semmes - weinstein monofilaments . diabetic retinopathy was defined as wisconsin grade 1.5 ( 7 ) , based on retinal photography . microalbuminuria was defined as an albumin - to - creatine ratio of > 2.0 mg / mmol ( 8) . depression was assessed using the 20-item center for epidemiologic studies depression scale ( ces - d ) ( score 16 ) ( 9 ) . diabetes - related symptom distress was measured with the revised version of the revised type 2 diabetes symptom checklist ( dsc - r ) ( 10 ) . tests ( percent ) and anova were performed to study differences in characteristics , comorbidities , and dsc - r variables for ngm , igm , and diabetes . because previous studies suggest that depression is associated with increased levels of symptom distress ( 11,12 ) , we included depression as a covariate in the anova to sort out the potential interaction between the dsc - r total scores and ngm , igm , and diabetes . mann - whitney u tests were performed for between - group comparisons regarding dsc - r , i.e. , 1 ) diabetes versus igm and 2 ) diabetes versus ngm . student 's t tests were used to determine the association between comorbidities and dsc - r scores in subjects with and without comorbidity . anova showed that worsening glucose metabolism , represented by ngm ( mean sd 6.1 7.9 ) , igm ( 6.5 7.1 ) , and diabetes ( 8.4 9.4 ) , was associated with increasing dsc - r total scores ( f = 3.1 , 2 d.f . in addition , we included depression ( ces - d score ) as a covariate in the anova to sort out the potential interaction . virtually the same dsc - r scores were found for the subjects with ngm ( 6.0 7.7 ) , igm ( 6.1 7.0 ) , and diabetes ( 8.5 9.7 ) , although scores were not statistically significant ( f = 0.99 , 2 d.f . , mann - whitney u tests revealed that diabetic patients reported a significantly greater burden of neuropathic pain ( p = 0.033 ) , sensibility symptoms ( p = 0.004 ) , and total symptom distress ( p = 0.005 ) than subjects with ngm but not those with igm ( supplemental table a1 , available in an online appendix at http://dx.doi.org/10.2337/dc08-1074 ) . subjects with ischemic heart disease had a significantly higher total dsc - r score compared with subjects with nonischemic heart disease . most strikingly , both the dsc - r score total and all subscale scores appeared to be 3-fold higher for subjects with depression ( ces - d score 16 ) than for those without depression at all three stages of glucose metabolism ( table 1 ) . this is the first study to demonstrate the association between glucose metabolism status and the level of diabetes - related symptom distress using the dsc - r score . the results presented provide supportive evidence of the validity and reliability of the dsc - r . the fact that subjects with depression reported significantly higher dsc - r levels compared with those without depression suggests that negative affect has a strong amplifying effect on diabetes symptom burden , representing higher illness intrusiveness . the association between diabetes symptoms and depressive mood could also be bidirectional , with diabetes symptoms contributing to the development of depressive symptoms ( 13 ) . yet , even after correction for depression we found that diabetic subjects report higher levels of diabetes symptom distress than subjects with ngm or igm , underscoring the importance of glucose metabolism status . in individuals screened for type 2 diabetes , relatively high levels of symptom distress likewise , in patients with established diabetes , high symptom distress despite relatively good glycemic control may point to elevated levels of depression . new longitudinal research on this complex relationship is warranted to further understand underlying mechanisms and to develop effective therapeutic strategies . the strengths of our study are the use of data from a population - based sample , the use of a standard measurement to determine glucose metabolism status ( i.e. , an oral glucose tolerance test ) , the availability of information on comorbidities , and the use of the validated dsc - r to determine diabetes - related symptom distress . further prospective research should help to clarify the course of symptom distress over time across different stages of glucose metabolism . in addition , determining the impact of different treatment strategies ( i.e. , diet , blood glucose lowering drugs , and insulin ) on the dsc - r levels among diabetes patients was beyond the scope of this study . however , given the increasing importance of patient - reported outcomes , future researchers should carefully explore the impact of diabetes medication on symptom distress as a measure of health - related quality of life .

objective the purpose of this study was to determine the associations between diabetes - related symptom distress , glucose metabolism status , and comorbidities of type 2 diabetes.research design and methods this was a cross - sectional sample of 281 individuals with normal glucose metabolism ( ngm ) , 181 individuals with impaired glucose metabolism ( igm ) , and 107 subjects with type 2 diabetes . we used the revised type 2 diabetes symptom checklist ( dsc - r ) to assess diabetes - related symptom distress.resultsthe total symptom distress score ( range 0100 ) was relatively low for diabetic subjects ( mean sd 8.4 9.4 ) , although it was significantly different from that for subjects with igm ( 6.5 7.1 ) and ngm ( 6.1 7.9 ) ( f = 3.1 , 2 d.f . , p = 0.046 ) . ischemic heart disease was associated with elevated dsc - r scores on three subscales , whereas depression showed higher symptom distress levels across all dsc - r domains.conclusionsworsening glucose metabolism is associated with increasing diabetes - related symptom distress . this relationship is attenuated by ischemic heart disease and particularly by depression .

in this issue of critical care , stroumpoulis and coworkers reported that a combination of vasopressin and epinephrine improve hemodynamics and return of spontaneous circulation ( rosc ) in an experimental cardiac arrest model . approximately 400,000 to 460,000 cardiac arrests occur out of the hospital each year in the usa . despite major advances in resuscitation science , overall survival from out - of - hospital cardiac arrest the patient 's initial cardiac rhythm is a principal determinant of resuscitation survival and neurologic outcome . a ventricular tachyarrhythmia ( ventricular tachycardia or fibrillation ) is the triggering event in up to 80% of cases and has the most favorable prognosis if it is treated promptly by defibrillation . approximately 25% of out - of - hospital cardiac arrest survivors require drug therapy for restoration of spontaneous circulation . however , if prompt defibrillation can not be performed and/or is unsuccessful and the resuscitation team must administer advanced life support drugs , the odds of survival to hospital discharge are under 10% . weisfeldt and becker proposed a three - phase model of resuscitation from cardiac arrest based on the changing physiologic needs of the patient : electrical , hemodynamic , and metabolic . for the first few minutes after the onset of ventricular fibrillation ( ' electrical phase ' ) , defibrillation may be all that is needed for successful resuscitation because the myocardial cells are still relatively rich in atp . after 3 to 4 minutes , depletion of myocardial atp diminishes the heart 's ability to resume effective contractions after defibrillation . attempts at defibrillation during this period are often unsuccessful or result in asystole or pulseless electrical activity . a brief period of effective cardiopulmonary resuscitation before defibrillation during this second ' hemodynamic phase ' can boost myocardial atp levels , increasing the likelihood of rosc after defibrillation . intravenous vasopressor support , using 1 mg doses of epinephrine every 5 minutes in adults or vasopressin 40 iu , is recommended by american heart association advanced cardiac life support guidelines to maximize oxygen delivery to the heart and brain and increase cellular atp . if spontaneous circulation is not restored for 8 to 9 minutes , then a cascade of cellular metabolic events usually leads to irreversible end - organ injury ( including anoxic brain damage and postresuscitation myocardial dysfunction ) . it is believed that reperfusion protection strategies mitigate cellular damages during this third ' metabolic phase ' . optimal vasopressor support during resuscitation should theoretically enhance aortic diastolic and coronary perfusion pressure as well as coronary and cerebral blood flow / oxygen delivery without increasing cellular oxygen demand . the principal hypothesis to explain why ' high dose epinephrine ' , which looked promising in animal models , did not improve survival in clinical resuscitation trials is that the increased coronary perfusion pressure did not increase myocardial oxygen delivery ( the majority of adult cardiac arrest victims , unlike most experimental animal models , have significant coronary artery narrowing ) sufficiently to offset the increased myocardial oxygen demand caused by epinephrine 's -adrenergic effects . vasopressin offers theoretical advantages over epinephrine in that it does not increase myocardial oxygen demand significantly and its receptors are relatively unaffected by acidosis . however , unlike epinephrine , it is not a myocardial stimulant . despite these differences in physiologic action , two large randomized clinical trials yielded virtually identical overall survival to hospital discharge when these agents were compared during in - hospital and out - of - hospital resuscitation in canada and europe , respectively . in the european study , survival to discharge was better with use of vasopressin than with epinephrine for the subgroup of patients in whom asystole was the initial rhythm . stroumpoulis and coworkers found that a combination of vasopressin and epinephrine resulted in higher aortic diastolic and coronary perfusion pressures , as well as better rosc , compared with that achieved with epinephrine alone in an 8-minute untreated ventricular fibrillation experimental model in large piglets . this is not a new finding , but it adds further evidence alongside the results of prior animal studies that showed that this combination improves survival [ 11 - 13 ] . there is also clinical evidence supporting use of a vasopressin / epinephrine ' combination ' ( usually in the form of sequential or alternating doses of the two drugs ) during resuscitation [ 6,14 - 16 ] . the best evidence to date comes from the out - of - hospital comparison of epinephrine versus vasopressin reported by wenzel and coworkers , which showed no difference in survival between treatment groups . however , there was better survival in the group of patients who received two blinded experimental doses of vasopressin followed by unlabelled epinephrine than in patients who received just repeated doses of epinephrine . the 2005 american heart association guidelines on cardiopulmonary resuscitation and emergency cardiovascular care recommend epinephrine ( 1 mg intravenously every 3 to 5 minutes ) and state that ' one dose of vasopressin may replace either the first or second dose of epinephrine ' . the 2005 european resuscitation council guidelines for resuscitation conclude that epinephrine , ' ... has been the standard vasopressor in cardiac arrest . there is insufficient evidence to support or refute the use of vasopressin as an alternative to , or in combination with [ epinephrine ] . ' however , based on the mounting clinical and experimental evidence , wenzel and lindner recently suggested that clinicians consider alternating epinephrine 1 mg intravenously with vasopressin 40 iu every 3 to 5 minutes . the verdict on a combination vasopressor strategy may come soon from a large ( > 2,000 patients ) prospective clinical trial that is underway in france to clarify the role of combination vasopressin / epinephrine therapy in out - of - hospital resuscitation .

optimal vasopressor support during resuscitation should theoretically enhance aortic diastolic and coronary perfusion pressure as well as coronary and cerebral blood flow / oxygen delivery without increasing cellular oxygen demand . intravenous vasopressor support , using 1 mg doses of epinephrine every 5 minutes in adults or vasopressin 40 iu , is recommended by american heart association advanced cardiac life support guidelines to maximize oxygen delivery to the heart and brain and increase cellular high energy phosphate levels . vasopressin offers theoretical advantages over epinephrine in that it does not increase myocardial oxygen demand significantly and its receptors are relatively unaffected by acidosis . however , unlike epinephrine , it is not a myocardial stimulant . despite these differences in physiologic actions , two large randomized clinical trials yielded virtually identical overall survival to hospital discharge when these agents were compared during inhospital or out - of - hospital resuscitation in canada and europe , respectively . more recent clinical and experimental evidence suggests that a combination of vasopressin and epinephrine used during resuscitation can improve hemodynamics and perhaps survival . the verdict on a combination vasopressor strategy may soon come from a large ( > 2,000 patients ) prospective clinical trial that is underway in france to clarify the role of combination vasopressin / epinephrine therapy in out - of - hospital resuscitation .

the indication of a minimally invasive therapy for a liver tumor lies in several criteria , highlighting the clinical condition of the patient , the number and size of nodules , and location of the lesions . in relation to this last point , some technical difficulties may negatively influence the effectiveness of this therapy , such as lesions located close to main vessels or biliary ducts , as well as lesions located in the liver periphery , mainly near the diaphragm . however , crossing the lung base and invasion of the pleural cavity are associated with an increased risk of complications , such as pneumothorax , hemothorax , alveolar bleeding , hemoptysis , tumor seeding , and severe postoperative pain . we report a case of radiofrequency ablation ( rfa ) of a lesion in the hepatic dome using an artificially induced pneumothorax to guarantee a safe and effective access . the institutional review board 's approval was not required for the present case report . a 60-year - old man presented with a hepatocellular nodule measuring 2.0 cm in the superior portion of segment viii [ figure 1a ] . due to difficulty of the transhepatic access and knowing the potential risks of transpulmonary access , it was decided to perform an artificially induced pneumothorax . 60-year - old man presenting with liver tumor in the hepatic dome underwent mri / ct of abdomen . ( a ) axial mri scan demonstrates a hepatocellular carcinoma nodule ( white arrow ) in the hepatic dome . ( b ) preinterventional ct image shows the same nodule ( white arrow ) after contrast media injection . a dynamic ct was obtained for identification of the target lesion and access planning [ figure 1b ] . then , a 22 g spinal needle ( spinocan , b. braun ; melsungen , germany ) was introduced to the right pleural cavity guided by ultrasound and ct [ figure 2a and b ] . initially , 100 cc of air was injected through a compact sterilized filter ( humid - vent ; louis gibeck ab , upplands vasby , sweden ) , intending to separate the visceral pleura . after that , a 14 g 20 cm single - lumen catheter ( arrow international inc , teleflex incorporated , reading , pa , usa ) was introduced by seldinger technique [ figure 2c ] . another 600 cc of air was injected under continuous clinical monitoring and repeated ct scans . the artificial pneumothorax promoted separation of the lung parenchyma , allowing a transpleural access guided by ct [ figure 2d ] . , the pneumothorax did not respond completely to aspiration by the single - lumen catheter , so it was decided to insert a 10-f wayne chest tube connected to a heimlich valve ( cook , inc , bloomington , in , usa ) in the right pleural cavity . the patient was discharged from the hospital the next morning with the chest tube placed , without symptoms . ( a ) ultrasound image shows a 22 g spinal needle ( white solid arrows ) crossing the parietal pleura ( white thin arrows ) . ( b ) ct image confirms the needle position ( white solid arrow ) within the pleural space . ( c ) a single - lumen catheter ( white solid arrows ) was placed in the right pleural cavity , allowing large amount of air injection ( white star ) . ( d ) subsequently , a radiofrequency probe ( white solid arrow ) was introduced into the middle of the lesion to be treated , through a transpleural access . the patient did not complain about pain for the next 24 h , and the drain was removed before hospital discharge . follow - up mri 2 months later demonstrated complete necrosis of the treated lesion [ figure 3 ] . 60-year - old man presenting with a hepatocellular carcinoma in the hepatic dome underwent percutaneous radiofrequency ablation . follow - up ( a ) axial and ( b ) coronal mri scans 2 months after the procedure demonstrated complete necrosis of the lesion ( white solid arrows ) . ablation of liver tumor is generally guided by ultrasonography ( us ) because of its advantages such as real - time imaging and absence of ionizing radiation , though for lesions in the hepatic dome , bowel interposition or pneumothorax could complicate needle visualization . therefore , ct is an excellent method for guidance , with great visualization of the lesion and adjacent organs along the proposed route . the ct - guided transpulmonary approach proved to be effective , however , with high incidence of complications such as major lung bleeding or pleural dissemination . to transpose the access difficulties , other techniques had been described , such as induction of saline solution or pneumothorax . injection of liquid or air in the pleural cavity decreases the incidence of complications because there is no damage to visceral pleura and lung parenchyma . despite the fact that intrathoracic saline solution can grant a great window for us guidance , ionic agents act as electrical conductor and may cause distant damages in non - target organs . an artificially induced pneumothorax , in turn , does not act as an electrical conductor or suffer from heat sink effect . in the present case , we demonstrated a safe and efficient rfa in the hepatic dome , performed according to the procedure outlined by de bare et al . for such applications , the air could be easily injected as well as aspirated by a spinal needle or catheter . percutaneous rfa of hepatocellular carcinoma has shown low complications rates , ranging from 0.9% to 5.0% . the most common complications are peritoneal hemorrhage , bile duct injury , abscess , and intestinal perforation . concerning the induced pneumothorax , some complications such as thoracic or shoulder pain , diaphragmatic paralysis , and tension pneumothorax may occur . furthermore , a track ablation could be performed carefully along the liver and the abdominal cavity . induced pneumothorax should be considered during ablation of hepatic dome lesions , mainly when the transhepatic access is not appropriate .

ablative therapies have become a great alternative to surgical treatment of hepatic nodules . some technical difficulties may negatively influence the effectiveness of this therapy , such as lesions located near the diaphragm . the transthoracic approach is commonly used to access these lesions . however , it is associated with an increased risk of complications , such as pneumothorax , hemothorax , alveolar bleeding , and others . we report a case of a radiofrequency ablation of a lesion in the hepatic dome , where an artificially induced pneumothorax was performed to guarantee a safe and effective access . the air was easily injected by a spinal needle and later aspirated by a single - lumen catheter . induced pneumothorax shoud be considered in ablation of hepatic dome lesions , mainly when the transhepatic access is not appropriate .

demonstration of coronary artery pathology in autopsies is vital for the elucidation of sudden death cases related to these lesions and for the development of new treatment approaches . congenital coronary artery anomalies are frequently observed in athletes , representing the second most common cause for athletic field deaths ( in about 20% of cases ) . the incidence of sudden cardiac death was reported to be 57% among 49 cases of anomalous left coronary artery . autopsy was conducted on a thirty - year male who died in a road traffic accident and brought dead to the hospital . heart dissection showed the absence of right coronary artery and the presence of left main trunk with unusual dominant course of the left circumflex coronary artery which was supplying whole of the right heart . however , the left anterior descending artery was normal [ figure 1 ] . cut section of the left circumflex coronary showed focal elevated areas [ figure 2 ] which microscopically showed [ figure 3 ] the features of pathological intimal thickening . the family members informed that he did not had any medical / cardiac signs and symptoms , and was absolutely normal before death . gross photograph of dissected heart showing only the left coronary artery with anterior descending and left circumflex branches gross photograph of dissected left circumflex coronary artery showing focal elevated areas in tunica intima microphotograph of the left coronary artery showing intimal thickening . coronary artery anomalies that entail a risk of sudden death are often associated with complex cardiac malformations but may occasionally be solitary . single coronary artery anomaly is most relevant clinically and associated with sudden cardiac death in young athletes and military personnel . a single coronary artery is an unusual congenital anomaly where only one coronary artery arises from the aortic trunk by a single coronary ostium and supply the entire heart . if a single common ostium is present , the pattern is considered to represent single coronary artery . the separate origin ( absent left main ) of the left anterior descending artery / left circumflex artery ( 30.4% ) and the anomalous origin of the left circumflex artery ( 27.7% ) are the two most common coronary anomalies . the anomaly observed in this case was the absent right coronary artery and an unusual dominant course of the left circumflex artery running along the posterior surface of the heart and supplying whole of the right heart , which is an extremely rare occurrence . to the best of our knowledge such an anomaly has only been reported twice in the english literature . the clinical outcome in patients with an anomalous coronary artery is heterogeneous with manifestations such as angina , syncope , myocardial infarction and sudden cardiac death . these are most common causes for sudden and unexpected death in young individuals , particularly during sporting activities and the greatest incidence of sudden death occurs during heavy physical exertion . the cause of sudden death varies from 25% for anomalous right coronary artery to 57% for anomalous left coronary artery . ischemia is the consequence of anatomical malformations , including the acute angle takeoff of the anomalous vessel , with a narrowed slit - like orifice that collapses in a valve like manner , thereby limiting the blood flow . younger patients ( 30 years old ) are reported to die suddenly or during exercise than older patients , despite their low frequency of significant atherosclerotic coronary artery disease . it was proposed that ischemia may be caused by sporadic spasm of the anomalous coronary artery induced by endothelial injury . hence one should be aware of the incidence of single coronary artery in particular , the rarest single dominant left coronary artery especially in athletes and military personnel , who can be screened and proper measures can be taken . in conclusion , we present a case with the absent right coronary artery and the presence of left main trunk with an unusual dominant course of the left circumflex coronary artery which was supplying whole of the right heart with normal left anterior descending artery . to the best of our knowledge such an anomaly has only been described twice in the english literature . performing autopsies for a better understanding of the coronary artery anomalies associated with sudden death

coronary artery anomalous course is rare , reported incidence is approximately 0.31.3% of patients undergoing coronary angiography and approximately 1% of routine autopsy examinations . a single coronary artery is an unusual congenital anomaly where only one coronary artery arises from the aortic trunk from a single coronary ostium , supplying the entire heart . we describe here a rare case with an unusual dominant left circumflex artery and absent right coronary artery .

anastomotic stricture is a potentially serious complication after uretero - ileal anastomosis in orthotopic urinary diversion . however , endourological procedures ( e.g. balloon dilatation , acucise cutting balloon , endoureterotomy or laser ureterotomy ) are minimally invasive options associated with lower morbidity but yield lower success rates . the memokath 051 stent ( pnn medical a / s , denmark ) has been shown to be an attractive long - term and cost - effective solution for uretero - ileal strictures . its thermosensitive shape memory allows softening at temperatures less than 10c and returning to a pre - formed shape and expanding at a temperature above 55c . it has a shaft diameter of 10.5 f and a fluted end that expands to 22 f. available lengths range from 30 to 200 mm . the memokath 051 has a tight spiral structure due to which it is resistant to extrinsic compression and prevents urothelial ingrowth . moreover , the stent adapts to the natural curves of the ureter , reducing the risk of ischemic injury to the ureteric wall and preserving ureteric peristalsis . we present a case of retrograde insertion of a thermo - expandable ureteric memokath 051 metal stent across a benign ureteroileal anastomotic stricture in a woman with a hautmann neobladder . a 70-year - old female patient with a history of muscle invasive bladder cancer and radical cystectomy with hautmann ileal neobladder 5 years ago has been treated for the last 2 years with balloon dilatation and conventional jj stent changes for a stricture at the level of the right uretero - ileal anastomosis . in order for renal function to be preserved in the long term , a percutaneous nephrostomy tube was placed . diagnostic work - up showed the stricture to be 3.5 cm with absence of metastatic disease . a technetium-99 m dimercaptosuccinic acid scan showed diminished function of the left renal unit ( right to left ratio 7525% ) . in order to remove the nephrostomy tube permanently , the benign nature of the stricture and the complexity of the case , insertion of a thermo - expandable ureteral memokath 051 metal stent was planned . the patient was placed in the lithotomy position with 15 elevation of the right flank . a nephrostogram under fluoroscopy was performed in order to delineate the exact length and position of the stricture . with the help of a 5 fr curved angiographic catheter , a 150 cm hydrophilic coated guide wire of 0.027 diameter was passed antegrade through the nephrostomy tube , into the neobladder . a 5 fr ureteric catheter was advanced over this wire , which was then replaced with a super stiff 0.035 diameter guide wire . by applying traction on both ends of the guide wire , a ureteric balloon dilator ( nephromax 12 fr , 4 cm , boston scientific ) was inserted and positioned at the level of the stricture and dilation was easily performed . after dilatation , a second polytetrafluoroethylene guide wire 0.035 diameter was inserted as a safety wire with the aid of a dual - lumen catheter . thereafter , a 6 cm/10.5 fr memokath 051 was back loaded to the stiff wire in a retrograde fashion according to the manufacturers instructions . after release of the stent , a nephrostogram showed free flow of the contrast from the right collecting system to the neobladder . an antegrade nephrostomogram was repeated the next day [ figure 1 ] , which showed direct passage of contrast to the neobladder and the nephrostomy tube was then closed . kidney function remained stable ( pre- and post - operative creatinine was 1.7 mg / dl ) , the patient remained asymptomatic and the tube was removed 48 h later . the patient was discharged with instructions for follow - up after 1 month and every 3 months afterwards for the first year with an xray - kub , creatinine and renal ultrasound . follow - up after 8 months shows the stent in place , stable creatinine and no dilation of the right kidney . infusion of contrast material through the nephrostomy shows contrast flow in the neobladder and confirms the right position of the stent the thermo - expandable ureteric metal stent has been available for the minimally invasive management of ureteric strictures for nearly two decades , with increasing indications of use . long - term data have shown durable relief of ureteric obstruction and support its routine use in endourology units . patency rates vary between 90% and 100% , with the most common long - term complication being that of stent migration , with a rate of 1420% . it is interesting that a 21% rate of spontaneous resolution of benign strictures has been reported after a mean time of 9 months of indwelling time . a serious drawback for its use is the stent cost , which however is offset if it is deployed for at least 12 months . the placement of a metallic stent in these strictures is more challenging than for strictures in the native ureter and requires technical expertise . access to the stricture is performed initially antegrade via the renal pelvicalyceal system , and this adds an extra difficulty for the endourologist for two reasons . first , a tortuous ureter that passes through the parietal peritoneum has to be negotiated and second , there is angulation of the anastomosis within the conduit . most authors recommend antegrade access for initial manipulations in order to establish a through - and - through guidewire to immobilize and straighten the uretero - ileal anastomosis . our case was even more demanding due to the neobladder configuration and native urethra that we had to overcome . further , distal identification of the stricture is more difficult after the dilatation , and this necessitates the accurate demarcation of the stricture at the early stages of the procedure . the stent must protrude into the conduit or the neo - bladder to be effective . during the follow - up of the thermo - expandable ureteric metal stents , the urologist should be aware that they are more prone to migration when they are placed for uretero - ileal strictures than other strictures . in our case , after 8 months the stent is still in place and , due to the high patency rate of this stent , follow - up with renal scintigraphy , at least in the short term , was not deemed necessary . such procedures are not common and further data with greater numbers and longer follow - up are required . placement of the thermo - expandable ureteric metal stent for benign uretero - ileal anastomotic stricture in orthotopic neobladder is feasible and effective .

technical challenges and increased morbidity of open reconstruction for uretero - ileal strictures have led to a search for minimal invasive treatments as an alternative solution . the insertion of a thermo - expandable ureteral memokath 051 metal stent across benign uretero - ileal anastomotic stricture in orthotopic neobladder has not been described in the english literature . herein , we describe a case of a woman with a hautmann neobladder and a 3.5 cm benign stricture of the right uretero - ileal anastomosis that was treated with insertion of a thermo - expandable ureteral memokath 051 metal stent .

a 57-year - old woman presented with a three - day history of photophobia and diplopia in the left eye . she was diagnosed with histologically confirmed nasal nktl of the right nasal cavity one month prior . the snellen visual acuity of the affected eye with correction was 1.0 / 0.63 , and the intraocular pressure in the right and left eyes was 17 and 18 mmhg , respectively . the patient had mild left eye conjunctival injection , ptosis , and swelling of the eyelid . opthalmoplegia , vitreous opacity , and an iris mass developed in the left eye ( fig . thirty - five percent of the cells obtained from the aspiration were morphologically atypical lymphocytes . skin biopsy confirmed neoplastic infiltration of small- to medium - sized pleomorphic lymphocytes with irregular nuclei , inconspicuous nucleoli , and scant cytoplasm ( fig . immunohistochemical staining was positive for cd56 ( fig . 2c ) and cd3 ( fig . the patient was diagnosed with nasal nktl with ocular involvement . after receiving radiotherapy at 900 cgy , her visual acuity improved and her anterior uveitis , vitreous opacity , and iris mass resolved ( fig . 3a and 3b ) . however , the patient 's condition continued to deteriorate despite radiotherapy and chemotherapy , and her ptosis and ophthalmoplegia gradually progressed . extranodal nktl , including nasal nktl , previously known as lethal midline granuloma , is a definitive diagnostic entity according to the world health organization lymphoma classification . however , histopathologically identical tumors may be identified at other extranodal sites , including the skin , muscle , gastrointestinal tract , liver , kidney , and retroperitoneal space . did report a case of histologically confirmed nasal nktl with intraocular involvement , however , in most cases intraocular involvement has not been confirmed histologically . the presence of intraocular involvement raises the possibility of leptomeningeal or central nervous system dissemination , and prompts consideration for external - beam radiotherapy to the eye and orbit in addition to systemic chemotherapy . nktl involving the ocular adnexa is generally a rapidly progressing disease , with a short survival from time of diagnosis , despite standard therapy . this was true in the current case as well ; the patient died three months after diagnosis . since nktl with ocular and orbital involvement is very rare and is characterized by rapid disease progression and a poor prognosis , nktl should be considered in the differential diagnosis of uveitis or orbital pseudotumor refractory to therapy .

herein , we report a case of nasal natural killer t - cell lymphoma ( nktl ) with intraocular involvement . a 57-year - old woman was referred due to a three - day history of photophobia and diplopia in the left eye . one - month previously , she was diagnosed with nasal nktl of the right nasal cavity . ophthalmic examination revealed conjunctival injection and ptosis . the left pupil was fully dilated and non - reactive to light . ocular motion was restricted on left - upper gaze . five days later , anterior uveitis developed and persisted despite topical steroid treatment . an orbital magnetic resonance imaging was without specific findings , however , ophthalmoplegia , vitreous opacity , and an iris mass were observed . a diagnostic anterior chamber aspiration was performed . aqueous humor aspiration revealed 35% morphologically atypical lymphocytes . after an intravitreal triamcinolone injection , radiotherapy and chemotherapy were administered ; this resolved the uveitis and iris mass . when refractory uveitis or orbital pseudotumor occurs in patients with nasal nktl , ocular and orbital involvement of the nktl should be considered .

we report here 2 patients with a common history of abdominal pain that was associated with acute onset of intestinal obstruction . in both patients , histopathology reports were consistent with pancreatic tissue in the submucosal part of the small bowels . a 72 year - old man presented to the er , because of acute onset of nausea , vomiting , gradual abdominal distention and severe abdominal pain . on physical exam , there were no signs of peritoneal irritation . blood tests were consistent with leukocytosis ( 17,000/mm ) , and a high crp level . ct - scan showed signs of mechanical obstruction , with some features of intestinal suffering ( fig . 1 ) .the patient was sent to the operating room after he had signed the informed consent . upon laparotomy , a well circumscribed mass about 100 cm proximal to the ileo - caecal junction , was found as the cause of this obstruction . the post - operative course was uneventful , and the histopatholgy exam was consisted with a submucosal pancreatic tissues , organized in clusters ( fig . 2 ) . a 24 year - old healthy man presented to the er for recurrent episodes of epigastric pain associated with nausea and vomiting . on physical exam ct scan showed layering effect on the small bowel consistent with enteroenteric intescusuption ( fig . the patient was sent to operating room after he had signed the informed consent . upon diagnostic laparoscopy there was a jejuno - jejunal intussusception and well circumscribed mass on the jejunum about 20 cm from the ligament of treitz . the post op course was uneventful and the histopathological exam was consistent with heterotopic pancreatic tissue on the small bowel ( fig . it s noteworthy mentioning , that both cases were reported in line with the scare criteria . a 72 year - old man presented to the er , because of acute onset of nausea , vomiting , gradual abdominal distention and severe abdominal pain . on physical exam , there were no signs of peritoneal irritation . blood tests were consistent with leukocytosis ( 17,000/mm ) , and a high crp level . ct - scan showed signs of mechanical obstruction , with some features of intestinal suffering ( fig . 1 ) .the patient was sent to the operating room after he had signed the informed consent . upon laparotomy , a well circumscribed mass about 100 cm proximal to the ileo - caecal junction , was found as the cause of this obstruction . the post - operative course was uneventful , and the histopatholgy exam was consisted with a submucosal pancreatic tissues , organized in clusters ( fig . a 24 year - old healthy man presented to the er for recurrent episodes of epigastric pain associated with nausea and vomiting . on physical exam there were no signs of peritoneal irritation . ct scan showed layering effect on the small bowel consistent with enteroenteric intescusuption ( fig . the patient was sent to operating room after he had signed the informed consent . upon diagnostic laparoscopy there was a jejuno - jejunal intussusception and well circumscribed mass on the jejunum about 20 cm from the ligament of treitz . the post op course was uneventful and the histopathological exam was consistent with heterotopic pancreatic tissue on the small bowel ( fig . it s noteworthy mentioning , that both cases were reported in line with the scare criteria . ectopic pancreas is congenital anomalies , in which pancreatic tissues lacking anatomical continuity with the pancreas , are found anywhere within the abdominal cavity . patients usually present with vague abdominal pain , , or with signs of obstruction . in 3 cases of gastric ep , in these 3 cases , patients presented signs of non - specific vague abdominal pain , and the diagnosis was made only post - operatively . in some papers , regarding its association with intestinal obstruction , few similar cases have been reported in the literature , , , , . in two cases , the obstruction was at the jejunal level , , and in two other patients it was at the level of the ileum , . laparotomy was performed on emergency basis in two cases , , where intussusception and peritonitis were diagnosed . in a young patient , ep caused gastric outlet obstruction , and patient lost about 15 kg in a short period of time . in about all cases reported in the literature , it s the best therapeutic option , for both , symptoms palliation and definitive diagnosis , especially to rule out malignant conditions . regarding our cases , both patients presented symptoms of intestinal obstruction , however , in one patient the occlusion was mechanical and situated distally , whereas in the other , the ep served most probably as an intussusceptum . in both cases , ep are rare condition , and symptomatic ones , causing intestinal occlusion are even rarer . both cases were diagnosed as intestinal occlusion and managed accordingly , and in both patients , the diagnosis was made after resection of the affected bowel segments . preoperative diagnosis is impossible , and in some cases reported in the literature , malignancy has been suspected . surgery is the best treatment option , for both , symptoms palliation and definitive histological diagnosis .

highlights2 cases of intestinal obstruction secondary to ectopic pancreas.ectopic pancreas causing intestinal occlusion is a very rare entity.in the first case , the ectopic tissue caused mechanical luminal occlusion in the distal ileal part , whereas the 2nd case presented intussusception sign , 20 cm distal to the treitz ligament.ectopic pancreas can serve as a lead point for ileo - ileal intussusception .

leiomyosarcoma ( lms ) of the inferior vena cava ( ivc ) is a slowly developing tumor which arise from smooth muscle cells of the wall of ivc in 38% cases.1 this tumor reside at the level of segment i represented by sub - renal ivc in 38% cases.2 a 50-year old patient , with no previous medical history , consulted for upper quadrant pain . ultrasound studies and computed tomography ( ct ) scan ( figures 1 , 2 ) revealed a multilobulated right mass , centered by the ivc invading along 7 cm . the right renal hilum was not invaded . magnetic resonance imaging ( mri ) showed an isointense t1 mass and hyperintense t2 emerging from the ivc adjacent to the right kidney ( figure 3 ) . , there was a sub - renal ivc tumor , totally obstructive , located 1 cm from the right renal vein ostium and 5 cm from iliac vein ( figure 4 ) . a resection of the ivc along with the tumor ( figure 5 ) was performed after clamping the ivc above the right renal vein . the post - operative period was complicated by the appearance of lower extremities deep vein thromboses at day 20 which well evolved with anticoagulant therapy . the diagnosis is established pre - operatively in only 10% cases.3 in tumors affecting segment i and ii , there is usually a colicky pain which can be either hepatic or renal . lower extremities edema is a rare manifestation which is due to the slow tumor growth that gives time for venous collateral circulation to develop . in our observation , ct - scan and abdominal mri allowed to precisely locating the tumor relatively to the ivc , its vasculature and within neighboring organs . it can be indicated when there is doubt in establishing the diagnosis or neoadjuvant chemotherapy is planned . in our case , segment i is the localization best suited for resection . if the tumor is not obstructing and limited to one venous wall , a lateral resection with suture can be done . if the tumor is large , totally obstructive , as in our case , wide excision with interruption of the ivc is performed . reconstruction is rarely performed as it is not needed in most cases ; because a well developed alternative circulation usually exist in the totally obstructive tumors.4 in suprarenal ivc , a prosthetic reconstruction might be necessary . the indications for prosthetics are incomplete obstruction of the vena cava or total resection along with many collaterals . however , prosthetic replacement remain a solution but controversial because of the risk of thrombotic events and infections . invasion of the renal veins especially right renal vein is a problem that can be encountered during surgery in tumors of segment i and ii . if the right renal vein is invaded , a reimplantation of the renal vein over the prosthetics , on the portal vein or renal autotransplantation in the iliac fossa is necessary to avoid right nephrectomy . 5 adjuvant treatment of sarcoma is controversial .

vascular leiomyosarcoma ( lms ) are unique . the inferior vena cava ( ivc ) is the most affected organ ( about 38% cases ) . we report the observation of a 50-year old woman who consulted for right upper quadrant pain . imaging studies revealed a retroperitoneal mass that mimic a lms of the ivc . the patient was operated . a resection of the ivc along with the tumor was performed without reconstruction . the management of lms is surgical and depends upon the location and tumor characteristics .

benign episodic unilateral mydriasis ( beum ) is an isolated cause of intermittent pupil asymmetry that might be caused by hyperactivity of the sympathetic nervous system or hypoactivity of the parasympathetic system . our case report of beum diagnosed in a young lady with migraine adds to the literature suggesting such an association . a 30-year - old lady with a history of migraine headache presented with a one - year history of chronic intermittent , bilateral , throbbing , and at times very severe , headache that often lasted for days and was associated with blurry vision , nausea , vomiting , photophobia , and phonophobia . severe episodes were sometimes associated with transient right eye mydriasis ( figure 1 ) , significant lower extremity weakness and at times confusion that all resolved with the resolution of her migraine episode . her past medical history was important for obesity , tobacco use , fibromyalgia , and depression . her medications at presentation were duloxetine hcl 60 mg daily , trazodone hcl 100 mg at night , gabapentin 900 mg three times a day , and clonazepam 0.5 mg twice a day as needed . she denied using illicit drugs , herbal , or other medications , including ones over the counter . physical examination revealed a fixed dilated right pupil ( figure 1 ) , decreased proximal and distal muscular strength ( 3/5 ) in the lower extremities with normal reflexes . the rest of the neurological and physical examination was unremarkable . her complete blood count , metabolic profile , and thyroid function test were unremarkable . she had normal cerebrospinal fluid analyses , head and neck magnetic resonance imaging and angiography , cervical , thoracic , and lumbar magnetic resonance imaging , electroencephalography , and electromyography of the lower extremities . based on her presentation , laboratory and test results , she was diagnosed with an unusual case of migraine with benign episodic unilateral mydriasis . she was started on divalproex sodium , initially 500 mg then increased to 1000 mg daily , which led to significant improvement of her migraine headache as well as pupil disturbance . causes of pupil asymmetry ( anisocoria ) range in seriousness from a normal , physiological condition ( seen in 20% of normal people ) to one that is immediately life - threatening , as seen with major stroke or intracranial bleed . other causes include medications , infection , aneurysm , iiird nerve palsy , closed - angle glaucoma , and trauma.1,2 benign episodic unilateral mydriasis is an isolated benign cause of pupil asymmetry.3 the episodes may be accompanied by blurred vision , orbital pain , headache , or photosensitivity.4 the underlying physiopathology is not always clear and may involve either parasympathetic deficiency or sympathetic hyperactivity affecting the iris.3,5 it can occasionally accompany migraine and some authors classify it as a limited form of ophthalmoplegic migraine . however , some cases have been described with no accompanying headache.3 the occurrence of mydriasis in ophthalmoplegic migraine can be due to functional exhaustion of parasympathetic fibers running within the iiird cranial nerve.6 other possible mechanisms include ischemia or oculomotor nerve demyelination caused by neuropeptides secreted at the level of circle of willis upon the activation of the trigeminovascular system causing edema and inflammation.7 according to one report of seven patients with beum associated with migraine , four had classic , one had common , and one had post - traumatic migraine . beum was also reported in migraine without aura or ophthalmoplegia , suggesting that it is a concomitant symptom.8,9 furthermore , a family history of migraine or headache was reported in patients with beum.4,8 a detailed medical history , including active medications , full physical examination , including a careful ophthalmic and neurological system evaluation , and if indicated , imaging studies , should be performed to rule out other possible underlying conditions that sometimes can be life - threatening . management and prognosis are determined by the underlying etiology . according to one case series report , patients with isolated benign episodic mydriasis appear to have a benign neurological prognosis , and do not require further neurodiagnostic studies.5 here we report a rare case of beum diagnosed in a young lady with a history of migraine , supporting the association between the two conditions . we call for more research to understand fully the underlying pathophysiology which would help in managing this condition .

pupil asymmetry or anisocoria can have benign or malignant causes , and be categorized as acute or chronic . it can also be a normal finding in about 20% of cases . benign episodic unilateral mydriasis is an isolated benign cause of intermittent pupil asymmetry . the exact pathophysiology is not always understood . according to one hypothesis , it is due to discordance between the sympathetic and parasympathetic systems . it is occasionally seen in patients with migraine . some authors consider it a limited form of ophthalmoplegic migraine . we report a case of benign episodic unilateral mydriasis diagnosed in a 30-year - old lady with a history of migraine who had extensive negative neurological evaluation .

birds were caught by mist - nets , banded ( ringed ) , and examined for ticks at the cernek bird ringing station ( 4136n , 3605e ) in the kizilirmak delta in turkey , an internationally important wetland area for birds ( 4 ) . we conducted the study during the spring and autumn migration seasons in 2010 and 2011 and in spring 2012 . the identified ticks were placed in tubes with steel beads and homogenized at the maximum speed ( 50 hz ) for 10 min in tissuelyser lt device ( qiagen , hilden , germany ) . rna was isolated according to the manufacturer s recommendations by using high pure viral nucleic acid kit ( roche applied science , mannheim , germany ) , but as a small modification , the homogenized tick mixture was kept at 37c for 1 h. in accordance with the manufacturer s recommendations , we obtained viral cdna using the revertaid first strand cdna synthesis kit ( thermo scientific , vilnius , lithuania ) . real - time pcr was performed by using the combination of primer pairs and the faststart taqman probe master kit ( roche applied science ) , as described by yapar et al . ( 6 ) , for each tick sample . cdna from patient samples , which previously had been determined as positive , were used as the positive control sample . cchfv small ( s ) segment ( encoding for the nucleocapsid protein ) specific primer pairs ( f3 : 3-gaatgtgcatgggttagctc-5 and r2 : 3-gacatcacaatttcaccagg-5 ) and same pcr conditions defined by schwarz et al . ( 7 ) were used in the pcr . sequence analysis was performed on the 260-bp pcr product , when positivity was detected , by using the primers of f3 and r2 in the abi 310 genetic analyzer ( applied biosystems , foster city , ca , usa ) . our sequences and genbank sequences were aligned in mega 5.1 ( http://www.megasoftware.net ) , and the phylogenetic tree was drawn on the basis of the 260 bp of the s segment of the cchfv genome . to compare the sequences and phylogenetic analysis , we used the maximum - likelihood method . we found attached ticks on 65 ( 0.5% ) of the 13,377 captured and banded birds , which represented 17 species . a total of 188 ticks collected on these birds belonged to ixodes , hyalomma , haemaphysalis , and rhipicephalus genera ( table ) . ( nymphs ) ( 127 [ 0.8% ] ) on great reed warblers ( acrocephalus arundinaceus ) ( 9.7% of banded birds ) and on european robins ( erithacus rubecula ) ( 0.5% of banded birds ) , respectively . the samsun cchfv partial sequences of the s segments obtained in this study have been deposited in genbank under the accession nos . kf727976 and kf727977 . the cchfv sequences obtained in the present study belong to genotype 4 ( figure 1 ) . phylogenetic tree based on the 260 bp of the small segment of the cchfv genome . the multiple sequence alignment was obtained by using mega 5.1 ( http://www.megasoftware.net ) , and the phylogenetic tree was constructed by the maximum - likelihood method using 1,000 bootstrap replicates of the sequence data . the tree is drawn to scale with branch length in the same unit as those of the evolutionary distance used to infer the phylogenetic tree . cchfv , crimean - congo hemorrhagic fever ; s. , south ; c. , central . ( nymphs ) on european robins , which migrate across turkey twice a year en route from their breeding sites to their wintering sites ( figure 2 ) and back . the probability of cchfv transportation by ticks among different regions and countries is high during migration of both bird species . because these birds stop several times during migration ( 9 ) , cchf in europe possibly could increase , especially at the stopover sites in southern europe , which provide suitable ecologic environments . a ) great reed warbler ( acrocephalus arundinaceus ) eastern migration routes ( red lines ) , breeding grounds ( yellow ) and wintering areas ( purple ) . bodies of water are blue , and nonbreeding / nonwintering areas are light green .. b ) european robin ( erithacus rubecula ) eastern migration routes ( red lines ) , resident grounds ( green ) , breeding grounds ( yellow ) , and wintering areas ( purple ) . bodies of water are blue , and nonbreeding / nonwintering areas are light green . although hyalomma ticks are the most commonly encountered ticks that carry cchfv in turkey , the virus also was detected in ixodid ticks , such as rhipicephalus spp . and haemaphysalis spp . picked up from humans and animals ( 10 ) . also , albayrak et al . ( 11 ) detected cchfv in i. ricinus ticks . because the 2 sequences detected showed similarity with cchfv genotype 4 , which was widespread in turkey ( 1 ) , whether the ticks were infected in turkey or infected earlier during bird migration is impossible to say . other studies have shown that cchfv could be transported by ticks on birds ( 1214 ) . by itself , transportation of infected ticks by birds might not be sufficient to cause the epidemics in turkey , but along with this , climate changes , environmental changes , increased number of sensitive animals , and tick and animal movements might play a role in spreading cchf ( 1 ) . in ecologically important regions , such as the kizilirmak delta , where resident and migratory birds are mixed , different microorganisms or ticks can be transferred among birds , and then carried by birds for long distances . therefore , knowing migration routes and what pathogens birds are infected with may help predict future epidemics in various countries and provide highlight the geographic regions where diseases could be detected ( 15 ) .

we investigated migratory birds role in spreading crimean - congo hemorrhagic fever virus ( cchfv ) through attached ticks . we detected cchfv rna in ticks on migratory birds in turkey . two isolates showed similarity with cchfv genotype 4 , suggesting a role for ticks in cchfv epidemics in turkey and spread of cchfv by birds .

the incidence of encephalocele is reported to vary in different parts of the world depending on the geographic region , gender of the affected infants , and nutritional supplementation during pregnancy , maternal age and parity . difficult or failed tracheal intubation is feared by all anesthesiologists , and there have been many attempts to develop a means of predicting it . difficult intubation in pediatric patients is especially important because the use of predictive tests , as a part of routine clinical practice , is limited in this patient group . the occurrence of associated congenital abnormalities might be a clue for potential difficulty of intubation . the purpose of this study is to describe the anesthetic problems encountered for these cases , and describe how these problems were managed . after our institutional ethical committee approval , we retrospectively reviewed the records of all patients with occipital encephalocele aged between 1 and 5 days , who were treated surgically in harran university hospital between january 2006 and july 2008 . hospital files of patients that had difficult intubations were evaluated for further knowledge related to difficult intubation . demographic characteristics , associated congenital abnormalities , and perioperative airway and/or respiratory complications were also recorded . all data were given as mean standard deviation ( sd ) with range with spss 11.0 ( spss inc , chicago , illinois , usa ) for windows . the demographic data of the patients is shown in table 1 . of the 17 cases , 11 were female ( 64.7% ) and 6 male ( 35.3 % ) and ages ranged from 1 to 5 days ( mean 2.1 ) , weighted between 2700 and 3500 g ( mean 3150 ) and head circumference is between 32 and 38 cm ( mean 34 ) . of these 17 patients , eight patients ( 47.1% ) were hydrocephalous , one patient ( 5.8% ) was dandy walker syndrome . micrognathia , macroglossia , restriction in neck movements were recorded as the reasons of difficulty intubation in six cases each . in all six cases , endotracheal intubation was achieved via multiple attempts and/or maneuvers , changing blades or using stylets . fiberoptic guidance and laryngeal mask airway two children were positioned in the lateral position and induced anesthesia with sevoflurane in 100% oxygen via face mask . two attempts of direct laryngoscopy ( miller blade size 0 ) in the lateral position showed just the tip of the epiglottis ( cormack - lehane grade 3 ) , and we could not intubate the child with this limited view . we lifted the baby off the operation table with the help of two residents [ figure 1 ] . we intubated the trachea with a 2.5-mm uncuffed , endotracheal tube , and proceeded uneventfully . four children were intubated with an alternative method . in this method , we used the silicone supports [ figure 2 ] . in our cases the silicone supports was put down on top of each other under the neonate body until the height matched that of the encephalocele sac . the neonate was put in the supine position with the body on the platform while a resident temporarily supported the head . the duration of surgery and anesthesia for these operations was 1.35 0.11 h and 1.51 0.19 h , respectively . no airway problems were encountered intraoperatively in our series . hemodynamics and end - tidal carbon dioxide were stable throughout surgery in every case . this retrospective study demonstrated our 3-year experience in anesthesia in 17 patients underwent surgically repairment of occipital encephalocele . reviewed the practice of anesthesia in 13,557 pediatric cases and reported that the risk of difficult intubation is higher in children with congenital malformation . anesthetic management of these children requires carefully attention because the size of occipital encephaloceles may vary from small to large masses . in this study , while one patient had a small head with a circumference of 31 cm only , another had an encephalocele with a circumference of 38 cm . sometimes we think that this method is very useful for anesthesiologists , especially in cases of large occipital encephalocele . we used this method because this method has some advantages such as needing less resident and preventing pressure on encephalocele sac and possible rupture . alternative approach is to lift the baby off the table , which we applied to two patients . we think this method is not helpful for patients with large occipital encephalocele because it needs two residents . , only one person is needed to manage the airway and pressure on the encephalocele sac is prevented . we used sevoflurane for induction and maintenance of general anesthesia for all patients because of getting rapid induction and not being irritable . we have presented here our experience in anesthesia management in patients with occipital encephalocele , who underwent surgical repair .

introduction : encephalocele , midline defect of cranial bone fusion , occurs most frequently in the occipital region . airway management in pediatric patients with craniofacial disorders poses many challenges to the anesthesiologist . the purpose of this study is to describe the airway problems encountered for such cases , and describe how these problems were managed.materials and methods : we reviewed the charts of occipital encephalocele newborn that were treated by surgical correction in harran university hospital during 20062008 . the collected data were categorized into preoperative , intraoperative , and postoperative data.results:the mean age of the patients was 5.17 days . of these 17 patients , eight patients ( 47.1% ) had hydrocephaly , one patient ( 5.8% ) with dandy walker syndrome . micrognathia , macroglossia , restriction in neck movements were recorded as the reasons in six cases each . no major anesthetic complication was found.conclusions:we reported perioperative management in 17 occipital encephalocele infant . comprehensive care during peroperative period is essential for successful outcome .

patients with eos usually present in the fourth and fifth decades of life unlike skeletal osteosarcoma patients who present in the fifth and sixth decades of life . there are also reports of eos involving the face , breast , abdominal wall , soft tissues of the back and retroperitoneum , and site of a vaccination scar . found that 10% of the patients had previous irradiation and 13% of the patients had history of trauma related to the site of eos . the genetic change most commonly associated with osteosarcoma is the loss of the p53 tumor suppressor gene on chromosome 17 and loss of retinoblastoma gene rb1 on chromosome 13 . a genetic association between paget 's disease and osteosarcoma has also been identified on chromosome 18q . other genomic changes , such as loss on 13q and 15q and gain on 1q and 8q , have been detected in patients with eos . the contribution of the major susceptibility genes on chromosome 10q ( ret proto - oncogene ) is well established in hd . in addition , potential modifying associations exist with chromosomes 2 , 9 , 13 , 20 , 21 , and 22 . variations of main ret proto - oncogene account for as much as 50% of familial and 2030% of sporadic cases . the etiology of the majority of sporadic cases is not clear , appearing to arise from combined cumulative effects of susceptibility loci on other chromosomes controlling the mechanism of cell proliferation , differentiation , and maturation . presence of genitourinary abnormalities in the form of horse - shaped kidney and azoospermia , and sporadic hd pointed toward a genetic abnormality in our patient . hence , we performed genetic screening at the common sites for both hd and osteosarcoma on chromosome 10 ( ret proto - oncogene ) and chromosome 17 ( p53 tumor suppressor gene ) . however , we found no abnormalities at both these loci , suggesting a role of combined cumulative effects of susceptibility genes on other chromosomes . inactivation or alteration of a gene located at this susceptibility loci could have been an early event in the development of eos in our patient . the cornerstone of treatment consists of radical surgery and polychemotherapy . due to the rare nature of this disease , no data from randomized studies concerning the type of chemotherapeutic combinations are available . in a small retrospective study , goldstein - jackson et al . reported favorable results when eoss were treated as conventional osteosarcomas . active chemotherapeutic agents for skeletal osteosarcoma consist of a combination of doxorubicin and cisplatin , with or without the addition of other drugs such as methothrexate , ifosfamide , and etoposide . the use of multi - agent chemotherapy has extensively improved the outcome for skeletal osteosarcoma . however , these treatments are very intensive , and therefore acute and delayed toxicities can be expected . doxorubicin can cause bone marrow toxicity and cardiotoxicity , whereas cisplatin can cause nephro - and ototoxicity . adjuvant radiotherapy does not increase the survival when patients are treated with effective surgery and chemotherapy , and increase the risk for secondary tumors . however , radiation should be considered in the setting of an unresectable or incompletely resected primary tumor . in this case , the tumor was completely resected , but the resection margins were close due to the orbital localization of the tumor , making wide resection impossible . the first case was an 11-year - old boy who developed an eos of the orbit following radiotherapy for retinoblastoma in infancy . the second patient was a 22-year - old otherwise healthy male with no predisposing factor . our case is the oldest patient ever detected with an eos in the orbit with no history of previous radiotherapy or trauma . one could suggest a possible association between eos and patients known hd , but this has never been reported in the literature before .

primary extraskeletal osteosarcoma ( eos ) is an extremely rare malignancy . in this report , the clinical course of a 32-year - old man presenting with proptoses is described . medical history included hirschsprung disease ( hd ) , horseshoe kidney , azoospermia , and vertebral anomalies . imaging of the orbit showed an oval , well - defined heterogeneous mass adjacent to the lateral wall of the orbit . the patient underwent a lateral orbitotomy and complete excision of the mass . the mass was not attached to the bone . histopathologic and immunohistochemical examination confirmed the diagnosis of an eos . the patient received chemotherapy and radiotherapy and is free of the disease 3 years after the diagnosis . genetic screening showed no mutations for both the ret proto - oncogene for hd and the p53 tumor suppressor gene for osteosarcoma .

proliferative vitreoretinopathy ( pvr ) occurs in 5%10% of patients and is the most common cause of failure of retinal detachment ( rd ) surgery . the silicone study further classified pvr into focal or diffuse posterior pvr , subretinal pvr , and anterior pvr.1 subretinal fibrosis is reported to occur in up to 15% of rhegmatogenous rds.24 retinal pigment epithelial cells may migrate on the detached outer retinal surface , proliferate , and differentiate into mesenchymal cells.5,6 the advent of spectral domain optical coherence tomography ( sd - oct ) enables imaging of hyperreflective subretinal fibrotic bands in chronic rds . to our knowledge , this is the first report of using sd - oct to document subretinal bands associated with chronic rd . we did not have approval from our institutional review board as it is not required at our institution ( university of miami- miller school of medicine ) for up to 3 patients . a 20-year - old female with high myopia presented with diminished peripheral vision in her right eye . her best - corrected visual acuity ( bcva ) was 20/25 oculus dexter ( od ) and 20/20 oculus sinister ( os ) . dilated fundus examination revealed a nasal shallow rd , lattice degeneration with multiple breaks superonasally , and a subretinal band inferiorly ( figure 1a ) . sd - oct demonstrated a band of subretinal fibrosis along with subretinal fluid ( figure 1b ) . however , 1 month later her bcva diminished from 20/25 to 20/40 od , which corresponded to progression of her detachment into the macula . her bcva improved to 20/25 and her retina remained attached with a persistent subretinal fibrotic band ( figure 1c ) at her 3-year follow - up . sd - oct confirmed the presence of persistent subretinal bands and resolution of subretinal fluid ( figure 1d ) . a 24-year - old male presented with no light perception vision in the right eye due to previous trauma and an rd in his left eye related to toxoplasmosis . the patient underwent three subsequent operations for recurrent rd including : 1 ) revision of the scleral buckle , 2 ) pars plana vitrectomy , sulfur hexafluoride ( sf6 ) gas injection , and repeat revision of the scleral buckle 1 month later , and 3 ) a pars plana vitrectomy , fluid air exchange , membrane peel , endolaser , and perfluoropropane ( c3f8 ) gas injection 9 years later . his bcva has remained 20/50 os , and the posterior segment examination revealed an encircling scleral buckle , peripheral laser scars , subretinal bands , and no recurrence of active toxoplasmosis was observed during his follow - up visit 37 years later ( figure 2a ) . sd - oct demonstrated a hyperreflective subretinal band corresponding to the subretinal fibrosis ( figure 2b ) . a 43-year - old female with history of high myopia and lasik ( laser - assisted in situ keratomileusis ) in both eyes presented after she noticed a visual field defect in her right eye . dilated fundus examination revealed an rd with a subretinal band temporally associated with lattice and multiple holes in the right eye ( figure 3a ) . she underwent a scleral buckling procedure with successful reattachment of her retina ( figure 3c ) . her bcva was 20/20 ou ( oculus uterque ) , and her examination by sd - oct at the 2-year follow - up showed a stable subretinal band temporally ( figure 3d ) . rd with pvr may be associated with development of fibrotic bands possibly causing failure of rd repair . based on the current classification scheme , subretinal bands are considered as part of more advanced pvr.7 however , successful reattachment after a single surgery for isolated subretinal pvr ranges from 89% to 95%.2,3,8,9 if removal of subretinal bands is required to achieve reattachment , the success rate decreases to 65% in one study.3 in addition , the presence of concomitant preretinal pvr alters management and reduces success rates.8 the current series demonstrates that successful retinal reattachment without removing subretinal bands can be accomplished by a scleral buckling procedure , with or without vitrectomy . however , subretinal bands causing a napkin - ring configuration are more likely to require pars plana vitrectomy , retinotomy , and excision of subretinal membranes . in conclusion , we present sd - oct findings of subretinal fibrotic bands in three patients who underwent successful anatomic reattachment without removal of the subretinal bands . in an era of multimodal imaging , oct studies are useful in documenting the extent and location of subretinal bands and may guide preoperative surgical assessment .

we report three patients with subretinal bands associated with retinal detachment in chronic retinal detachments who underwent successful retinal reattachment . subretinal bands before and after surgery can be identified on clinical examination and spectral domain optical coherence tomography . removal of subretinal bands is not mandatory to achieve retinal reattachment .

nasal dermoid sinus cysts ( ndsc ) are the most common congenital midline nasal lesion , accounting for 1% to 3% of all dermoid cysts , and 4% to 12% of head and neck dermoids ( 1 , 2 ) . ndscs may appear as a cystic mass or sinus opening on the midline nasal dorsum between the glabella and the columella at birth , or during early childhood ( 3 ) . complete excision of ndscs , regardless of extension , is essential to prevent recurrence , nasal deformity , infection , meningitis , and intracranial abscess formation ( 4 , 5 ) . among the surgical approaches used for the treatment of ndscs are : excision and primary closure , midline vertical incision , transverse incision , lateral rhinotomy , inverted - u incision , and external rhinoplasty ( 6 , 7 ) . open rhinoplasty is the preferred approach , since it provides advantages over the standard incisions , including better cosmetic results , wide exposure and more control over osteotomies , and better visualization of the cribriform plate ( 7 ) . the selection of the appropriate reconstruction technique , after dermoid resection , however , is also important . autologous septal or costal cartilage has been used for the repair of a dorsal defect ( 8 - 10 ) . among the limitations of autologous graft material in pediatric patients moreover , it may be necessary to reinforce the nasal dorsal skin , which is damaged by the disease itself as well as the resection procedure . thus , the availability of other graft material might help solve some of these complicated problems . here we report the successful management of ndsc using an open rhinoplasty approach and primary reconstruction using crushed septal cartilage and tutoplast - processed fascia lata ( tpfl ) . a 14-yr - old boy presented with pain , redness , and swelling of the nasal dorsum after being hit in the nose . at birth , this patient had a visible pit on the nasal dorsum , and intermittent discharge was present since early childhood . physical examination revealed a pit with discharge on a swollen and reddish dorsum ( fig . a preoperative magnetic resonance imaging scan revealed an enhancing soft tissue lesion on the nasal dorsum and a tortuous sinus tract , from the nasal skin to the nasal septum ( fig . the patient underwent complete removal of the ndsc by open rhinoplasty . following the transcollumelar and bilateral marginal incisions , both the lower and 3a ) and involving the upper lateral cartilage , dorsal septum , and nasal bones . the nasal pit was removed using a small elliptical skin incision , on the nasal dorsum and the lesion , and dissected from the upper lateral cartilages , dorsal septum , and nasal bone . however , the preexisting severe inflammation led to the rupture of the cystic lesion and severe adhesion to the surrounding tissue ; hence , complete en bloc excision could not be achieved . after removal of the lesion , there were significant nasal defects , including an open roof deformity and decreased projection of the nasal dorsum . both upper lateral cartilages were sutured to the nasal septal cartilage using 4 - 0 polydioxanone . the dorsal defect and irregularities were reconstructed using crushed cartilage harvested from the nasal septum and four - layered tpfl strips ( 3040 mm , tutoplast , tutogen medical gmbh , industriestrasse , germany ) , as described previously ( 11 ) . the tpfl was rehydrated in saline solution for more than 5 min before use and cut into long strips approximately 1 cm in width . the cephalic end of each four - layered strip had a stepladder pattern for smooth elevation of the radix area , and the caudal ends of all strips were rounded using iris scissors . this stack of tpfl strips was inserted into the dorsum , and crushed septal cartilage was inserted under the strips . this was followed by intercrural and interdomal sutures and an onlay graft to produce adequate nasal tip projection . the dorsal skin and rhinoplasty incisions were closed with 6 - 0 nylon sutures , and a nasal aqua splint was placed over the nasal dorsum . histologically , the lesion was lined by stratified squamous epithelium with adnexal structures , including hair follicles and sebaceous glands ( fig . the patient was satisfied with the cosmetic results , and there was no evidence of recurrence ( fig . most repairs of nasal defects after ndsc excision using an open rhinoplasty approach have used autologous septal and costal cartilages as graft materials for reconstruction ( 8 - 10 ) . although autologous cartilage is the material of choice for dorsal augmentation , the amount of harvested septal or conchal cartilage is often insufficient ; in addition , costal cartilage has been associated with donor site morbidity . although we initially attempted to reconstruct the defect in this patient using only septal cartilage , the amount harvested was only about 1.51.5 cm , an amount insufficient for dorsal augmentation . in the nose of asians , the septal cartilage is relatively small , and younger patients have even smaller amounts of cartilage and require preservation of the large l - strut . moreover , in this patient , some dorsal irregularity persisted , and the skin had been thinned by aggressive removal of the ruptured cystic lesion using a microdebrider . autologous fascia and costal cartilage were also available , but the patient 's parents were reluctant to approve a separate donor site incision for harvest . in addition , synthetic graft material has a considerable risk for infection and was therefore inappropriate in this boy , who had preoperative infection and inflammation . because of our considerable experience using tpfl for dorsal augmentation , we elected to use tpfl together with crushed septal cartilage to repair the dorsal defect of this patient . when tpfl is used for dorsal augmentation in rhinoplasty procedures , it can be easily cut into the desired shapes and multilayered . furthermore , the soft contour of tpfl enables it to be blended well with the overlying skin - soft tissue envelope . tpfl also has the advantage of a low infection rate , the absence of donor site morbidity , and minimal risk for displacement and extrusion . as in our patient , the sinus tract may be ruptured by severe inflammation , making it virtually impossible to completely remove the entire sinus tract . another potential benefit of tpfl is its ability to separate the remnant sinus tract from the dorsal skin , thus preventing recurrent skin infection . unpredictable resorption of tpfl has been observed in 3 ( 4.3% ) out of 69 patients reported in our previous study ( 11 ) . however , the patient did not show loss of volume at the two - year follow - up . the open rhinoplasty approach using tfpl can be a useful surgical option as illustrated by the patient presented here .

nasal dermoid sinus cysts are the most common congenital midline nasal lesion , accounting for 1% to 3% of all dermoid cysts , and 4% to 12% of all head and neck dermoids . selection of the appropriate reconstruction technique , after dermoid resection , is important for treatment . here we describe the successful management of a case with a nasal dermoid sinus cyst using an open rhinoplasty approach , and primary reconstruction using tutoplast - processed fascia lata and crushed septal cartilage .

actinomycosis , a rare chronic granulomatous infection induced by actinomyces species , colonizes the mouth , colon , and vagina ( 1 ) . mucosal disruption may lead to infection at virtually any anatomic site of immunocompromised body , such as head and neck , chest , abdomen , and pelvis region . in literature reports , about 50% of all cases appear in the head and neck region , with relatively rare appearance at other sites ( 2 ) . f - fluorodeoxyglucose ( fdg ) is a highly sensitive but not specific radiotracer that can accumulate at various inflammation and infection sites . only a few cases of actinomycosis appearance on f - fdg positron emission tomography / computed tomography ( pet / ct ) have been documented recenlty ( 23456 ) . here , we describe a rare appearance of pulmonary actinomycosis with intrapulmonary disseminated distribution and abscess formation on f - fdg pet / ct . we found that the involved bones demonstrated increased focal uptake of radiotracer on mtc - methylene diphosphonate ( mdp ) bone scintigraphy , which has not been reported to the best of our knowledge . a 41-year - old man was admitted to our hospital with complaints of cough for 8 months . in addition , he had right thoracodorsal and shoulder discomfort as well as wrist swelling pain for 4 months . physical examination found a palpable abscess at both right upper backside and right wrist . blood routine examination and serum tumor marker analysis revealed no abnormality . a chest ct scan revealed a 7.8 5.0 cm lung mass in the right upper lobe with spiculated margins and irregular chest wall infiltration , suggesting peripheral lung carcinoma . subsequently , a tc - mdp whole bone scintigraphy was performed to determine whether there were bone metastasis . 1a , posterior ) showed that the uptake of radiotracer was increased at the right third and fourth posterior ribs ( small black arrows ) as well as right multiple carpal bones ( large black arrow ) . regional chest and right wrist ( fig . 1b : b1 , single photon emission computerized tomography [ spect ] ; b2 , ct ; b3 , spect / ct fusion ) tomography imaging examinations found no osseous abnormality . the patient underwent f - fdg pet / ct for further evaluation since this imaging method had improved sensitivity compared to tc - mdp bone scintigraphy . 1d : d1 , pet ; d2 , ct ; d3 , pet / ct fusion ; fig . 1i , sagittal ) demonstrated a 7.8 5.0 cm mass in the upper lobe of the right lung with soft - tissue density and spiculated margins similar to results of the previous ct scan . the mass had intense f - fdg accumulation with maximal standardized uptake value ( suvmax ) of 13.3 . the infiltrative mass extended to adjacent chest wall , thoracodorsal muscle , and subcutaneous dermal tissues . the soft tissues at the right upper backside was swelling with a high activity ( suvmax = 9.2 ) . additionally , some opaque mottled shadows were distributed in multiple lobes of the right lung and the inferior lobe of the left lung ( fig . 1e2 , f2 , ct ; fig . 1e3 , f3 , pet / ct fusion ) . a ct - guided lung puncture biopsy was implemented to confirm the nature of the pulmonary mass . several days later , 4 - 5 ml yellowish pus was leaked out of the previous puncture pore . a little pus was collected by squeezing the puncture pinhole after sterilization . in the meantime , 0.5 finally , the patient received treatment with intravenous antibiotics ( piperacillin - sulbactam combined with levofloxacin ) followed by oral antibiotics ( cefuroxime combined with levofloxacin ) for 14 days . actinomycosis is a rare , chronic , and slowly progressive bacterial infection caused by several members of actinomyces . among six pathogenic species of actinomyces spp . , actinomyces israelii is the most common human pathogen . in our case , a less common species ( actinomyces odontolyticus ) was found . actinomycosis induces suppurative and granulomatous inflammation characterized by swelling with suppuration , sinus tract formation , and purulent discharge containing yellowish sulfur granules in severe cases . although it is usually involved in oral and cervicofacial infections , other sites of immunocompromised body can be infected . it is mainly caused by aspiration of oropharyngeal or gastrointestinal secretions into the respiratory tract ( 7 ) . the most common clinical symptoms of pulmonary actinomycosis are chest pain , productive cough , and dyspnea . these non - specific clinical presentations make pulmonary actinomycosis difficult to be diagnosed , often leading to misdiagnosis of malignancy rather than an infective disease ( 7 ) . only a few authors have recently reported pulmonary actinomycosis appearance on f - fdg pet / ct ( 4891011 ) . because there is not much difference in pet / ct findings between actinomycosis and malignancy , utilization of pet / ct to rule out malignancy seems unnecessary . therefore , pulmonary actinomycosis should be kept in mind for differential diagnosis of cases with intensive activity on f - fdg pet / ct . microbiological and pathological examinations with demonstration of actinomyces are indispensable for correct diagnosis of actinomycosis . in our case , ct - guided lung puncture biopsy was implemented and abscesses were punctured to culture the actual bacteria . to avoid contaminations with other external bacteria , the specimen should be collected prior to antimicrobial therapy and carefully transported to the laboratory in anaerobic media . in addition , histopathologic finding of yellowish sulfur granules within pus is often necessary for differential diagnosis of actinomycosis ( 12 ) . regarding treatment options , intravenous administration of penicillin should be given followed by oral penicillin or amoxicillin ( 13 ) . in cases of penicillin allergy or resistance , ceftriaxone , hyperbaric oxygen therapy may be used as an adjunct to conventional therapy when the disease process is refractory to antibiotics .

here we report a case of 41-year - old man with a soft tissue density mass at right upper lung and palpable abscesses at right upper backside and right wrist . 18f - fluorodeoxyglucose positron emission tomography / computed tomography demonstrated a 7.8 5.0 cm mass with soft - tissue density in the upper lobe of the right lung with high metabolic activity . the infiltrative mass extended to adjacent chest wall soft tissue . final diagnosis of pulmonary actinomycosis with multiple abscesses was made . the patient responded well to antibiotics treatment .

a 52-year - old male underwent evaluation for a 6-month history of intermittent abdominal pain . the pain did last 2448 h in each episode , and was associated with nausea and diarrhea . at admission , a colonoscopy was performed , which revealed a polypoid lesion in the cecum ( fig.1 ) . first reported in 1858 by mckidd 1 , intussusception of the appendix is an extremely rare entity , with an incidence of approximately 0.01% in patients undergoing appendectomy 1,2 . presentation varies from acute appendicitis symptoms to chronic abdominal pain , to alteration in bowel habit , to intermittent lower gastrointestinal bleeding . although more than 200 cases of appendiceal intussusception have been reported in the literature , very few have been diagnosed preoperatively by colonoscopy 2 . it is important to recognize the endoscopic appearance of this entity in order to avoid endoscopic removal and the potential hazard of an inadvertent polypectomy .

key clinical messageintussusception of the appendix is a rare condition . symptoms vary widely , whereas some patients are asymptomatic . diagnosis is generally challenging and few cases have been diagnosed by colonoscopy . it is important to recognize the endoscopic appearance of this entity in order to avoid the potential hazard of an inadvertent polypectomy .

neonatal tumors are rare ; however , congenital mesoblastic nephroma ( cmn ) is the most common neonatal renal tumor.12 the association between cmn and polyhyramnios , hypertension and prematurity has been well described ; and although cmn is a benign tumor , it could behave aggressively , resulting in catastrophic complications.35 we report a 28-week preterm newborn with a maternal history of polyhyramnios , hypertension in neonatal period and a right - sided renal mass which was confirmed as cmn by histopathology . a premature infant girl was born to a 28-year - old healthy woman ( g3 , p2 ) after 28 weeks of gestation . the infant was born apneic , cyanotic , and bradycardic , and was resuscitated successfully . on admission to the nicu , physical examination on the first day of life showed a palpable right flank mass occupying almost the whole of the right side of the abdomen . at the end of the first week of life , she developed hypertension that wascontrolled by hydralazine.4 serum creatinine , blood urea nitrogen and serum electrolytes were within normal limits . abdominal ultrasonography showed a 55 mm 40 mm heterogeneous solid mass involving the right kidney area . ct scan confirmed a large hypodense mass with heterogeneous enhancement arising from the right kidney with cortical enhancement ( figure 1 ) . ct scan of right kidney after stabilizing the cardio - respiratory status , the newborn underwent an exploration laporatomy at the age of 4 weeks . gross pathological examination showed a solid renal tumor weighing 50 grams and measuring 4 5 4.5 cm . no necrosis or hemorrhage microscopy revealed a moderately cellular spindle cell neoplasm infiltrating adjoining renal parenchyma , perirenal fat and renal sinus . however , the renal pelvis and calyces and the suprarenal gland were not infiltrated , no vascular invasion was seen . the kidney had an embryonic appearance ( figure 2 ) , which could be described as mesoblastic nephroma , mixed classic and cellular variant . congenital mesoblastic nephroma is the commonest neonatal renal tumor ; which mostly presents as a palpable abdominal mass.12 the differential diagnosis of a neonatal abdominal mass includes cmn , wilm 's tumor , neuroblastoma , rhabdoid tumor , clear cell sarcoma of the kidney , and renal cell carcinoma.12 although cmn is usually a benign tumor it can be aggressive in behavior.3 thus , total nephrectomy is curative.45 in this case , the abdominal mass was detected antenatally by ultrasonography . an increasing number of cases have been diagnosed antenatally especially with the widespread use of antenatal ultrasonography.67 there are two main subtypes of cmn . the classic ( typical ) type , which is the more common , is of a benign nature and has a better prognosis . the second , the cellular ( atypical ) variant , is less common and runs an unpredictable course.6 nevertheless , a mixed type , as in our case , has been reported.7 in our report , the mother had polyhydramnios , believed to be due to fetal polyurea , that required therapeutic amniocentesis . polyhydramnios , with or without hydrops fetalis , and/or preterm labor are associated with cmn.7 some cmns are associated with paraneoplastic syndromes such as hypertension ( due to hyperreninemia ) and hypercalcemia ( due to prostaglandin secretion from the tumor cells).68 our patient developed hypertension at the end of the first week of life and was controlled by hydralazine . this report is of a preterm infant girl , of 28-week gestation with cmn of mixed histology ( classic and atypical ) , polyhydramnios and hypertension . the right - sided abdominal mass was detected antenatally by ultrasonography , after which the patient underwent a total nephrectomy . based on the review of literature and the author 's knowledge , this is the youngest reported patient with cmn of mixed histology and hypertension .

congenital mesoblastic nephroma ( cmn ) is a rare renal tumor . it can be detected antenatally especially with judicious use of ultrasonography.a premature female neonate 28 weeks gestation , complicated by polyhydramnios , was born to a 28-year - old woman . an abdominal mass was detected antenatally . at the end of the first week of life , the newborn had hypertension that was controlled by hydralazine . ultrasonography and ct scan showed a right - sided renal heterogeneous solid mass . right nephrectomy was performed and the histology showed cmn .

penetrating carotid artery trauma may occur in consequence of incidental puncture during a number of procedures like the insertion of central venous catheters , which , if not performed meticulously , could give rise to a number of untoward complications.1 penetrating carotid artery trauma may also happen during many rare instances when the carotid artery is used as a portal for angiography , interventional angioplasty , extracorporeal membrane oxygenation ( ecmo ) , etc . this manuscript presents a case of neck hematoma in the wake of carotid artery trauma sustained during an unsuccessful attempt at interventional repair of aortic coarctation . the complication was treated surgically under general anesthesia , during which the cervical mass was evacuated and the coarctation was repaired . a 4-month - old boy was admitted to our university hospital due to an enlarged cervical mass following carotid attempt at interventional repair of aortic coarctation diagnosed via transthoracic echocardiography . the patient had previously been referred to the pediatric catheterization lab for the definite repair of the coarctation as well as pressure gradient correction ; however , initial attempts at introducing the guidewire into the arterial system ( first femoral , then brachial , and finally right carotid ) failed . the patient was thereafter transferred to the ward for further treatment , but his parents chose to take him home . three weeks later , the patient was admitted to the operating room for an emergency operation to evaluate and possibly evacuate the mass and also to assess and repair the carotid artery rupture and coarctation ( figures 1 and 2 ) . before the decision for surgery had been made , a doppler examination of the carotid artery ruled out a pseudoaneurysm of the carotid artery under the hematoma . volatile anesthetics , supplemented with ketamine , were planned for the patient ; and under standard non - invasive monitoring , 3mg / kg intramuscular ketamine plus 1.5% halothane was administered . with the patient breathing spontaneously under deeper levels of anesthesia , laryngoscopy showed that the glottis was not difficult for intubation . a peripheral intravenous line was established for muscle relaxants , and the trachea was intubated without any need for extra devices . additionally , an arterial line in the right brachial artery was established to commence invasive arterial monitoring . the left subclavian approach was chosen for the insertion of the central venous line at the first attempt , which demonstrated the central venous pressure waveform . the patient was operated on in the supine position with a roll below the neck to extend the neck gently and produce a thyroid position . the surgical approach was initially based on making the proximal and distal control of the right external carotid , but the hugeness of the cervical mass rendered it impossible . the skin over the mass was , therefore , gradually dissected and the mass , which was a hematoma around and inside the sternocleidomastoid muscle , appeared ( figures 3 and 4 ) . the hematoma was evacuated and the bleeding site , the punctured right external carotid , was repaired by direct suturing . subsequently , the sternocleidomastoid muscle was repaired , a hemovac drain was inserted , and the overlying skin was sutured . the patient was then placed in the right lateral decubitus position for a thoracotomy approach both to repair the coarctation of the aorta with a subclavian flap angioplasty and to close the patent ductus arteriosus ( pda ) . after this surgical procedure , the pressure gradient at the level of the coarctation was corrected , the left lung was re - expanded , the thorax was closed , and the skin was sutured . the patient was transferred to the pediatric cardiac intensive care unit and was extubated the following day . transthoracic echocardiography was indicative of no significant gradient across the repaired site of the coarctation , and the patient was discharged to the ward after 3 days . at three weeks follow - up , there was no significant lesion in the neck and transthoracic echocardiography demonstrated no residual coarctation . arterial interventions or angiography through the carotid artery , albeit not commonly practiced , may be utilized in rare cases in which the clinician has no good portal for arterial entry , e.g. in patients with weak pulses ; though it is not as much common as traumatic arterial complications in other arteries . inadvertent puncture of the common carotid artery is the most frequent complication of central venous catheter insertion and may result in central nervous system injury or airway compromise.3 one of the main untoward effects of carotid rupture , which is fortunately not common , is the effect of the enlarging mass on the upper airway . this effect may lead to gross mass effect , eventual fatal airway obstruction , and airway compromise.4 , 5 the case presented herein was a typical case of penetrating carotid artery trauma leading to a mass effect and a partially displaced airway . as a result , the coarctation was repaired in the next step after the repair of the carotid artery while the patient was in a stable situation . it is worthy of note that using the carotid artery for interventions or angiography in such cases may beget a number of deleterious effects;1 , 46 it is mandatory that this approach be taken cautiously with respect to the angiography catheter entry portal . if there is a no other arterial choice except for the carotid artery in diagnostic and interventional repair therapies of the aortic coarctation , the clinician should increase his / her attention for the possibility of post - procedure events . the reason is that in such cases , the blood pressure in the upper limb is increased due to the pathophysiology of the aortic coarctation ; the blood leakage from the ruptured artery is , therefore , much more severe than that in normal cases . such surgical cases require much more sophisticated cautious interventions on the part of the anesthesiologist , surgeon , and interventionist . this case demonstrated that a penetrating carotid artery puncture could lead to a number of life - threatening complications ; especially in a newborn with aortic coarctation .

a 4-month - old boy was admitted to our hospital following an unsuccessful attempt at interventional repair of aortic coarctation via the right carotid artery , which seemed to have given rise to the formation and growth of a cervical mass overlying the entry site . despite the initial anticipation of difficulty during intubation due to the pressure effect of the mass , anesthesia progressed uneventfully , the mass , which was a hematoma , was evacuated , and the coarctation was repaired . the patient was discharged after the operation . at three weeks follow - up , there was no significant lesion in the neck and transthoracic echocardiography demonstrated no residual coarctation .

acute epidural hemorrhage ( edh ) represents one of the most urgent neurosurgical lesions , and most frequently occur as a result of skull fracture with subsequent damage to the middle meningeal artery ( mma ) . in most cases , arterial bleeding focus could be identified and controlled in an open surgical field . on the other hand , recent advance in endovascular surgery there are some published reports to treat edh by using endovascular surgery without an open surgery.258 ) in the present study , we describe the intraoperative endovascular embolization to occlude a pseudoaneurysm and contrast leakage from the mma in case with brisk bleeding from the middle cranial fossa could not be controlled in an open surgical field . a 21-year - old female patient presented with headache via emergency room after traffic accident . computed tomography ( ct ) revealed an edh on the left temporal region ( figure 1a ) , and fractures of pelvic bone and both clavicles were also diagnosed via plane radiography . four - hour after an admission , she suffered abrupt neurological deterioration with 11 of glasgow coma scale score . follow - up ct showed an increased amount of edh ( figure 1b ) , and we decided to perform an emergency craniotomy and hematoma evacuation under the general anesthesia . during the surgery , massive bleeding from the base of middle cranial fossa was observed . even though aggressive hemostasis was attemped , we could not identify an origin of bleeding and foramen spinosum due to brain swelling and obscured surgical field . eventually , her systolic blood pressure was dropped to 60 mm hg with > 110 beat / min of heart rate . therefore , we decided to perform an intraoperative angiography after gauze packing into the middle cranial fossa . an angled 5 fr envoy guiding catheter ( cordis neurovascular , miami lakes , fl , usa ) was advanced into the left external carotid artery ( eca ) and intraoperative angiography was done . it demonstrated a large pseudoaneurysm and excessive contrast leakage from the mma which appeared torn and irregular ( figure 2a ) . the mma was catheterized using a excelsior sl-10 microcatheter ( stryker neurovascular , fremont , ca , usa ) upto the orifice of a pseudoaneurysm , and 1.4 ml of 33% n - butyl-2-cyanoacrylate ( nbca ) ( hystoacryl ; b. braun , melsungen , germany ) was infused occluding the pseudoaneurysm and mma ( figure 2b ) . completion control eca angiography was confirmed complete occlusion of pseudoaneurysm of mma and no contrast leakage from the mma ( figure 2c ) . after that , her vial sign became stable , and we could complete the operation after the achievement of complete hemostasis . postoperative ct revealed no residual edh with hyperdense materials in middle cranial fossa made by nbca . ct 4 months after the operation revealed decreased amount of hyperdense materials in middle cranial fossa and no residual hematoma ( figure 3 ) . after residual orthopedic problems were treated , she could be discharged 2 months after the brain surgery without any neurological deficits . acute edh is one of the most common intracranial traumatic lesions to require emergent surgery under general anesthesia.1 ) the bleeding source , typically a branch of the mma , can generally be controlled with bipolar diathermy . occasionally , the main trunk of the mma is ruptured because of a fracture involving the petrous bone . in these cases , hemostasis can be achieved by packing the foramen spinosum with bone wax or a combination of bone wax and surgicel.7 ) in the present case , however , we could not achieve adequate hemostasis due to the brain swelling and obscured surgical filed by brisk bleeding from the base of middle cranial fossa . it made the patient unstable during the surgery , and almost contributed to surgical mortality . endovascular access to mma is relatively easy , and embolization of mma could be achieved in a short time without systemic heparinization . we used nbca to occlude mma , and we achieved complete occlusion of pseudoaneurysm of mma . during the procedure , nbca was extravasated into the epidural space . because a dura matter was intact , however packed cottonoids and gauzes might be adhered with nbca and dura matter , however , it could be meticulously dissected with metzenbaum seizure and double - handed dissector , and almost removed . tiny pieces of cottonoids were remained , however , they did not lead to infection . intracranial pseudoaneurysms are less than 1% of all intracranial aneurysms , and mostly associated with head trauma about 70% of traumatic mma pseudoaneurysms are associated with a fracture crossing the mma in the temporal region.3 ) some authors favor an early treatment of the pseudoaneurysm of mma due to the possible severe consequences of acute and delayed hemorrhage.4568 ) whether the simple presence of a pseudoaneurysm , however , is an indication for intervention is unclear , especially in cases of small amount of edh which are not needed to operate.3 ) regardless of this controversy , intraoperative endovascular embolization of mma seems to be obviously beneficial , especially in case of brisk bleeding which could not be controlled in open surgical field . intraoperative angiography and endovascular embolization of mma was effective in achieving complete hemostasis in case with massive bleeding from the middle cranial fossa could not be controlled in an open surgical field .

a 21-year - old female presented with acute epidural hemorrhage ( edh ) on the left temporal region associated with skull fracture after traffic accident . she was neurologically deteriorated at four - hour after an admission , and follow - up computed tomography revealed increased amount of edh . under the general anesthesia , emergency craniotomy was performed . during the surgery , massive bleeding from the base of middle cranial fossa was observed . however , we could not identify an origin of bleeding and foramen spinosum due to brain swelling and obscured surgical field . consequently , her systolic blood pressure was dropped to 60 mm hg with > 110 beat / min of heart rate . therefore , we decided to perform an intraoperative angiography after gauze packing into the middle cranial fossa . intraoperative angiography showed a large pseudoaneurysm with massive contrast leakage of the middle meningeal artery ( mma ) . intraoperative endovascular embolization of the pseudoaneurysm and mma by using n - butyl-2-cyanoacrylate was done . after that , her vital sign became stable , and we could complete the operation after the achievement of adequate hemostasis . intraoperative angiography and endovascular embolization of mma was effective in achieving adequate hemostasis in case with brisk bleeding from the middle cranial fossa could not be controlled in an open surgical field .

a 6-year - old , 19 kg , child was admitted to our hospital with symptoms of proximal muscle weakness and a one - month history of difficulty climbing steps . preoperative laboratory tests were abnormal : aspartate transaminase ( ast ) 560 iu / l , alanine transaminase ( alt ) 773 iu / l , alkaline phosphatase 352 iu / l , lactate dehydrogenase ( ldh ) 4,280 iu / l , creatinine phosphokinase ( cpk ) preoperative echocardiography and pulmonary function test were considered to be unnecessary by the attending pediatrician . the patient received no premedication . upon arrival in the operating room , standard monitoring , including ekg , pulse oximetry , end - tidal capnography , transesophageal temperature probe , bispectral index ( bis vista , aspect medical systems , the netherlands ) and train - of - four monitor ( tof watch , organon , ireland ) were applied . anesthesia was induced with intravenous midazolam 1 mg , fentanyl 25 g , and continuous infusion of propofol . bis 40 - 65 was maintained and 30 ma at 2 hz tof was monitored during anesthesia . anesthesia was maintained with nitrous oxide 50% in oxygen and end - tidal co2 was maintained at 30 - 35 mmhg . intraoperative vital signs were as follows : blood pressure : 92 - 115/50 - 60 mmhg , heart rate : 100 - 107 bpm , oxygen saturation : 100% , and temperature : 36.3. propofol was stopped at the end of surgery . following recovery of tof to 90% , neuromuscular blockade was reversed with pyridostigmine 4 mg and glycopyrrolate 0.16 mg . the patient opened his eyes spontaneously , responded to verbal command , and was able to lift his head . muscular tone was re - evaluated but we did not check the tof . biopsy result confirmed duchenne muscular dystrophy . the disease is characterized by severe proximal muscle weakness , progressive degeneration , and fatty infiltration of the muscles . progressive and severe muscle atrophy and weakness cause loss of ability to ambulate by the teenager . dmd affects the muscles of respiration and is associated with dilated cardiomyopathy , which often leads to death secondary to cardiopulmonary causes . patients with dmd are vulnerable to the adverse physiologic effects of general anesthesia and procedural sedation . they are at increased risk of rhabdomyolysis , life - threatening hyperkalemia , malignant hyperthermia - like reaction and heart failure when they are exposed to inhalational anesthetics and succinylcholine . intravenous anesthetics such as propofol , ketamine and dexmedetomidine are reasonable alternatives and commonly used at variable doses [ 1 - 3 ] . however , there is no risk - free choice and a lack of prospective clinical studies to determine the safest anesthetic technique for dmd patients . in order to decrease risks such as rhabdomyolysis the responsiveness of dmd patients administered with standard dosage of nondepolarizing neuromuscular blocking agent differs from that in normal patients . delayed onset of blockade in dmd following administration of standard dose rocuronium , and the prolonged recovery from rocuronium - induced blockade necessitate the need for careful assessment of neuromuscular function . if rocuronium is administered in dmd , assessment of complete neuromuscular recovery ( tof ratio = 0.9 ) by quantitative measurement , such as acceleromyography , is mandatory . however , use of reduced electrical current for pediatric tof monitoring may be necessary [ 4 - 6 ] . using succinylcholine for the setup and stabilization of neuromuscular monitoring in children has been reported . however , this method is not appropriate for dmd . tof was not established for the child in the recovery room , and we monitored the muscle strength clinically . anesthetic induction dosage of propofol decreases with increasing patient age . as such , bolus dose used for children are much higher than that of adult . according to the pharmacokinetics of child , initial loading dose and maintenance dose target - controlled infusion ( tci ) system of propofol ( diprifusor , fresenius vial sa , france ) is based on adult pharmacokinetic model , and usage in children is limited . the limitation of age and body weight for the tci is 16 years - old and 30 kg respectively , and it is difficult to use in younger children . bispectral index monitoring has been developed as a tool for measuring the depth of anesthesia in adults , but the use of bis has generated several curious findings in children . the nervous system is functionally immature at birth , myelination is rapid during the first 2 years and completed by 7 years of age . but some studies showed the validity of bis in children older than 2 years [ 13 - 15 ] . monitoring with bis , bis is the most valid monitor to measuring the depth of anesthesia to date . in conclusion , we recommend anesthetists to pay close attention to the neurophysiologic and neuromuscular monitoring for patients with duchenne muscular dystrophic .

duchenne muscular dystrophy is a hereditary disorder characterized by progressive muscle weakness and contracture , and special care during anesthesia is needed in these patients . because inhalational anesthetics and succinylcholine can cause fatal results , intravenous anesthetics are commonly used . however , monitorings for the pediatric population are not otherwise specified . we report our experience of a 6 year - old boy that underwent muscle biopsy suspicious of muscle dystrophy under general anesthesia . the patient received midazolam , fentanyl , propofol and a small dose of rocuronium . he was monitored with bispectral index ( bis ) , acceleromyography ( tof ) . at the end of surgery , recovery of tof ratio to 90% was evaluated , followed by injection of pyridostigmine and glycopyrrolate . when reversal of neuromuscular block was confirmed quantitatively and clinically , the patient was extubated and he experienced no complication .

the association of drinking water hardness and cardiovascular diseases ( cvds ) has been studied since more than five decades ago . a study in england and wales showed that cardiovascular death - rates had a favorable effect in towns with harder water and had an adverse effect in towns with softer water . another studies showed that high levels of drinking water hardness can be protective against cvd . however , a review of ecological studies showed that the results are inconsistent between studies . a meta - analysis revealed a negative association between concentration of magnesium in water and cvd mortality . moreover , another review showed protective role of magnesium concentration in water against cvd in some case - control studies and one cohort study , but the analytical studies showed little evidence about the association of calcium and magnesium levels in drinking water and cvd risk . water hardness may be also associated with cvd risk factors , for instance positive correlations of water magnesium and calcium with blood pressure is documented . in the netherlands , another study found no overall association between calcium , magnesium or total hardness and ischemic heart disease or stroke mortality . water hardness may be also associated with cvd risk factors , for instance positive correlations of water magnesium and calcium with blood pressure is documented . case - control and cohort studies are more useful than ecological epidemiologicalstudies for investigating cause- and - effect relationships . the guidelines for drinking - water quality of the world health organization in 2011 reported that one of the case - control studies addressed the association between calcium and acute myocardial infarction and three reported the association between calcium and death from cvds . the aim of this study is to investigate the association of calcium and magnesium concentration of drinking water with cvds in urban and rural areas of a city in iran . this case - control study was conducted in 2012 in khansar county in isfahan province , iran . its area is 900 km and it includes 18 towns , 3 villages and one central city . we used the official data of the provincial health center regarding the chemical analysis data of urban and rural areas including the hardness , calcium and magnesium content of drinking water . data of patients hospitalized for cvd in the only specialty hospital of the city was gathered . by using the population of these areas , we calculated the relative frequency of cvd in urban and rural areas . we compared the frequency and relative frequency of cvds in the population studied according to the concentrations of ca and mg hardness in drinking water in 2010 and 2011 . we compared the frequency and relative frequency of cvds in the population studied according to the concentrations of ca and mg hardness in drinking water in 2010 and 2011 . the findings of the study in 2010 and 2011 are reported in table 1 . in 2010 , the increase in the calcium hardness above 72 mg / l , the prevalence of cvds in 1000 population decreased [ figure 1a ] ; in 2011 this decrease in cvds was observed for calcium hardness of more than 75 mg / l [ figure 1b ] . in 2010 , the level of mg hardness in water ranged from 23 to 57 mg / l . by increasing mg hardness level above 31 mg / l in 2010 [ figure 2a ] and above 26 mg / l in 2011 [ figure 2b ] , the number of cvd in 1000 people decrease . characteristics of variables studied in 2010 and 2011 cardiovascular patients in 1000 people according to the calcium hardness in water in ( a ) 2010 and ( b ) 2011 cardiovascular patients in 1000 people according to the magnesium hardness in water in ( a ) 2010 and ( b ) 2011 our study suggests favorable protective effects of water hardness , mainly water magnesium content , on cvds . several epidemiological studies have confirmed a negative association between water hardness and cvd among adults . however , some other studies did not confirm such relationship controversial results exist on the association of water hardness with cvd and their risk factors among the adult population . the health effects of hard water are mainly considered to be because of the effects of its dissolved salts , primarily ca and mg . some studies documented that the softer the water , the higher the cvd death - rates and suggested that water ca may have such protective role . most previous studies showed a significant inverse association between mortality from cvd and water levels of mg , but not ca levels . a review of the current literature reported that 10 of the 19 geographical studies published since 1979 , found a significant association , but two geographical studies from sweden , reported no association . moreover , one of the six case - control studies that considered ca , found significant protective effect of high ca levels ( > 70 mg / l ) in drinking water . mg is a protective element against soft - tissue calcification and has a role in prevention from myocardial infarction . many , but not all , of the geographical correlation , cohort and case - control studies suggested that a high mg water concentration protects against cvd mortality . likewise , a recent review confirmed the protective effects of water hardness , notably its ca level against many chronic diseases including cvds . the main limitation of the current study is its cross - sectional nature , thus the associations documented in this study should be considered with caution . the main limitation of the current study is its cross - sectional nature , thus the associations documented in this study should be considered with caution . water hardness , as well as calcium and magnesium content of drinking water may have a protective role against cvds . further experimental studies are necessary to determine the underlying mechanisms and longitudinal studies are required to study the clinical impacts of the current findings .

background : the aim of this study is to investigate the association of calcium and magnesium concentration of drinking water with cardiovascular disease ( cvds ) in urban and rural areas of a city in iran.methods:this case - control study was conducted in 2012 in khansar county in isfahan province , iran . we used the official data of the provincial health center regarding the chemical analysis data of urban and rural areas including the hardness , calcium and magnesium content of drinking water . data of patients hospitalized for cvd in the only specialty hospital of the city was gathered for the years of 2010 and 2011.results:in 2010 , water calcium content above 72 mg / l was associated with reduced number of cvds in 1000 population ; whereas in 2011 this decrease in cvds was observed for calcium levels of more than 75 mg / l . in 2010 , the level of water mg content ranged from 23 to 57 mg / l . by increasing mg hardness level above 31 mg / l in 2010 and above 26 mg / l in 2011 were associated with decreased number of cvds in 1000 people . decrease.conclusions:our study suggests favorable protective effects of water hardness , mainly water magnesium content , on cvds . water hardness , as well as calcium and magnesium content of drinking water may have a protective role against cvds . further experimental studies are necessary to determine the underlying mechanisms and longitudinal studies are required to study the clinical impacts of the current findings .

tight glycemic control in type 1 diabetes mellitus ( t1 dm ) patients is not possible because of hypoglycemia . diabetic patients are forced to change their lifestyle to adjust to the disease condition and survive it . the best way to manage diabetes would be to develop a therapy , which could adjust to the patient 's conditions . a 6-year - old boy presented with classic features of diabetic ketoacidosis , that is , weight loss and extreme weakness and osmotic features . the fasting blood sugar level was 300 mg / dl , postprandial glucose level was 467 mg / dl and hemoglobin a1c ( hba1c ) was 7.2% . he was administered with standard intravenous insulin and fluid , which finally brought down the fasting blood glucose level to around 120 mg / dl . his parents were worried due to frequent checking of blood glucose levels many times in a day . the patient remained unconscious in the middle of the night and was fed up with the frequent monitoring of blood sugar . the patient and the parents had severe anxiety , depression , frustration , and disgust . he was informed about degludec / injection tresiba , which is not yet approved in children because of lack of experience . the physician explained to them that there was nothing wrong in administering it and is not contra - indicated in t1 dm . the parents were also explained that insulin degludec may even help the child to convert from four injections to one injection a day , and from very frequent monitoring to once in a day . after reviewing the literature about insulin degludec , the patient was then put from basal - bolus to 2 bolus plus 1 basal and finally degludec at 16 u. over the period of time , blood sugar level came to normal at around 110 mg / dl - pre meal . the patient was trained very well that if he wanted to reduce the frequency of monitoring of blood sugar level , then he had to follow small frequent meals . this made him felt happy because once the sugar was controlled then small amount of sweets was also given . the outcomes of this case study were that in case of t1 dm the physician should not be very aggressive except during the first 2 weeks of admission . the physician should also try to convince the parents about line of treatment , and educate both the patients and the child . the dose may be gradually stabilized without being aggressive , and this also prevents frequent episodes of hypoglycemia . a 57-year - old female presented with a 13 year history of diabetes . due to the failure of oral hypoglycemic agents ( ohas ) in controlling her sugar levels , for the last 3 years , she was treated with biphasic insulin aspart 30/70 . she was a very frequent flier , a regular swimmer and socially very active , and this led her to have irregular meals . hence , she often go into frequent hypoglycemia and during the last 6 months the patient 's average blood glucose level during fasting were 170 mg / dl and postprandial glucose levels varied from 230 to 280 mg / dl . even after high sugar levels , she fortunately had normal kidney functions . patient was able to afford an insulin pump , so she was put on one . with the pump , these were the true feelings of a patient who was very active while she was on an insulin pump . the physician , after discussing with the patient , started her on insulin degludec and lifestyle modification , especially the diet component . she followed the dietary modification and over 2 months of time , fasting blood glucose was 110 mg / dl , post meals values were around 180 mg / dl . the doctor later reduced degludec from 44 u to 40 u and blood glucose was still improving without any episode of hypoglycemia in the last 3 months . the outcome of this case is that with this therapy and dietary modification , a desired level of blood glucose can be achieved , without hypoglycemic risk . an 80-year - old retired army officer , staying alone , has type 2 diabetes for the last 12 years and renal function test was normal and patient was on insulin along with other ohas . despite this , the patient was getting attacks of hypoglycemia , which scared the patient of unconsciousness and even death . the limiting factors were that the patient was staying alone and was dependent upon an attendant to get injections . during the weekends or holidays , the attendant was not on a regular time , and this led to irregular insulin injections , causing hypoglycemic episode to patient . this patient as well was put on insulin degludec and over a period the dose of degludec was also increased . the outcomes of this case are that degludec along with dietary modifications gave desired diabetes control without any hypoglycemia .

tight glycemic control in type 1 diabetes mellitus patients is associated with the risk of hypoglycemia . diabetic patients are forced to change their lifestyle to adjust to the disease condition and survive it . the best way to manage diabetes would be to develop a therapy , which could adjust to the patient 's conditions . here , i present few cases wherein switching to a long - acting basal insulin analog helped combat recurrent hypoglycemic episodes experienced by the patients .

filariasis is frequently encountered in asian , african and some of the south american countries . a large majority of the cases found in india are attributed to infection by wuchereria bancrofti , which mainly affects the lymph nodes and the lymphatic channels . we present a 30-year - old female who presented in surgical out patient department with complaints of palpable subcutaneous nodular swelling in the areolar region of the left breast for past 2 months with on and off pain , and itching and one episode of serous discharge . sonomammography was performed using a high frequency transducer of 5 - 17 mhz ( philips iu-22 ) that revealed a 6 3 mm cystic lesion containing numerous curvilinear echoes with 2 parallel walls and an anechoic central lumen , exhibiting vigorous twirling movements suggestive of filarial dance in the subcutaneous plane in the areolar region of the left breast . another 2 2 mm cystic lesion was noted deep to the above - mentioned cyst with a tubular channel interconnecting the two cysts . the cystic lesion also showed similar curvilinear echoes with vigorous twirling movement ( video 1 ) . few other tubular channels were seen adjacent to these cysts that were not converging towards the nipple and the cysts were presumed to represent focally distended lymphatic ducts ( figure 2a and b ) . real time sonogram shows characteristic vigorous twirling movement suggestive of filarial dance . color doppler examination of the lesion revealed red , blue , and mixed color doppler signals that were non - rhythmic , non - pulsatile , and rapidly changed in size and position ( figure 3a ) . spectral doppler trace showed the characteristic pattern of irregular worm movement ( figure 3b ) . fine needle aspiration of the cyst containing moving echoes yielded a straw - colored fluid and microscopic examination revealed the presence bancroftian microfilaria . there was eosinophilia in the blood ; however , the peripheral blood smear did not reveal any hemoparasite . the patient was treated with a regimen of diethylcarbamazine citrate 100 mg thrice daily for 2 weeks with a 2-week drug - free interval between each course for a period of 3 months . the follow - up ultrasound that was performed after 2 weeks of treatment showed prominent lymphatic channels in the subcutaneous tissue and significant reduction in the dilatation of the deep lymphatic channels . twirling movement of the worms was no longer seen that was suggestive of dead worms ( figure 4a and b ) . video 1 : real time sonogram shows characteristic vigorous tworling movement suggestive of filarial dance it is endemic all over india , except in jammu and kashmir , himachal pradesh , haryana , rajasthan , and the eastern states . it is distributed chiefly along the seacoast and along the banks of big rivers ( 1 ) . adult worms are found in the lymphatic vessels and lymph nodes of human beings only ; therefore , bancroftian filariasis is not a zoonotic disease . in lymphatic filariasis , the adult parasites live in the lymphatic vessels whilst their offspring , the microfilariae circulate in the peripheral blood and are available to the mosquito vectors when they feed on the human blood ( 2 ) . in lymphatic filariasis , the sites commonly involved are bronchial aspirate , cervico - vaginal smears and pleural and pericardial fluids ( 3 ) . breast lesions , though unusual , are not rare and swelling of the breast due to obstruction of the dermal lymphatics by the filarial worm has been documented ( 4 ) . lymphatic vessels of the breast get infested with the larvae , which causes lymphangitis , fibrosis , and lymphatic obstruction ( 5 ) . the patient usually presents with non - tender swelling ; the most common site being the upper outer quadrant ( 2 ) . our patient presented with small non - tender subcutaneous swelling in the left areolar region with on and off itching . however , changes of peau dorange and axillary lymphadenopathy have also been reported ( 6 ) . ultrasound is a valuable tool as real time ultrasound demonstrates the typical vigorous movement of the filarial worms termed as ( 7 ) first reported the real - time sonographic visualization of adult w.bancrofti in the scrotum of infected men , naming it the filarial dance sign. dreyer and colleagues first demonstrated the filarial dance in the breast ( 8) . ( 9 ) described the color motion artifact , which is produced by the swirling motion of the parasite . in our case also , typical filarial dance in the cystic lesion that was presumed to be a focally dilated lymphatic duct with living microfilarial worms showing color motion artifact was seen . ( 10 ) . to conclude , our case highlights the importance of sonomammography in the diagnosis of palpable and non palpable breast nodules in filarial endemic areas .

a 30-year - old woman presented with a palpable subcutaneous nodule in the areolar region of the left breast . sonomammographic examination revealed 2 cystic lesions showing typical filarial dance as vigorous twirling movement of multiple curvilinear echoes with mixed red blue color doppler signals that was non - rhythmic , nonpulsatile , and the characteristic pulse doppler trace due to irregular worm movement . real time sonographic demonstration of these typical features is pathognomonic for filariasis , especially in endemic areas and treatment should be initiated without delay on the basis of ultrasound .

advances in intensive care units as well as synchrony in multidisciplinary approaches allowed for expansion of possibilities in this field , such as conservative treatment . indications for surgical management in blunt solid abdominal organ injuries have diminished in recent years . by definition renal trauma grade iv according to aast ( the american association for surgery of trauma ) is laceration through the corticomedullary junction into the collecting system or vascular segmental renal artery ; vein injury with contained hematoma ; partial vessel laceration ; or vessel thrombosis . a 17year old male patient was referred to our hospital because of hematuria and left renal trauma grade iv due to a motorbike accident . on admission , the patient complained of left a ct scan of the abdomen revealed a grade iv trauma of the left kidney with multifragmentary renal ruptures , a large 7.5 cm retroperitoneal hematoma , and a urinoma ( figure 1 ) . shows a grade iv trauma of the left kidney with multifragmentary renal ruptures and a large retroperitoneal haematoma and urinoma 7.5 cm . the patient was thermodynamically stable and the renal function normal ( serum creatinine 0.96 mg / dl , blood urea nitrogen 21 mg / dl ) . therefore , a conservative approach was chosen and broad spectrum antibiotic administered , as well as bed rest , hydration , clinical monitoring , and urethral catheterization . ten days later he was discharged from our department and blood laboratory tests before discharge showed : hemoglobin ( 13 g / dl ) , creatinine ( 0.9 mg / ml ) , and crp ( 9.8 mg / dl ) . comparison of ct scans from two days and two weeks later revealed a decrease in the size of the hematoma from 7.5 to 3.5 cm ( figure 2 ) , with laboratory tests as follows : hemoglobin ( 14 g / dl ) , creatinine ( 0.9 mg / ml ) , and crp ( 1.3 mg / dl ) . ct scan two weeks later with a decrease of urinoma from 7.5 to 3.5 cm . radionuclide scan ( dynamic scintigraphy with 99 m technetium diethylene triamine pentacetic acid , dmsa ) revealed a left renal function of 32% and right renal function of 68% further clinical course was uneventful . at the last outpatient control , 1 year after the accident the patient was free from symptoms , renal function was normal , ct scan did not reveal hematoma or urinoma ( figure 3 ) , radionuclide scan revealed a left renal function of 38% and a right renal function of 62% , and hypertension was not noted . the vast majority of traumatic renal lacerations can be managed conservatively , avoiding additional loss of renal parenchyma . open surgical exploration is rarely indicated and is associated with a high likelihood of organ loss . especially in the case of an emergency surgical intervention in a clinically unstable patient , the nephrectomy rate nearly approaches 100% . even if primary conservative management is successful , as in this case , potential complications require the consequent follow up of these patients . apart from urinoma formation , secondary hemorrhages are seen in up to 25% of the grade iii iv renal traumas primarily caused by an arteriovenous fistula [ 4 , 5 ] . the frequency of post traumatic hypertension is estimated to be less than 5% , in our patient hypertension has not yet been recorded . according to the eau guidelines urinoma is usually managed with endourological techniques , our patient developed a urinoma that was managed conservatively . the comprehensive medical management of such patients requires lifelong regular control , as up to one third of the patients with grade iii iv renal traumas develop hypertension ( even many years after the trauma ) as well as hyperfiltration induced renal insufficiency [ 6 , 7 ] . indications for surgical management in blunt solid abdominal organ injuries have diminished in recent years . threatening bleeding and accompanying extraabdominal or intraabdominal lesion some studies verified that conservative management of major blunt renal trauma is appropriate . since nothing is known about long term outcomes in patients with severe renal trauma , prospective long term follow up studies are highly recommended .

renal trauma occurs in 15% of all trauma cases . the kidney is the most commonly injured genitourinary and abdominal organ.in this case we present a 17year old patient with a grade iv trauma of the left kidney with renal rupture , hematoma , and urinoma . he was referred to our hospital in hemodynamic stability ; therefore , a conservative approach was chosen.one year after the accident the patient was free from symptoms , and the renal function was normal ; ct scan revealed neither hematoma nor urinoma.since nothing is known on the long term outcomes in patients with severe renal traumas , long term follow up studies are highly recommended .

participants ( n = 2568 from 23 countries ) are asked to roll the die twice in the cup and to report the first roll . payment is according to reported roll , except reporting 6 earns 0 money units ( mu ; across subject pools mu in local currency are adjusted to equalise purchasing power ) . we used the same set of dice in all subject pools , and we also tested the dice for biasedness . this picture was taken by j.s . in the experimental laboratory of the university of nottingham . ( below - average ) prv countries ; the last 9 subject pools ( in red ) are from high ( above - average ) prv countries relative to the world sample of 159 countries . the horizontal black line refers to the uniform distribution implied by honest reporting and the blue step function to the distribution implied by the justified dishonesty benchmark ( jdb ) . for each subject pool we report the one - sample kolmogorov - smirnov test ( ks ) for discrete data in comparison with jdb ( ksd is the ks d value ) . stars above bars refer to binomial tests comparing the frequency of a particular claim with its predicted value under a uniform distribution . b. cumulative distributions for pooled data from subject pools from low and high prv countries , respectively . see supplementary analysis for further information . * p < 0.1 , * * p < 0.05 , * * * p < 0.01 . the blue line is a linear fit . the line marked mean claim is negatively related to a. government effectiveness ; b. constraint on executive ; c. fairness of electoral process and participation ; d. constraint on executive using the averages of the years 1890 to 1900 as a measure for distant institutional quality . see extended data table 1 and supplementary information for data description , references , and further analyses . the blue line is a linear fit . mean claim is negatively related to a. individualism ; b. traditional vs. secular - rational values ; c. survival vs. self - expression values . see extended data table 1 and supplementary information for data description , references , and further analyses . measures of prevalence of rule violations , economic and institutional variables , as well as cultural background of our subject pools data are country - level averages . control of corruption is a standard measure of corruption ; higher values indicate more corruption . shadow economy is measured in percent of the size of a country s gdp . political rights measures the fairness of electoral processes , political pluralism and participation , and the functioning of government ; higher scores indicate higher level of political rights . prevalence of rule violations is our self - constructed indicator based on a principal component analysis of control of corruption , shadow economy , and political rights . government effectiveness measures the quality of public service , independence from political pressure and policy implementation ; higher values indicate higher effectiveness . constraint on executive measures the institutionalised limitations on the arbitrary use of power by the executive ; higher values indicate better control . gdp per capita ( average of 1990 to 2000 ) is measured in us-$ 1000 ( ppp ) ) . individualism measures how important the individual is relative to the collective ; higher values indicate higher individualism . traditional vs. secular - rational values measures the importance of values such as respect for authorities ; higher scores indicate more secular values . survival vs self - expression values measure the importance of values surrounding physical and economic security ; lower scores indicate survival values are relatively more important than self - expression values . regression analysis of societal and individual determinants of dishonesty the explanatory variables are the scores of a country 's prevalence of rule violations in 2003 ; participants ' individual norms of honesty ( based on individual opinions about justifiableness of various acts of cheating ; higher scores indicate stronger norms ) ; participants ' beliefs in fairness ( the perceived fairness of most others ; a higher score indicates a higher belief ) . socio - demographic controls include age ; dummies for sex , urban residency , middle class status , being an economics student , and being religious ; and the percentage of other participants known to a participant . chi - tests reveal that socio - demographic controls are jointly insignificant in all models except model ( 2 ) , where they are weakly significant . our approach follows recent advances in the economic literature on institutions and culture ( see supplementary analysis for details and references ) . models ( 1 ) to ( 6 ) are ols ; models ( 7 ) to ( 10 ) use instrumental variables to identify causal relations . all regressions control for legal origin ( french , british , german , scandinavian ) . model ( 1 ) shows that both a frequently used measure for institutional quality ( constraint on executive ) and a frequently used measure for culture ( individualism ) are significantly correlated with prv . model ( 2 ) shows that past institutional quality ( constraint on executive in 1890 - 1900 ) can have long - lasting effects on prv . models ( 3 ) to ( 6 ) control for important variables proposed in the literature . models ( 7 ) to ( 10 ) report the results from instrumental variable estimation ( instrumented variables are in bold ) ; the instruments are assumed to have no direct impact on prv but only on the explanatory variable and thereby allow identifying a causal effect of either institutions ( as measured by constraint on executive ) or culture ( as measured by individualism ) on prv . settler mortality in european colonies ( 1600 - 1875 ) . to preserve degrees of freedom model ( 8) uses language ( grammatical rules ) and model ( 9 ) genetic distance as an instrument for culture . models ( 7 ) to ( 10 ) suggest causal effects of both the quality of institutions and culture ( individualism ) on prv .

deception is common in nature and humans are no exception1 . modern societies have created institutions to control cheating , but many situations remain where only intrinsic honesty keeps people from cheating and violating rules . psychological2 , sociological3 and economic theories4 suggest causal pathways about how the prevalence of rule violations in people 's social environment such as corruption , tax evasion , or political fraud can compromise individual intrinsic honesty . here , we present cross - societal experiments from 23 countries around the world , which demonstrate a robust link between the prevalence of rule violations and intrinsic honesty . we developed an index of the prevalence of rule violations ( prv ) based on country - level data of corruption , tax evasion , and fraudulent politics . we measured intrinsic honesty in an anonymous die - rolling experiment.5 we conducted the experiments at least eight years after the measurement of prv with 2568 young participants ( students ) who could not influence prv . we find individual intrinsic honesty is stronger in the subject pools of low prv countries than those of high prv countries . the details of lying patterns support psychological theories of honesty.6,7 the results are consistent with theories of the cultural co - evolution of institutions and values8 and show that weak institutions and cultural legacies9 - 11 that generate rule violations not only have direct adverse economic consequences but might also impair individual intrinsic honesty that is crucial for the smooth functioning of society .

interferons are well established agents for standard therapy in several malignancies , hepatitis c , idiopathic pulmonary fibrosis , and multiple sclerosis.1,2 despite this , adverse autoimmune effects associated with their use have been reported , including minimal change disease in the kidney,25 collapsing focal segmental glomerulosclerosis,6,7 membranous glomerulonephritis,8 acute renal failure,9 lupus nephritis,10,11 acute renal failure,12 and thrombotic microangiopathy.1318 these side effects are most often associated with interferon - alpha therapy , rather than interferon - beta . the mechanism for this is not clear . glomerular endothelial cells express and secrete adamts 13.17 the low activity of adamts 13 has been associated with the presence of an anti- adamts 13 igg antibody during treatment with interferon - alpha 2a,16 which could explain these adverse side effects , but no mechanism has been described to explain this with interferon - beta . in the following report , we describe a case of hemolytic uremic syndrome causing thrombotic microangiopathy and chorioretinitis after several months on treatment with interferon - beta which is much rarer . a 37-year - old woman was admitted to our hospital with acute renal failure , hypertension , subnephrotic proteinuria , nausea , and vomiting . she reported a 20-year history of multiple sclerosis , adequately controlled with steroids . she had been treated with interferon - beta due to a sensitive relapse affecting the spinal cord and both legs during the last five months . she reported a two - week history of mild fatigue and arthralgia in the left tarsus , treated with ibuprofen . on admission , she was well hydrated and , apart from pedal edema , physical examination was unremarkable . laboratory test results are shown in table 1 . a possible diagnosis of acute renal injury secondary to thrombotic microangiopathy associated with interferon - beta was suggested . chest x - ray was unremarkable and renal ultrasound showed kidneys of normal size with a normal echogenic cortex and no hydronephrosis . a kidney biopsy was performed and histological studies showed ischemic changes in 12 of 35 glomeruli studied ( figure 1a ) . some other glomeruli showed chronic glomerular microangiopathic lesions with duplication of the glomerular basement membrane ( figure 1b ) . the arterioles and intralobular arteries showed marked subintimal fibromucoid edema narrowing the lumen ( figure 1a arrows , figure 2a ) . an immunofluorescence study showed only fibrinogen deposits in the arterial wall ( figure 2b ) . the patient was finally diagnosed with thrombotic microangiopathy associated with interferon - beta , so the drug was withdrawn and immunosuppressive therapy was started with 1 mg / kg / day of prednisone because leg symptoms of multiple sclerosis had started immediately . doses of steroids were reduced and finally withdrawn over a period of one month , while glatiramer acetate was started . hematological abnormalities and serum lactate dehydrogenase levels returned to the normal range , and renal function slowly recovered a serum creatinine of 1.0 mg / dl . it is postulated that interferon - beta acts in this disease by inhibiting activation and proliferation of t cells.1,2 the drug is usually well tolerated , but constitutional side effects and autoimmune adverse events have been reported.19,20 the similarities between some manifestations of systemic lupus erythematosus , those of viral infections , and side effects of immunotherapy with recombinant interferons , such as fever , arthralgia , myalgia , and fatigue , are evident . in fact , interferon - alpha is a central mediator in systemic lupus erythematosus , and specific neutralizing antibodies are now in clinical trials for the treatment of this disease.21 our case can be immediately catalogued as a thrombotic microangiopathy - hemolytic uremic syndrome , which would explain the acute kidney injury . she had no symptoms or serological findings suggestive of systemic sclerosis , malignancy , malignant hypertension , or antiphospholipid syndrome . the presence of autoreactive antibodies , particularly antiphospholipid antibodies and antithyroid antibodies , are associated with an increased risk of interferon - beta antibodies in patients with multiple sclerosis on long - term therapy.22 however , we did not find any of these antibodies in our patient . kidney complications have not been directly attributed to multiple sclerosis.2 renal side effects including minimal change disease,25 collapsing focal segmental glomerulosclerosis,68 membranous nephropathy,9 lupus nephritis,10,11 acute renal failure,12 and thrombotic microangiopathy,1323 are most often associated with interferon therapy rather than with interferon - beta . the incidence of transient proteinuria during interferon - beta therapy is around 20% . to our knowledge , this is the fourth case of hemolytic uremic syndrome induced by interferon - beta . two of these patients were treated with corticosteroids and plasmapheresis , and another was only treated with supportive antihypertensive and antiproteinuric therapies . the fourth patient was diagnosed also with pseudo - sle and treated with immunosuppressants . in all of them the spectrum of kidney disease related to interferon - beta is shown in table 2 . the mechanism by which interferon could induce thrombotic microangiopathy lesions and nephrotic syndrome remains unclear.14 pleiotropic drugs such as interferon might disrupt complex pathways of complement regulation and play a role in endothelial damage.21 in recent years , mutation of complement system regulators ( factors h and i , and membrane cofactor protein ) have been directly implicated in the induction of atypical hemolytic uremic syndrome.24,25 however , we did not found any of these alterations in our case . furthermore , a recent publication described a case of low adamts 13 activity associated with the presence of an anti - adamts 13 igg antibody during treatment with interferon - alpha 2a.16 glomerular endothelial cells express and secrete adamts 13.17 it has been suggested that pre - eclampsia is also associated with decreased levels of adamts 13.18 the delayed appearance of thrombotic microangiopathy observed in our case and others suggest that the development of renal lesions may be the result of cumulative effects.14,22 in conclusion , thrombotic microangiopathy - hemolytic uremic syndrome induced by interferon - beta is an unusual side effect manifested as acute or subacute kidney injury . attempts should be made to detect it as soon as possible , and to clarify the mechanism of microangiopathy lesions .

interferon - beta is widely used for the treatment of relapsing multiple sclerosis . the drug is usually well tolerated , but autoimmune adverse effects , including kidney disease , have been reported . only a few cases of hemolytic uremic syndrome - thrombotic microangiopathy associated interferon - alpha have been described so far , and even fewer with beta - interferon . we report a patient who developed thrombotic microangiopathy during treatment with interferon - beta and improved after discontinuation and steroid therapy . complement cascade and antiphospholipid antibodies are investigated . the spectrum of renal diseases associated with interferon - beta treatment is also reviewed .

this case report describes an unstable ligamentous injury of the cervical spine , secondary to trauma suffered during an amateur rugby match . the proportion of these cervical spine injuries to be purely ligamentous after blunt trauma has been shown to be 0.6% , with half of this population presenting with neurological deficit . literature surrounding the incidence of cervical spine injury and associated spinal cord injury ( sci ) is reported between 0.8 and 13 times per 100 000 players per year [ 24 ] . fuller et al . estimated the incidence of spinal injuries to be 10.9 per 1000 player hours , in professional rugby . in an irish study reviewing 10 years of sport - related spinal injuries , rugby accounted for 16.3% of sports - related spinal injuries , with the majority of injuries occurred early in the season . we present a case in which trimodal imaging of the injury proved valuable , in terms of operative planning . a 33-year - old male was playing rugby union for his local club at the time of the injury . a ruch is defined as by the international rugby board ( irb ) as a phase of play in which one or more players from each team , who are on their feet , in physical contact , or close around the ball on the ground . in this driving ruck , immediately subsequent to the injury , he experienced full body paraesthesia , noting that he had pins and needles in his hands and feet , which resolved after 5 min . a logroll of the patient revealed cervical spine ( c - spine ) tenderness without further spinal tenderness . initial clinical examination did not elicit any neurological deficit . the lateral c - spine x - ray , taken as part of the trauma series , showed c4c5 subluxation , grade 1 anterolesthesis and an acute 30 kyphotic deformity at c4/5 level through the disc ( fig . 1 ) . the patient was maintained in triple immobilization and a computed tomography ( ct ) scan was obtained , this revealed bilateral c4/5 facetal subluxation with no obvious fractures ( fig figure 2:sagittal ct scan images showing a pure soft tissue bilateral c4/5 subluxation with bilateral naked facet sign . sagittal ct scan images showing a pure soft tissue bilateral c4/5 subluxation with bilateral naked facet sign . an orthopaedic consult was sought , the working diagnosis was a high - grade cervical sprain with compromise of the posterior ligamentous complex , bilateral facet joints capsules and potentially posterior longitudinal ligament ( pll ) . ultimately , to further elucidate ligamentous component of this injury and rule out disc herniation into the spinal canal , a magnetic resonance image ( mri ) of the c - spine was requested . the mr of the c - spine revealed c4/c5 subluxation ( flexion distraction injury ) with total rupture of posterior ligamentous complex including pll with acute traumatic disc herniation at c4/c5 level causing significant narrowing of the spinal canal at the level but no evidence of cord signal changes . partial injury to nuchal ligament with an acute injury to c6/c7 disc with posterior annular tear with disc extrusion , almost sequestration behind vertebral body of c7 towards left c7/t1 neural foramen , was also noted ( figs 35 ) . figure 3:sagittal t2-weighted mri images showing a disc herniation at c4/5 and a non - contiguous disc extrusion at c/67 behind the vertebral body of c7 . figure 5:axial t2 mri image at c6/7 level showing central annular tear with left paracentral disc extrusion behind the c7 vertebral body . sagittal t2-weighted mri images showing a disc herniation at c4/5 and a non - contiguous disc extrusion at c/67 behind the vertebral body of c7 . axial t2 mri image at c6/7 level showing central annular tear with left paracentral disc extrusion behind the c7 vertebral body . the impression of the spinal service was that this was an unstable ligamentous injury of the c - spine and would require operative intervention with an anterior cervical discectomy ( acdf ) for both levels with a possible c7 corpectomy to extract the herniated c6/7 disc fragment . this differed from the initial surgical plan , which was planned for a single - level surgery based on the ct findings . the patient underwent an awake fibre - optic intubation and was placed supine on a mayfield frame . general anaesthesia induced and a standard left smyth / robinson approach taken to anterior cervical spine with level c4c7 exposed and confirmed on image intensifier . the operative team proceeded to perform a discectomy at c4/c5 , with a noted tear in the pll . then the team moved to repeat a discectomy at c6/c7 , with a small midline tear in the pll noted . subsequently satisfactory decompression of the cord was achieved and stable somatosensory evoked potentials ( sseps ) and motor evoked potentials ( meps ) . standard fusion was done using a polyethyl ethylketone ( peek ) cage filled with biphasic calcium phosphate ( bcp ) and a single - level anterior locking plate was employed ( figs 6 and 7 ) . figure 6:lateral c - spine radiograph showing realignment of c - spine . figure 7:sagittal t2-weighted mri images showing decompression of the cord . patient had an uncomplicated post - operative recovery and was discharged from the hospital on day 3 . the phases of play in which the majority of cervical spine injuries occur has changed according to the literature , with more occurring in the tackle during open play since 2000 . the mechanism by which cervical spine injuries occur in rugby is subject to current academic debate . kuster et al . have proposed that these injuries are due to axial compression and associated buckling of the vertebral column , drawing upon case reports and experimental cadaveric studies . state the hyperextension model for cervical spine injury in rugby is supported with biomechanical studies displaying that flexion moments combined with axial compression and anterior shear forces can result in facet dislocations in the lower cervical spine . our patient has not returned to play rugby since his injury . in this case , the patient suffered a hyperflexion injury while playing amateur rugby . he suffered a c4/5 severe ligamentous injury with subluxation and a disc herniation associated with an extrusion of the disc at c6/7 , which was only detected with mri . the mri of the c - spine displays a pure soft tissue disc herniation at the non - contiguous level in the sub - axial cervical spine , with associated injuries giving rise to an unstable ligamentous injury of the c - spine requiring operative intervention . in conclusion , rugby football union is a common and popular sport , in which cervical spine injuries are occurring . we have described a unique cervical spine injury suffered during a rugby game and the usefulness of trimodal imaging in this case in fully characterizing the patient s injury .

the aim of this case report was to highlight the application of magnetic resonance imaging ( mri ) in elucidating serious and occult injuries in a single case of hyperflextion injury of a patient cervical spine ( c - spine ) . a chart and radiology review was performed to establish the sequence of care and how the results of imaging studies influenced the clinical management in this trauma case . plain radiographs and computed tomography ( ct ) imaging modalities of the c - spine revealed bilateral c4/c5 facetal subluxation with no obvious fractures ; however , the mr imaging of the c - spine revealed a non - contiguous and occult injury to c6/c7 disc with a posterior annular tear and associated disc extrusion . this altered the operative intervention that was initially planned . mr imaging proved an invaluable diagnostic addition in this particular case of cervical trauma in a rugby player following a hyperflextion injury , by revealing a serious non - contiguous and occult injury of the c - spine .

an oncocytoma is a tumor that is composed predominantly of polygonal oncocytes with abundant granular and intensely eosinophilic cytoplasm . it occurs in virtually every organ , most commonly the kidney , salivary glands , and thyroid . an oncocytoma that arises in the adrenal gland is very rare and usually benign and nonfunctioning . because of the unusual occurrence of functioning adrenocortical oncocytoma , particularly in childhood , we report here a case of adrenocortical oncocytoma in a girl presenting with virilization . we also discuss this case and review the literature in terms of pathologic classification and malignant potential . a 14-yr - old girl presented with deepening of the voice and excessive hair growth as well as change in body habitus since about 1 yr prior to admission . she was born at full term without perinatal problems and had no special health problems previously . her body weight was 69.5 kg ( 95 - 97 percentile ) , height was 165.1 cm ( 90 - 95 percentile ) and body mass index was 25.5 kg / m . physical examination revealed acne on the face and a male pattern of excessive hair on the chin , anterior chest wall , lower abdomen and inner thigh ( fig . her breast was tanner stage 2 , but her bone age ( 17 yr ) was advanced . we performed a hormonal study for evaluation of virilization , which revealed increased plasma testosterone ( > 1,500 ng / dl , reference range : 32 ng / dl ) and dehydroepiandrosterone sulfate ( dhea - s ) ( > 1,000 g / dl , reference range : 260 g / dl ) levels . plasma 17-hydroxyprogesterone ( 17-ohp ) was also increased ( 56.4 ng / ml , reference range : 2.3 ng / ml ) . plasma adrenocorticotrophic hormone ( acth ) was 5.1 pg / ml ( reference range : 5 - 37 pg / ml ) and plasma cortisol was 26.7 g / dl ( reference range : 4 - 11 g / dl ) . plasma lutenizing hormone ( lh ) was 0.24 miu / ml and plasma follicle - stimulating hormone ( fsh ) was < 0.1 miu / ml . the abdominal computed tomography ( ct ) scan and magnetic resonance imaging ( mri ) showed a 121214 cm - sized , well - circumscribed , huge mass which displaced the right kidney anteromedially ( fig . ct scan revealed a heterogeneous hypermetabolic mass only in the area between the liver and right kidney , which suggested a malignant retroperitoneal or adrenal tumor ( fig . we performed a low dose ( 0.5 mg q 6 hr for 48 hr ) and high dose ( 2 mg q 6 hr for 48 hr ) dexamethasone suppression test and the result of that was not compatible with cushing 's syndrome . the resected tumor was a huge encapsulated round mass , measuring 17.51514 cm in dimension and 1,100 gm in weight . . capsular or sinusoidal invasion was not noted and the subcapsular area showed a compressed normal adrenal cortex . these findings were compatible with the diagnosis of oncocytoma . at 2 weeks after removal of the adrenal tumor , virilization in an adolescent or woman can manifest as clitoral enlargement , increased muscle strength , acne , hirsutism , frontal hair thinning , deepening of the voice , and menstrual disruption due to anovulation . some of the possible causes of virilization in women are polycystic ovary syndrome , androgen - producing tumors ( of the ovaries , adrenal glands , pituitary gland ) , hypothyroidism , anabolic steroid exposure , congenital adrenal hyperplasia due to 21-hydroxylase deficiency ( late - onset ) , and hormone replacement therapy ( female - to - male ) ( 1 ) . tumors account for less than 0.2% of all pediatric neoplasms and 1.3% of all carcinomas in patients less than 20-yr - old ( 2 ) . adrenocortical tumors can be either functional or non - functional . in children , most tumors are functional , with 80 - 90% having endocrine manifestations at diagnosis and up to 94% secreting excess hormones on further evaluation ( 3 ) . most children ( 50 - 84% ) present with virilization ( pubic hair , accelerated growth and skeletal maturation , an enlarged penis or clitoris , hirsutism and acne ) due to excess androgen secretion . the adrenocortical oncocytoma is extremely rarely found ; only renal oncocytoma is commonly reported in children ( 4 ) . only a little more than 50 cases of adrenal oncocytoma have been reported in the literature ( 5 ) . the functioning adrenocortical oncocytomas have been reported so far in only 9 cases , of whom 7 patients were 40 - 60 yr of age and only 2 patients were 6 and 12 yr of age . the reported 2 children presented with virilization and pseudopuberty , and our patient also presented with virilization showing high serum testosterone and dhea levels . the major practical problem for our case was to differentiate benign from malignant adrenocortical oncocytoma after complete resection of the tumor . we considered her tumor as borderline because only one minor criterion was fulfilled according to the malignancy scoring system proposed by bisceglia ( 6 ) . as juliano et al . reported a case of metastatic adrenocortical oncocytoma ( 7 ) , we tried a metastatic work - up including a whole body bone scan , chest ct scan and pet - ct scan , which revealed negative findings . based on the borderline criteria of pathologic classification and no metastatic evidence however , she is being monitored by a physical examination and abdominal ct scan biannually for 2 yr , then annually for the next 5 yr . in conclusion , we report a very rare case of virilizing adrenocortical oncocytoma , particularly in a female child . although the malignant potential of this tumor is feeble and controversial , we must pay attention to recurrences because little is known of the tumor 's long - term natural history .

functioning adrenocortical oncocytomas are extremely rare and most reported patients are 40 - 60 yr of age . to our knowledge , only 2 cases of functioning adrenocortical oncocytomas have been reported in childhood . we report a case of functioning adrenocortical oncocytoma in a 14-yr - old female child presenting with virilization . she presented with deepening of the voice and excessive hair growth , and elevation of plasma testosterone and dehydroepiandrosterone sulfate . she had an adrenalectomy . the completely resected tumor composed predominantly of oncocytes without atypical mitosis and necrosis . a discussion of this case and a review of the literature on this entity are presented .

nosocomial infection has been recognized for over a century as both a critical problem affecting the quality of health care and a leading cause of morbidity , mortality and increased health care cost.1 stethoscopes are essential tools of the medical profession and because of their universal use might be a source of microorganisms that cause nosocomial infections . stethoscopes come in direct contact with numerous patients daily and their disinfection after each use is not an established practice . several studies in medical literature have demonstrated that many physicians stethoscopes are contaminated with pathogenic bacteria and could serve as a mode for transmission of infection.24 this phenomenon may be a particular problem in areas where the outbreak of multidrug resistant bacteria , such as , methicillin - resistant staphylococcus aureus ( mrsa ) , occurs or where patients with increased susceptibility to infection are to be found . we evaluated the problem by surveying the current practice of stethoscope hygiene among health care professionals and screened their stethoscopes for microorganisms during an outbreak of mrsa . king fahd hospital of the university is a 440-bed , primary through - tertiary care hospital located in the eastern province of saudi arabia . during a campaign to educate health care workers on the sources of mrsa , swabs were randomly taken from 100 stethoscopes used by different medical personnel in different hospital services . stethoscopes were collected while the staff completed a simple self explanatory questionnaire which explored the category of the health personnel , and the frequency of cleaning their stethoscopes ( never , once a year , once a week , daily or after each patient ) . cultures from stethoscopes were obtained by swabbing the diaphragm and the bell of the stethoscope with a sterile swab moistened with saline . these swabs were immediately streaked onto blood agar plates and incubated in air at 37c for 48 hours . cultures were identified by colony morphologic characteristics , gram stain characteristics , and standardized microbiological biochemical tests . there was , as expected , a predominance of microorganisms commonly found as cutaneous flora . results of cultures from 100 stethoscopes frequency of cleaning stethoscopes among 100 health care personnel the frequency of cleaning of stethoscope by the staff is shown in table 2 . many endemic pathogens are transmitted through hand carriage , and since the time of semmelweis , hand washing has been repeatedly shown to reduce the risk of nosocomial infections.5,6 however , transmission of infection through medical devices is also well documented . outbreaks of nosocomial infections attributed to electronic thermometers,7 blood pressure cuffs,8 and latex gloves9 have been reported . several studies have investigated the presence of pathogenic bacteria on stethoscopes as a source of infection.24 the results of our study demonstrate that stethoscopes that are utilized in clinical practice on a daily basis carry potentially pathogenic microorganisms . since normal skin flora consists primarily of gram - positive bacteria , it is not surprising that fewer gram - negative bacteria were isolated . the frequency of contamination of stethoscopes observed in this study is lower than the 70% to 100% reported in the literature.24 coagulase negative staphylococcus is a microorganism which frequently causes severe systemic infections , including catheter - associated and device - associated sepsis . however , small skin lesions are frequent and this route of exposure should not be underestimated . this is extremely important when treating patients with wounds or burns , or patients with catheters or tracheostomies . in our study , only 21% of the respondents regularly cleaned their stethoscopes . isopropyl alcohol has been shown to reduce bacterial colony counts when applied to the stethoscope diaphragm.3 regular disinfection of stethoscopes or disposable cover should be used to minimize the possibility of spreading infectious agents in hospitalized patients . this is especially important today , since hospitals now care for more immunocompromised patients than in previous times and also there is increased resistance of bacteria to available antibiotics.10

background : a stethoscope , an essential tool of the medical profession , can become a source of nosocomial infection.objective:to determine the frequency of bacterial contamination of stethoscopes as well as the practices used for cleaning them.methods:cultures were taken from 100 stethoscopes used by different medical personnel in different hospital services . the stethoscopes were collected while the staff filled in a questionnaire.results:thirty ( 30% ) out of the 100 stethoscopes surveyed were contaminated with microorganisms . the majority of organisms isolated were gram - positive bacteria ( gram positive bacilli 12% , gram - negative bacteria 9% , gram - positive cocci 9% ) . none of the stethoscopes grew methicillin - resistant staphylococcus aureus . overall , 21% of the health personnel cleaned their stethoscopes daily , 47% weekly , and 32% yearly . none of the health care workers cleaned their stethoscopes after every patient . nurses cleaned their stethoscopes more often than physicians and medical students.conclusion:stethoscopes may be important in the spread of infectious agents . their regular disinfection after use on each patient should be considered , particularly in such areas of the hospital , as the critical care units , and oncology units which house many patients with antibiotic - resistant organisms .

urinary tract infection ( uti ) is one of the most important and serious infections in children . it occurs in 35% of girls and 1% of boys up to 5 years , and peaks during infancy and toilet training period[1 , 2 ] . uti may lead to renal scarring , impaired glomerular function , early hypertension , and end stage renal disease in the future[36 ] . the periurethral area is colonized by both anaerobic and aerobic bacteria from the gastrointestinal tract , which serve as part of a normal defense barrier against pathogenic microorganisms . the most common risk factor leading to uti is urinary stasis that deranges this equilibrium . this can result from vesicoureteral reflux , bladder dysfunction , habitually infrequent or incomplete voiding , stones , and outflow obstruction due to labial adhesion or constipation[8 , 9 ] . other probable risk factors in girls include back to front wiping , poor perineal hygiene and bubble baths . in only one prospective case control study in 1996 that we found in literature , there was no difference in incidence of uti in children using different types of diapers . this study was done to investigate if the type of diapers used in infancy is a possible risk factor in uti in young girls . we compared the use of different types of diapers in two groups , ie children with definite uti and the control group without uti . this case control study was performed in girls under the age of two years hospitalized in mofid children 's hospital from october 2007 to march 2008 . female children under the age of two years admitted with their first uti were selected as cases and girls hospitalized for some other reasons ( mainly elective surgery ) , served as controls . we considered definite uti when the child had fever with positive pyuria ( urine wbc > 5 in hpp ) and positive urine culture ( colony count > 10/cc ) in clean catch or bag specimen . two groups were matched for age ( 1 month ) and for known risk factors for uti , like labial adhesion , constipation , style of buttock wiping and bubble bath . children with renal dysfunction or structural urinary tract anomaly except labial adhesion were excluded from this study . questionnaires were completed by a trained nurse ; relevant information included details of the types of diapers used prior to the first diagnosed uti , ie the use of superabsorbent , standard disposable or washable cotton diapers . the type and number of diapers used daily as well as the time of diaper change after defecation were documented . the study protocol was approved by the ethics committee of the shahid beheshti university of medical scinces . also , the data was summarized as follows : proportional values for qualitative variables and median ( minimum - maximum ) for quantitative variables . comparisons between the two groups were performed by paired t - test and mcnemar test and differences considered significant if p - value < 0.05 . one - hundred and eighteen children between 1 to 24 months were included in the study . fifty - nine pairs of cases , ( children with uti ) , and controls were matched . mean age was 9.57 months ( sd=6.06 ) in cases and 9.60 months ( sd=5.80 ) in control group . as shown in table 1 , superabsorbent diaper was used more frequently in cases than in controls ( 62.71% vs. 35.59% ; or=3.29 , p - value=0.005 ) . the difference between number of diapers used per day in controls ( 6.4 + 3.4 ) and cases ( 7.9 + 7.3 ) was not statistically significant ( p - value=0.15 ) . the time between defecation and diaper change was less than 5 minutes in majority of the children in both groups and was almost similar ( 89.83% in cases vs. 77.97% in controls , p - value=0.014 ) . there were no significant differences in other factors like level of family income and mother 's occupation in the two groups ( table 1 ) . the epidemiology of uti during childhood varies by age , gender and various other factors . the incidence of uti is highest in the first 2 years of life but decreases later in this study we have shown that the type of diaper could be a risk factor for uti in little girls . uti was seen more frequently with the use of superabsorbent diapers as compared to using other diapers . in one prospective case control study in 1996 , there was n't any difference in incidence of uti in children using three different types of diapers . the odds ratios for risk of contracting uti according to different diaper types used prior to the first uti diagnosis were 0.95 for superabsorbent diapers , 1.04 for standard disposable diapers and 1.00 for washable cotton diapers however , during the course of that study , use of cotton washable nappies had been replaced by the superabsorbent diaper , thus producing a bias and making the results unreliable . one explanation that could account for the different results in our study is the frequency of diaper changes in both groups . we expected that because of the high price of superabsorbent diapers , these types of diapers would be changed less often . however our study showed that there was no difference in number of daily diaper changes between the two groups . a logical explanation for difference in the incidence of uti between children with different types of diapers is insufficient ventilation in genital area with the use of superabsorbent diapers . the main source of uti is through the ascending route and it usually occurs as a consequence of colonization of the periurethral area by a virulent organism that subsequently gains access to the bladder , in superabsorbent diapers there is a suitable environment for bacterial overgrowth due to incomplete evaporation of urine , thus providing a ready source of infection . poor perineal hygiene and insufficient ventilation due to tight clothing is a predisposing factor for uti . in addition , it seems prudent to advise parents to keep the infant 's genital area dry thus reducing bacterial overgrowth and the consequent risk of infection . limitation of our study was an underestimation of case and control patients with washable cotton diapers , therefore we gathered children with this type of diapers and disposable diapers in one group known as ordinary diapers as washable cotton diapers are not ordinarily used nowadays , we recommend the use of diapers equipped with ventilation or with alarm systems such as a color change when wet . we also recommend that , to decrease bacterial overgrowth , the perineum should be dried thoroughly after washing all authors have no conflicts of interest to declare , and did not have any financial or non - financial conflict of interest .

objectiveurinary tract is one of the most common sources of infection in children under the age of two years . many known and unknown risk factors predispose to this important disease in children . this study was conducted to determine whether using a specific type of diaper plays a role in urinary tract infection ( uti ) in girls under the age of 2 years.methodsthis case control study was performed in hospitalized children ; girls with their first urinary tract infection were selected as cases , and those admitted for other reasons comprised the control group . two groups were matched for age ( 1 month ) , and other known risk factors for uti . type of diapers ( superabsorbent , standard disposable and washable cotton ) , used for these children during six months , from october 2007 to march 2008 , were compared in both groups.findings59 matched pair infant girls less than 2 years were selected . it was revealed that in cases with uti superabsorbent diapers were used more frequently than in controls ( odds ratio=3.29 , p - value=0.005 ) there were no significant differences in other factors like number of diapers used per day , the time between defecation and diaper change , mothers educational level , level of family income and mother 's occupation.conclusionthe use of superabsorbent diapers could be a risk factor for urinary tract infection in infant girls .

a healthy 12-year - old boy presented with complaints of a slow growing painless conjunctival mass of 3 years duration in the left eye . on examination , there was a subconjunctival , nontender , well - defined , multi - lobed , immobile , firm mass with scleral fixity at 5o - clock position of the left eye . there was no restriction of motility and the anterior and posterior segments in both eyes were within normal limits . ultrasound biomicroscopy of the lesion showed uniform internal low reflectivity . computed tomography of the orbits confirmed a well - defined mass in close relation to the globe with homogenous consistency and the extraocular muscles were seen distinctly separate from the mass . differential diagnosis based on clinical examination included atypical nodular scleritis , scleral nodule , hemangioma , parasitic cyst , or a myxoma . intraoperatively , the mass was found to be entirely subconjunctival and adherent to the sclera [ fig . however , it could be separated from the underlying sclera and excised in - toto [ fig . double freeze - thaw cryotherapy was done at its location over the sclera , and the conjunctiva was closed with 8 - 0 vicryl suture . the histopathological examination of the hematoxylin - eosin stained slides showed tumor consisting of spindle cells arranged in whorls and twisted configureuration , which were the antoni - a areas as well as the irregularly arranged , paucicellular antoni - b areas the cells were elongated with thin cytoplasmic processes , and the nuclei were arranged in parallel lines which assumed a typical picket fence the tumor cells also stained strongly positive for s100 , a highly sensitive marker for melanocytes and neural crest - derived cells ; thus confirming the diagnosis of epibulbar schwannoma [ fig . the patient did not have features of neurofibromatosis . at a follow - up of 26 months he has been advised annual follow - up and is being monitored for clinical signs suggestive of neurofibromatosis . ( a ) clinical photograph is demonstrating the nodular lesion seen as an elevation under the left lower eyelid . ( c ) intraoperative photograph showing the lesion , visible after incising the overlying conjunctiva . the mass was adherent to the sclera underneath but could be easily delineated and excised in - toto . ( d ) the excised mass was relatively avascular and firm in consistency ( a ) tumor consisting of spindle cells arranged in whorls and twisted configuration , which were the antoni - a are as ( h and e stain , 10 ) . ( b ) the slender schwannoma cells showing strong immunopositivity to s100 stain ( s100 stain , 10 ) non - interventional case series of 1643 consecutive patients with conjunctival tumors by shields et al . , there were no cases of conjunctival / epibulbar schwannoma . in a large review of 2455 conjunctival tumors by grossniklaus et al . , a single case of conjunctival schwannoma was reported in a 61-year - old male . le marchadour et al . described the electron microscopic features of conjunctival schwannoma in a 37-year - old male patient . the tumor almost entirely consisted of schwann cells that also enclosed myelinated fibers and were coated by a basal lamina . in between cell bodies , there were numerous schwann cell processes that were seen admixed with non - myelinated fibers . also , neuritic processes were seen and were encircled by schwann cell plasma membrane , leading to the formation of mesaxons . they usually present as a slow growing painless nodule on the conjunctiva / epibulbar surface . based on published literature , the age at presentation varies from 10 to 72 years , , [ table 1 ] . there is no obvious sex predilection with 6 of the 10 cases ( 60% ) reported being female . as elucidated by demirci et al . , since the bulbar conjunctiva is the most common site of this tumor , epibulbar schwannoma nils antoni , a swedish neurologist , described two distinct patterns of cellular architecture in the peripheral nerve sheath tumors . fibrillary , intensely polar , elongated appearing tissue type which was called tissue type a. these highly cellular regions were eventually referred to as antoni - a regions . he also described seemingly distinct , loose microcystic tissue adjacent to the antoni - a regions , and these came to be known as antoni - b regions . the conjunctiva receives its sensory nerve supply from the nasociliary nerve ; in addition there are autonomic nerves that are subconjunctival in location . it has been hypothesized that such epibulbar schwannomas possibly arise from any of these nerves . comparison of different characteristics of all the reported cases of epibulbar schwannoma available in literature the treatment of epibulbar schwannomas is complete excision . while excising , one must be careful to excise it in - toto and prevent damage to the underlying sclera . additional double freeze cryotherapy to the base , namely the underlying sclera , in addition to our case ; was done in only one of the reported cases . however , it is unlikely that cryotherapy has any role in preventing recurrence , given the benign pathology of the tumor . epibulbar or conjunctival schwannoma can be best described as a common tumor at an uncommon site . a systematic medline search was performed on pubmed using the key words : schwannoma , neurilemmoma , conjunctiva , conjunctival , and epibulbar . while this review relied on english articles , non - english language articles that had abstracts translated into english were also reviewed . a systematic medline search was performed on pubmed using the key words : schwannoma , neurilemmoma , conjunctiva , conjunctival , and epibulbar . while this review relied on english articles , non - english language articles that had abstracts translated into english were also reviewed .

schwannomas are benign , encapsulated , primary neurilemmal tumors composed of proliferating schwann cells . schwannomas are commonly seen in the orbit , but are rare on the epibulbar surface . herein , we report a case of a 12-year - old boy who presented to us with a slow - growing painless subconjunctival mass in the left eye . there was no intraocular extension of the mass and intra - operatively , the mass could be clearly delineated and was excised off the underlying sclera . histopathological examination of the mass showed typical features of schwannoma and immunohistochemistry helped to confirm the diagnosis . there was no recurrence of the lesion observed at follow - up 26 months after surgery . here , we describe this uncommon tumor and review the available literature . although rare , an epibulbar schwannoma should be considered in the differential diagnosis of an amelanotic , painless subconjunctival nodular mass . excision of the lesion is the recommended treatment .

drug hypersensitivity syndrome ( dhs ) , also known as drug rash with eosinophilia and systemic symptoms ( dress ) , is a severe drug reaction . the syndrome can also be caused by other drugs , such as allopurinol , sulfonamides , beta - lactam antimicrobials , antidepressants , and nonsteroidal anti - inflammatory drugs . allopurinol hypersensitivity syndrome ( ahs ) is characterized by rash , fever , and internal organ involvement . we report a case of an exceptional presentation of agep as a manifestation of ahs . a 67-year - old male was admitted to the dermatology department with history of widespread skin eruption since 10 days . he was started on allopurinol ( 200 mg / day ) for hyperuricemia 8 weeks earlier . clinical examination showed maculopapular eruptions involving trunk , arms and legs , as well as pinhead - sized follicular and nonfollicular pustules on patient 's face and trunk . laboratory investigations revealed a white blood cell count of 13.2 10/l with atypical lymphocytes and hypereosinophilia ( eosinophil count 1.9 10/l ) . iu / l ( reference range 340 ) and alanine aminotransferase 409 iu / l ( 345 ) . barr virus , hepatitis b , c , hiv , parvovirus b19 and human herpesvirus 6 . skin biopsy showed subcorneal spongiform pustules and some single scattered necrolytic keratinocyte [ figure 1 ] . the superficial dermis was edematous , with mixed inflammatory infiltration , including numerous neutrophils and rare eosinophils consistent with the diagnosis of agep . subcorneal spongiform pustules and some single scattered necrolytic keratinocytes the patient improved markedly within 72 h on prednisone 30 mg daily ( dose of 0.5 mg / kg body weight ) . full dose corticosteroid therapy was continued for two weeks , and tapered by 2.5 mg every 57 days , with a total duration of 3 months . causality assessment by the naranjo probability scale showed that ahs had probable causal association with the adverse effect . allopurinol hypersensitivity syndrome is a rare adverse reaction characterized by a spectrum of cutaneous reactions and systemic manifestations . ahs is more frequently associated with chronic renal insufficiency and concurrent use of thiazide diuretics . the exact pathogenesis of this syndrome is yet unclear , but different hypothesis are proposed . it seems to be related to the accumulation of allopurinol or oxipurinol a ( major metabolite of allopurinol ) in patients with renal insufficiency . immunological factors , genetic factors , and human herpes virus - type 6 are also implicated . cutaneous manifestations of hypersensitivity syndrome are heterogeneous , ranging from mild morbilliform exanthema to severe toxic epidermal necrolysis and stevens johnson syndrome . pustules in dhs differ from the typical pattern of agep , in which pustules are more numerous and predominant in main body folds . we scored both the european registry of severe cutaneous adverse reaction ( regiscar ) criteria for dress and euroscar agep score . the score was 7 for dress indicating a definite case and 5 for agep indicating a probable case . the clinical features which most differentiated dress from agep in our patient were the delayed onset ( 8 weeks ) , protracted course and multi - organ involvement . the first case of ahs with generalized nonfollicular pustulosis was described in a 47-year - old man . the second is a 65-year - old man who developed ahs manifested as generalized follicular pustulosis mimicking agep . allopurinol directly inhibits the lymphocytes enzyme purine nucleoside phosphorylase and may affect certain components of the immune system with an alteration of cd4/cd8 rate . the concomitant release of several mediators from eosinophils and/or activation of neutrophils might be important for the clinical evolution of lesions , with appearance of pustules , or aggravation of skin detachment , as well as internal organ involvement in dress . a drug reaction leading to folliculitis and then pustule formation has also been proposed . clinicians should be observant of cutaneous manifestations of hypersensitivity syndrome that may be a nonfollicular and/or follicular pustular eruption as well as the more commonly associated maculopapular rash or erythroderma .

allopurinol hypersensitivity syndrome ( ahs ) is a severe drug reaction . it is characterized by rash , fever , and internal organ involvement . it may present in different clinical forms . we present a case of acute generalized exanthematous pustulosis occurring as a manifestation of ahs .

he was known to have nonalcoholic steatohepatitis with cirrhosis and had suffered in the past two years from esophageal varix ruptures that were successfully treated using band ligation . upon admission , hemoglobin level was 8.1 g / dl and mean corpuscular volume 71 m . initial endoscopy of the upper gastrointestinal tract showed fibrous varices and grade 1 esophageal varices without any stigmata of recent hemorrhage , a small fundic varix that was actively bleeding and treated using band ligation . when reaching the duodenum , we found a large submucosal vermicular mass located in the first and second duodenum ( fig . 1a , arrows ) that had not beenpresent at the first endoscopy performed at the first variceal bleeding rupture two years earlier . second look endoscopy performed 48 h after admission confirmed the presence of grade 1 esophageal varices and the large submucosal blue - appearing vermicular mass located in the duodenum . endoscopic ultrasonography ( eus ) identified a large paraduodenal varix perforating the duodenal wall that protruded into the duodenal lumen ( fig . eus also identified a large collateral web of vessels located around the esophagus and which corresponded to a large portosystemic venous web of collaterals ( fig . the size of the duodenal varix urged us to propose a portal decompression by a tips instead of endoscopic therapy . at follow - up visit 30 days later , the patient had no signs of further bleeding and his hemoglobin level was 11.9 g / dl esophageal varices are responsible for up to 70% of all upper gastrointestinal bleeding episodes in patients with portal hypertension . a variceal source should always be suspected in any cirrhotic patient with acute upper gastrointestinal bleeding . diagnosis is usually established by the emergency endoscopist facing either active bleeding from a varix , or white nipple or clot adherent to a varix , and presence of varices without other potential sources of bleeding . the incidence of ectopic variceal rupture is as high as 15% of all variceal bleedings . ectopic varices are defined as natural portosystemic shunt leading to the appearance of venous collaterals anywhere in the abdomen . however , their appearance has been reported to be more frequent in cirrhotic patients having a history of previous abdominal surgery . furthermore , the prognosis from bleeding ectopic varices may be poor , with one study quoting more than 40% mortality at initial bleed from duodenal varices . our patient had several complications of portal hypertension including esophageal , gastric ectopic and rectal varices as well as portal gastropathy and colopathy . this particular case illustrates a typical ultrasonographic image of duodenal varice that was initially suspected at the first emergency endoscopy . it also illustrates the fact that previous band ligation treatment may influence the natural history of portosystemic shunt with the development of ectopic varices and participate to increase the size of the collateral web around the esogastric junction . the management of ectopic varices is difficult and must be performed by a multidisciplinary team . in the current case , sclerotherapy as well as band ligation were not chosen because we thought that if the entire varix could not be treated in one session , we might cause a wide defect in the varix that could precipitate a severe bleeding . a tips was chosen instead in our patient because this technique is extremely effective in controlling variceal bleeding with a control rate approaching 95% . however , due to worsening of liver function and encephalopathy , mortality remains unfortunately high . indeed , the 7 days and 6 month rates of survival after tips for ectopic variceal bleeding are 96 and 65% , respectively .

esophageal and gastric varices may complicate the course of cirrhosis as a direct consequence of portal hypertension . variceal hemorrhage has been reported to occur in 2540% of patients with cirrhosis [ gastroenterol clin north am 1992;21:149161 ] . occasionally , varices develop at sites other than the esophagus and are detected either when they bleed or incidentally during upper or lower endoscopy [ eur j gastroenterol hepatol 2006;18:11551160 ; digestion 2000;61:149150 ] . endoscopic treatment is often unsuccessful in these cases , and traditional treatment is usually surgical , while it has been suggested that bleeding can also be controlled by a transjugular intrahepatic portosystemic shunt ( tips ) [ endoscopy 1995;27:626627 ] . moreover , esophageal band ligation may interfere with the collateral web . we here report a case of an ectopic duodenal varix and the development of a large collaterals web that appeared after band ligation .

adrenocortical carcinoma ( acc ) is a rare , aggressive malignancy that features a correspondingly poor prognosis . its pathogenesis is poorly understood , especially at the molecular level , as the rarity of the disease renders comprehensive study difficult [ 1 , 2 ] . as a result , therapeutic options are currently limited , with medical and radiation therapy complementary to surgery , which is currently the only curative modality . the disease accounts for 0.2% of all malignancies and despite multimodal therapies , its prognosis remains poor , with a mean survival of < 30 months . an overall incidence of 0.52 per 1 million cases has been reported annually worldwide [ 1 , 2 ] . the disease shows a slight female gender preference and a bimodal age distribution , with the first peak in children < 5 years of age and the second peak in the fourth to fifth decade of life . a 63-year - old female patient presented left flank pain for 1 year associated with a palpated mass in this topography . in the previous 2 months , she had experienced nausea without vomiting and weight loss ( not measured ) . computerized tomography was performed for staging which revealed a large mass of 20.8 16.5 10.6 cm ( 1,902.6 cm ) in size with multiple areas of central necrosis and significant neovascularization in the left adrenal topography without a cleavage plane with pancreas and left kidney and thrombus in the inferior vena cava ( fig . 1 , fig . the patient was submitted to surgery with en bloc resection of the mass , body and tail pancreatectomy , splenectomy , left nephrectomy and thrombectomy of the inferior vena cava ( fig . histopathology revealed an adrenal carcinoma , a solid pattern , associated with low grade , 20 18 cm in size , and ki67 of approximately 5% . we observed capsule infiltration , an extensive necrosis area , clear margins and no lymph node involvement pathologic staging iv , american joint committee on cancer ( ajcc ) 2010 , european network for the study of adrenal tumors ( ensat ) stage iii . after 8 months of follow - up after surgery , the patient remained without any signs of recurrence on computerized tomography and magnetic resonance scans of the abdomen . written informed consent was obtained from the patient for publication of this case report and any accompanying images . accs arise from cells of the adrenal cortex , which are specialized steroidogenic cells that produce glucocorticoids , mineralocorticoids , and androgens . tumorigenesis may lead to abnormal hormone function , in approximately 6070% of adult patients , and result in excess production of steroid hormones that may clinically manifest as cushing 's syndrome , conn 's syndrome , virilization or feminization . however , a proportion of accs may be nonfunctional , and patients may present with nonspecific symptoms such as abdominal pain or fatigue . although acc is usually sporadic , it may be associated with familial cancer susceptibility syndromes including the li - fraumeni syndrome , beckwith - wiedemann syndrome , multiple endocrine neoplasia type 1 , and familial adenomatous polyposis . these tumors are classified as either nonfunctional or functional depending on whether they produce corticosteroids , androgen , estrogen or mineralocorticoids . although early studies reported that up to 50% of these were functional , more recent series have noted hormone secretion in up to 79% of accs . open surgery with transperitoneal access is the standard treatment of all patients with localized ( stage i ii ) and local advanced stage ( stage iii ) when complete resection can be achieved . laparoscopic adrenalectomy is a safe and effective procedure for pheochromocytoma and for patients with small accs ( < 8 cm ) without preoperative evidence of invasiveness , and adrenal masses that are judged to be only potentially malignant . the resection status ( r0 , r1 , r2 ) is a major predictor of prognosis for acc . a margin - free complete resection ( r0 resection ) , in fact , provides the only means to achieve long - term survival . in order to obtain such resection it is often mandatory to resect adjacent organs such as the wall of the vena cava , liver , spleen , colon , pancreas and/or stomach . clinical stages i and ii with tumors have a 5-year survival rate of 84 and 63% , respectively . stage iii patients have a 5-year survival rate of 51% and stage iv drops to 15% at 1 year . it is important to know that most patients surviving at 5 years are not cured but living with the disease and 85% of patients resected for cure will develop recurrence or distant metastasis . other factors negatively influencing the outcome are a size of > 12 cm , high mitotic activity , necrosis , high expression of ki67 and tp53 gene mutation positivity . in 2009 , a paper proposed a revision of the ajcc tnm classification , further called the ensat staging system . the major finding of their evaluation of the proposed international union against cancer ( uicc ) system is the observation that patients with stage ii disease do not differ considerably in their disease - specific survival from patients with stage iii disease . furthermore , patients who have stage iv disease with documented distant metastases have a poorer survival than patients who have uicc stage iv disease without distant metastases , thus clearing the improving prognostic potential . studies suggest that adjuvant mitotane can delay and possibly prevent a recurrence of the disease . in 2008 , a panel of international experts unanimously recommended adjuvant mitotane in patients with potential residual disease or a ki67 positivity on pathologic examination of > 10% . similarly , the same panel did not consider mitotane mandatory for patients with stage i or ii disease who underwent histologically proven total resection with clean margins ( r0 resection ) and a ki67 index of < 10% . the panel was undecided on whether to offer adjuvant therapy to stage iii acc patients following a r0 resection [ 1 , 5 ] . acc is a rare disease with poor prognosis . although most recurrences in the adjuvant setting occur at < 6 months postoperatively , this paper reported on a patient submitted to surgical multiorgan resection ( r0 resection ) , pathologic stage iv ( ajcc ) and ensat stage iii associated to adjuvant chemotherapy without signs of recurrence in an ongoing follow - up of 8 months .

adrenocortical carcinoma is a rare , aggressive malignancy that features a correspondingly poor prognosis . the disease accounts for 0.2% of all malignancies and despite multimodal therapies , its prognosis remains poor , with a mean survival of < 30 months . this paper reports on a patient submitted to surgical resection and adjuvant chemotherapy for adrenocortical carcinoma with an ongoing follow - up of 8 months after surgery and no signs of recurrence .

as transcriptional high - throughput sequence data are being generated , it is becoming clear that a large fraction of the data can not be annotated by comparison with existing genes using conventional methods , such as blast ( 1 ) . for example , a study of 10 human chromosomes shows that 15.4% of the nucleotides are transcribed , which is 10 times as many as expected from the annotation ( 2 ) . clearly , phenomena , such as junk transcription , are expected to account for some fraction of this transcription , but the same study also found that there are twice as many transcripts without a poly(a ) tail as transcripts with a poly(a ) tail in the cytosol . these results indicate that a significant portion of the existing transcription could be non - coding rnas . searches for novel non - coding rnas by comparative genomics are often highly dependent on a substantial amount of sequence similarity ( 3 ) . hence , genomic regions with low sequence similarity between related organisms remain to be systematically compared . the newly updated version uses a combination of a light weight energy model and sequence similarity to find common folds and alignments between two sequences ( 4 ) . other methods based on the work of sankoff have also been introduced ( 810 ) . local , where a single local fold and alignment between the two input sequences is produced . scan is used when the sequences have lengths that make the folding and aligning of the entire sequences prohibitive . the sequences can then be aligned by limiting the length of the resulting folds and alignments , i.e. a mutual scan for structural similarities between the two sequences can be carried out . here , we present a web server which provides a graphical output for the different types of comparisons . this graphical output enables the non - informatics user to navigate quickly to desired parts of the results . the web server ( and foldalign ) is especially suited for comparing sequences expected to be functionally related when the sequences are too diverged for similarity - based methods to work . the algorithm was previously tested on sequences with < 40% identity ( see supplementary material ) ( 4 ) . supplementary figure s2 shows novel performance results for global alignments , with similarity up to 70% identity . these results also show as expected that foldalign can be used when the sequences are > 40% identical . the default value scan compares the two sequences and reports a ranked list of the local folds and alignments . the other possible values are local which reports just a single local fold and alignment , and global which reports a single global fold and alignment . the maximum sequence length is 200 for global and local comparisons and 500 for scanning . for scanning , an email address can be provided for reporting when the results are ready . for scans , the score matrix found to be optimal for scanning in ( 4 ) is used . for local and global alignments , a novel score matrix optimized for global structure prediction is used ( see supplementary material ) . all types of comparisons use three parameters : maximum length difference ( delta ) , gap opening cost and gap elongation cost . obviously , for global alignments has to be longer than the length difference between the two sequences . this is not required for the other two types of comparisons , but setting to low will affect the quality of the alignment . the maximum value of is 15 for scan and 25 for local and global . for local and global alignment the gap opening cost depends on the rnas being aligned as observed by us and others ( 4,8 ) . supplementary figure s1 shows the average performance as a function of gap opening penalty for four different types of rna structures . an extra comment / id ( i d ) field is provided for the user 's convenience . there are two additional parameters for scan . maximum motif length ( lambda ) and maximum number of structures . the parameter maximum number of structures controls the maximum number of hits to be realigned and backtracked to produce a structure . if only the structure of the best hit is of interest , then this value should be set to one . the time needed to do an alignment varies from seconds ( short sequences and a small ) to several hours ( scan of 500 nt long sequences with = 200 and = 15 ) . examples of run times for different sets of parameters are available in the online documentation . when a job is submitted , its number in the server queue is reported . upon completion of a job , the web server produces a web page where the results are displayed and can be downloaded . the main parts of the outputs from the scan , global and local comparisons are shown in figures 1 and 2 . the typical output from a scan alignment the figure at the top shows the z - score for the best local alignment starting at each pair of positions along the two sequences . the bars at the top of the plot and on the left side indicate the location of the best alignments . the best alignment has a darker blue color than the others . to distinguish between alignments overlapping in one of the sequences , start and stop positions are colored yellow and red . a set of bars is drawn for each of the alignments for which a structure is produced and reported . the second main section is a list of the best scoring non - overlapping alignments between the two sequences . a maximum of 100 hits is included in the list on the web page , but the file with the entire list is one of the files available for download . the format of each line is : the name of sequence one , its start position , its end position , the name of sequence two , its start position , its end position , the foldalign score , the z - score , the p - value and the rank . the p - value is calculated using the island method , ( 12,13 ) , using the scores of the non - overlapping hits as the scores used for estimating the extreme value parameters . the distribution parameters can be found at the bottom of the page ( not shown in the figure ) . the p - value estimate is very crude since the distribution is estimated from very few alignment scores , and any non - random alignments will bias the estimate . the ns score is the foldalign score without the contribution from single strand sequence similarity . this score can be used to separate alignments that have a high score due to conserved structure from alignments that have a high score due to sequence conservation . the output from both local and global alignment shows the alignment score , the score without the contribution from the single strand substitution costs , the positions , the local identity of the sequences , the number of base pairs in the predicted structure , the sequences and the common structure ( figure 2 ) . foldalign performs structural alignment of two rna sequences or local structural alignment between structural similar regions in two sequences . the algorithm uses a combination of a light weight energy model and sequence similarity ( 4 ) . a foldalign web server is now available , which predicts alignments and structures for pairs of sequences . it can make three types of comparisons : scan makes a local alignment and reports a ranked list of the best local alignments . the input sequences can be long , but the length of the motif searched for is limited . the local comparison type makes a local alignment where the motif can be as long as the input sequence . the global comparison type folds and aligns the sequences from end - to - end . even though the sequence length , , and are limited on the web server , arbitrarily long sequences can in principle be scanned by using the foldalign software itself . and are then limited by the amount of memory available on the local machine . the trna structures were taken from the trna database and the surrounding sequences from genbank ( 14,15 ) . default parameters were used for the alignment . at the top of the output , there is a plot of the z - scores . it is followed by a ranked list of non - overlapping local alignments . in the example the locations of the best hits are marked with bars on the sides of the z - score plot .

foldalign is a sankoff - based algorithm for making structural alignments of rna sequences . here , we present a web server for making pairwise alignments between two rna sequences , using the recently updated version of foldalign . the server can be used to scan two sequences for a common structural rna motif of limited size , or the entire sequences can be aligned locally or globally . the web server offers a graphical interface , which makes it simple to make alignments and manually browse the results . the web server can be accessed at .

the majority of neuroendocrine carcinomas of the urinary bladder are small cell carcinomas . in recent years , a few cases of large cell neuroendocrine carcinomas ( lcnec ) have also been reported . we present a case of lcnec of the urinary bladder which achieved complete spontaneous remission . mr u is a 72-year - old male , with a background of clinical t2n0m0 high grade urothelial carcinoma bladder cancer treated with radical radiotherapy with neoadjuvant cisplatin 2 years previously . an us renal tract during this presentation showed a soft tissue mass in the base of the bladder causing bilateral ureteric obstruction . ct staging showed locally advanced malignancy with concern for local involvement of the mid rectum , there was no evidence of metastatic disease . he subsequently commenced on a combination of androgen deprivation therapy and neoadjuvant chemotherapy ( carboplatin / etoposide ) . after three cycles of chemotherapy , a pet - ct was done which showed no reduction of the large tumour volume and no metastatic disease . the patient was experiencing severe local symptoms and it was elected to go forward with radical pelvic surgery . he proceeded to a radical cysto - prostatectomy where the tumour was able to be dissected from the rectum . the tumour involved the bladder , with invasion through to adipose tissue and posteriorly at perivesical resection margins ( pt4a ) . in addition , there was a large gleason score 9 prostatic adenocarcinoma , distinct from the neuroendocrine carcinoma of the bladder and lacking neuroendocrine immune - histochemical positivity . nuclei are several times the diameter of lymphocytes ( lower left ) , nucleoli are prominent , and chromatin quite coarse . nuclei are several times the diameter of lymphocytes ( lower left ) , nucleoli are prominent , and chromatin quite coarse . some nuclear moulding is seen , as in the small cell variant . following surgery , the patient made a good recovery , however , represented 3 months later with significant deterioration in functional status , nausea , weight loss and anorexia . 2 ) demonstrated metastatic disease with the local recurrence of the mass extending into cystectomy bed . figure 2:ct - pet performed after surgery showing metastatic disease with local recurrence of the mass extending into the cystectomy bed . ct - pet performed after surgery showing metastatic disease with local recurrence of the mass extending into the cystectomy bed . the patient declined further intervention and was referred to hospice for end of life cares . following referral to the hospice he underwent a steady and gradual improvement in clinical status . he decided to live life to its fullest and went sailing around the mediterranean and pacific islands . following his travels he still felt well and a pet - ct was requested ( fig . the patient remains still disease free on imaging two years following his spontaneous remission ( 3 years since completion of any chemotherapy and his subsequent surgery ) . this case demonstrates a case of prolonged spontaneous remission in a patient with lcnec in the setting of previous chemo - radiotherapy for transitional cell carcinoma . the most common neuroendocrine tumour of the bladder is small cell neuroendocrine carcinoma , followed by carcinoid tumours ( typical and atypical ) , and lcnec . large cell neuroendocrine tumour of the bladder was first described in 1986 and , until now , 17 cases have been published in the international literature . the most common histology is mixed with urothelial cancer , followed by adenocarcinoma , squamous cell carcinoma and sarcomatoid urothelial cancer . our case presumably arose in a background of high - grade urothelial carcinoma , though none was found at the time of biopsy or cysto - prostatectomy . differential diagnosis of pure lcnec includes metastatic lcnec from pulmonary or gastrointestinal primary sites , local invasion of the bladder by poorly differentiated prostatic carcinoma in male patients , and primary bladder lesions such as large cell lymphomas and high - grade , undifferentiated urothelial carcinomas . the large cell variant of neuroendocrine carcinoma is considered closely related to the more common small cell variant , distinguished mainly by large total cell and nuclear size , prominent nucleoli and coarser chromatin . there is some evidence of biological differences in the two variants when occurring in the lung , but too few cases occur in the bladder for comparisons to be made . we believe that the present case is the 10th reported case of pure lcnec of urinary bladder proven post - cystectomy . pure lcnec of the urinary bladdersourceagesexsurgeryadjuvant chemotherapy or radiotherapystatus at last follow - upf / u periodhailemariam et al . 68mradical cysto - prostatectomyradiotherapy and chemotherapyalive with liver and bone mets16 months reported cases of pure lcnec of the urinary bladder bladder lcnecs have a rapid course and poor prognosis like the small cell variant . the role of surgery or chemotherapy in the treatment of lcnec of the urinary bladder is not clear due to the rarity of the disease . however , studies with pulmonary lcnec show they respond well to standard chemotherapy regimens used for the small cell variants . in our case , the follow - up period is 24 months following complete spontaneous remission of this very aggressive tumour and 3 years following surgical removal of the tumour . this suggests that although lcnec is generally characterized by a very poor prognosis , it may behave very unpredictably . although the overall prognosis is poor and most patient present with metastatic disease it can behave unpredictably . we believe this to be the first case of spontaneous remission of this disease . because of the rarity of these tumours , there are no prospective trials done to evaluate the optimum treatment and our case is important as it adds to the current literature on the behaviour of this rare variant of bladder cancer .

abstractlarge cell neuroendocrine carcinoma ( lcnec ) of the urinary bladder are rare . we present a case of a 72-year - old man who presented with back pain and acute renal failure . ultrasound showed a soft tissue mass in the base of the bladder causing bilateral ureteric obstruction . subsequent biopsy of this mass demonstrated neuroendocrine carcinoma . he was commenced on neoadjuvant chemotherapy ( carboplatin / etoposide ) and proceeded to a radical cysto - prostatectomy . histology revealed a lcnec involving the bladder , t4a with invasion through to adipose tissue and posteriorly at perivesical resection margins . in addition , there was a gleason score 9 prostatic adenocarcinoma , distinct from the neuroendocrine carcinoma . following surgery , the patient developed gross local - regional recurrence and refused further systemic therapy . however , 1 year following referral to palliative care , a further ct - pet showed complete spontaneous remission of his disease . there are only few case reports of lcnec of the urinary bladder therefore the pathogenesis and treatment protocol are still unclear . this case report highlights the unpredictable nature of this disease .

infliximab , a tumor necrosis factor- ( tnf- ) inhibitor , has become the standard therapy for active rheumatoid arthritis ( ra ) , but also has influences on bacterial , viral , and tuberculous infections . in particular , the reactivation of hepatitis b virus ( hbv ) is problematic and hbv - induced fulminant hepatitis following infliximab treatment has been reported in patients with crohn s disease and still s disease [ 36 ] . here , we describe an autopsy case of ra who developed precore mutant hbv - associated fulminant hepatitis after 1 year of infliximab therapy and subsequent methotrexate ( mtx ) withdrawal . a 73-year - old female , with a 10-year history of ra , had been treated with prednisone ( 510 mg / day ) and mtx ( 58 mg / week ) for 10 and 6 years , respectively . these were ineffective , and infliximab ( 150 mg every 8 weeks , 3 mg / kg ) was added between june 2004 and may 2005 , with liver function tests at each infusion . a pretreatment screening test showed that hbsag and hbcab were positive , and hepatitis c virus antibody was negative . an effective clinical improvement of joint disease was observed , and c reactive protein became normal . after the eighth infusion of infliximab , the liver function was found to be abnormal ( ast 291 iu / l ( normal < 40 ) , alt 331 iu / l ( < 40 ) ) , and mtx therapy was withdrawn . iu / l ) , and she was admitted to our hospital in june 2005 . on admission , she had jaundice and mild hepatic encephalopathy . iu / l , gtp 130 iu / l ( < 30 ) , ldh 364 iu / l ( 115 245 ) , total bilirubin 15.7 mg / dl ( < 1.0 ) , prothrombin time 8% ( > 70 ) , and nh3 168 g / dl ( 30 80 ) . her serological status was as follows : hbsag , hbeab , and igm hbcab were positive , while hbsab and hbeag were negative . hbv - dna was present with a level of 4.3 log copies per milliliter ( < 2.6 ) by polymerase chain reaction assay . therefore , a diagnosis of fulminant hepatitis due to the reactivation of hbv from a previously asymptomatic hbv carrier state was made , and lamivudine ( a reverse - transcriptase inhibitor of viral dna polymerase ) therapy ( 150 mg / day ) and plasma exchange were immediately performed . her condition temporarily improved , and liver transplantation was considered . however , her liver function deteriorated and an abdominal ct showed more atrophic liver . the liver was remarkably atrophied , weighing just 508 g , and more than 80% of the hepatocytes were necrotic . cd8-positive cytotoxic t lymphocytes had predominantly infiltrated , and amyloid a protein deposition as a result of ra was seen in the hepatic artery ( fig . 1 ) . c amyloid a immunoreactivity of the hepatic artery ( 10 ) liver histology . a massive hepatocytic necrosis ( h&e stain , 4 ) . our case suggests that infliximab treatment induces hbv precore mutation and the destruction of hbv - infected hepatocytes by cd8-positive cytotoxic t lymphocytes . the hbv precore mutation is involved in the pathogenesis of fulminant hepatitis , and the reactivation of a precore mutant hbv during infliximab therapy has been reported . tnf- has antiviral properties by inhibiting the replication of hbv dna and mediates apoptosis of cytotoxic t lymphocytes [ 9 , 10 ] . tnf- inhibitors may induce fulminant hepatitis following the reactivation of precore mutant hbv and the proliferation of cytotoxic t lymphocytes . in addition to infliximab , long - term mtx treatment and subsequent withdrawal may accelerate hbv reactivation . hbv - positive patients are at increased risk of fulminant hepatic failure after withdrawing immunosuppressive drugs such as mtx because of the hepatocytic attack following sudden reaction of the immune system . preemptive antiviral therapy with lamivudine is recommended as part of infliximab therapy for hbv - positive patients , even if they have normal liver function or an undetectable viral load [ 8 , 12 ] . the delayed onset of antiviral therapy in our patient may have been responsible for the fatal outcome . this case shows that infliximab therapy in an hbv - positive patient with ra can lead to fulminant hepatitis , possibly through the reactivation of precore mutant hbv and the proliferation of cytotoxic t lymphocytes . in addition , this case illustrates the importance of anti - hbv therapy before starting infliximab administration and withdrawing immunosuppressive drugs .

a 73-year - old female , who suffered from rheumatoid arthritis for 10 years , developed precore mutant hepatitis b virus - associated fulminant hepatitis after 1 year of infliximab therapy and subsequent methotrexate withdrawal . we emphasize the importance of preemptive antiviral therapy before starting infliximab administration and withdrawing immunosuppressive drugs .

spontaneous spinal edh is a rare clinical scenario , which may be secondary to variety of etiologies such as coagulopathic disorders , lymphomas , plasma cell myeloma , and very rarely , following lumbar puncture . central nervous system hemorrhages are an uncommon but severe complication of hemophilia , occurring in only 28% of children with hemophilia . urgent diagnosis is key to successful management as any delay can lead to rapidly progressive neurological deficits . we report a known case of hemophilia b who presented with backache and rapidly worsening paraparesis of short duration . he recovered gradually with conservative treatment with factor ix replacement therapy with close neurological and hematological monitoring . a 5-year - old male child , a known case of hemophilia b for 3 years , presented at the casualty with acute pain over the lumbosacral region and rapidly worsening paraparesis of 7 days duration . neurological assessment revealed bilateral lower limb paresis with hypotonia and motor power of 2/5 ( mrc grading ) , around 40% sensory impairment to all modalities of sensation from l1 dermatome below . the patient had urinary retention for which he was catheterized . a spinal mri done revealed a space - occupying lesion in the epidural space ventrally , extending from l4 to s2 level with heterogenously hyperintense signal on both t1 and t2 weighted images . hematological investigations of the patient revealed prolonged activated partial thromboplastin time ( aptt ) , normal prothrombin time , normal bleeding time , normal platelet count , and low factor ix ( < 1% ) . in consultation with hematologist , the patient was placed on factor ix replacement therapy in a dose of 100 u / kg initially , then 50 u / kg / day for 14 days to maintain factor ix activity up to 50% . the patient gradually improved neurologically and motor power improved to 4/5 at the time of discharge . he gradually improved and at 1 month following discharge , he was self - ambulatory with no residual weakness . the patient was doing well at last follow - up , 2 years following treatment . t1 and t2 weighted mri spine of the patient showing extradural hematoma from l4-s2 ventrally mri of the patient 6 months following treatment shows resolution of hematoma hemophilia is the most common hereditary bleeding disorder , with an incidence of 0.7 - 0.8/10,000 . hemophilia a is characterized by an inherited deficiency of factor viii while factor ix deficiency leads to hemophilia b ( christmas disease ) . spontaneous spinal edh is a rare entity in hemophiliacs , reported both in patients with hemophilia a and hemophilia b. there is no age bar for this condition as cases have been reported even in infants . spinal edh , by and large , pursues an acute course , although a chronic course has also been reported in the literature . the most common cause of death in hemophiliacs is intracranial hemorrhage ; however spinal hematoma can lend the patient a severe morbidity . patients with spontaneous spinal extradural hematoma usually present with rapidly progressive neurological deficits without any antecedent trauma . the investigation of choice remains noncontrast mr imaging , which can detect even small amounts of blood in the spinal canal . early and prompt treatment is the most important factor governing the outcome of the patient . treatment strategies include medical line of management consisting of replacement of the deficient factors , as in our case , or a combined medical surgical approach in which a decompressive procedure is performed under the cover of the deficient factor . sheikh et al . reported that aggressive factor ix replacement therapy resulted in complete neurologic recovery without the need for surgical decompression in a case of extensive spinal epidural hematoma , in a child with factor ix deficiency . recently published a review regarding spinal edh in pediatric hemophilia patients and concluded that observation and correction of coagulopathy remains a safe and effective treatment choice for patients with a stable neurological examination . they also concluded that a multidisciplinary treatment involving a pediatric hematologist , neurosurgeon , and pediatric intensive care unit to ensure timely correction of the coagulation disorder , maintenance of adequate factor levels , and close hemodynamic and neurological monitoring . kubota et al . , in a recent study , concluded that unless the neurological deficiency is progressing rapidly ; nonsurgical , conservative management is safe for a spinal extradural hematoma in patients with hemophilia , rather than attempting high - risk surgical management with inappropriate coagulation status . in a setting of coagulopathic disorder and rapid onset neurological deficits such as paraparesis or quadriparesis , spontaneous spinal epidural hematoma should be kept in the differential diagnosis . prompt investigation and management of patient with coagulation factor replacement therapy can have gratifying results . conservative therapy with replacement of deficient coagulation factor and close neurological and hematological monitoring usually result in a favorable outcome .

spontaneous spinal extradural hematoma is a rare clinical scenario which may be secondary to a variety of etiologies . spinal epidural hematoma is an extremely rare complication in hemophiliacs . it usually runs an acute course often leading to rapid onset of neurological deficits . mr imaging is the diagnostic modality of choice and early , prompt treatment will often yield fruitful results . we report a case of spontaneous spinal edh in a 5-year - old male child with hemophilia b , who was managed conservatively and was doing well at last follow - up , 2 years after treatment . the authors discuss the role of factor replacement therapy vis - a - vis surgery in such a scenario .

inflammatory bowel disease ( ibd ) is a group of inflammatory conditions of the colon and small intestine . an estimated 1.36.4% of patients with ibd experience cerebral venous thrombosis ( cvt ) at some point during the course of the disease . cvt tends to occur in young patients , and those who have both cvt and ibd are significantly younger than those with cvt without ibd . although many studies have shown that disease activity associated with cd contributes to a hypercoagulable state that aids in the development of cvt , the underlying mechanisms remain unclear . this case report describes the case of a 17-year - old male in whom cvt occurred during a nonactive phase of cd . a 17-year - old male visited the emergency room following the acute onset of right - side weakness and hypesthesia . he had no history of or risk factors for stroke , and the only noteworthy disease in his medical history was cd , which had been diagnosed 1 year earlier when he developed recurrent diarrhea and hematochezia . , he had no symptoms indicative of cd activity such as an increased frequency of bloody stools , abdominal pain and weight loss . the patient was normotensive , and a systemic review revealed a headache of moderate severity . a blood test revealed chronic anemia ( hb level 9.0 g / dl ; the total iron - binding capacity and red cell width were normal ) . his blood , sputum and stool cultures were negative ; a rectal examination did not reveal melena or diarrhea ; an electrocardiography demonstrated a normal sinus rhythm . a brain mri revealed an acute infarction of the left frontal cortex and a cortical subarachnoid hemorrhage ( fig . cvt was suspected because of his young age and the fact that he did not have any risk factors for stroke . the infarction in a nonarterial location , the cortical hemorrhage as well as the acute neurological deficits were illustrative . we confirmed the diagnosis via brain magnetic resonance venography , which revealed a segmental filling defect in the superior sagittal sinus and non - visualizability of some cortical veins bilaterally ( fig . a gastroenterologist was consulted in the context of the cd activity level ; no evidence of escalating disease activity ( such as an increased frequency of liquid stools , abdominal pain or weight loss ) was noted . the cd activity index ( cdai ) , which is widely used to quantify cd symptoms and the extent of disease activity in cd patients , was used to estimate his cd activity . the patient was also assessed for inherited and acquired thrombophilic disorders ; the factor v leiden mutation and prothrombin gene polymorphism were negative . laboratory findings were negative in terms of protein c and s deficiencies , hyperhomocysteinemia , vasculitis , and markers of other autoimmune diseases ( including thyroid function tests , fluorescent antinuclear antibody , rheumatoid arthritis factor , anti - thrombin iii antibody and anti - phospholipid antibody ) . with anticoagulation , the patient regained his independence with only mild hemiparesis 3 months after admission . although cvt is a rare complication in cd patients , the cvt is sometimes very serious , leading to mortality . however , it has been suggested that cvt is associated with several coagulation problems that develop during an ibd flare - up ; these include thrombocytosis , increased levels of fibrinogen , fibrinopeptide a , factors v and viii , the antithrombin factor iii deficiency as well as a deficiency of free protein s . the inflammation associated with ibd may promote the release of prothrombotic acute - phase reactants including factor viii , the von willebrand factor , lipoprotein a and fibrinogen while simultaneously reducing the levels of naturally occurring anticoagulant factors such as antithrombin , protein c , protein s , and tissue factor pathway inhibitors . thus , it is difficult to predict whether cvt will develop in a cd patient . this is important because , once cvt occurs , the patient deteriorates quickly and complications of the disease may become irreversible . in addition , the sequelae of cvt typically trigger a need for lifelong rehabilitation at enormous medical costs , and it might be impossible to modify the cd disease activity . it is also important to consider any psychological problems associated with medical disorders , especially in young patients . the most commonly used treatment strategy known to improve outcomes is anticoagulation with intravenous heparin or subcutaneous low - molecular - weight heparin until the patient is stabilized , followed by oral anticoagulation . however , achieving therapeutic anticoagulation with warfarin was difficult due to a presumed drug interaction with azathioprine and active gastrointestinal bleeding [ 11 , 12 ] . reported that thromboembolic phenomena were related to ibd activity only in ibd patients with cd , whereas katsanos et al . found active disease in 78.4% of ibd patients and quiescent disease in the other 21.6% . the characteristics of our case suggest that the risk of cvt is most likely related to cd per se rather than to disease activity associated with cd ; gastroenterologists treating cd patients should be aware of this risk . unfortunately , no single cause of the hypercoagulable state in cd patients has yet been identified . further research is necessary to clearly characterize the mechanism or mechanisms and to establish guidelines for clinicians who treat cd patients with cvt .

the development of cerebral venous thrombosis ( cvt ) as a secondary complication of crohn 's disease ( cd ) seems to be rare , but it is generally accepted that the disease activity of cd contributes to the establishment of a hypercoagulable state . here , we describe a case of cvt that developed outside the active phase of cd . a 17-year - old male visited the emergency room because of a sudden onset of right - sided weakness and right - sided hypesthesia . he had been diagnosed with cd 1 year before and was on a maintenance regimen of mesalazine and azathioprine . he did not exhibit any symptoms indicating a cd flare - up ( bloody stools , abdominal pain , complications , or weight loss ) . a brain mri scan revealed an acute infarction of the left frontal cortex and a cortical subarachnoid hemorrhage . additionally , a magnetic resonance venography revealed a segmental filling defect in the superior sagittal sinus and also the non - visualizability of some bilateral cortical veins . the characteristics of the present case suggest that the risk of cvt is most likely related to cd per se rather than disease activity associated with cd .

intralobar pulmonary sequestration ( ilps ) with an anomalous systemic arterial blood supply represents a mass of nonfunctioning parenchyma surrounded by visceral pleura ( 1 , 2 ) . unlike extralobar sequestration , radiographically demonstrated calcification in the ilps has been reported in some case series , but primary sequestration associated with typical hamartoma signs is really rare and has been described only once ( 3 , 4 ) . in this report , we present a rare case of giant hamartoma originated from primary ilps in a 39-year - old woman who had intracranial cholesteatoma . a 39-year - old woman with an abnormal mass in the right cerebellopontine angle was referred to neurosurgery department of our hospital . a giant mass in the right thoracic cavity was detected accidentally by x - ray in a routine preoperative screening process . subsequent thoracic computed tomography scan demonstrated a complex lesion ( size 15.1 12.3 15.7 cm ) occupying more than two - thirds of the thoracic cavity on the right side . the blood supply to the area of the lesion was from a systemic artery arising from the abdominal aorta , with venous drainage through the inferior pulmonary vein , suggesting an ilps ( figure 1 a - 1c ) . three months later , an open thoracic surgery for the intrathoracic lesion was performed under general anesthesia . intraoperatively , lesion appeared hypervascular , because of abundant systemic vascularization ( figure 2 a ) . the massive lesion was compressing the right lung and there was no communication between the tracheobronchial tree and sequestered lesion . based on the findings of 3d ct angiography , we explored the aberrant artery at the bottom of the chest and carefully ligated it with an absorbable silk suture . after the pulmonary ligament had been completely divided , the lower pulmonary vein was found to be widened . the lesion was adherent to adjacent pulmonary vein branches and was not easily dissected . to avoid major intraoperative blood loss , pathologic analysis showed a predominant fatty differentiation with fibroblastic , chondroid and osseous differentiation making the rest ( figure 2b - 2d ) . the postoperative recovery was uneventful and the patient was discharged from the hospital on the eighth postoperative day . ilps is usually diagnosed later than extralobar sequestration ( els ) , being found in childhood or adulthood when the patient presents with an infection ( 5 ) . up to 15% of patients , particularly in adult population , are asymptomatic when the sequestration is discovered incidentally ( 6 ) . in our case , the sequestration surrounded by visceral pleura , characteristically receives its blood supply from a systemic artery with venous drainage through pulmonary veins and was consistent with ilps . the lesions of ilps may be solid , fluid or hemorrhagic or may contain mucus . primary sequestration associated with typical hamartoma signs final histology was pulmonary hamartoma with predominantly adipose and cartilage differentiation , which is an unusual complication originated from primary sequestration . pulmonary hamartoma is a rare lung neoformation , usually symptomless and discovered incidentally , of a probable dysontogenetic origin with prevailing cartilaginous tissue and adult onset age ( 7 ) . in a series of 24 patients , gabrail and zara ( 8) concluded that pulmonary hamartomas are frequently accompanied by other developmental abnormalities and benign tumors . however , the entity described ( pulmonary hamartoma syndrome ) seems to be distinct from well - established multiple hamartoma syndrome ( cowden s syndrome ) . many interesting features characterize the case report : histopathological nature of the pulmonary hamartoma , the typical vascular structure related to intrapulmonary sequestration and intracranial cholesteatoma as shown by neurosurgery operation . . the combination of sequestration , hamartoma and cholesteatoma may be only an incidental combination , but this could indicate both possibilities as hamartomatosis change on underlying sequestration or sequestration associated with hamartomatosis syndrome .

the radiologic features of intralobar pulmonary sequestration ( ilps ) have been describe and include the identification of a feeding systemic artery with venous drainage through pulmonary veins . primary sequestration associated with typical hamartoma signs is really rare and has been described only once . we describe a patient with ilps whose radiographic findings were unusual for two reasons . first , computed tomography ( ct ) demonstrated a bulky mass in the pulmonary sequestration . the size of lesion and histopathology made it an unusual presentation . final histology study demonstrated pulmonary hamartoma with predominantly adipose and cartilage differentiation , which is an unusual complication originated from ilps . another sign also explains the second unusual feature , intracranial cholesteatoma , occurring concurrently with ilps .

etiological factors for spinal epidural hematoma are tumors , anticoagulant therapy , coagulopathy , hypertension , arteriovenous malformations , and trauma . most common presentation is sudden back or neck pain followed by a motor or sensory dysfunction with or without urinary retention . complete neurological recovery is possible with early diagnosis and urgent surgery while delay in the treatment of this condition causes permanent neurological deficit . a previously healthy 14-year - old boy was admitted with a 15-day history of back pain , urinary retention , and weakness of both lower limbs . the patient noted a rapid increase of his symptoms after 2 days with complete loss of power and sensation in lower limbs . the patient was admitted in some local hospital and came to us after 2 weeks of onset of symptoms . there was no history of spinal trauma , systemic disease , anticoagulant usage , or coagulopathy . neurological examination revealed bilateral sensory disturbance of all modalities below the level of fourth dorsal vertebrae level and spastic paraplegia . t1- and t2-weighted magnetic resonance ( mr ) images revealed a mass lesion suggesting an epidural hematoma , extending from dorsal vertebrae d1 to dorsal vertebrae d6 , localized in the ventral spinal epidural space . fourth and fifth dorsal laminectomy with left partial facetectomy was performed , and an epidural clot was totally removed using microneurosurgical technique . during surgery , no vascular abnormality was observed , but no dural pulsation was evident at the end of the procedure . an mr sean of the spine was performed 10 days after the operation which showed complete removal of hematoma [ figure 2 ] . ( a ) t1-weighted magnetic resonance imaging dorsal spine sagittal cuts , ( b ) t2-weighted sagittal cuts , ( c and d ) t2-weighted axial cuts showing a ventral spinal epidural hematoma extending from d1 to d6 vertebra and displacing and compressing the cord posteriorly ( a and b ) t2-weighted postoperative magnetic resonance imaging dorsal spine sagittal cuts , ( c and d ) t2-weighted axial cuts showing laminectomy defect with complete evacuation of ventral epidural hematoma ventral sseh , as our case , is even rarer with only four previous cases reported in the literature . the most common clinical presentation is sudden back or neck pain followed by motor and sensory dysfunction with or without urinary retention . the neurologic deficits are dependent to the localization of hematoma , horner or brown - sequard syndromes may be noted . the progression of the symptomatology and clinical signs is usually very rapid , as in our case , although slower progression over a few days has also been reported . most common causes of spinal epidural hematoma are the use of anticoagulants , coagulopathies ( hemophilia and leukemia ) , and procedures such as a spinal tap or epidural anesthesia . most authors have contended that sseh arises from epidural venous plexus in the spinal epidural space . because of fluctuations in intrathoracic and intra - abdominal pressures after exercise or other maneuvers , reversal of blood flow may induce rupture of a delicate vein in the valveless epidural plexus . ct findings are similar to intracranial epidural hematoma showing hyperdense lenticular collection . mr imaging is the best examination for diagnosis and follow - up . the dura mater is visualized as curvilinear low signal , separating the hematoma from the cord . the hematoma is isointense or slightly hyperintense on t1-weighted images and heterogeneous on t2-weighted images within 24 h of onset . later , hematoma gives a high signal on both t1- and t2-weighted sequences . in the absence of any signs suggestive of vascular malformation on mr imaging , preoperative angiography is not essential and need not delay the surgical procedure , because the timing of the surgery , together with the preoperative clinical state , determines the quality of the clinical result . posteriorly placed hematoma can be easily removed by doing laminectomy but anteriorly placed hematoma needs good exposure and microneurosurgical techniques to prevent retraction injury to the cord . partial facetectomy can be done in dorsal spine to enhance exposure , and lateral corridor can be used to prevent thecal sac retraction . complete and partial neurological recovery has been described in approximately 50% and 44% of the patients , respectively . recovery was significantly better when decompression was performed within < 36 h of the onset of the neurological deficit . in the majority of cases with sseh , the mainstay of treatment will remain surgical decompression of the neural structures and removal of the hematoma . the decision for conservative treatment has to be based on the severity of the neurological deficit and on the clinical course . prognosis of the patient with sseh depends on the severity of the neurologic deficit on admission and interval from onset of initial symptom to surgery . early diagnosis and prompt surgery are crucial to achieve the best neurological outcome and delay in the diagnosis and treatment may cause permanent neurologic deficit

spontaneous spinal epidural hematoma is very uncommon cause of spinal cord compression . it is extremely rare in children and is mostly located in dorsal epidural space . ventral spontaneous spinal epidural hematoma ( sseh ) is even rarer , with only four previous reports in childrens . we are reporting fifth such case in a 14 year old male child . he presented with history of sudden onset weakness and sensory loss in both lower limbs with bladder bowel involvment since 15 days . there was no history of trauma or bleeding diasthesis . on clinical examination he had spastic paraplegia . magnetic resonance imaging ( mri ) of dorsal spine was suggestive of ventral spinal epidural hematoma extending from first to sixth dorsal vertebrae . laminectomy of fourth and fifth dorsal vertebrae and complete evacuation of hematoma was done on the same day of admission . postoperatively the neurological status was same .

according to the world health organization , breast cancer is the most commonly diagnosed cancer worldwide . in western countries , the prevalence of metastases of breast carcinoma to the eye orbit is 4.6% . in order of declining frequency , ocular metastases of breast carcinoma appear in the highly vascular choroid , the anterior segment , orbital structures and the optic nerve . for orbital metastasis , breast carcinoma is considered to be the most prevalent primary tumor , accounting for 29 - 70% . the presence of metastases in this area usually reflects widespread hematogenous distribution , resulting in a poor prognosis . in 20 - 40% of the patients , twenty - five years after a primary diagnosis of breast carcinoma , a 72-year - old woman was sent to the ophthalmology department because of a painful right eye . a few weeks before , she had noticed a swelling on the temporal conjunctiva of that same eye . she had a history of lobular breast cell carcinoma of the right breast and had undergone lumpectomy with lymphadenectomy and adjuvant radiotherapy . visual acuity of the right eye was known to be low due to esotropia and amblyopia . slit lamp examination of the right eye showed chemosis with dilated vessels in the inferior temporal conjunctiva ( fig . the swelling was thought to hinder a normal tear film formation , giving rise to insufficient lubrication and causing the pain sensation . one month later , she visited the outpatient clinic because of a firm swelling , located at the superotemporal border of the right orbit , near the lacrimal gland . a ct scan showed unclear diffuse infiltration of the right and left orbit with staining of contrast in the lacrimal gland on the right side , as well as the retrobulbar area near the left optic nerve . a biopsy of the temporal conjunctival swelling and of the lacrimal gland of the right eye revealed lobular breast carcinoma metastases ( fig . the present case describes a 72-year - old woman with metastatic breast carcinoma to the bulbar conjunctiva and the lacrimal gland of the right eye . secondary tumors to the conjunctiva are extremely seldom and rarely mentioned in reviews on ocular metastases . a literature search revealed three papers with a total of six patients who showed a conjunctival metastatic lesion from breast carcinoma . patients presented with nonsuspect symptoms like a red eye , foreign body sensation , conjunctival swelling or a painless lesion with irregular and indistinct margins which was associated with dilated conjunctival vessels . metastatic lesions to the lacrimal gland are also quite rare . in patients with a lacrimal gland lesion , metastasis counted for 0.9% , all of which were due to secondary breast carcinoma . in conclusion , patients with a history of breast carcinoma may present with nonsuspect complaints , like conjunctival swelling , a lump , a red eye or foreign body sensation , which nevertheless may turn out to be breast cancer metastases , even 25 years after the initial diagnosis . unfortunately , prognosis of breast cancer metastasis to the conjunctiva is poor , and most of the described patients died weeks to months after the diagnosis . the authors have no conflict of interest to declare , nor sponsorships or funding to disclose .

a 72-year - old woman presented with a painful right eye . a few weeks before , she had noticed a red , swollen area in the conjunctiva of the same eye . on slit lamp examination , it appeared as chemosis and vascular injection ; artificial tears were prescribed . a month later , a firm mass developed on the superotemporal orbital rim , in the area of the lacrimal gland . a ct scan revealed infiltrative structures in both the left and right orbit , with contrast staining in the right lacrimal gland and near the left optic nerve . a biopsy was taken of the conjunctival swelling as well as of the lacrimal gland . both tissues showed infiltration with lobular breast carcinoma metastases .

a 38-year - old man presented with a swelling in the left upper eyelid for three years . clinical examination of the left eye revealed mild proptosis and downward and inward dystopia . magnetic resonance imaging ( mri ) of the orbit showed an extraconal mass in the left lacrimal gland region abutting the left globe and pushing it anteriorly and medially [ figure 1 ] . comparison with the previous mri scans done three and two years earlier showed no significant change in size of the lesion . a single nodular tissue measuring 2.5 2 1.5 cm was received for histopathological examination . routinely stained tissue sections revealed a lesion comprising nodular aggregates of small lymphocytes and diffuse occasional aggregates [ figure 2a ] . in between these were many large foamy histiocytes , some of which had lymphocytes and plasma cells entrapped within the cytoplasm ( emperipolesis ) [ figure 2b ] . most of the lacrimal gland was destroyed by the disease except for a few entrapped remnants of lacrimal ducts [ figure 2c ] . immunostaining showed the foamy histiocytes to be strongly positive for s-100 protein [ figure 2d ] and cd68 . a follow - up of about 24 months was available after surgical resection of mass . there has been no recurrence , neither has the patient developed any lymphadenopathy or any other systemic organ involvement . rosai - dorfman - destombes disease or sinus histiocytosis with massive lymphadenopathy ( shml ) is a rare non - neoplastic proliferative disorder of histiocytes that affects predominantly children and young adults , with a slight male predominance . nearly 87% of patients have bilateral cervical painless lymphadenopathy ; other nodal groups are less frequently involved.2 in some cases these extranodal manifestations represent the predominant or even exclusive manifestation of the disease . practically all organ systems have been recorded as being the site of the disease . classically , other sites include skin , upper respiratory tract , salivary gland , epidural space , bone and orbital soft tissue.5 the most frequent manifestation is an orbital soft tissue mass with proptosis . to the best of our knowledge , there are only three case reports with isolated lacrimal gland involvement.3,4 in the earlier three case reports along with our present case the lacrimal gland involvement was unilateral . in our present case , the lacrimal gland mass was very well circumscribed with no extension into orbital soft tissues . typically , rdd disease undergoes spontaneous remission after a protracted course but may develop recurrences . rarely , the involvement of vital organ may lead to death . in our cases no recurrence or any lymphadenopathy was noted in a follow - up period of 24 months . viruses like epstein - barr virus and human herpes virus 6 have been suggested . molecular studies done on involved tissue have failed to show evidence of clonality in keeping with their presumed reactive nature . rosai - dorfman - destombes disease presents in its most typical form as lymph node involvement with sinus histiocytosis.5 it is important to be familiar with the clinical and diagnostic histopathologic features of this entity especially when the disease presents extra - nodally in an isolated form . histopathologic features include the presence of lymphoid aggregates altering with pale - appearing areas composed of histiocytes and plasma cells.6 the diagnosis is based on the identification of large histiocytic cells showing emperipolesis.4 emperipolesis is the presence of intact lymphocytes , plasma cells , erythrocytes or polymorphonuclear leukocytes engulfed within the cytoplasm of the histiocytes.6 immunohistochemical stains are useful in differentiating rdd disease from other histiocytic disorders . the rdd histiocytes are strongly positive for s-100 and cd 68 as was observed in our case . no immunoreactivity to cd1a is observed ( cd1a is expressed in langerhans cells ) . to summarize , eyes and ocular adnexa , including lacrimal gland involvement represent the most common extranodal areas affected by rdd disease .

rosai - dorfman - destombes ( sinus histiocytosis with massive lymphadenopathy ) disease is an uncommon disease characterized by benign proliferation of histiocytes , with painless lymph node enlargement and frequent extranodal disease . orbital involvement occurs in 9 - 11% of cases . however , isolated rosai- dorfman - destombes disease of the lacrimal gland without any systemic involvement is very rare with only three case reports . we describe here one such young male patient with unilateral lacrimal gland swelling . excision biopsy revealed almost complete replacement of the lacrimal gland by lymphocytes , plasma cells and large pale histiocytes . the latter exhibited emperipolesis and stained positive for s-100 and cd68 on immunohistochemistry . patient is well and has no other manifestation or recurrence of the disease during a follow - up of 24 months .

the myelodysplastic syndromes ( mds ) are a group of clonal hematopoietic stem cell disorders characterized by cytopenia(s ) , dysplasia in one or more of the major myeloid cell lines , ineffective hematopoiesis , and increased risk for development of acute myeloid leukemia ( aml)(13 ) . approximately 50% of patients have a detectable cytogenetic abnormality during routine gtg - banded karyotyping ( 4 ) and the most commonly involved chromosomal changes observed are monosomy 5 or del(5q ) , monosomy 7 or del(7q ) , trisomy 8 , del ( 20q ) and del(9q ) ( 59 ) . several studies have established the prognostic value of cytogenetic analysis in mds both for survival and progression to aml ( 1012 ) . for example , an isolated 5q deletion is characterized by longer survival and lower progression rates to aml ( 12 ) . in mds rare abnormalities can be observed frequently ( 7 ) . to our knowledge , there has been no case report of del ( 6q ) in mds in iran , and there are only four case reports worldwide ( 1316 ) . a 66-year - old woman was admitted at imam khomeini hospitalcomplexes affiliated to tehran university of medical science , in august 2012 for further evaluation of moderate refractory anemia . in addition , she had received supportive cure in the form of transfusions . at admission , the patient had ill appearance without fever and showed no splenomegaly , lymphadenopathy , or organomegaly on physical examination . a complete blood evaluation revealed hematocrit of 24.7% , hemoglobin 7.7 g / dl , red cell count 2.26 10/l , mcv 109 fl , reticulocytes 2.2% of red blood cells , leukocyte count 9.4 10/l ( neutrophils 60% , lymphocytes 35% , monocytes 1% , eosinophils 1% ) and platelet count 288 10/l . blood film demonstrated red cell anisocytosis , with macrocytosis and moderate numbers of target cells . granulocytes showed shift to the left and dysplastic changes without ring sideroblasts and blasts comprised 1% of nucleated cells and + 4 iron store . the pathologic findings of aplastic anemia or bm failure diseases such as hypocellular or acellular marrow were not observed . diagnostic cytogenetic analysis performed on the diagnostic bone marrow sample revealed the karyotype as 46 , xx , del(6 ) ( q13q16 ) in all 20 metaphase cells which analyzed ( fig . the patient was diagnosed as having refractory cytopenia with unilineage dysplasia ( rcud)based on the 2008 who classification system.(17 ) . after her general condition recovered , she was discharged and followed up with cbc and liver function tests . in our patient , a diagnosis of rcud was made on the basis of peripheral blood and bone marrow findings . rcudincluding 10%20% of the cases of mds ( 18 ) and deletion of the long arm of chromosome 6 as the only diagnostic finding is rare in mds based on other studies ( 1316 ) . cytogenetic abnormalities associated with ra include del ( 20q ) , + 8 and abnormalities of chromosome 5 and/or chromosome 7 ( 11 ) . specific chromosomal abnormalities , such as -5/5q- and -7/7q- , play an important role in the development of new therapeutic options and clinical management of mds . however , due to cytogenetic heterogeneity , the additive prognostic impact of distinct single additional abnormalities remains obscure as yet . to our knowledge there are only four case reports worldwide about del ( 6q ) in mds ( 1316 ) . in 2000 , fernandez et al.(13 ) described the chromosomal abnormality 46 , xx , del ( 6)(q21 ) in a 18-year - old woman with ra and a 42-year - old man with refractory anemia with excess blasts in transformation it should be noted thatthe category of raeb - t has been abandoned in the new who classification of mds . the majority of patients which previously belonged to this group are now including in aml classification ( 19 ) . therefore this chromosomal abnormality can not attribute to aml progression . in 2006 , cen et al . ( 15 ) reported one case with 6q- in cytogenetic analysis of bone marrow cells of the 50 mds patients . ( 14 ) reported 46,xx , del(6)(q15q27 ) in a 72-year - old woman with refractory anemia ( mds - ra ) . the patient did not demonstrate of progression after 10 months of follow up . in 2011 , chaubey et al . ( 16 ) described two rare chromosomal abnormalities ( 6q- , 3q- ) with unknown prognostic significance . approximately 90%95% of patients with refractory anemia have low or intermediate international prognostic scoring system ( ipss ) ( 11 ) . in addition , when adjustments were made for age , patients with mds and transfusion dependency ( td ) had more than a two - fold higher risk for mortality and six - fold higher risk of leukemic transformation compared with patients without td during the initial three years after diagnosis ( 20 ) . on the other hand , mecucci et al . ( 21 ) recognized a significant correlation between myelodysplasia and previous exposure to toxic products such as alkylating agents . our patient was not exposed to toxic products and she had eastern cooperative oncology group ( ecog ) performance status of 1(22 ) with no evidence of progression after 12 months of follow - up . the genetic changes in the malignant cells of mds result mainly in the loss of genetic material , including probable tumor suppressor genes . it is noteworthy that deletions of the long arm of chromosome 6 and gain of chromosome 6 have already been described as poor prognosis in mds and aml ( 23 , 24 ) . the finding indicates that 6q- deletions are accompanied by structural and functional alterations of the c - myb locus which may be involved in the pathogenesis of leukemias and lymphomas ( 25 ) . due to the profound cytogenetic heterogeneity , the impact of many rare abnormalities such as del ( 6q ) in patients with mds is still unknown and further such cases are needed to find the impact of this abnormality on progress and prognosis . identification of all possible rearrangements is valuable for obtaining a better understanding of the leukemogenic process and for determining genetic pathways . these may also play an important role in the development of new therapeutic options in mds . ethical issues ( including plagiarism , informed consent , misconduct , data fabrication and/or falsification , double publication and/or submission , redundancy , etc ) have been completely observed by the authors .

abstractbackground : the myelodysplastic syndrome ( mds ) is a highly heterogenous disorder and karyotype analysis is helpful for diagnostic and prognostic estimation . deletion in long arm chromosome 6 ( 6q del ) as a sole abnormality is a rare event in mds . this is the first case report of del ( 6q ) as the only observed diagnostic change in iran . we also reviewed the literature of this cytogenetic lesion .

the cyst of anterior cruciate ligament ( acl ) is a known clinical entity , but its association with knee synovitis and melon or rice bodies is not documented . we report a rare case of ganglionic cyst of of the knee in association with diffuse synovitis and multiple melon or rice bodies in a 36 year old male . the case was treated arthroscopically with removal ofthe cyst of acl and multiple melon seed bodies . information regarding incidence , treatment , and outcomes for patients with synovial cysts and melon seed bodies is lacking . arthroscopic examination of joint gives the opportunity to diagnose such rare entity of the joint and also provide minimally invasive effective treatment of such pathology . the cysts of acl are known and described in the literature , but their association with diffuse synovitis and multiple melon or rice seed bodies has not been well documented . we report a rare case of a 36 year old gentleman who underwent arthroscopy of knee for swelling and locking , was found to have synovial cyst and melon seed bodies which were removed . the outcomes of such patients and recurrences have not been well described in the literature . a 36 year old gentleman presented with complaints of dull aching pain in the right knee for 6 months which was aggravated on walking and relieved on rest . patient did not give any history of fever , weight loss or instability while walking . there was no history of any other joint symptoms , comorbidities or long term drug intake . the clinical examination revealed parapatellar fullness over right knee without any local rise of temperature or tenderness . plain x ray revealed mild joint space reduction medially ( fig . 1 ) and mri revealed posterior horn degeneration of medial meniscus with mild effusion in the knee joint and a cyst behind the acl . 2 ) showed diffuse synovitis with well - defined cyst posterior to the acl ( fig . about 50 - 60 ml of synovial fluid mixed with multiple loose bodies ( fig 4 ) was removed . the histopathological examination revealed synovial chondromatosis and no evidence of tuberculosis or rheumatoid arthritis . at 3 month follow - up , there was complete resolution of swelling and pain with full range of motion of the knee . intraopertive picture showing diffuse synovitis and melon bbodies arthroscopic picture showing acl cyst & removal of melon bodies synovial fluid with melon seed bodies cysts associated with the anterior cruciate ligament ( acl ) are rare with an incidence of less than 1% . prevalence rates for cysts in two large mri series was 0.25% and 0.44% and in an arthroscopic series of 8,000 cases , only 49 ( 0.6% ) cystic masses were found to originate from the acl . a cyst in the mid - portion of the acl was first described by caan in 1924 , in the cadaver of an elderly man with no documented antemortem symptoms referable to the knee . the etiology of these lesions remains obscure , and a history of significant trauma is obtained in only a minority of cases . theories include post - traumatic mucinous degeneration of connective tissue mediated by local release of hyaluronic acid , herniation of the synovium into a defect in surrounding tissue , and even displacement of synovial tissue during embryogenesis . symptoms comprise anteromedial knee pain aggravated by changing direction while running , on squatting or with extreme flexion and extension , and may resemble those of internal derangement . a ganglion can arise as a cystic lesion from a tendon sheath or a joint capsule and contain a glassy , clear , and jelly - like fluid . a review of the literature reveals a controversial discussion about the clinical significance as well as the etiology of ganglion cysts arising from the cruciate ligaments . these case reports show that an intra - articular ganglion cyst of the cruciate ligaments is difficult to diagnose . a cyst does not necessarily have to be associated with specific clinical symptoms or a previous trauma . due to its multiplanar capability , mri is the imaging modality of choice for diagnosis of these lesions , and demonstrates fusiform swelling of the acl . the cysts return homogenously low signal intensity on t 1-weighted images and high signal intensity on t 2-weighted images which are articularly good at contrasting the cysts against an intact acl . arthroscopic evaluation and removal of synovial cysts and loose bodies although it was observed that most synovial cysts were asymptomatic and did not need any sort of treatment and were found incidentally . most patients have good or excellent results after arthroscopic excision of acl cysts ; postsurgical recurrence has not been reported . melon or rice bodies are free corpuscles of synovial origin with a cartilage - like appearance that may reach hundreds in number in the intra - articular space . rheumatologic or infectious pathologies that may produce synovial hypertrophy play a major role in the etiology . magnetic resonance imaging ( mri ) is the most useful diagnostic modality for detecting acl cysts and the treatment of choice in symptomatic cases is by arthroscopic surgery . information regarding incidence , treatment , and outcomes for patients with synovial cysts and melon seed bodies is lacking . arthroscopic examination of joint gives the opportunity to diagnose such rare entity of the joint and also provide minimally invasive effective treatment of such pathology . awareness about the association of an acl cyst with diffuse knee synovitis and melon bodies is crucial to reach an early diagnosis . arthroscopic surgery provides an accurate diagnosis and means to remove the melon bodies while carrying simultaneous synovectomy .

introduction : the cyst of anterior cruciate ligament ( acl ) is a known clinical entity , but its association with knee synovitis and melon or rice bodies is not documented.case report : we report a rare case of ganglionic cyst of of the knee in association with diffuse synovitis and multiple melon or rice bodies in a 36 year old male . the case was treated arthroscopically with removal ofthe cyst of acl and multiple melon seed bodies.conclusion:information regarding incidence , treatment , and outcomes for patients with synovial cysts and melon seed bodies is lacking . arthroscopic examination of joint gives the opportunity to diagnose such rare entity of the joint and also provide minimally invasive effective treatment of such pathology .

appropriate timing of treatment is the key to successful intensive care ; treatment that is delayed could be as detrimental as treatment that is premature . therefore , lange and colleagues , in this issue of critical care , reported on the time course of the expression of the different isoforms of the nitric oxide synthase ( nos ) - that is , neuronal nos [ nnos ] , inducible nos [ inos ] , and endothelial nos [ enos ] ( also known as nos1 , nos2 , and nos3 , respectively ) - of the tissue levels of nitrotyrosine and poly(adp - ribose ) , markers of nitrosative stress , and dna damage resulting from peroxynitrite formation as well as p65 , a mirror of the activation of the nuclear factor - kappa - b ( nf-b ) , in a well - established resuscitated ovine model of septic shock induced by pseudomonas aeruginosa pneumonia . ' downstream effects ' were evaluated by measuring nos activity , nitric oxide ( no ) production , and interleukin-8 concentrations . during the early phase ( that is , 4 to 12 hours after induction of pneumonia ) , enos expression was increased , and this coincided with increased tissue levels of nitrotyrosine , poly(adp - ribose ) , and nf-b activation , whereas in the later phase ( that is , until 24 hours of pneumonia ) , measurable nos activity and no production were related mainly to inos activation . the authors ' ' two - hit ' model of cotton smoke inhalation and subsequent instillation of live bacteria is characterized by a hyperdynamic circulation , hypoten - sion , tissue acidosis , and progressive impairment of gas exchange , lung mechanics , and morphological alterations typical of acute lung injury . furthermore , the model comprises resuscitation measures and thus allows the study of pathophysiological pathways in a clinically relevant , large - animal setting . numerous studies evaluated the no - related mediator orchestra and highlighted the friend - and - foe character of excess no formation : no not only is well established as a reactive nitrogen species ( rns ) , often referred to as a ' final mediator ' of sepsis - induced hypotension , but also acts as a scavenger of reactive oxygen species ( ros ) , such as the superoxide radical . this reaction , however , leads to the formation of the even more toxic peroxynitrite , which ultimately results in protein nitrosylation , dna damage , and activation of poly(adp - ribose ) polymerase ( parp ) . finally , the no production rate depends on the stimulus and the species , and therefore rodent data can not be transferred directly to the clinical scenario . endogenous no production in large animals is much closer to that of human beings , but although increased no production during sepsis is well established , these models yielded controversial results . the early and transient ( within minutes ) activation of parp would make parp inhibition an attractive approach , but unfortunately medical care is usually not available during this very early phase . moreover , the pathophysiological consequences of parp-1 activation are opposed to its vital role in the maintenance of genomic integrity through its function in base excision repair , and the effects of parp inhibition on dna damage and repair during shock are still a matter of debate . therefore , parp inhibitors are currently investigated in ischemia - reperfusion , oncology , and diabetes rather than in sepsis or acute lung injury , and peroxynitrite - neutralizing agents are a tempting alternative . several studies explored the potential of selective inhibition of nos isoforms under the assumptions that nnos and enos are constitutively producing homeostatic no and that inos responds to acute stimuli with excessive no production . in fact , the crucial role of enos expression seems to be unequivocal : enos activation improved microvascular perfusion and cardiac function in rodents , and enos polymorphism was associated with hypotension during human gram - negative sepsis . clearly , inos still seems to be a ' bad guy ' : several studies showed beneficial effects of various selective inos inhibitors on hemodynamics , lung function , deranged microcirculatory perfusion , coagulation disorders , and visceral organ injury [ 12 - 14 ] . recent data , however , suggest that nnos activation may also assume major importance , and a combined approach using selective nnos inhibition during the early phase ( 0 to 12 hours ) and inos inhibition during the later phase ( 12 to 24 hours ) yielded improved pulmonary function and attenuated nitrosative stress . finally , selective inos inhibition together with the ros scavenger tempol also afforded significant protection , further emphasizing the close interaction of no and oxidative / nitrosative stress . despite these encouraging results , nothing is simple or easy : in resuscitated murine septic shock , both genetic deletion and selective pharmacologic blockade of the inos were associated with markedly improved systolic contraction and catecholamine responsiveness but simultaneously deteriorated diastolic relaxation unfortunately , the authors did not report data on oxidative stress , so a complete overview of the whole rns - and ros - related mediator orchestra is only implicitly provided . nevertheless , the authors add an important piece to the complex puzzle of the no - related pathophysiological pathways : nitrosative stress ( that is , increased nitrotyrosine and poly[adp - ribose ] levels ) was aggravated only during the early phase up to 12 hours , whereas clear - cut increases in nos activity , no metabolites , and subsequently cytokines occurred only later on . interestingly , in this experiment , in contrast to previous reports from the same group , nnos synthesis was not increased . finally , despite its inherent protective properties , enos activation was also involved in the initiation of the septic response , and it remains to be elucidated whether this mirrors an adaptive or pathologic mechanism . how can we translate these data to daily care ? usually , there are few chances to intervene during the very early , evolving phase of sepsis and acute lung injury , and at the time of full - blown sepsis , all members of the mediator orchestra are already playing their ( un)coordinated , and unfortunately sometimes uncontrollable , concert . there are two ways to go from here , and both are worthy of being followed ! first , though extremely time- and resource - consuming , long - or longer - term large - animal models with a more prolonged observation period ( that is , days ) are needed and will probably yield valuable clues to the design of clinical studies . second , more studies on humans are warranted in order to better describe early and later phases of human sepsis in terms of nos , peroxynitrite formation , and parp activation . enos : endothelial nitric oxide synthase ; inos : inducible nitric oxide synthase ; nf-b : nuclear factor - kappa - b ; nnos : neuronal nitric oxide synthase ; no : nitric oxide ; nos : nitric oxide synthase ; parp : poly(adp - ribose ) polymerase ; rns : reactive nitrogen species ; ros : reactive oxygen species .

appropriate timing of treatment assumes particular importance in critical care . lange and colleagues recently reported on the time course of the different nitric oxide synthase ( nos ) isoforms , nitrosative stress , and poly(adp - ribosylation ) during pseudomonas aeruginosa pneumonia - induced ovine septic shock . initially , endothelial nos expression was increased together with markers of peroxynitrite formation , dna damage , and nuclear factor - kappa - b activation . later on , measurable nos activity and nitric oxide production resulted mainly from inducible nos activation . these results emphasize the need for long - term , large - animal studies investigated over days so that future therapeutic interventions can be better tailored and matched to the exact time course of the activation of the mediator orchestra .

congenital hearing loss is a relatively common disorder and it 's prevalence is approximately 1 in every 1,000 live births and about half of these cases are hereditary ( 1 ) . the pattern of inheritance can be dominant , recessive , x - linked and mitochondrial and more than one hundred genes could be involved in hearing impairment . most cases of hereditary hearing loss are nonsyndromic sensorineural hearing loss , with autosomal recessive forms the most common ( 1 ) . although a number of different genes have been associated with autosomal recessive nonsyndromic hearing loss ( arnhl ) , mutations in the gjb2 gene ( genbank m86849 , mim 121011 ) encoding connexin 26 ( cx26 ) , with chromosomal location 13q11 - 12 , known as dfnb1 ( mim 220290 ) , accounts for up to half of the cases of arnhl ( 2 , 3 ) . gene analysis in korean deafness patients also suggest that gjb2 mutations make up a major cause of congenital hearing loss ( 4 ) . however , few gjb2 mutations have been described to be associated with syndromic autosomal dominant hearing loss ( adhl ) in korea . herein we report a familial case of adhl presenting with palmoplantar keratoderma ( ppk ) associated with a gjb2 mutation , in a mother and daughter . a 3-yr - old korean female presented for evaluation of diffuse thickened scaly plaque on the palms and soles ( may 3 , 2007 ) . the physical examination revealed diffuse hyperkeratosis of the palms and soles and keratotic plaques on the knuckle areas , reported to be present since 2 yr of age ; there was no evidence of additional abnormalities including the teeth , hair or nails ( fig . diagnostic skin biopsies were obtained after written informed consent . a biopsy specimen from the sole showed compact hyperkeratosis , acanthosis with a well - formed granular layer , consistent with the diagnosis of ppk ( fig . her mother also had diffuse hyperkeratosis of the palms and soles , present since infancy , and biopsy from the sole of the mother 's foot showed similar features ( fig . , the patient was affected with bilateral severe to profound congenital sensorineural hearing loss that was diagnosed by an auditory brainstem response ( abr ) , and an otoacoustic emissions ( oae ) test at 6 months of age . the maternal grandfather , mother , and aunt also had a history of hearing loss and hyperkeratosis of the palms and soles with the onset in infancy ( fig . 3 ) . the combination of hearing loss and ppk inherited as an autosomal dominant trait led us to test for a mutation in the gjb2 gene in this patient and her mother . as shown in fig . 4 , mutation analysis of the entire gjb2 gene in the mother identified a heterozygous r75w mutation and v27i mutation . in addition , the mutation analysis of the entire gjb2 gene in the patient identified a heterozygous r75w mutation that caused an arginine to tryptophan substitution ( r75w ) at position 75 . cochlear implantation was done to modify hearing loss of the patient and further hearing rehabilitation had been provided by the hearing support service team in hospital . her functional auditory skills were slightly improved and attended to special school for the children who had hearing impairment . six gjb2 mutations have been found in association with syndromic ppk and adhl ( dele42 , g59a , r75q , h73r , g130v and including r75w ) ( 5 - 10 ) . the r75w mutation was previously reported in association with hearing loss and keratoderma in familial and sporadic cases ( 9 , 11 , 12 ) . in both the mother and the affected daughter therefore , this specific mutation must be the cause of hereditary hearing loss and ppk in her mother and the patient . similar to the present patient , r75w mutation in previous report ( 9 , 11 , 12 ) also has shown adhl associated with ppk . while , the v27i type of mutation , observed only in the mother , is relatively common in koreans with arnhl ( 13 ) , and this specific mutation may lead to the hereditary hearing loss in maternal family members including grandparents . thus , in this case , defect of cx26 , resulting from r75w mutation of gjb2 gene , was thought to be the cause of hereditary hearing loss and ppk in her mother and the patient . gap junctions are ensembles of gap junction channels formed by integral membrane proteins called connexins . cx26 , one of the human connexins , is found in a variety of cells and tissues , including the cochlea , epidermis of the palms and soles , hair follicles , and sweat glands ( 14 ) . cx26 contains four transmembrane domains with a cytoplasmic n - terminus , two extracellular loops , a single intracellular loop , and a cytoplasmic c - terminus . the extracellular loops , especially the first extracellular domain , are of critical importance for voltage gating , channel permeability , connexon - connexon interactions , and formation of gap junctions ( 15 , 16 ) ; mutations of this domain lead to interference with the activity of cx26 channels and consequently functional defects in gap junctions of the auditory organs . in addition to hearing loss , mutations in the first extracellular domain of cx26 are known to be associated with skin disease , which suggests that the first extracellular domain of cx26 is also essential for the correct formation and/or function of gap junctions in the epidermis of the palms and soles . likewise , it is well known that most pathogenic gjb2 mutations , including r75w mutation , causing both hereditary hearing loss and skin manifestations cluster in the first extracellular domain of the cx26 peptide ( 17 ) . this present case showed familial occurrence and a relatively early age of onset of ppk . in addition to hearing loss , a number of inherited ppks are also associated with a genetic predisposition to other conditions , including cancer and heart failure ( 18 ) . as a result , clinicians should be aware that infancy with inherited ppk may have additional medical problems . the genetic mapping and identification of genes may help with the diagnosis and prevention of other possible medical problems in such cases . in conclusion , the simultaneous occurrence of a gjb2 mutation in a mother and daughter suggests that r75w mutation cause adhl presenting with ppk . to the best of our knowledge , this is the first report of a gjb2 mutation associated with syndromic autosomal dominant hearing loss and ppk in a korean family .

gap junctions , which mediate rapid intercellular communication , consist of connexins , small transmembrane proteins that belong to a large family of proteins found throughout the species . mutations in the gjb2 gene , encoding connexin 26 , can cause nonsyndromic autosomal recessive or dominant hearing loss with or without skin manifestations . a 3-yr - old korean female and her mother presented to our clinic with diffuse hyperkeratosis of the palms and soles ( may 3 , 2007 ) . skin biopsies from the soles of both patients demonstrated histopathological evidence of palmoplantar keratoderma . the patient and a number of her maternal family members also had congenital hearing loss . the combination of congenital hearing loss and palmoplantar keratoderma , inherited as an autosomal dominant trait , led us to test for a mutation in the gjb2 gene in both patients . the results showed the r75w mutation of the gjb2 gene in both . in conclusion , the simultaneous occurrence of a gjb2 mutation in a mother and daughter suggests that r75w mutation cause autosomal dominant hearing loss presenting with palmoplantar keratoderma . to the best of our knowledge , this is the first report of a gjb2 mutation associated with syndromic autosomal dominant hearing loss and palmoplantar keratoderma in a korean family .

bacterial vaginosis ( bv ) is the most common infection of the female reproductive tract . bv refers to clinical syndromes resulting from replacement for normal lactobacilli vaginal flora with anaerobic bacteria including prevotella sp . reactive arthritis ( rea ) occurs worldwide in 1 - 4% of patients a few days to six weeks with an average of four weeks , after infection of the urogenital or enteric tract , and 30 - 70% of them are positive for human leukocyte antigen ( hla)-b27 . rea has been described in association with pathogens including shigella spp . , salmonella spp . , blastocystis hominis , cryptosporidia , chlamydia trachomatis , ureoplasma ureolyticum , mycoplasma hominis , neisseria gonorrhoea , streptococci , staphylococci , leptospira , borrelia , and mycobacterium tuberculosis we describe a 42-year - old woman presenting with rea affecting several joints and enthesitis who suffered from bv . a 42-year - old woman presented with a history of pain and swelling of her several joints . at first , she complained of tenderness and pain and warm swelling of her right ankle and after two days she also had a similar problem in her left ankle . after that , she had arthralgia in the knees and her right elbow and then swelling of the metarsal joints have developed within two weeks . she claimed that resting and ibuprofen could reduce , but not cure her swelling and pain . she hasnt had a history of diarrhea or dysuria or vaginal secretion , or sexually transmitted infections ( stis ) . , she had difficulty walking due to heel pain , a fever ( 38.8 c ) , swelling of her right ankle and right metatarsophalangeal joints . she had tenderness on both of achilles tendons attachments , the base of her right fifth metacarpal , and both patellar tendon insertions . laboratory data were normal for hemoglobin , leukocytes , erythrocyte , serum electrolytes , chemistry studies , and urine analysis except for erythrocyte sedimentation rate ( esr ) which was 42 and c - reactive protein ( crp ) was slightly elevated . tests for human immunodeficiency virus ( hiv ) antibody , hepatitis b and c , brucellosis antibody , chlamydia trachomatis antibody , cold agglutination test , rheumatic factor , lupus erythematosus ( le ) cells , antinuclear antibody ( ana ) and anti double stranded ( ds ) dna were negative . her pelvic examination revealed homogeneous white grey and malodorous vaginal discharge on the vaginal wall . her pap smear and gram - stained smear of vaginal swab showed benign cellular changes , predominance of gram - variable coccobacilli consistent with shift in vaginal flora , mild inflammation . the typical pattern of rea is an asymmetric mono or oligoarthritis , predominantly affecting lower extremities such as the knees , ankles , and feet . enthesitis , inflammation occurring at the point of attachment of skeletal muscles to bone , is common in rea and presents with a localized pain , swelling , and tenderness . the plantar aponeurosis and achilles tendon attachments to the calcaneum are very common and often cause heel pain and difficulty walking . this patient had tenderness on both of achilles tendons attachments , the base of her right fifth metacarpal , and both patellar tendon insertions and complained of difficulty walking too . however , diagnosis of rea is based on having mono- or oligoarthritis of the lower extremities and exclusion of other diagnosis . rea happens after a urogenital or enteric infection . the number of infectious agents associated with rea is gradually increasing . sti is also well recognized as a provoking cause of rea which named sexually acquired rea ( sara ) . although , rea linked to gardenella vaginalis infection is rare , but gardenella vaginalis as a triggering pathogen has been reported . based on our knowledge , two of them are female and others are male . finding a source of active infection and triggering agent might be helpful to diagnose and treat the patient successfully . the vaginal gram - stained smears used as a diagnostic test of bv and pap smear screening can be used for detecting bv with minimal costs and high specificity . the homogeneous white grey and malodorous vaginal discharge on the vaginal wall and pap smear and gram - stained smear results in our patient was compatible with bv and gardenella vaginalis . the recurrence of bv after treatment has been reported . the results of clinical trials indicate that a woman 's response to therapy and the likelihood of relapse or recurrence are not affected by treatment of her sex partner(s ) . early eradication of infection may reduce or limit the dissemination of bacteria or bacterial antigens to the joint , so the initiation of the subsequent rea might prevent . rea is a paradigm of a rheumatic disease in which the initiating infectious cause is known . early uses of antimicrobial drugs may prevent the development of musculoskeletal symptoms which are triggered by urogenital and some enteric infections . this patient recovered with metronidazole and no recurrent sign of rea has been observed during her six - month follow - up . bacterial vaginosis and gardenella vaginalis should be considered as one of triggering pathogens in differential diagnosis of rea . the identification of microbes or microbial elements in the joint or their possible persistence at other sites may help to determine a possible source and effective antimicrobial drugs may prevent the development of rea .

we report a 42-year - old woman with reactive arthritis induced by bacterial vaginosis who presented with oligoarthritis with an additive form , arthralgia , and enthesitis . she hasnt had a history of diarrhea or dysuria or vaginal secretion , or sexually transmitted infections ( stis ) . the laboratory tests were normal except for a high erythrocyte sedimentation rate ( esr ) . her pelvic examination revealed homogeneous white grey and malodorous vaginal discharge on the vaginal wall and pap smear and gram - stained smear of vaginal swab was consistent with bacterial vaginosis . she responded to metronidazole therapy and her six - month follow up hasnt shown recurrence of arthritis . as reactive arthritis ( rea ) is a paradigm of a rheumatic disease in which the initiating infectious cause is known , so early use of antimicrobial drugs may prevent the development of musculoskeletal symptoms which are triggered by infections .

a 60-year - old lady presented to her general practitioner with symptoms of fatigue , tiredness and generally being unwell . upon investigation , she was found to have iron deficiency anaemia ( haemoglobin 6.5 g / dl ) . she did not report any haematemesis or melaena , weight loss , abdominal pain or vomiting and had no significant family history or risk factors for gastrointestinal pathologies . following blood transfusion , she underwent endoscopy and was found to have a large polyp in the first part of the duodenum ( d1 ) . the polyp had a broad base of more than 1 cm in the proximal d1 and extended just proximal to the ampulla , involving the superior 180 degrees of the duodenal wall ( fig . 1 ) . multiple biopsies demonstrated duodenal mucosa with moderate chronic inflammation and foci of gastric epithelium . ct scan confirmed a solitary 6 3 cm duodenal polyp with duodenal intussusception ( fig . a repeat upper gastrointestinal endoscopy , again with multiple biopsies ( small intestinal type mucosa with villi lined by mildly dysplastic epithelium ) of this solitary polyp did not shed any further light on the subject . the lesion was further assessed by endoscopic ultrasound ( eus ) which excluded any invasive component and suggested possible duodenal lipoma based on ultrasound characteristics . endoscopic mucosal resection was not considered feasible due to the size of the lesion , as well as the large base with which it was attached to the first part of the duodenum . hence a transduodenal resection of the polyp in the submucosal plane was performed , with a sleeve of surrounding mucosa to excise all grossly abnormal mucosa . the patient made an uncomplicated recovery from surgery and was discharged home on the sixth postoperative day . final histopathological examination revealed the lesion to be a submucosal lipoma with overlying mucosal prolapse and reactive / regenerative atypia . an online search in pubmed for duodenal lipomas generated 174 articles , of which 26 were specific for duodenal lipomas and in english . of these 26 articles , 9 case reports were identified to have been published in the last 10 years . the age of the patients ranged from 46 to 81 years with 5 females and 4 males and most of them presented with melaena . the size of the tumours ranged from 2 to 5 cm and most of them were excised endoscopically . report two cases wherein , despite the tumours being small ( less than 3 cm ) , both patients were symptomatic with postprandial abdominal fullness and were treated with endoscopic excision . in our patient , the size of the lesion was 6 3 cm , which is the largest in comparison to the published reports . postoperative recovery was uneventful and on subsequent follow - up , her anaemia ameliorated and she did not suffer from any long - term complications . a study by mayo et al . showed that out of 4,000 cases of benign gastrointestinal tumours , 164 ( 4% ) were lipomas . the site most common was the colon ( 64% ) , followed by small intestine ( 26% ) , duodenum ( 4% ) , stomach ( 3% ) and oesophagus ( 2% ) . in the duodenum , lipomas tend to be noted mostly in the second part and are most often situated in the submucosal plane , but can also be subserosal and can be sessile or pedunculated . the occurrence of symptoms , if any , largely depends on the size of the lesion . tumours greater than 4 cm can produce symptoms of abdominal pain and discomfort or cause hypochromic microcytic anaemia due to ulceration and bleeding , or intestinal obstruction due to intussusception . acute haemorrhage is a rare complication with fewer than 15 cases reported [ 4 , 5 ] . direct visualisation at endoscopy will provide a clue to the nature of the tumour , however , as duodenal lipomas are submucosal , superficial biopsies will not be sufficient and deeper biopsies will be needed to provide a definitive diagnosis . imaging modalities such as ct scan and eus ct scan of the upper gastrointestinal tract can fairly accurately facilitate the preoperative diagnosis of lipoma based on low attenuation signals of 50 to 100 hounsfield units . eus features of a homogenous , hyperechoic mass within the submucosal layer are highly characteristic of duodenal lipomas and in addition , eus can also visualise the depth and invasion . the recommended treatment is endoscopic excision if the lesion is polypoid and of manageable size . for large and sessile lesions , endoscopic excision may prove technically difficult and increase the risk of bleeding and perforation , thus surgical excision would be the preferred approach in such cases . in conclusion , duodenal lipomas are uncommon benign tumours that can present with signs and symptoms of upper gastrointestinal bleeding . they can be suspected on endoscopic visualisation but may require ct or eus for further evaluation . endoscopic excision is the preferred treatment , but if the size of the lesion precludes endoscopy , surgical excision is the treatment of choice .

common causes of chronic upper gastrointestinal bleeding include oesophageal varices , gastroduodenal ulcers and malignancy , and patients mostly present with iron deficiency type anaemia . we present the case of a 60-year - old lady who presented with iron deficiency anaemia and on investigation was found to have a large duodenal polyp requiring surgical excision . on histological examination , the polyp was revealed to be a lipoma . we review the recent literature and formulate a management plan for this rare entity .

one of the rare causes for androgen excess is sex cord - stromal tumors of the ovary . , we report case of a 48-year - old woman who presented with hyperandrogenism due to bilateral ovarian thecoma . this , to the best of our knowledge , is the first case of bilateral thecoma presenting as hirsutism in a premenopausal woman . a 48-year - old woman presented with slowly progressive increase in terminal hair growth over the face for 8 years . she had regular periods , was married , had two children , last child birth was 30 years back . she did not experience any scalp hair loss , acne , voice change , galactorrhea , abdominal pain , abdominal distension , symptoms of raised intra - cranial tension and change in appetite or weight . her weight , height and body mass index were respectively 95 kg , 163 cm and 35.15 kg / m . her hormonal profile showed : thyroid - stimulating hormone 4.2 ( 0.27 - 4.2 miu / ml ) , luteinizing hormone ( lh ) 8.68 ( 2.4 - 12.6 miu / ml ) , follicle stimulating hormone ( fsh ) 7.06 ( 3.5 - 12.5 miu / ml ) , total testosterone 3.62 ( 0.029 - 0.408 ng / ml ) , dehydroepiandrosterone ( dheas)119.4 ( 60.9 - 337 g / dl ) , prolactin 17.70 ( 6.0 - 29.9 ng / ml ) and 8 am cortisol 10.39 ( 6.2 - 19.4 g / dl ) . over - night dexamethasone suppression and basal 17-oh progesterone was 1.19 g / dl and 1.9 ( 0.2 - 1.72 ng / ml ) respectively . magnetic resonance imaging ( mri ) scan of the abdomen and pelvis showed normal adrenals with bilateral ovarian masses [ figure 1 ] . t2 weighted magnetic resonance imaging of pelvis showing bilateral ovarian masses , which are homogeneous and mildly hyperintense ( arrows ) . there are no follicles seen in bilateral ovaries in the presence of normal dheas levels and normal appearing adrenals with bilateral solid mass lesions in both ovaries , virilizing ovarian tumor was considered . her post - operative period was uneventful and her testosterone levels were normalized ( 0.161 ng / ml on 3 post - operative day and 0.059 ng / ml a week later ) . serum testosterone , lh and fsh levels pre and post - surgery gross and microscopic picture : bilateral thecoma of ovary . ( b ) ( h and e , 40 ) and ( c ) ( h and e , 200 ) . ( d ) immunohistochemistry shows positivity for vimentin ( h and e , 100 ) . ( e ) special stains show the presence of intra - cellular lipids ( oil red o positive ) ( h and e , 200 ) ovarian tumors constitute < 0.2% of cases of hyperandrogenism and < 1% of all ovarian tumors . there are some rare benign ovarian tumors where there is a slow progression and long duration of symptoms before the diagnosis is established . the histological classification of ovarian tumors by the world health organization is based on histogenetic principles with regard to their derivation from coelomic surface epithelial cells , germ cells , and mesenchyme ( the stroma and the sex cord ) . the sex cord - stromal and steroid cell tumors of the ovary are classified as sex cord - stromal cell tumor ( granulosa and thecoma - fibroma ) , sertoli - stromal cell tumors , mixed or unclassified and steroid cell tumors . ovarian tumors that present with hyperandrogenism include leydig cell tumors , sertoli cell tumors , steroid cell tumors - not otherwise specified and ovarian thecomas . other rare ovarian causes of hyperandrogenism include gynandroblastoma , gonadoblastoma , ovarian carcinoid , surface epithelial tumors ( brenner tumor ) and metastatic tumors . tumoral cause of hyperandrogenism is suspected when the serum testosterone levels are significantly elevated ( > 200 ng / ml ) , rapidly progressive symptoms of virilization or pelvic mass in examination / imaging . as mentioned earlier , symptoms and signs associated with hilar leydig cell tumor may present as slowly progressive hirsutism . when imaging does not identify ovarian or adrenal mass and the results of serum dheas are normal , then it is considered an indirect evidence of ovarian tumor if serum testosterone levels exceed 200 ng / dl . these ovarian tumors secrete significant levels of testosterone or its precursor , androstenedione . both fibroma and thecoma of the ovary fibromas are composed entirely of spindle , oval or round cells forming variable amounts of collagen . the differentiation between thecoma and fibroma is occasionally imprecise and the term fibrothecoma has often been used . however , some authors have reported another peak between 20 and 40 years of age . rarely fibrothecomas present with endocrine manifestations related to hormonally active tumors . in a series of 24 cases of ovarian fibroma and fibrothecoma , thecomas are described in adolescents , young adults and older women and not in children . these tumors had presented as primary amenorrhea , secondary amenorrhea , hirsutism , menstrual irregularity or pressure effects . ovarian thecoma with virilizing manifestations were reported in both post - menopausal and premenopausal age groups . ovarian thecoma with concomitant post - menopausal bleeding , endometrial hyperplasia and endometrial carcinoma were reported in a post - menopausal woman . hysterectomy may be performed with oophorectomy especially in premenopausal woman who do nt want to keep their uterus because patients with thecoma may have endometrial hyperplasia or endometrial carcinoma . the treatment of choice for virilizing tumors of ovary and adrenals remains complete surgical excision . further decision regarding chemotherapy should be individualized based on biopsy , metastatic spread and complete excision . testosterone levels can be used as a marker of complete excision and for monitoring the subsequent course . our patient had complete normalization of serum testosterone levels and this correlated with the rise in serum lh and fsh levels to a post - menopausal range . after complete removal and normalization of androgen levels , the clinical manifestations due to hyperandrogenism will take months to resolve . to the best of our knowledge , this is the first case report of premenopausal hirsutism due to bilateral thecoma of ovaries . transvaginal ultrasound and mri aided in the pre - operative localization of tumor in both ovaries . in contrast to other virilizing ovarian tumors , which present as rapidly progressive hirsutism , ovarian thecoma ( like hilus cell tumors of ovary ) can present as slowly progressive hirsutism .

hyperandrogenism is a common disorder among women in the reproductive age group . one of the rare causes for androgen excess is sex cord- stromal tumors of the ovary . these are usually unilateral . here we report case of a 48 year old woman who presented with hyperandrogenism due to bilateral ovarian thecoma . androgen levels normalized following resection of the tumor . this , to the best of our knowledge , is the first case of bilateral thecoma presenting as hirsutism in a premenopausal woman .

since mccully1 first reported the relationship between homocysteine and atherosclerosis , hyperhomocysteinemia ( hhcy ) has come to be recognized as an independent risk factor for atherosclerotic vascular disease.2 further , some studies have demonstrated that hhcy might be involved in the pathophysiology of many neuropsychiatric disorders , including brain atrophy , epilepsy , and alzheimer s disease.35 here we report a patient diagnosed with spinal cord demyelination who presented with hhcy and a homozygous mutation of the gene encoding methylenetetrahydrofolate reductase ( mthfr ) c.677c > t . a 53-year - old man was admitted to our hospital with a 7-day history of numbness below thoracic level ( t)10 . cervical and thoracic spinal magnetic resonance imaging ( mri ) demonstrated that the spinal cord was swollen at cervical level ( c)7t3 , which was considered to represent inflammatory lesions ( figure 1a ) . we treated him with a 7-day course of intravenous prednisone 10 mg / day , and however , this was ineffective . to further distinguish it from the subacute combined degeneration of the cord , we tested his serum homocysteine , vitamin b12 , and folate , which revealed normal serum vitamin b12 ( 268.00 pg / ml ; normal range 211.00911.00 pg / ml ) , elevated serum homocysteine ( 44.49 mol / l ; normal range 515 mol / l ) , and decreased serum folate ( 2.70 ng / ml ; normal range 3.117.5 ng / ml ) . using a previously described method,6 we detected genetic polymorphisms rs1801133 ( c.677c > t ) and rs1801131 ( c.1298a > c ) on the mthfr gene , which encodes the methylenetetrahydrofolate reductase , a key enzyme in homocysteine metabolism . the results showed that the patient carried the tt genotype of the mthfr c.677c > t gene variant and no mutation was found in rs1801131 of the mthfr gene ( figure 2 ) . in light of these findings , the patient was treated with intramuscular cobalamin injections ( 500 g / day ) , vitamin b6 orally ( 30 mg / day ) , and folate orally ( 5 mg / day ) . he was discharged on oral cobalamin 500 g / day and oral vitamin b6 30 mg / day . a subsequent spinal mri performed 3 months after symptom onset showed near resolution of the original lesion ( figure 1b ) . mol / l , normal range 515 mol / l ; vitamin b12 544.10 pg / ml , normal range 211.00911.00 pg / ml ; folate 12.20 ng / ml , normal range 3.117.5 ng / ml ) . here we describe a patient with spinal cord demyelination whose symptoms , initial laboratory findings , and imaging results suggested acute myelitis , and in whom prednisone was ineffective . he had neither anemia nor abnormal serum vitamin b12 , and there were no other clinical or laboratory signs ( red blood cell count , mean corpuscular volume , hematocrystallin , and albumin values were normal ) of malabsorption . a spinal mri then showed that the lesion was not confined to the dorsal and lateral spinal cord . further , considering the acute onset and atypical clinical presentation , it seemed inappropriate to make a diagnosis of spinal cord demyelination , at least of a typical type . further examination revealed that , except for the elevated plasma homocysteine and folate deficiency , the patient was homozygous for the gene encoding mthfr c.677c > t . based on these findings , we treated the patient with b vitamins , and his symptoms gradually improved . as an important enzyme in folate and homocysteine metabolism , mthfr c.677c > t polymorphisms have been associated with hhcy.7 many studies have demonstrated that high levels of homocysteine can increase the risk of vascular disease,2,8 but reports of spinal cord demyelination and hhcy are rare . our case suggests that people with mutation of the mthfr gene combined with hhcy and folate deficiency might be highly susceptible to demyelination . first , mthfr mutation - induced disturbances in methylation process can result in a lower methylation rate of myelin basic protein , which plays an important role in the tight compaction of myelin sheath through association with acidic lipids on the cytosolic side of the membrane bilayer , and the decreased methylation of it could cause conformational change and splitting of the myelin sheet.9 jaiser and winston10 have proposed that methylation of myelin protein is limited by disorders of the methylation cycle , which also contribute to both copper deficiency myelopathy and spinal cord demyelination . second , hhcy is proatherogenic and prothrombotic , so increases the risk of ischemic disease , and might indirectly promote demyelination . further potential mechanisms for demyelinating neuropathy may relate to hhcy , which can promote neuronal hypersensitivity to excitotoxicity and apoptosis.5,11 successful treatment with vitamin b supports such speculation . the mutation was found by chance in our patient and we still do not know how it occurred . in addition to heredity , internal and external environmental factors , such as infection and radiation during growth and development , may also contribute to this mutation . however , the possibility that the mthfr c.677c > t genotype , especially in the presence of folate deficiency , could promote hhcy and affect the spinal cord can not be ignored.12,13 in summary , it is important to pay attention to plasma homocysteine , vitamin b12 , and folate levels in the patient with spinal cord demyelination or other myeleterosis . if these are abnormal , treatment with b vitamins should be started promptly , particularly in those who also have mthfr gene mutation .

hyperhomocysteinemia ( hhcy ) has been recognized as an independent risk factor for atherosclerotic vascular disease . here we report a patient who suffered from spinal cord demyelination combined with hhcy . the patient was admitted to our hospital with a diagnosis of acute myelitis . however , hormone therapy was ineffective . further investigations revealed that he had hhcy and a homozygous mutation of the gene encoding methylenetetrahydrofolate reductase ( mthfr ) c.677c > t , which is a key enzyme involved in homocysteine metabolism . in view of these findings , we treated the patient with b vitamins and his symptoms gradually improved . spinal magnetic resonance imaging performed 3 months after onset showed near recovery of the lesion . to our knowledge , similar reports are rare .

mercury can be found in three forms : elemental mercury , inorganic salts and organic compounds . elemental mercury ( the only metal which is in liquid form at room temperature ) is poorly absorbed ( < 0.01% of the dose ) from gastrointestinal ( gi ) tract after ingestion so systemic toxicity is not expected 1 . on the other hand , inorganic mercury compounds are absorbed in about 715% of doses after ingestion , while approximately 95% of any ingested organic mercury ( methylmercury ) is absorbed from gi tract 1 , 2 . in most cases , however , cases in which elemental mercury has been retained in the appendix have been described 3 , 4 , 5 , 6 . furthermore , cases of appendicitis after mercury ingestion have also been reported so prophylactic appendectomy has been proposed 6 , 7 , 8 . we describe a patient who ingested elemental mercury and was presented with elemental mercury aspiration and retention in the appendix . the patient was treated conservatively without development of appendicitis or clinical signs of mercury poisoning . a 27yearold forge worker was presented at the emergency department because of accidental ingestion of about 30 ml of elemental mercury ( 99.99% elemental mercury ) in the previous day . his vital signs were within normal ranges ( blood pressure : 125/70 mmhg , spo2 : 98% , pulses : 95/min , temperature : 36.8c ) while the clinical examination was unremarkable as well . furthermore , the ecg was normal and the complete blood count and also the blood chemistries were within the laboratory 's normal values . blood mercury levels at the day 2 after ingestion were 15.5 g / l ( normal value < 10 g / l ) while the 24h urine collection between days 2 and 3 after ingestion showed urine mercury levels 5.0 g / l ( normal values < 20 g / l ) . blood mercury levels at the day 5 after ingestion were 8 g / l . chest and abdominal xray showed the presence of highdensity elements in the left lung and in the intestines ( figs 1a , 2a ) . during hospitalization , the patient was given 75 mg of sennosides a+b orally for the first 3 days of hospitalization and succimer 1800 mg / day according the instructions of the local toxicology center . the serial abdominal xrays are shown in figure 1 . in the evening of day 3 after ingestion , the mercury filled the appendix while the patient remained asymptomatic ( fig . however , we decided to treat the patient conservatively and the patient was advised to lie down in trendelenburg position ( 30 ) and left lateral decubitus position every night from day 4 after ingestion . the following abdominal xrays at days 5 and 6 showed the excretion of mercury from appendix ( fig . ( a ) abdominal xray at the time of admission ( day 2 after ingestion ) . ( b ) chest xray after bal and minibal procedures . except the presence of mercury in the intestines we supposed that the patient aspirated an amount of mercury while trying to induce vomiting directly after mercury ingestion . however , because of its specific gravity and freeflowing properties , mercury can overcome the normal swallowing passing to the lungs and can lead to endobronchial hemorrhage and death 9 . despite the fact that mercury was located in small bronchioles in the left lower lobe we tried to remove it with bronchoalveolar lavage ( bal ) and minibal at the day 6 after mercury ingestion . 2b ) showed that our efforts were unsuccessful so the patient was treated with succimer antidote . elemental mercury ingestion is generally benign due to the low absorption levels from the gi tract . in fact , elemental mercury has been used traditionally in hispanic communities as a therapeutic approach in cases of gastrointestinal symptoms known as empacho.4 however , cases of systemic toxicity on the basis of a disrupted gastrointestinal mucosa or due to contamination of the peritoneum postoperatively have been reported 10 , 11 . furthermore , in some clinical conditions in which mercury remains for prolong periods in the gi tract , such as diverticulosis , there is a possibility of conversion of elemental mercury to organic by bacteria leading with that way to systemic toxicosis 9 . gastrointestinal complications after mercury ingestion such as granulomas and abscesses have also been reported 3 . as mentioned above , appendicitis is a possible complication of sequestration of ingested mercury in the appendix 6 , 7 , 8 . although prophylactic appendectomy is proposed as an invasive approach 5 , there are reports that conservative approach can be efficient and safe for the patient 3 , 4 . additionally , we believe that appendectomy of an appendix filled with mercury may not be safe because of the possible effusion of the mercury in the peritoneal cavity and the subsequent systemic toxicity , although similar reports have not published . to the best of our knowledge , it is the first report that an effort to remove aspirated mercury with bal was made . despite the fact that the procedure was not successful , we suggest that further criteria in using bal in such cases should be set . in the literature , there are reports of both mercury ingestion and aspiration in which patients were treated conservatively with chelating agents 12 , 13 , 14 . the placement of the patient with aspirated mercury in a negative pressure room for the protection of the staff from toxic exhaled vapors has also been reported 12 . in a case of mercury aspiration , postmortem findings two decades after aspiration showed extensive fibrosis and granuloma formation which attributed to the local irrigative action of mercury 15 . in our case , the increase in the mercury blood levels was primarily attributed to the inhaled mercury vapors from the aspirated mercury and secondarily to the absorption in the gi tract . gb : participated in management of the patient , conception of the work , writing the first draft of the article , drafting the article and final approval of the version to be published . ssp : participated in drafting the article , critical revision and final approval of the version to be published . pm : participated in drafting the article , critical revision and final approval of the version to be published . af : participated in management of the patient , drafting the article , final approval of the version to be published . ay : participated in management of the patient , drafting the article , final approval of the version to be published .

key clinical messagewe describe a patient with elemental mercury aspiration and retention in the appendix after elemental mercury ingestion . conservative management was proved to be successful for the removal of the mercury from the appendix while we do not suggest bronchoalveolar lavage in cases of small amount elemental mercury aspiration .

moreover , cavernous angiomas in the cerebellopontine angle ( cpa ) are extremely rare lesions with few cases reported in the literature . cavernous angiomas are benign lesions and present with cranial nerve palsies , hemorrhage , or hydrocephalus due to compression of the fourth ventricle . we report the radiologic and pathologic features of a left cerebellopontine lesion in a 50-year - old male , who presented with gait ataxia and lower cranial nerve palsy . a 50-year - old male presented with a four - month history of dysarthria , dysphagia , and unsteady gait . clinical examination revealed left lower cranial nerve palsy and cerebellar signs were positive on the left side ; there was no facial palsy or decreased hearing in the left ear . contrast enhanced computed tomography ( cect ) images of the brain revealed an enhancing hyperdense mass lesion in the left cpa [ figure 1 ] . preoperative contrast enhanced computed tomography of the brain showing a hyperdense lesion in the left cpa ; axial plane ( a and b ) , coronal plane ( c ) , and saggital plane ( d ) the retrosigmoid suboccipital approach was used to operate on the patient in the supine position with head turned to the right side and fixed to the sugita head frame . intraoperatively , there was a lesion of 3 3.5 cm size in the left cpa , which was vascular and bled when attempts were made to separate it from the surrounding structures . it was adherent to the petrous dura and a thin tail of the lesion extended into the internal auditory canal . histopathological examination of the specimen showed a lobular arrangement of dilated and congested vascular spaces lined by a single layer of endothelial cells [ figures 2a and b ] . postoperatively , there were no complications and the patient did not have further neurological deficits . ( a ) photomicrograph of cavernous hemangiomas with closely packed blood vessels of varying wall thickness without intervening central nervous system parenchyma ( h and e , 200 ) , ( b ) photomicrograph showing dilated vascular spaces lined by endothelium and separated by thin fibrous septa ( h and e , 200 ) extra - axial cavernous angiomas are rare and generally occur in the sinuses , meckel 's cavity , parasellar cavernous , and posterior fossa including the cpa and internal auditory meatus . recurrent hemorrhage from the sinusoids of the vascular malformation or from the neocapillary of the cyst wall and the osmotic transport of water into the cyst are thought to induce the growth of the cyst . cavernous angiomas confined only to the internal auditory canal ( iac ) are also reported . common and uncommon lesions of the cpa are listed in table 1 . in an analysis of unusual tumors in the cpa and iac , kohan et al . , found only one case of a cavernous angioma in a series of 426 patients with cpa and iac lesions ( 0.0023% ) . lesions in cerebellopontine angle the clinical and radiological appearance of extra - axial cpa cavernous angioma may mimic that of other cpa tumors ( meningiomas and schwannomas ) . cranial nerve palsies , due to long - term compression , is one mode of presentation . cavernous angiomas have a tendency to bleed and can present with intralesional bleed or as subarachnoid hemorrhage . cavernous angiomas with cystic degeneration they can be misdiagnosed as meningiomas or schwannomas on a ct scan . magnetic resonance imaging ( mri ) the mri features specific for cavernous angiomas include hypointense rim containing hemosiderin deposits on t2-weighted images ( t2wi ) or diffusion weighted imaging ( dwi ) and marked hyperintensity on t2wi . despite their extreme vascularity , cavernous angiomas before surgery can help in avoiding complications as cavernous angiomas have a tendency to bleed . as chronic compression by cavernous angiomas on cranial nerves or cerebellum causes symptoms , surgery is indicated for excision of these lesions . the lesion should be carefully dissected from the cranial nerves . in the present case , the lesion was well circumscribed and could be separated from the surrounding structures , including the lower cranial nerves , and was totally excised . though cavernous angiomas in the cpa are very rare , they should be considered for differential diagnosis when evaluating cpa lesions preoperatively for better intraoperative management and postoperative outcomes .

intracranial extra - axial cavernous angiomas are rare lesions . we report a rare case of extra - axial cavernous angioma in the cerebellopontine angle ( cpa ) in a 50-year - old male , who presented with lower cranial nerve palsy and gait ataxia . computed tomography ( ct ) scan of the brain showed a hyperdense lesion in the left cerebellopontine angle . the lesion was totally excised by the retrosigmoid approach and a pathological examination confirmed the lesion to be a cavernous angioma . following surgery , the lower cranial nerve palsy recovered significantly .

cervical myelomeningoceles ( cmmcs ) and meningoceles account for 15% of all neural tube defects . they have been published as isolated case reports in literature and very little is known about their follow - up . there are very few articles published in the literature , dealing with follow - up of cmmc.[25 ] here , we report a case of cervical meningocele in an asymptomatic patient who was detected to have myelocystocele with tethering on imaging and was surgically treated . a 6-year - old boy presented to us with the history of swelling in the posterior cervical region . past history revealed a cosmetic repair of similar swelling at the age of 3 days . on examination , he had a large midline posterior cervical swelling measuring 6 5 4 cm . the swelling was oval shaped , cystic with a wide sessile base and covered with full - thickness skin bearing scar of the previous surgery [ figure 1 ] . on examination , growth parameters , developmental assessment and neurological findings were normal . magnetic resonance imaging ( mri ) of the cervical spine revealed a fluid - filled cyst in the subcutaneous tissue of posterior neck consistent with meningocele . there was a communication seen between the meningocele and the cord through a spina bifida at sixth cervical vertebral level . the meningocele was associated with a syrinx just in front of the communication [ figure 2 ] . there was evidence of a second cyst within this meningocele consistent with myelocystocele [ figure 3 ] . there was no evidence of hydrocephalus , craniovertebral junction anomalies or dysraphism in other part of the spine . the sac was transected and another sac was found within this , which on exploration was attached to cord . the patient recovered and is doing well . clinical photograph showing cervical myelocystocele mri t1w image showing myelocystocele with syrinx and communication ( arrow ) mri t1w image showing a cyst within a cyst ( arrow head ) , typical of myelocystocele cervical myelomeningocoele is a rare condition , accounting for 15% of all neural tube defects . the mention of cmmc in literature is in terms of case reports or short series . there are very few series dealing with the follow - up of these patients.[25 ] classification and nomenclature of cystic cervical lesions is a controversial topic and various authors have suggested different classifications . defined cmmc as closed spinal dysraphism characterized by skin - covered posterior midline mass , a narrow posterior spina bifida and cerebrospinal fluid - filled cyst . pang et al . proposed a classification system based on the internal structure of the cystic lesions , dividing them into limited dorsal myelochisis containing fibroneurovascular stalk in a dural sac and split cord malformations containing two hemicords in a dural sac . divided these into 1 ) cystic spinal dysraphism of the cervical and upper thoracic region with a stalk of neuroglial or fibrovascular tissue , 2 ) myelocystoceles consisting of a second ependymal - lined cyst herniated inside a meningocele and 3 ) cystic spinal dysraphism of the cervical and upper thoracic region without a stalk or true meningocele . with respect to other classifications it can be called as limited dorsal myelochisis , myelocystocele and cyst herniation within meningocele . neurologically a patient with cmmc is well preserved when compared to those with thoracolumbar or lumbosacral myelomeningocele . the concept of tethering in cmmcs has not received much attention because of their rarity and very slight ascent of cervical cord during growth . however , several case reports have documented tethering in cmmcs which presents as neurological deterioration in the future.[1214 ] the role of imaging in asymptomatic cervical spinal dysraphisms has been emphasized in literature . magnetic resonance imaging is the modality of choice in these lesions , which helps to distinguish the subtypes of cmmc and to identify the associated anomalies of central nervous system like hydrocephalus , split cord malformations , tethering , syringomyelia and chiari malformations . surgical exploration of these lesions should be aimed at preventing infection , achieving cosmesis and most importantly releasing the tethered cord . simple excision of meningocele sac and transection of the communication can provide a cosmetic repair but can not prevent neurological deterioration due to tethering . neurological deterioration in patients undergoing cosmetic repair without detethering has been well documented , which made re - exploration and excision of tethering structures necessary . thus , resection of the lesion without detethering is insufficient and surgery of these lesions should include intradural exploration and detethering , with an aim to prevent neurological deterioration in the future .

dysraphisms involving cervical region are very rare and there are very few series describing their follow - up in literature . here , we report a 6-year - old boy who underwent postnatal cosmetic repair of posterior cervical cystic lesion and presented to us with a large recurrence with syringohydromyelia and tethering . tethered cord should be suspected in the presence of meningocele and intact neurology . treatment protocols of such complicated cervical spinal dysraphisms should include intradural exploration and detethering , with an aim to prevent neurological deterioration in future .

primary adenosquamous carcinoma ( asc ) of the liver is a rare subtype of cholangiocarcinoma . although the clinicopathologic characteristics and biologic behavior of asc of the liver have not yet been fully clarified , asc tends to present more aggressive clinicopathologic features and has a worse prognosis than cholangiocarcinoma . the clinical symptoms are not characteristic . however , fever and abdominal pain induced by cholangitis were reported . recently , we experienced a rare case of primary asc of the liver presented as liver abscess in a patient who had a previous history of liver abscess a 63-year - old man was admitted to korea university ansan hospital complaining of a 4-day history of fever and chill . he had a history of diabetes mellitus for 10 years and cholecystectomy due to gallstone 10 years ago . four years ago , he was admitted to hospital due to liver abscess . at that time hb 11.0 g / dl , platelet 338,000/mm , crp 6.2 mg / dl , esr 101 mm / hr , fasting blood glucose 236 mg / dl , ast 12 u / l , alt 12 u / l , alp 106 u / l , total bilirubin 0.1 mg / dl , total protein 6.9 g / dl and albumin 2.6 g / dl . hbsag and anti - hcv antibody were both negative . afp , cea and ca19 - 9 were 2.55 ng / ml , 20.8 ng / ml and 95.9 u / ml , respectively . abdominal ultrasonagraphy revealed a complex echoic mass in the left lobe of the liver ( figure 1 ) . abdominal computed tomography ( ct ) showed a central hypoattenuated and peripheral rim enhanced mass in the left lobe of the liver without lymph node enlargement ( figure 2 ) . these clinical and radiologic findings suggested a liver abscess . under the impression of liver abscess for evaluation of the complex echoic mass of the liver , ultrasonography - guided percutaneous liver biopsy was performed . the specimen contained an ovoid yellow - gray tumor measuring 655 cm with an eccentric necrosis ( figure 3 ) . however , the specimen had free resection margin and any vascular invasion was not seen . microscopically , the tumor was composed of two components : adenocarcinoma and squamous cell carcinoma components . the adenocarcinoma component was poorly differentiated whereas a few keratin pearls were observed in the squamous cell carcinoma component . thus , a diagnosis of asc in the liver could be made ( figure 4 ) . the patient was transferred to the oncology department and received chemotherapy with 5-fu and cisplatin . the levels of cea and ca 19 - 9 were within near normal range ( 5.3 ng / ml and 24.3 u / ml , respectively ) and the follow - up abdominal ct did not show cancer recurrence . carcinomas containing both adenocarcinoma and squamous cell carcinoma components have been referred to as asc , adeno acanthoma and mucoepidermoid carcinoma . since pianzola and durt described the first case of asc of the liver in 1971 , a few reports have described the clinicopathologic features of asc . most patients have a large mass and extensive invasion into the perihepatic region and lymphatics . in our case therefore , abscess formation might be a very rare complication in asc compared with common manifestations in cholangiocarcinoma . concerning the pathogenesis of asc in the liver , chronic inflammation of the bile duct , usually in association with infection and/or stones , has been proposed as an etiologic factor . such continuous irritations induce metaplastic changes in the biliary epithelium and lead to neoplasia . in our case , persistent inflammation from the choledochoduodenal fistula may have played a role in the development of asc . recently , maeda et al reported that most asc of the liver develops from a squamous change of the cholangiocarcinoma , using the cytokeratins in the liver . in concordance with maeda s proposal , our case showed the transitional area that adenocarcinoma transit to squamous cell carcinoma . the adenocarcinoma component arising from the main biliary duct seemed to differentiate into the squamous cell carcinoma component centrifugally . some report advocated radiation therapy , because squamous carcinoma is more sensitive to radiation therapy than adenocarcinoma . however , the majority of patients died within 1 year after hepatectomy or diagnosis . in our case , up to 8 months after operation , the patient was well and had no evidence of cancer recurrence .

primary adenosquamous carcinoma of the liver is a very rare type of cholangiocarcinoma and is defined as a cancer containing both squamous and adenomatous components in the same lesion . recently , we experienced a primary adenosquamous carcinoma of the liver presented as liver abscess . a 63-year - old man was presented with a 4-day history of fever and chill . the radiologic study showed a 4 cm - sized , central hypoattenuated mass with peripheral rim enhancement in the left lobe of the liver . ultrasonography - guided aspiration and biopsy suggested an adenocarcinoma with abscess in the liver . at laparotomy , the tumor occupied the left lobe of the liver and invaded the right diaphragm . an extended left lobectomy and a partial excision of the involved diaphragm were done . grossly , the tumor was 655 cm in size and had an eccentric necrosis . microscopically , the tumor was composed of adenocarcinoma and squamous cell carcinoma with a transitional area .

a 68-year - old man , who had the history of pterygium excision in his right eye 10 years ago , complained of irritation and congestion in that same eye , which were developed approximately 1 week prior to his visit to our department . slit - lamp examination showed a suspected uveal mass bulging through a focal scleral thinning site in the nasal region of the right eye [ fig . 1 ] . with the slit - lamp magnification topical therapy was started with 0.5% moxifloxacin solution 3 times a day , 0.1% fluorometholone solution 3 times a day , and dexamethasone ointment once a day . one month after the initial visit , the fb removal site was well - recovered . when the removed unknown fb was examined on a light microscopy , it was revealed to be not a parasite but suspected a larva state of an insect or seed of a plant [ fig . 2 ] . with botanist consultation , the fb was confirmed as a seed of the dicotyledones , which is a grouping formerly used for the flowering plants whose seed typically has two embryonic leaves . considering the patient 's history of growing vegetables , the fb might have entered the surface of conjunctiva accidently during the work . however , if it is impossible to distinguish the precise identity of the fb , based on history taking or clinical examination , then the genetic information ( 18s ribosomal deoxyribonucleic acid sequence ) of the organism need to be compared with those of other known organisms . an unknown mass embedded under the nasal bulbar conjunctiva posterior to limbus which was initially misdiagnosed as uveal prolapsed histopathologic features of a removed unknown foreign body . it was revealed to be a seed of the dicotyledones on light microscope examination ( magnification 100 ) there are several reports on ocular fbs involving larvae or wings of insects , beans , and plants . living organisms such as a larva or an insect wing can be diagnosed easily in most cases . however , a prolonged ocular fb combined with inflammation or granulation , like scleral thinning or necrotizing scleritis after pterygium surgery , can be difficult to be diagnosed . therefore , patients who display signs of prolapsed uvea or localized scleritis , possibility of a subconjunctival fb should be considered for differential diagnosis .

there are cases in which the presence of a foreign body ( fb ) is difficult to diagnose based on history taking or clinical examination . we report a case of subconjunctival fb confused with uveal prolapse . a 68-year - old man , who had the history of pterygium excision in his right eye , complained of irritation and congestion in that same eye . he also had the history of growing vegetables in a plastic greenhouse . it seemed to be a suspected uveal mass bulging through a focal scleral thinning site . on the basis of slit - lamp magnification , the lesion was presumed to be a hard and black keratinized mass embedded under the conjunctiva . histopathologically , the removed mass was revealed to be a seed of the dicotyledones . patients who show signs of prolapsed uvea or scleral thinning , possibility of a subconjunctival fb should be considered as differential diagnosis . in addition , a removed unknown fb should be examined histopathologically .

renal cell carcinoma ( rcc ) has a propensity to metastasize along the hematogenous route . about 20% to 25% of patients with rcc have distant metastases at presentation,1 and another 50% develop distant metastases or local recurrence after nephrectomy.2 metastasis can occur at any time , and the most frequent sites include the lungs , bones , liver , and brain . however , rccs can also metastasize to unusual sites , including the pancreas , thyroid , adrenal gland , skeletal muscle , and skin.3 clinical courses vary among patients with rcc who initially present with metastatic disease or who develop tumor dissemination during follow - up . rcc metastases to the stomach , including solitary synchronous gastric metastases from small , localized rccs , are extremely rare.4 we here describe a patient with a solitary synchronous metastatic gastric cancer originating from a small rcc who was treated with endoscopic resection . we also review the clinical characteristics of , therapeutic modalities for , and clinical outcomes of , patients with this disease . he had previously undergone surgery for a duodenal ulcer as well as an open cholecystectomy . , we did not detect abdominal tenderness or a mass , and laboratory tests revealed no abnormal findings . upper endoscopy showed an erosive lesion , approximately 0.6 cm in length , in the anterior wall of the midbody ( fig . a biopsy of the lesion showed tumor cells , consistent with metastatic rcc of the clear cell type . computed tomography ( ct ) showed a mass , approximately 5 cm long , in the right kidney , but there was no evidence of lymph node enlargement or metastatic lesions in the abdomen ( fig . positron emission tomography showed an isometabolic mass ( maximum standardized uptake value [ maxsuv ] , 2.7 ) in the right kidney ( fig . the resected specimen showed a well - defined lobulated mass , 5.23.83.5 cm in size , in the lower pole of the right kidney . the tumor cells had clear cytoplasm and round monotonous nuclei , both typical of clear cell carcinoma ( fig . the tumor was confined to the renal parenchyma , and no lymphovascular or renal vein invasion was apparent . microscopic examination of the resected specimen showed an ill - defined tumor , about 0.6 cm long , with clear resection margins , a finding consistent with metastatic rcc ( fig . upper endoscopy and a ct scan performed 6 months later showed no evidence of local recurrence or additional metastasis . although metastatic gastric cancers are uncommon , those observed are frequently metastases of lung cancer , breast cancer , and malignant melanoma.5 - 8 the clinical symptoms most commonly requiring endoscopy of patients with gastric metastasis include anemia , gastrointestinal bleeding , dyspepsia , and epigastric pain . metastases are most common in the gastric body , and are more likely to be single rather than multiple . grossly , such lesions can resemble submucosal tumors , with or without central depressions , or primary gastric cancers.8 the outcome of patients with metastatic gastric cancer is generally poor , because concomitant metastases to other organs are common.5 metastasis to the stomach from rcc is extremely rare,8 although we identified as many as 36 such patients ( including the patient described here , but excluding those identified on autopsy ) by systematic review of articles in english using a computerized search of the pubmed database ( table 1).1,9 - 37 we found that gastric metastases from rcc were more common in males than in females ( 26 males vs 9 females ) . median age at presentation was 67 years ( range , 48 to 84 years ) . among the most frequent clinical symptoms were upper gastrointestinal bleeding , anemia , and epigastric pain . the most frequent location of metastatic lesions was the gastric body , followed by the fundus and the antrum . the average period from rcc surgery to the presentation of gastric metastasis was approximately 6.5 years ( range , 0 to 24 years ) . twenty - one patients showed disseminated tumor spread to other organs , most frequently to the lungs . two patients presented with metastatic gastric cancer arising from rcc before detection of a renal mass ; one patient was diagnosed with advanced rcc with metastatic gastric cancer as well as lung and bone metastases,32 and the second patient , described here , had a single gastric metastasis arising from a small rcc without evidence of additional metastases . fourteen previously reported patients underwent surgical resection ( total / partial gastrectomy or wedge resection ) ; 3 ( including the patient described here ) received endoscopic resection / excision ; 7 were treated with systemic therapy including interferon , tamoxifen , sunitinib malate , or chemotherapy ; 6 received palliative embolization or endoscopic ablation for control of bleeding ; whereas 7 received no specific therapy . the clinical course in patients with rcc and metastasis to the stomach appears to be unpredictable . generally , the outcome of patients with rcc and gastric metastases is poor.5 some patients die within a few weeks , whereas six ( including ours ) showed no tumor recurrence at a median of 17 months ( range , 6 to 36 months ) after metastatectomy.20 - 22,28,35 of 10 patients with solitary gastric metastases , 6 underwent surgical metastatectomy , 2 underwent endoscopic resection ( snare polypectomy and endoscopic submucosal dissection ) , 1 was treated with sunitinib malate , and 1 received no therapy . five patients showed no evidence of tumor recurrence at a median of 12 months ( range , 6 to 18 months ) after metastatectomy , and one patient lived for 7 years after diagnosis . although the optimal treatment for patients with solitary gastric metastasis arising from rcc remains unclear , patients with potentially surgically resectable primary rcc and a solitary resectable metastatic cancer are candidates for nephrectomy and metastatectomy.38 patients in generally good condition showed prolonged survival after nephrectomy and complete resection of the metastatic lesion.4,38 - 40 similarly , complete metastatectomy may offer survival benefits in patients with solitary gastric metastases arising from rcc.21,28 the optimal treatment modality in a patient with a small , solitary gastric metastasis confined to the mucosa and submucosa may be endoscopic resection .

metastasis to the stomach from renal cell carcinoma ( rcc ) is extremely rare . usually , gastric metastasis seems to be a late event in patients with rcc and is accompanied by disseminated tumor spread to other organs . solitary synchronous gastric metastasis from small , localized rcc has rarely been reported . we report a case of 79-year - old man with synchronous gastric metastasis presenting with a single erosive lesion from pt1 rcc . the patient underwent radical nephrectomy and endoscopic resection for metastatic gastric cancer . the resected specimen showed an ill - defined tumor , approximately 0.6 cm long , with a clear resection margin . the morphologic features of the tumor cells were consistent with those of metastatic rcc of the clear cell type . at 6 months 's follow - up , the patient did not show local recurrence or additional metastasis on upper endoscopy and computed tomography scan .

the outcome of treatment modalities for urethral cancer has not changed during years , suggesting that there have been little advances in the management of this cancer [ 1 - 3 ] . classification of the urethral carcinoma is performed according to stage , location and histology of the tumor . squamous cell carcinomas are the most common pathological type ( 78% ) and 6% are adenocarcinomas . distal tumors ( glanular or penile urethra ) tend to present at a lower stage and have a better prognosis , which is an important prognostic indicator . the extent of tumor at diagnosis is a poor prognostic factor especially when the nodal metastasis is present [ 1,2 , 5 ] . urethral carcinoma has historically been managed by partial or radical penectomy for distal tumors or total penectomy with cystoprostatectomy for proximal tumors . recently , penile preservation surgery was performed in selected carcinomas of the distal male urethra [ 4 , 6- 8 ] . the case was a 44-year - old man presented with a 4 month history of gross initial hematuria and dysuria . endoscopic cold cup biopsies of the nodular mass showed a moderately differentiated squamous cell carcinoma . excision of the involved urethra followed by end- to - end bulbar urethroplasty was done for the patient . frozen - section biopsies of the excised urethra were sent for pathologic examination and the margins were tumor - free . the patient was held in complete bed rest position post - operatively for one week . the final pathology report showed a moderately differentiated squamous cell carcinoma of the urethra with negative tumoral margins . retrograde urethrogram did not show urethral extravasation , so foley catheter was discontinued three weeks after the surgery . external beam radiation was performed for the patient at the dose of 6000 rad , in 33 courses . postoperatively , the patient reported complete urinary continence without irritative or obstructive urinary symptoms and reported normal erections . after intravenous contrast injection , multi - slice abdominal pelvic ct scan was done every 6 months ; and no suspicious area of advanced disease was observed 2 years after the operation . there was not any evidence of urethral or bladder tumor in the 2-year follow - up with bi - annual cystoscopic examination . primary urethral tumor is an uncommon urologic cancer ; however , it presents a treatment dilemma for physicians [ 2 , 5 ] . due to small number of male urethral cancer cases reported over a long period with use of different modalities of treatment and significant physical and psychosexual complications , there is not a consensus on the treatment of these cancers . the most common site of urethral adenocarcinoma is in the bulbomembranous urethra [ 9 - 11 ] . if the extraurethral adenocarcinomas involving the urethra is excluded , then the histogenesis of this tumor would not be clear [ 9 , 12 ] . it has been postulated that these tumors may arise from heterotopic glands in and around the urethral mucosa or metaplastic urethral epithelium or malignant degeneration of persistent glandular elements that are embryonal rests or by neoplastic degeneration of goblet cells found in the urethral epithelium . urethral carcinomas in the proximal urethra present at a higher stage than distal tumors and have poor prognosis due to delay in diagnosis [ 6 , 14 ] . the treatment options for urethral cancers include transurethral resection of the lesion , open resection with end - to - end anastomosis , partial penile amputation and radical penectomy , radiotherapy has been reserved mainly for patients who have failed local control or refused surgical treatment . transurethral ablation or excision of tumor using laser are well documented penile- preserving surgical techniques . the recurrent rate is low for preinvasive lesions ; however , failure rate is very high for invasive tumors [ 16 - 17 ] . alternative organ - sparing treatment , especially in bulbomembranous urethra , is segmental excision of the involved area with end - to - end anastomosis . in our study , frozen - section biopsy during surgery can help the surgeon to resect the tumor completely . yachia and turani reported a case of adenocarcinoma of the bulbar urethra in which the patient refused radical procedures , so partial urethrectomy was performed while sparing the penis . our treatment modality for the patient with proximal urethral carcinoma included segmental urethrectomy accompanied by local radiotherapy . the outcome was satisfactory and the patient did not have any complains about urinary symptoms or erectile dysfunction . total penectomy with cystoprostatectomy is the standard management of proximal urethral carcinoma ; however , penile preservation surgery was preferred in some cases . we treated our patient with excision of the involved urethra followed by end to - end bulbar urethroplasty and external beam radiation and found no evidence of urethral or bladder tumor in the 2-year follow - up .

tumors of the male urethra are rare , comprising < 1% of urologic malignancies . this is a report of a 44-year - old man presented with a 4-month history of gross initial hematuria and dysuria . after examining the patient , we found a firm mass extending 1 cm within the proximal bulbar urethra and a pathologic report of moderately differentiated squamous cell carcinoma . he underwent excision of the involved urethra followed by end - to - end bulbar urethroplasty . external beam radiation was performed at the dose of 6000 rad , in 33 courses . the patient was followed by surveillance protocol and , no evidence of urethral or bladder tumor was found in the 2-year follow - up with bi - annual cystoscopic examination .

the bullous variant of darier s disease ( dd ) is a rare type characterized by histological and clinical overlap with hailey - hailey disease ( hdd ) . the following case report describes two cases of familial dd ; a 51-year - old woman who presented with erythematous plaques , covered by small blisters in axillary and inguinal areas , and the first patient s daughter , who presented with keratotic papules localized on the axillary and inframammary folds . these two cases are original by the predominant flexural distribution , and by a bullous form in the first case , clinically and histologically mimicking hhd . the bullous variant of darier s disease ( dd ) is a rare type with clinical and histological features suggestive of hailey - hailey disease ( hhd).1 it is a rare form , initially described by pels and goodman in 1939.2 there were only few similar reported cases.16 the following case report describes two cases of dd characterized by predominant flexural distribution and a bullous form , in one case , clinically and histologically mimicking hhd . a 51-year - old woman with no past medical history presented with pruritic lesions involving the flexural areas . she reported that her father and her daughter had similar skin lesions on skin folds . cutaneous examination revealed erythematous plaques , with macerated and eroded surface , partially covered by small blisters in axillary and inguinal areas , with parallel rhagades on the groins and the internal aspects of the thighs ( figure 1 ) . histopathological examination of a biopsy specimen revealed suprabasal clefting with acantholysis , and a few foci resembling a dilapidated brick wall. ? rare dyskeratotic cells were also observed with corps ronds ? in the stratum spinosum ( figure 2 ) . the patient was treated with topical antimicrobials , which lead to complete healing of the erosions , leaving no macroscopic changes , other than a temporary pigmentation . erythematous plaques , with parallel rhagades on the groins and the internal aspects of the thighs . the second report concerned the first patient s daughter , a 25-year - old with no past medical history , who presented with itching papules of skin folds . cutaneous examination revealed greasy brown - colored and keratotic papules of 13 mm in diameter , occupying the axillary and infra - mammary folds ( figure 3 ) . these lesions had begun 5 years ago and remained unchanged since that time , except for a slight exacerbation by heat , sweating , or friction . the histopathological examination of a cutaneous biopsy of one of the keratotic lesions revealed suprabasal clefts of the epidermis with acantholysis . there were also hyperkeratosis and focal dyskeratosis ( premature cornification with detached corps ronds ) ( figure 4a , figure 4b ) . keratotic brown - colored papules in the infra - mammary folds suprabasal clefts with acantholysis , hyperkeratosis and focal dyskeratosis . in view of the clinical and pathological findings in the mother and daughter , dd and hhd are both autosomal dominant calcium adenosine triphosphate synthase ( atpase ) disorders . even though they share some clinical and histopathological features , they correspond to two genetically distinct entities , and the presence of the two disorders in the same family seems to be unlikely . the authors believe that the previous reports of patients described as having both dd and hhd were probably cases of dd with flexural involvement.7,8 dd often begins in childhood before the age of twenty , and lesions develop slowly and remain static with no permanent clearance.9 in hhd , lesions , however , develop rapidly and may disappear entirely , leaving no residual lesions , except for temporary pigmentation.10 histologically , acantholytic suprabasal clefts can be seen in both disorders , but in hhd , acantholysis is usually incomplete , causing the well known dilapidated brick wall ? moreover , predominant dyskeratotic keratinocytes in dd can be a distinctive histological feature between these two genodermatoses.11 the bullous variant of dd is characterized by histological overlap with hhd , given the presence of extended acantholysis with foci of dilapidated brick wall ? appearance and few dyskeratotic cells , as shown in our first presented patient , who also has a striking clinical resemblance with hhd . our first patient had a late onset - flexural involvement with a relapsing course of the vesicles , leading to eroded surface , covered by parallel rhagades . all of these signs together with the presence of foci of dilapidated brick wall ? and the lack of profound dyskeratosis were in favor of hhd . however , this diagnosis was corrected by examining the daughter who presented with typical clinical and histological features of dd . the correlation of clinical and histopathological features in the mother and the daughter led to the diagnosis of bullous dd in the mother . the clinical and histopathological findings suggestive of hhd in cases of bullous dd have been previously reported.7,8 the current therapeutic alternatives for dd are based on keratolytic drugs , topical antimicrobials for infections , topical retinoids to reduce hyperkeratosis , and oral retinoids for severe cases , with variable clinical responses.12 in bullous dd , management should be different from that of the classic form and should take into account the risk of aggravation of macerated lesions by classic topical and systemic treatments . our first patient with bullous dd demonstrated a complete clearance of erosions under topical antimicrobials . the diagnosis of vesiculo - bullous dd is difficult in view of the histological and clinical overlap with hhd . subtle clinical and histological analysis may aid correct diagnosis and in doubt , molecular diagnosis is mandatory .

backgroundthe bullous variant of darier s disease ( dd ) is a rare type characterized by histological and clinical overlap with hailey - hailey disease ( hdd).case reportsthe following case report describes two cases of familial dd ; a 51-year - old woman who presented with erythematous plaques , covered by small blisters in axillary and inguinal areas , and the first patient s daughter , who presented with keratotic papules localized on the axillary and inframammary folds.conclusionthese two cases are original by the predominant flexural distribution , and by a bullous form in the first case , clinically and histologically mimicking hhd .

a 76-year - old woman was admitted to our hospital because of involuntary movement of the left distal extremities . two months earlier , she reported a peculiar feeling and occasional jerky and twisting movements of the left foot and hand when attempting to put on shoes or catch objects . the patient had a past history of hypertension and endometrial cancer that was treated . on initial examination , we found dystonic movement of the left hand and foot mainly occurring with action . on neurological examination , motor and sensation functions were preserved , but there were increased deep tendon reflexes , a positive barbinski s reflex and ankle clonus in the left extremities . the gait was small based and dystonia of the left foot was observed on initiation of gait . the brain mri revealed hyperintense lesions with irregular and nodular meningeal enhancement in the right fronto - parietal lobe , suggesting leptomeningeal metastasis ( figure 1 ) . the cerebrospinal fluid ( csf ) contained 54 white blood cells per ul ( 47% lymphocyte dominant ) ; the concentrations of protein and glucose were 79 mg / dl and 65 mg / dl , respectively . the search for a hidden primary malignancy included a whole body f - fluorodeoxyglucose positton emission tomography ( f - fdg pet ) . torso - pet scanning showed hypermetabolic signals in the gastric antrum and left upper lung , and the brain - pet scan showed hypermetabolic signals in the right fronto - parietal lobe , consistent with the brain mri findings ( figure 2 ) . endoscopic gastroduodenoscopy revealed a very large ulcerated infiltrating mass at the gastric antrum , and the biopsy showed an adenocarcinoma . two months later , after changing medication with clonazepam , the dystonia was slightly improved . basal ganglia dysfunction , with an imbalance between normal modulation of the direct and indirect pathways , is generally considered the basis for cortical disinhibition and abnormal motor output in the pathophysiology of dystonia.7 consequently , secondary dystonia is usually produced by lesions in the contralateral caudate nucleus , lentiform nucleus or thalamus , or in a combination of these structures.1 these lesions are thought to cause the abnormal functioning of the cortico - striato - pallido - thalamo - cortical loop leading to enhanced excitation of the premotor cortical area.8 however , recent studies have emphasized the role of abnormal cortical function , especially in the somatosensory system , for the development of dystonia.9 chuang and colleagues10 described that cortical lesions could be seen in up to 32% of patients with acquired hemidystonia . we identified 14 cases of symptomatic dystonia due to cortical lesions in the literature ( table 1).2,1118 there is often a latency between cerebral injury and the onset of dystonia , and such delayed development of dystonia might be related to aberrant reorganization of the cortico - striato - pallido - thalamo - cortical loop after a static lesion.3,10 though most cases of secondary dystonia due to stroke or trauma develop after a long latency from the initial brain lesion , a few cases of secondary dystonia due to neoplasm have presented as the initial manifestation of cortical lesion , similar to our case.14 functional neuroimaging showed overactivation of the primary sensorimotor cortex and underactivation of the prefrontal motor areas with focal hand dystonia.19 it is likely that hypermetabolism in the primary sensorimotor cortex , in our case , could have played a role in the genesis of dystonia . one prior report suggested that provoking the cerebral cortex can evoke dystonia in the extremities.19 clinical signs and symptoms of lm are usually attributable to the obstruction of normal csf flow , local tumor infiltration into the brain or spinal cord , alterations in the metabolism of underlying nervous tissues , or a combination of these processes . the common presenting signs of lm are headache , changes in mental status , focal neurological deficits , and seizures.5 there have been few reports on movement disorders such as dystonia secondary to lm . our patient presented with left hand and foot dystonia , as the initial clinical manifestation of leptomeningeal metastasis associated with gastric adenocarcinoma .

a 76-year - old woman gradually developed action dystonia of the left hand and foot . leptomeningeal metastasis of the right fronto - parietal area associated with gastric adenocarcinoma was found on the brain magnetic resonance imaging ( mri ) and positron emission tomography ( pet ) studies . we discuss the mechanisms involved in the development of secondary hemidystonia and review dystonia associated with cortical lesions .

correlation between mutations in cancer alleles and drug response is a key point to identify drugs or drug combinations that match the genetic profile of individual tumors . the identification of genetic determinants of drug response , by routine diagnostic approaches , is thus a clear priority of translational oncology . in non - small cell lung cancer ( nsclc ) genetic lesions affecting the epidermal growth factor receptor ( egfr ) pathway act as predictive markers of response to small inhibitors1 . inappropriate egfr overactivation is mainly consequent to somatic mutations occurring in those sequences which encode for the receptor tyrosine kinase ( tk ) domain2 . egfr amplification ( detected by fish in 20 - 40% of nsclcs , according to different studies ) seems to add a gain in response rates to gefitinb and erlotinib 3,4,5 . on the other hand , mutations affecting the egfr downstream transducers and mainly the kras oncogene have emerged as highly specific negative predictors of response to single anti - egfr agents 6 . here we describe the case of a 71 years old , currently smoker , caucasian man who came under our observation due to the occasional detection by standard chest x ray of a right pulmonary mass . during hospitalization , the patient underwent a total body ct scan that showed the presence of two solid parenchymal lesions : the first affecting the upper right lobe and a second nodule at the lower left lobe ; no mediastinal and extrathoracic masses were detected . the patient was then addressed to fluoroscopic ct - guided fine needle aspiration ( fna ) of the two lesions . in both cases the cytological analysis ( fig . 1 ) was consistent with adenocarcinoma , displaying a ttf-1 and p63 positive immunohistochemical profile . a diagnosis of bilateral synchronous nsclc ( adenocarcinoma ) was thus formulated . in order to evaluate the egfr / kras mutational profile , tumor genomic dna from formalin - fixed paraffin - embedded ( ff - pe ) corresponding samples was extracted and sequenced . interestingly two different egfr profiles were unveiled . indeed we found that the right lesion carried the egfr l858r somatic change , while no egfr mutations were detected by sequencing genomic dna extracted from the left nodule . absence of egfr amplification was documented by fish analysis on both lesions . besides the two masses harboured wild type kras sequences . on this evidence , the patient underwent a first line platinum - based chemo ( platinum - pemetrexed ) but a slight disease progression was documented subsequent to 4 cycles of treatment . after 6 months of treatment the egfr mutated lesion displayed a volume reduction of more than 50% ; the controlateral nodule showed a less but still significant ( 15 % ) reduction in both diameter and density ( fig.2 ) . overall ct scans were performed as a control after therapies and objective response was evaluated according to recist criteria7 . the above discussed case seems worth to be reported since it allows relevant some clinical considerations . interestingly , the lesion that was assessed as egfr wild type - by sequencing fna cytology specimen- actually displayed a slight response to the egfr inhibitor . this behavior could be coherent with the fact that some responses to egfr tkis have been described also in egfr wild type tumors8 . in addition , it could be hypothesized the presence of an egfr - mutated subclone . from this perspective , it should be note that direct sequencing might not be sensitive enough to detect low frequency of mutated egfr and kras even though it remains at the present among the most accurate approaches to mutational analysis for clinical running . a second key point is represented by the fact that routine histochemistry classified these tumors as two independent synchronous lung cancers . however based on their mutational profile , a potential chronology and a metastatic progression could be hypothesized . indeed preclinical data suggest that egfr mutations occur as an early event during nsclc onset 9 . besides it is known that the molecular status of egfr / kras may change during the distant spreading of nsclc 12,10 and a discordance between egfr mutations in primary tumors and their corresponding lymphatic and distant metastases has been already reported 11,12,13,14 . the heterogeneity found in this case has relevant clinical implications since it is reflected in disease staging and therapy : if the putative second lesion was a metastasis , that meant a stage iv - not resectable - disease , whereas if it was a synchronous primary tumor , it was potentially resectable15 . although an exhaustive immunoistochemical analysis could be helpful to clarify the association between the two lesions , this case undoubtedly demonstrates that regardless histochemistry , therapy should be tailored to the molecular profile and that a molecular profile can be linked to a clinical phenotype . a third issue is related to the opportunity to routinely perform egfr / kras mutational profile in front of adks aroused in males and active smokers . from this perspective , this report provides clear evidence for a need to a complete egfr / kras molecular profiling for all lung adks . finally this clinical report underlines the role of molecular diagnosis on cytological tumor sample as a routinely safe and accurate approach . coherently recent reports confirm the feasibility of these techniques in samples derived from us - guided fine needle biopsies 16,17 . however it should be noted that although mutational analysis on small sized samples may underestimate molecular heterogeneity that usually characterizes lung cancer and that , consequently , the fraction of the mutated egfr might be kept in consideration to define to what extent a lesion is mutated and consequently objectively sensitive to anti egfr therapy .

we discuss the case of synchronous bilateral lung cancers which feature the same histological phenotype and a different egfr mutational profile . both histological and molecular characterizations were performed on specimens derived thorough ct - guided fine needle aspiration . a first - line chemotherapy was unsuccessful . subsequent objective response to the egfr inhibitor erlotinib was clearly coherent with the sequencing data and the mutated nodule was effectively reduced ( > 50% ) after therapy , while the lesion assessed as egfr wild type featured a slight response . this report has two relevant implications . it points out that in case of multiple malignant lesions at time of diagnosis , molecular profiling should be as extensive as possible and it might contribute to clarify the association between the lesions found . besides the molecular analysis on cytology specimens could identify an accurate and safe diagnostic approach for clinical use .

acinic cell carcinomas ( acc ) are usually low grade , uncommon neoplasms constituting 2.54% of parotid gland tumors . papillary cystic variant ( pcv ) of acc is a rare tumor , the diagnosis of which is based on histopathological examination . it is composed of tumor with papillary and cystic growth patterns , with varying proportions of one or more cell types . it has mostly been reported in younger patients ( 16 - 40 years ) when compared to the classic type that characteristically presents in the fifth decade of life . although an uncommon tumor , it is important to recognize this variant as it has proved to be universally fatal within 10 years . we hereby report an unusual case of acc - pcv in a 20-year - old male which was considered a benign parotid mass both cytologically and radiologically . a 20-year - old male presented to the surgical clinic with a complaint of swelling on the left angle of mandible below the ear lobule since 4 months . it was 3.5 cm 2 cm in size , firm to hard with limited mobility and nontender . laboratory investigations revealed hemoglobin 16.2 gm% , total leucocyte count 6,800 cells/l , differential leukocyte count neutrophils 74% , lymphocytes 18% , eosinophils 5% , monocytes 3% , platelet count - 1.15 lac/l and blood sugar 120 mg / dl . contrast - enhanced computed tomography of neck showed a soft tissue nodular lesion isodense to the parotid ( attenuation 40 hu ) of size 2.3 cm 1.6 cm 1.3 cm with mildly enhancing peripheral walls in the left superficial parotid gland . few spiculated nodules were also seen in the right lung apart from patches of numerous centriacinar nodules . both lobes of the thyroid were normal in size , shape , attenuation and enhancement . grossly , the specimen measured approximately 6.5 cm 4.5 cm 3.5 cm with attached flap of skin measuring 3.5 cm 1.5 cm . cut surface showed a large cyst measuring 2.8 cm 1.5 cm filled with dark brown material [ figure 1 ] . histopathological examination showed a large well - circumscribed cystic space lined by numerous papillary projections few of them showing thin vascular cores [ figure 2 ] . many hemosiderin - laden macrophages and areas of hemorrhage are also seen [ figure 3a ] . these papillae were mostly lined by hobnail cells with round , vesicular nuclei , central nucleoli and eosinophilic to vacuolated cytoplasm [ figure 3b ] . gross specimen cut surface of which shows a large cyst measuring 2.8 cm 1.5 cm filled with dark brown material numerous papillae lying in cystic cavity ( black arrow ) , fibro collagenous tissue and adjacent normal salivary gland tissue ( white arrow ) ( a ) papillary growth pattern along with hemosiderin within the papillae . acinic cell carcinoma is an uncommon salivary gland tumor , making up 1% of all salivary gland neoplasms . they most often arise in the parotid gland , but may occasionally involve the submandibular , minor salivary or seromucinous glands . it is the least aggressive of salivary gland cancers with low malignant potential , but several recurrences and metastasis have been reported . few high - grade variants of acc are known such as papillocystic carcinoma or carcinomas with undifferentiated cells in the medullary pattern . lesions of the tail of the parotid gland are difficult to assess clinically and provide a diagnostic dilemma on imaging . most benign parotid tumors present as slow - growing , painless masses often in the tail of the parotid gland . defines the tail of the parotid gland as inferior 2 cm of the superficial lobe of the gland . in a study by hamilton on 117 parotid tail masses , seventeen types of parotid tail masses were identified out of which benign lesions were the most common . also as it was a slow - growing painless mass present in the parotid tail , clinically it was thought to be a benign parotid mass which was also supported by cytological examination . accurate localization of these lesions on imaging is essential to assist the clinical diagnosis and to prevent inadequate / incomplete excision and complications , especially damage to the facial nerve . the diagnosis of pcv of acc usually poses a challenge because of the cytoarchitecture that is different from classic type . cystic fluid in the case of acc - pcv dilutes the overall cellularity leading to a mistaken diagnosis of benign lesion as was seen in the present case . histopathologically , acc shows a myriad of architectural patterns : solid , solid - lobular , acinar - microcystic , papillary cystic , tubuloductal , follicular and macrocystic , and dedifferentiated . the solid and microcystic are the most common subtypes and the pcv accounts for one - fourth of acc . varying proportions of one or more of five cell types are seen including hobnail , acinar , intercalated , vacuolated , nonspecific glandular and clear cells . this is due to bulging of the apical portions of lumen lining cells into the lumen presumably after release of secretions . the clinical picture is not specific , and diagnosis is based on the histopathologic examination . the mean age of occurrence is in the fifth decade , but the pcv is reported to occur in younger patients compared to the classic type . the most significant differential diagnosis of acc - pcv is papillary carcinoma of the thyroid . thyroid ultrasonography , hormonal assays , and immunohistochemistry for thyroglobulin are helpful in differentiating these two lesions . timely diagnosis and treatment of acc - pcv is essential as it has been found to be universally fatal within 10 years . although most parotid masses are benign , removal is required for histopathologic confirmation because of the clinical and radiologic overlap . to conclude , the present case emphasizes the importance of histopathological examination in parotid masses as the imaging features as well as cytology lack the necessary specificity to differentiate benign from malignant masses , especially in a case of papillary cystic acc . it also highlights the need to consider malignant lesion in the differential diagnosis of parotid tail tumor .

acinic cell carcinoma ( acc ) is an uncommon low - grade tumor of the salivary glands that constitutes 2.54% of parotid gland tumors . papillary cystic variant ( pcv ) of acc is even rarer and can be diagnosed on histopathological examination only . it is important to diagnose this variant as it carries a poor prognosis when compared with other variants of acc and is known to be universally fatal in 10 years . the present case describes acc - pcv in a 20-year - old male , which presented as a slow growing parotid tail tumor and was misdiagnosed as a benign lesion both cytologically and radiologically . this case emphasizes the importance of histopathological examination in parotid masses as well as the need to consider malignant lesion in the differential diagnosis of a parotid tail tumor .

a clear fundus image of the surgical field is one of the most important factors influencing the surgical outcome . recently , use of a wide - angle viewing system has become popular , because it can easily provide a panoramic view of the surgical field . two types of wide - angle viewing system exist , with both contact and noncontact types available.13 while the contact type provides greater optical resolution and a wider field of view , the noncontact type is more popular because of the stability of the image against the tilt of the eyeball and the ease of manipulation . however , drying of the cornea during surgery , which causes poor visibility of the fundus , is one of the more serious problems when using the noncontact type . we have developed a new type of antidrying corneal contact lens that can be used with a noncontact wide - angle viewing system . we developed an extremely thin corneal contact lens that has a 0.2 mm thickness at the center and a clear meniscus made of polymethyl methacrylate . the lens has a loose curve that measures 8.45 mm for the front curve radius and 8.40 mm for the back curve radius , along with a large outer diameter that measures 13.0 mm ( figures 1 and 2 ) . we performed lensectomy , intraocular lens implantation , and 23-gauge pars plana vitrectomy with a wide - angle viewing system ( resight , carl zeiss meditec ag , jena , germany ) . to protect the cornea from drying , viscoelastic material was applied to the corneal surface , with the lens then positioned on top without any fixation ring required to hold it in place ( figure 3 ) . we developed an extremely thin corneal contact lens that has a 0.2 mm thickness at the center and a clear meniscus made of polymethyl methacrylate . the lens has a loose curve that measures 8.45 mm for the front curve radius and 8.40 mm for the back curve radius , along with a large outer diameter that measures 13.0 mm ( figures 1 and 2 ) . we performed lensectomy , intraocular lens implantation , and 23-gauge pars plana vitrectomy with a wide - angle viewing system ( resight , carl zeiss meditec ag , jena , germany ) . to protect the cornea from drying , viscoelastic material was applied to the corneal surface , with the lens then positioned on top without any fixation ring required to hold it in place ( figure 3 ) . this new type of corneal contact lens provided clear images of the surgical field without any drying of the cornea . the fundus area that was viewed with a combination of the contact lens and a wide - angle viewing system tended to be wider than the area available when using the wide - angle viewing system alone ( figure 4 ) . during operations using the noncontact type of wide - angle viewing system , continuous hydration of the cornea by an assistant or application of a viscoelastic material to the corneal surface is necessary if a clear view is to be maintained . however , the irregular surface of the viscoelastic material can lead to a poor quality of vision for the surgical field . recently , the usefulness of several types of corneal contact lenses that can overcome drying of the cornea has been demonstrated . ohji et al4 and ohno5 reported that use of a magnifying vitrectomy contact lens was advantageous , because it provided a wider fundus view in combination with the wide - angle viewing system . in their system , a magnifying contact lens is held on the cornea using a lens holder or a fixating ring . kamei et al6 compared a commercially available rigid gas - permeable contact lens ( 3 diopters ) with a system that used a balanced salt solution , a viscoelastic agent , a vitrectomy contact lens , and a soft contact lens , and found the rigid gas - permeable contact lens to be ideal for use during vitrectomy when performed with a wide - angle viewing system . the rigid gas - permeable contact lens is designed to be movable on the cornea in order to prevent hypoxic corneal damage in daily use . however , the lens might be unstable on the cornea during surgery , which seems disadvantageous when it is used in vitrectomy . ohno et al7 developed a new antidrying contact lens made of quartz with a diameter of 11.2 mm and a thickness of 1.0 mm . one of the disadvantages of using a quartz lens is its thickness , and because of its weight , this small diameter lens tends to show poor centering on the cornea . to overcome this problem , in contrast , the outer diameter of our lens is much larger , which leads to stabilization and prevents displacement of the lens without having to use a contact lens fixation ring . in addition , the very thin and lightweight characteristics of polymethyl methacrylate may also help contribute to the overall stabilization . because this prototype lens was designed to have a refractive power of zero on the cornea , no disadvantageous changes are seen in the panoramic view . thus , the design of this lens makes it possible to achieve a good quality intraoperative view , not only by preventing the cornea from becoming too dry , but also by providing a smooth surface over the entire cornea . therefore , this lens might be suitable for cases having a rough cornea following trauma or laser - assisted in situ keratomileusis , or during prolonged surgical procedures . when calculating the area of the fundus to be visualized using a ray tracing method , our contact lens with the wide - angle viewing system ( + 128 diopters ) provided a 109.9 and 133.7 degree fundus view , while the wide - angle viewing system alone provided a 108.1 and 125.9 degree fundus view in pseudophakic ( + 20 diopters , 6 mm in diameter ) and aphakic eyes , respectively ( table 1 ) . during the simulation , use of this lens led to a slightly wider field of intraoperative view , which could be another advantageous aspect when using our contact lens . in summary , this promising new lens can be used as an antidrying device during vitreous surgery with a noncontact wide - angle viewing system .

drying of the cornea during surgery , which causes poor visibility of the fundus , is one of the more serious problems when using a noncontact type wide - angle viewing system . we developed an antidrying corneal contact lens for use with this type of viewing system . the lens has a very thin meniscus made of polymethyl methacrylate with a large outer diameter . following application of the viscoelastic material , the lens can be put on the cornea without using a contact lens fixation ring . the larger diameter and lightweight characteristics of our lens contributed to its overall stability . the smooth surface of this prototype lens was able to achieve an intraoperative view of good quality , not only by preventing the cornea from drying , but also by providing a smooth surface over the entire cornea .

cutis laxa ( cl ) is a rare connective tissue disorder caused by defects in the elastic fiber network that can affect multiple tissues , predominantly the skin . redundant skin is often most noticeable on the neck , hands , and groin , but can also be seen on the face , creating a premature aging appearance . we report a case of a female child with generalized cl , developmental retardation , and abnormal thyroid function . congenital cl is a rare , hereditary connective tissue disorder resulting from the abnormality of elastic fibers . the clinical presentation and mode of inheritance show considerable heterogeneity . it can be subdivided into congenital and acquired forms . in congenital cl , three modes of inheritance have been described ; more and more gene mutations associated with elastic fibers have been reported . a number of metabolic disorders have also been found to be associated with inherited cl , such as menkes disease , congenital disorders of glycosylation , and others [ 4 , 5 ] . in humans , thyroid dysfunction is associated with alterations in skin architecture and homeostasis . in hyperthyroid individuals , the skin often presents with some of the following symptoms : softness , perspiration , heat , itching , generalized pruritus , chronic urticaria , vitiligo , and diffuse skin pigmentation . the epidermis is hyperkeratotic , alopecia may develop , and there is diffuse myxedema . a newborn with an autosomal recessive form of congenital cl has been reported in association with congenital hypothyroidism owing to isolated thyrotropin deficiency . this report was the first and the only one to show that cl was related with the thyroid . however , our patient was different from this case , with normal thyrotropin ( tsh ) , higher free triiodothyronine 3 ( ft3 ) and free thyroxine ( ft4 ) . a case similar to our patient has not been reported previously . we do not know whether these findings point to a relation between thyroid hormone metabolism and congenital cl . a 7-year - old female patient was admitted to our hospital due to developmental delay , growth retardation , and loose facial skin since birth . at 2 months of age , she was found to have a hoarse voice . at the age of 3 years however , she had no other symptoms , had normal intelligence , and was able to perform normal activities . her father had a similar skin disease , but his height was normal with about 5.6 feet ( 1.7 m ) . physical examination revealed that the patient 's height was 100 cm , weight 16 kg , and she had loose inelastic skin hanging in folds . loose and sagging skin folded over the neck , arm , and trunk , which gave her a prematurely senile appearance with her skin displaying obvious relaxation and elasticity ( fig . laboratory values revealed normal values for complete blood count , erythrocyte sedimentation rate , routine urine analysis , blood biochemistry , hepatic enzymes , and serum electrophoresis . skin biopsy specimens taken from the involved area were stained with hematoxylin - eosin and weigert stains . histological findings from the skin biopsy revealed a dermal collagen fiber glass - like swelling and perivascular lymphocyte infiltration . elastic fiber staining showed that the elastic fibers present were fragmented , arranged in disorder , and had a granular appearance ( fig . by contrast , the collagen structure , content , and distribution were normal ( fig . because of the growth delay , the thyroid functions were tested and revealed ft3 7.71 mmol / l ( normal range : 3.86.0 ) , ft4 24.80 mmol / l ( normal range : 7.914.4 ) , tsh 2.29 miu / l ( normal range : 0.345.0 miu / l ) , thyroid microsomal antibody 4.16% ( normal range : < 15 ) , and thyroglobulin antibody 9.23% ( normal range : < 30 ) . growth hormones and adrenocorticotropic hormones were all within normal ranges , as were skull radiographs . based on these clinical manifestations and laboratory examinations , the most likely diagnosis in our patient was cl syndrome with abnormal thyroid hormone levels . cl is characterized by abnormal elastic fibers resulting in loose , redundant , hypoelastic skin . typically , the skin in cl can easily be pulled away from underlying tissue and only slowly returns to its original position . there are no accurate incidence data available , and there is no known racial or ethnic predilection for cl . cl may be inherited as a dominant , recessive , an x - linked recessive disease or it may be acquired . the heritable forms of cl predominantly begin at birth , but it may be delayed until puberty or later with extracutaneous manifestations including pulmonary emphysema , umbilical and inguinal hernias , and gastrointestinal and vesico - urinary tract diverticuli . as the patient 's father had similar clinical manifestations and the generalized laxity of the skin and hernias were associated with cl , we concluded that his daughter had autosomal dominant cl ( adcl ) . systemic manifestations can range from mild to severe , including cardiac and pulmonary complications such as bronchiectasis and emphysema . many patients live normal life spans , although some of them with adcl experience serious systemic problems including aortic aneurysms , severe congenital lung disease , and pulmonary artery disease . the clinical presentation and the mode of inheritance show considerable heterogeneity . in the present case , the patient 's associated features include developmental delay , and growth retardation without severe systemic complications suggests autosomal dominant inheritance . severe short stature and dwarfism are common in autosomal recessive cl ii , de barsy syndrome , x - linked recessive cl , gerodermia osteodysplastica , and costello syndrome . a variety of cl syndromes have also been described , some of them exhibiting mental retardation , but only few reports suggested an association with the abnormal thyroid function . in the present case , it is unclear if short stature and dwarfism are part of the clinical presentation of cl or instead caused by the abnormal thyroid function . there is no effective drug treatment for anetoderma , but plastic surgery can be considered . after 3 months , her parents found no marked changes of the loose skin and height , which led to the discontinuation of the drug . now , her height is 115 cm , which is significantly lower than that of normal 10-year - olds . based on this case , cl is likely to be a complex disease accompanied by multiple anomalies , and there may be a dermatological clue to autoimmunity . we suggest that the finding of wrinkled skin or cl in a child with developmental delay should evoke a detailed endocrine and metabolic evaluation .

we describe a case of generalized cutis laxa ( cl ) in a 7-year - old female child . at 2 months of age , she was found to have a hoarse voice , and at 3 years , she was much smaller than her peers . her aging face and short stature caught our attention , and the treatment of the patient was accepted by our hospital . she underwent a thorough examination . x - ray of the wrist bone showed a markedly delayed bone age , and thyroid function tests revealed significantly elevated free triiodothyronine 3 and free thyroxine 4 levels , but thyrotropin was within the normal range . thyroid dysfunction and cl can be associated with lagged growth and development . whether her abnormal development was due to thyroid dysfunction or cl could not be ascertained . cl is possibly more complex than it has been supposed so far , and is therefore worth to be further studied .

the earlier reports are for the most part in accordance with more recent ones , despite being diagnostically less specific in psychiatric and hematological terms . these symptoms seem to fall into several clinically separate categories : slow cerebration ; confusion ; memory changes ; delirium , with or without hallucinations and/or delusions ; depression ; acute psychotic states ; and ( more rarely ) reversible manic and schizophreniform states . apart from these conditions , some cases of dementia , violent behavior and fatigue have also been documented to be caused by vitamin b12 deficiency . the psychiatric symptoms can develop long before anemia or spinal cord symptoms.[46 ] the most common neurological manifestations are sensory impairment in the form of paresthesias and decreased vibratory and touch sensations and ataxia . in various studies , the neuropsychiatric abnormalities were present in up to 28% of patients in the absence of any change in the hematocrit values or increase in the red cell mean corpuscular volume ( mcv ) . however , we could not come across a presentation as predominant negative symptoms associated with vitamin b12 deficiency in literature . a case of vitamin b12 deficiency is reported which presented with negative symptoms followed by neurological symptoms . mr . a , a 27-year - old single male , premorbidlywell adjusted , life - long vegetarian , without significant past or family history of psychiatric illness , presented to our out patient department ( opd ) in october 2005 with symptoms of 1 year duration . he started with complaints of forgetfulness for several weeks , followed by social withdrawal , paucity of speech , decreased interest in routine and pleasurable activities and apathy . his selfcare deteriorated markedly , appetite decreased grossly and he lost 57 kg weight in 45 months . members denied history of sadness of mood , ideas of worthlessness , hopelessness , guilt , nihilism , catastrophe , suicidal ideation , easy fatiguability ; loss of executive functions , agnosia , misrecognition , urinary or fecal incontinence ; manic and psychotic symptoms . olanzapine 5 mg / d . over the next 34 weeks , he perceived minimal improvement , but developed bilateral weakness of lower limbs resulting in limping and walking with support . neurological examination revealed bilateral loss of proprioception , vibration , light touch and pain sensation in the lower extremities . there was reduced motor strength and the reflexes were symmetrically diminished in the lower extremities with bilateral positive babinski sign . complete blood counts were essentially normal ; however , normochromic anisopoikilocytosis and macrocytes were seen in peripheral blood smear . serum vitamin b12 level was found to be markedly diminished less than 50 pg / ml ( normal : 200950 test results for intrinsic factor and parietal cell antibodies were found to be negative in his serum and the patient did not give consent for gastric biopsy . mr . a was diagnosed with subacute combined degeneration of spinal cord due to vitamin b12 deficiency . olanzapine was stopped and he was continued on intramuscular vitamin b12 injections . his psychological and neurological symptoms improved completely over the next 34 months and he is maintaining well for the last 2 years . negative symptoms of schizophrenia are characterized by restricted affect , diminished emotional range , poverty of speech , curbing of interests , diminished sense of purpose and diminished social drive . the primary negative symptoms usually show poor response to drugs and less long term recovery of functioning . the etiology of this patient 's vitamin b12 deficiency was not very clear , but when the patient was seen in his most prominent symptoms ( i.e. negative symptoms ) , anemia and spinal cord symptoms were not prominent . several factors in this case suggested an organic cause for this patient 's psychiatric symptoms , such as no family or past history of psychiatric illness , well adjusted personality without any history of substance abuse , predominant negative symptoms followed by neurological symptoms . once the organic nature of his psychiatric illness was realized , the patient was investigated accordingly and was given vitamin b12 supplementation . within 23 months of starting vitamin b12 supplementation , his psychiatric symptoms completely resolved and neurological symptoms improved markedly except minimal weakness in his lower extremities . this again suggests the etiology of negative symptoms is associated with vitamin b12 deficiency . though there is literature to suggest correlation between low folate levels and negative symptoms of schizophrenia , evidence for association of vitamin b12 or other nutritional deficiency with negative symptoms is lacking . this case suggests that predominant presentation with negative symptoms without other psychopathology can be a manifestation of an underlying vitamin b12 deficiency . therefore , complete case work up is recommended , including measurement of serum vitamin b12 level , in patients presenting only with predominant negative symptoms . we can only assume that had psychiatric symptoms been recognized and treated in time as a manifestation of vitamin b12 deficiency , development of neurological symptoms in this case could have possibly been prevented .

long lists of psychiatric illness or symptoms have been documented to be caused by vitamin b12 deficiency . we describe an atypical case of a young adult who presented with predominant negative symptoms followed by neurological symptoms consistent with vitamin b12 deficiency . the symptoms showed complete remission after vitamin b12 supplementation . the uniqueness of this case is that vitamin b12 deficiency presented with predominant negative symptoms without other psychotic and manic symptoms , which has not been reported previously .

glomeruloid hemangioma is a specific marker for poems syndrome.few cases of glomeruloid hemangioma without poems syndrome have been reported in the literature . glomeruloid hemangioma for a histologically distinctive , cutaneous , benign vascular tumor , which is considered to be a specific marker for the polyneuropathy , organomegaly , endocrinopathy , monoclonal gammopathy , and skin changes syndrome ( poems ) syndrome . clinically , it is characterized by a solitary or multiple blue - red papules and histologically it resembles renal glomeruli . the poems syndrome is a multisystem disease comprising of polyneuropathy , organomegaly , endocrinopathy , monoclonal gammopathy ( m - protein ) , and skin changes . contrary to what has been previously believed , glomeruloid hemangioma has recently been reported without any evidence of the poems syndrome . two young men , of age 18 and 22 years , presented with asymptomatic papulonodules of six months ' and ten years ' duration , respectively . one case had slowly progessing , multiple , pale , skin - colored papules and nodules present over the chin [ figure 1 ] . few of the papular lesions were translucent in appearance and one of the papules had a the second case had multiple , skin - colored , dome - shaped papules and nodules , present over the anterior and lateral aspect of neck , varying in size from 2 mm to 2 cm [ figure 2 ] . skin - colored papules and nodules on the chin , with a nipple - like projection skin - colored , dome - shaped papules and nodules on the neck both underwent routine laboratory tests , which revealed no abnormality . abdominal ultrasound was normal and endocrinological evaluation , comprising of thyroid function tests , serum estradiol , serum testosterone , serum prolactin , and serum cortisol were all within normal range . a nerve - conduction velocity study and a total body skeletal survey were normal . both cases had no abnormal findings on chest x - ray or computed tomography ( ct ) scans . inside these dilated vascular spaces , a conglomeration of capillaries lined by plump , swollen endothelial cells , filled with red blood cells , resembling renal glomeruli , were seen [ figures 3 and 4 ] . variable - sized , homogeneous , eosinophilic , periodic acid - schiff ( pas)-positive , diastase - resistant globules were present in the endothelial and stromal cells . photomicrograph showing glomeruloid aggregates of vascular structures in the dermis , resembling renal glomeruli ( h and e , 40 ) photomicrograph showing a glomeruloid structure with dilated capillaries lined by plump endothelial cells , filled with red blood cells ( h and e , 400 ) glomeruloid hemangiomas represent vascular tumors , which present as small , firm , red - to - violaceous , dome - shaped papules , papulonodules , subcutaneous bluish compressible tumors , wine - red sessile or pedunculated papules , or lesions with cerebriform morphology . they range in size , measuring few millimeters to few centimeters in diameter , and are located mainly on the trunk and proximal limbs . rarely , glomeruloid hemangioma involving the face has also been reported . it probably represents a reactive endothelial proliferation , wherein , the overproduction of the vascular endothelial growth factor ( vegf ) , il-6 , il-1 , tumour necrosis factor- , and estrogens are believed to be responsible for angioma formation . hemangiomas in the poems syndrome fall into four histopathological categories : microvenular hemangiomas , cherry hemangiomas , multinucleated cell angiohistiocytomas , and glomeruloid hemangiomas . they have been considered by some authors , to represent different stages in the development of the same lesion , which exhibit different degrees of endothelial proliferation , in response to angiogenic stimuli . tufted angioma is stated to be the most immature form of angiomatous lesions in the poems syndrome . a vascular lesion may appear well before the full blown poems syndrome develops , and therefore , a thorough evaluation and follow - up of patients with glomeruloid hemangioma , for potential development of this syndrome , is still recommended . the first case that was followed up for one year did not show any features of the poems syndrome and the other case was lost to follow - up . glomeruloid hemangioma may have a varied clinical presentation , as seen in our cases.glomeruloid hemangioma presenting with a nipple - like projection has not been reported to our knowledge.clinicians require a high index of suspicion to dignose glomeruloid hemangioma without the poems syndrome . clinicians require a high index of suspicion to dignose glomeruloid hemangioma without the poems syndrome .

glomeruloid hemangioma is a rare , histologically distinctive , cutaneous , benign vascular tumor , originally described by chan et al . glomeruloid hemangioma appears specific to polyneuropathy , organomegaly , endocrinopathy , monoclonal gammopathy , and skin changes syndrome ( poems ) , and is usually , but not always , related to castleman 's disease . we herein report two cases with glomeruloid hemangioma , without any features of the poems syndrome . glomeruloid hemangioma requires a high index of suspicion for diagnosis . it could rarely present as a solitary finding , without any evidence of an underlying poems syndrome .

steroid cell tumors of the ovary are rare hormone secreting tumors characterized by a steroid cell proliferation , of which there are three subgroups : steroid cell tumor not otherwise specified ( nos ) , stromal luteoma and leydig cell tumor . steroid cell tumors account for less than 0.1% of all tumors of the ovary , and of the three subtypes steroid cell tumor ( nos ) is the most common . . they may present as post menopausal virilization and have been reported in patients ranging in age from two and half years to 80 years . although , there are many case reports on steroid cell tumor ( nos ) of the ovary , reports of bilateral tumors are very rare , and one clinic pathological study had revealed the tumors to be bilateral in 6% of patients . a 54-year - old gravida two , para two post menopausal woman presented with a 6 month history of progressively worsening facial hair growth and receding hairline . there was no history of bleeding per vagina , abdominal pain , or distension . on examination she had greasy skin and although post - menopausal she had non - atrophic breasts . hormonal assessment revealed an inappropriately high serum follicle - stimulating hormone ( fsh ) level of 1.1 miu / ml ( post menopause > 30 miu / ml ) , serum luteinizing hormone ( lh ) level of 3.2 miu / ml ( post menopause > 30 miu / ml ) , and a serum testosterone level of 7.31 ng / ml ( normal 0.4 - 0.76 ng / ml ) . additionally , she had a serum dehydroepiandrosterone sulfate ( dhea - s ) level of < 15 g / dl ( normal 35 - 430 g / dl ) and a serum testosterone after dexamethasone suppression of 6.32 ng / ml ( not suppressed ) . the hormone profile demonstrated normal values for serum prolactin , cortisol , thyroid - stimulating hormone , and human chorionic gonadotrophin . imaging by transvaginal ultrasound and computed tomography of abdomen and pelvis revealed no ovarian masses . thus , ovarian venous sampling was carried out under radiological guidance , but the left ovarian vein cannulation was technically difficult and the procedure was relatively unsuccessful except for revealing that the right ovary was a source of testosterone in this patient [ table 1 ] . results ovarian venous sampling for serum testosterone and estradiol the patient , therefore , underwent hysterectomy and bilateral salpingo - oophorectomy . during the laparotomy , straw - colored ascitic fluid the right ovary was slightly enlarged ( 4 1.5 1.3 cm ) showing a slightly elongated , nodular appearance . left ovary was normal in appearance ( 3 1.6 1.4 cm ) [ figure 1 ] . microscopic sections of both ovaries revealed infiltrating islands and cords of tumor cells exhibiting round to oval nuclei and abundant eosinophilic cytoplasm [ figures 2 and 3 ] . there were no features to suggest malignant behavior such as nuclear atypia , hemorrhage , necrosis or increased mitotic activity . macroscopic appearance of resected specimen infiltrating tumor of the left ovary ( h and e , 40 ) islands of tumor cells in the right ovary ( h and e , 40 ) tumor cells are strongly positive for alpha inhibin ( h and e , 40 ) by 3 weeks post surgery , her serum testosterone had returned to normal range : 0.38 ng / ml and the clinical signs of hyperandrogenism resolved . she currently remains asymptomatic with normal testosterone levels and imaging at 6 months and 12 months after surgery . steroid cell tumor nos is usually composed of solid aggregates of cells with occasional nests or trabeculae . tumor cells are polygonal in shape with granular eosinophilic cytoplasm and sometimes show vacuolated cytoplasm . cytoplasmic lipofusin pigment may also be identified as a brown cytoplasmic pigment seen in this case . steroid cell tumors nos are differentiated from leydig cell tumors because of their lack of cytoplasmic reinke crystals which are eosinophilic rod shaped inclusions . the feature that makes this case unique is the presence of tumor cells in bilateral ovaries . although , there were tumor cell clusters in both ovaries , there was no evidence of capsular extension in either ovary . it could be argued that the second ovary may well be harboring the deposits of the malignant tumor in the 1 ovary . hayes and scully reported on certain histopathological features which correlate highly with clinically malignant behavior . these being two or more mitotic figures per 10 high power fields , necrosis , a diameter 7 cms , hemorrhage , and grade 2 - 3 atypia . the complete resolution of serum testosterone to normal levels following removal of ovaries was also against the possibility of malignant ovarian deposits . despite several reports of steroid cell tumor nos leading to a wide variety of clinical presentations , there has been very few cases reported on tumor occurring in bilateral ovaries . in the clinico - pathological analysis of 63 cases of steroid cell tumor nos by hayes and scully , they reported that in 6% of patients the tumors were bilateral . in another clinic pathological study of eight patients , the exact pathophysiology for bilateral occurrence is not very clear at this stage and warrants future evaluation . although the tumors were pathologically benign steroid tumor nos , as there have been reports of benign tumors with malignant behavior , careful follow up was carried out in this patient and at 1 year , there were no indication of metastasis .

steroid cell tumors of the ovaries are rare sex - hormone secreting tumors which are usually benign and unilateral . one previous study has estimated the tumors to be bilateral in 6% of patients . we report a case of post menopausal virilization where tumor histology revealed steroid cell tumor not otherwise specified with benign charactsristics . the presence of tumor in bilateral ovaries made this case unique .

before developing an acl - control strategy , we conducted a cluster - based , house - to - house survey in kabul city between july and september 2001 to collect data on the extent of acl . each of the city s 14 districts was divided into random clusters according to the district s population size , for a total of 90 sample clusters ; 30 neighboring households were surveyed in each cluster , with the first household selected at random . a team of medical staff diagnosed disease in household members on the basis of presence or absence of acl lesions or scars , number of lesions , and date of lesion onset ; members were interviewed to collect demographic data such as gender and age . because of logistic constraints , parasitologic diagnosis of acl lesions ( e.g. , microscopic examination or parasite culture ) was not carried out . however , in afghanistan , acl - like skin lesions from other causes are rare , and our experience suggests that clinical diagnosis has a sensitivity and specificity of > 80% and > 90% , respectively ( reithinger et al . , unpub . data ) . written approval to conduct the study was obtained from the afghan ministry of public health . informed consent was obtained from study participants ; all persons with active cases surveyed were offered free antileishmanial treatment at the healthnet international clinic . of 26,892 persons surveyed , 726 ( 2.7% ) and 5,900 ( 21.9% ) had active leishmaniasis lesions or scars , respectively . of those persons with acl lesions , the mean lesion number was 2.4 ( range 150 ) and the mean lesion duration ( to survey date ) was 9.1 months ( range 0.196 ) . a total of 26,887 observations , with full disease and demographic records , from 2,683 households from the 90 sample clusters , were used in logistic regression analyses with a binary outcome variable ( acl lesion or scar ) . four variables were created to assess the distribution of leishmaniasis cases : the prevalence of active lesions in other members of the same household , the prevalence of scars in other members of the same household , the prevalence of active lesions in the nearest neighbor households , and the prevalence of scars in the nearest neighbor households . the nearest neighbor households were defined in terms of the survey protocol . in a given sample cluster , the nearest neighbor to household 1 was household 2 ; the nearest neighbors to household 2 were households 1 and 3 ; and so forth . the logistic regressions with robust standard errors ( i.e. , clustering of households to control for within - household correlations ) were also adjusted for age ( continuous to year of age at last birthday ) , gender , and sample cluster ( categorical 90 levels ) . all analyses were conducted in stata 7 ( stata corporation , college station , tx ) . the regression analyses show that female patients are at significantly higher odds of having leishmaniasis lesions or scars ( table ) . odds of disease were associated with age : elderly people are at slightly greater risk of having active lesions ; and elderly persons are less likely to have leishmaniasis scars ( the drop in scar prevalence in persons > 12 years was significant , odds ratio : 0.994 ( 95% confidence interval [ ci ] 0.991 to 0.997 ) , p<0.001 ) . a person s probability of having an active lesion was increased greatly if other lesions appeared on persons in the same household ( figure , a ) and also ( but less so ) if other scars occurred on persons in the same household . a person s probability of having a scar was greatly increased if persons with active lesions or scars were present in the same household ( figure , b ) . finally , a person s probability of having a scar was greatly increased with the presence of persons with active lesions or scars in neighboring households ( figure , c ) . no significant clustering of acl lesions occurred between households ; however , the sample size was small for analyses . overall , these findings are consistent with clustering of acl transmission , including transmission in areas where previous transmission has occurred ( because of association with scar prevalence ) . when maximum likelihood methods ( 6 ) are used , the average annual force of acl infection ( ) was estimated to be 0.029 per year ( 29 cases/1,000 persons per year ; 95% ci 0.028 to 0.031 ) over the past 12 years ( figure , d ) abbreviations used : or , odds ratio ; ci , 95% confidence intervals ; d.f . , degrees of freedom ; ns , not significant . the statistical model controlled for age ( in years ) , sex , and sampling area ( a total of 90 areas ) , while the standard errors were adjusted for sampling of persons at the household level . the overall significance of the variable is shown rather than the ors for the 90 different sampling areas . a , the average probability of having a lesion at different levels of lesion prevalence recorded among other members of the same household ( open circles ) and the unadjusted fit ( solid line ) from the logistic regression . b , the average probability of having a scar at different levels of scar prevalence recorded in other members of the same household ( open circles ) and the unadjusted fit ( solid line ) from the logistic regression . c , average probability of having a scar at different levels of scar prevalence in nearest neighbor households ( open circles ) and the unadjusted fit ( solid line ) from the logistic regression . d , force of infection , , can be estimated from the age - prevalence data , where the proportion , p , of persons with acl at age a ( where a is age at last birthday plus 0.5 years ) is given by p(a ) = 1-exp(-a ) ( 6 ) . if one assumes that age - independent transmission started 12 years earlier ( 1 ) , was estimated by maximum likelihood by using the observed age - prevalence data for children < 12 y of age . currently six clinics provide leishmaniasis diagnostic and treatment services in kabul ; an estimated 20% of the total 67,500 patients ( based on the observed prevalence of 2.7% and a total 2.5 million population for kabul ) are diagnosed and treated . whether this fact alone could explain the extent and duration of the leishmaniasis epidemic in kabul is uncertain . our analyses show that persons are at high risk for active acl when a high proportion of persons with acl scars are in the same or neighboring households . the likely explanation for this finding is that sandfly distribution and abundance are patchy but stable over time . the high prevalence of persons with active acl in kabul and the comparatively high acl incidence show that acl - control strategies ( e.g. , increasing the number of clinics providing treatment facilities or providing personal protection methods against sandflies ) should be conducted soon . we demonstrate that a blanket - coverage acl - control strategy is not necessary : transmission of this disease is focalized , and interventions ( e.g. , household insecticide spraying , insecticide - impregnated bednets or chaddars ) ( 5 ) targeting households with a high proportion of persons with leishmaniasis lesions or scars or city districts containing a high number of high transmission clusters should have a major impact on transmission in kabul . the international donor community often considers acl to be of peripheral importance ( e.g. , the disease was not included in the basic package of health services for afghanistan ) ( 7 ) because this disease has no impact on death rates and patient treatment costs ( usually u.s.$15200 ) are not recovered . failure to implement a control strategy for this disease will likely lead to an increase in its impact and social stigmatization and represent further problems for a health infrastructure already crippled by 20 years of war .

a prevalence survey in kabul city showed that 2.7% and 21.9% of persons have active leishmaniasis lesions or scars , respectively . incidence of disease was estimated to be 2.9% ( 29 cases/1,000 persons per year ; 95% confidence interval 0.018 to 0.031 ) . disease was associated with age and gender ; logistic regression analyses showed significant clustering of cases .