“Atypical presentations” of celiac disease (CD) are the most common presentations

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Methods: A 38 year old Caucasian male with HIV infection on anti- reteroviral therapy for last 5 years was admitted with intermittent maroon colored stools for 3 days and hemoglobin decrease from 15.5 g/dl to 6.5 g/dl. He was treated for Kaposi’s sarcoma involving skin and stomach 5 years ago with complete remission. He had two similar episodes of GI bleed 2 years ago but the GI work-up including upper and lower GI endoscopy, enteroscopy, small bowel barium x-ray, abdominal CT scan and Meckel’s scan failed to localize the bleeding site. His recent CD4 count was 340 and viral load 1000. He never had any other opportunistic infections. On admission, he was orthostatic and pale otherwise rest of the exam- ination was unremarkable. A repeat colonoscopy revealed maroon blood throughout the colon. Small bowel enteroscopy upto mid small bowel did not reveal any bleeding site. An RBC tagged scan showed evidence of active bleeding from small bowel but abdominal angiogram did not detect the bleeding site or abnormal vasculature. On laparotomy, ten areas of submucosal sponginess with bluish discoloration were noted in the distal ileum from about 3 feet proximal to the ileocecal valve down to 5 cm from it. About 3 feet of the involved ileum was resected. Patient had an uneventful recovery without recurrence of GI bleed. Histopathological examination of these lesions revealed band like zones of fibrosis near submucosal lymphoid tissue along with increased number of congested submucosal vessels of various calibers, focally extending through muscu- laris mucosa into lamina propria. Special stains for organisms (AFB & GMS) were negative. Precise etiology of this nonneoplastic fibroinflam- matory process remains unclear. Conclusions: To our knowledge, this unique nonneoplastic fibroinflam- matory lesion of the small bowel presenting as overt GI bleeding in a patient with HIV who was previously treated for KS has not been reported so far. Although the exact etiopathogenesis of this lesion remains conjec- tural, it may be related to immune status of the patient and/or secondary to the therapy. 354 Role of endoscopic ultrasound in definitive diagnosis and staging of lung adenocarcinoma localized to superior mediastinum Shiro Urayama*. 1 Internal Medicine, University of California Davis, Sacramento, CA, United States. Purpose: We describe a particular role of endoscopic ultrasound in diag- nosis and staging of mass located in superior mediastinal region. Methods: Retrospective case review of a patient presented as having a unknown primary adenocarcinoma with mediastinal lymphadenopathy. Results: A 67y/o male presented with complaints of refractory nausea, vomitting and significant weight loss of 3 months duration. Upper endo- scopic evaluation revealed linear ulcerations in esophagus consistent with recurrent reflux symptoms, but no evidence of gross malignancy. CT scan evaluation of abdomen and chest showed paraesophageal & pretracheal/ subcarinal adenopathies without an evidence of primary tumor. Mediasti- noscopic biopsies of the lymph nodes showed evidence of adenocarcinoma. The patient’s symptoms had significantly improved after high dose proton- pump inhibitor treatment was initiated. We repeated upper endoscopy to evaluate for any evidence of primary upper GI tract malignancy. We also performed endoscopic ultrasonography during the second endoscopy to evaluate periluminal region. Mucosal examination showed healed esoph- ageal ulcers and rest of the upper GI tract to second portions were normal. EUS, however, showed 3cm mass lesion in the left superior mediastinum located just above the aortic arch, adjacent to esophagus, vertebral body, and left pulmonary field. Several paraesophageal adenopathies were also noted. More importantly, left adrenal gland was identified transgastrically and showed a focally enlarged appearance. Pancreas was normal. EUS- guided fine needle aspiration was completed of the superior mediastinal mass as well as the left adrenal gland. Both of these lesions showed adenocarcinoma and similar cellular appearance from the paratracheal lymph node lesion obtained at the time of mediastinoscopy. Thus, the final diagnosis and the staging was primary lung adenocarcinoma with stage IV with metastasis to left adrenal gland. Conclusions: This case illustrates the utility of endoscopic ultrasound in evaluation of mediastinal adenopathy with unknown primary malignancy as well as evaluation of superior mediastinal lesions in providing both diagnosis and staging of lung cancer. EUS provided not only the identifi- cation of primary malignancy but also accurately identified the advanced stage with positive metastatic cellular aspirate of the left adrenal gland during one session. 355 Software assisted detection of abnormalities of the GI tract for wireless capsule endoscopy Ofra Zinaty, M.Sc., Harold Jacob, M.D., Daphna Levy, M.Sc., Reuven Shreiber, M.D. and Arkady Glukhovsky, D.Sc.*. Yoqneam, Israel. Purpose: An ingestible wireless video capsule enables visualization of the small intestine beyond the reach of the endoscope. Detection of pathology is performed by a physician reviewing the recorded images. An algorithm for automatic detection of bleeding will assist the reviewer, thereby in- creasing the efficiency of the review process. Methods: A small bowel enteroscopy was performed using the Given® Diagnostic Imaging System. The blood detection algorithm automatically indicates suspicious images of the small bowel consistent with bleeding. The findings are presented to the reviewer as indicators on the time bar synchronized to the displayed video stream. The algorithm is based on detection of colorimetric abnormalities from an expected spectrum derived from spectral analysis of the video images. Each sample is compared to a reference representing blood, and to a reference representing healthy tissue of the patient. Each area of the image is assigned a value indicating the probability of the image to be a suspicious bleeding site. The probability indication function is based on a relative difference between the examined image sample and a reference sample of blood and healthy tissue. The reference of a healthy tissue is constructed using an adaptive approach. The blood detection algorithm is applied off-line, during the data processing phase. Results: The blood detection algorithm was verified by comparing results to 25 small bowel capsule enteroscopies that were interpreted by physi- cians. Based on this interpretation the following classification was made: 11 cases of bleeding, 6 cases of miscellaneous pathology, 4 cases with no diagnosed pathology, and 4 healthy volunteers. The automatic blood de- tection algorithm showed no false negative results with all known bleeding sites detected, and showed acceptably small amount of false positive results. Conclusions: The automatic detection algorithm may be of value in as- sissting the diagnostic procedure by prompting the physician to examine the sites of possible bleeding. This method may also be applicable to other pathologies. 356 “Atypical presentations” of celiac disease (CD) are the most common presentations Robert D. Zipser M.D., FACG 1 *, Sunil Patel, Donald W. Baisch 2 and Elaine Monarch 2 . 1 Medicine, Harbor-UCLA Medical Center, Torrance, CA, United States; and 2 Celiac Disease Foundation, Studio City, CA, United States. Purpose: The classic presentation of CD is childhood steatorrhea, weight loss and failure to thrive. In contrast, there is now increasing recognition that CD frequently has an adult onset without classic symptoms. Methods: To determine the most common presentations, officers of a large support group, Celiac Disease Foundation, did member surveys. All CD patients (n 1032) had diagnosis confirmed by small bowel biopsy. Results: At diagnoses, the median age was 46 years (n 968), and 14 patients were over age 80 years. Only 12% were diagnosed before the age of 10 years. The median body mass index (BMI) was 20 indicating that S113 AJG – September, Suppl., 2001 Abstracts

Transcript of “Atypical presentations” of celiac disease (CD) are the most common presentations

Methods: A 38 year old Caucasian male with HIV infection on anti-reteroviral therapy for last 5 years was admitted with intermittent marooncolored stools for 3 days and hemoglobin decrease from 15.5 g/dl to 6.5g/dl. He was treated for Kaposi’s sarcoma involving skin and stomach 5years ago with complete remission. He had two similar episodes of GIbleed 2 years ago but the GI work-up including upper and lower GIendoscopy, enteroscopy, small bowel barium x-ray, abdominal CT scanand Meckel’s scan failed to localize the bleeding site. His recent CD4 countwas 340 and viral load �1000. He never had any other opportunisticinfections.

On admission, he was orthostatic and pale otherwise rest of the exam-ination was unremarkable. A repeat colonoscopy revealed maroon bloodthroughout the colon. Small bowel enteroscopy upto mid small bowel didnot reveal any bleeding site. An RBC tagged scan showed evidence ofactive bleeding from small bowel but abdominal angiogram did not detectthe bleeding site or abnormal vasculature. On laparotomy, ten areas ofsubmucosal sponginess with bluish discoloration were noted in the distalileum from about 3 feet proximal to the ileocecal valve down to 5 cm fromit. About 3 feet of the involved ileum was resected. Patient had anuneventful recovery without recurrence of GI bleed. Histopathologicalexamination of these lesions revealed band like zones of fibrosis nearsubmucosal lymphoid tissue along with increased number of congestedsubmucosal vessels of various calibers, focally extending through muscu-laris mucosa into lamina propria. Special stains for organisms (AFB &GMS) were negative. Precise etiology of this nonneoplastic fibroinflam-matory process remains unclear.Conclusions: To our knowledge, this unique nonneoplastic fibroinflam-matory lesion of the small bowel presenting as overt GI bleeding in apatient with HIV who was previously treated for KS has not been reportedso far. Although the exact etiopathogenesis of this lesion remains conjec-tural, it may be related to immune status of the patient and/or secondary tothe therapy.

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Role of endoscopic ultrasound in definitive diagnosis and staging oflung adenocarcinoma localized to superior mediastinumShiro Urayama*. 1Internal Medicine, University of California Davis,Sacramento, CA, United States.

Purpose: We describe a particular role of endoscopic ultrasound in diag-nosis and staging of mass located in superior mediastinal region.Methods: Retrospective case review of a patient presented as having aunknown primary adenocarcinoma with mediastinal lymphadenopathy.Results: A 67y/o male presented with complaints of refractory nausea,vomitting and significant weight loss of 3 months duration. Upper endo-scopic evaluation revealed linear ulcerations in esophagus consistent withrecurrent reflux symptoms, but no evidence of gross malignancy. CT scanevaluation of abdomen and chest showed paraesophageal & pretracheal/subcarinal adenopathies without an evidence of primary tumor. Mediasti-noscopic biopsies of the lymph nodes showed evidence of adenocarcinoma.The patient’s symptoms had significantly improved after high dose proton-pump inhibitor treatment was initiated. We repeated upper endoscopy toevaluate for any evidence of primary upper GI tract malignancy. We alsoperformed endoscopic ultrasonography during the second endoscopy toevaluate periluminal region. Mucosal examination showed healed esoph-ageal ulcers and rest of the upper GI tract to second portions were normal.EUS, however, showed 3cm mass lesion in the left superior mediastinumlocated just above the aortic arch, adjacent to esophagus, vertebral body,and left pulmonary field. Several paraesophageal adenopathies were alsonoted. More importantly, left adrenal gland was identified transgastricallyand showed a focally enlarged appearance. Pancreas was normal. EUS-guided fine needle aspiration was completed of the superior mediastinalmass as well as the left adrenal gland. Both of these lesions showedadenocarcinoma and similar cellular appearance from the paratracheallymph node lesion obtained at the time of mediastinoscopy. Thus, the final

diagnosis and the staging was primary lung adenocarcinoma with stage IVwith metastasis to left adrenal gland.Conclusions: This case illustrates the utility of endoscopic ultrasound inevaluation of mediastinal adenopathy with unknown primary malignancyas well as evaluation of superior mediastinal lesions in providing bothdiagnosis and staging of lung cancer. EUS provided not only the identifi-cation of primary malignancy but also accurately identified the advancedstage with positive metastatic cellular aspirate of the left adrenal glandduring one session.

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Software assisted detection of abnormalities of the GI tract forwireless capsule endoscopyOfra Zinaty, M.Sc., Harold Jacob, M.D., Daphna Levy, M.Sc., ReuvenShreiber, M.D. and Arkady Glukhovsky, D.Sc.*. Yoqneam, Israel.

Purpose: An ingestible wireless video capsule enables visualization of thesmall intestine beyond the reach of the endoscope. Detection of pathologyis performed by a physician reviewing the recorded images. An algorithmfor automatic detection of bleeding will assist the reviewer, thereby in-creasing the efficiency of the review process.Methods: A small bowel enteroscopy was performed using the Given®Diagnostic Imaging System. The blood detection algorithm automaticallyindicates suspicious images of the small bowel consistent with bleeding.The findings are presented to the reviewer as indicators on the time barsynchronized to the displayed video stream. The algorithm is based ondetection of colorimetric abnormalities from an expected spectrum derivedfrom spectral analysis of the video images. Each sample is compared to areference representing blood, and to a reference representing healthy tissueof the patient. Each area of the image is assigned a value indicating theprobability of the image to be a suspicious bleeding site. The probabilityindication function is based on a relative difference between the examinedimage sample and a reference sample of blood and healthy tissue. Thereference of a healthy tissue is constructed using an adaptive approach. Theblood detection algorithm is applied off-line, during the data processingphase.Results: The blood detection algorithm was verified by comparing resultsto 25 small bowel capsule enteroscopies that were interpreted by physi-cians. Based on this interpretation the following classification was made:11 cases of bleeding, 6 cases of miscellaneous pathology, 4 cases with nodiagnosed pathology, and 4 healthy volunteers. The automatic blood de-tection algorithm showed no false negative results with all known bleedingsites detected, and showed acceptably small amount of false positiveresults.Conclusions: The automatic detection algorithm may be of value in as-sissting the diagnostic procedure by prompting the physician to examinethe sites of possible bleeding. This method may also be applicable to otherpathologies.

356

“Atypical presentations” of celiac disease (CD) are the mostcommon presentationsRobert D. Zipser M.D., FACG1*, Sunil Patel, Donald W. Baisch2 andElaine Monarch2. 1Medicine, Harbor-UCLA Medical Center, Torrance,CA, United States; and 2Celiac Disease Foundation, Studio City, CA,United States.

Purpose: The classic presentation of CD is childhood steatorrhea, weightloss and failure to thrive. In contrast, there is now increasing recognitionthat CD frequently has an adult onset without classic symptoms.Methods: To determine the most common presentations, officers of a largesupport group, Celiac Disease Foundation, did member surveys. All CDpatients (n � 1032) had diagnosis confirmed by small bowel biopsy.Results: At diagnoses, the median age was 46 years (n � 968), and 14patients were over age 80 years. Only 12% were diagnosed before the ageof 10 years. The median body mass index (BMI) was 20 indicating that

S113AJG – September, Suppl., 2001 Abstracts

most had normal weights (n � 589). BMI was over 25 in 10% of respon-dents, and 10 patients (1.7%) were obese with BMI over 31. Diarrhea wasa common initial symptom, but a few had constipation. Other frequentpresentations included anemia, fatigue, flatus, bloating, and abdominalpain. There were no symptoms in 5% of CD patients, suspected by familyhistory or incidentally diagnosed at examinations for unrelated disorders.The most frequent initial diagnosis was irritable bowel syndrome (36% ofrespondents). Possibly because the presentations were atypical for CD, themedian time from initial physician evaluation to biopsy-proven diagnosiswas 12 months, and 19% had symptoms for over 10 years prior to diagnosis(n � 978). There was a median number of 3 physicians seen for thesesymptoms prior to diagnosis (range 1 to 30, n � 635 respondents).Conclusions: This large survey documents that the classic presentation ofsevere malabsorption with childhood onset is uncommon in CD. Instead,the most common presentations are adults with symptoms mimickingirritable bowel syndrome or with evaluation for anemia. With the preva-lence of latent CD reported at 1:300, we encourage heightened awarenessof the presenting symptoms of this common disorder.

LIVER

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The effect of Cyclosporin-A on complement receptor function inpatients with primary biliary cirrhosisMohamed Na Al-Aghbar, J. Neuberger, Adrian LWF. Eddelston andRoger Williams*. Liver Unit, King’s College Hospital. London UnitedKingdom.

Purpose: Many immunological abnormalities have been described in pa-tients with primary biliary cirrhosis(PBC) including suppressor cell andC3b-receptor functional defects. The C3b-receptor functional defect wasconfirmed by in vivo and in vitro studies. It was shown to be due toblocking of the receptors by serum factors including complement compo-nents. The present study investigated the effect of Cyclosporin-A (Cy.A) onC3b-receptor function and complement activation in patients with PBC.Methods: Sheep Red Blood Cells (SRBC) coated with anti-SRBC IgM andcomplement (SRBC-M-C) were incubated with peripheral blood mono-cytes to detect the C3b-receptor function. That was done before and after9 months treatment of 8 patients with PBC and compared to 12 untreatedpatients. Complement activation was measured by C3-d assay in 10 un-treated patients and after 9 months treatment with Cy-A and compared to4 normal controls.Results: Nine months treatment of 8 patients with PBC with Cy-A (3.5mg/Kg) was associated with a significant improvement of C3b-receptor func-tion. The mean number of SRBC-M-C attached to 100 monocytes isolatedfrom 8 treated patients (mean � SD � 46.3 � 21.3) was significantlyhigher when compared with 12 untreated patients (24.3 � 10.8) P � 0.01.Complement activation as measured by C3d values after 9 months treat-ment with Cy-A was (0.83 � 0.45mg/dl) compared to (0.66 � 0.17mg/dl)in 10 untreated patients (p � NS) and (0.47 � 0.07) in 4 normal controls(p � 0.02).Conclusions: 1. Cyclosporin-A treatment corrected the abnormality ofC3b-receptor functional defect in patients with primary biliary cirrhosis.2. Complement was shown to be activated in patients with PBC.3. Complement activation persisted after Cy-A treatment. However, theincrease in complement activation after Cy-A trearment did no reach asignificant value in this study. This could be due to the small number ofpatients studied.

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Phenelzine induced acute liver failureAnwar Q Al-Anezi MD and Kevork Peltekian MD*. 1Gastroenterology,Queen Elizabeth II Health Sciences Centre, Halifax, NS, Canada.

Purpose: Acute liver failure (ALF), is the final common pathway of severehepatocyte injury. Over 50% of ALF cases in the United States are now dueto drug hepatotoxicity. Phenelzine is a potent monoamine oxidase (MAO)inhibitor. It is indicated in the treatment of nonendogenous depression.Common GI side effects include constipation, dry mouth, and elevatedserum transaminases. We report a case of ALF in a patient treated withphenelzine for over 4 weeks.Methods: A 45-year-old woman was admitted to the hospital because ofjaundice, mild abdominal pain, sleep disturbances and fatigue. For 4 weeks,nonendogenous depression had been controlled with phenelzine 60 mg/dayin 3 divided doses. there Was no history of recent alcohol consumption,other medication intake or previous hepatitis. on physical examination,jaundice and hepatic tenderness were noted. encephalopathy was not ob-served. AST 2806 U/L, ALT 1253 U/L, Total Bilirubin 197 Umol/L,Albumin 28 g/L, and INR 2.4. Hepatits A, B, C, ANA, ASMA, and AMAwere negative. Quantitative immunoglobulins, serum ceruloplasmin level,and hemoglobin A1C level were within normal range. HFE mutation wasnot present. Abdominal US showed gallstones but no biliary obstruction.Transjugular liver biopsy showed centrilobular congestion and hepatocel-lular necrosis with cholestasis.Results: Phenelzine was discontinued. During the following week thepatient’s condition deteriorated with encephalopathy, severe coagulopathyand hepatic coma. The king’s college prognostic criteria were used to guidemedical decision. Eventually, she underwent emergency orthotopic livertransplantation.Conclusions: Phenelzine hepatotoxicity has been reported in two cases inthe medical litrature. With the availability of safe and effective alternativeantidepressants, phenelzine use should be restricted. because most cases ofdrug-induced hepatotoxicity are rare, unpredictable, and not detected dur-ing clinical trials, postmarketing surveillance remains an essential means ofinsuring and monitoring prescription drug safety.

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Bile cast syndrome after orthotopic liver transplantationAnezi E. Aneziokoro1, John Losurdo1, Viviana Caban1 and SumanKaur1*. 1Division of Digestive Diseases, Rush-Presbyterian-St. Luke’sMedical Center, Chicago, IL, United States.

Purpose: Biliary tract complications following orthotopic liver transplan-tation (OLT) are associated with morbidity and have been reported in7–31% of patients. Bile cast syndrome is a known complication associatedwith multiple fixed filling defects in the intrahepatic or extrahepatic biliarytree. These filling defects are casts which conform to the contour of thesegment of biliary tree where they are found. The etiology of bile castformation is unclear, however theories include sloughing of the biliaryepithelium due to prolonged cold ischemia, ongoing ischemia, biliarystasis, infection, alteration of biliary milieu and chronic rejection have beenproposed. Our purpose is to determine the clinical characteristics andmanagement of patients with bile cast syndrome post-OLT.Methods: We reviewed the data of 460 consecutive OLTs performedbetween January 1993 and December 2000 at our institution. 37 patients(8.04%) with bile cast syndrome were identified. Clinical characteristics ofthese patients(pts) and their management was reviewed.Results: Indications for OLT were cirrhosis due to hepatitis C (HCV) in15pts (41%), Alcohol in 8pts (22%), Alcohol � HCV in 3pts, hepatitis B(HBV) in 3pts and other in 8pts(22%). The mean cold ischemia time was8 hours (range 4.02 to 16.10 hours). Mean time from OLT to diagnosis ofcasts was 167 days (7 days to 4.5 years). Casts were identified by endo-scopic retrograde cholangiography (ERC) in 33 patients and T-tube cholan-giography (TTC) in 4 patients. Other cholangiographic findings in additionto casts were anastomotic strictures in 17 patients, non-anastomotic stric-tures in 12 patients and no abnormalities in 7 patients. Indications forcholangiography were asymptomatic biochemical graft dysfunction (N �27), acute cholangitis (N � 6) and routine TTC (N � 4). Graft dysfunctionwas primarily cholestatic. All patients underwent therapeutic ERC withsphincterotomy, cast extraction and balloon dilatation of strictures (as

S114 Abstracts AJG – Vol. 96, No. 9, Suppl., 2001