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malignant thymic neoplasms can be broadly divided into thymomas and thymic carcinomas , both of which exist almost exclusively in the anterior mediastinum . thymomas have also been described in the neck , posterior mediastinum , lungs , base of the skull and pleural cavity [ 25 ] . very few case reports have described thymomas occurring in the middle mediastinum [ 25 ] . unlike thymomas , thymic carcinomas have only been observed in the anterior mediastinum . to our knowledge , this report will describe the first case of thymic carcinoma in the middle mediastinum . a 55-year - old caucasian female presented with productive cough , dyspnea , chest pain and weight loss . the patient 's medical history was significant for hypertension , hypercholesterolemia , dyslipidemia , chronic renal failure , gastroesophageal reflux disease and chronic anemia . she was a 30 pack - year smoker and her mother was diagnosed with lung cancer . an initial chest x - ray showed a large subcarinal mass . on computed tomography ( ct ) , a 6.2 5 cm tumor was detected in the subcarinal area ( figs 1 and 2 ) . a bronchoscopy with transbronchial biopsy was performed , and the resulting pathological analysis was suspicious for malignant cells ; however , no specific tumor type was identified . a positron emission tomography scan showed a large hypermetabolic subcarinal lesion with a standard uptake value of 14.3 , consistent with malignancy . the differential diagnosis at the time included an infected bronchogenic cyst , esophageal duplication cyst or a malignant subcarinal lymph node . thoracoscopic surgical resection was planned for diagnostic and therapeutic purposes . figure 1:subcarinal tumor on preoperative ct of the chest , coronal cuts . prior to surgical resection , esophagoscopy and bronchoscopy documented the absence of any communication of this mass to the esophagus and airway . one port was made at the mid - axillary line of the eighth intercostal space for the camera , in addition to two anterior and two posterior ports . the dissection of the tumor began at the level of the inferior pulmonary ligament and posterior mediastinum , all the way to the azygous vein . the esophagus was found completely adherent to the tumor , necessitating an esophageal myotomy for en bloc resection . the anterior dissection proceeded at the level of the posterior atrial wall , the inferior and superior pulmonary veins , the main trunk of the pulmonary artery , and the membranous areas of the right and left main stem bronchi . complete gross resection was observed at the conclusion of the operation . at the end of the procedure , air was insufflated into the esophagus to confirm the absence of any leaking from the mucosa . the specimen consisted of an encapsulated red - brown tissue weighing 57 g , and measuring 5.5 4.5 5 cm . some sheets of viable tumor cells were present , and these contained pleomorphic nuclei , vesicular chromatin , and prominent nucleoli . the tumor cells were strongly positive for cam 5.2 , but negative for ck7 , ck5/6 , ck19 , calretinin , ttf1 , cea , afp , hcg , plap , cd5 and cd56 . thus , thymic carcinoma was determined to be the most fitting diagnosis based on the morphology and staining . the patient was referred for adjuvant radiation . at the most recent follow - up visit 9 months after surgery , the patient was well with no evidence of recurrence on ct scan . all thymic carcinomas reported to date have been located in the anterior compartment of the mediastinum . to the best of our knowledge , this is the first case report of a thymic carcinoma occurring in the middle mediastinum . the differential diagnosis of middle mediastinal tumors usually includes bronchogenic cysts , enterogenous cysts , neuroenteric cysts , pericardial cysts and lymphangiomas . very selected cases of thymoma have been reported in the middle mediastinum [ 25 ] . these are attributed to the presence of ectopic thymic tissue in the subcarinal area due to failure of the thymus to migrate into the anterosuperior mediastinum during embryological development [ 25 ] . by way of the same process , it is therefore theoretically possible for these cells to undergo malignant degeneration into thymic carcinoma , although this has not been previously described . when compared with thymomas , thymic carcinomas have considerably worse prognosis . when possible , complete surgical resection is the treatment of choice for thymic carcinomas [ 1 , 6 ] . therefore , when planning surgical resection , it is important to consider malignant neoplasms as part of the differential diagnosis of middle mediastinal tumors . although rare , thymic carcinomas and other malignant tumors of the middle mediastinum should be resected with negative margins , in order to decrease the risk of local recurrence and improve prognosis . this is the first case report of a malignant thymic carcinoma occurring in the middle mediastinum at the subcarinal location . this pathology , although rare , should be added to the differential diagnosis of middle mediastinal tumors . when planning surgical resection of such tumors , complete excision with negative margins should be attempted to minimize chances of local recurrence and improve prognosis .
we report an extremely rare case of a thymic carcinoma occurring in the subcarinal location of the middle mediastinum , masquerading as subcarinal adenopathy . a 6.2 5 cm mass was detected on computed tomography scan and found to be hypermetabolic on positron emission tomography scan . transbronchial biopsy was performed and the mass was found to be suspicious for malignancy . a thoracoscopic resection of the mass was performed and found to be thymic carcinoma . the patient underwent a course of adjuvant radiation . to the best of our knowledge , this is the first reported case of a thymic carcinoma resected from the middle mediastinum .
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amyloidoses can be classified into three categories : primary localized cutaneous amyloidosis ( plca ) , secondary localized cutaneous amyloidosis , and systemic amyloidosis with cutaneous involvement . in the plca group , three types can be distinguished : macular , papular ( lichenoid ) and nodular forms . the first two are mainly located on the trunk , and cytokeratins serve as the amyloid precursors . here , amyloid deposition is limited to the papillary dermis [ 1 , 2 , 3 , 4 , 5 ] . however , in the rare cases of nodular plca , amyloid consists of aggregated kappa and lambda light chains , which can be found both in the dermis and the subcutaneous tissue [ 1 , 2 , 3 , 4 , 6 ] . furthermore , nodular plca can originate from systemic amyloidosis or progress to systemic disease [ 3 , 4 ] . here , we present a case of nodular plca on the temple of a 52-year - old woman . a 52-year - old lady presented with a soft , shiny , partially yellow , erythematous tumor ( 3.5 4.5 cm ) with telangiectasia on her right temple ( fig . she recalled having had this tumor for about 4 years and that it had significantly grown lately . the diagnosis was confirmed by electron microscopy , and the typical amyloid fibrils ( 710 nm in diameter ) were found ( fig . a punch biopsy taken during follow - up showed some remaining amyloid so that the patient is now scheduled to undergo a second surgery via curettage . the diagnosis of plca requires histological analysis of a skin specimen , complemented by immunohistochemistry and electron microscopy . histologically , eosinophilia as well as positivity in periodic acid - schiff , congo red , and thioflavin t stainings are characteristics of amyloid deposits [ 1 , 8 ] . immunohistochemistry with antibodies directed against cytokeratin and immunoglobulin light chains ( lambda and kappa ) allows to further distinguish between the different forms of plca . electron microscopy confirms the diagnosis of amyloidosis when the typical amyloid fibrils ( 710 nm in diameter ) are found . image quality improves significantly when the skin sample is fixed in karnofsky 's fixative ( glutaraldehyde ) instead of paraformaldehyde . for patients with nodular plca , it is recommended to asses for progression to systemic amyloidosis on a regular basis . this should include a full history and physical examination along with electrocardiogram , complete blood count , serum creatinine levels , serum liver - associated enzyme levels , serum electrophoresis , and urine examination . furthermore , an abdominal fat biopsy has been suggested as an easy method to detect a potential progression to systemic disease [ 3 , 7 ] . it has to be mentioned though that nodular plca is more frequent in asia and south america when compared to europe or north america .
a 52-year - old woman presented with a large partially yellow and erythematous tumor on her right temple . she reported that it had grown over the last 4 years . regional lymph nodes were impalpable . a punch biopsy showed eosinophilic material in the dermis and subcutis . immunohistochemistry showed positive staining for kappa and lambda light chains . electron microscopy showed the typical amyloid fibrils ( 710 nm in diameter ) . there was no evidence of systemic amyloidosis , paraproteinemia or underlying plasmacytoma . the tumor was completely removed via curettage . at follow - up , the patient presented in good health with no signs of relapse .
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palatal rugae are irregular , asymmetric ridges of mucous membrane extending laterally from the incisive papilla and the anterior part of the median palatal raphe . these structures have been used as internal cast reference points for quantification of tooth migration . a few investigators have shown the medial rugae region to be stable or show predictable changes post orthodontic therapy . the rugae patterns are completely formed by the 12 to 14 week of prenatal life and remain stable thereafter . these are unique to each person and show distinctiveness based on ethnic groups and hence are useful in forensic identification . because they are stable landmarks the palatine rugae play a significant role in clinical dentistry as well . the objective of this study was to analyze and characterize the rugae patterns , compare the rugae dimensions in various age groups and to ascertain any relationship between dimensional analysis and palatal depth . cross - sectional casts of 52 females and 48 males were selected from the archival section of the department of orthodontics , m. m. college of dental sciences and research , mullana ( ambala ) and studied employing following parameters : assessment of age ( according to erupted teeth).gender differentiation ( based upon records).division of medial palatal region into : a : distance between incisive papilla length and anterior limit of the anteriormost rugae.b : distance between incisive papilla and posteriormost rugae limits.lateral rugae dimensions . palatal depth ( measured from healthy gingival margin underneath the mesiolingual cusp to the deepest concavity of the palatal arch using a brass wire between two opposing points and measuring the vertical distance at the center).rugae patterns . division of medial palatal region into : a : distance between incisive papilla length and anterior limit of the anteriormost rugae.b : distance between incisive papilla and posteriormost rugae limits.lateral rugae dimensions . a : distance between incisive papilla length and anterior limit of the anteriormost rugae . palatal depth ( measured from healthy gingival margin underneath the mesiolingual cusp to the deepest concavity of the palatal arch using a brass wire between two opposing points and measuring the vertical distance at the center ) . no patient details were disclosed and ethical guidelines as per the declaration of helsinki were followed . correlations among a , b , lateral rugae patterns and palatal depth were calculated by mean s.d . correlations among a , b , lateral rugae patterns and palatal depth were calculated by mean s.d . followed by evaluation of p values . the rugae patterns identified were : common origin ; separate origin ; lateral branching ; secondary rugae and fragmentary patterns . females were found to have slightly higher predilection towards having the common , fragmentary and lateral branching rugae patterns . there was a stronger female predisposition for rugae with separate origin , whereas , the secondary rugae were equally existent in either gender . significant correlation was found between the a ( p=0.03 ) and b values ( p=0.02 ) on comparing the mean s.d . values of age groups between 12 - 13 years and > 14 years , respectively . hence , there is a corresponding anteroposterior increase in palatal dimensions in these age groups [ table 1 ] . this study showed no changes in the lateral rugae dimension and palatal depth with increasing age . the majority of the study cohort ( 57% ) had a palatal depth in the range of 1.6 - 2.0 cm [ table 2 ] . palatal rugae have been used as reference points for many purposes such as evaluating tooth movement pre- and post - orthodontic treatment , population studies and forensic identification . stability of medial palatal region has been a subject due to differences among various investigators . christou and kiliardis evaluated the vertical changes in the medial aspects of the rugae and concluded that these changes over time are due to the alterations in the vertical positioning of maxillary incisors and increase in lower face height . growth periods ( 12 - 13 years and > 14 years ) studied in this paper , showed a downward and forward movement of the maxilla in relation to the cranial base and also , changes in the size and shape of maxilla by structural remodeling . these phenomena can be explained by the deposition of new bone on the oral surface of the palate and at the alveolar crest . therefore , the changes in rugae dimensions can be the result of this differential growth in the palate and alveolar crest.[24 ] rugae patterns showed a strong female predilection for rugae with separate origins [ figure 1 ] whereas slightly higher incidences were noted for rugae with common origin , fragmentary and lateral branching patterns . there was an equal percentage of gender with secondary rugae pattern in the ethnic segment of north indian population studied [ table 1 ] . photograph depicting rugae patterns with secondary origin and fragmentary nature the purpose of this paper was to evaluate a cross - sectional patient database to analyze the anteroposterior stability of the medial rugae region . analysis of 100 study casts showed a significant difference in the medial rugae region ( p= 0.03 , 0.02 ) in a and b values . no significant changes were noted in the lateral rugae dimensions . the palate growth in this period was found to be non - significant . hence , it can be surmised from the analysis of the results that there is a differential growth spurt in the anterior and posterior palate during adolescence as is marked by the significant differences in the measurement values obtained through this study . palatal rugae can be studied as a strong indicator of ethnicity , gender differentiation and study of growth changes in the anterior maxilla . thus , rugae are important tools in clinical investigations involving forensic anthropology and developmental biology .
introduction : rugae patterns are significant markers for analyzing anteroposterior changes in adolescence , and forensic investigations.aim:the purpose of this study was to ascertain the gender - wise predisposition of rugae patterns and to analyze anteroposterior alterations along with any developmental changes in palatal depth.materials and methods : one hundred pre - treatment study cast models were obtained from the archives of the department of orthodontics , m.m . cdsr , mullana . parameters employed were : division of medial palatal region into a : distance between incisive papilla length and anterior limit of the anteriormost rugae ; b : distance between incisive papilla and most posterior rugae limits ; measurement of lateral rugae dimensions and palatal depth . statistical analysis : mean s.d . values were obtained and p values calculated.results:comparison of a and b showed a significant difference in the p values between the age - groups 12 - 13 years and > 14 years . rugae patterns with separate origins showed a predisposition for female gender.conclusion:a significant change in the anteroposterior medial rugae dimensions was seen in adolescent age groups alongside no variation in palate depth . hence , it can be concluded that differential growth potential is present in the premaxilla and can cause shift in medial palatal dimensions without altering the rugae patterns and palatal depth .
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median arcuate ligament syndrome ( mals , also called celiac axis compression syndrome or dunbar syndrome ) is known to be caused by compression of celiac artery ( ca ) by a fibrous arch that originates from the diaphragmatic crura on either side of the aortic hiatus and passes superior to the origin of the celiac axis . though it is still debated whether the compression of the ca can cause chronic mesenteric ischemia or not , there have been anecdotal case reports of surgical decompression of ca by dividing the medial arcuate ligament ( mal ) . the common clinical features of mals are chronic postprandial abdominal pain characteristically augmented by full expiration , nausea or vomiting , weight loss and audible epigastric bruit . the diagnosis of mals usually depends on the clinical features and radiologic finding of the focal narrowing at the proximal celiac axis on a lateral view of conventional aortography or computed tomography ( ct ) . while open surgical release of mal has been used for the treatment of this syndrome , laparoscopic release has been reported recently . however , it was usually ineffective owing to the refractory extrinsic compression of the ca by the tight ligament structure . we would like to report a case of surgical treatment of mals describing its clinical features , characteristic findings of diagnostic imaging study , and details of our surgical procedure . a 37-year - old female patient presented with chronic epigastric pain lasting 6 months , food phobia and weight loss of 10 kg during the previous 4 months . the pain was cramping aggravated after meals , and persisted for 20 - 30 minutes after meals . there were no other gastrointestinal symptoms such as diarrhea , constipation , gastrointestinal bleeding and nausea or vomiting . as past history , she underwent coil embolization of the bilateral ovarian veins at another hospital 2 months before visiting us under the impression of pelvic congestion syndrome , which did not improve her abdominal symptoms . physical examination showed mild abdominal tenderness at both lower quadrants without muscle rigidity . laboratory test showed normal range including serum amylase , liver enzyme and complete blood cell count . gastro - duodenoscopy showed chronic atrophic gastritis and 18-fluorodeoxyglucose positron emission tomography - ct showed no spe cific lesion with abnormal hot uptake . 1 ) showed downward angulation of ca and superior mesenteric artery ( sma ) close to their origins by the compression . to decompress ca , we approached through the upper mid line incision and lesser sac . postoperatively , epigastric postprandial pain was resolved and the patient could return to a normal diet . on a follow - up mal was first described by lipshutz as an anatomic structure that caused ca compression in 1917 . thereafter , harjola and dunbar et al . described it as a clinical syndrome causing nausea , vomiting and postprandial pain in 1963 and 1965 . the origin of ca and mal varies in its location from t11 to l1 , and occasionally , their locations are in conflict with each other when mal extends inferiorly or ca originates su periorly . during deep inspiration in the erect position during expiration , the condition is opposite , and compression causes the symptom and is thus called mals . at this point , it is proposed that mals is related with neurogenic pain from the compression and intermittent ischemia of splanchnic nerve plexus . this pain can be caused either by nerve stimulation leading to vasoconstriction or by direct sympathetic fiber irritation . splanchnic nerve plexus is an autonomic nerve plexus supplying upper abdominal organs ( stomach , liver , gallbladder , pancreas ) . this is located in front of diaphragmatic crura around the origin of ca and sma . to make a diagnosis of mals , other common causes of abdominal pain routine laboratory blood tests including amylase , lipase and tumor markers for hidden malignancy , esophago - gastro - duodenoscopy , liver , pancreas and kidney ultrasonography are usually used . a typical feature is focal narrowing of ca with poststenotic dilatation aggravated during deep inspiration . nowadays standard treatment of mals is an open surgical division of mal followed by a dramatic symptom relief . recently , a lapa roscopic approach can be attempted but carries the risk of arterial injury and massive hemorrhage . three series of case reports showed mean rates of open conversion due to bleeding at about 20% , but despite that , laparoscopic treatment showed no other morbidity or mortality and shortened hospital stay . this is probably due to the extraluminal compression by mal , which should be solved outside of the ca . in cases of recurrent symptom after surgical decompression of ca , angioplasty is beneficial .
median arcuate ligament syndrome is a rare cause of abdominal pain which results from compression of the celiac artery ( ca ) or rarely , the superior mesenteric artery by a ligament formed by the right and left crura of the diaphragm . we report a case of open surgical decompression of the ca by division of the median arcuate ligament for a 37-year - old female patient who had suffered from chronic postprandial epigastric pain and severe weight loss . we described clinical features , characteristic angiographic findings and details of the surgical procedure for the patient with this rare vascular problem .
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a 38-year - old woman was admitted to our outpatient clinic with accelerating back pain and fatigue following a kick to her back by her husband two days previously . on her physical examination , an ecchymotic area on her back between the scapulae was observed . she had pallor , her blood pressure was 80/60 mmhg in both arms , and was tachycardic on auscultation . st segment elevations were observed in the d1 , avl , and v2 leads , along with accelerated idioventricular rhythm ( fig . 1 ) . transthoracic echocardiography demonstrated akinesia of the anterior septal , apical , basal - mid septal , and basal - mid anterior walls , and her ejection fraction was 20% . we detected a dissection of the left main artery , the left anterior descending artery ( lad ) , and the circumflex artery , originating from the middle portion of the left main coronary artery ( lmca ) ( figs . 2 and 3 , supplemental videos 1 , 2 ) . a saphenous vein was grafted to the distal lad . during the intraoperative evaluation of the epicardial vessels , our team of cardiac surgeons did not plan to place a bypass graft to the circumflex artery because it was thin and non - dominant . since the patient was hypotensive under noradrenaline and dopamine infusions , she was transferred to the cardiovascular surgery intensive care unit on an extracorporeal membrane oxygenator ( ecmo ) and intra - aortic balloon pump ( iabp ) . during follow - up , her blood pressure remained low , at approximately 60/40 mmhg , despite aggressive inotropic and mechanical support . on the second postoperative day , although the patient s lad artery had been revascularized by a saphenous vein graft , her left ventricular ejection fraction remained as low as 10%15% , leading to ventricular failure . asystole and cardiovascular arrest then quickly developed , and despite aggressive cardiopulmonary resuscitation , she died . coronary artery dissection after blunt chest trauma is an extremely rare condition that can be fatal , and some cases are detected in postmortem examinations . left main coronary artery dissection is even rarer . multiple mechanisms exist leading to coronary artery dissection , including intimal tears due to a deceleration injury , compression of the artery between the heart and sternum , coronary spasm , and impairment of the coronary flow by a dissection flap or a superimposed thrombosis . coronary artery dissection is detected most commonly in the lad ( 76% ) , the right coronary artery ( 12% ) , and the circumflex artery ( 6% ) [ 4 , 5 ] . in necropsy series , the most common cause of acquired non - atherosclerotic coronary artery disease is spontaneous coronary artery dissection , and the lad is the artery in which this condition is most commonly detected . the risk factors for spontaneous coronary artery dissection are exercise , arteriosclerosis , cardiovascular disease , oral contraceptive use , marfan syndrome , systemic lupus erythematosis , and connective tissue disorders . however , the time from injury to coronary artery occlusion may vary , ranging from immediately after the trauma to five weeks later . bedside electrocardiography ( ecg ) provides important clues about coronary artery dissection after blunt anterior chest and back trauma . it has been previously reported that patients with baseline ecg changes on admission should be monitored for 24 hours . the ecg may be normal on presentation , but was found to demonstrate st abnormalities in 63% of patients who are admitted for blunt thoracic trauma within 24 hours of observation . our patient presented with st segment elevation the in d1 , avl , and v2 leads , along with accelerated idioventricular rhythm . some emergency therapeutic options exist for patients with spontaneous coronary artery dissection . in some previous case reports , the patients were managed by primary percutaneous coronary angioplasty , especially patients without lmca lesions . however , surgical treatment remains most common treatment and is associated with the best outcomes . an internal mammary artery graft was not utilized in our patient , because she was in cardiogenic shock , and the surgical team tried to save time by using a saphenous vein graft . in order to prevent death , all available measures were used by our surgical team , including intravenous inotropic agents , iabp , and ecmo . nevertheless , using the internal mammary artery for revascularization may have been more effective for restoring left ventricular function . additionally , complete revascularization , including the circumflex artery , spontaneous coronary artery dissection is a rare and potentially fatal complication of blunt chest trauma in younger patients , and early diagnosis and prompt treatment can be life - saving . physicians should be aware of this possibility when evaluating patients in emergency conditions after blunt trauma of any kind . the 12-lead ecg , cardiac bio - markers , and transthoracic echocardiography , along with other imaging modalities , are important in the management of suspected coronary artery dissection in trauma patients .
we present the case of a 38-year - old woman admitted to our outpatient clinic with accelerating back pain and fatigue following a kick to her back by her husband . upon arrival , we detected st segment elevation in the d1 , avl , and v2 leads and accelerated idioventricular rhythm . she had pallor and hypotension consistent with cardiogenic shock . we immediately performed coronary angiography and found a long dissection starting from the mid - left main coronary artery and progressing into the mid - left anterior descending ( lad ) and circumflex arteries . she was then transferred to the operating room for surgery . a saphenous vein was grafted to the distal lad . since the patient was hypotensive under noradrenaline and dopamine infusions , she was transferred to the cardiovascular surgery intensive care unit on an extracorporeal membrane oxygenator and intra - aortic balloon pump . during follow - up , her blood pressure remained low , at approximately 60/40 mmhg , despite aggressive inotropic and mechanical support . on the second postoperative day , asystole and cardiovascular arrest quickly developed , and despite aggressive cardiopulmonary resuscitation , she died .
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a fit and healthy 25-year - old male presented with palpitations from a regular broad complex tachycardia ( bct ) of left bundle branch block ( lbbb)-like morphology , 330-ms cycle length , and no discernible p waves . intravenous adenosine terminated the bct , resting electrocardiogram ( ecg ) was not pre - excited , and echocardiogram was normal . during the electrophysiologic study , retrograde conduction was central and decremental . 1a shows that conduction switched from nodal ( narrow qrs complexes with distinct his potential solid blue arrow ) to the atriofascicular pathway ( lbbb - like complexes and interpolation of the his potential dotted blue arrow ) during wenckebach - pacing maneuver . 1b shows that the intellatip mifi ablation catheter was used to map the atriofascicular pathway potential in sinus rhythm . the 8-mm tip ablation catheter has three radially positioned , equally spaced mini - electrodes , 2 mm from the tip . bipolar recordings were made between these mini - electrodes : m12 , m23 , and m31 . this enabled precise signal localization and clearer identification of the pathway potential than the conventional ablation distal bipolar recording ( blue dotted box , fig . the gains on the catheter mini - electrodes were 5000-fold greater , and those in the conventional distal pole of the ablation catheter were 10,000-fold greater . radiofrequency ablation ( rfa ) at this site ( 60 c , 70 w , and 120 s ) resulted in no pathway conduction and non - inducible bct . atriofascicular pathways consist of fibers arising from the right atrial free wall and insert at or adjacent to the distal right bundle . they usually only conduct in an anterograde manner , participating in the anterograde limb of an antidromic atrioventricular reciprocating tachycardia ( avrt ) with lbbb - like morphology and decremental properties . standard therapy involves targeted rfa around the tricuspid annulus as guided by pathway potentials also known as mahaim ( m ) potentials , . these m potentials can be as large as the his deflection or can be small and narrow with a low amplitude . furthermore , unintentional mechanical trauma by catheter manipulation can result in transient abolition of these potentials from a few minutes to a few hours , . these m potentials are also recorded only in close proximity to the atrial insertion site , and thus , accurate localization and ablation at this site result in successful abolition of this pathway , , . the intellatip mifi catheter has been shown to delineate local electograms better in the isthmus than the conventional bipolar electrode during atrial flutter ablation . the signal amplitude in the mini - electrodes has been noted to be higher than that of the conventional bipolar catheter . we used the same principle in this case to make use of the special characteristics of the novel ablation catheter to localize the atrial insertion point accurately . furthermore , the m potentials , as recorded from the distal mini - electrodes , were clearer and of higher amplitude compared with those recorded using the conventional distal pole of the ablation catheter . we did not compare this with a standard ablation catheter in the same patient to keep the costs of the procedure within reasonable limits and to avoid causing unintentional mechanical trauma to the atrial insertion site .
atriofascicular pathways are a rare cause of antidromic atrioventricular reciprocating tachycardia . the intellatip mifi ablation catheter ( boston scientific , ma , usa ) is a novel ablation catheter that allows enhanced signal clarity with highly localized electrograms . this is the first report of this catheter being successfully used to map and ablate the atriofascicular pathway potentials .
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cutaneous amyloidosis were classified into primary cutaneous amyloidosis ( pca ) , secondary cutaneous amyloidosis , and systemic cutaneous amyloidosis . pca is a rare , chronic progressive skin disease , defined as deposition of amyloid in previously apparent normal skin without systemic involvement . its prevalence were rarely reported . until now , there are fewer than 40 published cases worldwide . etiological factors associated with pca is still unknown , but its striking familial occurence suggests the role of genetic . we report a case with peculiar mottled pigmentation originally referred for vitiligo , but later proved as acd by histopathological examination . the disorder was thought to be familial as his siblings were affected with the similar condition . a 12-year - old boy presented with asymptomatic , generalized mottled hypo- and hyper - pigmented lesions of 6-year duration . the hypopigmented macules were first noticed on his lower extremities and had been slowly progressing to involve almost the entire body ( figure 1a ) . he did not have any history of systemic or cutaneous disease before the onset of the lesions . history of trauma , rubbing of the skin with any material , and extensive sun exposure was denied . his 11-year - old sister and 6-year - old brother experienced similar yet milder symptoms . physical examination revealed an extensive , discrete , pigmented macules distributed nearly all over the body in symmetrical pattern . he was referred to our hospital for skin biopsy with the initial differential diagnoses of vitiligo and pityriasis alba . histopathological examination ( figure 2 ) showed uneven distribution of melanin in the epidermis and deposits of pale pink amorphous material in the papillary dermis . based on clinical presentation and the histopathology examination , we made the diagnosis of primary cutaneous amyloidosis , presented as amyloid cutis dyschromica . the patient was treated with oral acitretin 25 mg per day . by the third month , some improvement was observed as the pigmented macules were slightly lightened ( figure 1b ) . the patient could tolerate the treatment , as there was no significant increase of transaminases and the lipid profile . the diagnosis of acd in our patient was not readily recognized as it mimicked , to some degree , other relatively common disorders with pigmentation feature . that , combined with its low prevalence , had eluded previous attempts at the correct diagnosis and treatment . the peculiar asymptomatic mottled pigmentation is much likely seen in poikiloderma , but as our case showed , without the corresponding signs such as telangiectasia or atrophy . furthermore , our patient was otherwise healthy , showing no signs that might indicate systemic disorders or photosensitivity , e.g. dermatomyositis , lupus erythematosus , or xeroderma pigmentosum . as defined by morishima , acd characterized by diffuse speckled hyperpigmentation with hypopigmented spots without papulation , atrophy , and telagiectasia , mild or no itching , onset before puberty , and focal amyloid deposition in the papillary dermis . duh begins in infancy or childhood , and unlike acropigmentation of dohi , might encompass the whole skin surface with exception of face . for definite diagnosis , histopathologic examination should be shought . the most common epidermal findings of pca were hyperkeratosis , irregular acanthosis with thinning of rete ridges , and expansion of dermal papillae by amyloid deposition . the finding of amyloid bodies in the papillary dermis was crucial in establishing the diagnosis of acd in our patient and disproved duh , in which such deposition was absent . its visualization under polarized light , showing apple - green birefrigence , confirmed the presence of amyloid . special histochemical stains were helpful for confirming the existence of amyloid . in our case , we tried to obtain histopathological examination of the siblings , but the parents denied the request to perform biopsy on the grounds that their clinical appearances were quite similar that the histopathological findings would likely be the same . multiple factors such as race , genetic , and enviromental may play collective roles , making variable degrees of cutaneous amyloidosis . although most cases of acd were sporadic , many have also reported positive family history of pca , suggesting that the important role of genetic factors in its pathogenesis . our patient s siblings experienced similar yet milder symptoms that the disorder was thought to be familial . , familial relationship was found in 5 of 10 patients , and consanguinity was denied in all . amyloidosis cutis dyschromica is assumed to be a congenital disorder and exposure to sunlight is thought to be the major causal factor . the lesions in our patient were more pronounced on exposed parts of body , that we thought sun exposure might be an important cause . acitretin was given and seems to be effective because it may act by minimizing keratinization defect that causes keratin degeneration to amyloid as proposed by some to be the pathogenesis of this disorder . qiao et al . in his series has convincingly shown 100% positivity to immunohistochemical staining for keratin , ck34 e12 and ck5/6 , that the amyloid is thought to be of epidermal origin . that points at a disturbance of keratinocyte repairs after irradiation with ultraviolet . at the third month his serum transaminase and lipid profile were only slightly increased , that we think the drug was safe to be continued . however , it is interesting to note that some authors consider certain populations of asia , e.g. chinese , japanese and thai , particularly susceptible . therefore , it is possible that several cases of this elusive disorders exist , but are often misdiagnosed . on the other hand , the treatment of acd still remains a challenge .
amyloidosis cutis dyschromica ( acd ) is an extremely rare type of primary cutaneous amyloidosis . to date there are fewer than 40 published cases worldwide ; some were reported affecting several family members . its resemblance to other common pigmentation disorders makes it rarely recognized at first sight . our patient , the 12-year - old firstborn son of non - consanguineous parents presented with generalized mottled pigmentation starting from lower extremities . his siblings suffered from similar condition . the clue for diagnosis is the amyloid deposition in the papillary dermis . the etiology of acd is still unknown , but genetic factors and ultraviolet radiation are implicated . it is proposed that disturbance of keratinocyte repair following ultraviolet radiation results in amyloid deposition . the treatment remains a challenge . oral acitretin treatment , thought to repair keratinization defect , gave a slight improvement in our case . our is the first case of acd reported in indonesia .
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nonsteroidal anti - inflammatory drugs ( nsaids ) are the most commonly used medications during the dental treatment for their creditable efficacy in reducing pain and inflammation.1,2 however , the burden of their unwanted side effects is high particularly with traditional nsaids.1,2 this is more likely due to chronic use and reflecting the fact that nsaids are used extensively in the more vulnerable elderly population.1 the reported adverse effects include gastrointestinal and cardiovascular events , alterations in renal function , effects on blood pressure , hepatic injury , and platelet inhibition which can lead to increased bleeding.1 this case report describes an unusual rare adverse event of the use of ibuprofen for pain control post restorative treatment . a 26-year - old , otherwise healthy male has reported to the restorative clinic at al - farabi college , riyadh for investigation and management of pain affecting the posterior left region of the maxilla . his history of chief complaint showed typical presentation of severe pain due to advanced buccal cervical carious lesion . based on clinical findings , radiographic interpretation , and vitality tests , the carious lesion was removed and replaced by composite material ( filtek p90 , 3 m ) . in addition , cleaning and shaping were performed using crown down technique with protaper rotary instruments ( dentsply ) . the patient reported to has an increased erectile function and libido after 2 h from taking the ibuprofen tablet . he has reported that has done three sexual intercourses with his wife at that day . on the next day , he was curious , and he repeated taking the ibuprofen tablet . surprisingly , he had the same experience of his first use of ibuprofen of having increased libido and erectile function that has lasted for at nearly 6 h. he contacted us to question this unusual experience . his medical history was re - reviewed thoroughly with an endocrinologist , and nothing was mentioned . furthermore , tests for complete blood count , testosterone , thyroid stimulating hormone , cholesterol pre and post ibuprofen intake were assessed , and all results came up as normal . the unwanted side effects are well - reported . to our knowledge , this is the first report to present an unusual side effect of increased libido and erectile function post use of ibuprofen . we searched the literature using medline , accessed via the national library of medicinepubmed interface ( http://www.ncbi.nlm.nih.gov/pubmed ) , for articles relating to the existence of reports of adverse events of erectile dysfunction in relation to the use of ibuprofen or nsaids written in english from 1966 to september 2014 . we used the following mesh terms ( nsaids , ibuprofen , erectile dysfunction , libido , and adverse event ) . two relevant reports were found and presented in table 1 . these included studies reported sporadic erectile or sexual dysfunction associated with the use of different types of nsaids . in an animal model study , uqochukwu et al . ( 2011 ) found that the treatment with nimesulide has an impact on the testosterone and estradiol levels . however , at the doses studied , there were no significant changes in testicular architecture except for mild degenerative changes.5 the most recent study showed that the aspirin was effective in improving lithium - related sexual dysfunction in men with stable bipolar affective disorder.6 with no doubt , we can not speculate that ibuprofen can improve sexual dysfunction , but nsaids could have a role in treating such patients .
nonsteroidal anti - inflammatory drugs are the most commonly used medications for pain control in dentistry . the reported adverse effects include gastrointestinal and cardiovascular events , alterations in renal function , and effects on blood pressure , hepatic injury , and platelet inhibition which can lead to increased bleeding . this case report describes an unusual rare adverse event of the use of ibuprofen for pain control post restorative treatment . a 26-year - old , otherwise healthy saudi male reported an unusual side effect of increased libido and erectile function post use of ibuprofen . the medical and laboratory tests have failed to identify a link between this rare adverse event and either underlying conditions or possibly related etiology . this case represented a puzzling challenge with no clear explanation .
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maxillofacial injuries have the potential to cause airway compromise and are associated with pain and swelling causing difficulty in mouth opening , chewing and deglutition . it helps in intraoperative pain relief and also helps in early post operative rehabilitation of maxilla facial trauma patients mandibular nerve block is often performed for diagnostic , therapeutic and anesthetic purposes for surgery involving mandibular region . we report a case where intraoperative and post - operative pain in a case of unilateral fracture mandible was effectively managed through intermittent mandibular nerve block via a catheter . a 30-year - old male with right sided parasymphyseal fracture mandible was scheduled for open reduction and internal fixation . the patient had difficulty in opening the mouth due to pain ( 3 cm ) . informed written consent for the nerve block was obtained and visual analogue scale ( vas ) of 0 - 10 , was explained to patient . in the operation theater , neuromuscular blockade was achieved with vecuronium and anesthesia was maintained with o2 and n2o using controlled ventilation . the right side of the face was prepared for mandibular nerve block with lateral extraoral approach [ figure 1 ] . arrow showing skin site of epidural needle insertion for mandibular nerve block an 18-gauge i.v . cannula was inserted at midpoint of lower border of the zygomatic arch and was advanced perpendicular to face until it contacted the lateral pterygoid plate . the length of the cannula outside the skin was marked and cannula was redirected slightly posterior to reach behind the posterior border and was advanced further by 0.5 cm . catheter was tunnelled subcutaneously and the filter was attached to its other end [ figure 2 ] . for surgical analgesia , a bolus dose of 4 ml of 0.25% bupivicaine was given through the catheter . fentanyl 1mg/ kg i.v . was given only when there was more than 20% increase in heart rate or blood pressure above base line . the surgery lasted for 2 h. at the end of surgery , neuromuscular blockade was reversed and the trachea extubated . following extubation , the patient was conscious and pain free and then shifted to ward . post operatively , he received 4 ml of 0.25% bupivacaine through the epidural catheter every 12 h for two days . vas score was measured immediately after surgery and thereafter at 1 , 2 , 4 , 6 , 12 , 24 , and 48 h respectively . patient was observed for numbness at the surgical site , need for rescue analgesia , complications ( nausea , vomiting ) . numbness in area of lower jaw line was present throughout the period but subsided after discontinuation of local anesthetic through epidural catheter . he was discharged on fourth post operative day with advice for follow up in o.p.d . mandibular nerve block can be used to manage intra as well as post operative pain in cases of fracture mandible . we performed lateral extra oral approach because of restricted mouth opening and the need to retain the catheter for post operative analgesia . 18 g cannula instead of epidural needle was used to minimize the bleeding from pterygoid plexus of veins . the cannula was advanced further after contacting lateral pterygiod plate so that the catheter comes in vicinity of mandibular nerve and there is no displacement during jaw movements . the catheter was further tunnelled subcutaneously to prevent dislodgement and filter was used to prevent infection . there was excellent post operative analgesia achieved with this technique as shown in decreased pain scores , both static as well as dynamic .
mandibular nerve block is often performed for diagnostic , therapeutic and anesthetic purposes for surgery involving mandibular region . advantages of a nerve block include excellent pain relief and avoidance of the side effects associated with the use of opiods or non - steroidal anti - inflammatory drug ( nsaids ) . a patient with maxillo facial trauma was scheduled for open reduction and internal fixation of right parasymphyseal mandibular fracture . the mandibular nerve was approached using the lateral extraoral approach with an 18-gauge i.v . cannula under general anesthesia . he received 4 ml boluses of 0.25% plain bupivacaine for intraoperative analgesia and 12 hourly for 48 h post operatively . vas scores remained less than 4 through out observation period . the only side effect was numbness of ipsilateral lower jaw line , which subsided after local anesthetic administration was discontinued . patient was discharged after four days .
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synchronous primary lung cancers are uncommon and the occurrence of synchronous non small cell lung carcinomas ( nsclc ) with different histological morphologies within the same lobe is rare . the aim of this report is to discuss surgical and oncological management of this entity together with a review of the current literature . a 61-year - old female smoker ( 42 pack years ) was found to have left upper zone shadowing on chest x ray following a history of weight loss . her performance status was 1 with a forced expiratory volume in one second ( fev1 ) of 1.94 litres . positron emission tomography in conjunction with computed tomography ( pet - ct ) showed two nodules in the left upper lobe . there was a 2 cm spiculated lesion in the anterior segment with a standardised uptake value ( suv ) max of 5.6 units ( fig 1a ) and a 2.2 cm cavitating lesion in the apico - posterior segment with a suv max of 2.7 units ( fig 1b ) . coronal view pet ct scan of a high uptake nodule in the left upper lobe coronal view of pet ct scan of moderate uptake synchronous nodule in the left upper lobe a left thoracotomy with upper lobectomy was performed and the intra - operative findings were consistent with the radiological appearances seen in the pet - ct scan . there was a 2 cm infiltrating squamous cell carcinoma in the anterior segment with vascular invasion ( pathological ( p ) t2a according to tnm 7th edition ) and a separate 2.4 cm adenocarcinoma with associated broncho - alveolar spread in the apical segment ( pt1b ) . an adjacent peribronchial lymph node was positive for tumour ( pn1 ) , however due to the poorly differentiated morphology of the tumour cells it was unclear as to which tumour it originated . cisplatin and vinorelbine adjuvant chemotherapy was administered with no overt complications ; however recurrence of the disease was discovered radiologically one year post - operatively in the form of a left hilar soft tissue mass with bony metastases . the patient subsequently received radiotherapy without complications but a staging ct chest / abdomen / pelvis performed three months later showed progressive disease with left lung collapse in addition to multiple , palpable subcutaneous nodules . these features were in keeping with the patient s symptoms of worsening breathlessness and wheeze . further investigations including bronchoscopy showed a tumour in left main bronchus which was treated with laser ablation and placement of a tracheo - bronchial stent . histological assessment of this nodule showed a poorly differentiated adenocarcinoma , which was confirmed with immunohistochemical techniques . specialist genetic analysis of the subcutaneous lesion was performed , which showed the presence of an egfr mutation sensitive to anti - egfr tyrosine kinase inhibitors . this enabled the oncologists to use monoclonal antibody therapies ( anti egfr tki ) and the patient was treated for one month with iressa ( gefitinib ) . she responded well with significant decrease in the size of the metastatic subcutaneous nodules . due to her progressive disease the patient deceased with an overall survival ( from the time of the lung resection ) of 20 months synchronous primary lung cancers ( splc ) were first described in 1924 by beyreuther h ( 1 ) . the true incidence of these remains uncertain but evidence has shown figures between 1% to 8% ( 2 ) . the criteria for the diagnosis of splc , which was proposed by martini and melamed in 1975 , are that synchronous tumours are physically distinct and that the histology is different surgical resection of splc was recommended by many of the reviewed authors ( 3 - 5 ) . rostad et al studied the outcome and characteristics of synchronous primary lung cancers . in 15,308 lung cancer resection cases , 94 patients were found to have synchronous non small cell lung cancers , nsclc , 9 patients had synchronous lung cancers with different histological morphologies and only 2 ( 0.01% ) patients had synchronous lung cancers with different histological morphologies within the same lobe . the relative survival rate in patients with different histological morphologies ( n=9 ) was 12.7% . patients with similar morphologies had a better outcome with a relative survival rate of 29.2% , although the difference was not statistically significant ( p=0.24 ) . the authors concluded that surgical resection should be offered to patients with synchronous lung cancers who are operable with respectable tumour . ( 3 ) lymph node metastases were found to be a statistically significant prognostic factor . in a study of 92 patients who had surgical resections for multiple synchronous primary lung cancers , the results showed the 5-year survival were 52.5% and 15.5% for patients without and with lymph node metastasis respectively ( p = 0.001 ) . ( 3 ) skin metastases from lung cancers are not uncommon , and their presence is a poor prognosis factor ( 6 ) . recently anti - egfr tki molecules have been introduced for the treatment of advanced malignancies including lung cancers , however long term treatment resistance remains a therapeutic challenge . our patient had a disease free survival of 12 months post surgery despite a positive lymph node , but she later developed recurrence with distant subcutaneous metastases and an endobronchial mass , which was treated palliatively . she received anti egfr1-tk inhibitors based on genetic analysis from the metastatic adenocarcinoma subcutaneous lesions and her initial response was good . patients with resectable synchronous primary non small cell lung carcinomas within the same lobe should be offered surgical resection after careful pre - operative staging . skin metastases should be biopsied and egfr testing should be requested in order to determine the originating the tumour and to assess the patients suitability for anti egfr - tki treatment .
we report the case of a sixty one year old female diagnosed with two synchronous primary lung cancers located within the same lobe . surgical resection was performed , followed by adjuvant chemotherapy . the patient developed distant bone and skin metastases one year post - surgical resection . in this report we discuss the multimodality therapy used to treat this patient .
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dr clark is the director of the centre for gambling research at ubc , which is supported by funding from the province of british columbia and the british columbia lottery corporation ( bclc ) . dr clark has provided paid consultancy to cambridge cognition ltd . on issues relating to neurocognitive assessment . dr clark has not received any other direct or indirect payments from the gambling industry or any other groups substantially funded by gambling to conduct research or to speak at conferences or events .
billieux et al . ( 2015 ) propose that the recent proliferation of behavioral addictions has been driven by deficiencies in the underlying research strategy . this commentary considers how pathological gambling ( now termed gambling disorder ) traversed these challenges to become the first recognized behavioral addiction in the dsm-5 . ironically , many similar issues continue to exist in research on gambling disorder , including question - marks over the validity of tolerance , heterogeneity in gambling motives , and the under - specification of neuroimaging biomarkers . nevertheless , i contend that the case for gambling disorder as a behavioral addiction has been bolstered by the existence of clear and consistent functional impairment ( primarily in the form of debt ) , coupled with the development of a public health approach that has given emphasis to product features ( i.e. the structural characteristics of gambling forms ) as much as individual dispositions ( the addictive personality ) .
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gastric volvulus , an entity seen in both pediatric and adult patients , occurs when the stomach twists upon itself . this event may be transient , producing few if any symptoms , or may lead to obstruction or even ischemia and necrosis . pare described the first case of gastric volvulus in 1579 in a patient with diaphragmatic injury from a sword wound . acute gastric volvulus in pediatric and adult patients has been reported but chronic organo - axial gastric volvulus with diaphragmatic eventration has not been reported . this report describes a rare case of gastric volvulus with a review of the literature . he had a feeling of fullness and discomfort in the upper abdomen for 2 months . twenty years ago , he was told that his chest radiography , performed due to routine survey for military service , was abnormal . but he had received no specific measures since then and he had been relatively healthy . a thorough review of symptoms was performed but did not disclose any respiratory or cardiovascular symptoms . on clinical examination , he appeared not acutely ill looking and his body temperature was 36c , blood pressure 120/70 mmhg , respiratory rate 24/min . auscultation of the chest showed diminished breathing sound at the left lung base and heart sound was normal . no abnormalities were observed in cbc , liver chemistry , serum amylase and stool examination . the chest radiograph showed the left hemidiaphragm to be located at an unusually high intercostal space with large air - fluid level ( figure 1 ) . upper gastrointestinal series demonstrated the typical appearance of an organo - axial volvulus of the stomach ( figure 2 ) . the barium - filled stomach was twisted on an axis from the pylorus to the esophagus . the patient refused surgical or endoscopic correction of the volvulus and has been followed in the outpatient clinic for more than 6 months without symptom aggravation . the stomach is relatively fixed at the esophageal hiatus and the pylorus by the four gastric ligaments . the gastrophrenic ligament and the retroperitoneal attachment of the second part of the duodenum provide the superior and inferior fixation . the gastrohepatic ligament tethers the lesser curve , the gastrocolic ligament connects the stomach to the transverse colon , and the gastrosplenic ligament tethers the greater curve . the clinical symptoms depend upon the extent or degree of rotation , obstruction and associated defect . borchard s triad of pain , violent retching and inability to pass a nasogastric tube6 should lead to a strong clinical suspicion of acute gastric volvulus . an acute volvulus is an emergency situation , with either obstruction or strangulation of the stomach and requires expeditious surgery . in comparison , this explains why the diagnosis is often delayed in the elderly or after complication has occurred . . gastro - esophageal reflux may give rise to epigastric pain , which is intermittent during the periods of engorgement or gastric emptying . gastric ulceration is caused by localized ischemia and acidity within the herniated stomach or mucosal congestion due to venous obstruction of the herniated stomach . angina - like pain and electrocardiographic abnormalities may make the differential diagnosis difficult in the elderly . the diagnosis is confirmed by the presence of a large , unusual gas - filled viscus in the chest or abdomen on plain radiographs . if necessary , a barium swallow study can define the anatomic changes more exactly . on barium examination , the characteristic findings are 1 ) esophagogastric junction lying lower than normal , 2 ) reversal of the greater and lesser curvatures , 3 ) pylorus pointing downward , 4 ) greater curvature crossing the esophagus , 5 ) two air - fluid levels and lowering of the gastric fundus . secondary gastric volvulus is more frequent than idiopathic volvulus , therefore the diagnosis of gastric volvulus can be made after a thorough search for possible causative factors . these conditions have been reported as follows ; para - esophageal hernia , traumatic rupture of the diaphragm , eventration of the diaphragm and phrenic nerve injury . in infants and children , 15 ( 33% ) among 46 patients with gastric volvulus had diaphragmatic eventration . organo - axial volvulus is commonly associated with diaphragmatic hernia and usually manifests as an acute event . diaphragmatic eventration is suggested when a part or all of the hemidiaphragm is located at an unusually high level in the thorax . it does not refer to a defect or hole in the diaphragm with discrete edges , but rather to a diffuse or localized bulging of the diaphragm itself . acquired lesions are usually related to phrenic nerve injury , which may be diverse in origin . radiological investigation combined with fluoroscopy of the diaphragm our patient showed no diaphragmatic movement during respiration , so he had complete left hemidiaphragmatic eventration . acute gastric volvulus and symptomatic chronic gastric volvulus require operative treatment . if the volvulus is secondary , definitive treatment must include correction of the associated abnormalities . this is done by advancing the endoscope just past the point of torsion , turning and locking the tip of the instrument , and rotating it 180 degrees . with rapid diagnosis and modern treatment , the rate of mortality from acute gastric volvulus is now about 15% to 20% . if the stomach is strangulated , the mortality rate of emergency surgery is 4060% . in recognized cases of chronic gastric volvulus
gastric volvulus occurs when the stomach rotates about its longitudinal axis ( organo - axial volvulus ) , or about an axis joining the lesser and greater curvatures ( mesentero - axial volvulus ) . primary gastric volvulus , making up one third of cases , occurs when the stabilizing ligaments are too lax as a result of congenital or acquired causes . secondary gastric volvulus , making up the remainder of cases , occurs in association with a paraesophageal hernia or other congenital or acquired diaphragmatic defects . while gastric volvulus may occur acutely , especially in children , it may not be clinically apparent and discovered incidentally . the authors present a case of chronic organo - axial volvulus of the stomach secondary to left hemidiaphragmatic eventration with a review of the relevant literature .
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dilatation of various lengths and severity of the common bile duct ( cbd ) , entitled choledochal cyst , has been detected in utero and usually presents with icterus in infancy , clinically mimicking biliary atresia and neonatal hepatitis1 ) . younger children and occasionally infants tend to present with painless jaundice , and older children present with recurrent abdominal pain , which was actually due to acute pancreatitis12 ) . postnatally , ultrasonography ( us ) is the initial diagnostic modality of choice , allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge . magnetic resonance cholangiopancreatography ( mrcp ) and endoscopic retrograde cholangiopancreatography ( ercp ) has superseded the use of computed tomography ( ct ) for preoperative anatomical delineation of the pancreaticobiliary tract . here we present the biggest choledochal cyst reported in infancy in the literature to our knowledge . a term female baby was born by normal delivery route after consanguineous marriage , at house , in syria as the 6th child of her parents . this family was living the civil war in syria at that time . during 4 months period abdominal distension had increased . because of restlessness and growing abdominal distension but as a result of investigations they were sent home because of normal laboratory results . after 15 days they admitted to hospital because of jaundice and abdominal cyst she had referred to our hospital ( in turkey ) with the preliminary diagnosis of abdomianl cyst and hepatitis developed due to compression of cyst . on her admission , us revealed giant abdominal cyst with thin wall and liquid - debris level extending from right upper quadrant to pelvic region . it was suspected that bile duct 's dilatation was due to abdominal giant cyst 's pressure . she was operated with the differential diagnosis of duplication , omental or mesenteric cyst . at operation , a giant type 1a choledochal cyst , 160 mm in diameter , was surprisingly detected ( fig . serum levels of bilirubin decreased sharply and the patient was discharged without any problems on the tenth postoperative day . a choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts2 ) . type ia is a cystic dilation of the cbd ; type ib is a focal segmental dilation of the distal cbd ; type ic is a fusiform dilation of both the common hepatic duct and cbd . in type ii , the cyst forms a diverticulum from the extrahepatic bile duct . type iii , also known as choledochocele , is a dilation of the distal cbd lying mainly within the duodenal wall . type iv is essentially type i anatomy with either intrahepatic bile duct cyst ( iva ) or choledochocele ( ivb ) . some authors refer to caroli 's disease with multiple cystic dilations of the intrahepatic biliary tree as type v23 ) . in our case there was a cystic dilation of the cbd as a type ia choledochal cyst with minimal dilatation of intrahepatic bile ducts . this malformation primarily affects girls ( 4:1 ) and about 80% become symptomatic during childhood . choledochal cysts remain relatively uncommon in western europe and the united states , although they are appreciably more common in asia . obstructive jaundice is the main presentation symptom in children , but abdominal pain is the commonest symptom in adults . the classical triad of pain , jaundice and a palpable mass is uncommon , occurring in no more than 6% in one uk series4 ) . the complications of congenital cystic dilatation of the bile duct are biliary stone formation , progressive biliary cirrhosis with portal hypertension , and carcinoma . fusiform lesions are never large enough to be palpable while multiple intrahepatic type 4 lesions cause predisposition to stone formation and sepsis . a wide variety of imaging techniques are available which noninvasively reconstruct biliary anatomy and give an excellent idea of biliary function . although us is the first described imaging method to determine the cbd cyst , ct , mrcp , and ercp are superior to assess the extention of the cyst and associated pathologies such as cholangitis , pancreatitis and pancreaticobiliary junction anomaly . in our case , we used us and ct to identify the abdominal mass ' origin . because of the diameter of the cyst , cbd cyst was not thought in the differential diagnosis . cbd malformations should be kept in mind as a differential diagnosis of the cystic mass regardless of size , and patient 's age . surgery is the main choice of treatment but some centres in south american and asia have reported ercp and sphincterotomy alone as definitive treatment for mild fusiform dilatation although their long - term prognosis is not known3 ) . at operation , it was difficult to make the differential diagnosis of our giant cyst . it was important to make the dissection of the cyst carefully in order to avoid the iatrogenic injury . following the diagnosis of choledochal cyst was confirmed , hepaticojejunostomy was easier depending on the wide common hepatic duct of our huge cyst . tang et al.5 ) reported a study that involves 62 children ( average age of 2.3 years ) who had cysts with the average diameter of 42 mm ( range , 12158 mm ) . there is no information about the child'a age with the 158-mm cyst . as our knowledge we report the biggest choledocal cyst case in infancy in the literature . in conclusion , cbd malformations should be kept in mind as a differential diagnosis at the cystic mass regardless of cyst 's size , and patient 's age , especially in children presented with abdominal pain , jaundice , and palpable mass .
choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts . postnatally , ultrasonography is the initial diagnostic modality of choice , allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge . symptoms depend on the age at presentation . common bile duct malformations should be considered as a differential diagnosis of a cystic mass regardless of the cyst 's size or patient 's age , especially in children presenting with abdominal pain , jaundice , and palpable mass . to the best of our knowledge , we report the largest choledochal cyst in infancy .
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bilateral shoulder dislocation are most commonly posterior type . these are most commonly due to seizure disorder and electrocution . to best of our knowledge there are only few cases of similar kind are reported in literature . we hereby report a interesting case of posttraumatic , bilateral anterior dislocation of shoulder without associated fracture in a 45 old women without any predisposing pathoanatomy . a 45-year - old women presented to casualty with sudden onset of pain and restriction of movement in both shoulders fallowing trauma . immediately post trauma she had severe pain and restriction of both shoulders . on examination arms were abducted and externally rotated . there was loss of round contour of shoulder with increased vertical diameter of axilla anteriorly . bilateral traumatic anterior shoulder dislocations are rare and are seen as a result of unique mechanism of injury . in our case patient bilateral shoulder dislocations are usually posterior type and are almost pathognomonic of seizure disorder or electrocution . though anterior dislocation of shoulder is commonest bilateral simultaneous dislocation is very rare[1 - 7 ] . we hereby report a case of posttraumatic , bilateral anterior dislocation of shoulder without associated fracture in a 45 old women . a 45-year - old women presented to lok nayak hospital , new delhi , india in august 2010 with sudden onset of pain and restriction of movement in both shoulders fallowing trauma . she had no history of seizure , epilepsy , previous shoulder dislocation or instability in other joints . on examination arms . there was loss of round contour of shoulder with increased vertical diameter of axilla anteriorly . radiological examination revealed bilateral anterior dislocation of the shoulders without any associated fractures(figure 2 ) . radiograph showing bilateral shoulder dislocation concentric reduction of bilateral shoulder joint achieved closed reduction done by milch technique after intraraticular lignocaine injection . majority of the bilateral shoulder dislocations are of posterior type most commonly seen during convulsion , electric shock or hypoglycaemic seizures . posterior type is common in these conditions due to violent contractions of the muscles of the shoulder girdle [ 8 - 10 ] . unlike bilateral occurrence of anterior shoulder dislocation is rare because of the fact that one extremity takes the brunt of the impact . to best of our knowledge only three cases of bilateral anterior dislocations are reported in literature . in two of the three cases reported were sequential , one sided followed by contra lateral side dislocation . in our case impact is same on both shoulders at the same time . the mechanism of anterior dislocation is forced extension , abduction and external rotation of the arm . in our case mechanism of injury mechanism of injury , systemic disease and associated fractures in various similar cases is depicted in table 1 . croswell and smith reported a case of bilateral anterior dislocation of the shoulder without any fractures in a bench - pressing athlete . in an unusual mechanism of injury weight on the bar forced his arms into hyperextension in the mid - abducted position . the humeral shaft gradually pivoted on the bench and the humeral heads were slowly dislocated interiorly by the weight of the bar . sandeep s and sudhir k reported a case of sequential bilateral anterior dislocation in which the left shoulder dislocated first due to trauma followed by atraumatic dislocation of the right shoulder . sreesobh k v et al reported a case where atraumatic right shoulder dislocation was followed by traumatic dislocation of the left . this type of dislocation involves a unique type of mechanism injury and in our case it was fall on pointed elbow causing forced extension . traumatic bilateral anterior dislocations without any pathologic lesion are very rare with only few cases reported in literature .
introduction : bilateral shoulder dislocation are most commonly posterior type . these are most commonly due to seizure disorder and electrocution . anterior shoulder dislocations occurring bilaterally without any predisposing factors are very rare . these types of injuries are due to trauma with a unique mechanism of injury . to best of our knowledge there are only few cases of similar kind are reported in literature . we hereby report a interesting case of posttraumatic , bilateral anterior dislocation of shoulder without associated fracture in a 45 old women without any predisposing pathoanatomy.case report : a 45-year - old women presented to casualty with sudden onset of pain and restriction of movement in both shoulders fallowing trauma . immediately post trauma she had severe pain and restriction of both shoulders . on examination arms were abducted and externally rotated . bilateral shoulder movements were painful and restricted . there was loss of round contour of shoulder with increased vertical diameter of axilla anteriorly . radiological examination revealed bilateral anterior dislocation of the shoulders without any associated fractures . closed reduction done by milch technique after intraraticular lignocaine injection . mri of bilateral shoulder showed no pathological lesion . both shoulders were immobilized with a shoulder immobilizer for three weeks.conclusion:most of the bilateral shoulder dislocations are posterior type seen in seizure disorders . bilateral traumatic anterior shoulder dislocations are rare and are seen as a result of unique mechanism of injury . in our case patient had a fall on her elbows causing forced extension . if diagnosed and treated promptly completely normal function of the shoulders can be restored .
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maculopapular exanthema ( mpe ) is the most frequent clinical manifestation of nonimmediate allergic reactions due to drugs and t helper 1 ( th1 ) cytokines and cd4 ( + ) t cells have been shown to play an important role in its pathogenesis . pyrazinamide is used in the management of tuberculosis ( tb ) in combination with other drugs . the common side effects due to pyrazinamide are hyperuricemia ( gout ) , hepatotoxicity , nausea , vomiting , flushing , dysuria , arthralgia , and sideroblastic anemia . we hereby report a case of maculopapular rash due to pyrazinamide in a patient undergoing antitubercular treatment . we also established the causality , severity , and preventability of the suspected adverse drug reactions ( adrs ) . a 19-year - old patient of bersarai area , new delhi , belonging to a lower middle class family , visited a microscopic center situated in delhi government dispensary of bersarai area with complaints of cough with expectoration and fever , loss of appetite , and weight for the past 1 month . the sputa were examined as per revised national tuberculosis control programme ( rntcp ) by zeihl - neelson ( zn ) staining for acid - fast bacillus ( afb ) and were found to be negative . chest radiograph showed bilateral upper zone infiltration . on the basis of the above clinical examination and observation he was referred to the directly observed treatment ( dot ) center of his area for the initiation of category i antituberculosis therapy as per national rntcp guidelines according to his weight ( 40 kg ) . category i antituberculosis therapy includes isoniazid 600 mg ( 2 tablets ) , rifampicin 450 mg ( 1 capsule ) , pyrazinamide 1500 mg ( 2 tablets ) , and ethambutol 1200 mg ( 2 tablets ) . ( after the second day of therapy ) , the patient visited dot center with generalized maculopapular rashes all over the body and more on both shoulders and upper and lower limbs . a diagnosis of antitubercular drug - induced maculopapular rash was made by the medical officer . rashes were round in shape , raised from the body surface , appeared reddish in color , and hot on touch . therefore , pyrazinamide was stopped on the advice of the medical officer and other antituberculosis medicines were continued with the addition of oral antihistaminic and the patient was kept under close observation for evaluation . he tolerated isoniazid , rifampicin , and ethambutol but on inclusion of pyrazinamide , rashes reappeared in the same part of the body . he is on regular follow - up with disappearance of rashes and signs and symptoms of tuberculosis . we carried out the causality assessments as per the naranjo algorithm and preventability and severity assessments as per the hartwig scale . the causality assessment revealed a probable association ( naranjo score 7 ) between the adr and pyrazinamide . higher rates were found in elderly patients who are likely to be receiving multiple medications for long - term illnesses . maculopapular rashes consist of macules ( distinct flat areas ) and papules ( raised lesions ) . the rash is usually bright red in color and the skin may feel hot with burning sensation or itch . the whole of the skin surface may be involved , though the face is often spared . up to 5% of the patients receiving penicillin , sulfonamides , phenytoin , or gold , erythema multiforme has been reported in one patient following pyrazinamide administration for cutaneous tuberculosis related to a pleural fistula . daily antituberculosis treatment ( att ) was initiated in this patient with isoniazid , rifampicin , ethambutol , and pyrazinamide . after 26 days of therapy , maculopapular erythematous lesions appeared , and biopsy results confirmed the diagnosis of erythema multiforme . the rash disappeared with the discontinuation of all drugs , but reappeared when rifampicin and pyrazinamide were reintroduced 5 days later . the patient developed the rash on the third day ( after second dosing day of therapy ) after initiating antituberculosis therapy and disappeared after few days when the drug ( pyrazinamide ) was stopped . the causal relationship between the drug and the adr was found to be probable . generalized maculopapular skin rash was a common adr reported in an investigational trial of ofloxacin ( 800 mg a day ) and pyrazinamide ( 1500 mg a day ) . the management of such reactions needed withdrawal of the suspected drug and management of symptoms , if any . in this study , the suspected drug was stopped immediately following the adr and antihistamines were added to manage associated itching due to drug reaction , to which patient responded well . the severity assessment revealed the adr to be moderate ( level 3 ) , suggesting that the suspected drug should be withheld , discontinued , otherwise changed , and/or on antidote or other treatment is required . since this patient did not have any past history of skin reaction due to pyrazinamide or any other drugs , therefore this reaction was unpreventable . since pyrazinamide is a common drug used in tb management , and tb is also a common problem in countries like india , the dermatological manifestations due to pyrazinamide gain attention . upon occurrence of dermatological manifestations , the patients may become noncompliant , which is one of the common causes with other anti - tb drugs for treatment failure in tb therapy . although skin reactions due to pyrazinamide are not well reported , one should be suspicious of maculopapular rashes due to pyrazinamide also . upon occurrence , the suspected drug/(s ) should be stopped immediately and the patient should be managed symptomatically . the patients undergoing treatment on an outpatient basis should be counseled for the early recognition of dermatological manifestations .
pyrazinamide is a commonly used first - line antitubercular drug . gastric - related adverse drug reactions are common with pyrazinamide . dermatological manifestations due to pyrazinamide are rare . this study aimed find out the dermatological manifestations / adverse drug reaction ( adr ) due to pyrazinamide . we reported a case of maculopapular rash caused by pyrazinamide in a patient on antituberculosis treatment using structured questionnaires . the patient developed maculopapular rashes on receiving combination antituberculosis treatment . the rashes disappeared after stopping the suspected drug . the patient was rechallenged with pyrazinamide , which led to reappearance of a similar type of rash . the causality , preventability , and severity were assessed using the naranjo algorithm and hartwig scale . since pyrazinamide is a commonly used drug in tuberculosis and which is a common infectious disease in developing countries , with the similar reports , we can predict early case detection and can prevent the occurrence of similar reactions in future .
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primary lymphoma of the parotid gland is an uncommon entity . predominantly ( 84 - 97% ) parotid lymphomas are non - hodgkin lymphomas ( nhl ) and most of them are of b - cell origin . there may be coexisting lymphadenopathy of the cervical region . however , to categorize them as a primary from the parotid , the first clinical manifestation should arise in the parotid gland . we report a case of primary nhl of the parotid gland in a 61-year - old male that was diagnosed on fine needle aspiration cytology ( fnac ) with further histological confirmation . a 61-year - old male presented with a painless swelling in the right parotid region for the last 1 year and right sided cervical swelling for the last 3 months . on examination , a mass of 5 cm 5 cm was identified in the parotid region . in addition , the patient had an enlarged right sided level v cervical lymph node . hematological investigations were within normal limits ( hemoglobin 11.8 gm / dl , total leukocyte count 6000/cumm and a differential count of 66% neutrophils , 28% lymphocytes , 4% eosinophils and 2% monocytes , platelet count 1.6 lakhs / cumm ) . his erythrocyte sedimentation rate was elevated with 58 mm at the end of the first hour . contrast - enhanced computed tomography ( cect ) revealed a lobulated mass of 7 cm 5 cm 2.5 cm arising from the deep lobe of the parotid . fnac was advised from both the parotid swelling and the enlarged right sided level v cervical lymph node . smears were moderately cellular and comprised of large atypical lymphoid cells with high n : c ratio , irregular nuclear contour , vesicular chromatin , and prominent nucleoli and scanty agranular cytoplasm . the background showed lymphoglandular bodies alongwith normal - appearing salivary gland acini and ducts [ figure 1a ] . subsequently , immunocytochemistry ( icc ) was performed on the papanicolaou - stained smears without destaining . these cells were positive for leukocyte common antigen ( lca ) , cluster of differentiation ( cd ) 20 ( inset ) and negative for cd3 and cytokeratin . in view of the icc findings , a diagnosis of high grade nhl favoring diffuse large b - cell lymphoma ( dlbcl ) was offered . ( a ) fnac of the parotid mass shows moderately cellular smears comprising of large atypical cells with scant cytoplasm , irregular nuclei , vesicular chromatin , and prominent nucleoli . background shows normalappearing salivary gland acini and ducts ( arrow ) ( mgg , 400 ) . inset : b - cell marker ( cd20 ) positivity of the tumor cells ( icc , 100 ) ( b ) cervical lymph node fnac shows similar cytomorphology ( mgg , 100 ) fnac from the cervical lymph node showed similar cytomorphology [ figure 1b ] . in view of the above findings , a diagnosis of high grade b - cell nhl favoring dlbcl involving the right parotid gland with secondary involvement of the level v cervical lymph node was made . excision biopsy of the cervical lymph node revealed diffuse effacement of the architecture with sheets of atypical lymphoid cells with vesicular chromatin and scanty cytoplasm . based on the above histological and ihc findings , a diagnosis of dlbcl was made . correlating with the clinical history and radiological findings , final diagnosis of a primary dlbcl of the right parotid gland with secondary involvement of level v cervical lymph node was made . the patient was treated with six cycles of rituximab - cyclophosphomide - hydroxydoxorubicin - oncovin - prednisolone ( r - chop ) chemotherapy . malignant lymphoma of the parotid gland is relatively rare and constitutes about 4 - 5% of extranodal lymphomas , and 1 - 4% of all parotid tumors . facial nerve paresis and associated cervical lymphadenopathy may be a feature as well . in the present case they may be associated with autoimmune diseases such as sjgren 's syndrome . in the present case , the lymphoma may originate from the intraparotid lymph nodes or from the parenchyma ( mucosa - associated lymphoid tissue [ malt ] ) or both . in view of this , lymphoma primarily affecting the parotid gland to refer to lymphoma affecting the parotid region . the differentials of malt lymphoma are lymphoepithelial sialadenitis ( lesa ) and warthin 's tumor . the cytology of lesa reveals a mixture of acinar cells , epithelial , and myoepithelial cells admixed with polymorphous lymphoid cell population . warthin 's tumor is considered a benign primary parotid gland neoplasm that is composed of a mixture of oncocytic cells , basal cells , and stroma that contains numerous lymphocytes usually arranged in papillary and cystic structures . by contrast , the diagnosis of non - malt high grade lymphomas is usually straightforward , as they have overt cytological atypia . the criteria for primary parotid lymphoma , as suggested by hyman and wolff , include the first clinical manifestation in the parotid gland , histologically involving the parotid gland parenchyma and malignant nature of the lymphoid infiltrate . in the present case , the parotid swelling developed earlier than the cervical lymph node and the parenchyma was infiltrated by malignant lymphoid cells , meeting all the criteria as suggested by hyman and wolf for primary lymphoma of the parotid . lymphoma of the salivary gland is highly chemo - radiosensitive and , therefore , a timely diagnosis is important . moreover , surgical management of parotid lymphoma patients carries a high risk of morbidity due to infiltrative nature of the neoplasm . the role of fnac thus becomes important in such a setting for an early definitive diagnosis .
primary lymphoma of the parotid gland is relatively rare and constitutes about 4 - 5% of extranodal lymphomas . the majority of them is non - hodgkin lymphoma ( nhl ) and is b cell in nature . we report a case of primary diffuse large b - cell lymphoma ( dlbcl ) of the parotid gland in an elderly male . the case was diagnosed on fine needle aspiration cytology ( fnac ) of the right parotid gland as high grade b - cell nhl and confirmed on histopathology as dlbcl . in correlation with the clinicoradiological findings , the case was diagnosed as primary parotid dlbcl . the case highlights the role of fnac as a timely and useful diagnostic tool .
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a 53-year - old caucasian male was evaluated for bright red blood per rectum with colonoscopy . the patient 's past medical history was significant for end - stage liver disease due to hepatitis c and hepatitis b , asthma , and erythropoietic porphyria . the patient had prior episodes of hematemesis which required multiple sessions of esophageal variceal rubber band ligation treatment . the patient complained of bright red blood per rectum for a four month period without clinical evidence of active or massive bleeding . family history was notable for colon cancer in the patient 's father at age 60 . his medications included propanolol , lasix , aldactone , lactulose , thiamine , and folate . pertinent review of symptoms was negative for fevers , chills , weight loss , diarrhea , constipation , melena , weakness , or loss of consciousness . physical examination revealed a well - developed , well - nourished male in no acute distress appearing his stated age . vitals signs were as follows : blood pressure 108/58 mm hg , heart rate 66/min , respiratory rate 18/min , and temperature 36.9c . in addition , abdominal examination revealed a 2 2 cm umbilical hernia and a right - sided inguinal hernia . recent complete blood count prior to colonoscopy revealed a hemoglobin of 11.5 g / dl , hematocrit of 34.6% , and a platelet count of 36,000 with a mcv of 81.3 . the patient had positive serologies proving both infection with hepatitis b and c. the patient 's hepatitis c viral load was undetectable . recent abdominal ultrasound was notable for hepatomegaly , nodular and heterogeneous in consistency , massive splenomegaly , and ascites . colonoscopy was significant for internal hemorrhoids as well as cecal and proximal ascending colon varices without any endoscopic evidence of bleeding ( fig . reported cases of varices have been found in the stomach , small bowel , biliary tree , colon , rectum , and even the site of a surgical ostomy . the stomach and the rectum are the most common sites of extraesophageal varices and up to 30% of variceal bleeding may originate from an extraesophageal location . a history of abdominal surgery may predispose a patient to develop varices of the lower gastrointestinal tract [ 5 , 6 ] . other causes include biliary atresia , biliary sclerosis , congestive heart failure , superior mesenteric vein thrombosis , and inferior mesenteric vein thrombosis . a smaller number are of idiopathic origin . due to the patient 's history of esophageal varices as a result of portal hypertensive gastropathy this case of cecal and ascending colon varices is presented because of the uniqueness of this anomaly within the colon and most notably the cecum . in the literature , a total of seventeen cases ( including this one ) of cecal varices have been reported [ 2 , 5 , 6 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ] . the cases of cecal varices that have been reported have all presented clinically with massive lower gastrointestinal bleeding necessitating urgent management with blood products and surgical or endoscopic intervention . to our knowledge this is the first case of cecal varices reported that has not resulted in massive lower gastrointestinal bleeding . he did not demonstrate any episodes of massive gastrointestinal bleeding requiring immediate intervention as in the previous cases reported . given the colonoscopic finding , we believe that the internal hemorrhoids and not the cecal varices may have contributed to his clinical presentation . by reporting our experience we emphasize that cecal varices are very rare and may also be present in stable patients without massive lower gastrointestinal bleeding . because of the paucity of data , the proper management of patients with colonic varices is unknown . treatment of cecal varices has included colectomy as well as transjugular portal - systemic shunt in cases of varices attributed to portal hypertension . there are no established guidelines for the treatment of extraesophageal varices due to the scarcity of cases reported . he is clinically stable and has no clinical evidence indicating a history of active bleeding from those varices . however , further study is needed to determine whether sclerotherapy , band ligation , or even surgical treatment is effective for bleeding associated with cecal varices . careful patient follow - up should also be instituted for those who are incidentally noted to have cecal varices but have not bled .
since the original description of colonic varices in 1954 [ n engl j med 1954;250:434 - 438 ] , fewer than 100 cases have been reported in the literature . particularly , even fewer cases of cecal varices have been reported . more than 75% of these cases have been due to portal hypertension . our objective is to contribute a rare case with an uncommon presentation to the medical literature . we present the case of a 53-year - old male with hepatitis c and hepatitis b liver cirrhosis who presented for outpatient colonoscopy . the indication for colonoscopy was bright red blood per rectum and iron deficiency anemia . a significant amount of varices were noted in the cecum and proximal ascending colon . no endoscopic evidence of colonic bleeding was noted . this is the first reported case of cecal varices not presenting with massive lower gastrointestinal bleeding .
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twenty - eight post mortem lower eyelids ( 14 right , 14 left ) from 16 japanese cadavers were examined and divided into 2 groups . one group of 14 lower eyelids of 7 cadavers ( 7 right , 7 left ; 3 males , 4 females ) with an average age at death of 80.4 years ( range : 73~97 ) , were sagittally incised from the lower lacrimal punctum ( figure 1 ) . the other 14 lower eyelids of 9 cadavers ( 7 right , 7 left ; 4 males , 5 females ) with an average age at death of 76.3 years ( range : 69~95 ) were incised from the lower lacrimal punctum with 5 degrees lateral inclination to the sagittal plane ( figure 1 ) . all cadavers were registered in the cadaveric service of aichi medical university , with proper consent from our institutional review board and approval to use them for educational purposes and studies . all methods for securing human tissue were humane and complied with the tenets of the declaration of helsinki . in the first group , which underwent a strict sagittal incision , 10 canaliculi of 7 cadavers ( 6 right , 4 left ; male 5 eyelids , female 5 eyelids ) were interrupted at the halfway point of the vertical portion and so the distal part of the canaliculi were not included ( figures 2a , b ) . however , 4 canaliculi of 4 cadavers ( 1 right , 3 left ; male 1 eyelids , female 3 eyelids ) did include the whole length of the vertical portion . in the second group with 5 degrees laterally inclined incision , all specimens included the whole length of the vertical portion ( figures 3a , b ) . most vertical portions of the lower lacrimal canaliculus examined here demonstrated a laterally inclined course of approximately 5 degrees ( figure 4 ) . the course of the laterally directed vertical portion of the lacrimal canaliculus should be considered not only in lacrimal probing , syringing , and trephining but also in eyelid surgeries . the 5 degrees lateral inclination of the vertical portion significantly emphasizes the need of a laterally directed insertion and lateral lid traction during the passage of a lacrimal probe , syringing needle or trephine to accord with the true course of the vertical portion . as well , since the vertical portion takes its lateral course within 1 mm laterally from the lacrimal punctum ( whitnall 1979 ; hwang et al 2005 ) , surgeons , during eyelid surgery , need to recognize the area within 1 mm laterally from the punctum as being a zone with a potential for serious lacrimal canalicular injury . the vertical portion changes its direction medially because of the inward eyelid movement ( kakizaki et al 2005 ) caused by the contraction of horner s muscle . at this time , the closure of the vertical portion may become insufficient , because the relative angle of the muscle of riolan and the vertical portion of the lacrimal canaliculus becomes milder . to maintain the relative perpendicular angle to each other , although all the cadavers were in an eye - closed state , and the muscle tonus of the orbicularis oculi had been completely lost , this state would have been similar to the eye - opening state in live humans whose muscle tonus of the orbicularis oculi is little . as the lacrimal probing syringing and trephining is usually performed in eye - opening state , the present result is reasonable for the true clinical situations . first , as the study samples were all taken from elderly cadavers , we may not be able to apply the results to younger generations . confirming our results in a younger patient population through clinical procedures such as lacrimal probing is possible . our experience is that the lateral inclination of a probe usually enables easy lacrimal probing , even in young generations . second , as we did not examine samples of caucasians or blacks , we can not conclude that they have the same inclination . however , to date , racial variations have not been reported with regard to the lacrimal drainage system ; therefore , we may be able to speculate that they have the same inclination . in conclusion , most vertical portions of the lower lacrimal canaliculus are directed laterally at approximately 5 degrees . surgeons need to recognize the true course so as not to cause injury to the vertical portion of the lower lacrimal canaliculus . this anatomical view will be helpful for a better understanding of the lacrimal drainage system .
the nomenclature of each part of the lacrimal canaliculus , for example the vertical portion , does not always reflect the true course . since we have sometimes observed findings suggesting the so called vertical portion of the lower lacrimal canaliculus inclined laterally , we re - examined the course of the vertical portion . twenty - eight postmortem lower eyelids in 16 japanese were examined and divided into 2 groups . the first group was 14 lower eyelids of 7 cadavers . eyelids were incised sagittally from the lower lacrimal punctum . the second group was 14 lower eyelids of 9 cadavers ; these were incised from the lower lacrimal punctum with 5 degrees lateral inclination to the sagittal plane . in the first group , 10 canaliculi of 7 cadavers were interrupted at the halfway point of the vertical portion . four canaliculi of 4 cadavers included the whole length of the vertical portion . in the second group , all specimens included the whole length of the vertical portion . most vertical portions of the lower lacrimal canaliculus demonstrated a laterally inclined course of approximately 5 degrees , although some took a completely vertical course .
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isolated nccm was first described in 1984 , but it only regained recognition in the last decade . now , it increasingly attracts scientific attention , especially because the condition has not been fully understood so far and is thus a subject of ongoing investigations on its pathology , development , clinical course , and therapy ( 1 ) . nccm is a primary genetic cardiomyopathy , caused by a defect in endomyocardial morphogenesis . as a result , the muscle of the ventricle is built out of trabeculae with intratrabecular recesses , giving a typical appearance of muscular bands in a spongy mashwork. this specific morphology is reminiscent of the myocardium during early embryogenesis . between the 4th and 18th week of cardiogenesis , a non - compacted structure is essential for the nutrition of the cells , since the muscle is being supplied primarily by diffusion of blood that flows in the intratrabecular spaces . when the angiogenesis of coronary arteries is not accompanied by a simultaneous regression of trabeculae , nccm is occurring ( 2 ) . mostly , they encode for sarcomere and cytoskeleton proteins , such as tafazzin ( taz ) , lim domain binding protein 3 ( ldb3 ) , -dystrobrevin ( dtna ) , lamin a / c ( lmna ) , etc . the exact prevalence is not known and it is assumed to bear a significant number of undiagnosed cases . in echocardiography , it is found in 1 of 2,000 studies . since the diagnosis is defined by structural features , which can only be evaluated by cardiac imaging , mostly transthoracic echocardiography . a diagnosis can be made in the presence of three factors : 1 ) a thickened left ventricular wall consisting of two layers with a maximum ratio of non - compacted to compacted myocardium > 2:1 at end - systole in pax , 2 ) color doppler evidence of flow within the deep intertrabecular recesses , 3 ) prominent trabecular meshwork in the lv apex or midventricular segments of the inferior and lateral wall ( 3 ) . various criteria systems have been described ; however , they all disembogue in the structural features and quantifications . cardiac mri is an advanced option for gaining a closer motion insight of the myocardium . it is especially useful if the morphology is overlapped with other cardiopathies ( 4 ) , due to its enhanced spatial resolution , improved tissue characterization , and lack of ionizing radiation ( 5 ) . the disease may remain silent along the entire life or appear unspecifically , depending to what extent the heart function is affected : dyspnea , fatigue , limbs edema , limited physical capacity , and exercise intolerance . furthermore , tachycardia has recently been found associated with nccm , leading to hypotension . syncopies have not been mentioned so far in possible signs of the disease but are plausible since it is a result of cerebral hypoperfusion . in advanced stages , the prognosis is difficult to predict and has to be individualized , but it can be geared to the resulting cardiac impairment grade . since there is no specific treatment so far , the essential approach is the early recognition of any cardiac dysfunction , prevention of complications and symptom - based therapy , including ace inhibitors , beta - blockers , and aspirin . more aggressive treatments such as surgical interventions have been reported in severe nccm cases ( 6 ) . in our case , the symptoms were still limited to situations where our patient is physically challenged . thus , she was advised to forego intensive sports and advised to have regular check - ups with a cardiologist ( including loop recording and tte ) . patient 's children have been advised to undergo a cardiologic check - up as well . its molecular genetic basis is not yet fully clear , and the same is true of its diagnosis , treatment , and prognosis . the author has not received any funding or benefits from industry or elsewhere to conduct this study .
a 41-year - old female was brought to the er after a presyncope and absence episode while riding a bike . she recalled no prodromi . her physical and neurological examinations were unremarkable ; past medical , family , substance , and travel history bland . she reported a constant tiredness , sporadic slight cephalgia , responsive to acetaminophen , and a recent syncope while jogging , resulting in a fall , circumstances of which she could not recall . non - compaction cardiomyopathy is a type of cardiomyopathy that was first described 25 years ago . its molecular genetic basis is not yet fully clear , and the same is true of its diagnosis , treatment , and prognosis . further study of these matters is needed .
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cardiac perforation is a dreaded complication of transvenous pacemaker , cardiac resynchronization therapy defibrillator ( crt - d ) , and implantable cardioverter - defibrillator ( icd ) lead placement because of the potential for critical morbidity and mortality . we report the case of a 45-year - old woman affected by severe dilated cardiomyopathy who was admitted to our cardiology department for an upgrade of a previously implanted pacemaker . the patient received a vvi pacemaker implant for complete atrioventricular block 22 years earlier and an upgrade ( ddd mode ) 11 years later . leads were inserted through the left subclavian vein : ( a ) an active fixation double catheter in the right ventricle , ( b ) a bipolar active fixation lead in the right auricle ( fig . 1 ) , and ( c ) a bipolar lead introduced from the coronary sinus into the antero - lateral vein . the previously implanted pacemaker and one atrial lead were removed ( the old unipolar lead was left in the right ventricle ) and a crt device was connected to the new implanted leads . after satisfactory r - wave sensing ( > 5 mv ) and pacing thresholds ( < 1.0 v at 0.5-ms pulse width ) had been demonstrated , the patient underwent defibrillation threshold testing ( dft ) to ensure proper device function . sustained ventricular tachycardia and ventricular fibrillation were induced to make certain that the device was able to constantly sense , detect , and terminate arrhythmias with a shock at 25 j. during skin closure , the patient went into cardiac arrest with pulseless electrical activity . cardiopulmonary resuscitation maneuvers were performed immediately and echocardiography showed intrapericardial effusion that was partially drained . after 1 h , pulseless electrical activity persisted , the patient was declared dead , and resuscitation attempts were halted . the unexpected fatal outcome resulted in an allegation of medical negligence against the operating cardiologists . a board of physicians consisting of a forensic doctor and a heart surgeon conducted a post - mortem examination and analysis of the medical records . at autopsy , approximately 180 cm of blood and clots were found inside the pericardial cavity . careful inspection of the aorta showed a deep lesion , 2 mm in length , on the antero - lateral aspect of the vessel ( fig . 1 ) 2 cm above the valvular plane ( fig . the lead metal extremity was implanted at the base of the auricle on the medial wall . the right atrium was very thin , with an average thickness of 2 mm . histology showed abundant adipose tissue in its context , endocardial fibrosis , and numerous foci of inflammatory infiltration by various elements including lymphocytes and monocytes as well numerous plasma cells and eosinophils , clearly indicating myocarditis that was not recent . the ascending aorta was incised and approximately 2 cm above the noncoronary cusp , a pinpoint injury related to the lesion described on the aortic adventia ( fig . the pathogenetic mechanism underlying this patients death can be explained in the following manner : the active lead ( fig . 1 ) , positioned in the right atrium medial aspect , perforated the thin atrial wall and the lateral aspect of the aortic wall . after aortic perforation , the lead initially remained inside its wall , which explains why the device interrogation revealed satisfactory r - wave sensing ( > 5 mv ) and pacing thresholds ( < 1.0 v at 0.5-ms pulse width ) . the patient eventually underwent dft to guarantee proper device function . at this point , the active lead , positioned inside the aorta like a cork , slipped out , causing cardiac tamponade . in a review of relevant literature , we identified only five cases , , , , of atrial damage by a pacemaker lead with concomitant aortic wall perforation , as shown in table 1 . however , not all reports indicate whether the lead responsible for the cardiac laceration was active or passive . a board of physicians conducted a post - mortem examination and their findings did not support a direct allegation of medical negligence against the operating cardiologists who performed the icd implant . the board analyzed the guidelines for cardiac pacing and cardiac resynchronization therapy and considered the indications for the procedure performed on the patient to be correct . the increased heart size shifted the right atrial appendage closer to the adjacent lateral aspect of the aorta . at the same time , the remarkable thinness of the right atrium was conducive to wall perforation . the perforation was not necessarily a result of malpractice in electrode positioning , but was also caused by other factors such as the pressure exerted during cardiac contraction . moreover , since she was bearing a pacemaker , the patient could not undergo magnetic resonance imaging before the procedure to highlight the thicknesses of the cardiac wall . in addition , her pre - existing myocarditis undoubtedly reduced the chances of successful resuscitation . antonino m. grande , affirms on behalf of all co - authors that we have no conflicts of interest in connection with this article .
a 45-year - old woman with dilated cardiomyopathy was admitted for the upgrade of a previously implanted pacemaker . echocardiography showed intraventricular dyssynchrony and a low ejection fraction ( 0.35 ) . treatment with a cardiac resynchronization therapy defibrillator ( crt - d ) was selected and the device was implanted . crt - d interrogation revealed proper function . following procedure termination , the patient went into cardiac arrest and died despite resuscitation attempts . an autopsy revealed that the medial aspect of the right atrium was pierced by an active lead and that the aorta had a deep lesion , 2 mm in length , on its lateral aspect . we explain the probable pathogenesis of this patients death .
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the mitral valve is located far from midline sternotomy , but is very close to the interatrial groove . as our experience grew and we learned from embryology , we found that a more cental left atriotomy was advantageous , and possible by interatrial groove dissection using ultrasonic scalpel . under general anesthesia , midline sternotomy is performed and the ascending aorta is cannulated for arterial inflow , and the superior vena cava and right atrial appendage are cannulated for venous outflow . the fad pad covering the interatrial groove is dissected under cardiopulmonary bypass by ultrasonic scalpel . the dissection is carried out in the exact groove of sondergaard until accessing beneath the thin atrial septum with careful attention paid to the coronary sinus vein ( fig . the aorta is cross - clamped and a vertical incision is made onto the left atrium , leaving enough room for the atrial wall to close . using a self - retaining retractor , atrial tissue above the anterior mitral annulus this makes a tractile and stretched wide view of the mitral valve possible because the left atrium wall hook - up is optimal . this is quite different from the right side left atriotomy , which usually leaves a redundant left atrial wall to be hooked up . because the mitral valve is such a complex anatomic structure and the maneuvers involved in correcting a regurgitant valve may vary from the simple to the very complex , adequate exposure is an absolute requirement in every operative plan . the first critical step to standard valve repair is the complete and thorough development of the sondergaard plane reflecting the right atrium off the left atrium to the atrial septum . this was first described in the 1950s by the danish surgeon sondergaard to expose the atrial septum for noncardiopulmonary bypass treatment of atrial septal defects . regardless of claims from previous procedures , it should always be possible to dissect out the groove without significant difficulty . the complete and full development of the groove is crucial for obtaining adequate exposure of the mitral valve . with this technique , via blunt and sharp dissection with ultrasonic scalpel , we had no need for any other incision for mitral valve repair or replacement , whether for primary surgery or reoperation . this incision brings the surgeon very close to the mitral valve even in the most difficult anatomic situations . once the right atrium is dissected off the left atrium , a generous incision in the left atrium is made , avoiding the atrial septum . we have been adopting this incision and approach in 10 consecutive patients and have found that the aortic cross - clamp time decreased significantly from an average 75 minutes to 48 minutes compared to right - side left atriotomy patients . a more central incision onto the left atrium
abstractexposure of the mitral valve is of critical importance in mitral valve surgery . the mitral valve is located near the interatrial groove . as we accumulated experience and learned from embryology , we found that a more central left atriotomy was advantageous , and was made possible by interatrial groove dissection using an ultrasonic scalpel . this crucial finding prompted us to report the technique .
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sertoliform endometrioid carcinoma of the ovary ( sec ) is an uncommon variant that bears histologic similarity to sertoli and sertoli - leydig cell tumors ( slts ) . recognition of this tumor is important as it is a well - differentiated , low - grade malignancy that displays a good prognosis when confined to the ovary . a 55-year - old postmenopausal female patient presented with mass per abdomen since 2 years and pain for 2 days . ultrasonography showed multicystic lesion measuring 12 cm 11 cm in the left ovary [ figure 1 ] . ultrasonography showing multicystic lesion measuring 12 cm 11 cm in the left ovary on gross examination , ovary weighed 550 g , measured 13 cm 11 cm 7 cm , showed solid and cystic areas on cut section [ figure 2 ] . microscopy showed round to solid tubules lined by pseudostratified columnar epithelium with elongated nuclei resembling sertoli tumor - like pattern along with conventional endometrioid tumor [ figures 35 ] . inset : outer surface showing blackish areas due to torsion microphotograph showing sertoliform endometrioid carcinoma showing tubules and tightly packed nests of tumor cells separated by fibrous stroma . individual tumor cells having vesicular nuclei , prominent nucleoli , and moderate cytoplasm ( h and e , 400 ) microphotograph showing foci of conventional endometrioid carcinoma ( h and e , 400 ) microphotograph showing foci of conventional endometrioid carcinoma and sertoli cell tumor - like pattern ( h and e , 200 ) immunohistochemistry ( ihc ) showed tumor cells strongly immunoreactive for epithelial membrane antigen ( ema ) , cytokeratin ( ck ) but negative for inhibin [ figures 68 ] , thus confirming the diagnosis of sec of left ovary . sertoliform endometrioid carcinoma showing cytokeratin positivity ( immunohistochemistry , 200 ) sertoliform endometrioid carcinoma showing epithelial membrane antigen positivity ( immunohistochemistry , 400 ) sertoliform endometrioid carcinoma showing inhibin negativity ( immunohistochemistry , 400 ) endometrioid carcinoma of ovary resembling sex cord - stromal tumor is a rare variant of endometrioid adenocarcinoma that focally looks like a sex cord - stromal tumor with sertoli , leydig , or granulosa cells . ordi et al . , in their study , reported that sec typically demonstrates the following characteristics , while slts do not : ( 1 ) presence of areas with usual pattern of endometrioid carcinoma , ( 2 ) presence of mucin at the apical borders of the tumor cells . other factors that may favor sec would include squamous or squamoid areas , well - developed cilia , the presence of endometriosis , or a concomitant adenocarcinoma of the endometrium . immunostains for alpha - inhibin is positive in most neoplastic sertoli cells but negative in the cells of endometrioid carcinoma , while positive ema and ck immunostains favor sec . despite histologic similarity misir and sur in their study report that in slts , the patient age tends to be younger , with an average age of 25 years and clinically , up to 50% of slt patients may exhibit endocrine manifestations . in contrast , sec occurs almost exclusively in postmenopausal women with an average age of 68 years . virilizing symptoms , though uncommon in endometrioid carcinoma , may be encountered in the sertoliform variant , thus compounding the diagnostic difficulty . in our case , usually , solid portion of endometrioid adenocarcinoma is considered as grade 3 according to the international federation of obstetrics and gynecologists grading system . sec should be considered as grade 1 ( well - differentiated ) , despite the presence of solid , sex cord - like proliferation as it carries good prognosis when confined to ovary . according to these suggestions , our case is graded as well - differentiated ( grade 1 ) based on the histological features of foci of conventional endometrioid carcinoma . the case is presented for its rarity . in view of relatively good prognosis of sec as compared to endometrioid tumor , identifying this variant by an extensive sampling of the specimen is mandatory .
sertoliform endometrioid carcinoma of the ovary ( sec ) is an uncommon variant that bears histologic similarity to sertoli and sertoli - leydig cell tumors . we report an interesting case of sec in a 55-year - old female with a left ovarian mass with torsion . histology revealed an sec , featuring foci of typical endometrioid carcinoma , and areas composed of uniform , small , hollow tubules lined by columnar cells with apical cytoplasm . tumor cells were strongly immunoreactive for ema and cytokeratin but negative for inhibin ; thus , confirming the diagnosis of sec ovary . recognition of this tumor is important as it is a well - differentiated , low - grade malignancy that displays good prognosis when confined to the ovary .
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aneuploid strains were generated by sporulation of the above polyploid strains , followed by karyotype stability tests and determination as described in fig . qpcr assays were designed with primers in non - coding regions on each chromosome arm ( supplementary table 1 lists primer sequences ) . dna samples were prepared by alkaline lysis , and qpcr reactions were performed in 384-well plates using a biomek fx ( beckman coulter ) to assemble 10 l reactions and an abi 7900ht ( applied biosystems ) for cycling . equal amounts ( od600 ) of aneuploid and euploid control cultures were spotted , using the biomek fx robot , onto omnitrays containing various solid media and grown under conditions listed in supplementary table 2 . omnitrays representing three biological replicates of each tested condition were scanned on an hp scanjet 4070 desktop scanner . whole - cell lysates were prepared from 50 ml cycling yeast cultures by bead - beating . the ms / ms datasets were searched using sequest23 against a database of 11,986 sequences , consisting of 5,816 s. cerevisiae non - redundant proteins ( ncbi ) , 177 contaminants and 5,993 decoy sequences . all statistical analyses were performed in the r environment25 using standard packages and custom scripts . aneuploid strains were generated by sporulation of the above polyploid strains , followed by karyotype stability tests and determination as described in fig . qpcr assays were designed with primers in non - coding regions on each chromosome arm ( supplementary table 1 lists primer sequences ) . dna samples were prepared by alkaline lysis , and qpcr reactions were performed in 384-well plates using a biomek fx ( beckman coulter ) to assemble 10 l reactions and an abi 7900ht ( applied biosystems ) for cycling . equal amounts ( od600 ) of aneuploid and euploid control cultures were spotted , using the biomek fx robot , onto omnitrays containing various solid media and grown under conditions listed in supplementary table 2 . omnitrays representing three biological replicates of each tested condition were scanned on an hp scanjet 4070 desktop scanner . whole - cell lysates were prepared from 50 ml cycling yeast cultures by bead - beating . the ms / ms datasets were searched using sequest23 against a database of 11,986 sequences , consisting of 5,816 s. cerevisiae non - redundant proteins ( ncbi ) , 177 contaminants and 5,993 decoy sequences . all statistical analyses were performed in the r environment25 using standard packages and custom scripts .
aneuploidy , referring here to genome contents characterized by abnormal numbers of chromosomes , has been associated with developmental defects , cancer , and adaptive evolution in experimental organisms19 . however , it remains unresolved how aneuploidy impacts gene expression and whether aneuploidy could directly bring phenotypic variation and improved fitness over that of euploid counterparts . in this work , we designed a novel scheme to generate , through random meiotic segregation , 38 stable and fully isogenic aneuploid yeast strains with distinct karyotypes and genome contents between 1n and 3n without involving any genetic selection . through phenotypic profiling under various growth conditions or in the presence of a panel of chemotherapeutic or antifungal drugs , we found that aneuploid strains exhibited diverse growth phenotypes , and some aneuploid strains grew better than euploid control strains under conditions suboptimal for the latter . using quantitative mass spectrometry - based proteomics , we show that the levels of protein expression largely scale with chromosome copy numbers , following the same trend observed for the transcriptome . these results provide strong evidence that aneuploidy directly impacts gene expression at both the transcriptome and proteome levels and can generate significant phenotypic variation that could bring about fitness gains under diverse conditions . our findings suggest that the fitness ranking between euploid and aneuploid cells is context- and karyotype - dependent , providing the basis for the notion that aneuploidy can directly underlie phenotypic evolution and cellular adaptation .
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a woman in her 30s with acquired immunodeficiency syndrome ( aids ) diagnosed by the fourth - generation hiv testing , with a cd4 count of 3 ( 1% ) cells / mm , presented with weight loss and cachexia . she was started on lamivudine , zidovudine , and nevirapine and then developed hypersensitivity rash and hepatitis . at that time , she also had pancytopenia ( white blood cells 2,950/l , neutrophils 79% , lymphocytes 13% , hemoglobin 9.6 g / dl , hematocrit 29% , platelets 325,000 10/l ) and intra - abdominal lymphadenopathy . she was treated initially for presumptive disseminated mycobacterial infection with isoniazid , rifampicin , pyrazinamide , ethambutol , and clarithromycin . she improved clinically , and her antiretroviral regimen was restarted after three weeks with lamivudine , tenofovir , and efavirenz . later on , she had gained weight , and laboratory results showed the resolution of pancytopenia and hepatitis . two months later , the antibiotic regimen was readjusted to ciprofloxacin , clarithromycin , and streptomycin injection ( for one month ) according to the susceptibility , and her clinical improvement was remarkable . two months after the initiation of antiretroviral therapy ( art ) , she had an hiv viral load of < 40 copies / ml and a cd4 count of 1 ( 2% ) cell / mm ; however , she had recurrent transaminitis . she was given prednisolone 20 mg / day with a tapered dose for five weeks for the treatment of hepatitis possibly due to immune reconstitution inflammatory syndrome . five months after the initiation of art , her cd4 count was 13 ( 3% ) cells / mm , with an hiv viral load of < 40 copies / ml . she developed cytomegalovirus retinitis and received intravitreal ganciclovir injection and oral valganciclovir for six weeks . she then developed abdominal pain , with a palpable large suprapubic mass , for three weeks . a computed tomography ( ct ) scan of the abdomen showed a large lobulated mass ( 8.5 10 cm ) with the epicenter at the right adnexa and with the invasion of the uterus , right ovary , right lateral wall of the sigmoid colon , appendix , superior wall of the urinary bladder , and right distal ureter , causing right ureter obstruction and a moderate degree of right hydronephrosis . multiple matted lymph nodes were present along the mesenteric root and in the para - aortic , aortocaval , and retrocaval regions . focal circumferential wall thickening of the left side of the jejunum caused moderate intraluminal narrowing , with evidence of small bowel obstruction ( fig . she underwent exploratory laparotomy ; intraoperative findings revealed a large pelvic mass with nodular surface and with dense adhesion to the omentum , the anterolateral wall of the uterus , the bladder , and the sigmoid colon . only partial resection of the omentum and a biopsy of the mass at the anterior aspect of the uterus were performed . acid - fast and gomori methenamine stains of the tissue specimen were negative ; however , microbial culture was not sent for laboratory analysis . her hospital course was complicated by wound infection / dehiscence , with the formation of an enterocutaneous fistula . she suffered from abdominal pain as well as malnutrition due to the nature of the unresectable residual mass and the enterocutaneous fistula . she was discharged home on palliative care , with antimicrobial therapy , and was lost to follow - up . disseminated m. simiae infection presented with pancytopenia , hepatitis , and lymphadenopathy , similar to mycobacterium avium infection in an aids patient.1 effective antimicrobial treatment of m. simiae included rifampicin and ciprofloxacin . our patient had an abnormal host immune response to infection , malakoplakia , which occurred after m. simiae infection , a condition that has not been previously described in the literature . however , we were unable to demonstrate a direct correlation due to the lack of microbiological data from tissue specimens . malakoplakia has a gross appearance of round , oval , or mushroom - shaped yellowish structures.2 histologically , there is a proliferation of histiocytes with abundant granular eosinophilic cytoplasm ( known as von hansemann histiocytes ) that contain the pathognomonic michaelis gutmann bodies.2 these bodies are round to oval in shape and are visible as deeply basophilic structures , which are typically described as target - like and are periodic acid schiff stain - positive and calcium - positive.2 pathogenesis of malakoplakia is associated with the lysosomal dysfunction of macrophages in the intracellular killing process of ingested organisms and/or a defect in elimination , resulting in the accumulation of partially degraded bacteria within the cytoplasm and phagolysosomes of histiocytes , forming michaelis gutmann bodies around the undigested bacteria.2 the most common infectious etiologies described are bacteria , such as escherichia coli , rhodococcus equi , pasteurella multocida , and mycobacterium tuberculosis.211 malakoplakia often occurs in immunocompromised patients , such as those with aids , organ transplant recipients ( mostly solid organ transplants ) , and in one case a patient who had undergone stem cell transplantation.5,1114 malakoplakia affecting a major organ could result in various organ dysfunctions . one of the most serious adverse events reported was irreversible renal allograft dysfunction due to malakoplakia in a renal transplant recipient with e. coli infection , pyelonephritis , and bacteremia.14 in an allogeneic stem cell transplant recipient , pleural malakoplakia caused by r. equi , presenting with a large tumor mass , required a lobectomy.12 in patients with residual disease , successful results have been reported by treating the underlying infection , reducing immunosuppression , improving the bactericidal activity of monocytes with a cholinergic agonist , such as bethanechol , and administering a multivitamin supplement.4,15,16 this is the first report of malakoplakia occurring after m. simiae infection .
malakoplakia in an acquired immunodeficiency syndrome ( aids ) patient with disseminated mycobacterium simiae infection presented with a large pelvic mass that caused organ dysfunction from mimicking a tumor . malakoplakia is a rare , chronic granulomatous abnormal host response toward infectious agents , presenting as a tumor - like lesion . this is the first report of pelvic malakoplakia after disseminated m. simiae infection in an aids patient .
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postoperative complications of blepharoplasty range from skin changes to vision - threatening emergencies.1 some of these complications occur early in the postoperative period , such as retrobulbar hemorrhage , infection , and eyelid hematoma . other complications occur later in the postoperative period , such as eyelid malposition , strabismus , scar and over- and under - resection of skin or orbital fat.1 there has been a case report of persistent lateral hooding after upper lid blepharoplasty that has been treated with lacrimal gland repositioning.2 to the best of our knowledge , we report the first case of lacrimal gland fistula after upper lid blepharoplasty . she had a history of repeated lid swelling for almost 3 years before the age of 10 years . the patient underwent bilateral blepharoptosis repair at 12 years of age , re - operation on the right lid at the age of 18 years , and laser resurfacing of periocular region at the age of 20 . on examination , the patient had bilateral blepharoptosis ( margin reflex distance of + 2.5 mm on the right and + 2 mm on the left side ) , dermatochalasis with mild lateral hooding , and medial and preaponeurotic fat protrusion . upper blepharoplasty included skin - orbicularis muscle flap excision , medial fat excision , and partially preaponeurotic fat excision through a hole in the center of the septum . she had an uneventful postoperative follow - up with the exception of lateral hooding of the right lid . an elliptical lateral hooding excision was performed 2 months after upper blepharoplasty . at 1 week post - hooding excision , there was wound dehiscence with clear watery drops discharging from the wound [ figure 1 ] . post - lateral hooding excision right upper eyelid wound dehiscence and watery drops show a fistulous tract from lacrimal gland to the wound the patient was scheduled for repair of the wound and repositioning of the lacrimal gland . there was a tract from the lacrimal gland to the skin of the upper eyelid at the site of wound dehiscence . the lacrimal gland was repositioned into the lacrimal gland fossa using 4 - 0 prolene suture . the postoperative course was uneventful out the last visit at 6 months after repair [ figure 2 ] . last follow up ( 6 months ) after repair of the wound and repositioning of the right prolapsed lacrimal gland despite the facile nature of the blepharoplasty , high patient expectations can make this procedure quite challenging to the surgeon . in order to attain a good result and avoid patient dissatisfaction , the surgeon must perform a careful history and physical exam and address specific patient complaints and expectations.3 blepharochalasis is a rare eyelid disorder characterized by exacerbation and remission of painless edema , eventually leading to atrophy of the periorbital skin . these episodes of eyelid swelling usually become less frequent with age , and eventually most cases enter a relatively quiescent stage . ptosis is a common finding in blepharochalasis ; however , the levator function is preserved.4 surgical management should be performed during the quiescent phase of blepharochalasis to avoid recurrent bouts of lid swelling leading to further ptosis and lid atrophy . surgeons advocate that blepharochalasis symptoms should be quiet for 6 - 12 months before surgical treatment is contemplated.4 our case did not report any exacerbation for at least 15 years prior to the recent operation . a prolapsed lacrimal gland occasionally occurs due to atrophic changes in the septum in patients with blepharochalasis . in cases of frank preoperative prolapsed lacrimal gland , a blepharoplasty and suspension of the lacrimal gland should be performed.4 our patient did not present with a prolapsed lacrimal gland preoperatively . she only had post - blepharoplasty lateral hooding on the right side without a palpable lacrimal gland . we assume that blepharoplasty and consequently right - side hooding excision pulled an undetected partially prolapsed lacrimal gland inferiorly into the wound and resulted in the formation of a fistula . a similar case has been reported with hooding after blepharoplasty due to lacrimal gland prolapse ; however there was no history of blepharochalasis or postoperative wound dehiscence and fistula.2 repositioning of the lacrimal gland to the right side was successful without postoperative sequelae out to 6 months postoperatively . the procedure can complex especially in the context of chronic inflammatory conditions.5 this case report illustrates an uncommon post - blepharoplasty complication in patients with blepharochalasis . surgeons who perform blepharoplasty should have a high index of suspicion for lacrimal gland herniation in the presence of lateral hooding specially in patients with a history of blepharochalasis .
to report the first case of lacrimal gland fistula after upper eyelid blepharoplasty for blepharochalasis . standard upper blepharoplasty and the hooding excision were performed in a female with blepharochalasis . the patient developed a fistulous tract with tearing from the incision few days after hooding excision . fistula excision and lacrimal gland repositioning were performed . there were no complications after the repositioning procedure ( 6 months follow up ) . prolapsed lacrimal gland and fistula formation can occur after upper blepharoplasty hooding excision .
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cross sensitivity is defined as sensitivity to one substance that renders an individual sensitive to other substances of similar chemical structure . cross sensitivity has been reported commonly among various lactam antibiotics and sulfonamides . for the diagnosis of cross sensitivity , various methods like prick test , oral challenge test is considered as the most reliable . due to broad antimicrobial spectrum , frequently reported adverse reactions with fluroquinolones are nausea , abdominal pain , diarrhea , dizziness , restlessness , headache , depression and somnolence or insomnia . . immediate type of reactions are more common than delayed type of reactions . frequently reported immediate hypersensitive reactions are urticaria , anaphylactic shock , rashes and erythema . ciprofloxacin and norfloxacin are the most common drugs causing hypersensitivity reactions , while levofloxacin is the least common with incidence of 1 per million population . however , we could not find a case of immediate hypersensitivity reaction with levofloxacin or case report of cross sensitivity between ciprofloxacin and levofloxacin . here with , we report a case of cross sensitivity between ciprofloxacin and levofloxacin for immediate type of hypersensitive reaction . seven years old male child ( 24 kg weight ) admitted in paediatric ward , sir takhtsinhji general hospital , government medical college , bhavnagar , gujarat , india with complaints of fever , headache , vomiting and abdominal pain for 7 days . fever was of high grade , intermittent and associated with chills , which was followed by colicky abdominal pain . he had a past history of similar attacks two times , at that time pain was relieved after oral treatment . various investigations like hemoglobin , total and differential wbc count , platelet count , esr , urine and stool examination were normal . as per surgical opinion conservative treatment was started with ciprofloxacin infusion ( 20mg / kg / day in two divided doses ) , inj . metronidazole ( 30mg / kg / day , in three divided doses ) , inj . five minutes after starting infusion of ciprofloxacin , patient developed itching followed by rashes at the site of infusion . on dermatological examination , reaction started from the site of injection and spread upward involving whole arm up to chest . infusion was stopped immediately and injection dexamethasone ( 0.5mg / kg ) was given along with injection chlorphenaramine maleate ( 0.1mg / kg ) . levofloxacin ( 10mg / kg / day ) was added in treatment in place of ciprofloxacin from next day under close observation . immediately after levofloxacin infusion , itching and rashes in arm appeared so , infusion was withdrawn . ceftriaxone ( 100mg / kg / day ) injection added in the place of fluoroquinolones along with metronidazole . it was moderate in severity according to modified hartwig and siegel 's scale and non preventable as per modified schumock and thornton scale . all fluoroquinolones have similar core structure ( 4-oxo-1 , 4-dihydroquinoline ring ) with fluorine atom attached at position 6 except , first generation quinolone , nalidixic acid . norfloxacin , a second generation fluroquinolone , is the result of replacement at c-7 methyl side chain with piperazine group , while replacement of n-1 ethyl group of norfloxacin with cyclopropyl group generates ciprofloxacin . levofloxacin , third generation flouroquinolone , has modification of piperazine group with methyl group at c-7 . although changes in position 1 , 7 and 8 generated different fluoroquinolones , core structure remains same . 54.5% immediate type of reactions to fluoroquinolones are ige mediated and occurs due to binding of ige to 7 position of core structure of fluoroquinolones . patient had a past history of two similar attacks , at that time he might be treated with fluoroquinolones , which may resulted in production of ige antibodies . as a result , treatment with fluoroquinolones this time might have caused the reaction . ciprofloxacin and levofloxacin have similar core structure ; this may be the reason for the cross sensitivity in this case . it is advisable to avoid other fluoroquinolones when hypersensitivity reaction detected to one and should be shifted to other class of antimicrobials . overall risk of adverse effects especially hypersensitivity reactions with fluoroquinolones is low but due to widespread use , such rare hypersensitivity reactions should be kept in mind . improvement in diagnostic tests like detection of ige antibodies , cellular tests and skin test may help in preventing such adverse drug reactions .
seven years old male child ( 24 kg weight ) diagnosed as a case of sub acute appendicitis treated with ciprofloxacin , immediately developed multiple erythmatous papules . reaction subsided after withholding ciprofloxacin and treatment with dexamethasone and chlorpheneramine maleate . it was developed again when treated with levofloxacin and subsided after withdrawal . ige binding at 7th position of core structure of fluoroquinolones likely to be the mechanism . as all the fluoroquinolones have similar core structure , hypersensitivity to one may have cross sensitivity to other fluoroquinolones . it is advisable to avoid other fluoroquinolones and switch over to other group of antibiotics when hypersensitivity to one occurs .
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female genital tuberculosis ( fgtb ) is common in developing countries and associated with significant morbidity in the form of menstrual dysfunction , infertility , tubal block , peritubal adhesions , intrauterine adhesions , and perihepatic adhesions . fallopian tubes are involved in about 90% of the cases with findings such as congested tubes , tubercles , or caseous nodules on them , peritubal adhesions , and tubal blockage at various levels such as cornual block , mid - tubal block , multiple tubal blocks , or fimbrial block , hydrosalpinx , pyosalpinx , and tubo - ovarian masses . a 25-year - old woman with family history of pulmonary tb was presented to the gynecology outpatient department with primary infertility and oligomenorrhea of 3 years . on examination , vaginal examination demonstrated anteverted uterus with fullness and induration in both fornices . on investigations , hysterosalpingogram which was already performed by the referring doctor for infertility before diagnosis of fgtb was made showed bilateral tubal block . endometrial aspirate performed in premenstrual phase showed positive polymerase chain reaction based on the amplification of the 240 bp region of the mpt 64 gene using primers mpt 1 ( 59-tccgctgccagtcgtcttcc-39 ; nt 460479 ) and mpt 2 ( 59-gtcctcgcgag tctaggcca-39 ; nt 700681 ) with equipment using amplitron thermocycler ( barnstead / thermolyne ) , it also tested positive for culture for mycobacterium tuberculosis using mycobacterium growth indicator tube-960 . , there were findings of tb in the pelvis in the form of multiple tubercles on tubes and uterus , bilateral hydrosalpinx with beading of tubes , and few caseous nodules . when methylene blue dye was injected in the uterus , the fallopian tubes became distended and blue colored with beading looking with alternate constrictions and dilatation looking - like blue pythons diagnosis of genital tb was made , and she was started on anti - tb therapy . her postoperative period and follow - up were uneventful . left fallopian tube with distension , multiple ballooning , and blue coloration with fimbrial block showing blue python sign although gold standard in diagnosis of fgtb is demonstration of mycobacterium tuberculosis either on microscopy or culture of endometrial biopsy or presence of epithelioid granuloma on histopathology , they are positive in only few cases leading on to missing of diagnosis in many cases . polymerase chain reaction on endometrial biopsy is a sensitive and rapid method for detecting mycobacterium dna ( mpt 64 gene ) but can be false positive and may not be able to differentiate between infection and disease . gene xpert has recently been introduced in both pulmonary and extra - pulmonary tb as a sensitive and specific method , but its data in fgtb are very sparse . imaging modalities such as ultrasound , computed tomography scan , magnetic resonance imaging , and positron emission tomography scan have main role in tubo - ovarian masses . hysterosalpingography is generally avoided in a suspected case of fgtb due to risk of flare up of the disease but may demonstrate tubal block and other tubal patterns , especially in advanced case . hysteroscopy is useful in endometrial disease and may show pale looking endometrium with endometrial cavity being partially or completely obliterated by adhesions of varying grade ( asherman 's syndrome ) which may involve ostia . however , laparoscopy is the most reliable tool to diagnose fgtb , especially for tubal , ovarian , and peritoneal disease . there can be tubercles on peritoneum or tubes , tubo - ovarian masses , caseous nodules , encysted ascites , various grades of pelvic adhesions , hydrosalpinx , pyosalpinx , beaded tubes , tobacco pouch appearance , and inability to see tubes due to adhesions . other authors have also found laparoscopy very useful in diagnosis and management of genital and peritoneal tb with the advantage of avoidance of laparoscopy which is more traumatic and hazardous especially in a case of abdominopelvic tb . in a case of fgtb , if there is no cornual block , there is a partial or complete fimbrial block with multiple blocks at various parts of tube as is typical of fgtb , dye enters the tube but remains in various parts of tubes with alternate dilatation and constriction making the tube look like a blue python as happened in the present case . the new sign case is easily diagnosed by gynecologists during routine laparotomy performed for infertility patients with suspected fgtb and can aid in its diagnosis
female genital tuberculosis ( fgtb ) is an important cause of infertility in developing countries . various type of tb salpingitis can be endosalpingitis , exosalpingitis , interstitial tb salpingitis , and salpingitis isthmica nodosa . the fallopian tubes are thickened enlarged and tortuous . unilateral or bilateral hydrosalpinx or pyosalpinx may be formed . a new sign python sign is presented in which fallopian tube looks like a blue python on dye testing in fgtb .
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porokeratoses are a group of cutaneous entities of unknown etiology , characterized by disordered epidermal keratinization and , at histological examination , by the presence of coronoid lamella , a column of parakeratotic cells extending through the stratum corneum . disseminated superficial porokeratosis ( dsp ) is a distinct form of porokeratosis that , clinically , is characterized by numerous , small , superficial keratotic papules with verrucoid aspects , an atrophic center and peripheral keratotic border . only 7 cases of this association have been described in the literature ( table 1 ) . we report a eighth case of this type and an atypical immunohistochemical method used to characterize the amyloid substance . a 76-year - old woman was referred to our department in june 1999 . she reported a 20 years history of an erythematous and squamo - papular eruption located on the extremities , without itching or pain . the patient had noted a progressive extension of these lesions in the last year and an exacerbation of the lesions during the summer . upon physical examination , numerous papules with a size of less than 15 mm , an atrophic center and hyperkeratotic borders were distributed on the neck , chest and extremities . palms , soles , mucous membranes and nails were spared , and the patient denied any family history of similar skin lesions . we examined a skin biopsy that revealed atrophy of the epidermis with hyperorthokeratosis and presence of homogeneous and compact cornoid lamella of keratin , typically oriented sideways with respect to the epidermal tissue ( fig . 1 a ) . the superficial dermis showed a slight chronic inflammatory infiltrate with vascular ectasia and edema . moreover , in the papillary dermis a compact deposit of a cellular eosinophilic material suspicious for amyloid was observed ( fig . 1b ) . to demonstrate the epithelial origin of the amyloid , we performed two immunohistochemical stainings by using specific moabs to pan - cytokeratin ae1-ae3 . interestingly , there was a striking difference between the two results : in fact , the acellular deposits , mentioned above , were negative for ckae1-ae3 , whereas a strong immunoreactivity was demonstrated for ck5 , supporting a specific origin of the amyloid substance from the basal cell layer of the epidermis ( fig . in 1937 , andrews first described dsp and introduced this term to indicate a clinical variant of mibelli 's porokeratosis . later , chernoski and freeman proposed a possible actinic etiology of this dermatosis and coined the term disseminated superficial nowadays , this term is generally accepted in european dermatological literature , and this definition is based on clinical and histological findings . dermatological manifestations are typically confined to sun exposed areas , with actinic induction and exacerbations . in the literature , two types of localized cutaneous amyloidosis ( lca ) are described : primary lca ( macular amyloidosis and lichen amyloidosis ) , which is not associated with other dermatoses or systemic involvement , and secondary lca , which is associated with inflammatory , hamartomatous or neoplastic skin disorders . the mechanism by which dsp induces dermal amyloid deposits is not clear , but piamphongstant et al . first suggested that this process can derive from degenerated epidermal keratinocytes . we hypothesize that a mutant keratinocyte clone is responsible for induction of porokeratotic lesions , because these necrotic epidermal keratinocytes ( colloid bodies ) might be transformed into amyloid by dermal macrophages and fibroblasts . immunohistochemical staining has shown an overexpression of p53 protein in porokeratotic lesions ; this is a tumor suppressor protein , an important gatekeeper and effector of the cell cycle . mutations of the p53 gene in all forms of porokeratosis , also in dsap , create a permissive state of uncoordinated cell cycling , and predispose cells to death . in our case , the lack of systemic involvement led to our hypothesis of a secondary dermal deposition of amyloid proteins and the existence of a close relationship between these two processes . this hypothesis was confirmed by positive staining with congo red and immunohistochemical staining with the anticytokeratin moabs strongly positive for ck5 , just below the epidermal porokeratotic zone in close proximity to the cornoid lamella . this cytokeratin is , in fact , strongly represented in the basal cell layer and these results indicate that the dermal deposits were amyloid originating from the epidermis ( type ii keratin ) . nowadays , more studies are necessary to clarify the exact mechanism that leads to secondary deposition of dermal amyloid in porokeratotic disease and the frequency of this association . we also suggest utilization of moabs for ck5 as a first - line target in these conditions , encouraged by the immediate and strong positivity of this protein in immunohistochemical studies , whereas pan - cytokeratin at first produced negative results .
only 6 cases with an association of disseminated superficial porokeratosis with dermal amyloid deposits are reported in the literature . we present the case of a 76-year - old woman who presented with a disseminated superficial porokeratosis . histological examination revealed amyloid deposits in the upper dermis , which were typed with routine he stains , congo red stains and anticytokeratin antibodies ( ae1-ae3 and ck5 ) . positive staining with congo red and , moreover , with ck5 ( a cytokeratin strongly represented in the basal cell layer of the epidermis ) indicates an epidermal origin of this protein .
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hemangiomas are common lesions of the head and neck area , and they predominantly originate from the lips , tongue and buccal mucosa . the capillary type is lobulated and it lacks a capsule , it is purplish in color and it infiltrates the involved glands . microscopically , solid masses of cells and multiple anastomosing capillaries that replace the acinar structure of the gland are seen . the cavernous type is formed by dilated blood vessels or sinusoids lined by endothelium ( 1 ) . cavernous hemangioma of the external auditory canal ( eac ) and tympanic membrane is rare , but this lesion is more common in the eac . according to the previously reported cases ( 2 - 8 ) , cavernous hemangioma seems to most frequently arise in the posterior portion of the eac . however their incidence in the tympanic membrane is very rare and there have been only 4 such case reports in the literature according to a medline search from 1970 to 2008 ( 9 - 12 ) . we report here the 5th case of isolated cavernous hemangioma that was limited to the tympanic membrane and we review the relevant literature . a 49-year - old man patient was referred to our department for evaluation of right - sided pulsatile tinnitus that he had experienced for the previous 2 years . the patient denied having hearing loss or vertigo and he had no evidence of facial palsy . endoscopic examination of the eac revealed a red - colored soft non - pulsatile mass that occupied part of the tympanic membrane ( fig . the tympanic membrane was partially obscured by the mass and it was not clearly mobile . a pure tone audiogram ( pta ) showed high frequency mild sensorineural hearing loss in the right ear at 3,000 - 8,000 hz ( fig . 2 ) . temporal bone computerized tomography ( ct ) showed an isolated soft tissue mass just lateral to the tympanic membrane . a tympanomeatal flap was elevated to reveal a mass with involvement of the tympanic membrane . the mass was spongy on palpation , and it was removed en bloc with little bleeding . the defect of the tympanic membrane was reconstructed by type i tympanoplasty and using a piece of fascia taken from the temporal muscle . pathologic examination of the specimen showed a well - demarcated nodular lesion composed of dilated vascular spaces lined by a single layer of flat endothelial cells ( fig . the postoperative endoscopic examination showed an intact state of the neo - drum with good ventilation . in 1983 , andrade et al . ( 10 ) reported the first case of cavernous hemangioma limited to the tympanic membrane in a 59-year - old man with a contralateral ear infection . according to the previously reported cases , cavernous hemangioma seems to most frequently arise in the posterior portion of the eac . conductive hearing loss , tinnitus and aural fullness may be present . in the present case , the differential diagnosis includes aural polyp , glomus tumor , arteriovenous malformation , granulation tissue and carcinoma of the eac . a temporal bone ct scan is the first choice for the evaluating the location and size of the lesion and middle ear involvement . the majority of the previously reported cases showed a normal middle ear . only 1 case reported by magliudo et al . ( 8) had cavernous hemangioma that initially involved the middle ear space through a small perforation in the tympanic memrane and the tumor had reached the handle and the short process of the malleus . for an advanced lesion , diagnostic angiography should be obtained to identify the blood supply to preoperatively assess the safety and feasibility of performing embolization ( 5 ) . surgical treatment with en bloc excision is generally curative , and preoperative embolization is usually not necessary except for an advanced lesion . the endaural approach is generally used . in this case , we removed the mass by the postauricular approach due to the size of the mass .
cavernous hemangioma seems to most frequently arise in the posterior portion of the external auditory canal . however , they rarely occur in the tympanic membrane . a 49-year - old male patient was referred for evaluation of right - sided pulsatile tinnitus that he 'd experienced for the previous 2 years . temporal bone computerized tomography showed an isolated soft tissue mass just lateral to the tympanic membrane . there was no evidence of bony erosion or middle ear invasion . the patient underwent excision of the mass using a postauricular approach . the mass was removed en bloc and the defect of the tympanic membrane was repaired by tympanoplasty type i. there was no recurrence after 1 year of follow - up .
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pica actually refers to the latin word for magpie , a bird thought to have odd feeding habits . incidence of pica has also been linked to iron and zinc deficiency . whether the pica is an eating disorder or obsessive compulsive disorder is still controversial . the patient was a 27 year old young indian lady living in the baruipur with no previous psychiatric or medical history . patient complained of an unusual sensation in her abdomen for the last 3 months while her parents gave a history of habitual consumption of nails for the same duration . the patient was a single child to her parents and she was living in the suburbs since her birth . she had attended public schools and had completed her graduation on arts from a college in kolkata . soon after her marriage , she expressed sexual aversion with history of dyspareunia . within a month of her marriage , she found it extremely difficult to live in her in - laws house and preferred to stay at her parental home , although her parents repeatedly insisted her to go back to her in - laws residence . depressed and also admitted that for the last 3 months , she was having a sense of hopelessness and worthlessness over her future days . vanished. she temporarily enjoyed the gritty and spiky sensation of nails along her throat , but at night she used to have a vague abdominal sensation which she described to be funny. according to her mother , she did not want to go back to live with her husband . on close interviewing of the patient , initially her response to same- sex relationship was guarded but later it was found that she was actually having such a relationship with another lady of her age for the past 3 years . she also accepted that she used to consume glass piece occasionally since last 3 years but never disclosed them to anyone . she had once had an x - ray of her abdomen done 2 years back due to complaints of low back pain which was inconclusive ; perhaps glass beads which are radiolucent were missed . no further investigation was done . the patient was decently dressed in a pastel shade salwar with a boyish cut hairstyle . on mental state examination on interviewing the husband , it was known they had never had any sexual intercourse since marriage . on routine examination , possibility of iron deficiency anemia , serum ferritin abnormality and zinc deficiencies were ruled out . x ray of abdomen showed multiple radio opaque pointed objects in her gut ( ingested iron nails ) . the patient had been nurtured in an overprotected environment by her parents in her early childhood days . however immediately following her high school , she had to take many responsibilities of the family all of a sudden because of loss of job of her father . she was more bonded to her mother and in the elderly , often avoided her father because he was alcoholic . the cultural , psychodynamic , and physiological aspect of this case is a unique one . the patient 's initial experimentation with ingestion of glass beads , though in a very remote sense , may be culture based considering the fact that the practice of ingesting such beads and sharp metallic objects has been reported . considering the fact that the patient was initially nurtured in an overprotected environment by her parents and suddenly being exposed to a comparatively hostile situation where she found a discouraging picture of the members of the opposite sex from that of her alcoholic father , the patient 's inclination toward same - sex relationship can be explained . psychologically , the inclination of the patient toward same sex relationship and her unfamiliarity and uneasiness with the members of the opposite sex led to marital dissatisfaction . also , since the patient had to take many burdens of her family at an age which may be considered quite an early one considering the indian standards and her socioeconomic profile , this probably has caused her to resort to some , harsh robust means like ingestion of nails which may have been an act to depict her her intelligent quotient as measured by wechsler 's adult intelligence scale had been found to be 98 which fall in normal age . as in most cases of pica , she initially did not come with a complaint of pica . rather , she had reported to the hospital with complaints of unusual sensation in her abdomen . the present case presented a unique conglomeration of cultural , psychodynamic , and physiological determinants which together contributed to the expression of pica in comparison with previous reports and reviews . however , an interesting fact in this case is the age of the patient which is quite unusual for pica to occur . this perhaps necessitates further research in dealing with the epidemiology and other aspects of this quite rare but interesting disease entity .
pica has been considered as the ingestion of inedible substances or atypical food combinations . pica has been reported widely in pediatric age group and often found to be co existing with obsessive compulsive or major depressive disorder . reports of pica in elderly age group are relatively uncommon and rarely does it have an adult onset . in this article we present a case of adult onset pica . a young lady with unusual sensation in her abdomen was found to consume iron nails over years and there was history of dyspareunia since her marriage three months back . on query it was known that the lady is having same sex relationship over years . there unique conglomeration of cultural , psychodynamic and physiological determinants which together is responsible for this unusual habit of this lady . moreover the onset of the disease at a late age and different psychodynamic issues make the case all the more interesting . whether the pica is an eating disorder or obsessive compulsive disorder is still controversial . pica has been mentioned in diagnostic and statistical manual iv tr . the present case report warrants the need to look into this entity more closely with regards to its occurrence and etiology .
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intraspinal juxtafacet cysts ( commonly , synovial or ganglion cyst ) can be an uncommon cause of lower and radicular pain , neurogenic claudication , and cauda equina syndrome2,9,10,13 ) . spinal synovial cysts generally arise from a degenerated facet joint and often presenting as a gradual onset of back pain and potentially , chronic progressive radiculopathy is a much less common occurrence , and patients may present with acute onset of symptoms3,13 ) . we describe a case in which hemorrhage into a right l2 - 3 facet synovial cyst caused acute back pain and radiculopathy and required surgical excision . a 72-year - old - woman with a past history of hypertension presented to the clinic with acute back pain radiating to the anterior surface of the right thigh and leg . the right straight - leg - raise test elicited pain in the leg at 30 which was exacerbated further by ankle dorsiflexion . magnetic resonance imaging ( mri ) revealed the presence of a cystic formation in the right l2-l3 facet joint with arthritis compressing the l3 right root and the dural sac ( fig . , the mri displayed a hyperintense abnormality on t1-weighted images and hypointense on t2-weighted images consistent with hemorrhage . the posterior elements from l1 to l3 were exposed and right l2-l3 laminectomy and facetectomy were performed . a brownish mass was found in continuity with the right l2-l3 facet joint adherent to the dural sac . the extradural hematoma was visualized and evacuated , effectively decompressing the right 2 nerve root and sacral sac . microscopically , there was a synovial cyst with synovial cell lining , neovascularization , hemosiderin microdeposits , and blood ( fig . one month after the operation , the patient had no symptoms in her lower back and leg . spinal synovial cyst can be defined as a soft tissue mass located extradurally along the medial border of a degenerated facet joint , especially l4 - 5 level1,4,8,11,12 ) . these cysts are filled with clear or xanthochromic fluid and have a synovial - like epithelial lining with a demonstrable connection to a joint capsule3,11 ) . if the synovial cell lining and the communication with a joint capsule are absent , the cyst is classified as ganglion11 ) . intraspinal synovial cysts are rare but have been documented with increasing frequency because of the improvement of neuroradiological imagings4 ) . an mri is considered a good modality for diagnosis . on mri , cysts appear as well - circumscribed , epidural mass lesions that are hypo- and hyper - intense on t1 and t2 weighted images , respectfully . bleeding into a synovial cyst may cause a sudden expansion of the cyst leading to compression of the epidural space result in root compression symptoms . hemorrhage into a synovial cyst results in an acute increase in pain and radicular symptoms , including neurological deficits12 ) . hemorrhagic presentation can be caused by anticoagulation treatment , trauma , disc herniation , vascular anomaly , and neoangiogenesis in the cyst4 ) . in addition , high vascularization of the cyst can cause hemorrhage in spite of minor trauma or just spinal instability . synovial cyst hemorrhagic events in some cases can occur despite the absence of trauma or coagulopathy13 ) . as a result , other yet to be determined risk factors for synovial cyst hemorrhage occurrence exist prompting the need for more studies . non - hemorrhagic synovial cysts are occasionally treated by percutaneous aspiration with successful resolution of symptom6 ) . the injection of corticosteroid agents into the facet joint may be an treatment option . despite conservative treatment , recurrence of cyst with symptoms the natural history of spinal synovial cysts is unknown , but spontaneous remissions of a synovial cyst on follow up imaging with improved symptoms have been reported5 ) however , pain can be treated with steroid injections and bed rest in substantial number of patients . we report of a case in which hemorrhage into a right l2 - 3 facet synovial cyst causing an acute back pain and radiculopathy . treatment by resection of the cyst and evacuation of the hematoma led to complete neurological recovery . synovial cysts of the lumbar spine are an uncommon cause of back and radicular pain . rarely , these cysts can go through hemorrhagic transformation by trauma , anti - coagulopathic state , or for unknown reasons . hemorrhage into a cyst is an uncommon occurrence that can lead to acute symptom . in most cases , surgery is the treatment of choice for symptomatic hemorrhagic synovial cysts . surgery involving the evacuation of the hematoma and/or cyst can result in complete resolution of acute symptoms and neurologic deficit .
synovial cysts of the lumbar spine are an uncommon cause of back and radicular pain . these cysts most frequently present as back pain , followed by chronic progressive radiculopathy or gradual onset of symptoms secondary to spinal canal compromise . although less common , they can also present with acute spinal cord or root compression symptoms . we report of a case in which hemorrhaging into a right l2 - 3 facet synovial cyst caused an acute onset of back pain and radiculopathy , requiring surgical excision .
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bednar tumor or pigmented dermatofibrosarcoma protuberans ( dfsp ) is a rare , aggressive , cutaneous tumor that constitutes 1 - 5% of all dfsps . a 40-year - old female presented with right shoulder swelling present since few months and gradually increasing in size . a complete excision was performed without any prior fine needle aspiration cytology or needle biopsy . the tumor was just beneath the skin , fairly circumscribed , non - capsulated extending into the subcutaneous fat and measuring 5 4 4 cm . grossly , focal areas of pigmentation were noticed towards the periphery of the tumor ( fig . the tumor was a spindle cell lesion abutting the skin and invading the underlying subcutaneous fat . 2 ) . on higher magnification , the spindle cells were uniform appearing with a moderate amount of eosinophillic cytoplasm and plump nuclei with tapering edges . the pigment - laden cells showed coarse brown black pigment obscuring the nuclei and positivity for masson - fontana stain . it is considered to be a tumor of intermediate grade and very infrequently reported in the literature . it is seen in young to middle - aged adults in the fourth decade with very occasional cases in the pediatric age group . the preferred sites are the shoulder region as in our case as well as the trunk , extremities and the head and neck . grossly , the tumors have been described to be multilobular with pigmentation , irregular surface and firm nodules growing deep within the subcutaneous tissue . in this case , the cut surface of the fairly circumscribed tumor showed trabeculations and peculiar pigmentation in the subepidermal portion , which has not been described in the literature . it has to be distinguished from other pigmented cutaneous spindle cell lesions like pigmented neurofibroma , psammomatous melanotic schwannoma , neurocristic cutaneous hamartoma and desmoplastic malignant melanoma . however , our case had classic features of dfsp , and the above lesions were ruled out on histopathology itself . neuro - ectodermal differentiation or melanocytic colonization are the two proposed theories for histogenesis for the bednar tumor . it has also been reported in association of dermal melanocytosis ( nevus of ito ) , and based on the immunohistochemistry , the cell of origin is thought to be a neuromesenchymal cell . bednar tumor can rarely undergo malignant transformation in form of fibrosarcoma with repeated recurrences and distant metastasis . hence , a close follow - up of these cases is always necessary , which is being done in our case . to conclude , though rare , it is important for the histopathologists to be aware and recognize this unusual entity and distinguish it from other pigmented spindle cell lesions . ethics committee approval and patient consent has been obtained . published research complies with the guidelines for human studies and animal welfare regulations . the patient has given informed consent , and the study protocol has been approved by the institute 's committee on human research .
bednar tumor is an uncommon variant of dermatofibrosarcoma protuberans . also known as pigmented dermatofibrosarcoma protuberans , this tumor is of intermediate grade . it is seen in adults and has a predisposition to affect the shoulder region . we report a rare case of bednar tumor in a 40-year - old female patient . the diagnosis of bednar tumor must be considered while reporting pigmented subcutaneous spindle cell lesions .
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symptomatic adrenal adenoma usually presents with systemic symptoms . depending on the function of the adenoma , the patient can present with pheochromocytoma - like symptoms ; primary hyperaldosteronism and cushing syndrome ( weight gain , weakness , depression , and bruising ) . a 41 year - old lady presented with multiple metatarsal and phalangeal fractures of the both feet without significant injury . laproscopic r adrenalectomy was performed and histological study confirmed adrenal cortical adenoma with adjacent cortical atrophy suggestive of a functioning adenoma . symptomatic adrenal adenoma usually presents with systemic symptoms . depending on the function of the adenoma , the patient can present with pheochromocytoma - like symptoms ( palpitations , sweating , headache , abdominal pain and labile hypertension ) ; primary hyperaldosteronism ( hypertension and unprovoked hypokalemia ) and cushing syndrome ( weight gain , weakness , depression , and bruising ) . we reported a case of bilateral adrenal adenoma who presented with multiple insufficiency fracture of both feet . a 41 year - old lady presented to us with fracture of the base of her left fifth metatarsal after an inversion sprain ( fig 1 ) . she had sudden onset of left forefoot pain eight months later without any preceding injury . radiographs showed fracture of her left second metatarsal with callus formation ( fig 2 ) . radiographs showed old fractures of bases of bilateral fifth metatarsals , proximal phalanges of bilateral third toes , left second metatarsal with callus formation and crack fracture of the proximal phalanx of the left fourth toe ( fig 3 ) . she was newly diagnosed to have diabetes mellitus but the sensation of the feet was still intact . overnight dexamethasone suppression test ( ondst ) was 660nmol / l which was non suppressible . the serum cortisol reached peak level of 699 in low dose short synacthen test ( ldsst ) and the acth level was less than 1.6 . computed tomogram showed bilateral adrenal adenoma ( fig 4 ) . in order to plan for surgery , the functionality of the adenomas needed to be determined . radiographs showed old fractures of bases of bilateral fifth metatarsals , proximal phalanges of bilateral third toes , left second metatarsal with callus formation and crack fracture of the proximal phalanx of the left fourth toe . multiple metatarsal and phalangeal fractures of the foot of different ages without any significant injury are uncommon . this lady is a housewife and not involved in any activity that can contribute to fatigue fracture . dexa scan was performed as insufficiency fracture was suspected and the result confirmed the presence of osteoporosis . overnight dexamethasone suppression test ( ondst ) , urine cortisol level , low dose short synacthen test ( ldsst ) and the acth level was compatible with adrenal cushing syndrome . adrenal adenoma is a cause of cushing syndrome that is surgically treatable . the presence of adenoma in both adrenal glands needs further study of the functionality of the tumours for surgical planning . histological study confirmed adrenal cortical adenoma with adjacent cortical atrophy suggestive of a functioning adenoma . the clinical significance of this report is that it demonstrates the multiple phalangeal and metatarsal fractures can be the sole presentation of adrenal adenoma . causes of secondary osteoporosis should be investigated in pre - menopausal patient with insufficiency fracture of the feet .
introduction : symptomatic adrenal adenoma usually presents with systemic symptoms . depending on the function of the adenoma , the patient can present with pheochromocytoma - like symptoms ; primary hyperaldosteronism and cushing syndrome ( weight gain , weakness , depression , and bruising).case report : a 41 year - old lady presented with multiple metatarsal and phalangeal fractures of the both feet without significant injury . dexa scan showed evidence of osteoporosis . investigations showed that the picture was compatible with adrenal cushing syndrome . computed tomogram showed bilateral adrenal adenoma . adrenal cortex scintigraphy with np-59 scan showed hyperfunctioning right adrenal adenoma . laproscopic r adrenalectomy was performed and histological study confirmed adrenal cortical adenoma with adjacent cortical atrophy suggestive of a functioning adenoma . post - operatively , she was put on hydrocortisone replacement and recovered well.conclusion:adrenal adenoma can present with insufficiency fractures of the feet .
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we thank all the members of the cytoskeleton and cancer group and our collaborators , who collectively participated to the original manuscripts . this work was supported by grants from anr-13-jjc - jsv10005 , la ligue nationale contre le cancer and fondation arc pour la recherche sur le cancer . v.m . is supported by funding from equipe labellise ligue nationale contre le cancer 2011 .
accumulation of type i collagen fibrils in tumors is associated with an increased risk of metastasis . we recently demonstrated that the collagen sensor discoidin domain receptor 1 ( ddr1 ) interacts with type i collagen fibrils to allow proteolysis - based cancer cell invasion through the formation of a new class of invadosomes , termed linear invadosomes .
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in 2007 , a 14-year - old male killer whale at a marine park in san antonio , texas , usa , died suddenly without notable premonitory signs . on gross examination , mild multifocal meningeal hyperemia and petechial parenchymal hemorrhage focally extensive tan discoloration and fibrosis were present in the right accessory lung lobe with associated hemorrhage and congestion . tissues fixed in 10% buffered formalin were processed routinely and stained with hematoxylin and eosin for histologic examination . inflammatory lesions of the central nervous system were focused in gray matter of the medulla oblongata , pons , mesencephalan , and cerebellum . blood vessels demonstrated mild to moderate acute necrosis and lymphocytic and contained plasmacytic and neutrophilic infiltrates within vascular walls . encephalitis was characterized by perivascular lymphocytes and fewer plasma cells expanding the virchow - robbins spaces . predominant lesions in the lungs were areas of chronic and active abscessation amid a focally extensive area of mixed inflammation and fibrosis . they were characterized by central ulcerations with necrosis and a mixed inflammatory infiltrate surrounded by variable fibrosis and a rim of epithelial hyperplasia . changes in spleen , lymph node , and kidney included acute edema , congestion , and vascular dilation . conventional diagnostic assays were performed for aerobic , anaerobic , and fungal microbes in liver , lung , kidney , cerebrospinal fluid , and brain . the final diagnosis was fulminant peracute bacteremia and septicemia secondary to a primary viral infection associated with nonsuppurative encephalitis . published etiologic considerations for cetacean nonsuppurative encephalitis include morbillivirus and protozoal infections ( 2 ) . a dna microarray with highly conserved sequences from > 1,000 viruses was selected to screen for known and novel viruses ( 3 ) . total rna was extracted from brain tissue and hybridized to a microarray as described ( 4 ) . analysis of the resulting hybridization pattern demonstrated a strong hybridization signal to many oligonucleotide probes on the microarray from the family flaviviridae , in particular to wnv . consensus reverse transcription pcr primers ( 5 ) targeting wnv were used to confirm the microarray results . hq610502 ) yielded a sequence with 99% nt identity and 100% aa acid identity to wnv strain ok03 ( genbank accession no . eu155484.1 ) , a strain originally identified in oklahoma , usa . to further support a wnv diagnosis , we performed immunohistochemical staining on brain tissue . rockville , md , usa ) with peroxidase - tagged goat antirabbit immunoglobulin g ( dakocytomation , carpinteria , ca , usa ) bridge and 3-amino-9-ethylcarbazole ( dakocytomation ) as the chromogen . this staining demonstrated abundant wnv antigen within the cytoplasm of a small number of neurons and glial cells and in fewer macrophages in the brain tissue ( figure ) . brain specimen from killer whale ( orcinus orca ) with west nile virus infection that died at a marine park , san antonio , texas , usa , 2007 . neurons and glial cells demonstrate abundant intracytoplasmic west nile viras antigen . we evaluated wnv exposure within the same cohort , as well as a geographically distant cohort of whales by using serologic testing . all testing was performed at the same laboratory by using a standard plaque - reduction neutralization test . in this assay , a 90% neutralization cutoff was used ( 6 ) . a 90% plaque - reduction titer serum from the affected whale and 5 cohort killer whales from the same marine park in san antonio as well as 5 whales housed at another facility in orlando , florida , usa , were evaluated . in each facility , the animals have regular contact with each other . the facilities are geographically separated so the animals do not have exposure to those in the other park . all 6 animals from texas had 90% plaque - reduction titers > 10 , ranging from 40 to 80 . these findings broaden the known host tropism of wnv to include cetaceans in addition to previously known pinnipeds . although we can not definitively attribute the cause of death of this whale to wnv , the observed lesions are consistent with those caused by wnv in other animals . the serologic results demonstrate that subclinical infections can occur and that exposure can be variable . both bexar county , texas , and orange county , florida , have had wnv in wildlife since 2002 . mosquito management practices are similar in both facilities and have been expanded since this diagnosis . differences in wnv prevalence or mosquito numbers may have played a role in the different serologic results . health evaluations of free - ranging and captive cetaceans should include wnv serology to assess exposure rates . this report focuses on killer whales , but the loafing behavior ( stationary positioning at the water s surface ) is commonly seen in many coastal dolphins , thereby increasing the likelihood of mosquito bites and exposure to wnv . serologic screening of bottlenose dolphins ( tursiops truncatus ) from the indian river lagoon demonstrated wnv titers ( 7 ) . as with many species of birds and mammals , wnv infection carries a risk for zoonotic transmission . until the implications of this infection in marine mammals are better understood , biologists and veterinarians working with cetaceans should consider this possibility . potential viral shedding can occur through the oropharygeal cavity and feces as well as through blood and organs during necropsies . finally , our study demonstrates the broad applicability of using panviral microarray - based diagnostics . even though pcr diagnostics are well developed for wnv , the agent was not initially considered as a potential pathogen in this species . panviral microarray can be used not only to identify novel viruses but also to detect unsuspected agents .
in 2007 , nonsuppurative encephalitis was identified in a killer whale at a texas , usa , marine park . panviral dna microarray of brain tissue suggested west nile virus ( wnv ) ; wnv was confirmed by reverse transcription pcr and sequencing . immunohistochemistry demonstrated wnv antigen within neurons . wnv should be considered in cases of encephalitis in cetaceans .
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gastrointestinal stromal tumors ( gist ) represent less than 1% of all primary tumors of the gastrointestinal tract ( git ) and commonly affect patients in the middle and older age groups with equal gender distribution . . their sub mucosal location can produce local obstructive symptoms , particularly when arising in the esophagus or the small intestine . most gists usually present with vague upper abdominal pain , fullness or gastrointestinal ( gi ) bleeding . sometimes they are found incidentally during barium studies , endoscopy or abdominal scans performed for other reasons . a 40-year - old man presented with a history of rectal bleeding for two days , with a reported loss of more than 1 liter of fresh blood . despite intensive fluid management with whole blood and normal saline , colonoscopy was not attempted because of continuous bleeding . tc-99 m labeled red blood cell ( rbc ) scintigraphy [ figure 1 ] showed accumulation and movement of tracer in the mid - abdomen , corresponding to the region of the jejunal loops . a diagnosis of active gastro - intestinal bleed ( likely to be from the jejunum ) was made , and the patient taken up for exploratory laparotomy . a polypoidal exophytic mass about 4 cm in size was found in the jejunal wall , 40 cm from the duodeno - jejunal junction . the mass along with the adjoining jejunal loops was resected , followed by end - to - end anastomosis . gross examination of the specimen [ figure 2a and b ] showed a polypoidal mass ( measuring 3.2 cm in its largest dimension ) arising from the serosal aspect , producing a bulge in the central part of the mucosa , which was ulcerated and could be the possible source of bleeding . microscopic examination [ figure 2c ] showed a well - circumscribed tumor in the submucosa . the mass showed a spindle cell tumor arranged in long interlacing fascicles with characteristic perivascular arrangement of tumor cells . the tumor cells were moderately pleomorphic with mitotic rate of 2/50 high power field ( hpf ) . following surgery , the patient made a satisfactory recovery and was discharged on the seventh post - operative day . dynamic scintigraphy using tc-99 m labelled red blood cells showing accumulation and movement of tracer in the mid - abdomen ( arrows ) the resected specimen from the jejunum ( a and b ) a polypoidal mass arising from the serosal aspect , with an ulcerated bulge in the central part of the mucosa ( c ) microscopic examination shows a spindle cell tumor arranged in long interlacing fascicles with perivascular arrangement of tumor cells ( d ) immunoperoxidase staining showing cd-117 positivity gists are derived from cajal cells or their precursors and most commonly occur after the age of 50 years in the stomach ( 60% ) , jejunum and ileum ( 30% ) , duodenum ( 4 - 5% ) , rectum ( 4% ) , colon and appendix ( 1 - 2% ) , and esophagus ( < 1% ) , rarely as apparent primary extra - gastrointestinal tumors in the vicinity of the stomach or intestines . the role of labeled rbc scintigraphy in acute gastro - intestinal bleeding is well - established and its application in rare cases has also been reported . however , few authors have discussed the role of tc-99 m rbc scintigraphy in discovering a bleeding gist . surgery is the primary treatment of choice for patients with localized or potentially resectable gist lesions and is recommended if bleeding is present . the scottish intercollegiate guidelines network ( sign ) recommends that nuclear scintigraphy should be considered to assist in localization of bleeding in patients with significant recent lower gi hemorrhage . although colonoscopy is the initial diagnostic modality of choice to localize the bleeding site for patients presenting with acute severe hematochezia , it is difficult when patients present with profuse bleeding . in these situations arteriography or labeled rbc scintigraphy is warranted . as a non - invasive investigation that requires no specific patient preparation , there is also no need of any iodinated contrast that may cause contrast - nephropathy in acutely ill - patients . since the risk of malignancy was low in our patient , he has been kept under follow - up with no adjuvant treatment . this report highlights the advantage of this non - invasive investigation in hemodynamically unstable patients by detecting and localizing the site of an active gi bleed .
the role of 99m - technetium labeled red blood cell ( rbc ) scintigraphy in acute gastro - intestinal bleed is well - established . the authors report a case of a bleeding gastrointestinal stromal tumor ( gist ) incidentally discovered on tc-99 m rbc scintigraphy .
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retinitis pigmentosa ( rp ) was defined as a group of hereditary disorders that diffusely involve photoreceptor and pigment epithelial function characterized by progressive visual field loss and abnormal electroretinogram . rp also has changes at vitreoretinal interface , such as vitreoretinal traction and preretinal membrane , which causes macular disorders such as cystoids macular edema and macular hole . we report a case of vitreoretinal traction syndrome associated with rp , which spontaneously resolved after posterior vitreous detachment ( pvd ) . a 81-year - old woman presented with bilateral loss of peripheral vision and defective dark adaptation 15 years before . the best - corrected visual acuity was 20/28 and 20/28 in the right and left eyes , respectively . slit - lamp examination showed bilateral retinal arteriolar narrowing and intraretinal pigment deposition ( bone spicule pigmentation ) in both eyes ( figure 1a ) . the visual fields of the patient were markedly constricted to the central 10 in both eyes ( figure 2 ) . on june 2 , 2006 , retinal detachment was detected in the nasal periphery in the right eye ( figures 1b and 1c ) . at that time , pvd was confirmed with weiss ring ( figure 3 ) . fluorescein angiography showed a hyperfluorescence due to atrophy of retinal pigment epithelium but no active leakage in the detached area ( figure 1c ) . the detached area was within the area where the visual field was lost , and we observed a clinical course of this eye without any treatment . retinal detachment was kept localized and not extended . five months later , pvd further progressed beyond the arcade area , accompanying retinal hemorrhage around the detached retina . in this case , an eye with rp was complicated with tractional retinal detachment , possibly associated with increased vitreoretinal traction secondary to the incidence of pvd . the retinal detachment was limited within the area where the visual field was lost and was not progressive . eight months later , spontaneous resolution of tractional retinal detachment resulted from progression of pvd , which was suggested by transiently increased traction - related retinal hemorrhage . eyes with rp may have abnormal liquefaction of vitreous and altered distribution of vitreoretinal adhesion . the incidence of pvd in the posterior pole might result in the focal remarkable traction in the peripheral retina and tractional retinal detachment . eight months later , the retinal detachment was resolved because local vitreoretinal traction was weakened with extention of pvd beyond the arcade vessels . rp has vitreomacular traction - related pathologic events involving vitreomacular traction syndrome , cystoids macular edema , and macular hole.13 such changes in this patient with rp might result in a tractional retinal detachment . rani et al4 reported that a 50-year - old man underwent a tractional retinal detachment involving the posterior pole with a taut posterior hyaloids surface . in this study , vitrectomy was not performed because the patient opted for no surgical intervention in view of poor prognosis . on the other hand , it was reported that vitrectomy was effective for vitreous opacity,5 cystoid macular edema,6 exudative retinal detachment,7 and macular holes8 in rp . however , regarding the indication to perform a vitrectomy , the balance of the expected gain in visual function and usual risks of vitrectomy and rp - related risks , such as phototoxicity worsening of visual field loss , and cystoid macular edema . it might be a choice that a patient with tractional retinal detachment in rp is observed without surgical treatment , if the detached area is not progressive .
we present a case of retinitis pigmentosa with vitreoretinal traction - associated retinal detachment . the retinal detachment was detected in the nasal periphery . no retinal breaks and no active vascular leakage were observed by fundus scopy and fluorescein angiography , respectively . however , 8 months later , the tractional retinal detachment was spontaneously resolved with posterior vitreous detachment .
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metastases to the breast from extramammary malignancies are rare and account for 0.43% of all breast malignancies . lymphoma , melanoma , sarcoma , lung carcinoma and ovarian tumor are common extramammary primary malignancies that metastasize to the breast [ 1 - 3 ] . cases of metastasis from primary colorectal neoplasm to the breast is extremely rare . in searching medline and embase including non - english literature , and handsearching the references , we describe the features of metastatic breast cancer from adenocarcinoma of the colon in a 63-year - old woman . a 63-year - old woman was referred to a breast surgeon due to a mass with suspicious metastatic axillary lymph node discovered incidentally on positron emission tomography - computed tomography for follow - up study after colon cancer surgery without other metastatic lesion ( fig . four years ago , she underwent a subtotal colectomy for stage t3n1m0 sigmoid colon cancer with colonic obstruction and subsequent chemotherapy with 5-fluorouracil and leucovorine . two years after the operation , a 2.8 cm sized solitary metastatic lung nodule on the right middle lobe was identified on chest computed tomography for follow - up study for which she underwent pulmonary wedge resection , followed by chemotherapy with oxaliplatin and 5-fluorouracil . physical examination revealed a 2 cm sized firm nodule in the lower inner quadrant of the right breast without evidence of axillary or supraclavicular lymphadenopathy . the mass was not identified on mammography , but ultrasonography showed 1.1 cm sized mass in the lower inner quadrant of the right breast without enlarged lymph node in the ipsilateral axilla . serum tumor markers were all in normal ranges ( carcinoembryonic antigen 4.2 ng / ml , carbohydrate antigen 15 - 3 6.2 u / ml ) . subsequent magnetic resonance imaging showed 1.8 cm sized ill - defined irregular shaped enhancing mass in the lower inner quadrant of the right breast and enlarged lymph node at the ipsilateral axilla compatible with a nodule suspicious for metastasis . breast specific gamma imaging using tc - mibi showed a lesion with increased uptake in the lower inner portion of the right breast with no lesion uptake in the axilla . the tumor cells in the breast mass were cuboidal to columnar with eosinophilic cytoplasm and pseudostratification , compatible with the features of adenocarcinoma of colorectal origin ( fig . immunohistochemical study revealed the following results : tumor cells were strongly positive for cdx2 , cytokeratin ( ck)20 and negative for ck7 , thyroid transcription factor-1 , estrogen receptor , progesterone receptor , c - erbb2 ( fig . 4 ) . these findings are consistent with metastatic adenocarcinoma of the colon or rectum . primary breast cancer is one of the most common malignancies and leading cause of death from malignant disease in women . a malignant mass in the breast can easily be misinterpreted as breast cancer and inappropriate surgical intervention could be carried out . although there are few instances of breast metastasis from colorectal malignancy , correct diagnosis is crucial to avoid unnecessary surgical intervention . generally , metastases to the breast from extramammary malignancies are characterized by rapidly growing , mobile masses that are easily palpable but do not cause overlying skin or nipple retraction , or bloody nipple discharge . core biopsy allows histological assessment of the tissue , helping in identifying the origin of the carcinoma . on the histologic finding , the periductal and perilobular location of the tumor with the absence of in situ ductal carcinoma in the surrounding breast specimen favor a metastasis . immunohistochemistry enables a more confident differentiation between a primary breast adenocarcinoma and a colorectal adenocarcinoma metastasis to the breast . for our patient , diagnosis was finally reached after immunohistochemistry finding . the majority of colorectal carcinomas are usually ck7-negative and ck20-positive , while the majority of primary breast carcinomas is ck7-positive and ck20-negative . and the positive immunostaining for cdx2 is a highly sensitive and specific marker of colorectal carcinoma . our case showed these findings as well ; tumor cells were positive for cdx2 , ck20 and negative for ck7 . recently , some authors have suggested that the expression of ck20 can be identified in primary breast cancer in contrast to the conventional immunohistochemistry results . so , clinicians and pathologists should examine multiple immunohistochemistry markers and combine them as an approach for final diagnosis . according to recent reports , the management of metastatic breast mass from colorectal adenocarcinoma should be diagnostic and palliative . advocated that surgical excision should be avoided in the view of short life expectancy and risk of seeding to the skin . suggested that excisional biopsy was usually appropriate and provided adequate local control . if the diagnosis for the breast mass is unclear , unnecessary intervention , for example mastectomy or lymph node dissection of axilla , can be performed . therefore , when breast mass is identified incidentally on radiologic study during follow - up after operation for colorectal carcinoma , clinicians should keep the possibility of breast metastasis from colorectal adenocarcinoma in mind . additionally , periodical examination of the breast after colorectal cancer surgery may be helpful to detect the metastasis early .
a 63-year - old woman was referred to a breast surgeon with a breast mass discovered incidentally during follow - up study after colon cancer surgery . invasive adenocarcinoma was revealed on core needle biopsy . wide excision of the breast including the tumor was performed . on standard histological examination the tumor showed features of moderately differentiated adenocarcinoma . the immunohistochemistry study revealed positive results for cytokeratin ( ck)20 and cdx2 , but negative for ck7 . these are typical characteristics for colon cancer . considering her history of subtotal colectomy for sigmoid colon cancer , it is presumable that the mass in the breast was of colonic origin , and it was an extremely rare case of metastasis to the breast from primary colorectal neoplasm . although the instance is rare , clinicians should keep the possibility of breast metastasis from colorectal cancer in mind for early and correct diagnosis .
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a 41-year - old woman with a history of liver cirrhosis presented with recurrent portal hypertension and bleeding from esophageal varices . three months prior to this admission , a tips was created with a 10 mm 7 cm wallgraft ( boston scientific , natick , ma ) . however , the stent 's anticoagulation was inadequate ( international normalized ratio [ inr ] : 1.02 - 1.21 ) after placement . the initial attempts at recanalization of the completely occluded stent via the transjugular approach were unsuccessful . therefore , a percutaneous transsplenic approach guided by ultrasound was used to gain entry to the splenic vein and portal vein for catheterization of the occluded stent . to perform the ultrasound guided puncture of the splenic vein through the spleen , we used a skater introducer set ( angiotech , pbn medicals , denmark ) . a 22-guage chiba needle was introduced into the splenic vein , then a 0.018 inch guide wire was placed through the chiba needle into the splenic vein , and a 6-fr dilator system was placed over the wire . we then exchanged the 0.018 inch guide wire with a 0.035 inch guide wire , followed by a 5-fr introducer sheath and a 4-fr kmp catheter ( cook medical inc . , however , residual thrombosis of the stent was noted in combination with occlusion of the hepatic vein ( fig . so , we performed catheter - directed intrathrombus thrombolysis with urokinase ( taiwan green cross co. , taipei , taiwan ) . in brief , the distal end of a 4 fr rc1 catheter ( cook medical inc . , bloomington , in ) with home - made side holes ( n = 10 , made by needle - sticks into the distal 5-cm segment of the catheter ) was wedged into the thrombosed tips stent . then , catheter - directed intrathrombus thrombolysis with a solution of 48 vials of urokinase ( 60,000 u / vial ) in 500 ml normal saline at a drip rate of 21 ml / hr ( i.e. , 2 vials / hr = 2,000 u recanalization was performed in the following day and a wallstent endoprosthesis ( 14 mm 9 cm , boston scientific , natick , ma ) was placed , resulting in the successful opening of the shunt ( fig . the catheters were removed and the transsplenic tract was embolized with 4 serial coils ( mwce-35 - 8/4-tornado , cook medical inc . , she is doing well five months after discharge and an ultrasound study indicates that the stent remains patent . transjugular intrahepatic portosystemic shunt is now considered the procedure of choice for accessing the portal system for management of refractory variceal bleeding . certain complications , including a high reintervention rate due to occlusion of the stent , have raised concerns regarding its application ( 2 , 3 ) . yet recent reports have suggested that using a covered prosthesis improves the tips patency to longer than two years ( 4 , 5 ) . although a covered stent had been employed in the patient presented here , thrombosis occurred early ( i.e. , within 3 months ) . transjugular intrahepatic portosystemic shunt failure requires recanalization of the stent or placement of serial or parallel stents ( 6 , 7 ) . neointimal hyperplasia at the ends of the stent may completely occlude the outflow hepatic venous tract ( 1 ) and this can make reintervention difficult . as in this case , an alternative route is needed when attempts at recanalization via a conventional transjugular approach fail . the splenic vein drains directly to the portal vein , and its size and straight route make this approach an ideal option ( 5 , 8) . the transsplenic approach has been used for other indications , as reviewed by tuite et al . ( 8) , but we are unaware of any other report of using the percutaneous transsplenic approach to recanalize an occluded tips stent . additionally , we believe that the overnight infusion of a thrombolytic agent contributed to successful recanalization in our case ( 9 ) . concerns regarding post - procedural splenic bleeding and the reported difficulty some clinicians have experienced negotiating the pathway have limited the use of the transsplenic route . a cautious , image - guided approach reduces the risk of splenic hemorrhage ( 5 , 8) and obliteration of the transsplenic tract with coils upon completion of the procedure also assists in preventing post - procedural bleeding ( 8) . no significant splenic bleeding occurred in our patient , despite her liver impairment , during or following the intervention . subtherapeutic anticoagulation ( inr < 2 ) probably contributed to the thrombosis and subsequent occlusion in this patient . there is no routine anticoagulation protocol to follow for tips patients . we prescribe anticoagulation for only the patients with tips dysfunction ; however , there is no reference available for the effectiveness of this regime . as a general rule for preventing thromboembolism , chronic oral anticoagulant therapy with a vitamin k antagonist is administered and the dose is adjusted to achieve an inr of 2.0 to 3.0 ( 10 ) . in summary , we have reported on a rare case of using the transsplenic approach for recanalization of a tips stent by thrombolysis . transsplenic entry to the portal system is useful in recanalizing a stent that is difficult to reach through the usual transjugular route .
a 41-year - old woman with liver cirrhosis had recurrent portal hypertension and bleeding from esophageal varices due to complete occlusion of a previously inserted transjugular intrahepatic portosystemic shunt stent . because recanalization of the stent by the transjugular approach was unsuccessful , ultrasound - guided entry to the splenic vein and portal vein was used . after catheter - directed intrathrombus thrombolysis , successful opening of the stent was achieved and a stent was placed . we herein report a rare case in which thrombolysis and recanalization of a tips stent were performed via a percutaneous transhepatic approach .
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in the current issue of critical care , de prost and colleagues evaluated the impact of protective and injurious ventilation strategies on lung neutrophil distribution and activation in sheep 2 hours after endotoxin infusion . the protective ventilation strategy consisted of 8 ml / kg tidal volume and titration of positive end - expiratory pressure ( peep ) to achieve a plateau pressure of 30 cm h2o . peep was not applied in the injurious ventilation protocol , and tidal volumes of 14 to 18 ml / kg were delivered . positron emission tomography ( pet ) imaging and n-[nitrogen]-saline infusion were performed to evaluate regional lung perfusion and shunt fraction . cellular metabolic activities were measured by using an f - fluorodeoxyglucose ( f - fdg ) infusion protocol , and lung leukocyte infiltration was determined by histological analysis . their results show that protective ventilation was associated with better gas exchange and lower shunt fraction in dependent lung regions prior to endotoxin infusion . endotoxin infusion worsened gas exchange in both groups , but less so in sheep receiving protective ventilation . protective ventilation also attenuated f - fdg uptake and phosphorylation after endotoxin infusion , particularly in dependent areas of the lungs . the authors conclude that a protective ventilation strategy that optimizes alveolar recruitment and minimizes alveolar distension may mitigate neutrophil activation in the lung , particularly in dependent areas , during early experimental acute lung injury ( ali ) . use of the sheep model in this study had several advantages . with a size comparable to that of the human , the ovine model allowed an analysis of physiological endpoints that are clinically relevant and permitted the application of ventilation strategies that are similar to those used in clinical practice . the tidal volumes and peep applied in the protective strategy are similar to those advocated for use in the clinical setting . alternatively , a study by takeuchi and colleagues described a method of pressure - volume curve analysis to identify the most appropriate peep during mechanical ventilation in an adult sheep model of ali , which may further optimize lung protection and would be interesting to apply in the model used by de prost and colleagues . the endotoxin infusion model has the advantage of being reproducible and easily titrated and is known to induce neutrophil accumulation in the lungs . however , the protocol appears to have induced minimal histological evidence of lung injury as indicated by lung injury scores of 0 to 1 ( on a scale of 0 to 4 ) in both groups . given the lack of demonstrable lung injury at the histological level , it is possible that differences in normal lung recruitment and hemodynamics contributed to the dissimilarities in gas exchange and shunt fraction observed between groups . however , changes in alveolar - capillary integrity that were not detectable by light microscopy may also be present early after endotoxin infusion and could have contributed to the observed gas exchange perturbations . evidence indicates that dysregulated inflammation and the inappropriate accumulation and activation of leukocytes , especially neutrophils , contribute to the pathogenesis of ali . furthermore , investigators have postulated that protective ventilation strategies decrease regional lung inflammation in subjects with ali . the assessment of cellular metabolic activity by using pet imaging and f - fdg infusion along with the evaluation of lung perfusion and ventilation , as performed by de prost and colleagues , is an informative , non - invasive approach that provides useful data regarding regional differences in cellular metabolic rate . the technique may have practical utility since studies have shown that evaluation of f - fdg uptake may be valuable in predicting respiratory failure and evaluating therapy in clinical and experimental models of ali [ 8 - 10 ] . the present study extends previous reports by documenting the impact of ventilation strategies on regional metabolic activity and neutrophil accumulation early during the course of ali . although differences in cellular metabolic activity were observed between groups , neutrophil accumulation in the lungs was not different when sheep receiving protective or injurious ventilation were compared . the authors interpret that finding as possibly being indicative of increased neutrophil activation in sheep receiving injurious ventilation . that conclusion is based , in part , on previous studies that showed neutrophil activation to be the primary factor contributing to increased f - fdg uptake and phosphorylation during ali . however , as noted by the authors , it is unclear whether the alterations in f - fdg uptake and phosphorylation observed in their analysis are entirely specific for neutrophils . it is possible that the metabolic rates of other leukocyte and non - leukocyte cell populations were affected . evidence indicates that alterations in macrophage , epithelial , and endothelial cell functions occur during ali . further studies are needed to identify the cell populations that are affected and the functional importance of the observed alterations . overall , the study by de prost and colleagues provides new insights into the impact of ventilation strategies on regional cellular metabolic activity during early ali . more investigation is needed to extrapolate their findings into the clinical setting and determine the functional importance of their findings . it is hoped that the group will perform follow - up studies to determine whether the observed changes in regional leukocyte activation are predictive of progressive respiratory failure and pulmonary injury as well as better define the specific cell populations involved . 18f - fdg : f - fluorodeoxyglucose ; ali : acute lung injury ; peep : positive end - expiratory pressure ; pet : positron emission tomography .
protective mechanical ventilation is currently accepted as a key strategy for the management of acute lung injury ( ali ) and its most severe form , acute respiratory distress syndrome . the study by de prost and colleagues in the current issue of critical care provides new insights into the impact of ventilation strategies on pulmonary function , gas exchange , and regional cellular metabolic activity during early ali in sheep . the group reports that a protective ventilation strategy may attenuate neutrophil activation in dependent lung regions during early experimental ali . this is an innovative report that provides the basis for further study .
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in this issue of critical care , lesur and colleagues report the differential profile of stress response in septic and non - septic patients . adrenocorticotropic hormone ( acth ) and acth / cortisol ratio were lower whereas baseline cortisol , procalcitonin ( pct ) , and stromal cellderived factor-1-alpha ( sdf-1 ) were higher in septic patients than in non - septic patients . furthermore , a probability score incorporating acth , cortisol , and pct by multivariate logistic regression analysis predicted sepsis better than sepsis score or pct did . the response of hypothalamic - pituitary - adrenal ( hpa ) axis to the sustained stress of severe illness has been the focus of many studies in recent years . in healthy subjects , cortisol secretion by adrenal cortex is regulated by acth secretion by the pituitary , which in turn is regulated primarily by hypothalamic secretion of corticotropinreleasing hormone ( crh ) , whereas cortisol inhibits both acth and crh production through a negative feedback [ 2 - 4 ] . however , hpa stress response during sepsis is much more complex and is poorly defined . plasma cortisol levels may be low , normal , or high in sepsis but nonetheless inadequate to control the inflammatory response and meet the elevated metabolic demand . this effect is termed relative adrenal insufficiency ( rai ) , also known as critical illness - related corticosteroid insufficiency ( circi ) . other factors are involved in the hpa stress response during sepsis . in rodent models , arginine vasopressin ( avp ) was shown to increase endogenous adrenal acth secretion . apelin , a neuropeptide originating from paraventricular and supraopitc nuclei , acts on hpa axis regulation by releasing crh and acth and by reducing avp . copeptin , a 39-amino acid glycopeptide , makes up the pre - pro - vasopressin molecule together with neurophysin ii and avp and serves as a surrogate marker to assess avp plasma concentrations in septic shock . in normal rats , the chemokine sdf-1 and its receptor colocalize with avp in magnocellular neurosecreatory neurons , resulting in an inhibition of avp - induced release . the dissociation of acth and cortisol levels in late phase ( lasting many days to weeks ) , which is different from that of the acute phase ( hours to a few days ) of an illness , indicates that alternative pathways not mediated by acth are involved . limited clinical studies prove that the dehydroepiandrosterone ( dhea ) level is very low in septic shock , whereas its sulphate and the cortisol / dhea ratio might be prognostic markers and signs of exhausted adrenal reserve in critical illness . tissue resistance to corticosteroid action may also play an important role in sepsis and can be caused by either defects in the corticosteroid receptor or postreceptor alterations and may not be defined accurately based on plasma cortisol levels . despite the uncertainty of the definition and diagnostic criteria , clinical studies show that patients with rai are at a significantly higher risk of hospital mortality and this has been the driver for corticosteroid replacement therapy in severe sepsis / septic shock [ 2 - 4 ] . this is not surprising upon review of the aforementioned complexity and unknowns of hpa stress response . furthermore , it must be acknowledged that the decision to treat with stress - dose corticosteroids is based on clinical criteria rather than on the inconclusive results of adrenal function tests . the dissociation of acth and cortisol levels observed in the study is more compatible with neuroendocrine characteristics of prolonged critical illness , although the authors claimed to include patients within the first 24 hours of admission . the clinical significance of the predictive model is hindered by the unavailability of acth or cortisol measurements at the bedside and by the fact that the predictive value of sepsis score or pct has not been consistently validated in clinical trials . despite all of these limitations , the study by lesur and colleagues undoubtedly expands our understanding of the complex neuroendocrine network regulating hpa stress response in human sepsis . we believe that further investigation into the mechanism is warranted before we plan a successful strategy for corticosteroid replacement in sepsis . acth : adrenocorticotropic hormone ; avp : arginine vasopressin ; circi : critical illness - related corticosteroid insufficiency ; crh : corticotropin - releasing hormone ; dhea : dehydroepiandrosterone ; hpa : hypothalamic - pituitaryadrenal ; pct : procalcitonin ; rai : relative adrenal insufficiency ; sdf-1 : stromal cell - derived factor-1-alpha .
the hypothalamic - pituitary - adrenal ( hpa ) axis response in sepsis remains to be elucidated . apart from corticotropin - releasing hormone , adrenocorticotropic hormone , and cortisol , many other neuroendocrine factors participate in the regulation of hpa stress response . the hpa response to acute and chronic illness exerts a biphasic profile . tissue corticosteroid resistance may also play an important role . all of these add to the complexity of the concept of relative adrenal insufficiency ' and may account for the difficulty of clinical diagnosis and for the conflicting results of corticosteroid replacement therapy in severe sepsis / septic shock . the study by lesur and colleagues expands our understanding of the mechanism , and further study of hpa stress response is warranted .
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a 65-year - old man without any symptoms was referred for an operation on the aortic root and ascending aortic aneurysm . he had undergone a total correction of tetralogy of fallot ( tof ) and aortic valve replacement with mechanical valvular prosthesis 22 years earlier ( in 1987 , at the age of forty - three ) . in august 2009 , a huge aneurysmal dilatation ( 9 cm ) of aortic root and proximal ascending aorta , severe tricuspid regurgitation , and good left ventricular ejection fraction were found on echocardiography . computed tomography showed a huge saccular aneurysm of the aortic root and proximal ascending thoracic aorta ( fig . 1 ) . the patient underwent a bentall procedure involving a composite conduit with a mechanical valvular prosthesis . the operation also included pulmonary valve replacement using a bioprosthesis , tricuspid annuloplasty , subaortic pannus resection , maze procedure , and coronary artery bypass surgery ( saphenous vein to proximal right coronary artery ) . because the right coronary artery orifice was deviated to the left side and was close to the aortic annulus , coronary artery bypass surgery was chosen despite availability of the button technique . after the operation , there was the microscopic finding of cystic medial degeneration in the ascending aortic wall ( fig . he was discharged on his 37th postoperative day . at follow - up , he remains well 17 months after this latest procedure . in 1997 , dodds and colleagues described the first series report about progressive aortic regurgitation and aortic root dilatation after complete , uncomplicated repair of tof . a long - standing volume overload of the aortic root may cause aortic root dilatation in adults with a repaired tof . the right to left shunt through the ventricular septal defect in unrepaired tof increases the volume overload effect on the aortic root . another causative mechanism for progressive aortic root dilatation intrinsic histological changes were found in tof patients ; including medionecrosis , fibrosis , cystic medial necrosis , elastic fragmentation and elastic lamellae disruption . our patient had initial reparative surgery at the age of forty - three in 1987 . the phrase of the enlarged aorta was described in the record of the first operation . , we did not know why correction was not performed in the initial operation . in 2002 , an operation for ascending aortic aneurysm was recommended , but he refused any surgery at that time . the pathologic conditions of aortic root and ascending aorta in repaired tof are no longer a benign problem . in recent reports , these are the first reports of aortic dissection in tetralogy of fallot . after an initial curative operation for tof , meticulous monitoring of aortic root and ascending
surgical repair of the tetralogy of fallot is one of the most successful operations in the treatment of congenital heart diseases . we report the case of a 65-year - old man who had an aortic valve replacement at the time of complete repair of the tetralogy of fallot at the age of forty - three . he subsequently had progressive aortic root and ascending aorta dilation to 9 cm . the aortic root and ascending aorta replacement was done using a composite valve - graft and was performed along with other procedures . thus , meticulous follow - up of aortic root and ascending aorta after corrective surgery for tetralogy of fallot is recommended following initial curative surgery .
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in central europe patients with muscle - invasive bladder cancer ( mibc ) have relatively poor prognosis . this includes also patients initially undergoing radical surgical treatment . in order to find underlying reasons , we have previously estimated the timing of radical cystectomy in a multicentre study involving 575 polish patients . however , one of the most important limitations of the abovementioned study was the inclusion criterion of being cystectomised instead of being diagnosed with mibc . consequently , the data on final treatment in the whole population of mibc patients are still limited . within this short communication the aim of this study was to describe patterns of care in polish patients with newly diagnosed mibc . this is a multicentre retrospective cohort study involving 296 consecutive patients with primary mibc diagnosed in the years 20122013 in 13 polish urological centres . in all patients the diagnosis was made based on histological examination of surgical specimens from transurethral resection of the bladder tumour . the mean age of the cohort was 72.1 years and male - to - female ratio was 3.2 : 1 ( 225 vs. 71 ) . differences between cystectomised and non - cystectomised patients were evaluated with u mann - whitney test and test for quantitative and qualitative variables , respectively . comparison of patients depending on qualification for radical cystectomy table presents absolute , median , or percentage values . haemoglobin ; rc radical cystectomy data on nicotine use was available only in 220 patients ( 77.2% of cohort ) among 121/285 ( 42.5% ) patients disqualified from rc , 32/121 ( 26.4% ) patients were qualified for a second step of transurethral resection of the bladder tumour ( turbt ) intentionally followed by systemic chemotherapy , four ( 3.3% ) patients after complete turbt were qualified for adjuvant intravesical chemotherapy only , while the remaining 85 ( 70.2% ) patients were qualified for palliative treatment in the form of chemotherapy and/or radiotherapy and/or best supportive care . despite poor outcomes of treatment of patients with mibc in central europe , data on patterns of surgical and medical management , as well as its quality , are still unavailable . we performed a retrospective study aimed at the description of further treatment in patients diagnosed with primary mibc . the most important finding of our analysis is the high percentage of patients qualified for radical treatment . a clinically important fact is that that these numbers would probably be higher if we excluded from the analysis patients with metastatic disease , who are not candidates for rc by definition . available data on patterns of care in mibc patients published in the last 10 years present significantly lower rates of curative treatment , covering 2152.5% of patients [ 47 ] . however , the studies cited above included more patients and/or were based on cancer or national registries . some portion of patients included in these analyses was treated with radiotherapy alone , which nowadays is not regarded as a radical approach . we have also found that patients disqualified from curative treatment were older , had lower bmi values , lower haemoglobin concentration , and declared lower rate of nicotine abuse and shorter time interval between first symptom to diagnosis . while age alone should not influence clinical decisions , it is suggested that older mibc patients are less frequently qualified for radical surgery , and it is well established that the morbidity related to rc is increased within this group [ 912 ] . low haemoglobin concentration , as well as malnutrition is associated with shorter survival after rc [ 9 , 13 , 14 ] . moreover , abnormal bmi value increases the risk of surgical complications [ 12 , 15 , 16 ] . our findings on nicotine use and time from first symptom to diagnosis are both surprising and unexplainable . considering the pathogenesis and clinical course of the disease , one can suspect that these results are fortuitous . finally , they are of no practical significance . the study 's strengths are its multi - institutional character , involvement of both academic and non - academic urological departments , and enrolment of a representative cohort of patients . the most important limitation of the study is the lack of data on lymph node and distant metastases . as available clinical staging is limited to regional status , among patients disqualified from surgery there are both patients unfit for surgery and patients with initially metastatic disease . their differentiation in the present study was not performed . because the majority of polish patients with primary mibc receive curative treatment , the stage of the disease alone seems not to be the leading cause of poor survival . however , the appropriateness of qualification for rc and treatment quality needs to be assessed for a final conclusion on the factors influencing outcomes of treatment in poland . the results presented within this paper come from post hoc analysis of data collected during a multicentre study aimed at oncological characterisation of a large cohort of polish patients with primary urothelial carcinoma of the bladder . the study was produced under the auspices of the residents section of the polish urological association . all the investigators , but two ( m.s . and p.r . ) , were urologists in training .
a potential reason for poor survival among patients with muscle - invasive bladder cancer ( mibc ) in poland is initial disqualification from curative treatment due to advanced stage of the disease or low performance status . the aim of this study was to describe patterns of care in patients with newly diagnosed mibc.this is a multicentre retrospective cohort study involving 296 consecutive patients with primary histologically diagnosed mibc . therapeutic decisions and potentially underlying clinical factors were analysed.full clinical data was available for 285 patients . one hundred and sixty - four ( 57.5% ) patients were qualified for radical cystectomy ( rc ) , 32 ( 11.2% ) patients for a second step of transurethral resection of the bladder tumour ( turbt ) intentionally followed by systemic chemotherapy , four ( 1.4% ) patients after complete turbt were qualified for adjuvant intravesical chemotherapy only , while the remaining 85 ( 29.8% ) patients were qualified for palliative treatment in the form of chemotherapy and/or radiotherapy and/or best supportive care . patients disqualified from curative treatment were older ( 78 vs. 69 years , p < 0.02 ) , had lower bmi values ( 24.5 vs. 25.7 kg / m2 , p < 0.02 ) , lower haemoglobin concentration ( 11.6 vs. 12.9 mg / l , p < 0.02 ) , declared lower rate of nicotine abuse ( 50.5% vs. 72.1% , p < 0.02 ) , and had a shorter time interval between first symptom and diagnosis ( 30 vs. 60 days , p = 0.02).as the majority of polish patients with primary mibc receive curative treatment , the stage of the disease alone seems not to be the leading cause of poor survival . however , appropriateness of qualification for rc and treatment quality needs to be assessed for final conclusion on the factors influencing outcomes of treatment in poland .
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myocardial bridging is defined as an epicardial coronary artery that goes intramurally through the myocardium beneath the muscle bridge . while generally benign , myocardial bridges can cause ischemia , ventricular tachyarrhythmias , atrioventricular block , and sudden cardiac death.1)2 ) for symptomatic patients , various therapeutic approaches have been attempted , but the optimal treatment of myocardial bridging still remains controversial.3)4 ) coronary stenting has been another therapeutic option with medical and surgical treatment , but the high risk of perforation and high rate of in - stent restenosis have limited its use.5 - 10 ) recently , we experienced a patient with a perforated coronary artery after implantation of a drug - eluting stent ( des ) that was successfully rescued by deployment of covered stent in symptomatic myocardial bridging . a 46 year - old woman who had no coronary risk factors , presented with exertional chest pain for several weeks . the chest pain was typical for angina pectoris and depressed st segments were noted at the exercise test . echocardiography revealed normal left ventricular ( lv ) systolic function { ejection fraction ( ef)=72% } without any regional wall motion abnormality . we performed coronary angiography which showed significant stenosis ( up to 80% ) aggravated by severe myocardial bridging at the mid - portion of the left anterior descending ( lad ) artery ( fig . so we decided to do a percutaneous coronary intervention ( pci ) at the lad lesion . through a 7 fr judkins guiding catheter , predilatation was performed with a maverick balloon catheter ( 2.515 mm , boston scientific , natick , ma , usa ) at 10 atmospheres for 20 seconds . we deployed a taxus stent ( 3.516 mm , boston scientific , natick , ma , usa ) according to the size of the predilated balloon catheter . but the middle segment of the lesion was not compliant , so the stent was not fully expanded with nominal pressure . , the coronary artery was perforated and some extravasation of contrast media was observed in the pericardium ( fig . but , the patient 's vital signs were stable ( blood pressure 115/70 mmhg ) with only a mild increase in heart rate . however , because the perforated site was entrapped intramurally through the myocardium in the interventricular groove , there was no evidence of accumulated blood at the dependent position of the pericardium on fluoroscopy or echocardiography . so we decided to observe the patient with close monitoring of symptoms and vital signs . but echocardiography showed no evidence of pericardial effusion . on an intravascular ultrasound ( ivus ) study , a large perforated site and a perivascular hematoma were observed in the mid - portion of the deployed stent ( fig . 1c ) . based on the ivus findings , a jo covered stent ( 319 mm , jomed international ab , helsingborg , sweden ) was deployed at the perforation site . four months after the procedure , ct angiography showed no evidence of residual hematoma or pseudoaneurysm ( fig . 2a and b ) . follow - up coronary angiography performed at 8 months after the procedure showed good distal flow with minimal stenosis at the proximal edge of the stent ( fig . 2c ) . although coronary stenting is an effective interventional approach to improve symptoms in selected patients with myocardial bridging , it is associated with a high risk of coronary perforation.5 - 7 ) the reason for this phenomenon is not clear . autopsy findings showed that tunneled segments in myocardial bridging tend to be deficient in vascular smooth muscle density , which may be more prone to vascular disruption during high inflation pressures during pci.11)12 ) another study revealed that the vessel area in the myocardial bridge segment was significantly smaller than that in the adjacent reference segments proximal and distal to the myocardial bridge throughout the cardiac cycle.13 ) this finding might explain the higher rate of coronary perforation associated with coronary stent implantation for myocardial bridges . the histological and anatomical differences in tunneled coronary arteries may require an adjustment in stent diameter and inflation pressures to help reduce the risk of coronary perforation . in our case , we selected an oversized stent , resulting in coronary perforation . pre - interventional ivus may be helpful in selection of an appropriate size of stent and , in particular , in cases that require high inflation pressures for optimal stent implantation . although coronary perforation is an uncommon complication following pci , it usually causes a catastrophic result including cardiac tamponade , emergency coronary artery bypass surgery , or pseudoaneurysm formation , with the potential for late coronary rupture and death.14)15 ) but , in myocardial bridging as observed in our patient , because the perforation site and extravasated blood are mainly confined in the interventricular groove , perforation itself usually does not cause hemodynamic instability . we were able to evaluate the lesion using ivus to decide on implantation of a covered stent after perforation . coronary stenting in myocardial bridging has been associated with a high restenosis rate.9 ) possible factors for this include shear stress from persistent external compression from myocardial bridges , causing neointimal proliferation , and long stent and recoil phenomena when inadequate pressures are used for stent deployment . a des was chosen as a feasible alternative to surgery because it had the ability to reduce restenosis . in our case , we performed pci with a des , which also showed good distal flow with minimal restenosis at the proximal edge of the stent at follow - up angiography . our case showed that , even though coronary perforation had occurred in myocardial bridging , the perforation site was confined in the interventricular groove . therefore , it could be managed more easily than conventional coronary perforation .
we successfully rescued a patient whose coronary artery perforated following implantation of a drug - eluting stent ( des ) , by deploying a stent - graft in symptomatic myocardial bridging . our case demonstrated that coronary perforation could be handled without difficulty when perforated myocardial bridging is confined to the interventricular groove
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the deleterious impact of hydrosalpinx on fertility is best exemplified by studies showing a 50% reduction in ivf pregnancy rates in their presence ( 1 ) . chlamydia infection and pelvic inflammatory disease are associated with an increased risk of both tubal infertility and ectopic pregnancy , with the association proportionate to the number of infections(2 ) . various theories have been proposed to explain the observation of lower ivf pregnancy rates in the setting of hydrosalpinx , to include mechanical effects , embryo and gametotoxicity , decreased expression of key implantation molecules , and/or a direct effect on the endometrium leading to intrauterine fluid formation(3 ) . drainage of hydrosalpingeal fluid into the endometrial cavity may exert a mechanical effect by washing the transferred embryo from the cavity . cultured epithelial cells isolated from hydrosalpinx affected tubes produce a fluid which is hostile to both spermatozoa and early mouse embryo development(4 ) . multiple studies have demonstrated deficiency in endometrial markers of embryo receptivity in the presence of hydrosalpinx . il-2 , t lymphocytes , cd3 + , cd8 + , cd4 + , alpha v beta 3 integrin , lif , mmps and hoxa10 have all been shown to be decreased in the endometrium from women with versus without hydrosalpinx(5 - 8 ) though molecular inflammatory changes in the setting of hydrosalpinx are documented , a hysteroscopic endometrial phenotype in the presence of hydrosalpinx has not been well characterized . eighteen months prior to presentation , she was diagnosed with a chlamydial infection by cervical culture and treated with a single dose azithromycin . a negative chlamydia culture was confirmed four months prior to presentation . as part of her initial infertility evaluation at our center , a hysterosalpingogram ( hsg ) was performed in a window of doxycycline prophylaxis which demonstrated bilateral hydrosalpinges , with the right tubal diameter measuring greater than the left and bilateral tubal occlusion ( figure 1 ) . hysterosalpingogram demonstrating bilateral distal tubal occlusion with hydrosalpinges staged combined endoscopy was performed in the operating room . hysteroscopy revealed endometrial inflammation as evidenced by diffuse hyperemia and mucosal oedema ( figure 2a ) . laparoscopic findings were significant for a right sided hydrosalpinx measuring over 2 centimeters in diameter and dense adhesions of the bowel to the left hydrosalpinx and ovary . she underwent right salpingectomy and left tubal occlusion at the visible isthmic region via clip placement . she experienced an unremarkable postoperative course and was scheduled for in vitro fertilization ( ivf ) treatment . hysteroscopic appearance of endometrial cavity immediately pre - salpingectomy ( a ) and six months after interruption of communication with hydrosalpinges ( b ) . for each series , images from left to right depict right cornual , fundal and left cornual regions , respectively . six months later , the patient underwent hysteroscopic endometrial cavity evaluation in preparation for ivf . at hysteroscopy , the patient subsequently underwent ivf with transfer of a single blastocyst culminating in the delivery of a healthy infant at term . given the importance of tubal patency in non - ivf treatment and the detrimental impact of tubal pathology such as hydrosalpinx , oviductal evaluation represents an important part of the initial female infertility work up . given the trend among women to present for infertility care later in the reproductive lifespan , patients undergoing ivf may not have had prior tubal evaluation via either of the standard modalities . transvaginal ultrasound in the detection of hydrosalpinx has been reported , albeit with user - dependent sensitivity . the present case suggests that an inflamed endometrial appearance may be a hysteroscopic sign of a communicating hydrosalpinx requiring dedicated tubal study prior to initiating fertility treatment as depicted in figure 2 , the endometrial surface appears erythematous with friable and tortuous vascularity in the setting of a hydrosalpinx , and normal appearing after surgical interruption of hydrosalpingeal drainage . subsequent ivf treatment and transfer of a single blastocyst resulted in an intrauterine pregnancy , with subsequent term delivery , indicative of functional embryo receptivity on a molecular level , although no molecular evidence is available for confirmation . in view of these findings , we propose an inflamed appearance at hysteroscopy for endometrial cavity screening should prompt a dedicated study to rule out hydrosalpinx prior to proceeding with ivf treatment . to date , such a hysteroscopic endometrial phenotype in the presence of hydrosalpinx has not been described .
we report the hysteroscopic findings in a 22 year old nulligravid patient with bilateral communicating hydrosalpinges . the inflamed hyperemic endometrial cavity encountered preoperatively normalized at second look hysteroscopy six months after bilateral tubal interruption . the patient underwent successful ivf with culmination in a singleton , live birth . we propose that an inflamed appearance at hysteroscopy , done for endometrial cavity screening , should prompt a dedicated study to rule out hydrosalpinx prior to proceeding with ivf treatment . to date , such a hysteroscopic endometrial phenotype in the presence of hydrosalpinx has not been well characterized .
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role of n - acetylcystein ( nac ) in adults with non - acetaminophen induced liver failure was described in few studies in literature . studies were particularly relevant to countries where , liver transplantation facilities are limited or unavailable . found a significantly improved transplantation free survival at 3 weeks and at 1 year with the use of nac in non - acetaminophen related liver failure , the benefit being confined to those with early hepatic encephalopathy . dengue infection is prevalent in southeast asia , and according to the epidemiological unit , ministry of health , sri lanka , during the last 4 months of the year 2012 , 11148 suspected dengue cases and 46 deaths have been reported . the elevation of transaminases is usually less than five - fold greater than upper limit of normal . however , levels more than five - fold were reported 36.8% and 74.4% of patients with classical dengue and dengue hemorrhagic fever ( dhf ) respectively . fulminant hepatitis tends to occur more often in dhf or dengue shock syndrome compared to classic dengue infection and case fatality rate of 50% being reported . although , nac has shown benefit in non - acetaminophen related liver failure , it was not well studied in dengue associated severe hepatitis . a previously healthy 54-year - old mother of three admitted with 3 day history of fever , headache and body ache . physical examination on investigation on the day of admission revealed platelets 84,000/cumm and haematocrit ( hct ) 37% . on the 2 day , she was transferred to intensive care unit ( icu ) as her platelets dropped to 41,000 per cu mm and hct increased to 47% . liver transaminases showed mild to moderate rise with ( ( ast ) aspartate transferase ) 302 u / l and ( ( alt)alanine transferase ) 262 u / l and patient was given total dose of 10 g of acetaminophen over 3 days at the time . she had stable hemodynamics apart from heart rate of 121 beats / min , but she developed right side moderate pleural effusion , icterus , mild ascites and right hypochondrial pain . in the icu , she deteriorated further , with a decline of glasgow coma scale ( gcs ) to 11 , but no focal neurological signs . urgent computed tomography brain was done and it neither showed intracranial hemorrhage nor evidence of increased intracranial pressure . her liver functions continued to deteriorate and liver enzymes reached peak value of ast 16261 u / l and alt 4545 u / l , ( pt / inr ) prothrombin time / international normalized ratio 1.7 and total bilirubin 5.9 mg / dl on 4 day of admission ( 7 day of illness ) . intravenous nac was started at 100 mg / kg / day as an infusion and continued for 5 days with liver failure regime . marked improvement in liver enzyme was noted and sgot and sgpt levels dropped by more than half by 48 h of treatment . on the 9 day of admission , liver function revealed ast 300 u / l , alt 223 u / l and pt / inr 1.2 , and her conscious level improved to gcs of 15 . during the course of illness , she had mild gum bleeding and few ecchymotic patches with lowest platelet count of 18,000 per cu mm . her serology was positive for dengue antibodies but negative for hepatitis a and b. hepatitis e serology was not done due to unavailability . co - infection of malaria was not excluded as she was from neither endemic area nor her symptomatology typical of malaria including fever pattern . possibility of leptospirosis can not be excluded in this case as serology was not done . however , she did not have any exposure and her renal functions were never abnormal . during her follow - up visit at 2 weeks after discharge , she had normal liver profile and did not have any evidence of chronic liver disease . both virus itself and dysregulated immune response to virus are being described as possible mechanisms of liver damage in literature . although , severe hepatitis associated with dengue fever is a rare occurrence , it carries significant mortality and morbidity . nac , mostly used in acetaminophen poisoning , acts through its antidote effect of repletion of hepatocellular glutathione stores . nac scavenges free radicals , improves antioxidant defense and acts as a vasodilator to improve oxygen delivery and consumption . these properties of nac have been postulated to improve outcome in patient with dengue associated acute liver dysfunction . concluded that benefit is seen when nac is used early stage of liver failure rather than late stage . a retrospective analysis on nac in dengue associated liver failure by kumarasena et al . showed that 5 patients who survived out of 8 were in early ( coma grade 1 , 11 ) liver failure stage at the time when nac was started . this case report also supports the view that intravenous administration of nac is safe and benefits patients , if started in early stage of liver failure . this patient was treated with intravenous nac 100 mg / kg / day infusion for 5 days compared to 150 mg / kg bolus over 15 min followed by 12.5 mg / kg / h for 4 h and then 6.25 mg / kg / h for 72 h was given by kumarasena et al . in their retrospective analysis . reported a pediatric patient with dengue associated liver failure successfully treated with nac and they have given intravenous nac 100 mg / kg / day for 6 days . large randomized trials should be carried out to establish its efficacy along with appropriate dosage , timing , and duration of treatment .
although , n - acetylcystein ( nac ) has shown benefit in non - acetaminophen related liver failure , it was not well studies in dengue associated severe hepatitis . we report a case of dengue hemorrhagic fever associated severe hepatitis ( encephalopathy grade 2-drowsy and intermittent disorientation ) treated with nac resulted in good outcome without hepatic transplantation .
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all strains were derived from haploid cells by4741 ( mating type a , euroscarf ) . cooperator strain has an intact suc2 gene , defective his3 gene ( his31 ) , and yfp expressed constitutively by the adh1 promoter ( inserted using plasmid prs401 containing met15 ) . the mutant cheater strain lacks the suc2 gene ( euroscarf y02321 , yil162w::kanmx4 ) , has an intact his3 gene , and has tdtomato expressed constitutively by the pgk1 promoter ( inserted using plasmid prs301 containing his3 ) . 2 and 5a were done using a strain containing yfp driven by the suc2 promoter ( inserted using plasmid prs306 containing ura3 ) . co - culture experiments were performed in 5ml batch culture at 30c using synthetic media ( minus histidine ) supplemented with 5% sucrose and variable concentrations of glucose and histidine . the 20% sucrose stock solution was filter - sterilized and stored with 1 mm tris ph 8.0 to prevent acid - catalyzed autohydrolysis . serial dilutions were performed daily ( 23 hours of growth ) such that the starting optical density was 0.0025 , corresponding to ~150,000 cells . equilibrium data in figures 1c and 3b , c were recorded after five days of competition between the two strains . all strains were derived from haploid cells by4741 ( mating type a , euroscarf ) . cooperator strain has an intact suc2 gene , defective his3 gene ( his31 ) , and yfp expressed constitutively by the adh1 promoter ( inserted using plasmid prs401 containing met15 ) . the mutant cheater strain lacks the suc2 gene ( euroscarf y02321 , yil162w::kanmx4 ) , has an intact his3 gene , and has tdtomato expressed constitutively by the pgk1 promoter ( inserted using plasmid prs301 containing his3 ) . 2 and 5a were done using a strain containing yfp driven by the suc2 promoter ( inserted using plasmid prs306 containing ura3 ) . co - culture experiments were performed in 5ml batch culture at 30c using synthetic media ( minus histidine ) supplemented with 5% sucrose and variable concentrations of glucose and histidine . the 20% sucrose stock solution was filter - sterilized and stored with 1 mm tris ph 8.0 to prevent acid - catalyzed autohydrolysis . serial dilutions were performed daily ( 23 hours of growth ) such that the starting optical density was 0.0025 , corresponding to ~150,000 cells . equilibrium data in figures 1c and 3b , c were recorded after five days of competition between the two strains .
the origin of cooperation is a central challenge to our understanding of evolution13 . microbial interactions can be manipulated in ways that animal interactions can not , thus leading to growing interest in microbial models of cooperation410 and competition11,12 . in order for the budding yeast s. cerevisiae to grow on sucrose , the disaccharide must first be hydrolyzed by the enzyme invertase13,14 . this hydrolysis reaction is performed outside of the cytoplasm in the periplasmic space between the plasma membrane and the cell wall . here we demonstrate that the vast majority ( ~99% ) of the monosaccharides created by sucrose hydrolysis diffuse away before they can be imported into the cell , thus making invertase production and secretion a cooperative behavior15,16 . a mutant cheater strain that does not produce invertase is able to take advantage of and invade a population of wildtype cooperator cells . however , over a wide range of conditions , the wildtype cooperator can also invade a population of cheater cells . therefore , we observe coexistence between the two strains in well - mixed culture at steady state resulting from the fact that rare strategies outperform common strategies the defining features of what game theorists call the snowdrift game17 . a model of the cooperative interaction incorporating nonlinear benefits explains the origin of this coexistence . we are able to alter the outcome of the competition by varying either the cost of cooperation or the glucose concentration in the media . finally , we note that glucose repression of invertase expression in wildtype cells produces a strategy which is optimal for the snowdrift game wildtype cells cooperate only when competing against cheater cells .
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nontyphoidal salmonella species are important food borne pathogens and acute gastroenteritis is the most common clinical manifestation accounting for about 70% of cases . splenic abscess is a very rare complication of nontyphoidal salmonella infections since the presence of antibiotics . here a 63-year - old woman from eastern part of turkey was admitted with the complaint of back pain , vomiting and nausea since 20 days . laboratory findings were as follows : white blood cell ( wbc ) count was 14670/mm ( 88.9% polymorphonuclear cells ) , haemoglobin 9.5 g / dl , platelet count 183000/mm , erythrocyte sedimentation rate 76 mm / h , and c - reactive protein ( crp ) 58 mg / dl ( normal value < 5 mg / l ) . an abdominal ultrasound revealed a hypoechogenic cystic structure with a diameter of 6172 mm in the upper part of the spleen with calcifications ( figure 1 ) . salmonella enteritidis was yielded from culture of the samples of the abscess obtained during the operation . it was sensitive to ampicillin , cotrimoxazole , cephalosporins of third generation , and ciprofloxacin . after isolation of the s. enteritidis from abscess , the patient was questioned in detail , it was learned that the patient had diarrhoea and fever existed 2 days and recovered without antibiotic treatment one month ago . the usual clinical presentation of nontyphoidal salmonella infection is self - limited gastroenteritis , however bacteraemia and focal extraintestinal infections may occur . risk of bacteraemia and focal extraintestinal infections are high in individuals with comorbidities such as malignancy , human immunodeficiency virus ( hiv ) , diabetes mellitus , and patients receiving immunosuppressive therapy . invasive nontyphoidal salmonellae disease is a major cause of mortality in african children and hiv - infected african adults . any tissue or organ may be seeded hematogenously by nontyphoidal salmonella and may form a local infection , become obvious months or even years after the initial bacteraemia producing characteristic clinical syndromes . some serovars of salmonella show a higher tendency for causing bacteraemia and these serovars differ in different countries . non - typhoidal salmonella serovars s. typhimurium and s. enteritidis are cause of invasive disease in industrialized countries , also they are predominant in african region . s. enteritidis had the highest blood invasiveness among non - typhoidal salmonella species in malaysia . our patient had diabetes mellitus and probably s. enteritidis bacteraemia occurred during the course of diarrhoea one month ago which lead to bacterial seeding in the spleen . antibiotic treatment for patients with mild to moderate gastroenteritis due to non - typhoidal salmonella is not indicated in healthy adults . however antimicrobial therapy should be initiated for patients who are severely ill and for patients with risk factors for extraintestinal spread of infection . the symptoms of splenic abscess are usually nonspecific the most frequent symptoms and signs are fever , abdominal pain and tenderness over left upper quadrant , splenomegaly , leucocytosis , and left lower chest abnormalities . our patient had back pain , vomiting , nausea and leucocytosis which were not specific for splenic abscess . computed tomography remains the gold standard and the most sensitive tool for the definitive diagnosis of splenic abscess . ultrasonography has 76% , ct has 96% sensitivity for the detection of abdominal masses . in our patient , diagnosis was confirmed with ct . at present , splenectomy is the gold standard for treatment while ultrasound- or ct - assisted percutaneous drainage and antimicrobial therapy can be considered as therapeutic alternatives especially in the presence of an isolated abscess . we preferred splenectomy for treatment as the causative microorganism was not known initially . in conclusion , although non - typhoidal salmonella gastroenteritis is rarely resulted with splenic abscess , patients with comorbities are at increased risk .
splenic abscess is a very rare complication of non - typhoidal salmonella infections . we report a case of splenic abscess caused by salmonella enteritidis . the patient is a 63-year - old woman with diabetes mellitus and underwent splenectomy . this case suggests that the patients with comorbities are at increased risk for invasive infections in non - typhoidal salmonella infections .
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a 60-year - old patient presented in the outpatient department with chief complaints of hematuria with off and on episode of urine retention . there was history of passage of small stones in urine occasionally for last few days . on ultrasonography he was found to have 35 g prostate with slightly thickened bladder wall with a large stone in the urinary bladder . x - ray kub showed a large radio - opaque shadow in the pelvic region [ figure 1 ] . patient underwent open cystolithotomy and large bladder stone [ figure 2 ] with multiple small stones were retrieved . patient was started on alpha blockers in the immediate postoperative period and foley 's catheter was removed on the eighth postoperative day . at follow - up of three weeks after the surgery the patient was voiding in good stream with minimal post - void residual urine . as the name implies this variety of stone has a characteristic shape resembling a child 's toy [ figure 3 ] . these types of stone are commonly described in the veterinary literature with common occurrence in cattle , cats and dogs . dogs are mostly commonly affected and canine jackstones are usually composed of silica . depicting child toy called jackstone calcium oxalate is the most common component of urinary calculi . calcium oxalate monohydrate calculi are usually smooth and black , whereas stones comprising calcium oxalate dihydrate tend to be irregular and yellow . prostatic diseases , previous lower urinary tract surgery , metabolic abnormalities , upper urinary tract calculi , intravesicular foreign bodies , spinal cord injuries , transplant surgery etc . the presentation of vesical calculi varies from completely asymptomatic to symptoms of suprapubic pain , dysuria , intermittency , frequency , hesitancy , nocturia , and urinary retention . other common signs include terminal gross hematuria and sudden termination of voiding with some degree of associated pain referred to the tip of the penis , scrotum , perineum , back , or hip . the discomfort may be dull or sharp and is often aggravated by sudden movements and exercise . assuming a supine , prone , or lateral head - down position may alleviate the pain initiated by the stone impacting the bladder neck by causing it to roll back into the bladder . in our case the prostate is the likely cause of this stone . enlarged prostate probably restricts the calculus into its eccentric location and contributes to the growth of stone by causing stasis of urine . it is important to recognize the characteristic shape of the jackstones as they are susceptible to lithotripsy . we did not offer lithotripsy as this modality is known to be less efficacious in case of vesical calculus .
jackstone calculi are urinary tract stones that have a specific appearance resembling toy jacks . they are almost always composed of calcium oxalate dihydrate consist of a dense central core and radiating spicules . they are usually light brown with dark patches and are usually described to occur in the urinary bladder and rarely in the upper urinary tract . their appearance on plain radiographs and computed tomography in human patients has been described .
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we quantified the results of entry screening for influenza a(h1n1)pdm09 at auckland international airport . using the information generated during screening , we retrospectively estimated the number of infected travelers who actually passed through the airport . to estimate the sensitivity of screening , we then compared screening findings with the expected number of infected travelers who passed through the airport . ethical approval was received from the northern x regional ethics committee of the new zealand ministry of health . the numbers of crew members on inbound international aircraft were estimated by using averages for flights into auckland . the number of travelers detected at each step and referred to the next step of the screening process was obtained from auckland regional public health service records . a confirmed case was one that met the current case definition ( as published on the ministry of health website , www.health.govt.nz ) and one for which rt - pcr result was positive . we estimated the number of infected travelers screened as the total number of confirmed cases in new zealand during this period , multiplied by the proportion of overseas - acquired cases , and the proportion of international travelers arriving at the airport . on april 30 , 2009 , nonseasonal influenza a ( h1n1 ) was made notifiable , and these data were collated on the national surveillance database ( episurv ) ( www.surv.esr.cri.nz/episurv ) . the proportion of infected travelers who acquired the infection overseas was extrapolated from ministry of health records of the first 100 cases of pandemic ( h1n1 ) 2009 because this information was not collected for all travelers with confirmed infection . the proportion of travelers who passed through the airport was determined from statistics new zealand ( www.stats.govt.nz ) arrivals records . confidence intervals were calculated by using the online calculator for screening on open epi ( 4 ) . during the screening period , 456,518 international travelers were screened ; 406 ( 0.09% ) of these were referred for medical assessment . of those , rt - pcr results were located for 89 ( 82% ) , among which 4 were positive . the expected number of infected travelers estimated to have passed through the border during the screening program was therefore 69 , giving an estimated sensitivity of 5.8% ( 95% ci 2.3%14.0% ) . this form of border screening is therefore unlikely to have substantially delayed spread of the pandemic into new zealand in 2009 . limitations of influenza screening include the high proportion of asymptomatic infected travelers ( 5 ) , incubation of infections acquired before or during a flight ( 3 ) , reliance on self - identification , limitations of case definitions , and limitations of thermal scanning ( 6 ) . modeling data have shown that the ability of border screening to delay global pandemic influenza is closely linked to the effectiveness of the screening process or travel restriction used . to delay influenza spread by 1.5 weeks , border restrictions need to reduce imported infections by 90% ( 7 ) . the potential effectiveness of screening arriving travelers to prevent or delay influenza epidemics has been debated . mathematical models and literature reviews have argued for ( 7,8 ) and against ( 911 ) this approach . some authors have found that entry screening for respiratory conditions or influenza a(h1n1)pdm09 is insensitive and not cost - effective ( 12 ) . this study has several limitations , particularly with regard to estimating the number of infected travelers who would have passed through the airport during the screening period . most cases of illness acquired overseas would probably not have been notified , particularly those in patients with mild illness who did not see a doctor or who saw a doctor but did not receive a diagnosis . the estimated proportion of overseas - acquired cases was based on data from the first 100 cases and would have decreased as the pandemic progressed . the net effect of these factors is unknown , but they would probably have increased the estimated number of undetected infected travelers passing through screening , thereby further reducing the estimated sensitivity of screening . it might provide public assurance and confidence that something is being done ( 14 ) . the communication of health information and advice on how to seek treatment is consistently recommended as a pandemic prevention strategy ( 12,15 ) and is usually delivered as part of border screening programs . these benefits need to be balanced against the considerable resources used , opportunity cost ( resources used for this activity and thereby unavailable for other activities ) , uncertain effectiveness , and inconvenience of border screening . to delay or prevent influenza entry at borders , influenza screening needs to be considerably more effective than the mostly passive program described here . we hope that during this interepidemic period , a major international review of the role of international air travel in the dissemination of emerging infectious diseases will be conducted to identify effective interventions . such a review should consider systemwide approaches , including exit screening , standardized health declarations , active screening of individual passengers ( including use of rapid laboratory tests and thermal scanning ) , passenger tracking , policies and practices that support sick travelers wishing to defer travel , and circumstances where airline travel should be suspended entirely .
entry screening for influenza a(h1n1)pdm09 at auckland international airport , new zealand , detected 4 cases , which were later confirmed , among 456,518 passengers arriving april 27june 22 , 2009 . on the basis of national influenza surveillance data , which suggest that 69 infected travelers passed through the airport , sensitivity for screening was only 5.8% .
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the structures of acl and mcp in the gas phase were optimized at the b3lyp/6 - 31 g * level . the structures of the water clusters were determined with classical molecular dynamics trajectories ( see details in the si ) . the cluster models include solvents that have at least one of the atoms within 3 and 4 from acl and mcp , respectively . the geometry of the acl and mcp in the water clusters was further optimized at the b3lyp / cc - pvdz and cam - b3lyp / cc - pvdz levels , respectively . in the optimization , the water molecules were fixed at the md structure and replaced by point - charges ( tip3p charges ) . we also fixed the c atom next to the o atom of acl . in mcp , the atomic coordinates of the central c atom were fixed . the hf orbitals were transformed into mos localized within each fragment ( solute and solvent ) . our transformation uses reference orbitals ( rmos ) obtained with external calculations for isolated molecules . , we show the populations at the fragments . in the perturbation - selection step of the sac - ci calculations , a set of threshold , levelfour , as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be re - organized for online delivery , but are not copy - edited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors
intermolecular interactions regulate the molecular properties in proteins and solutions such as solvatochromic systems . some of the interactions have to be described at an electronic - structure level . in this study , a commutator for calculating the excitation energy is used for deriving a first - order interacting space ( fois ) to describe the environmental response to solute excitation . the fois wave function for a solute - in - solvent cluster is solved by second - order perturbation theory . the contributions to the excitation energy are decomposed into each interaction and for each solvent .
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once believed to be rare , trichotillomania is now thought to affect as much as 4% of the population . usually beginning in early childhood or adolescence , most patients with trichotillomania do not seek treatment until 17 years of age . patients with trichotillomania often hide their hair - pulling behavior , and the disorder is often suspected by typical dermatological findings , such as alopecia . eating the part of hair pulled out is a common practice and trichorhizophagia is a new term to denote the habit of eating the root of hairs pulled out , associated with trichotillomania . here we report a case of trichotillomania with trichorhizophagia in schizophrenia and discussed the various treatment options . a 58-year - old , married , hindu , unemployed male , a confirmed case of schizophrenia of 30 years duration with no family or past history of psychiatric illness , no history of any medical or surgical illnesses , presented in our opd with pulling hair and eating the hair root for the last 5 years . prior to the consultation in our hospital , he was on irregular treatment with haloperidol . he used to pluck hair from the scalp and eyebrows and developed patches of hair loss in these areas . usually he plucks one or two hairs at a time and plays with hair for some time or rubs the root of the hair along the lips and then discards it . at times he bites the hair and swallows the bitten part containing the root of hair discarding the rest . the patient admitted hair - pulling behavior and reported a kind of pleasure in doing this activity . he has not attempted to resist this habit and it was not a concern for him . there were patches of alopecia on the scalp and right eyebrows with no local inflammation , itching , or pain . he was started on olanzapine and the doze was titrated to a maximum dose of 20 mg per day over a period of 1 month . he showed significant improvement of schizophrenic symptoms except hair pulling behavior . since behavior therapy was not possible at this stage as the patient was not cooperative , escitalopram 10 mg was added to the previous regime for controlling the hair pulling behavior . after 3 months of combined therapy he almost completely stopped the hair pulling behavior , subsequent biting , and eating hair roots without any exacerbation of psychotic symptoms . there was regrowth of scalp hair as well . till date , the patient is maintaining improvement and is attending our opd with regular follow up . trichotillomania is considered to be a rare disorder encountered in clinical practice . although trichotillomania was reported to occur with many psychiatric disorders , the exact prevalence rate was not reported . other comorbid conditions reported include dissociative experiences , dementia , parkinson 's disease , partial seizures , and prader- willi syndrome . possible hypothesized causes include a biological basis , as well as hair pulling in response to life stresses . some of the ssris especially escitalopram , fluoxetine , and fluvoxamine are found to be effective . recently , there was a report of resistant trichotillomania treated with risperidone augmented with fluoxamine . the index case had compulsive plucking of hair and eating the hair root for the last 5 years , which was not part of any delusions or hallucinations . although olanzapine has shown efficacy as monotherapy against psychosis , addition of escitalopram only produced marked improvement in compulsive behavior . this case points to the efficacy of combination of olanzapine and escitalopram particularly in patents showing psychotic symptoms along with trichotillomania . moreover , in patients with hair pulling behavior , it is prudent to inquire about trichophagy because it can lead on to rare but potentially life - threatening condition called trichobezoar .
trichotillomania is a disorder characterized by chronic hair pulling that often results in alopecia . eating the part of hair pulled out is a common practice and trichorhizophagia is a new term to denote the habit of eating the root of hairs pulled out , associated with trichotillomania . many psychiatric disorders are prevalent among patients with trichotillomania . here we report a case of trichotillomania with trichorhizophagia in a 58-year - old man with schizophrenia . the various treatment options are also discussed .
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as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be reorganized for online delivery , but are not copyedited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors .
abstracthelically chiral n , n , o , oboron chelated dipyrromethenes showed solutionphase circularly polarized luminescence ( cpl ) in the red region of the visible spectrum ( em(max ) from 621 to 663 nm ) . the parent dipyrromethene is desymmetrised through o chelation of boron by the 3,5orthophenolic substituents , inducing a helical chirality in the fluorophore . the combination of high luminescence dissymmetry factors ( |g lum| up to 4.7 103 ) and fluorescence quantum yields ( f up to 0.73 ) gave exceptionally efficient circularly polarized red emission from these simple small organic fluorophores , enabling future application in cplbased bioimaging .
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aggregation and bordering behaviors were measured essentially as described2 ; values report the average fraction of three or more behavioral assays of 150 animals each . average locomotion speed was calculated by tracking 20 animals for 10 minutes with an automated tracking system30 . for rmg - selective expression of transgenes , loxp - flanked lacz sequence containing a transcriptional stop , three repeated polya sequences , and two repeated mrna cleavage sequences was inserted upstream of npr-1::sl2::gfp under the control of the flp-21 promoter ( flp-21::loxstoplox::cdna(gfp , npr-1 , tetx , or pkc-1(gf ) ) . transgenic animals containing this plasmid strong and consistent expression was observed in rmg and m2 ; adl , asj , and ask were seen weakly and inconsistently . for ascaroside chemotaxis assays , washed animals were placed in the center of a 4-quadrant plate with ascarosides in alternating quadrants , and scored after ten minutes . a chemotaxis index ( c.i . ) was calculated as ( # of animals on pheromone quadrants # of animals on buffer quadrants)/(total # of animals ) . in the cartoon in fig . 4 a cocktail of three ascarosides was used ; individual ascarosides and other combinations are in supplementary fig . calcium imaging of the aia and ask neurons was performed in a custom - fabricated microfluidic device , essentially as described26 . for ask imaging , the transgene kyex2866 was used , with gcamp2.2b ( gift from loren looger ) expressed under the sra-9 promoter . for aia imaging , ask fluorescence was recorded in the neuronal cell body , and aia fluorescence was measured in the dorsal aia process in the nerve ring .
innate social behaviors emerge from neuronal circuits that interpret sensory information based on an individual 's own genotype , sex , and experience . the regulated aggregation behavior of c. elegans , a simple animal with only 302 neurons , is an attractive system to analyze these circuits . wild social strains of the nematode caenorhabditis elegans aggregate in the presence of specific sensory cues , but solitary strains do not1,2,3,4 . here we identify the rmg inter / motor neuron as the hub of a regulated circuit that controls aggregation and related behaviors . rmg is the central site of action of the neuropeptide receptor gene npr-1 , which distinguishes solitary strains ( high npr-1 activity ) from wild social strains ( low npr-1 activity ) ; high rmg activity is essential for all aspects of social behavior . anatomical gap junctions connect rmg to multiple classes of sensory neurons known to promote aggregation , and to ask sensory neurons , which are implicated in male attraction to hermaphrodite pheromones5 . we find that ask neurons respond directly to pheromones , and that high rmg activity enhances ask responses in social strains , causing hermaphrodite attraction to pheromones at concentrations that repel solitary hermaphrodites . the coordination of social behaviors by rmg suggests an anatomical hub - and - spoke model for sensory integration in aggregation , and points to functions for related circuit motifs in the c. elegans wiring diagram .
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it is characterized by the diversion of portal venous blood away from the liver , by either end - to - side or side - to - side shunt . a two - and - half - year - old child presented with respiratory distress , poor growth , and loss of appetite for 2 months . computed tomography ( ct ) showed mild cardiomegaly with collapse / consolidation of bilateral lung lobes . incidentally , there was the presence of an end - to - side shunt between the right branch portal vein ( pv ) and the inferior vena cava ( ivc ) with fusiform aneurysmal dilatation of the pv continuing into the left branch , which abruptly ended after a short distance . there was aplasia of the right branch of the pv [ figures 1 and 2 ] . the hepatic artery appeared normal . the splenic and superior mesenteric veins had normal orientation , and these vessels joined to form the main pv . a diagnosis of congenital extrahepatic portocaval shunt ( ceps ) ( abernethy malformation type 1 ) with pv aneurysm was made . since the patient was asymptomatic and liver enzymes were normal , the patient was advised follow - up . there was the presence of end - to - side shunt between the right branch of pv and the ivc ( solid thick arrow ) , fusiform aneurysmal dilatation of the pv , which was continuing into the left branch ( thin arrow ) coronal multiplanar reconstruction ( mpr ) shows the presence of end - to - side shunt between the right branch pv and the ivc ( b ) ( arrow ) with fusiform aneurysmal dilatation of the pv ( a ) . the portal venous system and ivc develop between the 4th and 10th weeks of embryonic life by selective apoptosis of some portions of the vitelline , which may lead to the potential for congenital portosystemic shunts . type i ceps ( congenital absence of the pv ) is an end - to - side shunt between pv and the systemic circulation . here all the splanchnic venous return enters the systemic circulation , and the liver is not perfused with portal venous blood at all . type ii ceps is a partial side - to - side shunt between the portal and systemic vein , where only a fraction of the splanchnic venous return bypasses the liver parenchyma . ceps are also associated with an increased frequency of hepatic neoplasms.[37 ] it has been proposed that the diversion of hepatotropic substances in the splanchnic venous blood , such as insulin and glucagon , away from the liver results in alterations of development , function , and regenerative capacity of the liver . this diversion , along with increased arterial hepatic flow , may contribute to the development of hepatic neoplasms . currently , a diagnosis of abernethy malformation is usually made by noninvasive cross - sectional imaging techniques such as ultrasound , ct , or mri , which show the shunt and any intrahepatic pv branches . however , liver biopsy may be necessary in patients with suspected type 1 malformation since an occasional patient may have small pv radicles which can not be seen on ultrasound but can be observed on liver biopsy . pv aneurysm is a rare clinical entity having a focal fusiform or saccular dilatation of more than 20 mm . aneurysms of the pv may occur either proximally at the junction of the superior mesenteric vein and splenic vein or more distally in the pv radicals . congenital origin is suggested based on the discovery of variations in the embryologic development of the pv and may be associated with multiple vascular malformations . the origin of acquired pv aneurysms is more commonly secondary to cirrhosis and other hepatic diseases . pancreatitis can be included as an extrahepatic cause . in the majority of cases , patients are clinically asymptomatic . the majority of patients with abernethy malformation have other associated anomalies such as liver and cardiac abnormalities . described a case of 24-year - old man having ceps type-2 with pv aneurysm during an investigation for nonspecific abdominal pain . determining the patients with type i malformation need clinical , biochemical , and imaging follow - up . however , those patients with type 2 malformations need surgery or percutaneous transcatheter coil placement . in conclusion , although ceps is a rare anomaly , it must be recognized early to prevent the consequences of metabolic derangements by appropriate surgical treatments . , the detection of tiny intrahepatic portal venous radicals may be beyond the resolution limits of the available imaging methods . the limitation of imaging in determining the type of shunt in some cases should be recognized , and a histopathological confirmation of the type of shunt may be crucial in deciding the treatment course .
abernethy malformation is an extremely rare anomaly of the splanchnic venous system . we describe multidetector computed tomography findings of an incidentally detected abernethy malformation with portal vein aneurysm in a two - and - half - year old child . the computed tomography scan was performed for the evaluation of respiratory distress , poor growth , and loss of appetite .
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parent - of - origin effects is a broad term that encompasses two distinct phenomena parent - of - origin effects on transcription , and parent - of - origin effects on mutation rates . a parent - of - origin effect on transcription , or genomic imprinting , results from epigenetic modification of the genome which , in turn , results in unequal transcription of parental alleles . for these imprinted genes , expression of the alleles is dependent upon the sex of the parent from which they were inherited ( 1 ) . a parent - of - origin effect on mutation rate , however , refers to the preferential occurrence of some spontaneous mutations in either the father 's or the mother 's germ line . the mechanisms by which these spontaneous mutations arise depend upon the parental germ line in which the mutation occurred . for example , base substitutions , arising from errors during replication , tend to be paternal in origin , owing to the greater number of cell divisions in spermatogenesis as compared with oogenesis ( 2 ) . oocytes are arrested in prophase of meiosis i until sexual maturity , when one oocyte per month is selected to resume the cell cycle . it is thought that the longer the oocytes are arrested in meiosis , the greater the chance for a nondisjunction event to occur ( 3 ) . advanced parental age seems to influence the development of some , but not all , of these mutations ( also referred to as the paternal or maternal age effect ) ( 2 ) . in 1998 , the catalogue of imprinted genes and parent - of - origin effects was first published ( 4 ) . this catalogue served as the basis for the development of a more comprehensive , searchable , online database , made publicly available in 1999 . the original database included 41 imprinted genes , and other parent - of - origin effects , including some records on the parental origin of spontaneous mutations ( 5 ) . we have added recently a comprehensive section on spontaneous mutations that show a bias with respect to their parental origin . this new part of the database can be searched according to mutation type , disorder , chromosomal location , gene name and inheritance pattern . outcomes of the search are presented in a tabular format with the following information : disorder , inheritance pattern , incidence of disorder , gene name , chromosomal location , evidence of a paternal or maternal age effect , mutation type and any recurrent mutations associated with a parent - of - origin effect , number of paternal mutations , number of maternal mutations and pubmed reference ( e.g. table 1 ) . in the case of base substitutions , data are separated according to the type of base substitution ( missense mutation , nonsense mutation or splice site mutation ) , whether the mutation is a transition or transversion mutation , and whether the base substitution falls within a cpg dinucleotide . for deletions and insertions , the distinction is made between large deletions and insertions ( > 20 bp ) and small deletions and insertions ( < 20 bp ) . this size distinction is made based upon the possibility of different mechanisms contributing to these different types of mutations , and therefore potentially different parental origins ( 2 ) . in general , large deletions do not appear to have a parent - of - origin effect , whereas small deletions tend to be more paternal in origin . currently , > 1700 mutations with a parent - of - origin effect are catalogued in this database . the other major section of the database includes known imprinted genes and observations of other putatively imprinted genes . of the 464 database entries , 152 entries describe 85 unique imprinted genes in humans , mice , cattle , sheep , pigs , rats and marsupials , as well as 14 genes for which the evidence of imprinting is conflicting or provisional . an additional 186 entries report parent - of - origin effects in the transmission or linkage of simple and complex genetic conditions including human diseases and animal quantitative traits . the imprinted gene and parent - of - origin effect database is housed at the university of otago in dunedin , new zealand and can be accessed at . the database is maintained by the corresponding authors who welcome submissions and comments and is updated as new literature is published . submissions to the imprinted gene database should be directed to i.m.m . and submissions to the parental origin of de novo mutations database example of report for parental origin of de novo mutations showing base substitutions within a cpg dinucleotide ad , autosomal dominant ; xd , x - linked dominant ; xr , x - linked recessive ; p , point mutation ; ms , missense mutation ; ns , nonsense mutation ; cpg , mutation in a cpg dinucleotide ; ts , transition mutation ; tv , transversion mutation .
the imprinted gene and parent - of - origin effect database ( ) consists of two sections . one section catalogues the current literature on imprinted genes in humans and animals . the second , and new , section catalogues current reports of parental origin of de novo mutations in humans alone . the addition of a catalogue of de novo mutations that show a parent - of - origin effect expands the scope of the database and provides a useful tool for examining parental origin trends for different types of spontaneous mutations . this new section includes > 1700 mutations , found in 59 different disorders . the 85 imprinted genes are described in 152 entries from several mammalian species . in addition , > 300 other entries describe a range of reported parent - of - origin effects in animals .
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this is a widely endorsed public perception of fear , derision and avoidance of the mentally ill . one reason could be lack of awareness , but other reasons abound . to take an example , popular films seem to suggest that people with mental illnesses : are mass - murdering , homicidally inclined violence junkies;have themselves to blame for not being strong enough to battle illness;have been visited by the divine wrath of an unforgiving god . are mass - murdering , homicidally inclined violence junkies ; have themselves to blame for not being strong enough to battle illness ; have been visited by the divine wrath of an unforgiving god . in this case , stigma works insidiously when internalized to erode the sense of self - worth or social relevance . it works at various levels to instill a deep level of insecurity . to take an example , childless women experience self - stigma . questionable person proves his claim to normalcy by citing his acquisition of a spouse and children , and oddly , by ttesting to his spending christmas and thanksgiving with them . people 's perceptions are not going to change overnight that is not to imply that everyone thinks alike . a community 's attitude toward the mentally ill plays a paramount role in treatment - seeking , drug compliance and rehabilitation . i hope that this article will help raise greater levels of awareness and help combat stigma against the mentally ill .
stigma against people with mental illness is a very complex public health problem . there could be diverse reasons for this ranging from : lack of awareness;fear of a dimly - comprehended and much - misunderstood illness;illogical generalizations ; anddisrespect for the heterogeneity of life . the result - for the mentally ill - could well be diminished access to social determinants of healthcare , employment , and housing . in addition , people with mental illnesses are exposed to numerous health risks such as malnutrition , drug abuse , violence and homelessness . maybe this explains nondisclosure of illness in an increasingly degenerate civil society .
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when detection is delayed or the rupture occurs spontaneously , it sometimes resembles acute kidney injury because ascites , oliguria and increasing serum creatinine levels are observed in patients with intraperitoneal urinary leakage . a 37-year - old woman presented with post - operative acute abdominal distension and an increasing serum creatinine level 7 days after total abdominal hysterectomy for uterine myoma . her serum creatinine and urea nitrogen levels were elevated , but the serum beta2-microglobulin level was within normal limits . the patient had no history of kidney disease , and her serum creatinine level on post - operative day 1 was normal ( 0.76 mg / dl ) . sodium , potassium and chloride levels in the ascitic fluid were 37 , 19 and 76 meq / l , respectively , which differed markedly from the serum electrolyte levels ( table 1 ) . laboratory findings of two cases of intraperitoneal urine leakage because these symptoms may also be caused by drug - induced nephropathy , all medications were stopped . however , no decrease in ascites or in the serum creatinine level was observed . since the patient s urine volume had decreased , a urinary catheter was inserted . the perforation was closed surgically , and a subsequent retrograde cystography did not reveal urinary leak . retrograde cystography exhibiting bladder perforation in case 1 ( a ) and case 2 ( b ) . a 70-year - old woman with a history of radiotherapy for cervical cancer 16 years earlier presented with progressive abdominal distension over a 2-week period . on admission sodium , potassium and chloride levels in the ascitic fluid were 21 , 23 and 78 meq / l , respectively , which differed from the serum electrolyte levels ( table 1 ) . after placement of the catheter in the bladder , the ascites disappeared , and the serum creatinine level decreased to the normal range . we performed retrograde cystography , but bladder perforation was not detected . because the patient s condition had improved , she was discharged from the hospital . three months after discharge , the patient was re - admitted with massive ascites and an increasing serum creatinine level . to determine the cause of serum creatinine elevation , we performed technetium-99 m diethylenetriaminepentaacetic acid ( tc-99 m dtpa ) renography , which showed extravasation of tc-99 m dtpa into the peritoneal cavity . retrograde cystography revealed a small perforation in the bladder ( figure 1b , arrow ) . intraperitoneal urinary leakage is characterized by an increase in the serum creatinine level caused by reabsorption of creatinine in the urine through the peritoneal membrane , oliguria and ascites . because most cases of intraperitoneal urinary leakage are the result of blunt trauma , leakage without obvious trauma the incidence of bladder injury after total abdominal hysterectomy is 0.1% . because most bladder injuries are identified intraoperatively , delayed appearance of urinary leakage however , the literature includes one report of delayed intraperitoneal urinary leakage after caesarean section . although spontaneous bladder perforation is uncommon , several cases have been reported in association with intravesicular obstruction , infectious lesion of the bladder , bladder diverticulum , bladder carcinoma , chemotherapy , and alcohol or substance abuse . therefore , acute kidney injury with massive ascites and peritonitis should be distinguished from urinary leakage . however , our two cases had abdominal pain , but rebound tenderness , which is a sign of peritonitis , was not noted . because bladder rupture without signs of peritonitis has been reported , symptoms of peritonitis may sometimes be unclear . although a correlation between serum and ascitic electrolyte levels has not been clearly established , some papers have reported this correlation [ 79 ] . sodium , potassium and chloride levels in ascitic fluid are nearly identical to those in serum ( ascites vs. serum ; sodium 133.1 6.6 vs. 131.8 6.3 mmol / l , potassium 4.1 0.8 vs. 4.3 0.9 mmol / l and chloride 107.2 7.6 vs. 101 7 mmol / l ) in cirrhosis patients . in peritoneal dialysis patients , electrolyte levels in peritoneal dialysate that are retained longer than 24 h are nearly identical to those in serum ( peritoneal dialysate vs. serum ; sodium 141.4 vs. 140.6 meq / l , potassium 4.7 vs. 5.1 meq / l and chloride 108.5 vs. 102.7 meq / l ) . another report noted that the ratio of sodium , potassium and chloride between serum and transudates which include ascites is 0.95 , 0.740.80 , and 0.950.99 , respectively . however , in our two cases , the ascitic sodium , potassium and chloride levels differed markedly from their serum levels . both serum beta2-microglobulin and creatinine levels are usually elevated in patients with acute kidney injury . this was not the case in our first patient , and this discrepancy may be a clue to the presence of intraperitoneal urinary leakage . the reason for this discrepancy has not been elucidated ; however , beta2-microglobulin , due to its larger molecular size , may not be absorbed through the peritoneal membrane similar to creatinine . in addition , although we did not measure urea nitrogen and creatinine in the ascitic fluid , the higher levels in ascites than in the blood are additional clues suggesting the possibility of intraperitoneal urinary leakage . in summary , we report two cases with intraperitoneal urinary leakage resembling acute kidney injury . these cases suggest that bladder perforation should be considered in the differential diagnosis of acute kidney injury with massive ascites .
ascites , oliguria and increasing serum creatinine levels are often noted in patients with acute kidney injury . however , these presentations are also observed in patients with intraperitoneal urinary leakage . bladder perforation without obvious trauma is sometimes mistaken for acute kidney injury . we report two cases of bladder perforation resembling acute kidney injury . the first case was a 37-year - old woman with delayed intraperitoneal urinary leakage following total abdominal hysterectomy , and the second was a 70-year - old woman with spontaneous bladder perforation . although the initial diagnosis in both cases was acute kidney injury , rupture of the urinary bladder was later identified .
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nutritional status is very important especially in old age population because any imbalance in nutritional status has negative effect on quality of life and causes increase morbidity and mortality . for instance , phytobezoar , a bezoar consist of fruit and vegetable fibers , can lead to small bowel obstruction and has risk factors such as intake of large amount of food with high - fiber content and inadequate chewing ( 1 , 2 ) . furthermore , aging process and some of related physiologic changes can predispose one to phytobezoar formation . we describe a case of small bowel obstruction due to phytobezoar following large amount of pomegranate seeds intake a few days before admission as an example of increased morbidity relating to unusual dietary habit . a 61 year - old man was admitted to emergency department of nemazee hospital , shiraz university of medical sciences , shiraz , iran , in 2015 with bowel obstruction . ct scan showed a mottled - appearing lesion in terminal ileum with air bubbles and dilated proximal bowel loops ( fig . three consecutive ct images show mottled - appearing lesion in terminal ileum five cm before ileocecal valve with air bubbles and dilated proximal bowel loops in favor of phytobezoar ( black arrows ) causing obstruction . note normal non - distended distal ileum just distal to bezoar ( white arrows ) because our patient had consumed large amount of pomegranate seeds five days before admission and with no history of previous abdominal surgery , phytobezoar became first diagnosis as cause and scheduled for surgery . during laparotomy , there were impacted materials in favor of phytobezoar just before ileocecal valve causing complete obstruction of small - bowel . the blockage resolved with pushing the intestinal contents into large bowel . the patient was discharged after passing unremarkable postoperative course and was doing well at two months follow up visit . nutritional status is very important especially in old age population because any imbalance in nutritional status has negative effect on quality of life and causes increase morbidity and mortality . one of the problems relating to dietary habit is phytobezoar formation in the gastrointestinal tract . bezoars are masses produced by accumulation of undigested material such as fruit , hair , and milk in gastrointestinal tract more frequently in stomach ( 3 , 4 ) and one of the most common types of bezoar is phytobezoar ( 5 ) , composed of fruit and vegetable fibers . phytobezoar as a cause of small bowel obstruction has risk factors such as intake of large amount of food with high - fiber content and inadequate chewing ( 1 , 2 ) . inadequate chewing can result of dental problems such as difficult chewing following loss of teeth that frequently seen in older age group ( 6 ) . other factor in aging population is loss of intestinal elasticity , as a physiologic change commonly seen in this group , and resultant constipation ( 6 ) may be considered as another reason for increased chance of intestinal phytobezoar formation . in other words , intestinal phytobezoar formation is a multifactorial entity , involving dietary and alimentary factors ( 7 ) and dental hygiene . therefore , good dietary habit and proper dental hygiene in lifetime are necessary for maintaining a healthy population especially in older adults . small bowel obstruction is another area for consideration that is a common disease and result from many causes such as adhesion , hernia , inflammation , tumor and bezoar . two to three percent of small bowel obstructions are due to bezoar ( 7 ) . in addition , most frequent manifestation of bezoar is complete intestinal obstruction ( 8) and computed tomography is useful in its diagnosis ( 1 ) . findings of bezoar - induced small bowel obstruction on ct scan are intraluminal mass containing air bubbles and dilated bowel loops proximal to mass and normal distal loops ( 1 ) . the dietary habit of having large amount of vegetables and fruit in asian countries can be result in phytobezoar formation as relatively common cause of small bowel obstruction in the absence of previous gastric surgery ( 4 ) . small bowel obstruction due to bezoar may need surgery for treatment and this entity rarely improves with conservative therapy , hence it is important to consider this diagnosis as the reason for obstruction ( 1 ) . in this way , in patients with small bowel obstruction that have history of recent consumption of vegetables or food with high fiber content and no past history of surgery , phytobezoar should be kept in mind with high index of suspicion . 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 .
nutritional status is very important especially in older adults because of its effects on quality of life . phytobezoar , for instance , that can lead to small bowel obstruction has risk factors such as excessive consumption of foods with high fiber content and inadequate chewing . these factors are related to dietary habits . furthermore , aging process and some of related physiologic changes can predispose one to phytobezoar formation . we describe a 61-yr - old man presented to the emergency department of nemazee hospital , shiraz university of medical sciences , shiraz , iran , in 2015 with small bowel obstruction due to phytobezoar following large amount of pomegranate seeds intake a few days before admission as an example of increased morbidity relating to unusual dietary habit .
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drug - coated balloon has been developed as an alternative to drug - eluting stents ( des ) . results of several preclinical and clinical studies indicate that short - term exposure of injured arteries to paclitaxel eluted from regular percutaneous transluminal angioplasty and ptca balloons may be sufficient to reduce late lumen loss and restenosis rates during a critical period of time after angioplasty of diseased coronary and peripheral arteries.- we present a case of particularly proliferative instent restenosis treated with a new type of drug eluting balloon . a 68-year - old man with severe silent ischemia was admitted to our center for elective coronary angiography and percutaneous coronary intervention . the patient had bypass grafting of left anterior descending ( lad , with mammary artery ) , first obtuse marginal branch and right coronary artery ( rca ) 6 years before . three years after the surgical procedure , he developed unstable angina and angiography showed occlusion in both vein grafts . he underwent percutaneous transluminal coronary angioplasty with des in the obtuse marginal branch and left main and with a bare metal stents vision 3.5 18 mm in the proximal and 3.0 23 mm ( abbot vascular , abbott park , illinois , usa ) in the distal dominant rca ( figure 1a and 1b ) . after one year , he had recurrent angina and a complete occlusion of previous bare - metal stents of rca was noted . the patient underwent repeated coronary angioplasty with des and extensive reconstruction of the rca was accomplished using two promus ( boston scientific , usa ) 3.5 28 mm and a 3.5 15 mm followed by another 3.0 12 mm from the proximal to the distal portion of rca with excellent angiographic results ( figure 1c ) after high pressure over- dilation with 4.0 12 mm sprinter nc balloon ( medtronic , minneapolis , minnesota , usa ) . nevertheless after 8 mo the patient developed severe stress myocardial ischemia in the inferior territory associated with mild effort angina . the coronary artery angiography revealed diffuse proliferative and subocclusive in - stent restenosis of previous des ( figure 1d ) . the patient was considered at risk for repeated bypass surgery , so a percutaneous ballaoon angioplasty with a medicated balloon was scheduled . because the in - stent restenosis was too long to treat with a coated balloon , that usually can be used only one time for each inflation , an infusion balloon such as the genie ( acrostak ag , stegackerstrasse 14 , winterthur , switzerland ) was selected . the device is composed of a balloon with two heads at each distal extremity which allow for stopping the blood flow for at least 80 s ( ideally 120 s ) and a central chamber with micro - holes which is filled up with liquid paclitaxel ( 130 - 170 mol ) ( figure 1e ) , and can be implanted at a low pressure ( 2 atm ) . thus , the rca was wired and dilated with multiple inflation of a standard 3.0 30 mm sprinter balloon and then , four dilatation along the entire rca was accomplished with a 3.5 28 mm genie catheter . the immediate final ( figure 1f ) as well as 6 mo follow - up coronary angiographic results ( figure 1 g ) were excellent . the patient is free from symptoms and silent ischemia at 9 mo clinical and instrumental follow - up . the presented case suggests that drug - infusion balloon can offer an effective therapeutic option in selected patients with very extensive in - stent restenosis after des implantation . although the number of published trials and patients treated with drug eluting - balloon is still limited , and its effectiveness in treating in - stent restenosis , and in particular in the treatment of restenosis after des has been only suggested but not yet proved , there is some promise for drug - coated stent for such purpose . most drug - coated balloon can not be used for more than one inflation and thus resulted useless in long segment treatment . the genie catheter has been used in in - stent restenosis after bare - metal stent implantation . however , to the best of our knowledge , this is the first report about its use in very long proliferative and occlusive in - stent - restenosis after des treatment .
drug - coated balloon has been developed as an alternative to drug - eluting stents for in - stent restenosis but the performance of drug infusion balloon in such setting has not been previously described . we present a case of particularly aggressive in - stent restenosis after drug eluting stent implantation treated with a new kind of drug infusion balloon developed in order to overcome the impossibility to inflate regular drug - coated balloon for several dilatation .
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a 26-year - old female was admitted to the neurology unit for fever , severe temporal , parietal and occipital headache , paresthesia of the hands and involuntary blinking of the left eye which had started 10 days before . a transient episode of aphasia she had undergone dental procedures some months before without any antibiotic prophylaxis . on physical examination a holosystolic murmur was heard . the erythrocyte sedimentation rate and the c - reactive protein were slightly increased while the white blood count was normal . a computed tomography scan detected two small areas of hyperintensity compatible with subarachnoid haemorrhage in the left parietal lobe ; a smaller area with the same characteristics was detected in the right parietal lobe . magnetic resonance imaging ( mri ) and angio - mri revealed an irregular , nodular image of 4 mm with high flow , in the left parietal lobe , interpreted as a vascular malformation ; two smaller areas with similar characteristics were observed in the left and the right parietal lobe . angiography revealed three small aneurysmal dilatations along the course of the left paracentral lobular artery , the left superior parietal artery and the left angular artery ( figure 1 ) . aneurysms were interpreted as possible mycotic aneurysms and an echocardiography was requested because infective endocarditis was suspected . a trans - thoracic ecocardiography confirmed the mitral prolapse with moderate insufficiency and revealed thickened mitral lembs . a follow up transesophageal ecocardiography documented remarkable reduction of the thickness of the lembs of the mitral valve and an improvement of the mitral regurgitation . a follow up mri of the brain showed hemosiderin deposits as a result of bleeding . a follow up scintigraphy showed the resolution of the accumulation on the mitral valve and at brain level . according to the most recent guidelines of the american heart association , issued in 2007 , antibiotic prophylaxis is no longer indicated in patients with mitral prolapse undergoing dental procedures , as it was in the previous edition . this decision has been criticized by some authors who reported cases of infective endocarditis occurring in patients with such a cardiac defect and undergoing dental procedures without any prophylaxis . though infective endocarditis in these patients can not be attributed to dental procedures for sure , we believe a more prudent approach should be considered . the diagnosis of endocarditis is based on the duke criteria . in our case , the following occurred : one major criteria ( major echocardiographic findings ) and four minor criteria ( fever , embolism , predisposing heart condition and minor microbiological criteria ) . positive leukocyte scintigraphy is not included in the duke criteria ; however , in our case , it was consistent with the diagnosis of endocarditis with cerebral embolism . some data suggests scintigraphy is of little value in the evaluation of patients with suspected endocarditis , since vegetations consist mainly of masses of fibrin , clotted platelets , blood cell debris , bacteria and only a few leukocytes . other studies suggest a positive granulocyte scan correlates with high activity of the inflammatory process and predicts a poor prognosis for the patients concerned . probably , more evidence is needed to define the role of scintigraphy in the diagnosis of infective endocarditis . indications for therapy with daptomycin approved by the fda include staphylococcus aureus bloodstream infections including right - sided endocarditis and daptomycin is also considered as an alternative option for the empirical treatment of endocarditis on native valves and the treatment of endocarditis due to gram positive bacteria . daptomycin is not generally recommended for infections of the central nervous system since there is no adequate evidence on its penetration in the cerebral parenchyma and the cerebrospinal fluid . however , in our case , we considered cerebral mycotic aneurysms as caused by the infection of the vascular side of the wall of the vessels . our report suggests that daptomycin is safe and effective in case of left endocarditis with cerebral embolism .
a young girl was admitted for fever , headache , paresthesia of the hands , involuntary blinking of the left eye and aphasia . imaging revealed mycotic cerebral aneurysms and finally infective endocarditis was diagnosed and successfully treated with daptomycin . she had a history of mitral prolapse and she had undergone dental procedures some months before without any antibiotic prophylaxis , according to the 2007 guidelines of the american heart association .
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the british thoracic society have provided guidelines that detail a systematic approach to the investigation of unilateral pleural effusion . our case highlights the potential for serious harm when pleural procedures are performed without bedside pleural ultrasound . pulmonary tuberculosis ( tb ) is a leading cause of death worldwide , especially in developing countries . diagnosis can be made from sputum for microscopy for acid - fast bacilli ( afb ) and tb culture . pleural tb usually presents with symptoms such as pleuritic chest pain , cough , and fever and it is recommended that when pleural tb is suspected , patients should undergo thoracocentesis and a blind closed pleural biopsy ( bcpb ) . in our institution , bcpb is not performed , which posed a dilemma since the patient was deemed too unwell for a thoracoscopy . our patient was an 86-year - old man , never smoker , who presented to a regional hospital with a 4 weeks history of nonproductive cough , dyspnea , and left pleuritic chest pain . he had migrated to australia from korea 40 years ago , and his only significant medical illness was atrial fibrillation for which he was receiving oral digoxin 125 mcg daily . at the time of hospital presentation , blood tests demonstrated leukopenia 3.2 10 9/l and lymphopenia 0.76 10 9/l . c - reactive protein was 145 mg / l ( normal < 5 mg / l ) . however , the medical officer did not use image guidance and mistakenly attempted the thoracocentesis on the right hemithorax instead of the left side . chest x - ray showing moderate amount of left sided pleural effusion in our institution , we performed a bedside pleural ultrasound - guided thoracocentesis . the pleural fluid analysis revealed the fluid to be an exudate ( protein 54 g / l and lactate dehydrogenase 289 u / l ) . since the cause of the effusion was unknown , 1 week later a 2 thoracocentesis was performed which was also nondiagnostic . the patient underwent a computed tomography ( ct ) chest which revealed a moderate amount of left - sided pleural effusion and an irregular left upper lobe linear nodular opacity [ figure 2 ] . our institution does not offer an induced sputum test , and consequently , the patient underwent a bronchoscopy , and bronchial lavage was performed on the left upper lobe . computed tomography chest showing left sided pleural effusion and apical pulmonary nodular opacity clinically , the patient continued to deteriorate and was now bed bound . to obtain a pathological diagnosis , it was decided that a pleural biopsy must be performed as a next step investigation . bcpb equipment was not available in our institution . due to his poor performance status , furthermore , there was no discrete pleural tissue that could be biopsied using ct image guidance . six weeks later the bronchial lavage culture grew mycobacterium tb , which was sensitive to first - line anti - tb agents . hence , he was commenced on standard daily regimen antibiotic treatment consisting of isoniazid 300 mg daily , pyridoxine 25 mg daily rifampicin 600 mg daily , pyrazinamide 1500 mg daily , and ethambutol 800 mg daily were prescribed for 2 months followed by isoniazid and rifampicin for 4 months . over the course of the 6 months , finally , 8 months after he initially presented to hospital , he was discharged from the respiratory clinic after completion of anti - tb treatment . we believe that this case report has highlighted three pertinent issues in the management of patients ' with pleural effusion : wrong side thoracocentesis , lack of equipment to perform bcpb and induced sputum . it is estimated that approximately 178,000 thoracenteses are performed per year in the united states . typically , thoracocentesis is a safe procedure but can result in significant complications including pneumothorax , hemorrhage , and death . despite these efforts , wrong side thoracocentesis can still occur . they found that absence of verification images to be cause in almost 50% of cases . consequently , there has been a trend to the widespread implementation of ultrasonography , training , and restriction of thoracentesis to experienced health care professionals . another reason for the delay in diagnosis was the lack of availability of equipment to perform a bcpb in our institution . however , it has now been demonstrated that if the pleural biopsy is guided by an imaging technique ( ultrasound or computed tomography ) , its yield is even better . the increasing availability of video - assisted thoracoscopy and the low prevalence of tb effusions in developed countries has led to the declining use and experience with bcpb . the third reason for delays in the diagnosis of tb was the lack of availability of induced sputum in our institution . a randomized study previously demonstrated that induced sputum has a greater diagnostic yield and is more cost - effective than bronchoalveolar lavage in detecting active pulmonary tb in patients who can not produce spontaneous sputum . induced sputum is performed with hypertonic saline solution delivered by nebulizer and samples can be obtained readily . this is in contrast to bronchoscopy , where immediate availability is not always possible due to staffing , bronchoscopy suite availability , and time constraints . this case highlights certain health care system deficiencies that resulted in delays and potential harm to our patient with pleural tb . importantly , these deficiencies are readily amenable to correction , and our health care service is currently undertaking reform to rectify these errors .
we report the case of an elderly asian man where a medical error and diagnostic delays obscured the diagnosis of pleural tuberculosis ( tb ) . the patient was hospitalized for evaluation of a unilateral pleural effusion . initially , the patient was subjected to a pleural aspiration on the wrong side due to a lack of bedside ultrasound guidance . subsequently , the patient underwent several investigations but not a blind closed pleural biopsy ( bcpb ) due to a lack of equipment . furthermore , the patient was deemed to be too sick to undergo a thoracoscopic pleural procedure . eventually , a bronchoscopy was performed , and washings from the right upper lobe were cultured , which established the diagnosis of tb . this case highlights the need to use bedside ultrasound in the investigation of pleural effusions , the role of bcpb especially in frail patients and finally the utility of bronchoscopy in establishing a diagnosis of pleural tb .
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the highest prevalence is seen in sephardic jews from iran and iraq that is 1:3000 who also often have an associated coagulation factor vii deficiency ( 14 ) . dubin johnson syndrome manifests with an intermittent jaundice in the first two decades of life . pregnancy or intake of oral contraceptive may provoke manifestation of the disease . except for the jaundice the patient is a 22-year old male that came to the office 3 year ago for the first time with fever , jaundice , fatigue and dark urine . he had no history of alcohol intake or any drug use ( herbal or chemical ) . liver function test were impaired and all serological studies was normal but havab igm was positive , the patient was being treated with the suspicion of acute viral hepatitis a ( table 1 ) . paraclinical evaluations from first presentation until present ast= aspartate aminotransferase ; alt= alanine transaminase ; alp= alkaline phosphatase ; inr= international normalized ratio ; alb= albumin ; lkm ab= liver kidney microsome antibody ; asma= anti - smooth muscle antibody ; ana= antinuclear antibody ; ab= antibody ; ag= antigen ; ig= immunoglobulin ; hbs= hepatitis b surface ; hbc= hepatitis b core ; hcv= hepatitis virus type c ; hiv= human immunodeficiency virus ; hav= hepatitis a virus ; tsh= thyroid stimulating hormone ; s.p= serum protein ; ema= endomysial antibody ; p - anca= perinuclear anti - neutrophil cytoplasmic antibody in the recent past 3 years total bilirubin has been fluctuates between 8.9- 13.5 mg / dl and the direct part between 7.4 - 9.5 mg / dl . despite the regression of symptoms and reduction of liver enzymes to the normal levels , the jaundice remained persistent ( table 1 ) . abdominal sonography in 3 years ago revealed nothing significant but mild hepatomegaly with heterogenous echo . recently , liver biopsy was done and containing a specimen consisting of two pieces of small creamy needle - shaped dark brown tissue totally measuring 3 cm in length and 0.1 cm in diameter . individual hepatocytes contain abundant course brown pigment granules especially in perivenular areas portal tracts show mild lymphocytic infiltration . he had one episode of upper gi bleeding that esophagogastroduodenoscopy revealed grade b esophagitis and small duodenal ulcer . in urine djs is listed as a rare disease by the office of rare disease ( ord ) of the national institutes of health ( nih ) ( 6 , 7 ) . in this situation the diagnosis is based on clinical and laboratory findings especially liver biopsy . despite the normal liver enzymes , only the bilirubin is higher than normal level that is mainly conjugated part . in liver biopsy the total coproporphyrin in urine is normal , but 85 - 90% of urinary coproporphyrin is type i , whereas in normal persons 75% of urinary coproporphyrin is type iii ( 7 , 9 , 10 ) . in our patient the jaundice was being provoked after a viral hepatitis and despite comprehensive work up , only direct hyperbilirubinemia was seen .
elevated serum level of bilirubin is a common manifestation which is occurred in several diseases . hyperbilirubinemia can manifest either conjugated or unconjugated . conjugated or direct hyperbilirubinemia usually are caused by hepatocellular diseases or cholestatic liver diseases . merely conjugated hyperbilirubinemia is the main manifestation of two congenital syndromes , including dubin - johnson and rotor syndrome ; however it can be seen in some patients with recurrent benign intrahepatic cholestasis . this article reports a patient with dubin- johuson syndrome as a benign and rare condition .
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we administered intravitreal bevacizumab injection to eight eyes of eight patients of various etiologies ranging from retinal vein occlusion to diabetic retinopathy , age related macular degeneration amd , and choroidal neovascular membrane cnvm . the best corrected visual acuity of seven out of eight patients was poor and ranged from 20/120 to finger counting . the details of the procedure , complications were discussed with the patients , and written consent taken . injection bevacizumab was procured by a patient five days prior to the date of appointment and stored under recommended conditions ( i.e. , below 4c ) . on the day of injection , under aseptic precautions , the vial was opened in operation theatres , hood was not used due to its nonavailability . the syringe was then capped with a 30-gauge needle and kept on a sterile tray . best corrected visual acuity of patients and indication for intravitreal injection the eye of each patient was prepared following standard aseptic procedures ( i.e. , lids cleaned sequentially first with spirit and then with 10% povidone - iodine ) . fornices were flushed with normal saline and 1 drop of 10% povidone - iodine was instilled 2 min before the procedure . intravitreal bevacizumab injection was administered into the superotemporal quadrant , 4 mm from the limbus . different needles were used each time after cleaning the surface of vial with spirit , thereby administering multiple pricks in the vial . all of them were asked to come next week for follow - up or earlier if patient experienced severe discomfort . four of the eight patients reported to the hospital on the 3rd day after injection with complaints of pain , watering , and diminution of vision . two patients who did not report by the 4th day were contacted and recalled for an examination . six out of eight patients had absent fundal glow along with presence of cells and flares [ table 2 ] . clinical findings in patients following intravitreal injection of bevacizumab these six patients were clinically diagnosed to have endophthalmitis and were administered intravitreal antibiotics ( injection ceftazidime 2.25 mg in 0.1 ml and injection vancomycin 1 mg in 0.1 ml ) on the same day of presentation . vitreous samples and drug vial were sent for culture sensitivity in two different laboratories which turned out to be sterile . intravitreal antibiotics were repeated after 48 h. all patients were closely followed up and remaining drug was discarded . while pegaptanib and ranibizumab are labeled for intravitreal use , bevacizumab is labeled for use in cancer therapy and is currently being used off - label for the treatment of ocular neovascular diseases . because of its off - label use , bevacizumab is supplied in much larger volumes than those needed for single intravitreal injection . thus , hospitals and compounding pharmacies must divide the larger volume of bevacizumab into smaller units suitable for single - use , individual doses . contaminants could possibly be introduced during the compounding process and compromise the sterility of the aliquoted drug . multiple pricks ( procedure common in india ) were made in vial to prepare administrating dose for eight patients . an alternative protocol suggested is that small aliquot of drug should be prepared using single prick technique , i.e. , 0.5 in . 26 gauge needle should be inserted into rubber cap of vial and drug should be drawn into different 1 ml syringes , every time changing only the syringe , leaving the needle in place . next group should be administered injection after one week , i.e. , after the first follow - up of the previous batch . in this way , we would be able to minimize incidence of cluster endophthalmitis and detect possible contamination in the compounded aliquoted drug before it is administered to the next batch . if required , each eye should be injected using drug from different lots under sterile conditions . six out of eight patients had endophthalmitis , remaining two patients though belonging to different age groups ( 50 and 72 years ) , did not develop endophthalmitis . the possible reason for this could be the inherent immunity against the causative organism or the quantity of causative organism in the inoculums could have been below the threshold required for endophthalmitis . presentation of cases with signs and symptoms of endophthalmitis and response with intravitreal injection of antibiotics led us to assume infective pathology despite the negative culture report . the possibility of tass syndrome in these patients was considered , but review of literature suggests that series of patients , who developed tass syndrome in canada , had reported as early as 24 h. the final visual outcome was poor even with aggressive treatment . all patients had worse visual acuity at the end of follow - up than on injection day . four patients in our study presented on day 3 , while two patients reported on day 4 , after intravitreal injection . three out six patients with endophthalmitis showed improvement in visual acuity with intravitreal antibiotic therapy , as compared to pretreatment level . visual acuity remained same in two cases , while it deteriorated drastically in one case even after aggressive treatment [ table 3 ] . as the approval of intravitreal use of bevacizumab and its subsequent availability in the market in single dose ( 0.05 ml ampoules ) is still awaited , using the single - dose vial and aliquoting into smaller doses for multiple uses , is the call of the day . however , the present incident highlights the risks of microbial contamination and the need to stay vigilant against preexisting contamination within the vial or its access to the drug via multiple pricks . the alternative protocol , as described previously , is recommended to increase the safety margin of the intravitreal injection of bevacizumab .
the risk of endophthalmitis is always a concern when an intraocular procedure is performed . intravitreal injection is a frequently used method for therapeutic management of many diseases , affecting the posterior segment of the eye . hence , it is important to assess the risk of complications , especially endophthalmitis . most studies conducted concentrate on risk assessment from single use from single drug vial . the present article reports the occurrence of cluster endophthalmitis following multiple intravitreal bevacizumab injections from a single vial . intravitreal injection of bevacizumab was administered to eight eyes of eight patients . administered dose was prepared from single 4-ml vial of bevacizumab and was injected in the eye , after patient preparation and under aseptic conditions . the procedure was repeated for the remaining patients , thereby imparting multiple pricks in the same vial . four of the eight patients reported to the hospital on the 3rd day after injection with complaints of pain , watering , and diminution of vision . two patients reported the following day with similar complaints . two patients who did not report by the 4th day were contacted and recalled for an examination . all the patients were thoroughly examined using slit lamp biomicroscopy and indirect ophthalmoscopy . six out of eight were clinically diagnosed to have endophthalmitis and were administered intravitreal antibiotics . the present report highlights possibility of microbial contamination of the drug vial or during compounding process . however , from the present incident , we are encouraged to stay vigilant and wary of contamination
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multiple myeloma is a malignant neoplasm that is characterized by a monoclonal proliferation of plasma cells . the clinical manifestations of the disease occur as a result of an expanding plasma cell mass in the bone marrow and other factors produced by these cells such as monoclonal immunoglobulin , bence - jones proteins and osteoclast activating factors . the common clinical signs and symptoms of multiple myeloma include pain in the bone , fatigue , anemia and infectious diseases . oral and maxillofacial manifestations as an initial sign or symptom of multiple myeloma are rare . in 12 - 15% of cases , oral involvement can be apparent as swelling , orofacial pain , mobility of teeth , numbness and paresthesia , hemorrhage , fracture and root resorption . we hereby present a case of multiple myeloma with first clinical manifestation as generalized gingival enlargement . a 58-year - old male patient was referred to our department because of the generalized enlargement of gingiva . the enlargement was first noted by the patient 6 months prior to the referral , and progressed steadily since then . the intraoral examination revealed soft , granular , friable , non - tender , and red / magenta enlargement that bled spontaneously . the enlargements were present on both buccal and lingual / palatal sides [ figure 1 ] . on physical examination , grade iii mobility was observed in 16 , 17 , 18 , 36 , 37 . the medical history of the patient included epilepsy for which he took prescribed medication with no occurrence of seizure for last 10 years . based on clinical presentation , a provisional diagnosis of inflammatory gingival enlargement routine blood investigations were done along with hiv and hepatitis b and sputum examination to rule out any leukemic infiltration and enlargement associated with tuberculosis . orthopantomography was advised , which revealed severe bone loss in 16 , 17 , 18 and 36 , 37 regions . after phase i therapy and consultation with the physician , gingivectomy was performed in anterior mandibular region and excised tissue was sent for histopathological examination . the patient was followed up every week [ figure 2 ] but after 1 month the clinical examination revealed recurrence of enlargement in the anterior mandibular region [ figure 3 ] . the subepithelial zone showed infiltration by sheets of plasma cells mainly mature with few being less differentiated [ figure 4 ] . based on these findings , serum protein electrophoresis and urine analysis for bence - jones proteins was carried out . subsequently , patient was advised to undergo cranial and pelvic radiography [ figures 6 and 7 ] . the patient was diagnosed as a case of multiple myeloma and chemotherapy was started with thalidomide . gingiva 10 days after excisional biopsy was taken recurrence after 1 month of biopsy areas of ulceration and sheets of plasma cells multinucleate and binucleate plasma cells pelvic radiograph showing osteolytic lesions skull radiograph showing osteolytic lesions normal gingiva 1 month after chemotherapy multiple myeloma is the most aggressive plasma cell neoplasia and most common primary malignancy of bone . it has a predilection for areas of active hematopoiesis such as the lumbar spine , ribs , and pelvic bones . jawbone involvement in multiple myeloma is common and often occurs in the advanced stages of the disease . jaw involvement in multiple myeloma was reported by bruce and royer to have a prevalence rate of 28.8% ( 17 of 59 total cases ) . epstein et al . , reported that 14.1% of 783 multiple myeloma cases had oral manifestations . lesions such as swelling , orofacial pain , mobility of teeth , numbness and paresthesia , hemorrhage , fracture and root resorption are more frequently found in the mandible than in the maxilla , especially in the posterior third and angle of the jaw , perhaps because of greater hematopoietic activity in these areas . a radiographic survey of patients with multiple myeloma reveals multiple well - defined punched out radiolucencies involving bone . these radiolucent areas of bone contain the abnormal plasma cell proliferations that characterize the disease . in our case , type 2 bone involvement was seen , which is by far , the most common presentation of multiple myeloma . in conclusion , dental surgeons can play an important role in the early recognition of oral lesions with underlying systemic disease , thus preventing the morbidity and mortality associated with such pathologies .
multiple myeloma is a malignant neoplasm that is characterized by a monoclonal proliferation of plasma cells . oral and maxillofacial manifestations as an initial sign or symptom of multiple myeloma are rare . a 58-year - old male patient presented with generalized gingival enlargement for last 6 months . based on clinical presentation , a diagnosis of gingival hyperplasia was made . after phase i therapy , excisional biopsy was taken in anterior mandibular region and excised tissue was sent for histopathological examination . the histopathology report revealed a lining of stratified squamous epithelium with foci of ulceration . the subepithelial zone showed infiltration by sheets of mainly binucleate and multinucleate plasma cells , few cells being less differentiated . rounded cytoplasmic inclusion bodies were identified in many of these cells . after a series of clinical investigations , a case of multiple myeloma was diagnosed . patient presenting with generalized gingival hyperplasia should be worked up for systemic disease like multiple myeloma .
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chest pain is a typical symptom of acute coronary syndrome ( acs ) , of which there are three common types : unstable angina ( ua ) , st segment elevation ( stemi ) of myocardial infarction ( mi ) , and non - st - segment elevation of mi ( nstemi ) [ 1 , 2 ] . symptoms of acs in the absence of chest pain are dyspnea , nausea , sweating , neck or jaw and arm pain . ear pain and sore throat are rare symptoms of acs , which makes diagnosis and treatment difficult and challenging . we present a rare case of a patient presented to the emergency department of our hospital with symptoms of earache and sore throat for cardiac ischemia . a 53-year - old african - american female presented to the ed complaining of earache and sore throat for 3 days . she described the pain as a burning sensation that was worst when she woke up in the morning and got better as the day progressed . the patient denied chest pain , shortness of breath , nausea , vomiting , or dizziness . the patient worked as a telemarketer , described her job as very stressful , and claimed to smoke one to two cigarettes per week . her medical history was significant for dyslipidemia , newly diagnosed type 2 diabetes , and chest discomfort diagnosed 4 months ago at a different hospital as stemi with complete occlusion of the rca . she was treated at the hospital by placement of a bare - metal stent in her rca . current home medications included clopidogrel , aspirin , statin , beta - blocker , metformin , and nitroglycerin sublingually as needed for chest pain . her vital signs on arrival at the ed were : bp of 131/82 mmhg , pulse of 70 beats per minute , respiratory rate of 17 breaths per minute , temperature of 98 f , and oxygen saturation of 98% on room air . because of multiple risk factors , in addition to a symptom that could potentially be a referred cardiac pathology , an ecg , chest radiograph , and cardiac biomarkers were ordered at the triage . cardiovascular , pulmonary , abdominal , ear , nose , and throat examinations were unremarkable . the initial ecg displayed no st segment elevation , but t wave inversion in inferior leads that was unchanged from a previous ecg . a presumptive diagnosis of acs/ nstemi was made on the basis of an elevated troponin combined with the ecg changes . after the acs protocol was initiated , a cardiology consultation was requested , and the patient was admitted to our hospital . on admission , the patient continued to complain of throbbing ear pain that fluctuated now between the two ears , but again denied chest pain or shortness of breath . on the 2nd day it was found that the previous bare - metal stent in the posterolateral branch ( distal rca ) was completely occluded , and no balloon could be advanced beyond that area . a new lesion in the proximal / ostial rca greater than 70% occlusion was opened with a successful placement of a pci at the ostial rca using a drug - eluting stent . following this , her troponin level had declined to 0.63 ng / m on the 3rd day , and the patient was discharged with a referral for cardiac rehabilitation . we concluded on follow - up that otalgia in this patient was an atypical symptom of referred angina pain . the patient was seen 2 weeks later at the cardiology clinic of our hospital and reported no complaints about her condition or recurrence of ear pain . an ecg revealed no changes from the previous one done 2 weeks earlier at the time of discharge . the underlying pathophysiology of the referred otalgia in this patient can be explained by the autonomic dysfunction of the auricular branch of the vagus nerve often termed alderman s nerve . this branch of the vagus innervates the inner portion of the outer ear , and also controls the skeletal muscles including the superior , middle and inferior pharyngeal constrictors . the sinoatrial ( sa ) node we suggest that partial occlusion of the rca promoted damage of the parasympathetic fibers of the right vagus , and was the cause of referred otalgia and pharyngitis in our patient . sensory nerves are affected by type 2 diabetes [ 47 ] , but whether such a preexisting condition predisposes a person to angina - referred otalgia or pharyngitis is not clear . to our knowledge , symptoms of ear pain for cad and acute arterial occlusion in a patient have rarely been discussed in the literature . we found only one report ; in this report the correct diagnosis of cardiac ischemia was missed in two patients who were initially treated for primary ear pain . in conclusion , clinicians should be aware and recognize that ear and throat pain may represent symptoms for cardiac ischemia .
a rare case of a patient with unusual symptoms of earache and sore throat for cardiac ischemia is presented . a diagnosis of non - st - segment elevation myocardial infarction ( nstemi ) was made based on initial elevation of troponin and an abnormal electrocardiograph ( ecg ) . percutaneous coronary intervention ( pci ) performed with stent placement in the occluded coronary vessel was followed by a decrease in troponin level and complete resolution of the ear and throat pain and patient recovery from cardiac ischemia .
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intracranial foreign bodies frequently result from trauma , including penetrating injury , and rarely involve wooden materials compared with metallic materials . when intracranial penetrations of wooden objects occur through the transnasal or transorbital route , physical examinations may reveal no abnormalities , and the objects may be difficult to visualize with conventional radiography . we present a case involving the transnasal intracranial penetration of a wooden branch that resulted in a delayed intracranial infection due to initial misdiagnosis . a 48-year - old man presented to the emergency department with complaints of headache the day before . two days earlier , he fell to the ground in a drunken state , and he could not clearly remember the incident . at the emergency department , no neurological or physical abnormalities , such as external contusions or cerebrospinal fluid rhinorrhea , were observed . computed tomography ( ct ) that was performed upon admission showed a round hypodense signal in the left frontal area , which suggested pneumocephalus , and a small amount acute subdural hematoma in the left frontotemporoparietal area ( figure 2 and 3 ) . the patient was admitted to the neurosurgical department for close observation , and prophylactic antibiotics were administered for the pneumocephalus . brain magnetic resonance imaging ( mri ) revealed a fistula from the left nasal cavity to the frontal lobe with enhancement along the tract and ventricular lining ( figure 4 ) . the foreign body was a wooden branch ( 11 cm long and 0.7 cm wide ) covered with brain tissue ( figure 5 ) . after the operation , the patient received intravenous antibiotics ( ceftriaxone plus vancomycin ) for over 2 months . a microbiologic culture study on the foreign body showed gram - positive cocci ( s. aureus ) . intracranial penetrating injuries by foreign bodies represent only 0.4% of all head injuries and usually occur in children because of falls.17 ) intracranial foreign bodies that cause severe intracranial infections can be fatal.8 ) miller et al.7 ) reviewed 42 cases with intracranial penetrating injuries and found that 64% developed central nervous system infections , 45% had additional brain abscesses , and the death rate was 25% . generally , the time from the injury to the clinical intracranial infection presentation is long , but it can vary from a few days to several years.5 ) therefore , careful history taking and thorough physical and radiologic examinations are essential . however , intracranial wooden foreign bodies are difficult to detect with plain radiographic techniques and distinguish from periorbital fat and air in the nasal cavity on ct images.48 ) the ct attenuation values of wooden foreign bodies vary depending on their water content . freshly cut wood has a relatively high physical density because of its high water content ; therefore , it is difficult to distinguish it from soft tissues , such as muscle and vitreous.3 ) conversely , when wood dries , the water is gradually replaced by gas , and its density is difficult to distinguish from that of fat or air.3 ) consequently , intracranial wooden foreign bodies have variable densities over time and are difficult to distinguish from the surrounding structures . tasneem et al.11 ) described a case involving a radiolucent intracranial wooden foreign body and the usefulness of the lung window setting for detecting wooden foreign bodies on ct images . careful review of ct images with the lung window setting can be helpful for detecting a wooden foreign body . unlike ct images , mri is more useful for detecting wooden foreign bodies that appear hypointense on t1- and t2-weighted mri scans and that can be distinguished from air or fat.69 ) in addition , infected lesions exhibit gadolinium enhancement . the initial ct image of the patient showed a single area of air in the frontal lobe . steudel and hacker10 ) reviewed 508 cases with acute head injuries and found that 49 ( 9.7% ) had a pneumocephalus on brain ct scans , with 5 ( 1% ) showing a single air bubble and 6 ( 1.1% ) having an intracerebral or intraventricular location . however , they did not experience a case involving a single air bubble with an intracerebral or intraventricular location . these findings suggest that patients with a pneumocephalus with a rare presentation need to be examined more carefully . patients with retained intracranial wooden foreign bodies frequently develop delayed infectious complications , and surgical removal is necessary , even in the absence of symptoms.178 ) our patient experienced late - onset seizures , which could have been secondary to gradual gliosis , progressive granulomatous changes , or delayed abscess formation , as has been noted in cases with retained foreign bodies.2 ) therefore , if intracranial injury from a wooden foreign body is suspected , careful history taking and imaging are very important . once the diagnosis of an intracranial foreign body is confirmed , surgical removal of the foreign body is required . the present case illustrates the necessity for special attention to patients suspected of having pneumocephalus with a rare presentation during the initial examination . early surgical removal of the intracranial foreign body is necessary to prevent complications .
intracranial wooden foreign bodies are rare . in addition , such objects are difficult to identify with conventional radiographic techniques , such as x - ray radiography or brain computed tomography . a 48-year - old man presented to our emergency room with a headache . even though he had a history of trauma , he had no external wounds and showed no neurological deficits at the initial examination . he was initially diagnosed with trauma - related pneumocephalus . he developed a delayed intracranial infection and underwent surgery to remove the wooden foreign body . the present case illustrates the necessity for special attention to patients suspected of having pneumocephalus with a rare presentation during the initial examination . early surgical removal of the intracranial foreign body is necessary to prevent complications .
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the retrorectal space is an uncommon area where tumors occur and these include primary tumors of neurogenic , osteogenic , and congenital origin ; in addition to metastatic and inflammatory processes . congenital lesions include chordomas ( remnants of notochord ) , teratomas , anterior sacral meningoceles , and developmental cysts ( dermoid , epidermoid , enteric duplication , and tailgut cysts ( tgcs ) ) . tgcs , also known as retrorectal cystic hamartomas , are a rare congenital lesion thought to arise from the remnants of the embryonic postanal gut . hjermstad and helwig were the first to publish their findings in 1988 , and since then there have been no large case series reported . from review of the literature done by killingsworth and gadacz ( keyword = tailgut cyst or retrorectal cystic hamartoma , limits = english ) , there have been 43 cases with confirmed diagnosis of tgc since their report . a 15-year - old girl presented with the complaints of lower abdominal pain and constipation occasionally . however , on per rectal examination , there was a mass bulging from the posterior rectal wall , firm , and non - tender , with regular surface and smooth mobile rectal mucosa over it . an ultrasonogram ( abdominal ) revealed a large cystic lesion present in the left lower abdomen and the left ovary could not be seen separately . the patient then underwent a contrast - enhanced computed tomography ( cect ) of the abdomen and pelvis which revealed a well - defined 12 13 9 cm multiseptated lesion in the presacral space which was pushing the rectum laterally and urinary bladder superiorly and abutting the sacrum and coccyx posteriorly [ figure 1a and b ] . the lesion was showing peripheral and septal calcification , few hyperdense nonehancing areas and few ossified fragments within it . a provisional diagnosis of mature cystic teratoma was made and the patient underwent exploratory laparotomy wherein a large tubular tense cystic mass resembling fluid - filled intestinal loop filled with thick mucoid material was present in the presacral space [ figure 2 ] . the two ends of the tube were merging at the coccyx . the mass was displacing the sigmoid colon and rectum laterally and urinary bladder anteriorly . en masse removal was done . cect abdomen showing multiseptated pre sacral mass compressing the rectum and displacing bladder superiorly intraoperative picture showing a tubular fluid - filled structure displacing the bowel loops the patient had an uneventful postoperative recovery . the histopathological examination revealed it to be a retrorectal cystic hamartoma with areas of intestinal ( large and small ) and gastric epithelium . a solitary solid area within it had intestinal lining with area of squamous epithelial nests , haphazardly arranged muscle bundles , nerve bundles , and serous acini with few cystic spaces [ figure 3a c ] . histopathological image showing ( a ) gastric mucosa , ( b ) ectopic gastrointestinal gland and ( c ) ectopic pancreatic epithelium the retrorectal space is a potential space developed when a mass displaces the rectum anteriorly . the pelvic peritoneal reflection forms the superior border , and the levator ani and coccygeus muscles form the inferior border . the differential diagnosis of masses within this space is broad and includes primary tumors of neurogenic , osteogenic , and congenital origin ; in addition to metastatic and inflammatory processes . congenital lesions include chordomas , teratomas , anterior sacral meningoceles , and developmental cysts ( dermoid , epidermoid , enteric duplication , and tgcs ) . excluding inflammatory lesions , developmental cysts are the most common masses in the retrorectal space . . only one case of a retrorectal cystic hamartoma occurred in a 2-year - old child and very few cases have been reported in teen aged girls , as in our case . the differential diagnosis for a retrorectal mass can be narrowed using a combination of diagnostic tools to reach a preoperative diagnosis of a developmental cyst . due to their location , almost all retrorectal tumors will be palpable on rectal examination , and developmental cysts will manifest as extrinsic masses . ct and magnetic resonance imaging ( mri ) are useful imaging modalities that help in making a preoperative diagnosis . however , the definitive diagnosis and treatment is through complete surgical excision and pathological examination of the specimen . preoperative biopsy should not be attempted ( unless the mass is surgically unresectable at presentation ) due to risk of spreading dysplastic cells through weakened cyst walls . in addition , tissue obtained from biopsy is often not extensive enough to show all the histology features necessary for diagnosis . dermoid and epidermoid cysts are both lined with stratified squamous epithelium ; however , only dermoid cysts contain dermal appendages ( hair follicles , sweat glands , and tooth buds ) . epidermoid cysts are formed from inclusion of epidermal elements at the time of neural groove closure in the meninges . rectal duplication cysts are lined by typical gastrointestinal epithelium ( often with crypts , villi , and glands ) and are surrounded by two well - formed layers of smooth muscle with nerve plexuses . tgcs , or retrorectal cystic hamartomas , are predominantly multicystic and can contain a variety of epithelia between cysts or even within the same cyst . epithelial types include stratified squamous , transitional , mucinous or ciliated columnar , and cuboidal mucus secreting . in contrast to enteric duplication cysts , tgcs have disorganized smooth muscle fibers within the cyst wall and do not contain neural plexus . retrorectal hamartoma or tgc should be considered as a possible differential in any case of perirectal cyst , irrespective of age and gender .
the retrorectal space is an uncommon seat for neoplastic masses . retrorectal hamartoma or tailgut cyst ( tgc ) is an uncommon developmental cystic lesion occurring in this space which mostly occurs in middle - aged females . we recently cared for a 16-year - old girl who presented with vague lower abdominal pain and occasional constipation . per rectal examination revealed an extraluminal mass bulging from posterior rectal wall . preoperative radiological investigations revealed by suggested it to be a mature cystic teratoma . the patient underwent exploratory laprotomy with en masse excision of the cyst . histopathological examination of the specimen showed it to be a tgc . this case highlights the possibility of a tgc as a differential for retrorectal cystic lesions and the need to completely excise them given the possibility of future malignant transformation .
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ewing sarcoma / primitive neuroectodermal tumor ( es / pnet ) , previously thought to be separate tumors , is now treated as the same tumor ; both have similar immunohistochemical characteristics and chromosomal translocation . they are malignant tumors composed of undifferentiated small round cells , usually affecting children , adolescents , and young adults . generally es / pnet affects the bones and deep soft tissues , although other organs such as the pancreas , small bowel , esophagus , kidneys , prostate , ovaries , vagina and rectovaginal septum have been reported ; this is termed as extraskeletal es / pnet . to the best of our knowledge , only 5 cases of gastric es / pnet have been reported in the english language literature . a 31-year - old healthy female patient was admitted to the surgical ward due to upper abdominal pain and coffee ground vomiting of 3 days duration . the patient had no other complaints and was hemodynamically stable . rectal examination revealed melena . a nasogastric tube was inserted and revealed coffee ground secretions . upper endoscopic examination revealed a large ulcerated mass located at the lesser curvature of the stomach , with oozing of blood . biopsy revealed tumor cells showing positive immunoreactivity for cd99 ( fig 1 ) , fli1 , vimentin , and ki67 , and negative immunoreactivity for cytokeratin , s100 , cd20 , cd3 , cd79a , pax5 , cd30 , cd43 , dog-1 , cd68 , cd163 , cd33 , mpox , and desmin . es / pnet was suspected and fluorescence in situ hybridization ( fish ) analysis was ordered , which was positive for the ewsr1 gene rearrangement ( 11 : 22 translocation ) . total body computed tomography ( ct ) showed a hypodense mass measuring 9 cm at the lesser curvature of the stomach , with compression on the splenic vein ( fig 2 ) . positron emission tomography - ct ( pet - ct ) revealed pathological uptake of fluorodeoxyglucose at the gastric mass and lymph nodes at the gastrohepatic ligament ( fig 3 ) . the patient refused neoadjuvant treatment , and thus surgery was performed . on exploration of the abdomen , the mass was adhering to the pancreatic tail and mesentery of the transverse and descending colon , along with abnormal pathological lymph nodes at the greater curvature . histopathological examination revealed the mass measuring 11 cm in diameter to be an es / pnet invading the gastric wall , pancreas , and splenic hilum , without involvement of the left adrenal . three years postoperatively , the patient is doing well , with no evidence of disease recurrence . es , a term used to describe tumors that lack neuroectodermal differentiation , and pnet , used to describe tumors that exhibit neuroectodermal features , are now treated as a single entity . tumor cells are rich in glycogen , and pseudorosette formation characterizes the tumor 's morphological differentiation . the diagnosis can be made by immunohistochemical staining for a monoclonal antibody to cd99 ( hba/71 , 12e7 , and 013 ) , which is positive in almost all cases of es / pnet [ 5 , 6 , 7 ] . another immunohistological reagent that can be used for diagnosis is the intermediate filament vimentin , which is usually positive . markers showing variable immunohistochemical staining include s100 , chromogranin a , synaptophysin , and neuron - specific enolase . for tumors occurring in older patients or at unusual sites , fish or reverse transcription polymerase chain reaction can be used for diagnosis [ 3 , 8 ] . these are used to test for the presence of genetic mutation ( 11 : 22)(q24:q12 ) translocation ( ews / fli1 fusion ) , which is an essential criterion for the diagnosis of es / pnet , although sometimes these tests are negative [ 9 , 10 ] . due to the poor results of surgery alone as a treatment modality for extraskeletal es / pnet , the current recommendation is multimodal treatment including surgery , chemotherapy , and radiotherapy . the chemotherapeutical agents used as standard therapy for es / pnet include vincristine , doxorubicin , cyclophosphamide , ifosfamide , and etoposide . of these 5 cases , 3 were females and 2 males , with an average age of 46.6 years ( range 1468 years ) and an average tumor size of 8.5 cm . most of these patients presented with abdominal pain , and 1 presented with an abdominal mass . two cases had hepatic metastasis , 1 had lymph node and peritoneal metastasis , 1 no metastasis , and 1 was not reported . only 2 patients ( of the 3 with metastasis ) herein , we describe the 6th case of es / pnet of the stomach . due to the patient 's refusal of chemotherapeutical treatment three years postoperatively , the patient is doing well , with no evidence of disease recurrence . primary gastric es / pnet is a very rare tumor , and few cases are reported in the literature . due to its rarity , written informed consent was obtained from the patient for publication of this case report and accompanying images ; it is available for consultation . they confirm that the manuscript has not been published elsewhere and is not under consideration by another journal .
ewing sarcoma / primitive neuroectodermal tumor ( es / pnet ) is a tumor of small round cells arising in skeletal tissues . these tumors rarely arise in the stomach . we present a 31-year - old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage . upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature . biopsy revealed tumor cell immunoreactivity positive for cd99 , vimentin , and ki67 ( an index of proliferation ) . these findings were compatible with gastric es / pnet . the fluorescence in situ hybridization analysis result for the ewsr1 gene rearrangement ( 11 : 22 translocation ) was positive . the patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found . the mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon . total gastrectomy , distal pancreatectomy , splenectomy , and left adrenalectomy were done . the patient refused adjuvant treatment . she is free of disease 3 years after surgery .
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to explore the experiences of patients with cancer who were offered a nurse navigator in their course of illness before the in - hospital period . development has fragmentized healthcare systems in many countries , and coherence is now desired . among interventions suggested to reduce the fragmentation and improve delivery of care are help from patient navigators , where patients are offered extra help in a defined area by e.g. , a nurse [ nurse navigator ( nn ) ] . a phenomenological - hermeneutical longitudinal study was performed among danish gynecological patients from before an in - hospital period to two months after discharge . not all could use the help from nn . those who could , attached affectional bonds to nn and experienced benefit from her presence as well as her help . many had a feeling of deep - felt disappointment and felt rejected when the contact to nn stopped . resources for nn should be prioritized to patients who can use the help , and not stop prematurely . the traditional division and thinking by healthcare professionals are challenged , if all patients should be helped .
purposeto explore the experiences of patients with cancer who were offered a nurse navigator in their course of illness before the in - hospital period.theorydevelopment has fragmentized healthcare systems in many countries , and coherence is now desired . among interventions suggested to reduce the fragmentation and improve delivery of care are help from patient navigators , where patients are offered extra help in a defined area by e.g. , a nurse [ nurse navigator ( nn ) ] . patients experiences are of major interest , but have seldom been thoroughly investigated.methodsa phenomenological - hermeneutical longitudinal study was performed among danish gynecological patients from before an in - hospital period to two months after discharge . nn offered extra information , coordination , logistic services and emotional talk . semi - structured interviews provided data to the primarily open - minded analysis.resultsnot all could use the help from nn . those who could , attached affectional bonds to nn and experienced benefit from her presence as well as her help . many had a feeling of deep - felt disappointment and felt rejected when the contact to nn stopped.conclusionresources for nn should be prioritized to patients who can use the help , and not stop prematurely . the traditional division and thinking by healthcare professionals are challenged , if all patients should be helped .
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although in most cases ( 8090% ) the ingested foreign body will pass uneventfully through the gastrointestinal tract , sharp foreign bodies such as toothpicks are associated with an increased risk of gastrointestinal perforation and bleeding . the most common causes of the accidental ingestion of toothpicks include alcoholism , rapid food intake and decreased palatal sensitivity . although the complications caused by swallowed toothpicks are more likely to be detected and resolved by surgical procedure in selected cases when it is possible to locate the position of the toothpick , endoscopic removal can result in the rapid relief of symptoms [ 3 , 4 ] . a 57-year - old caucasian woman with no previous medical history was admitted to the department of internal medicine , division of gastroenterology , clinical hospital split . she complained of fever and abdominal pain located in right upper quadrant for one week . vital signs were as follows : blood pressure 120/80 mm hg , pulse rate 100 bpm and core body temperature 39.0c . laboratory evaluation revealed a wbc count of 12.5 10/l with a left shift and c - reactive protein concentration of 123.6 mg / l while urinalysis as well as other relevant laboratory data were within reference values . abdominal ultrasound examination was performed because of a clinical presentation mimicking acute cholecystitis , but it was within normal ranges . however , contrast - enhanced computed tomography ( ct ) scan with three - phase protocol showed an unclearly outlined lesion within the liver parenchyma with a longer diameter of 2.1 cm and which could not be clearly separated from eccentric radiopaque thickening of the stomach antrum in an extension of more than 4 cm ( fig . 1 , fig . this finding indicated gastroscopy , which revealed a sharp wooden foreign body protruding from the antrum mucosa and the whole wooden foreign body ( toothpick ) was successfully removed by snare extraction without complications . after the extraction of the foreign body the patient remembered that two weeks earlier when consuming lamb she might possibly have swallowed a toothpick . the patient was also treated with a proton pump inhibitor ( pantoprasole ) , the intravenous administration of crystalloids and ceftriaxone for seven days . impaction , perforation and obstruction most often occur in areas of acute angulation or physiologic narrowing . it is difficult to estimate the incidence of accidental ingestion of toothpicks , mostly because the literature covering this issue is anecdotal . in contrast to this , it is well known that toothpick lesions to the gastrointestinal tract are often associated with significant morbidity and mortality [ 2 , 3 , 4 ] . clinical presentation of toothpick gastrointestinal injury includes generalized or local peritonitis , intestinal obstruction or gastrointestinal hemorrhage . in some cases toothpicks migrated outside the gastrointestinal tract and were found in the pleura , pericardium , ureter or bladder [ 2 , 5 , 6 , 7 ] . ingested toothpicks in the esophagus and small intestines are presented by dysphagia or intestinal colic while foreign objects remaining in the stomach are often asymptomatic . early diagnosis and retrieval of the toothpick is critical for reducing morbidity and mortality [ 3 , 8 , 9 ] . therefore , we think that a patient presenting with gram - positive bacteremia and no sign of free perforation of the gastrointestinal tract is very illustrative because this demonstrates the necessity of thinking of foreign body ingestion as a cause of obscure bacteremias . we would like to emphasize how important it is to make a very careful anamnesis in order to determine swallowed foreign objects , such as a toothpick , as a rare cause of bacteremia . moreover , there are no relevant physical or laboratory findings characteristic for a swallowed toothpick . ct images are useful in acquiring missing clinical information such as toothpick location . in cases without free perforation ,
although most ingested foreign bodies usually pass through the gastrointestinal tract asymptomatically , toothpick injury to the gastrointestinal tract is often associated with significant morbidity and mortality . toothpick perforation of the gastrointestinal tract is frequently reported but , to the best of our knowledge , bacteremia caused by an impacted toothpick within the gastric mucosa has not yet been described . here , we report the case of bacteremia caused by an accidentally swallowed toothpick . the toothpick was impacted deeply in the gastric mucosa and was first seen and localized on contrast - enhanced computed tomography ( ct ) . ct scan is a very useful imaging technique in such situations since we lack typical and relevant physical findings or laboratory studies that go with accidentally swallowed objects , in this case a toothpick . flexible endoscopy was successful in extracting the whole toothpick . in cases without free perforation , flexible endoscopy is the treatment of choice in toothpick removal from the upper gastrointestinal tract .
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ectopic gastric mucosa and polyps related to brunner 's gland hyperplasia are the most common polyps . serrated polyps are characterized by infolding of the crypt epithelium , resulting in a saw - tooth appearance . a polyp with serrated morphological features has been classified histologically as hyperplastic polyp in the past . until the late 1990s , colorectal polyps were generally divided into two major subtypes : hyperplastic polyps and adenomatous polyps . in 1990 , serrated came from the observation of the saw tooth - shaped infoldings of the surface and crypt epithelium of these polyps that was similar to that of hyperplastic polyps . serrated polyps are now classified into 3 distinct types by histologic and genetic characteristics : hyperplastic polyp , sessile serrated adenoma , and transitional serrated adenoma ( tsa ) . a serrated adenoma is a precursor lesion for colorectal carcinoma ( crc ) . the serrated neoplasia pathway has been associated with carcinogenesis of serrated adenoma , which is different from the traditional adenoma - carcinoma sequence [ 5 , 6 ] . serrated polyps are commonly found in the colorectum but have rarely been described in other parts of the gastrointestinal tract . because of the rarity of identifying such a lesion in the small bowel , the natural history , prognosis , and appropriate management recommendations are unclear . serrated adenomas in the small intestine may represent more aggressive lesions with high malignant potential than those in the colon and rectum , according to some reports . we should consider the existence of serrated adenoma of the duodenum and its higher virulence . we report a case of a slow - growing early adenocarcinoma arising from a traditional serrated adenoma of the duodenum , which was diagnosed and treated by an endoscopic mucosal resection . a 66-year - old man with no significant medical history underwent esophagogastroduodenoscopy ( egd ) for general examination ( fig . 1 ) . he had a 1-cm sized , yamada type iv polyp in the second portion of the duodenum . it was an elevated mucosal lesion with focal white patch and was located at the proximal site of the major papilla . the follow - up egd was done after 2 years ( fig . 2 ) . we recommended the patient to resect the polyp ; however , he preferred regular follow - up examination every year . there was no change in the shape , size , and pathologic finding of the polyp for two consecutive years . he underwent another egd for general medical check - up 3 years later ( 5 years after the first detection ) ( fig . the size of the polyp was slightly increased , but the shape of the polyp was not changed . the lesion was raised by means of a submucosal injection of hypertonic saline tinted with indigo carmine and resected by using a snare . the pathologic result revealed a 0.8 0.5-cm sized , well differentiated tubular adenocarcinoma ( fig . 5 ) . carcinomas are multifocally spread on the traditional serrated adenoma , and the proportion of adenocarcinoma component is approximately 50% . abdominal pelvic computed tomography and positron emission tomography showed no other solid organ involvement or metastasis . he is now in good health and we will perform surveillance follow - up egd after 1 year . benign serrated polyps are commonly found in the colorectum but have rarely been described in other parts of the gastrointestinal tract . histology disclosed an adenomatous growth with unlocked saw tooth - like glands with high - grade dysplasia . tsas were found in the esophagus , the stomach , the duodenum , the pancreas , and the gallbladder . increased awareness of the existence of serrated neoplasms in the upper digestive tract rubio 's review indicated that 53.4% ( n = 39 ) of the 73 tsas of the upper digestive tract showed a simultaneously growing invasive carcinoma . following that original publication [ 13 , 14 , 15 ] , 35 additional cases of tsa of the duodenum appeared in the literature ; 28.6% ( n = 10 ) of the 35 cases showed invasive growth . of 73 cases of tsa of the upper digestive tract reported in the literature so far , 53.4% ( n = 39 ) had invasive carcinoma . although the causes for this aggressive behavior remains elusive , it would appear that not only the degree of cellular severity , but also the histological configuration ( i.e. , with unlocked serrations ) might have played a particular role in their virulence . in rosty et al 's study , high - grade dysplasia was present in six of the serrated adenomas ( 46% ) . one case was an adenocarcinoma resembling a serrated adenocarcinoma of the colorectum , with an adjacent serrated adenoma . this high frequency of high - grade dysplasia suggests that these adenomas may represent aggressive lesions with high malignant potential . serrated adenomas in the small intestine may represent a distinct morphological subtype of adenoma with a biological significance that is different from those in the colon and rectum . in the current case , it is consistent with other reports that serrated adenoma in the small bowel is more virulent than those in the colon . tsas of the duodenum should be radically excised , either endoscopically or surgically to rule out the possibility of a synchronously growing invasive adenocarcinoma or to prevent cancer progression . in conclusion , this present case distinctively showed a slow growth but had adenocarcinoma arising from serrated adenoma of the duodenum . we should consider the existence of serrated adenoma of the duodenum and excise it radically owing to its high virulence .
serrated polyps are classified into 3 distinct types : hyperplastic polyp , sessile serrated adenoma , or transitional serrated adenoma . a serrated adenoma is a precursor lesion for colorectal carcinoma . serrated polyps are commonly found in the colorectum but have rarely been described in other parts of the gastrointestinal tract . serrated adenomas in the small intestine may represent aggressive lesions with high malignant potential , according to some reports . a 66-year - old man with no significant medical history underwent esophagogastroduodenoscopy ( egd ) for general examination . he had a 1-cm sized , yamada type iv polyp , with focal white patch in the second portion of the duodenum . the biopsy result revealed gastric metaplasia and chronic inflammation . he wanted regular follow -up examinations . the follow - up egds were done every year . there were no changes in the shape and size of the polyp . the pathologic findings were unchanged . then , he underwent egd for general medical check - up again 5 years after the first detection . the size of the polyp was slightly increased . the biopsy result revealed serrated polyp , unclassified . endoscopic mucosal resection was done . the pathologic result revealed a 0.8 0.5-cm sized , well differentiated tubular adenocarcinoma . carcinomas are multifocally spread on the traditional serrated adenoma , and the proportion of the adenocarcinoma component is approximately 50% . the tumor had invaded the lamina propria but confined to the mucosa . the resection margins were negative , and no lymphovascular invasion or perineural invasion was seen . abdominal pelvic computed tomography and positron emission tomography showed no other solid organ involvement or metastasis . surveillance follow - up egds were done after 3 months and 1 year . there was no evidence of recurrence .
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a 32-year - old healthy caucasian lady presented complaining of recent deterioration of vision in her left eye . at presentation , her best corrected visual acuity ( bcva ) was 20/20 in her right eye and counting fingers in her left eye ( le ) . fundus examination and fluorescein angiography revealed findings consistent with arteriovenous communications of the retina or racemose hemangioma , in the posterior pole of the le with the presence of macular ischemia . complete and systemic examination was unremarkable , excluding the possibility of wyburn - mason syndrome . eight years after presentation , findings and bcva in the le have remained stable , with no extension of the retinal ischemia or development of neovascularization . although extensive retinal ischemia has been reported to result in complications such as retinal or iris neovascularization , in our case the macular ischemia has not expanded further over a period of 8 years . however , due to this macular ischemia the patient unfortunately lost her central vision . racemose hemangioma is rare . the development of extensive retinal ischemia including macular ischemia resulting in rubeosis has been reported . we describe a case of racemose hemangioma which spontaneously developed macular ischemia alone , resulting in poor visual acuity . this finding has remained stable over a follow - up period of 8 years with no further complication . a 32-year - old healthy caucasian lady presented complaining of recent deterioration of vision in her left eye ( le ) . at presentation , her best corrected visual acuity ( bcva ) was 20/20 in her right eye and counting fingers in her le . the anterior segment and intraocular pressures were normal in both eyes . fundus examination and fluorescein angiography revealed findings consistent with arteriovenous communications of the retina or racemose hemangioma , in the posterior pole of the le with the presence of macular ischemia ( figures 1 and 2 ) . complete and systemic examination including mri scan was unremarkable , excluding the possibility of wyburn - mason syndrome with arteriovenous malformations of the optic nerve and midbrain . eight years after initial presentation , findings and bcva in the le have remained stable , with no extension of the retinal ischemia or development of neovascularization . the lesions have been reported either to remain static or regress12 or to enlarge gradually . vision may be affected directly due to macular involvement or by producing hemorrhage or exudation.3,4 based on archer et al the angioma in our case was grade 3 , although there were no systemic findings.5 we present a case of racemose hemangioma which spontaneously developed macular ischemia . although extensive retinal ischemia has been reported to result in complications such as retinal or iris neovascularization,1 in our case the macular ischemia has not expanded further over a period of 8 years . however , due to this macular ischemia the patient unfortunately lost her central vision . it has been postulated that either an enlarged malformation using part of the blood supply of the retina may cause ischemia , or there was a partial thrombosis which caused circulatory stasis within the lesion.6,7
purposeto report a rare case of racemose hemangioma which developed spontaneous macular ischemia.methodsa 32-year - old healthy caucasian lady presented complaining of recent deterioration of vision in her left eye . at presentation , her best corrected visual acuity ( bcva ) was 20/20 in her right eye and counting fingers in her left eye ( le ) . fundus examination and fluorescein angiography were performed . the patient had regular follow - up appointments over a period of 8 years.resultsfundus examination and fluorescein angiography revealed findings consistent with arteriovenous communications of the retina or racemose hemangioma , in the posterior pole of the le with the presence of macular ischemia . complete and systemic examination was unremarkable , excluding the possibility of wyburn - mason syndrome . eight years after presentation , findings and bcva in the le have remained stable , with no extension of the retinal ischemia or development of neovascularization.conclusionalthough extensive retinal ischemia has been reported to result in complications such as retinal or iris neovascularization , in our case the macular ischemia has not expanded further over a period of 8 years . however , due to this macular ischemia the patient unfortunately lost her central vision .
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gierke , in 1905 , described a lesion of adrenal gland containing fat and myeloid elements . a 40-year - old man referred to department of endocrinology with adrenal mass and hypertension . he was diagnosed with hypertension 3 years back , initial bp was 180/110 mm hg , was started on anti - hypertensive treatment . on examination , there were no neurocutaneous markers or marfanoid habitus , no features of cushing 's syndrome , 24-hour urine metanephrines level was 3000 micrograms / day ( normal < 900 micrograms / day , the test was done after stopping all interfering drugs ) . ultrasonography revealed 9.8 8.5 cms well - defined predominantly hyperechoic lesion , faint hypoechogenicity originating from right suprarenal region abutting the upper pole of right kidney and lower surface of right lobe of liver suggestive of right adrenal mass . cect of abdomen showed 9.8 8.5 cm well - defined , well - circumscribed heterogenous hypoattenuated mass lesion noted in right suprarenal region and minimal enhancement on contrast with 80 to 100 hf units of attenuation suggestive of myelolipoma of right adrenal gland [ figure 1 ] . abdominal contrasted computerized tomography showing well - defined non - homogeneous mass of right adrenal origin in view of hypertension , adrenal mass , and elevated 24-hour urine metanephrines ( > 3 times ) , possibility of pheochromocytoma was considered . patient underwent surgery , and well encapsulated right adrenal tumor ( weight : 500 gm ) was excised [ figure 2 ] . immuno - histochemistry of specimen revealed positive for chromogranin a , suggestive of catecholamine - secreting granules in the tissue [ figure 4 ] . gross specimen of removed mass h and e staining revealing features of myelolipoma with mature fat cells , suspended with plenty of normal hematopoitic marrow elements with congested blood vessels . ( original magnification , 100 ) immuno - histochemistry of specimen revealed positive for chromogranin a the patient had remission in hypertension . adrenal myelolipomas are uncommon benign tumors of adrenals , composed of adipose and hematopoietic tissue in varying proportions , a result of metaplasia of reticuloendothelial cells . as these tumors are usually more than 5 cms in diameter , they can be easily detected on ultrasound . the lesion is typically seen as a well - encapsulated heterogeneous supra - renal mass of low density with negative attenuation values , interspersed by dense myeloid tissue and with or without specks of calcification . the diagnosis of adrenal myelolipoma in our case was suggested by the established ct scan criteria [ figure 1 ] and the typical histopathological features [ figure 3 ] . functionality of the adrenal mass in this patient was suggested by the presence of hypertension and elevated metanephrines . catecholamine secreting adrenal myelolipoma was confirmed by the absence of any evidence of pheochromocytoma on hpe and documentation of positive staining for chromogranin a on ihc [ figure 4 ] . brogna et al . , reported a giant cortisol secreting adrenal myelolipoma , but our patient was clinically and biochemically eucortisolic . to the best of our knowledge , only two case reports are available on catecholamine - secreting adrenal myelolipoma in the world literature . tamidari et al . have reported a case of a large , right - sided catecholamine , secreting adrenal myelolipoma with increased 24 hours urinary metanephrines . udupa et al . have reported a large adrenal myelolipoma with increased 24 hours urinary vanillylmandelic acid ( vma ) levels . all these patients became normotensive and biochemical abnormalities normalized following surgery similar to the patient in our case . the association of adrenal myelolipoma and hypertension may not be entirely coincidental , as it may be associated with catecholamine secretion , as seen in our case .
co - occurrence of adrenal incidentaloma with hypertension calls for evaluation of endocrine causes including pheochromocytoma , cushing 's disease , and primary aldosteronism . we are reporting 40-years - old man who presented with hypertension and adrenal mass . he had elevated metanephrines , histology of resected adrenal mass revealed adrenal myelolipoma , and immuno - histochemistry was positive for chromogranin a. both his blood pressure and urinary metanephrines returned to normal after surgery . the association of hypertension and adrenal myelolipoma may not be entirely coincidental , as it may be associated with secreting catecholamine . literature on such an uncommon association is reviewed briefly as well .
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since the first description was more than 20 years ago , arrythmogenic right ventricular cardiomyopathy ( arvc ) is increasingly recognized from pathology to diagnosis . the main pathologic feature is the gradual replacement of the right ventricular myocardium by fibrous tissue and fat . patients with arvc often die at a young age due to the fatal ventricular arrhythmias whereas arvc of an elder person is rare and different from that of young people . in this report , an unusual case of arvc with a long , natural history in a middle - aged woman is presented . a 54-year - old woman was presented with progressive weakness , severe edema , and moderate shortness of breath on exertion for 9 years . subsequently , she was found to have monomorphic ventricular tachycardia ( vt ) with left bundle branch block - type morphology . rest electrocardiogram displayed atrial fibrillation rhythm , epsilon wave , and negative t - waves in leads v1 to v4 [ figure 1 ] . it could be found from the echocardiography that there was severe dilation of the right ventricle , with a poor right ventricular ( rv ) systolic function and severe tricuspid valve regurgitation and an electrophysiological study showed vt of a right ventricular outflow tract or rv apex origin . as a diagnosis of arvc was most likely , the patient underwent a magnetic resonance imaging scan which revealed diffuse areas of fat tissue at the right ventricular wall especially at the free wall [ figure 3 ] . at the same time , multiple small aneurysms were also found . based on the clinical presentation and work -- up , a definite diagnosis of arvc was made . she was treated with various antiarrhythmic agents and angiotensin - converting enzyme inhibitors ( aceis ) , diuretics , and -blockers . despite aggressive medical therapy , her clinical conditions continued to deteriorate , and the tricuspid valve repair was performed because of severe tricuspid valve regurgitation . the general and cardiac biopsy findings demonstrated diagnosis was consistent with arvc [ figure 4 ] . ( b ) 12-lead ecg with inverted t - waves and postexcitation epsilon wave in leads v1 to v4 echocardiography image in the parasternal long axis showing severe dilation of the right ventricle and severe tricuspid valve regurgitation mri showing the dilation of rv and transmural fibrofatty replacement in the rv free wall ( a ) a gross photograph demonstrating the right ventricular with extensive fatty replacement primarily involving the lateral wall . ( b ) typical histologic features of arvc , ongoing myocyte death with early fibrosis and adipocyte infiltration arvc is a heart muscle disease which is characterized by prominent , severe ventricular arrhythmias . however , the criteria have been found to be poorly sensitive and specific , especially in the early stage of the disease . the new criteria continues with these same categories : global or regional dysfunction and structural alternations ; tissue characterization of the wall ; repolarization abnormalities ; depolarization abnormalities ; and arrhythmia and family history . the categorization provides more measurable criteria removing some of the interpretation in the old criteria . arvc is an important cause of sudden cardiac death in people less than 65 years of age . they include chest discomfort , palpitations , presyncope , syncope , and unexplained heart failure . although the right ventricle is mainly involved in arvc , the left ventricle may be progressively affected thus resulting in biventricular failure . the heart muscles of the patient in our case were replaced by fibrous tissue and fat . the electrophysiological study carried out on the patient showed that there were a lot of low - voltage districts in the heart . and this caused the failure of radiofrequency catheter ablation ( rfca ) on vt . so the treatment of arvc is yet focused on icds for the prevention of sudden cardiac death . heart transplantation is unusual in arvc but it has been performed at the end stage of heart failure . because of cardiac myocyte loss ( fat and fibrous tissue replacement ) in the right ventricle , the patient would benefit when she accepted an operation of heart transplantation , not the tricuspid valve repair for her severe heart failure . heart failure may be more obvious in elder patients . although arvc is an inherited disease of the heart muscles involving the right ventricle , the left ventricle may be progressively affected thus resulting in a biventricular failure .
arrhythmogenic right ventricular cardiomyopathy ( arvc ) is a kind of heart muscle disease characterized by the gradual replacement of the right ventricular myocardium with fibrous tissue and fat . it could be the major cause of sudden cardiac death with ventricular tachycardia , and there is a variation in the history of the disease . we reported an unusual case of arvc in a middle - aged woman with congestive heart failure as her first presentation for a long time .
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behet 's disease ( bd ) is characterized by recurrent aphthous stomatitis , genital ulcers , and various skin lesions . bd can also involve other systems such as ocular , gastrointestinal , articular , neurological and cardiovascular systems . the frequency of cardiovascular involvement is estimated to be 4 - 46%.1)2 ) common vascular manifestations are thrombophlebitis and arteritis , which occur in as many as one - third of the patients.3 ) aseptic endocarditis is a rare manifestation of bd and is mostly found in the form of intracardiac thrombus or endomyocardial fibrosis.3)4 ) nonrheumatic tricuspid valve stenosis ( ts ) is extremely rare , and to our knowledge , it has never been reported in the english literature in patients with bd . we describe a case of a female bd patient with aseptic tricuspid valve ( tv ) endocarditis presenting as ts . a 39-year - old female was admitted to kyungpook national university hospital with a 3-month history of dyspnea on exertion and abdominal distension . signs of right heart failure such as pitting edema , palpable liver and neck vein distension were noted on physical examination . she had been diagnosed with bd four years ago . although her previous clinical courses fluctuated , she did not show any signs or symptoms of bd since one year before admission . at the time of admission , evidence of bd disease exacerbation was absent ; esr 20 mm / hr ( reference 0 - 20 mm / hr ) , ferritin 71.50 ng / ml ( reference 13 - 150 ng / ml ) . transthoracic echocardiography ( tte ) showed normal left ventricular function , normal aortic and mitral valve function , morphology , and a moderate to large amount of pericardial effusion . however , precise evaluation of right heart was difficult on tte due to poor echo window . transesophageal echocardiography showed severe ts with an ill - margined echogenic mass , and a mild to moderate amount of pericardial effusion ( figs . 1 and 2 ) . any other possible causes of ts , such as cardiac tumors , carcinoid syndrome , marantic endocarditis , and wegener 's granulomatosis were not detected . symptoms of right heart failure gradually progressed despite the appropriate steroid and immunosuppressive therapy.5 ) she was operated for ts . the thickened tv was removed and was replaced with an artificial valve ( edwards - mira 31 mm ) . pathologic examination showed valvulitis consisting of fibrinoid necrotic material and inflammatory cells ( figs . 3 and 4 ) these pathologic findings were consistent with those of previous reports presenting aseptic endocarditis in bd.6)7 ) after the tv replacement , she remained free from symptoms of right heart failure with immunosuppressive therapy and anticoagulant therapy . endocarditis in bd may be limited to the valve leaflets or may spread to the ventricular or atrial wall and can result in serious complications , such as valvulopathy , organized thrombus or endomyocardial fibrosis.3)4)9 ) however , most cases of endocarditis in bd were detected in the form of organized thrombus or endomyocardial fibrosis.3)4 ) not only an intracardiac thrombus but also a massive endomyocardial fibrosis could cause serious functional obstruction of the tv.3)4)9 ) however , overt ts due to valvulitis has not been reported , even though the affected valve leaflets could either be thickened or replaced by fibrous tissues . also , we could not find any evidence of other combined sequelae of endocarditis , such as intracardiac thrombosis , endomyocardial fibrosis or valvulopathy of other valves . mcdonald et al.10 ) reported for the first time , a case of endocarditis involving the normal mitral and aortic valves in a patient with bd . histologic examination of these valves showed mononuclear infiltration with a few polymorphonuclear leukocytes and no fibrin deposits . madanat et al.6 ) reported a case of bd and endocarditis with left atrial thrombosis mimicking myxoma . postoperative pathologic examination revealed yellowish valvulitis involving the mitral valve leaflet with deep ulcerations on the valve surface , covered by fibrinous and necrotic masses with a significant growth of granulation tissue . in other case reports of endocarditis in bd , several granulomas were found within the central portion of the vegetations and polymorphonuclear cells and lymphocytes infiltrated the small vessels near the vegetations.7 ) in our case , the pathologic examination showed vegetations , consisting of fibrinoid necrotic material , granulation tissue , and inflammatory cells , which were predominantly mononuclear cells . therefore , ts was caused due to valvulitis as a possible sequelae of endocarditis in bd . as in this case , the cardiac lesions in bd might progress insidiously in the absence of concurrent signs or symptoms of bd , or they were even diagnosed at autopsy.10)11 ) therefore , it might be difficult to detect endocarditis in the early stage . these findings suggest that screening for cardiac involvement is required for early detection of endocarditis or other heart diseases in patients with bd .
aseptic endocarditis is an uncommon complication of behet 's disease ( bd ) . we describe a rare case of a 39-year - old female who had bd with aseptic endocarditis of the tricuspid valve ( tv ) presenting as tricuspid stenosis . she was diagnosed with bd four years ago . the mucocutaneous lesions were well - controlled with colchicine and short courses of corticosteroids . she remained free of signs and symptoms of bd for one year without any medication . three months before admission , she gradually developed dyspnea on exertion and peripheral edema . echocardiography revealed dilated right atrium and markedly thickened tv with severe stenosis . tv replacement was performed . pathologic examination of the valve showed fibrinoid necrotic material and inflammatory cell infiltration . blood cultures and cultures of the excised valve were negative for microorganisms .
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rhabdomyolysis is characterized by muscle necrosis and the release of intracellular muscle contents into the systemic circulation . the spectrum of the syndrome ranges from asymptomatic serum muscle enzymes elevation to life - threatening extreme enzyme elevations , electrolyte imbalances , and acute renal failure . we report an elderly lady with a combination of risk factors who developed rhabdomyolytic acute renal failure . a 65-year - old lady was suffering from type 2 diabetes for the past 30 years , hypertension for the past 20 years , and coronary heart disease for the past 10 years . the medications included clopidogrel 75 mg / day , amlodipine 10 mg / day , frusemide 40 mg bds , and insulin . a week before presenting to us , a cardiologist had added atorvastatin 10 mg / day to her prescription . she presented to us with complaints of severe generalized myalgia , difficulty in assuming upright posture from sitting position , and difficulty in walking of 1 week duration . she also complained of swelling of feet , face , nausea , loss of appetite and noticed decreased urine output , and reddish discoloration to urine for the last 3 days . there was no fever , history of trauma , viral exanthem , severe exercise , seizure , uncontrolled blood glucose , and use of herbal medication preceding the illness . on examination , she was well - built and well - nourished , and had pedal edema and facial puffiness . she was afebrile with pulse rate of 60 beats per min and blood pressure of 160/90 mm hg . neurological examination showed 2/5 power in all four limbs , absent deep tendon reflexes , and muscle tenderness with no sensory involvement . urinalysis showed glucose 2 + , ketone bodies negative , blood positive , red blood cells nil , and white blood cells 1 - 2/hpf . her hemoglobin was 11.4 g / dl , total leukocyte count 18 , 300 per mm , platelet count 5.0 lakh per mm , esr 20 mm after 1 h , electrocardiogram showed tall peaked and widened t waves with proximal limb steeper than distal limb , and the chest radiograph was normal . ultrasound abdomen showed right kidney 9.3 3.7 cm and left kidney 9.2 3.2 cm . the urine and blood cultures were sterile , hiv , hbsag , anti - hcv antibodies , anti - hav igm , and anti - hev igm were negative . myalagia , reddish discoloration to urine , deterioration of renal function , elevated sgot , creatinine kinase , and increased urine myoglobin led to the diagnosis of rhabdomyolysis . levothyroxine replacement was initiated at a dose of 50 g / day , increased after 15 days to 100 g / day . the following three risk factors for the onset of rhabdomyolysis were identified : use of statin , undiagnosed hypothyroidism , and co - administration of amlodipine and clopidogrel . frusemide was stopped as she had hypokalemia before the onset of illness which was again a risk factor for rhabdomyolysis . after seven sessions of hemodialysis the urine output improved and serum creatinine stabilized at 3.2 mg / dl . however , it is difficult to directly compare the incidence of statin myopathy in clinical trials with real world clinical practice given the inconsistent definitions . the common risk factors for the development of a statin - induced myopathy include high dosages , increasing age , female sex , renal and hepatic insufficiency , diabetes mellitus and concomitant therapy with fibrates , cyclosporine , macrolide antibiotics , warfarin , and digoxin . individual statins differ in their risk of inducing rhabdomyolysis , with some patients developing this syndrome when switching from one statin to another . it is probable that genetic factors play a role in the pathogenesis of this syndrome . the temporal relation between statin therapy and the onset or resolution of myopathy is not fully defined . a retrospective study of 45 patients with statin myopathy at a tertiary center revealed a mean therapy duration of 6.3 months before symptom onset and a mean duration of 2.3 months for symptom resolution after discontinuation of statin therapy . patients in primo study developed muscle symptoms after a median of 1 month after initiation of statin therapy , ranging up to 12 months after initiation . hypothyroidism was reported as a predictor of statin - associated myopathy ( or 1.71 ; ci , 1.10 - 2.65 ) in primo study . the likely mechanisms of renal impairment in hypothyroidism are the reduction in glomerular filtration rate due to the lower cardiac output and renal blood flow , thyroxine may mediate tubular secretion of creatinine , hypothyroidism may increase creatinine release from muscle , and rhabdomyolysis . it is possible for two different substrates of the same metabolizing enzyme to compete for catalytic sites on the same enzyme ; through competitive inhibition , one substrate may gain access to these sites whereas the other is excluded . this process results in metabolism of the drug that successfully accesses the catalytic sites of the enzyme , whereas the excluded drug is metabolized at a significantly slower rate . in the present patient the present patient provided a caution that hypothyroidism and interaction with other drugs should be considered when patients were going to be initiated on statins .
rhabdomyolysis is a syndrome characterized by muscle necrosis and the release of intracellular muscle contents into the systemic circulation . we report a patient with chronic kidney disease who had deterioration of renal function due to combination of risk factors like hypothyroidism and interaction of amlodipine and clopidogrel with statins .
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we assembled snp array data from 29,589 unrelated people and 222 nuclear families genotyped at 490,000910,000 snps from the candidate gene association resource ( care ) , studies at the children s hospital of philadelphia ( chop ) , the african american breast cancer consortium , the african american prostate cancer consortium and the african american lung cancer consortium . to build a recombination map , we used hapmix to localize candidate crossover positions , and implemented a markov chain monte carlo ( mcmc ) that used the probability distributions for the positions of the filtered crossovers to infer recombination rates for each of 1.3 million inter - snp intervals . we also implemented a second mcmc that models each individual s set of crossovers as a mixture of a shared ( s ) map similar to the european decode map and an african - enriched ( ae ) map , and then assigns each individual an ae phenotype corresponding to the proportion of their newly detected crossovers assigned to the ae map . we imputed genotypes at up to three million hapmap2 snps using mach , and then tested each of these snps for association with the ae phenotype and other recombination - related phenotypes . we identified 2,454 candidate african - enriched hotspots with increased recombination rates in the yri vs. ceu maps , and in the ae vs. s maps , and searched for motifs enriched at these loci , thus identifying a degenerate 17-bp motif . to study the structure of prdm9 , we measured the length of the prdm9 zinc finger array and genotyped rs6889665 in yri , ceu and the care nuclear families ; we also carried out imputation based on 1000 genomes project short read data to infer the alleles individuals carry , among 29 previously characterized in a sequencing study of prdm9 .
recombination , together with mutation , is the ultimate source of genetic variation in populations . we leverage the recent mixture of people of african and european ancestry in the americas to build a genetic map measuring the probability of crossing - over at each position in the genome , based on about 2.1 million crossovers in 30,000 unrelated african americans . at intervals of more than three megabases it is nearly identical to a map built in europeans . at finer scales it differs significantly , and we identify about 2,500 recombination hotspots that are active in people of west african ancestry but nearly inactive in europeans . the probability of a crossover at these hotspots is almost fully controlled by the alleles an individual carries at prdm9 ( p<10245 ) . we identify a 17 base pair dna sequence motif that is enriched in these hotspots , and is an excellent match to the predicted binding target of african - enriched alleles of prdm9 .
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the work of wu and colleagues is in accordance with the recent concept of sepsis - induced immunosuppression . there is now agreement that many severe septic patients survive the first critical hours of the syndrome but eventually die later in a state of immunosuppression that is illustrated by patients ' difficulty to fight the primary bacterial infection , decreased resistance to secondary nosocomial infections and reactivation of viral infections normally solely pathogenic in the immunocompromised host . consequently , immunostimulatory therapies might be used to restore immune functions in the most immunodepressed patients . in the absence of any specific clinical signs of immune failure , however , it is beforehand critical to determine the best biological tools ( markers of septic patients ' immune failure ) enabling patient stratification . the most frequently assessed biomarker in the field to date is undeniably the measurement of hla - dr expression on circulating monocytes ( mhla - dr ) . there appears to be general consensus that diminished mhla - dr is a reliable marker for the development of immunosuppression in critically ill patients . indeed , decreased expression of this marker is regularly reported to be associated with higher mortality / risk for nosocomial infections in critically ill patients . more than 100 articles on this topic have been published in different icu conditions , including sepsis , trauma , burns , and stroke . it is becoming increasingly clear that the critical point after injury is the recovery of normal mhla - dr . schematically , mhla - dr rapidly returns to normal values ( generally in less than 1 week ) in injured patients with uneventful recovery , whereas this parameter remains constantly decreased in patients with adverse outcome/ secondary septic complications . in line with this hypothesis , wu and colleagues showed that low mhla - dr was associated with increased mortality in severe sepsis . most importantly , the authors propose that , more than a single value at a given time point , the dynamic change of mhla - dr over time would be a better predictor of mortality . indeed , in their study , single measurements of mhla - dr within the first week after patient admission ( either days 0 , 3 or 7 ) had no predictive value regarding mortality . in contrast , results expressed as dynamic parameters ( that is , between two time points ) provide excellent predictive values , especially calculated between days 0 and 3 or between days 0 and 7 ( areas under the curve of 0.92 and 0.94 , respectively , in receiver operating characteristic analysis ) . most importantly , after multivariate analysis , the authors show that these two parameters remain the sole independent predictors of mortality with an elevated significant odds ratio . overall , the present results confirm the concept that patients who do not start to restore normal immune functions are those who are going to die . these results are in agreement with two recent studies in which a weak slope of mhla - dr recovery was associated with increased risk of secondary infections in a mixed icu population and in trauma . this outcome could have important consequences in patient management , by potentially allowing for the administration of tailored therapies aimed at restoring immune functions based on dynamic changes of immunological parameters . first , the study is monocentric in a small cohort of surgical patients ( that is , not necessarily representative of the whole septic population ) that present with relatively elevated mhla - dr values ( > 50% ) in comparison with results from the literature ( usually below 50% in severe septic patients ) . this moderate severity and the lack of statistical power due to the small size of the cohort may explain surprising results after multivariate analysis ( sequential organ failure assessment and acute physiology and chronic health evaluation ii scores were not significantly different between survivors and nonsurvivors , odds ratio with very large confidence intervals ) . indeed , the standardized recommended method for expressing mhla - dr results is as numbers of antibodies bound per cell and not as the percentage of positive cells . as an example , the authors suggest that a difference of 4.8% in mhla - dr between days 0 and 3 is of significance . although most probably correct from a statistical perspective , this threshold is hardly applicable in routine / technical practice because such a small percentage difference could be due to measurement variability by flow cytometry . overall and beyond these limitations , appropriately acknowledged by the authors , this study confirms that after injury ( for example , severe sepsis ) survivors tend to progressively normalize mhla - dr , contrary to non - survivors . this biologic parameter could thus provide critical information when assessed as a dynamic variable over time . this potential aspect now deserves to be validated in multicentric clinical studies using standardized flow cytometry protocols .
increasing evidence suggests that the secondary phase of sepsis ( that is , after the first proinflammatory hours ) is characterized by the occurrence of a systemic failure of the immune system . in the most immunodepressed patients , therapies could be used to restore normal immune functions . however , biomarkers need to be developed to beforehand specifically identify these patients . of these biomarkers , diminished monocyte hla - dr expression has rapidly become the most popular . herein , novel perspectives regarding monocyte hla - dr assessed as a dynamic parameter in septic patients will be discussed in the context of a recently published study investigating daily evolution of monocyte hla - dr with regard to 28 day - mortality after severe sepsis .
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since its inception , cell communication and signaling ( ccs ) has been published by biomed central as an open access journal . biomed central is an independent publisher committed to ensuring high quality publications in the fields of biomedical research . articles published in ccs are freely available to everyone online , and are archived in internationally recognized free repositories . although it is still young , ccs has been moderately successful , as indicated by the several thousand accesses to the manuscripts published throughout 2004 . of course , becoming established will require more years , but the quality of the publications that have been accepted by our editorial board is a sign of good health . thanks to an open access policy , articles that are published become freely and instantly available to any person connecting to the world wide web . because articles are intended to remain available at no cost forever , they can be read , downloaded and printed in perpetuity . copies of the published manuscripts are also archived and searchable in pubmed central , the us national library of medicine 's full - text repository of life science literature , and also in repositories at the university of potsdam in germany , at inist in france and in e - depot , the national library of the netherlands ' digital archive of all electronic publications . since the authors hold coyright for their published work , they can make their articles freely available on their institution 's website . the copyright policy also stipulates that the authors grant anyone the permission to reproduce and disseminate the article , provided that no errors are introduced and that it is adequately cited . as a comparison , several journals now offer free access to their articles on line , but it is generally , either for a limited period of time or only after 6 to 12 month following publication . thus , open access offers several benefits to authors and readers in the scientific community and the general public . first the authors are assured that their work is widely disseminated and that it is likely to be cited more often than when it is published in a journal whose access is limited to subscribers . at a time when politicians in many different countries are urging the scientific community to better communicate with the general public , this aspect is of prime importance . another major consequence is that there is no financial barrier to the dissemination of knowledge . the impact of a country 's economy on access to knowledge is considerable and is often under evaluated or ignored by those who live in wealthy environments . as long as a researcher has internet access , he or she can read open access articles ( although enabling them to get internet access is , admittedly , a big issue ) . contributing to the cost of publishing by paying apcs is comparable to paying a toll for driving safely and more rapidly on good quality highways . multiple clean , fast and safe lanes kept in good condition , with same services provided to all customers . any publishing of quality , traditional or electronic , involves processing that is generally paid by the scientific community ( either as authors , readers or subscribers ) . it may seem to some authors that the requested fee of 525 us$ is exceedingly high but , in truth , it is very low compared to the revenues made per article in the traditional publishing model , which some have suggested were as high as us$3000 , if not higher . the apc pays for the article to be freely accessible , and for the processes required before inclusion in pubmed and archiving in pubmed central , e - depot , potsdam and inist . authors can circumvent the charge by getting their institution to become a ' member ' of biomed central , whereby an annual membership fee covers the apcs for all authors at that institution submitting to any journal published by biomed central in that year . current members include nhs england , the world health organization , the us national institutes of health , harvard , princeton and yale universities , and all uk universities . many funding agencies have also realized the importance of open access publishing and have specified that their grants may be used directly to pay apcs . on behalf of the editorial board of ccs , i wish to reassure readers and authors that we are committed to evaluating manuscripts on the basis of their scientific quality , not on whether author 's can pay article - processing charge . if an author is unable to afford the apc , the editor - in - chief will be able to waive payment , if deemed necessary . , we firmly believe that publishing open access can only help scientific communication and improve results , hence becoming an excellent service to society . we do hope that you will support our effort towards this end by submitting manuscripts to cell communication and signaling .
in this article we briefly review the reasons and advantages that underly our publisher 's decision to introduce article - processing charges ( apc ) for manuscripts submitted to cell communication and signaling . the charge is an attempt to develop a new business model for distributing biomedical information and has been accepted in a number of other journals . apcs will enable biomed central to continue to provide their excellent service and will help to establish our journal .
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ghent university multidisciplinary research partnership bioinformatics : from nucleotides to networks ; fund for scientific research ( fwo)flanders ( belgium ) ( postdoctoral research fellowship to f.i . ) ; institute for the promotion of innovation through science and technology in flanders ( iwt - vlaanderen ) ( phd to k.p . ) ; proteomexchange project , funded by the european union 7th framework program under grant agreement number [ 260558 to n.h . ] ; prime - xs project , funded by the european union 7th framework program under grant agreement number [ 262067 to k.g . and l.m . ] .
we here present the online protein processing resource ( toppr ; http://iomics.ugent.be/toppr/ ) , an online database that contains thousands of published proteolytically processed sites in human and mouse proteins . these cleavage events were identified with combinded fractional diagonal chromatography proteomics technologies , and the resulting database is provided with full data provenance . indeed , toppr provides an interactive visual display of the actual fragmentation mass spectrum that led to each identification of a reported processed site , complete with fragment ion annotations and search engine scores . apart from warehousing and disseminating these data in an intuitive manner , toppr also provides an online analysis platform , including methods to analyze protease specificity and substrate - centric analyses . concretely , toppr supports three ways to retrieve data : ( i ) the retrieval of all substrates for one or more cellular stimuli or assays ; ( ii ) a substrate search by uniprotkb / swiss - prot accession number , entry name or description ; and ( iii ) a motif search that retrieves substrates matching a user - defined protease specificity profile . the analysis of the substrates is supported through the presence of a variety of annotations , including predicted secondary structure , known domains and experimentally obtained 3d structure where available . across substrates , substrate orthologs and conserved sequence stretches can also be shown , with icelogo visualization provided for the latter .
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baseline flt imaging in this patient with metastatic malignant melanoma demonstrated splenic ( s ) , peritoneal ( p ) and bm metastases . after 14 days of treatment with a novel antiangiogenesis agent the upper abdominal peritoneal deposit ( vertical arrow ) had substantially decreased activity while the lower abdominal focus could no longer be visualised . uptake at sites of baseline abnormality in the spleen ( horizontal arrow ) and right femoral bm ( oblique arrow ) were relatively photopaenic compared to adjacent normal tissues . fdg pet scanning ( not shown ) demonstrated no change over the same period . most clinical studies of hypoxia imaging have utilized the nitroimadozole , [ f]fluoromisonidazole ( fmiso ) . slow blood pool clearance and high lipophilicity contribute to significant background activity and relatively low contrast between hypoxic and normal tissues . a new agent [ f]fluoro - azomyacinarabinofuranoside ( faza ) has lower lipophilicity as demonstrated by low brain uptake in the left panel . more rapid blood clearance with similar absolute uptake in hypoxic tissue leads to higher contrast as demonstrated in this comparative study of faza ( left ) and fmiso ( right ) scans in a patient with locally advanced retropharyngeal cancer .
despite the excellent clinical performance of fluorodeoxyglucose ( fdg ) as a cancer - imaging agent for positron emission tomography ( pet ) , false positive and false negative results can be problematic in some clinical settings . radiopharmaceutical development has recently focussed on the search for new pet tracers that could complement or replace fdg in such settings . due to the general availability and favourable physical properties of fluorine-18 , much effort has been directed to fluorinated compounds . the most promising of these are discussed .
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tuberculosis , one of the oldest diseases known to affect human being , is caused by bacteria belonging to the mycobacterium tuberculosis complex . transmission usually takes place through airborne spread of droplet nuclei produced by patients with infectious pulmonary tuberculosis . oral mucosa is a rare location for tubercular infection , and it may either be primary or more often secondary infection . different areas of oral cavity like floor of mouth , soft palate , gingiva , lips , hard palate can be involved ; however , hard palate and tongue are the commonest sites of involvement for oral tuberculosis . a 40-year - old male patient presented to our outpatient department because of difficulty in swallowing solid food for two months . this patient also complained of a painless ulceration in his soft palate , malaise , and weight loss since last two months . there is no history of rise of temperature , cough , hemoptysis , hoarseness of voice , regurgitation of food . clinical examination revealed an irregular area of 3 2 centimeters over the soft palate and uvula . hard palate , tonsil , tongue , pharynx were within normal limit [ figure 1 ] . the regional cervical group of lymph nodes was not palpable when the patient presented to us . palatal ulcer before treatment erythrocyte sedimentation rate was raised ( 77 mm / hour ) . nasal endoscopy , fiberoptic laryngoscopy , barium swallow x - ray of esophagus , and x - ray paranasal sinus ( water 's view ) were within normal limit . punch biopsy was taken from the ulcerated lesions over the soft palate under topical anesthesia ; bleeding controlled by application of local pressure . histopathological examination of the tissue revealed presence of epithelioid cells , mononuclear inflammatory cells , langhans type of giant cells forming granulomas with focal caseous necrosis [ figure 2 ] . staining for acid - fast bacillus granulomatous inflammation pattern on histology ( h and e , 400 ) in accordance with the existing guidelines , the patient was administered anti - tubercular medication ( cat 1 four drugs for two months followed by two drugs for four months ) . his difficulty in swallowing reduced rapidly ; his ulceration regressed within one and half months of chemotherapy . although tuberculosis has definite affinity for lungs , it can affect any part of body including oral cavity . oral manifestation of tb tuberculous involvement of the oral cavity is extremely rare , with incidence ranging from . 05 - 5% . an intact and healthy mucosa seems to provide a sufficient barrier to mycobacteria , with saliva also helping to control the organisms . although oral tuberculosis has been well - documented , tuberculous lesions of the upper aerodigestive tract have become rare . oral tuberculosis most commonly results from contact of infected sputum with oral mucosa or hematogenous dissemination in an older individual with pulmonary disease . in contrast , cases of primary infection arising through direct mucosal invasion by mycobacteria are uncommon and typically are seen in young patients , who often present with cervical lymphadenopathy with or without cutaneous sinus formation . the sites demonstrating the most frequent involvement with primary tuberculosis are the gingivae , vestibular mucosa , and extraction sockets . mucosal lacerations and dental extractions have been implicated as predisposing an individual to the development of oral tuberculosis . traditionally , the diagnosis of tuberculosis has been made on the basis of clinical , radiographic findings , and sputum examination . the diagnosis of oro - facial tuberculosis can be quite challenging , mainly because of a lack of definite signs and symptoms . according to pandit et al . , ( 1995 ) , when considering the overall prevalence of tuberculosis of indian population , the presence of epithelioid cell granuloma is indicative of disease unless proven otherwise . 1991 ) reported two cases of primary tb of the oral cavity where smears and culture for afb , from the oral lesion and the sputum , were negative . they confirmed the diagnosis solely on the basis of history and histopathological examination , which only revealed giant cells and epithelioid cells . oral ulceration can be a manifestation of primary syphilis and fungal diseases , and non - infectious processes such as chronic trauma and squamous cell carcinoma . multinucleated giant cells of langhans type are frequently seen in various granulomatous lesions such as tuberculosis , leprosy , syphilis , sarcoidosis , crohn 's disease , eosinophilic granuloma , and certain fungal diseases . lepromatous leprosy lesions are associated with the involvement of superficial nerves leading to anesthesia and paresthesia , which may cause unrealized trauma leading to ulcers and secondary infection . crohn 's disease manifests itself as granulomatous nodules and ulcers in the oral cavity along with associated gastrointestinal symptoms . fungal lesions such as histoplasmosis , blastomyosis , and coccidiodomycosis should also be considered during diagnosis of an oral lesion . microscopically , organisms can be identified with stains such as hematoxylin and eosin ( h and e ) , periodic acid - schiff ( pas ) , or methenamine silver . sporangia may be found free within necrotic tissue or within the epithelioid cells and giant cells of the granuloma . fungal cultures can be an aid in identification of specific fungal species . in our patient , we got epithelioid cell granuloma , also the giant cells and caseous necrosis in histopathologic examination ; his mantoux test elicited a strongly positive reaction with an induration of 14 18 mm after 72 hours , and patient was managed solely with anti - tubercular chemotherapy .
a 40-year - old male patient presented to our clinic with history of dysphagia and ulceration in the palate for two months . after history - taking and thorough clinical examination , investigations like routine blood parameters , chest skiagram , sputum for acid - fast bacilli , ultrasonography of the abdomen , and biopsy from the palatal lesion were performed . no evidence in support of pulmonary or abdominal tuberculosis was found . histopathological examination of the biopsy revealed granulomatous inflammation with langhans giant cells and caseation necrosis . diagnosis of primary tuberculosis of soft palate was made . anti- tubercular regimen ( cat i ) for 6 months was prescribed , and we got a dramatic response noted within 15 days . as isolated tuberculosis of soft palate is a very rare entity , one should , therefore , consider it in any case of chronic ulcer of the soft palate . response to cat 1 was excellent in our case .
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a 17-year - old male was referred to emergency department immediately after a gsi . on arrival , he was conscious , the vital signs were within normal limits , and no neurological deficit was noted . the right globe was ruptured and light perception was negative on the right eye . on physical examination , a single bullet entry hole on the right posterior scapular area was detected [ fig . ( a ) particular appearance of skin wound including small contusion , skin introflection , and simple ecchymosis with frayed margins in the right posterior scapular area . ( b and c ) the preoperative appearances of the globe in emergency and operation room the only detectable exit wound was the right orbit . the route of the bullet was identified by computed tomography ( ct ) scans obtained in several projections and signs of the damage along the path of the bullet entering from the right scapular region and leaving the body from the right orbit were confirmed [ fig . identified route was ; entrance from the right posterior scapular region , passing neighboring to right lung , moving upward to the cranium by side of the carotid artery and the vein , fracturing lateral and posterior wall of the maxillar sinus , entering the orbit fracturing the orbital floor , and leaving the body through the orbit perforating the right eye [ fig . orbital computed tomography , sagittal section ; along the path of the bullet , there were signs of emphysema and hematoma in the soft tissue and at the right parapharyngeal , the masticator and the inferiotemporal muscles due to penetrating injury . furthermore , bone fracture fragments were observed along this path , especially in the lateral and the posterior wall of the maxillar sinus primary reparation under general anesthesia was performed . however , as the double perforation was so severe no postoperative visual function was preserved . unusual presentations of bullet trajectory in gsi can create surgical and/or medico - legal diagnostic problems . since the face and neck region is packed with the vital structures in a relatively small volume of space , even the smallest of movements by a penetrating missile may injure a major vein , artery , and main nerve trunk simultaneously . moreover , especially the injuries to the neck and maxillofacial region could end with high morbidity and mortality . gsi to the orbit , especially the ones penetrating the globe could have devastating effects on all intra- and peri - orbital structures . as the bullet has both forward and rotatory movements , it possesses much higher amounts of kinetic energy to cause more damage in the eye . the energy is dissipated as the bullet slows within the soft tissues or the orbit . high - velocity injuries also cause secondary damage due to the fragmentation of bone , which is shattered by the missile on impact and enhance the injury . nature and severity of the damage and preservation of visual function depends on the direction of impact and the part of globe involved . in case of perforating injury , loss of vision is common as in our case or even loss of eyeball and late enophthalmos in many cases . primary evisceration may be needed in cases with severely ruptured globe if reconstruction is not possible . on the other hand in closed injuries , globe concussion , retinal detachment , optic nerve avulsion , or chorioretinal lacerations some unusual routes of bullet in gsi are reported in the literature . in these awkward injuries , the prediction of the trajectory is very difficult without additional radiological investigations . especially in case of any high velocity projectile wounding , the physician must be aware of the fact that the bullet 's course will not be a linear but most probably a complicated one . the entry wound and the exit wound should be both carefully explored . over - concern with the entry wound ct is the procedure of choice to detect any hemorrhage , air , bullet , bone fragments , hemothorax , nerve lesion , musculoskeletal lesions , and vessels injuries . ct imaging is also useful for assessing the missile path and the anatomical structures at risk . prognosis of the injury depends on the course of the bullet or shrapnel fragment and the multidisciplinary team approach . moreover , even the crime investigation might be enlightened by the demonstration of the bullet 's route . herein , a very unusual route of a bullet entering from the scapular area , passing through the neck and ending with eye perforation is reported . the accurate detection of entrance and exit wounds , path and extent of tissue damage were difficult . although the area pierced by the bullet was rich in neurovascular structures - many of which are extremely important - by chance the patient did not suffer any life - threatening injury . as the dynamics of the shot was investigated , he was thought to be injured with a trajectory from below to above while he was running away from the gunshot . the knowledge of the path of the missile , an open - minded approach , interdisciplinary care , and close clinical observation of the patient are critical for the assessment of management in atypical gunshot wounds .
herein , an awkward case of globe perforation with a bullet - entering from the right posterior scapular region and leaving the body from the right orbit through the eye - is reported . route of the bullet could be devastating - as it passed through the neck and the maxillofacial region - however by chance no vital damage occurred . its path was assessed by plain radiography and computed tomography scans . sometimes prediction of the trajectory is very difficult without additional radiological investigations . especially , in the case of any high velocity projectile wounding , physician must be aware of the fact that the bullet 's course will not be a linear but most probably a complicated one . prognosis of the injury depends on the path of the bullet or shrapnel fragment , close clinical observation , an open - minded approach , and the multidisciplinary care . moreover , even the crime investigation might be needed .
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a 26-year - old man was admitted to the emergency department with a 24-hour history of diffuse abdominal pain that had started in the epigastric area , then localized at the right lower quadrant ( rlq ) , followed by nausea and vomiting . physical examination revealed a mcburney incision scar . tenderness and rebound tenderness were noted in the rlq during palpation . white blood cell ( wbc ) count was 17400 cells / mm with a neutrophil percentage of 78% , whereas c - reactive protein ( crp ) was in normal reference ranges . contrast - enhanced computed tomography ( cect ) scan of abdomen and pelvis showed pericecal free pelvic fluid , cecal inflammation and inflammatory changes in the rlq with a dilated tubular structure extending from the base of the cecum ( figure 1 ) . yellow arrow : periceceal free pelvic fluid , cecal inflammation ; red arrow : right lower quadrant with a dilated tubular structure ( stump appendicitis ) . after adhesiolysis , a remnant suppurative appendiceal stump 5 cm . in size was noted . the postoperative period was uneventful and the patient was discharged on the seventh postoperative day . histopathological examination confirmed stump appendix 5 cm in size with features of local peritonitis ( figure 2 ) . on surface epithelium ulceration , all the layers of appendix wall leukocyte infiltration ( hematoxylin and eosin 100 ) . most patients diagnosed with stump appendicitis present with typical symptoms and findings of acute appendicitis , including pain that starts periumbilically and migrates to the rlq with anorexia , nausea and vomiting . leukocyte count and crp levels tomography scan is more useful than ultrasound to diagnose stump appendicitis , as ultrasonographic findings are not characteristic [ 2 , 4 ] . cect scan can reveal findings that support the diagnosis of stump appendicitis , such as inflammatory changes in pericecal region , thickening of cecal wall , abscess formation , presence of fluid in right paracolic area , and air - filled tubular structure [ 5 , 6 ] . clinical diagnosis of stump appendicitis may be difficult due to underlying conditions like mental retardation , pregnancy , immune suppression and steroid use . medical history of appendectomy can also lead to delay or even missed diagnosis of stump appendicitis . therefore , stump appendicitis should be considered in differential diagnosis of patients with acute abdomen indication , appendectomy or mcburney s incision scar . cecal diverticulitis should also be considered in differential diagnosis of stump appendicitis since cecal diverticulitis is clinically indistinguishable from acute appendicitis . it has been reported that almost 70% of patients with cecal diverticulitis underwent surgery based on preoperative diagnosis of acute appendicitis , and correct preoperative diagnosis was made in only 5.3% of 318 patients . it is also reported that time interval from initial appendectomy to stump appendectomy may vary from 2 months to 50 years [ 1 , 9 ] . in the present case , rate of perforation for stump appendicitis ( detected during surgery ) approaches 68% and length of hospital stay increases due to delayed diagnosis . appropriate stump length to be left after appendectomy is 35 mm to prevent stump appendicitis . stump appendicitis has been reported after both open and laparoscopic appendectomy ; there is very little difference between various surgical techniques in terms of increase in incidence of stump appendicitis . in both open and laparoscopic appendectomy , a longer stump can be obstructed with fecalith , which may lead to chronic inflammation causing ischemia of appendiceal wall and eventually perforate and/or suppurate . while some authors do not support this idea , incidence of stump appendicitis has increased relative to the increase of laparoscopic appendectomy . in an open or laparoscopic approach , careful appendix artery dissection of taenia coli appendiceal cecal junction identification is very important , especially for subserous appendix . in the present case , retrospective examination of patient s medical records of first appendectomy revealed multiple abscesses and adhesions in right iliac fossa ( rif ) . it is important to understand that a history of appendectomy is , by itself , insufficient to exclude diagnosis of appendicitis . presence of mcburney scar may be a warning for surgeons to consider stump appendicitis during an emergency examination . identification of appendiceal base by tracing taenia coli to appendix is very important to prevent stump appendicitis . appendiceal stump of less than 5 mm in length can minimize incidence of stump appendicitis . careful evaluation of clinical and computed tomography ( ct ) scan findings may prevent delay in diagnosis , and decrease morbidity and length of hospital stay .
stump appendicitis is an acute inflammation of remnant appendix , a rare complication of incomplete appendectomy . it may present as acute abdomen with history of appendectomy , which may cause delay in diagnosis . therefore , incomplete appendectomy should be considered as a differential diagnosis of acute abdomen in patients with medical history of appendectomy . the present case is one of stump appendicitis 6 months after appendectomy . stump appendectomy was performed and the patient was discharged 7 days after the operation without any complication .
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tracheobronchopathia osteochondroplastica ( to ) is a rare benign airway disease typically characterized by the presence of multiple rock - garden - like nodules in the lower trachea and upper main bronchi ( 1 ) . because of the absence of cartilage in this region of the airway , these nodules involve the anterior and lateral walls of the trachea and the bronchus , sparing the posterior membranous wall ( 2 ) . several reported cases have demonstrated successful surgical intervention and bronchoscopic laser therapy for advanced symptomatic patients ( 2,3 ) . we herein report the successful bronchoscopic resection of a symptomatic localized polyp due to to using a high - frequency snare . an 80-year - old japanese man was admitted to our hospital for the evaluation and management of multiple tracheobronchial polyposis and right middle lobe atelectasis . he had a history of polyarteritis nodosa and had been treated with corticosteroids ( prednisolone 6 mg / day ) . chest computed tomography ( ct ) revealed diffuse calcified lesions throughout the cartilaginous regions of the trachea and bronchi , right middle atelectasis , and airway polyps ( 4 - 9 mm ) in the left trachea and the left main bronchus ( fig . 1 ) . the bronchoscopic findings showed diffuse edematous mucosal lesions with polyposis on the left side of the trachea , the right middle bronchus and the left main bronchus ( fig . 2 ) . a spirometric analysis demonstrated an obstructive impairment , and the forced expiratory volume in one second ( fev1 ) was 1.36 l , and fev1% was 43% . a transbronchial biopsy to make a diagnosis of the airway polyp was performed , and endoscopic mucosal resection was also carried out using a high - frequency snare to improve ventilatory insufficiency . to was pathologically confirmed in the resected submucosal cartilaginous tissue , and mature ossifications were also observed in the tissue ( fig . after resecting the airway polyp , the spirometric data of the fev1 and fev1% improved from 1.36 l to 1.69 l and from 43% to 93% , respectively . a : a coronal view of the chest mediastinal window shows diffuse calcified lesions throughout the cartilaginous regions of the trachea and bilateral bronchi . noncalcified endobronchial airway polyps are also seen on the left side of the trachea and the upper side of the left main bronchus ( white arrows ) . b : a transverse view of the chest mediastinal window demonstrates right middle lobe atelectasis and an endobronchial airway polyp with small calcified lesions ( white arrow ) in the right middle lobe bronchus . there are no remarkable abnormal findings in the trachea ( a ) and carina ( b ) , however , bronchoscopy showed a diffuse edematous mucosa with polyposis in the trachea ( a ) , carina ( b ) , right middle bronchus ( c ) and left main bronchus ( d ) . a : an endobronchial polyp lesion obtained from the left main bronchus demonstrated submucosal calcification , ossification and cartilage formation surrounded by chronic airway inflammatory cells . b : an enlarged view shows the polyp lesion to consist of submucosal ossification and inflammatory cells . the comprehensive etiology of to remains to be elucidated , however , chronic airway infections , irritant exposure , several metabolic disorders and genetic factors have been proposed to be causative factors of to ( 3,4 ) . this patient showed typical chest ct findings ( fig . 1 ) and unusual bronchoscopic features ( fig . long - term corticosteroid administration might be a potential explanation for the atypical bronchoscopic findings . tajima et al . reported that bone morphogenetic protein-2 ( bmp-2 ) played an important role in nodule formation and might synergistically act with transforming growth factor 1 ( tgf-1 ) to promote an inductive cascade of to nodules ( 5 ) . the airway polyp in our patient did not include mature ossifications in contrast to the previously reported cases ( 2,4 ) , and the long - term corticosteroid administration in this patient might be related to these pathological atypical findings , such as the suppression of calcified lesion formation . however , there has so far been no report describing the effects of corticosteroids on initiating and enlarging airway polyp formation ; thus , further studies are necessary to clarify the mechanism of airway polyp formation and effective treatment . reported that chronic airway inflammation might be an important factor in the formation of to , and they discussed the potential clinical effects of inhaled corticosteroids to improve the symptoms in patients in the early stage of this disease ( 3 ) . no guidelines have yet been established for the management of to , and systemic or inhaled corticosteroid treatment might be one of treatment choices for to without any problematic clinical symptoms , as seen in the present patient . in conclusion , we herein reported a rare case of to accompanied by unusual bronchoscopic features , such as multiple tracheobronchial polyposis , which was successfully treated using a high - frequency snare . to is a benign disorder , however , to may cause various clinical symptoms and spirometric impairments that necessitate the resection of airway polyps . physicians should therefore be aware of this disease and its clinical symptoms and include it in the differential diagnosis .
tracheobronchopathia osteochondroplastica ( to ) is a rare benign airway disease that is characterized by the presence of multiple rock - garden - like nodules on bronchoscopy . to is a slowly progressive disease of the trachea and major bronchi , which is typically characterized by such symptoms as a persistent nonproductive cough , dyspnea and wheezing . the clinical features of to are variable , and asymptomatic patients may incidentally be diagnosed during the work - up for other diseases . we herein report a rare case of to accompanying multiple tracheobronchial polyposis in which bronchoscopic resection of the airway polyp using a high - frequency snare was successfully performed .
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redox biological signaling has significantly developed over the last couple of decades with small molecules such as no , co , h2s , and h2o2 being identified as competent signaling agents . nitroxyl ( hno ) , the oneelectron reduced / protonated form of no , has been discussed as a signaling agent , but the lack of specific & selective detection methods hampers a better understanding of its biology . significant advances in hno detection have occurred with the development of new copper and phosphinebased fluorescent probes and new electrochemical & mass spectrometric methods . with these mechanistically different ways to detect hno , questions regarding hno 's biology and endogenous formation can be approached . the most significant result is that the azaylide intermediate generated from the reaction of these probes and hno rapidly and reliably undergo a staudinger ligation resulting in fluorescence and a stable amide byproduct . reaction of these probes with rsno gives a similar azaylide , but in this case , the staudinger ligation does not occur or proceeds inefficiently . flow cytometry experiments show that these reactions also occur in cells and that these probes only detects hno in cells . one of the challenges starting this project was to not let our previous ideas about the chemistry dictate what experiments to do . we knew that rsno reacts with phosphines to yield azaylides that should undergo ligations and yield fluorescence . should we continue if we were going to see that our probes were not selective ? zhengrui miao noticed that no one had ever really directly compared the response of these probes to hno and rsno . he did this comparison and found fluorescence generation from hno treatment was greatly enhanced compared with rsno , and these findings initiated the study . the overall lesson is to keep an open mind and do n't plan the outcome of your experiments !
abstract invited for this month 's cover picture is the group of prof . s. bruce king at the department of chemistry of wake forest university . the cover picture shows a prefluorescent phosphinebased probe reacting with nitroxyl ( hno ) and s nitrosothiol ( rsno ) , nitrogen oxidederived biological signals . both species react with the prefluorescent probe , but only the product from the hno reaction can complete a further chemical ligation pathway that results in fluorescence , indicating the presence of hno . the product of the probe with rsno does not complete this ligation and does not generate a fluorescent species . these phosphinebased probes thus demonstrate a selectivity for hno over rsno based on their chemical reactivity and can be used in biological systems to differentiate these species . for more details , see the communication on p. 110 ff . read the full text of the article at 10.1002/open.201500200
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premature ovarian aging ( poa ) is defined by elevated age - specific basal follicle stimulating hormone ( fsh ) cut - off levels with menstruation . the age - specific cut - off level under the age of 33 years ( our patient 's age ) is 7.0 miu / ml . there are four main causes for pof , namely , idiopathic , genetic , autoimmune , and viral causes . can we extend causes of pof to poa ? if causes are similar and we evaluate women for poa , can we delay the process of pof ? with these thoughts , we evaluated this patient and we are reporting the case . a study by gleicher et al . titled concluded that presumed underlying etiologies of poa follow a similar distribution pattern as reported for pof . they proved the hypotheses that poa is a precursor stage of pof and hence requires similar evaluation . she had an elevated basal fsh level of 28 miu / ml 3 months back . her height was 1.68 m and she weighed 50 kg with a body mass index of 19 kg / m . a transvaginal ultrasound scan showed a normal - sized uterus but ovaries were not visualized . the repeat basal fsh level ( after 6 weeks ) was 27 miu / ml . since the basal fsh level was above the age - specific cut - off level ( 7 miu / ml for 33 years of age ) , diagnosis of poa was considered and karyotype was requested . jacobs et al . described the first association of triple x syndrome with pof in 1959 . a total of 21 cases of pof with triple x syndrome have been reported in the literature , but to the best of our knowledge , this is the first case report of poa with triple x syndrome . genetic causes comprised approximately 16% of the total in the study conducted by gleicher et al . both autosomes and x chromosomal involvement they are turner mosaicism , partial x chromosome deletion , x chromosome mosaicism , x chromosome inactivation , and fmr 1 ( fragile site mental retardation x gene ) . x chromosome partial deletions are more common , while balanced x chromosome to autosome translocation of xq13q26 is rare , but documented . autosomes involved are at the following gene loci : 3q , 13q , 14q , 17q , 15q , and 11p . genetic defects are proposed to cause poa and pof by increasing atresia of ovarian follicles due to apoptosis or failure of follicle maturation and thus decreasing the pool of primordial follicles . triple x syndrome women also suffer from psychiatric disorders like schizophrenia , eeg abnormalities , scoliosis , and genitourinary malformations . poa with triple x syndrome and primary infertility is treated with ovulation induction by gonadotrophins , because of elevated basal fsh values . prenatal diagnosis for pregnant women with triple x syndrome is definitely required as there will be 25% chance of x chromosomal abnormalities in the offspring . hence , we conclude that it is essential to consider karyotyping for all cases of poa , and age - specific basal fsh values will help us detect cases of poa .
genetic aberrations comprise one - third of women with premature ovarian aging ( poa ) . x chromosome abnormalities are seen in these women . we report a case of a 29-year - old lady with primary infertility and poa . she was phenotypically normal and her basal follicle stimulating hormone level was above the age - specific cut - off . karyotype was triple x syndrome .
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although various prostheses have been used for anterior chest wall reconstruction , selection of the procedure depends on the surgeons experience . the case of a patient who underwent reconstruction of the anterior chest wall using a titanium plate sandwiched between two polypropylene mesh sheets is reported . a 79-year - old woman was referred to our department with a diagnosis of recurrent chondrosarcoma . the first operation for sternal chondrosarcoma included sternal resection and reconstruction with polypropylene mesh and a musculocutaneous flap . however , 18 months after the first operation , computed tomography revealed five tumors located on the anterior chest wall and another tumor located in the subcutaneous tissue on the right chest wall . the tumors were considered metastatic lesions , with no evidence of enlarged mediastinal lymph nodes or distant metastases on radiographic examination . thus , it was judged that complete resection was possible , and the patient underwent subtotal sternectomy , total resection of the body and partial resection of the manubrium sternii , together with partial resection of the 1st5th ribs and costal arch , with a surgical margin of more than 1.0 cm for each tumor . this resection left a defect measuring 17 14 cm on the anterior chest wall . reconstruction of the defect was undertaken with a titanium plate ( titanium mini mesh sheet , 01 - 13155 , 132 82 mm ; thickness 0.5 mm , stryker leibinger & co. , germany ) sandwiched between two polypropylene mesh sheets . the lowermost and the uppermost layer consisted of a polypropylene mesh , and the sheet was fixed to the manubrium and each rib with absorbent suture . the middle layer was a titanium plate , which was fixed to the manubrium and costal arch directly by absorbable # 2 polyfilament braided suture and pulled toward each rib stump ( fig . 1 ) . . no paradoxical movement of the rib cage was noted during respiration in the postoperative period . twelve months after operation , the patient had maintained excellent range of motion without instability or lordosis ( fig . b chest wall defect after subtotal sternectomy and resection of the 1st5th ribs and costal arch . c the middle layer consists of a titanium plate fixed to the manubrium and costal arch , pulled to each rib stump . the lowermost layer is a polypropylene mesh sheet . d the uppermost layer consists of a polypropylene mesh sheet fixed to the manubrium and each ribfig . 2postoperative chest x - ray and computed tomography scans showing the titanium plates secured to the manubrium and ribs surgical images . a local recurrent tumors on the chest wall . b chest wall defect after subtotal sternectomy and resection of the 1st5th ribs and costal arch . c the middle layer consists of a titanium plate fixed to the manubrium and costal arch , pulled to each rib stump . the lowermost layer is a polypropylene mesh sheet . d the uppermost layer consists of a polypropylene mesh sheet fixed to the manubrium and each rib postoperative chest x - ray and computed tomography scans showing the titanium plates secured to the manubrium and ribs sternal tumors are uncommon ; however , they are of different pathological types , such as sarcoma and metastatic tumors of the breast , thyroid , kidney , and colon . king et al . recommended a 4-cm free margin for highly aggressive primary tumors and 2-cm margins for metastatic , benign , or low - grade malignancies to avoid local recurrences . in any case , complete resection of the sternal tumor results in a wide defect on the anterior chest wall . the ideal prosthetic material should be easily available , durable , easily usable , adaptable , rigid , resistant to infection , translucent to radiographs , and of low cost . generally , polypropylene mesh sheets or polytetrafluoroethylene patches ( e - ptfe ) covered with a musculocutaneous flap are used . however , their rigidity is insufficient to protect intrathoracic organs . various prostheses have been used , with sufficient rigidity , such as sandwiched polypropylene mesh and stainless steel mesh , methyl methacrylate sandwiched between polypropylene mesh , titanium plate - supported methyl methacrylate sandwich , titanium plate with gore - tex dual mesh , and composix mesh . however , methyl methacrylate is not easy to handle and is difficult to adapt to the shape of the patient s chest . titanium mini mesh sheet has strong rigidity , no plasticity , translucency to radiography , magnetic resonance imaging ( mri ) compatibility , and biocompatibility . we think that the combination of a metal material and a mesh is an appropriate prosthesis , because of its durability , ease of use , adaptability , rigidity , and translucency to radiography . the advantages of the present procedure are based on the easy use of the titanium plate , irrespective of the shape of the defect and the physiological nature of the material . the titanium plate is used to provide protection for intrathoracic organs , while the polypropylene mesh is flexible in both vertical directions and thus allows movement of the chest wall during breathing . in conclusion , the procedure with the titanium plate sandwiched between two polypropylene meshes achieved good fixation and flexibility . in patients who require extensive anterior chest wall and sternal resection ,
extensive sternal resection carries the risk of difficult reconstruction and surgical complications . a 79-year - old woman underwent sternal resection and reconstruction for sternal chondrosarcoma . however , 18 months after the first operation , she developed six metastatic tumors on the anterior chest wall . she underwent subtotal sternectomy and rib resection , leaving a defect measuring 17 14 cm . reconstruction of the anterior chest wall using a titanium plate sandwiched between two polypropylene mesh sheets is described . this method is potentially applicable to extensive anterior chest resection , and its advantages compared with conventional prostheses are rigidity , flexibility , and usability .
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asphyxiation by an inhaled foreign body is a leading cause of accidental death among children younger than 4 years . in a recent series of 103 children with foreign body aspiration ( fba ) , 64% of the patients were boys and the majority ( 73% ) was younger than 3 years of age . the most common symptoms were sudden choking crisis ( 74% ) and paroxysms of cough ( 73% ) . the most sensitive and specific clinical features were choking ( 86% ) and witnessed aspiration episode ( 89% ) , respectively . available chest radiographs revealed radio - opaque objects in 27% of patients . in another series , fba was suspected by the parents in 59% of patients while witnessing of choking episode was the most important historical event to pinpoint an early diagnosis of fba in children . most aspirated foreign bodies are organic materials ( 81% ) , nuts , peanuts ( 59% ) , seeds , and fruits being the most common . although jellies may also be aspirated in the lungs of small children consuming sweets , there are no reports describing characteristics of gummy or jelly sweets fba in the literature . today , haribo0 is the biggest manufacturer of gummy and jelly sweets in the world , with its products mainly consisting of gummi bears , other jelly sweets and liquorice . we present a case of chewing gummi bear particles aspiration in a 5-year - old child . this is the first time that an extended haribo lung is described after a secret a previously healthy 5-year - old girl presented with a 24-hour history of sore throat , chest pain , and shortness of breath at the pediatric intensive care unit , university hospital , heraklion , greece . on physical examination a posteroanterior chest radiograph revealed right lung collapse and emphysema of the left lung , with tracheal deviation and mediastinal shift [ figure 1 ] . thoracic computed tomography scanning showed extensive multiple obstructions of the distal airways of the right lung which were initially suggestive of disseminated fba [ figure 2 ] . emergency bronchoscopy was performed and multiple small chewing gummi bear ( haribo ) particles impacted in the orifices of the right main bronchus and right lobar and segmentalinic bronchi were successfully removed and aspirated . next day chest radiograph result was normal and the patient was discharged uneventfully without any complication . chest radiograph showing tracheal deviation ( black arrows ) , mediastinal shift ( white arrow ) , left - sided hyperinflation , and low lung volumes and diffuse haziness on the right consistent with atelectasis ct scan displays multiple obstructed lobar and segmentalinic bronchi ( black arrows ) in the atelectatic right lung . sudden onset of cough ( 72% ) , dyspnea ( 64% ) , and wheeze ( 60% ) are the predominant symptoms and signs . the majority of foreign bodies ( 88% ) lodge in the bronchial tree ( right - sided 52% ) , with the remainder catching in the larynx or trachea . only 11% of the foreign bodies are radio - opaque on radiograph , with chest radiographs being normal in 17% of children . obstructive emphysema ( 53% ) and normal chest radiograph ( 34% ) are the most frequent radiological findings . clinical and radiological findings of pneumonia and atelectasis are significantly more common in the groups with negative bronchoscopy or with delayed diagnosis . however , in toddlers with unexplained acute respiratory distress with refractory parenchymal infiltrates , unrecognized fba should be considered . although rigid bronchoscopy is the traditional diagnostic gold standard , the use of computerized tomography , virtual bronchoscopy , and flexible bronchoscopy is increasing . aspiration of gummi bears may cause a silent choking episode leading to life threatening severe respiratory complains , even in children older than 4 years . in a recent healthy lifestyle in europe by nutrition in adolescence ( helena ) cross - sectional survey girls selected more fruit juice , water , herbal infusions , and sweets . gummy and jelly sweets have become a clear favorite , attracting a loyal fanbase which is constantly growing throughout the world . the chewing gummi bear ( haribo , bonn , germany ) , a dancing bear molded from fruit gum [ figure 3 ] , has inspired a million different variant innovations in size , animal , shape , color , and flavor . this is the first time that a lung filled with gummi bears is described after fba in a child ( medline search ) . the gummi / gummy bear is a dancing bear molded from fruit gum foreign body asphyxiation and ingestion needs a focus on education of parents and child caregivers regarding age , appropriate food , risk of play with small items , but also of older children consuming gummy or jelly sweets . legislation for gummy sweets could be extended to children up to the age of 6 years , and to similar products marketed for children . in conclusion , we have reported our experience with a first case of a haribo lodged in the right main bronchus and right lobar and segmentalinic bronchi . aspiration of gummy or jelly sweets may cause a silent choking episode leading to life threatening severe respiratory complains . clinicians should keep a high index of suspicion for silent asphyxia episodes in children consuming gummy or jelly sweets , even in those older than 4 years old . labels on these products should now include warnings for the danger of suffocation in all age 's children .
inhalation of foreign bodies , a leading cause of accidental death , is most common in preschool children . in this article we report our experience with a 5-year - old greek girl who presented with a 24-hour history of sore throat , chest pain , and shortness of breath . emergency bronchoscopy was performed and multiple small chewing gummi bear ( haribo ) particles impacted in the orifices of the right main bronchus and right lobar and segmentalinic bronchi were successfully removed and aspirated . aspiration of gummi bears , which is for the first time reported , may cause a silent choking episode leading to life - threatening bronchi obstruction at multiple sites , even in children older than 4 years .
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a 53-year - old man presented with transient acute onset of left - sided numbness and speech disturbance one week prior to admission . transient ischemic attack or acute cerebral infarction were possible diagnoses , so mri and mr angiography were performed , showing an occlusion of his right mca at the proximal m1 segment without any acute ischemic lesion or infracted area on mri , including diffusion - weighted images . the next day , a conventional angiogram was taken and revealed a tapered occlusion at proximal m1 segment of right mca , collateral pathways through perforating arteries , rich pial collateralization from distal anterior cerebral artery , and a shift of the watershed zone ( fig . these findings were compatible with the long - standing stage of the occlusion of mca rather than the acute occlusion . he also has an incidental saccular aneurysm at left posterior communicating artery ( p - com ) origin . the brain single photon emission computed tomography ( spect ) showed mildly decreased perfusion in right middle cerebral artery territory and the vascular reserve was also decreased mildly after acetazolamide injection ( fig . an echocardiogram and myocardial spect excluded cardiac embolus as the etiology of the occlusion . although the patient had no past medical history , untreated hypertension and diabetic mellitus were found during evaluation . in this setting of presumably chronic occlusion the patient was initially treated with antiplatelet and circulating drug and carefully observed for two weeks . after two months , the left p - com aneurysm was managed by the microsurgical aneurysmal neck clipping and there were no developing or new neurologic symptoms after surgery . immediate postsurgical computed tomography angiogram ( cta ) showed persistent right mca occlusion and complete aneurysm clipping ( fig . the brain cta of 21 months after neck clipping developed recanalization of the previously occluded right mca . a subsequent conventional angiogram confirmed nearly complete patency of the right mca with focal mild stenosis , normal blood flow through mca , and the normalization of the shift of watershed zone ( fig . our patient experienced late spontaneous recanalization and restoration of blood flow by an unknown mechanism within 21 months , without any neurologic deficit . spontaneous recanalization of the occluded mca has been a common finding in acute ischemic stroke . several studies demonstrated that most recanalization may occur in the acute phase of stroke , within approximately 48 hours after onset [ 2 , 5 ] . a recent meta - analysis of thrombolytic therapy attempted to quantify spontaneous recanalization in ischemic stroke . spontaneous recanalization occurred in 21.4% of patients within 24 hours and in 52.7% of patients by a week . although the analysis of these data had limitations , including the variety of times examined , but the suggestion is that the natural history of cerebral embolus is dissolution and spontaneous recanalization over a period of time . like these studies , spontaneous recanalization of mca , in which occlusion is apparent in the acute phase have occasionally occurred in the subacute phase of stroke . but late recanalization in the chronic phase has not been reported to the best of our knowledge . unlike recanalization of occluded artery in acute stroke , the spontaneous recanalization of a long - standing occlusion of extracranial artery has been only anecdotally reported [ 7 - 9 ] . the mechanism by which recanalization of chronically occluded carotid arteries occurs is still little known . in 1999 , suggested the possibility of occlusions resulting from ulcerated plaque thrombosis presenting long - term recanalization by thrombolysis . colon et al . , in 1999 , published a series of four cases of spontaneous recanalization of the internal carotid artery , which through imaging examinations and intraoperative finding , proved to be a hypertrophy of vasa vasorum , causing reperfusion of the distal to the occlusion . another possible mechanism is that , in the case of myointimal hyperplasia or atherosclerotic disease , the neovascularization is induced , which in the long term can allow perfusion distal to vessel occlusion . persistence of some embryonic vessels can also account for the complete non - occlusion of the whole internal carotid artery segment , allowing action of varied mechanisms for vessel recanalization . nowadays some groups have been following patients with carotid occlusions , with the aim of better evaluating the natural history of such lesions . verlato et al . , in 2000 , published a cohort study including 41 patients with carotid occlusion . the mean follow up periods were 44.5 months . in one case with asymptomatic carotid occlusion of 41 patients , spontaneous recanalization was identified at three years after the diagnosis , remaining without symptoms for the whole period . the mechanism of late recanalization of chronic occlusions of mca could be one as remarked above or not . unlike the extracranial carotid artery , the intradural cerebral artery has a little chance to the hypertrophy of vasa vasorum or the revascularization within a plaque due to the nature of intracranial artery , including the lack of vasa vasorum and the smaller caliber . in our case , we cautiously speculate that the recanalization process could be that the repeated embolic formation and accumulation were progressed to complete obstruction for some period of time in focal thrombotic area . after antiplatelet therapy , the thrombolysis occurred gradually instead of repeated embolic accumulation . finally , spontaneous recanalization occurred at this late stage . the angiographic changes in our patient illustrate that the chronic mca occlusion were spontaneously recanalized by an unknown mechanism within 21 months , with a good clinical outcome . the incidence , mechanism , and ideal management of this extraordinary finding remain unclear . through this rare case , further investigation and studies into the underlying mechanism of spontaneous recanalization should be performed .
early spontaneous recanalization of the middle cerebral artery in acute ischemic phase artery is not uncommon , whereas the late spontaneous recanalization of chronic occluded artery is a very rare phenomenon and exact incidence and the timing of this event have not been quantified . we present a case in which late spontaneous recanalization of long - lasting middle cerebral artery occlusion occurred in the absence of surgical , endovascular and thrombolytic treatments .
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sciatic hernias are one of the rarest types of hernia and often pose diagnostic difficulty to clinicians . imaging is often required to confirm the diagnosis and usually involves computed tomography ( ct ) or magnetic resonance imaging ( mri ) . to our knowledge , we report the 115th case of sciatic hernia in the literature who had a falsely negative ct but had the diagnosis confirmed using ultrasonography . an 80-year - old lady was referred to the on - call surgical team by her gp with a 3-week history of a right - sided swelling of the buttock . she had a past medical history of hypertension , osteoarthritis and pemphigus vulgaris and a past surgical history of a perianal abscess requiring incision and drainage . she was allergic to penicillin and took regular oral betamethasone , xylometazoline nasal spray and topical aqueous cream . she did not consume alcohol , was a non - smoker and lived in warden - controlled accommodation . on examination , her cardiac , respiratory and abdominal examinations were normal . digital rectal examination revealed a non - tender stool - filled rectum with no palpable masses . on standing , a swelling on the medial aspect of the right buttock became apparent which was easily reducible , had audible bowel sounds and a positive cough impulse . she was reviewed by the on - call consultant who discharged her with a working diagnosis of possible obturator hernia , with a plan for an outpatient ct of her pelvis and follow - up in clinic . ct scan of the pelvis did not identify a cause for the swelling ( fig . 1 ) . due to the positional nature of the swelling , a gluteal ultrasound was organized , which revealed a large colonic sciatic hernia ( fig . 2 ) . as the patient had minimal symptoms and was not keen for surgical intervention , a plan for conservative management was agreed and the patient was discharged from clinic . figure 1:ct of the patient 's pelvis demonstrating a normal right sciatic foramen ( arrowed ) . us hip rt : confirms reducible herniation of colon in the right sciatic region into the buttock. ) ct of the patient 's pelvis demonstrating a normal right sciatic foramen ( arrowed ) . hip rt : confirms reducible herniation of colon in the right sciatic region into the buttock. ) sciatic hernias are one of the rarest types of hernia and often pose diagnostic difficulty to the clinician . a sciatic hernia is defined as herniation of intraperitoneal contents through either the greater or lesser sciatic foraminae ( fig . the majority of sciatic hernias are found in women ( 77% ) with more than one - third of these being aged 60 or over . the contents are variable and hernias containing ovaries , ureters , bladder , small and large intestine , omentum and dermoid cysts have been reported [ 16 ] . half of patients report non - specific abdominal or pelvic pain and one third have a mass on clinical examination . sciatica , intestinal obstruction , urinary sepsis and hydronephrosis have also been described [ 15 ] . diagnostic laparoscopy or laparotomy is often required to fully evaluate the sac contents and to repair the defect [ 2 , 3 , 5 ] . in symptomatic patients , surgical repair is indicated due to the high risk of bowel strangulation [ 1 , 3 ] . figure 3:pelvis demonstrating the greater ( red ) and lesser ( green ) sciatic foraminae . diagnosis by clinical examination alone is possible in only the minority of cases and imaging is frequently used to confirm the suspicion of sciatic hernia . commonly used imaging modalities are computerized tomography ( ct ) [ 25 ] and mri [ 3 , 4 ] , particularly if the sciatic nerve is thought to be involved . currently , the most commonly used imaging modality of choice is a ct scan with the patient supine . however , if the hernia is only apparent in a dependent position , this can produce a false - negative result , as in our case . the benefit of ultrasound is that it allows for real - time positional assessment of the region of interest , to reduce the risk of missing this rare but significant hernia . due to their rarity , sciatic hernias are often not considered in the differential diagnosis . the authors recommend that all patients presenting with non - specific abdominal or pelvic pain associated with a gluteal swelling should have sciatic hernia considered amongst their differential diagnosis . for patients in which there is a high degree of clinical suspicion for a sciatic hernia and a negative ct , the use of ultrasonography for positional defects may be a useful aid to confirming the suspected diagnosis .
sciatic hernias are one of the rarest types of hernia and often pose diagnostic difficulty to clinicians . we report a case of an 80-year - old lady with a sciatic hernia who had a falsely negative computed tomography ( ct ) but was found to have a colonic hernia on ultrasonography . the authors recommend that for patients in which there is a high degree of clinical suspicion for a sciatic hernia and a negative ct , ultrasonography may be considered as a useful imaging modality to confirm the diagnosis .
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encouragingly , the boolean model was able to make highly accurate predictions on spatial and temporal gene expression patterns . indeed , only several predictions among the 2,772 time - space - gene combinations were at odds with experimental data . this may not seem surprising because the grn is based on interpretations of huge masses of expression data , perturbation data and cis - regulatory data . proceeded to perform more stringent tests of the model by asking how it would respond to four perturbations : extinction of the expression of the delta gene , global expression of the pmar1 gene , extinction of hox11/13b expression , and most challengingly , the transplantation of four cleavage skeletogenic micromeres into the animal pole of an otherwise normal embryo that possessed its own set of four micromeres at the vegetal pole . ( the intra - embryo transposition of blastomeres harkens back to the classical era of experimental embryology . ) the authors emphasized that except for the hox11/13b test , the perturbation results they sought to reproduce were not used in building the grn . ( transplanted micromeres could of course not have been part of the grn , which is for a normal embryo . ) the results of these perturbation tests were in nearly perfect agreement with the experimental data , which led the authors to two conclusions : the grn contained sufficient information to provide a system - level causal explanation for sea urchin development , and the boolean computational model was a useful tool for in silico testing of grn and making predictions upon perturbations . the test of blastomere transplantation demonstrated the critical role of intercellular signaling between the different spatial domains in development . the cells in these domains obviously need to work cooperatively to ensure precise and robust developmental progression and thus information on the regulatory state of each cell must be able to diffuse spatially . we enjoy so many examples of such events in development , e.g. , the wnt pathway in drosophila development to mention just one of many examples , but we have no case in which a paracrine signaling pathway amidst a developmentally determinative cluster of cells can be put into the context of a grn as detailed as the one peter et al . have defined . from the information theory perspective , intercellular thus , the developmental process is also a diffusion process of the genomic regulatory information . related domains of this emerging field include molecular information theory and information networks in the data mining field from both of which the modeling of intercellular signaling may benefit . went so far as to suggest that the gene regulatory models could sufficiently explain all the gene expression patterns in sea urchin development , without considering non - coding rnas . for example , a recent study identified long noncoding rnas ( lncrna ) in zebrafish embryogenesis and revealed that lncrnas were specifically enriched in early - stage embryos . ironically , davidson and his longtime partner roy britten were the first to postulate such a role . is there any information loss from the continuous data to boolean data , from gene regulatory logic to boolean logic ? what would be a good cutoff for converting the expression level to on or off ? if a grn is not available in a given case , can a boolean model be directly inferred and tested using raw experimental data ? if so , what kinds of experimental data are most suitable and in what way can the causal structure be best captured in the boolean model ? theoretical frameworks of causal inference from observational data have been studied in machine learning for decades . possibly the established causal inference algorithms can expand this newest chapter in grn - ology from the pioneering davidson lab into more general settings . can this work provide insights into other animal development , e.g. , mouse , human or into human embryonic stem ( es ) cell differentiation ? is it possible to apply the general computational framework to test grn models derived from biological processes in addition to development , such as immunology and postnatal neurogenesis in the subventricular zone of the brain to mention only two of many frontiers before us that beckon for the grn approach ? with the advances of next generation sequencing technology and its cost now rocketing downward , a large amount of data will soon be in hand for various biological processes across diverse phyla . much of this momentum now comes from the sea urchin embryo and from the scientific mind of eric davidson .
eric davidson at caltech has spent several decades investigating the molecular basis of animal development using the sea urchin embryo as an experimental system1,2 although his scholarship extends to all of embryology as embodied in several editions of his landmark book.3 in recent years his laboratory has become a leading force in constructing gene regulatory networks ( grns ) operating in sea urchin development.4 this axis of his work has its roots in this laboratory s cdna cloning of an actin mrna from the sea urchin embryo ( for the timeline , see ref . 1)one of the first eukaryotic mrnas to be cloned as it turned out . from that point of departure , the davidson lab has drilled down into other genes and gene families and the factors that regulate their coordinated regulation , leading them into the grn era ( a field they helped to define ) and the development of the computational tools needed to consolidate and advance the grn field .
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the definition of a gerbode defect , according to the society of thoracic surgeons congenital heart nomenclature and database project is true left ventricular ( lv ) to right atrial ( ra ) communication . however , no sources provide a definition encompassing congenital right ventricular ( rv ) to left atrial ( la ) communication . zacharkiw and stimpson recently described this pathology as a mirror - image gerbode defect in a patient following atrioventricular ( av ) septal defect repair . in the present study , the case of a congenital la - rv shunt in an adult is presented and the classification of such defects is discussed . a 74-year - old woman was referred to our center with a worsening history of orthopnea , paroxysmal nocturnal dyspnea , and peripheral edema . transthoracic echocardiography revealed lv hypertrophy , a dilated left atrium , severe mitral valve insufficiency , and pulmonary hypertension ( 60 mmhg ) . a careful review of the preoperative transesophageal echocardiography ( tee ) revealed a clear jet across a small defect between the rv and la ( figs . 1 , 2 ) . the pericardium was entered through a midsternal incision and a patch was fixed using glutaraldehyde . following the establishment of cardiopulmonary bypass ( cpb ) , a vertical cleft separating the anterior leaflet into two hemileaflets was observed on the mitral valve ( fig . aspirator - guided inspection showed that the defect was located between the la and the rv ( fig . the cleft was closed without tension after resection of the abnormal chordae attached to its edges . direct suture was not technically possible due to the fibrous reaction of the edges of the cleft with an area lacking valvular tissue . instead , the edges of the cleft were resected and the anterior leaflet of the mitral valve was reconstructed using an autologous pericardial patch . however , the saline test revealed regurgitation at both commisures . the patch was then replaced with a 27-mm porcine bioprosthetic valve ( biocor ; st . paul , mn , usa ) which was implanted in a supra - annular fashion with the use of interrupted , pledgeted , 20 everting mattress sutures . rewarming was initiated , the atriotomy was closed , the heart was de - aired , and the cross - clamp was removed . the av junctions are the area of the heart where the atrial myocardium is inserted into the base of the ventricular mass . partial av septal defects are malformations with two av valve orifices and no interventricular communication , whereas complete av septal defects have a common av valve orifice and extensive interventricular communication . lv to ra atrial communications , known as gerbode defects and lv - ra shunts , are encountered from time to time and are caused by surgical mishaps , trauma , and endocarditis . however , we could find only three cases of mirror - image gerbode defects ( la - rv shunts ) that have been reported to date in the literature [ 2,46 ] . of those cases , only one was congenital , while in the other cases , the defect emerged after the repair of an av septum . the previously reported congenital case was a 39-year - old woman with a common av junction and partially separated right and left av orifices , and the shunt was exclusively from the la to the rv due to overriding of the left av valve . however , in our case , each atrium was connected to its own ventricle through separate leaflets . additionally , a cleft in the anterior mitral leaflet existed , which may have been linked developmentally , on the basis of different degrees of failure of fusion of the av endocardial cushions . a literature review demonstrated that it is virtually impossible to categorize the spectrum of av septal defects into satisfactory and noncontroversial subgroups with regard to patients such as ours . we believe that diagnosis and surgical treatment strategy will become easier if case - specific morphologic and functional variables are analyzed .
gebode defect , that can accurately be treated surgical repair , is defined as a true communication between left ventricle and right atrium . a 74-year - old woman with a worsening history of ortophnea and peripheral edema was hospitalised . a communication between right atrium and left ventricle was diagnosed using transeusophageal echocardiography . the defect was repaired and mitral valve was replaced with a biologic valve . it would be beter to tailor surgical strategy for each case with atrioventricular canal defect after preoperative transeusophageal echocardiography and peroperative direct sight .
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the juice was extracted from the fruits and subjected to centrifuge . in the supernatant equal amount of ethanol was added and kept it in refrigerator for 30 min , then centrifuge the mixture and dry at room temp . fine powder was collected as bio - polymer . for the granulation 1% , 3% and 5% solution of polymer was made in distilled water and appropriate amount of lactose ( 10 gm ) was added to it . then granules were subjected to resize with sieve#16 . before compression of granules , 2%talc and 2% fine powder were mixed well . for the preparation of sustained release tablets containing rifampicin same procedure was followed with 3% and 5% of polymer for preparation of sustained release tablets . additional tablets of 100 mg , 200 mg and 400 mg were prepared using 5% concentration of the polymer . the biopolymer from citrus limetta was isolated and evaluated for release retardant property in sustained release tablet . among the three formulations , fm-3 shows hardness of 5moh , friability of ( .90 ) , weight variation of ( 5.0 ) . it had t50% and t 80% of 1.34 hrs and 2.52 hrs at ph ( 1.2 ) respectively . in - vitro release study for tablets prepared by using 5% conc . of polymer graph for cumulative % drug release vs time of rifampicin at ph ( 1.2 ) observation table for release study the results indicated that the selected biopolymer had a good release retardant property thus it can be concluded that the selected biopolymer can be utilized as low cost natural biocompatible and biodegradable agent .
the advantages of biopolymers over synthetic polymers are low cost , natural origin , free from side effects , biocompatible , bio - acceptable , environmental friendly processing , local availability , better patient tolerance as well as public acceptance . sustained release tablets containing rifampicin was prepared by adding 100 mg polymer and 50 mg drug and granules . same procedure was followed with 3% and 5% of polymer for preparation of sustained release tablets . additional tablets of 100 mg , 200 mg and 400 mg were prepared using 5% of the polymer . the results indicated that the selected biopolymer had a good release retardant property thus it can be concluded that the selected biopolymer can be utilized as low cost natural biocompatible and biodegradable agent .
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its effect is mediated via interactions with several receptors in the central nervous system and results from a combination of antidopaminergic , anticholinergic , antihistaminic , and weak antiadrenergic actions . the therapeutic effects of chlorpromazine are frequently accompanied by unwanted side effects that include sedation , autonomic , endocrine , and neurological effects . to date tinnitus is a common adverse reaction ( adr ) to several drugs and may occur during long - term therapies or after a single drug administration . even though not life threatening , tinnitus may be discomforting ; it may also be irreversible despite drug withdrawal . to date , over 130 drugs have been described to be potentially ototoxic , among which the most common inducers of tinnitus are aminoglycosides and other antimicrobials . we report on a suspect adr to chlorpromazine that occurred in a 12-year - old boy , affected by severe generalized anxiety disorder . he received initially a benzodiazepine therapy , which was switched to chlorpromazine ( 6.25 mg / day orally ) because of the absence of a significant clinical response . ten days after treatment with chlorpromazine , the patient experienced an enhanced sensitivity to sounds accompanied by perception of noises of the buzzing or ringing type . information about the patient 's medical history did not report conditions that may have predisposed to the onset of the disturbance manifested . moreover , the patient was in overall good health and had never suffered from hearing disorders . in view of the medical history , the inability to discontinue therapy with chlorpromazine resulted in an objective worsening of the patient 's symptoms , which are still present to date . the naranjo adr probability scale identified the relationship between the patient 's development of adr and the drug as possible . this is the first report on a case of tinnitus related to the administration of chlorpromazine . chlorpromazine is an antagonist of several dopamine cochlear receptors that play an important role in the sensory process by modulating afferent auditory nerve activity . dopamine , released from the terminals of lateral olivocochlear efferent fibers , is protective against acoustic trauma , hypoxia , and ototoxicity . in this context , the dopamine antagonist activity of chlorpromazine may result in a higher risk of ototoxicity . it controls the cochlear blood flow , acting on the precapillary sphincters and increasing the microcirculatory flow . clinical evidence indicates that h1 histamine agonists are effective in reducing tinnitus via improving vestibular compensation of the microcirculation . chlorpromazine antagonism on h1-receptors may thus play a role in counteracting the vessel modulatory effect of histamine and by this means have contributed to tinnitus development in our patient . acetylcholine is the major neurotransmitter in the olivocochlear efferent pathway , which is a feedback control system to the inner ear comprising a medial olivocochlear pathway projecting to outer hair cells and a lateral olivocochlear pathway projecting to dendrites of cochlear nerve fibres . in this context , the anticholinergic effects of chlorpromazine may have inhibited efferent signalling via the 9/10 nicotinic acetylcholine receptor complex in the outer hair cells which is known to be protective against acoustic injury . another action of chlorpromazine that may have contributed to generate tinnitus in our patient is its antagonism of serotonergic receptors . serotonin is one of the neurotransmitters acting on the auditory pathways ; in particular it is involved in sound detection , location , and interpretation . serotonin is currently believed to be one of the most important neurotransmitter involved in the perception of tinnitus . indeed , serotonin reuptake inhibitor drugs reduce the intensity of tinnitus acting directly on nerve conduction of the auditory stimulus , particularly in the central auditory pathways . in this scenario , it is thus conceivable that antagonism at serotonin receptor levels caused by chlorpromazine causes auditory disorders leading to tinnitus . finally , a role for an action of chlorpromazine on gamma amino butyric acid ( gaba ) can not be excluded . gaba inhibits auditory system and systemic administration of a gaba transaminase inhibitor improves tinnitus by suppressing hyperactivity in the auditory system . the neurotransmitter serotonin , involved in a large variety of physiological functions , behaves as a neuromodulator by strengthening the gaba system . chlorpromazine , by decreasing the availability of serotonin , may lead to decreased gabaergic activity and this action may have contributed to tinnitus development . we can not establish which among the actions of chlorpromazine described above has been predominant in the tinnitus - inducing action we observed , and the most likely possibility is that tinnitus resulted from a synergism among these different actions . a predisposition of the patient to develop tinnitus following chlorpromazine can not also be ruled out . receptors for serotonin , histamine , dopamine , and gaba are polymorphic and the presence of specific single nucleotide polymorphisms that acted as predisposing factors may be present in this specific patient . in addition chlorpromazine is substrate of cytochrome p4502d6 ( cyp2d6 ) , a highly polymorphic isoform of cytochrome . genetically determined functional variations of this cytochrome may be present in the patient and may also have contributed to the onset of tinnitus . to our knowledge , this is the first report on the development of tinnitus following chlorpromazine administration . although there is no information on dechallenge and rechallenge , the inability to discontinue therapy with chlorpromazine resulted in an objective worsening of the patient 's symptoms . this clinical case is of great clinical interest as chlorpromazine is not currently included among potentially ototoxic drugs ; paradoxically phenothiazines can be prescribed to alleviate symptoms related to disorders of the vestibular system .
chlorpromazine is a well - known antipsychotic agent that binds with a variety of receptors in the central nervous system . to date , chlorpromazine has never been associated with onset of hearing disorders and tinnitus . we report on an unexpected suspect adverse reaction to chlorpromazine that occurred in a 12-year - old boy , affected by severe generalized anxiety disorder . after treatment with chlorpromazine , the patient experienced an enhanced sensitivity to sounds accompanied by perception of noises of the buzzing or ringing type . this clinical case is of great clinical interest as chlorpromazine is not currently included among potentially ototoxic drugs .
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unless otherwise stated , all chemicals and solvents were purchased from commercial suppliers in reagent- or technical - grade quality and used directly as received without further purification . the bis(arylimino)acenaphthene ligands were prepared from acenaphthenequinone and the corresponding substituted anilines in anology to literature methods.[14 , 27 ] the rhenium complex 1 was synthesized as previously reported . general procedure for the preparation of 2 and 3 : equimolar amounts of re(co)5cl ( 0.05 g , 0.14 mmol ) and the corresponding bis(arylimino)acenaphtene ligand were refluxed in dry toluene ( 4 ml ) for 30 min . complete precipitation of the product was obtained upon cooling the reaction mixture to room temperature and slow addition of n - hexane . as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be re - organized for online delivery , but are not copy - edited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors
rhenium(i ) carbonyl complexes carrying substituted bis(arylimino)acenaphthene ligands ( bian - r ) have been tested as potential catalysts for the two - electron reduction of carbon dioxide . cyclic voltammetric studies as well as controlled potential electrolysis experiments were performed using co2-saturated solutions of the complexes in acetonitrile and acetonitrile water mixtures . faradaic efficiencies of more than 30 % have been determined for the electrocatalytic production of co. the effects of ligand substitution patterns and water content of the reaction medium on the catalytic performance of the new catalysts are discussed .
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measurement of cardiac output ( co ) requires use of invasive or minimally invasive devices ; the use of noninvasive and minimally invasive devices has gained popularity in recent years . the bioreactance technique is a relatively new , continuous , totally non - invasive technique for measuring co that is easily implemented . this new technique involves analyzing phase shifts of a delivered oscillating current that occur when the current traverses the thoracic cavity , and differs from traditional bioimpedance techniques that rely on analysis of changes in signal amplitude . most validation studies in critically ill patients have shown good correlation and/or agreement of bioreactance values compared with co values obtained using other devices in patients admitted after cardiac surgery [ 2 - 4 ] . however , validation in critically ill patients is lacking . as part of the internal evaluation of a bioreactance device before its implementation in the unit ( evaluation of new non - invasive monitoring systems before introduction in the unit does not require the approval of the ethics committee in our institution ) , we compared co values obtained using the bioreactance technique ( nicom system ; cheetah medical inc . , portland , or , usa ) with those measured using semi - continuous cardiac output by thermodilution ( cco ) with a pulmonary artery catheter ( vigilance , edwards lifesciences , irvine , ca , usa ) . in 11 patients the co values were compared at study inclusion and each time a relevant change in hemodynamics and/or in therapeutics ( for example , fluid challenge , inotrope or vasopressor infusions ) was observed ( table 1 ) . patient characteristics data in parentheses represent maximal dose , range ( g / minute for norepinephrine and g / kg.min for dobutamine ) . we recorded bioreactance co ( average of five values over a 5-minute period ) just after obtaining the pulmonary artery catheter cco ( average of five cco values over a 5-minute period ) . we collected 141 pairs of measurements ( 3 to 23 per patient ) ; the duration of monitoring was at least 3 hours but never exceeded 24 hours . there was poor correlation between the two techniques ( correlation coefficient r = 0.145 ) ( figure 1 ) . to limit the time effect , we randomly selected one pair of measurements for each patient - but this did not improve the results ( r = 0.13 ) . bland and altman analysis with correction for multiple measurements showed wide limits of agreement ( figure 2 ) . the time course of co was not well tracked either , sometimes with opposite trends between the two devices . correlation between pulmonary artery catheter semi - continuous cardiac output by thermodilution and bioreactance cardiac output . pulmonary artery catheter semi - continuous cardiac output by thermodilution and bioreactance cardiac output : bias and agreement . co , cardiac output ; pac - cco , pulmonary artery catheter semi - continuous cardiac output by thermodilution . the bioreactance technique is dependent on diffusion of electrical current , so interstitial edema may interfere with measurements ; we believe this is the most probable explanation for the poor correlation . whatever the reason , these data suggest that caution should be applied when using bioreactance devices in critically ill patients .
measurement of cardiac output ( co ) using minimally invasive devices has gained popularity . in 11 patients we compared co values obtained using the bioreactance technique - a new continuous , totally non - invasive co monitor - with those obtained by semi - continuous thermodilution using a pulmonary artery catheter . we obtained co measurements at study inclusion and after any relevant change in hemodynamic status ( spontaneous or during fluid challenge , inotrope or vasopressor infusions ) . there was a poor correlation between the two techniques ( r = 0.145 ) . these data suggest that caution should be applied when using bioreactance devices in critically ill patients .
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the study was approved by the joint ethics committee of the institute of neurology and the national hospital for neurology and neurosurgery , london , uk . all structural and functional scans at time 1 and time 2 were acquired from the same siemens 1.5 t sonata mri scanner ( siemens medical systems , erlangen , germany ) . the structural images were acquired using a t1-weighted modified driven equilibrium fourier transform sequence with 176 sagittal partitions and an image matrix of 256 224 , yielding a final resolution of 1 mm [ repetition time / echo time / inversion time = 12.24 ms / 3.56 ms / 530 ms ] . pre - processing of 66 structural images ( 33 participants 2 time points ) used spm8 ( http://www.fil.ion.ucl.ac.uk/spm ) with the dartel toolbox to segment and spatially normalize the brains into the same template ; with and without modulation . modulated images incorporate a measure of local brain volume while unmodulated images , used with proportional scaling to correct for global grey matter , provide a measure of regional grey matter density . previous studies have shown the correlations between brain structure and cognitive ability are better detected by grey matter density . images were smoothed using an 8 mm isotropic gaussian kernel at full width half maximum ( fwhm ) . the relationship between change in iq and change in brain structure was investigated by entering the appropriate pre - processed images ( modulated or unmodulated grey or white matter ) into within subjects paired t - tests , with change in iq ( viq , piq or fsiq ) and year of scan as covariates . the degree to which time 2 iq was predicted by changes in brain structure was investigated in a hierarchical regression analysis with time 1 iq entered before change in brain structure . details of the functional imaging paradigm have been reported elsewhere and are summarised in supplementary information ) .
intelligence quotient ( iq ) is a standardized measure of intellectual ability that taps a wide range of cognitive skills1 . across life span , iq is generally considered to be stable with scores at one time point used to predict educational achievement and employment prospects in later years1 . neuro - imaging allows us to test whether unexpected longitudinal fluctuations in measured iq are related to brain development . here we show that verbal and nonverbal iq can rise or fall in the teenage years , with these changes in performance validated by their close correlation with changes in local brain structure . a combination of structural and functional imaging showed that verbal iq changed with grey matter in an area that was activated by speech , while nonverbal iq changed with grey matter in an area that was activated by finger movements . by using longitudinal assessments of the same individuals , we eschewed the many sources of variation in brain structure that confound cross sectional studies . this allowed us to dissociate neural markers for verbal and nonverbal iq and to show that these general abilities are closely linked to the sensorimotor skills involved in learning . more generally , our results emphasize the possibility that an individual s intellectual capacity relative to their peers can weaken or strengthen in the teenage years . this would be encouraging to those whose intellectual potential may improve ; and a warning that early achievers may not maintain their potential .
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takayasu s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches . as a result of intimal fibroproliferation , segmental stenosis , occlusion , dilatation , and aneurysmal formation of the involved vessels it is an uncommon disease , with an approximate incidence of 23 cases per year per million individuals and usually affects young female of asian ascendance during their second and third decades of life . we describe a case of a previously healthy caucasian female whose takayasu s arteritis presented as an association of aortic and main left coronary aneurysms with severe aortic insufficiency . a 26-year - old caucasian female was admitted to our hospital with a 3-week history of fatigue , malaise , exertion dyspnea , ortophnea and paroxysmal nocturnal dyspnea . there was a marked difference on the blood pressure measurement between the arms ( 140/90 mmhg on the right arm and 90/60 mmhg on the left ) . the brachial pulse could not be felt on the left arm and a systolic murmur was heard on the left infraclavicular area . on cardiac auscultation , a diastolic murmur ( + + + + /vi ) was heard on the aortic area , and there were some crackles in the basal regions of both lungs . her erythrocyte sedimentation rate ( esr ) was 64 mm on the first hour and the result of the serum c - reactive protein ( crp ) was 24 mg / dl ( normal : 06 mg / dl ) . based on the 1990 american college of rheumatology criteria , a diagnosis of takayasu s arteritis was made . a high - resolution thorax computed tomography ( ct ) showed a 4-cm aortic aneurysm spanning the ascending and the proximal descending portions , as well as the aortic arch . cineangiocoronariography confirmed the findings of the ct and also revealed a severe aortic insufficiency and a large main left coronary aneurysm ( figure 1 ) . a three - day course of intravenous high - dose methylprednisolone ( 1000 mg ) was administered , as well as medications for the management of the heart failure ( diuretics , digoxin , angiotensin - converting enzyme inhibitor ) , which resulted in a remarkable improvement in general symptoms . methotrexate was started at 15 mg / week as a steroid - sparing medication . takayasu s arteritis is primarily a chronic inflammatory vasculitis characterized by stenosis of large and medium sized arteries . the coronary arteries are involved in about 10% of cases , but aneurysm formation , especially affecting the main left coronary , is a very rare finding . destruction of the elastic fibers in the media of the vessel is the leading pathogenic mechanism of aneurysms formation . in some situations of massive aortic regurgitation coronary aneurysms predispose to thrombus formation and acute myocardial infarction , even in patients receiving aspirin and/or warfarin . on the other hand , revascularization under inflammatory circumstances carries a higher risk of complications , such as stenosis , suture line dehiscence and pseudoaneurysm formation . a case of successful surgical resection of a giant right coronary artery has been reported , even though the best surgical timing in takayasu s arteritis still remains controversial , as long as even when asymptomatic and with no serological evidence of current inflammation ( normal esr ) , up to 44% of these patients show some degree of histologic active disease . to our knowledge , this is the first report of an association of aortic and coronary aneurysms with severe aortic insufficiency in a takayas s arteritis patient . the complexity of this case , allied to the absence of previously described medical interventions specific to this situation , certainly turns it into a therapeutic challenge . proper follow - up is warranted , to get nearer to an accurate decision about the best moment to choose for a surgical approach , considering its costs and benefits when dealing with vasculitic vessels and its inner complications .
takayasu s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches . as a result of intimal fibroproliferation , segmental stenosis , occlusion , dilatation , and aneurysmal formation of the involved vessels may develop . it is an uncommon disease and usually affects young asian female patients during the second and third decades of life . coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding . we describe a case of a previously healthy 26-year - old caucasian female whose takayasu s arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency .
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