Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The histological lesions found in patients with hepatic disease and positive anti-mitochondrial antibodies (AMA) by immunofluorescence were compared with those in 10 subjects of the same age and sex with negative AMA. The majority of the patients with positive AMA were suffering from primary biliary cirrhosis, the diagnosis being based upon the histological appearance (Stage 1 of Scheuer's classification: 40%) or the presence of clinical signs of cholestasis with a compatible histological appearance (40%). None of the remaining patients (20%) with positive AMA nor the 10 individuals with negative AMA showed histological signs suggestive of primary biliary cirrhosis. The absence of certain clinico-biological factors, with the exception of Stage 1 histology, gives a high degree of value to a high level of AMA in the diagnostic of PBC.
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PMID:[Diagnostic value of anti-mitochondria antibodies]. 84 Jun 32

Twenty-two patients with clinical, biochemical, immunological and pathological characteristics compatible with primary biliary cirrhosis were studied. There were 17 women and 5 men with a mean age of 57.4 +/- 15.2 years and a mean follow-up of 24.1 +/- 20.1 months. Four of them expired during the follow-up and eighteen patients now survive. The most common complaints were fatigue (63.6%) and itching (59.1%). Only one case (4.5%) was asymptomatic in this series. The major physical findings were jaundice (50%) and hepatomegaly (50%). The significant laboratory findings were: elevation of alkaline phosphatase (91% of the cases greater than 3 times the upper limit of normal), gamma-glutamyl transpeptidase (100% of the cases greater than 4 times the upper limit of normal), aspartate transaminase (95%) and alanine transaminase (100%), presence of anti-mitochondrial antibodies (91%), antinuclear antibodies (73%) and the elevation of IgM (88%). One case was associated with ulcerative colitis. Pathological staging in this series revealed 57.9% of stage II, 26% of stage III, 10% of stage IV and 5.3% of stage I. All patients with granuloma survived but 4 of the 5 patients with cholestasis died during follow-up. The results show that the features in this series of PBC were similar to those observed in western countries. The very high ALP and gamma-GT level as well as only one asymptomatic case in this series, suggest that our patients were diagnosed at a late stage. The reason(s) for the higher positivity of ANA, particularly the speckled type and a lower rate of associated auto-immune disease requires further study. Liver biopsy in predicting a prognosis is valuable.
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PMID:[A clinicopathological study in primary biliary cirrhosis]. 135 58

40 women, average age 52.5 years, with varying stages of primary biliary cirrhosis, were observed. One third of them suffered from a mild anaemia, mean plasma concentrations of ALAT were increased four times and those of AP six times. Despite the hepatocellular damage products of the liver synthesis such as transport proteins or coagulation factors were found to be normal or enhanced. 60% of the patients had a hypercholesterolaemia. The risk factors low density lipoprotein (LDL)- and very low density lipoprotein (VLDL)-cholesterol showed normal levels, but the protective factor high density lipoprotein (HDL)-cholesterol was clearly increased. Apart from the low blood pressure in most of the patients and the absence of other risk factors these observations explain, why patients with PBC and hypercholesterolaemia don't usually develop arteriosclerotic complications. Only in case of severe cholestasis a lipid constellation comes into being accompanied by high risk for the blood vessels, but in these cases the terminal stage of PBC limits the survival. Positive correlations between markers of cholestasis and lipid parameters let an enhanced production and simultaneous impaired excretion of cholesterol be assumed.
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PMID:[Lipid metabolism disorders in primary biliary cirrhosis (PBC)]. 191 Apr 90

The paucity of the intrahepatic bile ducts, also known as ductopenia, is a well recognized disorder in pediatric patients. Recently, however, a similar disorder has been reported in adults and termed idiopathic adulthood ductopenia (IAD). We describe a 30-year-old patient with a 15 year history of episodes of jaundice. During icteric episodes, serum levels of bilirubin and alkaline phosphatase were markedly elevated. Between attacks, totalling more than 30, the patient was asymptomatic, but bilirubin and alkaline phosphatase levels were mildly elevated. No neonatal jaundice was present in the patient's history. PBC, PSC and drug-induced cholestasis were excluded. Two needle biopsies of the liver, taken within a 13 year interval, were available. The lobular architecture appeared progressively disturbed by porto-centro-portal bridging septa. In both biopsies, a destructive cholangitis was found. In the last biopsy, the majority of the septal and interlobular ducts appeared severely damaged and, in three out of seven portal tracts, the interlobular bile duct had disappeared. In the parenchyma, the main feature was a severe mainly canalicular bilirubinostasis. The patient described illustrates that IAD may have a clinical picture indistinguishable from benign recurrent intrahepatic cholestasis. The etiology of the disease, in this as in other patients, remains unknown.
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PMID:Idiopathic adulthood ductopenia presenting with chronic recurrent cholestasis. A case report. 200 71

Primary biliary cirrhosis is a chronic liver disease of unknown etiology characterized by slowly progressive intrahepatic cholestasis due to an inflammatory destruction of small intrahepatic bile ducts. The clinical course of PBC is variable ranging from a few years in rapidly progressive cases to a normal life-expectancy in a proportion of asymptomatic cases. The typical patient is a middle-aged woman who may present with pruritus, increasing pigmentation of the skin, and eventually jaundice. The level of serum alkaline phosphatase is almost invariably elevated, serum mitochondrial antibodies are present in more than 90 per cent, and an elevated serum IgM is usually present. PBC is associated with many immunologic abnormalities and appears to be a classic autoimmune disease. Some of the immune defects may be epiphenomena; others such as a marked defect in suppressor T cell function seem to be related to the pathogenesis of the disease. All drug therapy that is aimed at slowing the disease process is experimental. A place for immunosuppressive drugs in the management of PBC would be anticipated. However, no drug has to date been definitively shown to have a beneficial effect on the disease. Currently, the main treatments used are aimed at preventing or correcting the complications of intractable cholestasis. Patients with PBC and evidence of hepatic decompensation and/or poor quality of life make good candidates for liver transplantation. The current aim of therapy is to find an effective regime of immunosuppression that will make hepatic transplantation redundant for this disease.
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PMID:Primary biliary cirrhosis. 265 67

Thirty-one patients with primary biliary cirrhosis were observed in Picardy during the decade 1975-1984. Mean annual incidence was 2.6 per 10(6), and point prevalence, 13 per 10(6) inhabitants in 1984. Asymptomatic cases (without clinical manifestations of cholestasis) constituted 32% of all patients. In five of the 21 symptomatic patients, ascites and/or digestive hemorrhage due to ruptured esophageal varices revealed the disease. Nine patients (2 with initially asymptomatic PBC) died during the follow-up period. Cholelithiasis was present in 10 patients (32%) and one case of hepatocellular carcinoma was found at autopsy.
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PMID:[Asymptomatic forms of primary biliary cirrhosis seen in 10 years in Picardie]. 294 18

While cholestatic jaundice usually develops in the advanced stages of primary biliary cirrhosis, our series of this disease disclosed that 8 of the 88 cases presented cholestatic jaundice in the early histologic stages (stages 1 and 2). These patients frequently presented esophageal varices (57%) and showed a low incidence of positive mitochondrial antibodies compared to the non-jaundices cases in the same histologic stages. Histologic changes of livers from the jaundiced patients failed to show fundamental differences from those of the non-jaundiced patients, except for the presence of bile plugs and extensive bile duct loss in the former. Deposition of orcein-positive granules and increased hepatic copper content, suggesting prolonged cholestasis, were rather advanced, and inflammatory changes in the portal tracts and piecemeal necrosis were rather mild in the jaundiced patients. These data suggest that cholestatic jaundice occurs in a few patients in the early histologic stages on a biopsy with extensive bile duct loss and features of prolonged cholestasis; it is not clear whether such patients are a small separate cluster in PBC, or not.
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PMID:Pathologic study of primary biliary cirrhosis of early histologic stages presenting cholestatic jaundice. 321 71

Acute hepatitis A is diagnosed by IgM-anti-HAV antibodies. Cytotoxic immune reactions seem to play a key role in the pathogenesis of hepatitis A, which is an acute self-limited disease. - Acute hepatitis B is diagnosed by IgM-anti-HBc antibodies and can thus be differentiated from other HBV-associated liver diseases. The detection of HBV-DNA in serum serves to differentiate between HBsAg/anti-HBe positive patients, with active viral replication and progressive chronic liver disease, and HBV-DNA negative asymptomatic HBsAg-carriers usually with normal liver histology. In the pathogenesis of HBV-associated liver diseases cytotoxic immune reactions against virus-infected hepatocytes are thought to mediate liver cell destruction. Membrane expressed virus as well as host antigens are candidate target antigens for these immune reactions. Hepatitis delta is always associated with acute and chronic HBV infection. Acute hepatitis delta is diagnosed by delta-RNA in serum and later by antibodies against the delta virus. Little is known about the pathogenesis of delta virus infection. Autoimmune type chronic active hepatitis (CAH) is classified into classical autoimmune type "lupoid CAH" with antinuclear antibodies and liver membrane antibodies as markers, liver-kidney-microsomal (LKM) antibody positive CAH, CAH associated with autoantibodies against a soluble liver antigen (SLA), and CAH associated with high titers of anti-smooth muscle antibodies (SMA). In the pathogenesis of autoimmune type CAH an antibody-mediated cellular cytotoxicity (ADCC) may play a significant role. Primary biliary cirrhosis is to be differentiated as a clinical syndrome associated with cholestasis and anti-mitochondrial antibodies (AMA). By immunoblotting and radioimmunoassay at least two PBC-specific subtypes of AMA can be defined. Immune reactions against self-antigens are thought to be involved in the pathogenesis of PBC, although the precise reactions are unknown.
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PMID:[Immunology of liver diseases]. 331 82

In PBC, common features beyond cholestasis and presence of mitochondrial antibodies are signs of complement activation and high levels if IgM. In order to characterize this IgM physicochemically and immunologically, we studied IgM from 15 patients with PBC in comparison with that from patients with high levels of polyclonal IgM without signs of liver disease (idiopathic hyper-IgM-emia) and normals. Agarose electrophoresis, immunofixation, gel filtration, and ultracentrifugation studies gave no evidence of any abnormal IgM populations such as complexes, aggregates, 7S IgM, or oligoclonality. However, IgM in PBC is highly cryoprecipitable, precipitable with 2.5% PEG (mol. wt. 6000) and binds to conglutinin. In addition, purified IgM from PBC patients rapidly converts complement factor C3 in fresh normal serum, mainly via the classical pathway, in contrast to IgM in the same concentration from normals or patients with idiopathic hyper-IgM-emia. IgM from PBC patients behaves like an immune complex, although it has the same molecular size and electrophoretic properties as normal IgM. No evidence of any antigen(s) bound to IgM in vivo in PBC was found in this study.
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PMID:IgM in primary biliary cirrhosis. Physicochemical and complement activating properties. 706 68

Liver transplantation is the therapy of choice in the final stage of PBC, PSC and biliary atresia. UDCA leads to remission of the symptoms and the clinic in the early stages not only of these diseases but of cholestasis of pregnancy and of cholestasis in cystic fibrosis as well. In benign intermittent cholestasis only phenobarbital but not UDCA seems to be efficacious. The efficacy of UDCA in chronic active hepatitis and alcoholic hepatitis is not yet guaranteed.
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PMID:[Treatment of cholestatic liver diseases with phenobarbital and ursodeoxycholic acid]. 748 96


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