| Autoimmune cholangitis is a rare chronic cholestatic liver disease. We describe the case of a 65-year-old woman with celiac disease who presented to us with fever, jaundice and weight loss. serum biochemical study showed marked increase in alkaline phosphatase and gammaGT levels. Antinuclear antibodies were positive, while antimitochondrial and anti-smooth-muscle antibodies were negative. Liver biopsy was compatible with primary autoimmune cholangitis. The patient was successfully treated with azathioprine and methylprednisolone. We describe here the uncommon association of autoimmune cholangitis with celiac disease and review the prevalence of liver diseases in patients with celiac disease. ( info) |
2/440. Hepatocellular carcinoma with fibrolamellar pattern in a patient with autoimmune cholangitis. A 75-year-old woman with a 15-year history of autoimmune cholangitis underwent orthotopic liver transplantation because of progressive liver decompensation. A clinically unsuspected hepatocellular carcinoma was found. A portion of the tumor showed fibrolamellar differentiation. Hepatocellular carcinoma, either with the usual pattern or with a fibrolamellar pattern, is rare in the setting of primary biliary cirrhosis, but has been seen in the setting of autoimmune hepatitis. Autoimmune cholangitis is a relatively recently recognized form of autoimmune liver disease whose association with hepatocellular carcinoma has yet to be determined. ( info) |
3/440. Negative conversion of antimitochondrial antibody in primary biliary cirrhosis: a case of autoimmune cholangitis. Autoimmune cholangitis is a clinical constellation of chronic cholestasis, histological changes of chronic nonsuppurative cholangitis and the presence of autoantibodies other than antimitochondrial antibody (AMA). It is uncertain whether this entity is definitely different from AMA positive primary biliary cirrhosis (PBC), though it shows some differences. We report a case of autoimmune cholangitis in a 59-year-old woman, who had been previously diagnosed as AMA-positive PBC associated with rheumatoid arthritis, has been converted to an AMA-negative and anticentromere antibody-positive PBC during follow-up. The response to ursodeoxycholic acid treatment is poor except within the first few months, but prednisolone was dropping the biochemical laboratory data. ( info) |
4/440. Hepatobiliary dysfunction as the initial manifestation of disseminated cryptococcosis. A case of hepatobiliary dysfunction as the initial manifestation of disseminated cryptococcosis is described. The patient was admitted with symptoms of hepatitis with cholestatic jaundice. Antibody tests for hepatitis b and C and human immunodeficiency virus were negative. The patient continued to deteriorate clinically. Eventually, the patient succumbed to hepatic failure. autopsy disclosed systemic cryptococcosis that caused extensive necrosis of the liver. In review of the literature, only nine cases of cryptococcal infection presenting as hepatitis, cholangitis, and cholecystitis as initial manifestation were reported. Four of these patients had been subjected to exploratory laparotomy for clinical suspicion of acute abdomen. One patient developed cirrhosis as a result of cryptococcal hepatitis. Two patients succumbed to hepatic failure. cryptococcosis is known to occur commonly in immunocompromised patients, yet only two reported cases presenting as hepatitis were associated with immunocompromised status. ( info) |
5/440. Prolonged recovery after extended right hepatic lobectomy in a patient with severe blunt liver injury and laceration of the vena cava. A report of case with special references to autotransfusion and complications of biliary decompression. A patient with severe blunt liver injury and laceration of the vena cava who underwent a successful extended right hepatic lobectomy is reported. The use of autotransfusion unit saved the patient from exsanguination. His postoperative course was complicated by renal and hepatic failure, bile leakage, and persistent jaundice due to cholangitis. Prolonged choledochal drainage via T-tube obviously acted as a source of infection. The use of autotransfusion, choledochal drainage and the proper timing of its removal, the treatment of vena cava lesions and jaundice due to cholangitis in patients with severe liver trauma are discussed. ( info) |
6/440. Hepatic sickling crisis mimicking recurrent cholangitis. A 22-year-old man with homozygous sickle cell disease presented with recurrent fever, right upper quadrant pain and jaundice. Liver biopsy confirmed the diagnosis of hepatic sickling crisis; the symptoms responded to hydroxyurea therapy. Hepatic vasocclusive crisis can diagnosed on liver biopsy, and need not be a diagnosis of exclusion. ( info) |
7/440. Obstructive jaundice and acute cholangitis due to papillary stenosis. Papillary stenosis is characterized by fixed fibrosis leading to structural outflow obstruction and it is usually secondary to inflammation and fibrosis from the chronic passage of gallstones, episodes of acute pancreatitis, chronic pancreatitis, sclerosing cholangitis, peptic ulcer disease, and cholesterolosis. However, obstructive jaundice with or without acute cholangitis which leads the physician to suspect the presence of malignancy as a cause is a rare manifestation of papillary stenosis. We report here a case of papillary stenosis presenting with obstructive jaundice and acute cholangitis. The lesion was so difficult to exclude the presence of malignancy preoperatively and intraoperatively that a pylorus-preserving pancreaticoduodenectomy was performed. Histologic examination of the resected specimen revealed fibrosis, adenomatoid ductal hyperplasia, and mild chronic inflammation of the papilla of Vater and distal common bile duct. ( info) |
8/440. Case report: two cases of biliary papillomatosis with unusual associations. Papillomatosis arising from the biliary tree is a well recognized but rare entity. We encountered two patients with this condition. However, one of them had associated hepatocellular carcinoma and cirrhosis and the other had concomitant recurrent pyogenic cholangitis. To our knowledge, these associations have not been reported before. We, therefore, present these clinical problems and highlight the added difficulty in the management of these patients. ( info) |
9/440. Adenomyomatous hyperplasia of the papilla of Vater: A sequela of chronic papillitis? A case of adenomyomatous hyperplasia of the papilla of Vater is described. The lesion presented as a small polypoid tumor projecting into the duodenal lumen, causing obstruction and dilatation of the common bile duct. Serial cross-section of the ampulla showed diffuse thickening of the muscular layer corresponding to Oddi's sphincter, with resulting narrowing of the lumen. Many ductal or glandular components were dispersed within the mucosa and the muscular layer and were admixed with lymphocytes, a few lymphoid aggregates, and fibrosis. Based both on the absence of cellular atypia and the presence of inflammation, fibrosis, and preservation of the normal architecture of the ampulla, we favor the interpretation that this hyperplastic lesion represents a sequela of chronic papillitis. The different diagnoses for this lesion are presented along with a review of the literature. ( info) |
10/440. Successful pregnancy in a woman with secondary biliary cirrhosis with portal hypertension from recurrent pyogenic cholangitis. A case report. BACKGROUND: pregnancy in women with secondary biliary cirrhosis due to recurrent pyogenic cholangitis is extremely rare. Little information is available on the effect of pregnancy on the disease and vice versa. CASE: A patient who had secondary biliary cirrhosis due to recurrent pyogenic cholangitis complicated by splenomegaly and portal hypertension had a successful pregnancy. Although she had a history of esophageal variceal bleeding before this pregnancy, there was no such bleeding during pregnancy. She had an uneventful antenatal course except that her liver enzyme level fluctuated slightly. The serum bilirubin level increased during the third trimester of pregnancy but returned to the prepregnant level after delivery. CONCLUSION: Termination of pregnancy may not be the only option for management. The management protocol for patients with primary biliary cirrhosis complicating pregnancy, which includes regular fetal surveillance and monitoring of maternal liver function, should be considered for pregnant women with secondary biliary cirrhosis. ( info) |