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)

The characteristics of the primary sclerosing cholangitis are a diffuse inflammation with fibrous thickening and narrowing of the biliar ducts. Two observations replying to strict characteristics are related. From attacks of angiocholitis, the disease generally progresses till a parmanent icterus and death occurs in a state of biliar cirrhosis. In the liver, the micro pathology consists in fibrous rings constricting the segmentary interlobular ducts. The diagnostic is mainly difficult and the prognostic is fatal in average time of 4 years of evolution althought we used corticoids that were administrated by local method as well as in the classical general way.
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PMID:[Surgical problems of the primary sclerosing cholangitis (author's transl)]. 60 51

The characteristics of primary sclerosing cholangitis are a diffuse inflammation with fibrous thickening and narrowing of the biliary ducts. All patients who have stones or have been operated on for biliary disease (risk of traumatic stenosis) must be excluded in discussions of this disease. Two observations illustrating the strict definition of its characteristics are related. From attacks of angiocholitis, the disease generally progresses to a permanent jaundice. Death occurs in a state of biliary cirrhosis. Liver histopathologic changes consist of fibrous rings constricting the segmentary interlobular ducts. Physiopathologic changes of the disease are difficult to define precisely except in cases with coexistent ulcerative colitis (1/3 of cases). The diagnosis is difficult. Illustrative examples are presented: A syndrome of stenosis in a patient in whom no stones were found during the operation but the lesions were typical of the passage of stones. The prognosis was good. One patient seemed rather typical but the rapid evolution and finally a finding of carcinomatous tissue led to the diagnosis of cancer of the biliary duct. One patient had cirrhosis with extrahepatic stenosis. Histologic studies, cholangiolitis and obstructive pericholangiolitis indicated the correct diagnosis. The disease is usually fatal within four years of onset despite administration of corticoids locally and systemically.
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PMID:Surgical problems in primary sclerosing cholangitis. 89 6

Sclerosing diseases of the biliary system encompass a spectrum ranging from primary sclerosing cholangitis (usually of the extrahepatic biliary tree) to primary biliary cirrhosis of the intrahepatic bile canaliculi. In a study of 35 patients with primary intra- and extrahepatic biliary sclerosis, age of onset, sex distribution, symptomatology, associated diseases, radiographic abnormalities and chemical profile were considered. The difficulty of differentiating sclerosing cholangitis and biliary cirrhosis from other causes of obstructive jaundice preoperatively was stressed, in addition to points of differential clinical and laboratory findings. The etiology of these entities as well as the possibility that they represent variant clinical manifestations of the same disease process were also considered. Mechanical and pharmacological treatment alternatives that were attempted included drainage procedures, the easiest and most widely used of which was the T-tube. However, this could prove to be a source of infection and should therefore be removed early, inasmuch as cholangitis represents a major cause of morbidity. Steroids have been used with varying effectiveness; subjective improvement was generally attained, although objective improvement has been difficult to document. When choleuretics and cholestyramine were administered, we noted significant palliation. Antibiotics were reserved for treatment of cholangitis. Until the underlying etiology of this rare malignant sclerosing process is found, only symptomatic treatment can be offered.
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PMID:Sclerosing cholangitis and primary biliary cirrhosis--a disease spectrum? 92 53

Nine cases of primary sclerosing cholangitis were reviewed as to methods of diagnosis, association with other disease states and clinical course of the patients. There were four cases of primary sclerosing cholangitis occurring alone, four cases associated with inflammatory bowel disease (three with chronic ulcerative colitis and one case with proctosigmoiditis) and one case associated with porphyria cutanea tarda. All cases of primary sclerosing cholangitis occurring alone, progressed to secondary biliary cirrhosis, however, none of the cases associated with chronic ulcerative colitis progressed to secondary biliary cirrhosis. In all cases, the diagnosis was established by operative findings and biopsy results. The mode of clinical presentation was similar in all cases and was characterized by slowly progressive jaundice. Intravenous and oral cholangiography were not useful in establishing a diagnosis but endoscopic retrograde cholangiography offers preoperative diagnostic hope and use for follow-up evaluation. One case with ulcerative colitis had a Strongyloides infection and the organism was found in the fibrotic duct and pericholedochal lymph nodes. The etiological considerations are reviewed and the classification of sclerosing cholangitis associated with ulcerative colitis, as primary, is discussed. Therapeutic modalities are discussed, though therapy is mainly empirical at present.
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PMID:Primary sclerosing cholangitis. A report of nine cases and clinical review. 127 36

Primary sclerosing cholangitis (PSC) is a disease of the bile ducts which frequently is associated with inflammatory bowel disease. The disease is characterized by stenoses and saccular dilatations of the intra- and/or extra-hepatic bile ducts. Endoscopic retrograde cholangiography is the mainstay of diagnosis of this disease. A liver biopsy gives additional information and is particularly useful to rule out secondary cirrhosis. In a series of 50 patients the 5-year survival was 85%. The etiology is unknown, but in addition to inflammatory bowel disease the disease is associated with the autoimmune haplotype HLA-A1, B8, and DR3. Antibodies to perinuclear antigens in leukocytes are present in 65% of patients with PSC. Ursodeoxycholic acid therapy causes an improvement of symptoms and a decrease of elevated liver enzyme values. There are no drugs as yet with a proven effect on fibrosis or cirrhosis in this disease. Liver transplantation in this disease has a reported 4-year survival of 88%.
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PMID:Primary sclerosing cholangitis. An unresolved enigma. 129 52

Homozygous alpha 1-antitrypsin deficiency (PiZZ phenotype) is known to be associated with increased risk of cirrhosis and primary liver cancer. Although a relationship between heterozygous alpha 1-antitrypsin deficiency and chronic liver disease was suggested recently, it is still a matter of controversy whether such patients are at increased risk of liver cancer. The goal of this study was to determine the prevalence of heterozygous alpha 1-antitrypsin deficiency of different phenotypes among patients with primary hepatobiliary cancers. We studied 82 patients with primary hepatobiliary cancer; 59 had hepatocellular carcinoma and 23 had bile duct carcinoma. alpha 1-Antitrypsin quantitation and phenotyping were performed in each patient using standard methods. The distribution of the various Pi phenotypes was compared with that found in a normal population and reported elsewhere. Odds-ratio and chi 2 tests were used to measure the relative risk and the significance of association, respectively, between primary hepatobiliary cancers and heterozygous alpha 1-antitrypsin deficiency. Four patients in each of the cancer groups were heterozygous. Among the hepatocellular carcinoma patients, three had the PiMS phenotype and one had the PiMZ phenotype. Of these four heterozygous patients, only two had cirrhosis; one had cryptogenic cirrhosis and the other had hepatitis B virus-related cirrhosis. One noncirrhotic patient with a PiMZ phenotype had a fibrolamellar carcinoma. Of the four patients with bile duct carcinoma, three had the PiMS phenotype and one had the PiMZ phenotype. Of the four heterozygous patients, two had primary sclerosing cholangitis without associated inflammatory bowel disease and one patient had had previous biliary operations.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Lack of increase in heterozygous alpha 1-antitrypsin deficiency phenotypes among patients with hepatocellular and bile duct carcinoma. 131 55

The immunohistochemical detection of the c-erbB-2 oncopeptide (p185erbB2) has been shown to be a valid marker for over-expression of this oncogene. To evaluate the possible relevance of gene expression to the proliferation of hepatocytes and bile ducts in human disease, the authors applied a monoclonal anti-p185 antibody to formalin-fixed, paraffin-embedded tissues from 67 examples of benign proliferative and neoplastic hepatic lesions and fetal liver. Focal membrane-based reactivity for the oncopeptide was detected on tumor cells in two of eight hepatocellular carcinomas and on tumor cells and adjacent bile ducts and hepatocytes in four of six cholangiocarcinomas. Each of the latter four lesions were in patients with primary sclerosing cholangitis. No reactivity was obtained in examples of hepatoblastoma, mixed cholangiocarcinoma-hepatocellular carcinoma, bile duct adenoma, or hepatocellular adenoma. Weak staining for p185erbB2 also was seen in two of seven cases of (sub)massive hepatic necrosis and two examples of postnecrotic cirrhosis, all of which were secondary to either hepatitis B or C virus infection. No other benign proliferative lesions were labeled by the anti-p185 antibody, including cases of chronic allograft rejection, necrosis secondary to hepatic artery thrombosis, metabolic-associated and nonmetabolic-associated cirrhosis, focal nodular hyperplasia, and nodular regenerative hyperplasia. The authors' results indicate that c-erbB-2 may be amplified in specific neoplastic and hepatitis B virus and hepatitis C virus infectious lesions of liver. The authors postulate that: (1) c-erbB-2 immunoreactivity may be a marker for malignant transformation in primary sclerosing cholangitis; and 2) overproduction of p185erbB2 may be an epiphenomenon of hepatitis B virus or hepatitis C virus infection.
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PMID:Immunoreactivity for c-erbB-2 oncopeptide in benign and malignant diseases of the liver. 137 19

To assess the role of the hepatitis C virus in patients with unexplained chronic liver disease, we tested for the presence of anti-hepatitis C antibody (anti-HCV) in the stored serum of patients with cryptogenic cirrhosis and a variety of other chronic liver diseases. The anti-HCV assay was performed by both the enzyme-linked and recombinant immunoblot methods in 16 patients with cryptogenic cirrhosis. Eight of these 16 patients (50%) were seropositive. Six of these eight patients were born outside of the United States, compared with only one of eight seronegative patients (p = 0.021). Of the anti-HCV-positive cryptogenic cirrhotic patients, 50% also had markers of previous hepatitis B infection, compared with only 12.5% of seronegative patients. Evidence of anti-HCV positivity was found in 10%, 19%, 0%, and 0% in patients with alcoholic cirrhosis, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis, respectively. We conclude that in a suburban American population, hepatitis C accounts for a significant percentage of patients with presumed cryptogenic cirrhosis. Unrecognized risk factors may account for a higher prevalence of HCV in foreign-born patients with cryptogenic cirrhosis. A low prevalence of anti-HCV positivity is found in other forms of chronic liver disease.
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PMID:Prevalence of anti-HCV in cryptogenic cirrhosis in a suburban Detroit community. 137 12

Ursodeoxycholic acid (UDCA) allows symptomatic treatment of cholestatic liver diseases such as primary biliary cirrhosis, primary sclerosing cholangitis, intrahepatic biliary atresia, and cholestasis of cystic fibrosis. Patients should be treated at an early stage of the disease in order to prevent progression to cirrhosis. Since UDCA has no toxic effects longterm treatment with this substance is possible without the risk of undesired side effects. In patients with primary biliary cirrhosis and rapid progression of the disease, UDCA may be combined with an immunosuppressive substance (i.e. cyclosporin). In primary sclerosing cholangitis, biliary atresia and cholestasis of cystic fibrosis, UDCA at present seems the only treatment of which a benefit for the patients can be expected. In endstage disease liver transplantation is indicated. The role of UDCA in chronic hepatitis and alcohol induced liver disease needs to be clarified in further studies. Whether the improvement of laboratory tests in such patients indicates amelioration of the course of disease, still is unclear.
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PMID:[Treatment of cholestatic liver diseases; the role of ursodeoxycholic acid]. 144 78

To assess the prevalence and significance of chronic ulcerative colitis in patients with severe autoimmune hepatitis and to determine the frequency of cholangiographic and histologic features of primary sclerosing cholangitis in those with colitis, 105 patients who had been screened by annual proctoscopic examination were studied. Patients with features of colitis were compared to counterparts without colitis who had been matched by age, sex, disease severity and treatment regimen. Seventeen patients (16%) had findings of chronic ulcerative colitis. Twelve of these underwent cholangiography and five (42%) had features of primary sclerosing cholangitis. Patients with and without cholangiographic abnormalities were indistinguishable by clinical, laboratory, immunoserologic, and histologic features. Fibrous obliterative cholangitis was present in only two patients, including one with normal cholangiography. Patients with colitis entered remission less frequently (59 vs. 94%, p less than 0.05), failed treatment more commonly (41 vs. 6%, p less than 0.05) and progressed to cirrhosis more frequently (75 vs. 25%, p less than 0.05) than counterparts without colitis. Patients with colitis but normal cholangiography, however, responded satisfactorily to therapy. We conclude that chronic ulcerative colitis can coexist with severe autoimmune hepatitis in the absence of primary sclerosing cholangitis or hepatitis C infection. Under such circumstances its presence does not adversely influence treatment outcome. Primary sclerosing cholangitis cannot be excluded by routine examinations and its presence is associated with a poor treatment response. Cholangiography should be considered in all patients with autoimmune hepatitis and colitis, especially in those recalcitrant to therapy.
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PMID:Frequency and significance of chronic ulcerative colitis in severe corticosteroid-treated autoimmune hepatitis. 150 Jun 96


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