Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This case report documents a patient who presented with retained common bile duct (CBD) stones, recurrent attacks of cholangitis, CBD stricture, and secondary biliary cirrhosis. Endoscopic or surgical treatment of choledocholithiasis must be considered early in treatment.
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PMID:Distal common bile duct stricture and secondary biliary cirrhosis due to choledocholithiasis. 181 43

Twelve patients with portal hypertension caused by biliary cirrhosis were treated from January, 1983- June. 1990. There were 6 women and 6 men. Age ranged from 20 to 50 years with an average of 35.5 years. In all patients, a significant feature of this series was the long period of biliary obstruction caused by bile duct stones (N = 7), congenital atresia of biliary tract (N = 1), Caroli disease (N = 1), sclerosing cholangitis (N = 1), sclerosing carcinoma of biliary tract (N = 1), and benign postoperative stricture of biliary tract (N = 1). The incidence was 6.6% in portal hypertension; 2.2% in biliary tract disease and 18% in intrahepatic cholelithiasis. Esophagogastric varices were demonstrated in 6 cases. In 5 cases with or without esophageal varices, varices around the extrahepatic bile duct were detected before or during operation. Some complications including poor liver function, dilated collaterals around extrahepatic bile duct and biliary infection with underlying biliary stones existed simultaneously, therefore the management of these cases was very difficult. There was no single approach to patients with portal hypertension caused by biliary cirrhosis and it should be treated as an individual basis.
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PMID:Portal hypertension caused by biliary cirrhosis. 188 48

The long-term prognosis of extrahepatic biliary atresia after surgical restoration of bile flow is still controversial. An ongoing process of cirrhosis and the development of portal hypertension continue to create frequent and frustrating management problems. Clinical features, hepatic function, echotomography aspect, calcium-phosphorus metabolism and serum levels of 25-OH-D-3 were evaluated in 12 anicteric patients with extrahepatic biliary atresia successfully treated in a period from 1974 through 1987. Seven of these children had a total of 21 episodes of cholangitis. In five patients liver biopsy, obtained at the time of the external diversion closure, showed a biliary cirrhosis. Growth, development and hepatic function were normal in all children studied; one patient had esophageal varices. The serum levels of 25-OH-D3 in patients without oral supplementation of vitamin D are lower than normal. This deficit can be corrected by oral administration of vitamin D. Our study revealed that the children with successful portoenterostomy appeared to thrive normally and that they tolerated the relatively mild liver damage. We believe that Kasai operation should be done in all patients with extrahepatic biliary atresia and that the liver transplantation is to be reserved only in those with unsuccessful Kasai. In our experience external diversion was not useful to prevent cholangitis and moreover it complicates the hepatectomy in case of transplantation.
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PMID:[Long-term prognosis of patients with extrahepatic biliary atresia successfully treated with surgery. Our experience]. 194 2

A 17-year-old patient suffering from Crohn disease (CD) and liver cirrhosis is presented. At an advanced stage of the disease, he died of a concomitant urosepsis. Autopsy showed that the liver cirrhosis was caused by sclerosing cholangitis. This very rare complication of CD in adolescence is discussed.
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PMID:Crohn disease with sclerosing cholangitis and liver cirrhosis in adolescence. 195 60

Cyclosporine appears to have abrogated age as a contraindication to kidney transplantation in the elderly, although it is unclear whether this is true for other types of solid organ transplantation. We performed a retrospective analysis of liver transplant recipients who were 60 years of age and older (n = 23) versus recipients of primary transplants who were 18 to 59 years of age (n = 84). Indications in recipients over 60 included alcoholism (6), postnecrotic cirrhosis (6), cancer (4), primary biliary cirrhosis (3), sclerosing cholangitis (2), and one patient with polycystic liver disease. There were no important differences in the initial transplant hospitalization or the incidence of infection and rejection between the two groups. No patient in the over-60 population required retransplantation. Actuarial patient survival is 83% at 2 years for recipients 60 years of age and above compared to 76% patient survival in adult recipients who are under the age of 60. Liver transplant recipients over the age of 60 years have excellent patient and graft survival and the same postoperative morbidity as recipients who are under 60 years of age. Therefore, advanced age does not appear to be a contraindication to orthotopic liver transplantation.
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PMID:Orthotopic liver transplantation in patients 60 years of age and older. 199 39

Noninfectious obliterative cholangitis results from biliary tract inflammation in clinical conditions such as biliary atresia and sclerosing cholangitis. The purpose of this study was to develop an animal model of noninfectious biliary tract inflammation and fibrosis. An implantable osmotic pump was connected to a catheter placed into the gallbladder of hamsters. Phorbol myristate acetate (PMA) was infused into the biliary tract for periods of 6 hours to 28 days. After 7 days the animals developed neutrophil infiltration, cellular necrosis, and edema of the biliary ducts. After 14 days, the animals demonstrated intrahepatic cholestasis with bile duct fibrosis and acute and chronic inflammatory cell infiltration. By 28 days pronounced portal fibrosis was present, some of which created an early bridging cirrhosis pattern. In addition there was evidence of neocholangiogenesis. We conclude that long-term PMA infusion into the biliary tract generates an inflammatory response characterized by obliterative cholangitis and fibrosis, sharing many of the histologic features of human biliary atresia. This model may provide a relatively simple technique for investigating the process of nonpyogenic biliary tract inflammation.
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PMID:Experimental obliterative cholangitis. A model for the study of biliary atresia. 200 17

Black and brown pigment gallstones are morphologically, compositionally, and clinically distinct. Black stones form primarily in the gallbladder in sterile bile and are associated with advanced age, chronic hemolysis, alcoholism, cirrhosis, pancreatitis, and total parenteral nutrition. Brown stones form not only within the gallbladder but also within the intrahepatic and extrahepatic ducts; they are uniformly infected with enteric bacteria and are usually associated with ascending cholangitis. Brown stones are related to juxtapapillary duodenal diverticula and are the predominant type of de novo common bile duct stones. Cholecystectomy is usually curative in black pigment stone disease, whereas stones often recur after cholecystectomy for brown stone disease. The pathogenesis of black stones is probably related to nonbacterial, nonenzymatic hydrolysis of bilirubin conjugates. At the pH of bile, this results in two monohydrogenated bilirubin anions that precipitate with calcium ions. Bilirubin monoconjugates that are increased in several conditions, such as Gilbert's syndrome and chronic hemolysis, may play a pivotal role in black stone formation as a source of unconjugated monohydrogenated bilirubin and as a possible co-precipitant with calcium. The precipitation of calcium carbonate and phosphate is influenced by local gallbladder factors. Brown pigment stones are formed in bile infected with enteric bacteria that elaborate hydrolytic enzymes: beta-glucuronidase, phospholipase A, and conjugated bile acid hydrolase. The resulting anions of bilirubin and fatty acids form insoluble calcium salts. We used nb/nb mice with a chronic hemolytic anemia as a model of hemolysis-induced black stone disease. The presence of 40% bilirubin monoconjugates in mouse gallstones indicated the importance of this moiety in the pathogenesis of black stones. Other data obtained by marrow transplantation experiments in mice revealed the relative importance of genotype versus the hemolytic anemia on determinants such as biliary bile acid composition and mucin secretory glands in the mouse gallbladder neck. Additional physical chemical studies of the interaction of unconjugated bilirubin in model bile solutions will be helpful in further delineating the pathogenesis of both black and brown pigment gallstones.
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PMID:Pigment gallstone disease. 202 17

The authors describe extensive strictures of the hepaticocholedochus, ranging from 2.5 cm in length to total involvement of the duct, in 15 patients with primary extrahepatic portal hypertension. In 7 patients they were combined with cystic dilatation of the intrahepatic bile ducts. All patients were under 40 year of age, the ages of 10 of them ranged for 18 to 30. There were 9 males. ++Non-operative treatment of the disturbed bile drainage proved ineffective: remittent jaundice was encountered in all cases. The most frequent complications were cholangitis (6 cases), biliary cirrhosis of the liver (3) and the formation of spontaneous biliary fistulas (2).
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PMID:[Lesions of the biliary system in primary extrahepatic portal hypertension]. 204 25

This article summarizes our present knowledge concerning drug-induced cholestasis by lesions of the bile ducts. Acute or chronic ductular and ductal cholestasis are induced by a few drugs. The acute form may mimic viral hepatitis or biliary tract obstruction. The outcome is generally good. The mechanism may be immunoallergic. The clinical presentation of the chronic form resembles that of primary biliary cirrhosis or sclerosing cholangitis. Its prognosis is generally good and characterized by a complete recovery. However, an evolution to biliary cirrhosis is sometimes possible. The mechanism could also be immunoallergic. Extrahepatic cholestasis is frequently observed after intra-arterial infusion of floxuridine. The prognosis is generally poor. The mechanism of this sclerosing cholangitis may be ischaemic.
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PMID:[Secondary drug-induced cholestasis with bile duct involvement]. 205 47

A case of ectasing cholangitis, following intra-arterial chemotherapy with discontinuous infusion of fluorouracile, is described. The severe destruction of the intra-hepatic biliary ducts led to the constitution of large cavities in continuity with the biliary tree. These cavities were responsible for images which were mistakenly thought to be metastases. Consequently, secondary biliary cirrhosis developed, requiring orthotopic liver transplantation.
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PMID:[Ectasing cholangitis and secondary biliary cirrhosis following intra-arterial hepatic chemotherapy. Treatment by liver transplantation]. 206 Jul 45


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