Methods: A 71-year old man was admitted for coronary artery bypasssurgery and underwent endotracheal intubation and surgery without event.He was intubated for 5 days. Post extubation he developed coughing whileeating. Physical examination was normal and videofluoroscopic bariumswallow was not diagnostic. An esophagogastroduodenoscopy and brocho-scopy were planned at a simultaneous session to exclude a suspected TEfistula. An Olympus G100 video upper endoscope was passed under directvision into the esophagus.Results:At 18 cm from the incisors, a small opening to a possible fistuloustract was seen in the esophagus. The surrounding area was devoid oferythema, ulceration or tumor. The rest of the esophagus and stomach werenormal. As the endoscope was withdrawn, a 2 mmopening to the fistuloustract was again visualized. Using a spray catheter, 0.5 ml of methylene bluewas injected into the opening. An identical upper endoscope was then usedto perform tracheoscopy. Approximately 4 cm into the posterior trachea, anedematous mucosal protrusion with surrounding methylene blue stainingwas noted. The scope was advanced to the major carina and revealed noother abnormality. The patient was taken to the operating room where asmall tracheal and esophageal tear with a fibrotic tract was found at the 4th

and 5th tracheal rings. The patient underwent closure of the tracheal andesophageal communication with repair utilizing a Bovine pericardial patch.Conclusions: This case illustrates the usefulness of directed methyleneblue instillation during ‘same-sitting‘ esophagoscopy and tracheoscopy asa method of diagnosing TE fistula.

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Two siblings with heterozygous phenotype SZ Alpha-1-Antitrypsindeficiency and end-stage liver diseaseYoon David K M.D., Kueppers Friedrich M.D., Genta Robert M M.D.,FACG, Khaoustov Vladmire I M.D., Klintbalm Goran B M.D., YoffeBoris M.D.* Baylor College of Medicine, Houston, Texas, UnitedStates.

Purpose: The most common type of protease inhibitor (PI) deficiency isthe alpha-1-antitrypsin (AAT) deficiency which is one of the most frequentlethal genetic diseases. AAT deficiency affects Caucasians and 10 % of theUS population of European descent are heterozygous carriers. At the levelof transcription and mRNA stability everything appears normal, but due toa single genetic point mutation causing a Glu342 to Lys substitution in therough endoplasmic reticulum of the AAT molecule the folding of theprotein to its tertiary structure is slowed down. This results in a decreasedlevel of AAT level in the plasma and intracellular aggregation of AAT.Patients with homozygous type PI ZZ develop cirrhosis due to intracellularstorage of AAT and are diagnosed by very a low level of AAT (15 % ofnormal levels) ?? phenotyping. The relationship of heterozygous states tothe development of liver disease remains controversial with only fewpublished case reports. We report two siblings, ages 42 (WF) and 47 (WM),with heterozygous type PI SZ AAT deficiency without any other risk factorfor liver disease who developed end-stage liver disease and underwent livertransplantation.Methods: The sera, lymphocytes, and liver were obtained during clinicalfollow up. AAT levels, phenotyping, and immunohistologic assessmentwith specific mouse monoclonal AAT-Ab were done on the liver speci-mens. Twelve control liver specimens were analyzed for any reactivity tothe monoclonal AAT-Ab.Results:AAT levels were only moderately decreased and were 74 mg/dLand 90 mg/dL (140–470), respectively. Immunochemistry with mousemonoclonal AAT-Ab showed a prominent accumulation of AAT in thehepatocytes. Liver specimen from 12 control patients revealed no reactivityto the monoclonal antibodies.Conclusions:Since heterozygous AAT deficiency such as PI SZ is usuallynot associated with an end-stage liver disease, near normal levels of AATare often ignored. Here, we report a first presentation of heterozygous AATdeficiency PI SZ in two siblings who developed liver disease and under-went liver transplantation. Our case accentuates the need for phenotypingand identification of individuals carrying the Z allele of AAT deficiency

and immunohistologic assessment with specific monoclonal AAT-Ab inpatients with suspected AAT deficiency.

706

A case of bleeding jejunal ulcer due to vasculitis from hydroxyureaYousuf Khurshid M.D.; Kathula S. M.D.; Hillman N. M.D.; Pacheco J.M.D.; Barde C.J. M.D., FACG; Gopalswamy N. M.D., FACG. VAMedical Center/Wright State University, Dayton, Ohio.

Case:A 54-year-old Caucasian male presented with melena and dizziness.He was on hydroxyurea for myelofibrosis. Patient was hemodynamicallyunstable. Rectal exam revealed bright red blood. NG tube aspirate wasclear. His hematocrit was 16.2%. Blood urea nitrogen was 58mg/dl andcreatinine was 1.1mg/dl. Platelet count, PT and PTT were normal. Hereceived four units of packed red blood cells (PRBC) and fluids. EGD andcolonoscopy were normal. Melena continued and the patient was transfusedsix more units of PRBCs. During push enteroscopy, apart from fresh clotsin the jejunum no distinct lesion was noted. During exploratory laparotomywith intraoperative enteroscopy a 5mm shallow bleeding ulcer was foundin the distal jejunum. Wedge resection of the ulcer was done. Microscopicexam revealed ulceration with features compatible with hypersensitivityvasculitis. Common causes of vasculitis were ruled out and hydroxyureawas suspected as the source of his vasculitis. Hydroxyurea was discontin-ued and no further bleeding was noted. The patient was discharged in stablecondition. During two months of follow-up there has been no recurrence ofbleeding. His hematocrit was 31.6%. The patient is currently on busulphanfor his myelofibrosis.Discussion:Jejunal ulcers are uncommon causes of gastrointestinal bleed-ing. Push enteroscopy and intraoperative enteroscopy have been helpful inidentifying small bowel lesions and in directing appropriate treatment. Themain adverse effects of hydroxyurea are marrow suppression, megaloblas-tic anemia and GI disturbances. Vasculitis and cutaneous ulcers fromhydroxyurea are well known adverse effects. Painful oral ulcers have alsobeen reported, but gastrointestinal ulceration is not known. To our knowl-edge this is the first report of bleeding jejunal ulcer due to vasculitis causedby hydroxyurea. Hydroxyurea should be considered as a cause of vasculitisand gastrointestinal bleeding.

707

Pylephlebitis, portal vein thrombosis, hepatic abscess; unusualcomplications in a teenager with Crohn’s diseaseZapata Fernando MD, Ruzal-Shapiro Carrie MD, DeFelice Amy MD,Kazlow Philip MD, FACG. Columbia University, New York, NY, USA.

Pylephlebitis, portal vein thrombosis and hepatic abscesses are unusualcomplications of Crohn’s disease. Our patient, a 17 year old male had beendiagnosed with Crohn’s disease one year prior to the present illness. Hisclinical course had been uneventful, and his only medication was me-salamine 800 mg PO TID.

The patient re-presented with a one week history of prolonged fever to106°F, increased abdominal pain, vomiting and diarrhea and a recent sixpound weight loss. The physical exam was only notable for mild abdominaltenderness in the right lower quadrant. Lab data at that time includednegative blood cultures, a normal CBC, ESR of 40 mm/hr, normal chem-istries and normal liver enzymes except for GGT of 138. Oral Metronida-zole and Ciproflaxcin were added to the therapeutic regimen. However, thespiking fevers continued. The patient returned for a Gallium scan three dayslater which was negative. The patient was admitted to the hospital andplaced on intravenous steroids and IV antibiotics. Within three days hebecame afebrile and had a significant resolution of his symptoms. Twoweeks later, the patient presented with the same constellation of symptoms.Blood cultures drawn at that time were again negative. Repeat blood workwas essentially unchanged from the previous admission. The patient wasthen given a one week course of IV steroids and antibiotics. Within the first48 hours he once again experienced a resolution of his fevers and had amarked clinical improvement.

2616 Abstracts AJG – Vol. 95, No. 9, 2000

Two weeks later the patient presented again with the same symptoms,but now with new onset of back pain. Spinal X-ray showed mild thoraciclordosis. Blood work at this time was notable for an ESR of 103. A repeatCT scan now revealed two large hepatic abscess cavities and absence ofblood flow to the right portal vein. The patient was started on IV antibioticsand underwent successful percutaneous drainage of the abscess cavities. Heultimately completed five months of home antibiotic therapy with periodicCT scans illustrating a resolution of the hepatic abscesses. Hepatic abscessand protal pylephlebitis are rare complications of inflammatory boweldisease. It is thought to occur through bacterial seeding of the bloodstreamspossibly via the mechanism of translocation. A hypercoaguable state whichis often found in IBD patients may predispose them to these complications.In patients with IBD and unexplained fevers, hepatic abscess and portalpylephlebitis must be given serious consideration when formulating adifferential diagnosis.

708

Severe protein-losing enteropathy secondary to chronic NSAID useZein Claudia O. MD, Nehra Vandana MD. Mayo Clinic and MayoFoundation, Rochester, Minnesota.

Introduction: Approximately 60% of patients on chronic NSAID therapydevelop intestinal inflammation which is frequently associated with low-grade bleeding and protein loss. The protein loss is typically mild. Wepresent a case of a patient with severe protein-losing enteropathy secondaryto NSAID use.Case Report: A 40 year-old male was referred for evaluation of mildanemia. For two years prior to presentation the patient was taking Diclofe-nac at a dose of 100mg bid for progressive low-back discomfort. Thepatient was otherwise asymptomatic. Physical examination was unremark-able. Laboratory studies showed a hemoglobin of 10.7 g/dL. A serumprotein electrophoresis showed total protein of 4.0 (6.3–7.9), albumin 2.0(3.4–4.8), gamma-globulin 0.3 (0.7–1.7). Other laboratory tests were nor-mal. Urinary protein elimination over 24h was normal. After a 24h stoolcollection, Alpha-1 Antitrypsin clearance was found to be 607 mL/24h (NRd 27). The fat content was 1.9g/24h (2–7 g/24h). an EGD showed reactivegastropathy (confirmed on biopsy). The small bowel appeared normal(normal biopsy). A colonoscopy showed a shallow ulceration at the ileo-cecal valve consistent with NSAID-associated injury. The terminal ileumappeared normal. CT scan of the abdomen and pelvis, Upper GI and smallbowel XR were unremarkable. Lymphoscintigraphy was normal. AHMPAO white blood cell scan showed diffuse increased activity in thesmall bowel and colon consistent with an inflammatory process. TheNSAID was stopped and the patient was started on Tramadol for his backpain. An albumin level checked 7 weeks after stopping NSAID showedmarked improvement (from 2.0 to 3.2 g/dL). Ten weeks after stoppingNSAID a repeat stool collection for Alpha-1 Antitrypsin clearance showeddramatic improvement (from 607 to 176 mL/24h).Conclusion: This case illustrates the occurrence of severe protein losingenteropathy secondary to chronic NSAID use. Although most cases ofintestinal protein loss secondary to NSAID are mild, in rare cases markedlysevere loss can occur.

709

Spontaneous clearance of chronic hepatitis C during pregnancyZein Claudia O., Abu-Lebdeh Haitham S., Zein Nizar N.* Mayo Clinicand Mayo Foundation, Rochester, MN, United States.

Purpose: Introduction: Spontaneous clearance of the hepatitis C virus(HCV) once chronic hepatitis is established is extremely rare. We presenta case of spontaneous clearance of HCV during pregnancy. Pregnancy ischaracterized by significant changes in the maternal immune system.Among these uterine decidual and placental cells produce a large array ofcytokines which contribute to deviation of the immune response from Th1to Th2. Changes in circulating and local T-cell subsets is also described.

Methods: Case Report: A 27-year old woman with an established diag-nosis of HCV infection and persistently elevated transaminases for at leastthree years was seen at Mayo Clinic in August of 1998. A blood transfusionin 1995 was presumed to be the source of HCV acquisition. At the time ofinitial evaluation at Mayo, transaminases were 43 upper limit of normal,HCV antibodies were detected by EIA ??, and HCV RNA was detected byPCR. RNA quantitation by PCR was 2.64 mEq/mL and HCV genotype was1a. A liver biopsy showed portal hepatitis consistent with active chronichepatitis C without fibrosis. The patient received no therapy for HCVinfection or any other antiviral agent. Fourteen months after the initial visitthe patient became pregnant. Her transaminases, still elevated at the be-ginning of pregnancy (AST 69, ALT 117), gradually reached normal valuesafter the 5th month of pregnancy. Four months after delivery transaminasescontinued to be normal. HCV RNA by PCR was persistently negative ontwo separate occasions 2 weeks apart, confirming spontaneous clearance ofHCV RNA.Discussion:Spontaneous clearance of chronic HCV infection is extremelyrare. This case illustrates spontaneous HCV RNA clearance and normal-ization of transaminases during pregnancy in a patient with previouslydocumented chronic hepatitis C. We postulate that changes in the maternalimmune system, maybe related to pregnancy, influenced the outcome ofthis case. In most cases of HCV infection the host’s immune system failsto mediate resolution of the infection, leading to chronic and persistentinfection. Further investigation of the changes in immunity elicited bypregnancy and the outcome of chronic and acute HCV infection in pregnantwomen may improve our understanding of immune mechanisms for HCVclearance.

710

Hepatitis A presenting with meningoencephalitisZeng N. M.D., Zuckerman M.J. M.D., FACG. Texas Tech UniversityHealth Sciences Center, El Paso.

Background: Meningoencephalitis may rarely herald the onset of infec-tious hepatitis.Case report: A 20 year old Hispanic male without previous medicalproblems presented to the ER after having a seizure followed by decreasedmental acuity for 2 days. The seizure was initially tonic clonic and becamepartial jerking in nature, focused only on the face and left arm. Associatedsymptoms included fever, nausea, vomiting, drowsiness, and generalizedmuscle weakness. There was no rash, jaundice or history of head trauma,drug abuse, alcoholism, animal or insect bites, or blood transfusion. He hadrecently traveled to Juarez, Mexico and eaten seafood. On examination, hewas afebrile, anicteric, slightly obtunded and disoriented. Initial laboratoryinvestigations showed normal CBC, electrolytes, liver function tests andchest x-ray. Blood and urine cultures were negative. Serology was negativefor EBV, HIV and VDRL. Head CT scan, MRI and EEG were normal.Cerebrospinal fluid was normal. While in the hospital, his neurologicalcondition improved. However, his SGOT and SGPT became elevated atday five (364 and 412) peaking at day six (1768 and 888), with bilirubinelevated at day six (1.8), and peaking at day ten (3.7). The serum hepatitisA IgM antibody was positive and HBsAg and anti-HCV were negative. Thepatient was discharged in stable condition and had no recurrent seizure.Hepatic enzymes gradually returned to normal. Review of literature: A totalof 31 cases of meningoencephalitis preceding, coincident with or after theonset of the icteric phase of infectious hepatitis have been documented inthe literature (17 cases were reported before current viral hepatitis serol-ogies were available, 12 cases were hepatitis A and 1 hepatitis B, one bothA and C). Neurological manifestations in the 12 hepatitis A patientsincluded mental status changes (7) or generalized tonic-clonic seizures (5).All patients recovered without neurological sequelae.Conclusion: Meningoencephalitis is a rare extrahepatic manifestation ofHAV infection. The identification of HAV as a cause of acute viralencephalitis by readily available serologic methods reduces the need forextensive costly diagnostic evaluations and for potentially toxic empirictreatment.

2617AJG – September, 2000 Abstracts