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)

Obstructive jaundice, pruritus, and malabsorption developed in twin brothers in infancy. Early liver biopsy specimens showed intracellular and canalicular cholestasis with normal bile ducts. By the age of 3 years, both had cirrhosis and portal hypertension. Each died during the teen years from hepatocellular carcinoma. These brothers represent the tenth reported family with familial cholestatic cirrhosis, and they are the first patients with this syndrome in whom hepatoma developed.
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PMID:Hepatoma in familial cholestatic cirrhosis of childhood: its occurrence in twin brothers. 21 1

In the last decade, the primary, biliary liver cirrhosis was diagnosed in 17 female patients aged between 33 and 72 years. The most frequent complaint were itching and jaundice. Hepatomegaly and itching predominated in the clinical signs Laboratory tests have shown and increase in alkaline phosphatase activity, gamma-glutamyltranspeptidase, and alanine-aminotransferase activities, accelerated ESR and decrease in blood serum albumins. Immunological abnormalities were found in 15 patients, including 12 with antimitochondrial antibodies. Liver biopsy was carried out in all patients enabling to diagnose the primary cirrhosis in 14 of them. Duration of the disease was between 1 and 9 years. Immunosuppressive treatment was carried out in 10 patients, and symptomatic treatment in the remaining 7 patients. No difference in the effect of therapy on actual health state of patients was seen.
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PMID:[Primary biliary liver cirrhosis in patients treated at Szczecin hospitals in 1978-1988]. 166 45

The Pitt-UNOS Liver Transplant Registry maintains a computerized database of all orthotopic liver transplantations (OLTX) performed in the United States since October 1, 1987. Recipient data, which are collected at the time of transplantation and at specified follow-up time periods, are reported to the Registry directly, while donor data are transmitted through UNOS Central. During the first year of registration (October 1, 1987 through September 30, 1988), 1,561 liver transplantations were performed on 1,377 patients; 146 patients received 2 livers and another 19 patients received 3 livers. There were 29 multiorgan transplants, including 18 liver/kidney, 7 liver/pancreas, 1 liver/small bowel, 2 liver/kidney/heart, and 1 liver/kidney/pancreas. Among the 52 centers reporting at least 1 liver transplant during this time period, 34 (65%) performed less than 20 procedures and only 7 (13%) performed at least 50 transplantations. Sixty-three percent of the donors were male; 63% were less than 25 years of age and in 72% the cause of death was motor vehicle accidents, gunshot wounds, or cerebrovascular accidents. Recipients were older than donors, 37% above age 45. About 25% of the recipients required intensive care or life support. The majority suffered from cirrhosis of cholestatic, autoimmune, viral, alcoholic, or unspecified etiology. The 6-month cumulative survival following OLTX was 76%, and the 6-month retransplant-free survival rate was 69%. Survival rates at 1 year were 72% and 64%, respectively. Factors associated with patient survival were diagnosis of liver disease and work (UNOS) status. Patients with fulminant liver failure or malignancies had the poorest survival, and patients with cholestatic cirrhosis or other cirrhosis had the best.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pitt-UNOS Liver Transplant Registry. 248 29

To determine whether portal lymphadenopathy in primary biliary cirrhosis is caused by deposition of lipofuscin pigment in sinus histiocytes and to compare primary biliary cirrhosis with other liver diseases a retrospective study on a consecutive series of 169 livers obtained at transplantation was carried out. There were grouped into eight diagnostic categories: primary biliary cirrhosis (n = 51), primary sclerosing cholangitis (n = 10), extrahepatic biliary atresia (n = 6), chronic rejection (n = 9), cirrhosis (other causes) (n = 38), primary liver neoplasia (n = 21), acute liver disease (n = 20), and retransplantation (other) (n = 14). Lymph nodes were present in 66 specimens. Fifty of these contained granules of lipofuscin pigment. The highest incidence of lymph node enlargement and the largest amounts of pigment were present in cases of primary biliary cirrhosis. A similar pattern of lymph node enlargement was also commonly observed in other chronic cholestatic conditions (primary sclerosing cholangitis, biliary atresia, chronic rejection). Much less pigment was seen in nodes draining livers with non-cholestatic cirrhosis or primary tumours. Nodes were not found in acute liver disease. It is concluded that portal lymphadenopathy associated with lipofuscin is a common finding in various chronic cholestatic liver diseases. The pathogenesis of this lesion is uncertain. Most cases are asymptomatic with enlarged nodes which may be detected only at laperotomy or necropsy and may be wrongly attributed to neoplastic disease. Diagnostically, the finding of large amounts of lipofuscin in enlarged portal lymph nodes is a good indicator of underlying chronic cholestatic liver disease.
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PMID:Portal lymphadenopathy associated with lipofuscin in chronic cholestatic liver disease. 258 29

Liver cirrhosis is relatively rare in children as compared to adults; frequently it is diagnosed too late. Biliary cirrhosis of early childhood is often the result of neonatal cholestatic syndromes. Beyond infancy, cirrhosis as a consequence of chronic active autoimmune hepatitis or of Wilson's disease may be prevented, if causal therapy is begun in time. Hence paediatricians should exclude both diseases in all children with elevated transaminases and clinical features of a liver disorder.
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PMID:[Liver cirrhosis in childhood--etiology, diagnosis and conservative therapy]. 353 72

A model of long-term cholestasis with the following recovery of intestinal bile outflow was developed on rats. In two experimental series choledochoduodenostomy was applied after 10- and 20-day cholestasis. It was established that in 10-day cholestasis perilobular hepatic fibrosis developed and bile outflow recovery in this period caused complete normalization of the liver structure a month later. 20-day cholestasis produced absolute parenchymal and stromal disorganization and the onset of biliary liver cirrhosis. Bile outflow recovery in such animals normalized only parenchymal, but not stromal structure; sclerotic processes were seldom reversed. Thus, the above cholestasis model precipitates the onset of liver cirrhosis and the technique of intestinal bile outflow recovery makes it possible to study the reversibility of pathologic hepatic changes.
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PMID:[Sclerotic changes in the liver in experimental cholestasis and their reversibility following restoration of bile outflow]. 373 May 76

The clinical value of measuring biliary acids in various chronic liver disease was investigated. The sample examined included 17 healthy subjects, 16 patients with active chronic hepatitis, 15 with cirrhosis of the liver and 14 with cholestatic cirrhosis. The following parameters were considered in each patient: blood bilirubin, gamma GT, alkaline phosphatase, cholinesterase, blood cholesterol, Quick time. The total pool of biliary acids was assayed by the enzymatic method on samples taken in the morning before breakfast and two hours after intake of 600 mg ursodeoxycholic acid. Total biliary increased with the progression of the pathological condition. Unlike all other indicators biliary acid assays after oral loading with ursodeoxycholic acid makes it possible to distinguish between subjects with active chronic hepatitis and those with cirrhosis of the liver.
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PMID:[Bile acids in chronic hepatopathies]. 380 85

Two siblings with intrahepatic cholestatic cirrhosis and their brother, who had a potentially related disease at the time of accidental death, are presented. The onset of disease occurred during adolescence in all 3 cases. The initial sign was mild jaundice or portal hypertension. There was no abnormality in the countenance, cardiovascular system, or vertebral column. Except for the brother who died from an accident, jaundice gradually increased. Death followed due to cirrhosis. Liver biopsy specimens of these 2 patients showed diminution of interlobular bile ducts with no significant cholangitis. At autopsy, the livers of the 2 patients showed biliary cirrhosis without extrahepatic biliary obstruction. In both cases there was an accessory lobe on the right hepatic lobe. Histologically, septal bile ducts showed pronounced papillary proliferations of the epithelium; there was also a decrease in the number of small interlobular bile ducts. Excess copper accumulation in the liver was ascertained. It is suggested that the disease in the 2 autopsied cases is intrahepatic cholestatic cirrhosis due to hypoplasia of the intrahepatic biliary trees.
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PMID:Familial intrahepatic cholestatic cirrhosis in young adults. 400 5

The ferrochelatase deficiency in protoporphyria leads to accumulation of protoporphyrin in erythrocytes and liver. Consequences are protoporphyrinemia with photosensitivity and liver damage (fibrosis, cirrhosis) with cholestasis. The latter are unpredictable and can be observed in about 10% of the patients. Protoporphyrin, the physiological main component of hepatocellular porphyrins, has a hepatotoxic effect in the high-concentrated crystalline storage form. The obligatory hepatobiliary excretion of the lipophil, erythropoietic increased accumulating protoporphyrin in protoporphyria strains the excretory function of the liver. Its restriction is followed by an exzessive protoporphyrin accumulation, which leads to protoporphyrin-induced, progressive cholestatic cirrhosis, icterus, and aggravation of the extrahepatic protoporphyrinemic cutaneous manifestation. In case of hepatobiliary complications a coproporphyria of diagnostic relevance develops with inversion of isomers. Simultaneously, the fecal protoporphyrin excretion decreases. After liver transplantation hyperbilirubinemia, protoporphyrinemia and coproporphyrinuria significantly went down. A protoporphyrinemia of about 20% of preoperative values reflects the persisting hereditary enzyme defect and the continuity of the metabolic disease.
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PMID:[Cholestatic erythrohepatic protoporphyria: porphyrin metabolism before and after liver transplantation]. 748 27

The pathogenesis of the vasodilatation associated with liver cirrhosis is not fully understood, but it has recently been postulated that it may be related to an increase in nitric oxide production. The aim of this study was to compare the response of isolated aortic rings from normal and cirrhotic rats to two vasoconstrictors, phenylephrine and U46619, a thromboxane analogue. Biliary cirrhosis was induced by ligation of the common bile duct; a sham operation was performed in control animals. Five weeks later, the aorta was removed and dissected into rings for study in organ chambers. Concentration-response curves were obtained for the two vasoconstrictors from rings with intact endothelium and from rings denuded of endothelium. We found that the vasoconstriction produced by phenylephrine was decreased in cirrhotic vessels both with and without endothelium, but the response to U46619 was not modified by cirrhosis. Concentration-response curves for phenylephrine were also obtained from rings in which the synthesis of nitric oxide and prostaglandins was inhibited by NG-monomethyl-L-arginine and indomethacin, respectively. Nitric oxide synthase inhibition restored normal contractility of the rings with and without endothelium. This beneficial effect was not observed when cyclo-oxygenase activity was blocked with indomethacin. This study suggests that cirrhotic vessels are hyporeactive to vasoconstrictors and that this effect is mediated through increased nitric oxide production. The improvement observed after inhibition of the nitric oxide pathway in denuded rings led us to suggest that cirrhosis also induces nitric oxide synthase in smooth muscle cells, as previously observed by others in septic animals.
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PMID:The endothelial and non-endothelial mechanism responsible for attenuated vasoconstriction in cirrhotic rats. 757


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