Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over a period of 18 months the development of hepatitis after intake of oxyphenisatin, a laxative, was established in 14 patients by re-exposure to the drug. The characteristic feature was nonspecific upper abdominal pain up to colic-like pain, lact of appetite, nausea or vomiting, and pruritus. The biochemical changes were those of chronic hepatitis with varying severity of biliary stasis and abnormal immunofluorescence. On re-exposure there was a particularly remarkable rise in GLDH activity. The histological picture showed acute inflammatory changes in the biliary passages on re-exposure, while the liver cells were clearly involved only secondarily. At a latter point the histological picture became non-specific. At laparoscopy there were different stages of minor periportal hepatic fibrosis to marked postnecrotic liver scars with portal hypertension and decompensation. Early diagnosis is difficult but crucial to the patient's fate, because this form of hepatitis regresses completely after oxyphenisatin has been stopped. Laxatives containing this drug should be withdrawn from the market.
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PMID:[Oxyphenisatin-induced liver disease (author's transl)]. 12 99

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

Among 424 children with liver disease, 20 had alpha1-antitrypsin deficiency associated with protease inhibitor ZZ phenotype. This disorder manifested itself as cholestasis in early infancy in 19 children. Jaundice and pruritus cleared in 16 of these by 7 months of age, but hepatomegaly and laboratory evidence of mild hepatic dysfunction persisted in all. Biliary cirrhosis and portal hypertension eventually developed or was suspected in eight, and hypoplasia of intraheptic bile ducts was demonstrated in another four. Routine screening revealed intermediate alpha1-antitrypsin deficiency in 16 other children with various types of liver disease. The phenotype in these patients was MZ, MS, or SZ. PAS-positive granules were present in liver of all patients with the ZZ phenotype and in none with other phenotypes. The findings indicate that manifestations and prognosis of this inherited liver disease are extremely variable.
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PMID:Alpha1-antitrypsin deficiency and liver disease in children: phenotypes, manifestations, and prognosis. 108 71

Tests for cell-mediated immunity and presence of autoantibodies were performed in a mother and daughter with primary biliary cirrhosis. Lymphocytes transformation to phytohemagglutinin, delayed cutaneous response to one or more skin test antigens, and percentage of peripheral T and B lymphocytes were normal in both patients. Although successful in the mother, dinitrochlorbenzene sensitization was not achieved in the daughter. Histocompatibility antigens 1 and 8, elevated levels of IgM, and antibodies to mitochondria, smooth muscle, and skeletal muscle were present in both patients. However, the clinical course was more severe in the daughter who developed portal hypertension with bleeding esophageal varices requiring portacaval anastomosis. Except for intermittent pruritus, the mother has remained asymptomatic.
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PMID:Immunological studies in familial primary biliary cirrhosis. 108 42

Portal hypertension has been regarded as an uncommon and late complication of primary biliary cirrhosis (PBC). 24 patients with PBC were investigated for portal hypertension. Esophageal varices were present in 20, 50, and 90% of the patients 1, 3, and 9 years, respectively, after the onset of pruritus and/or jaundice. Portal hypertension was responsible for gastrointestinal bleedings in 11 patients; bleeding was the first clinical manifestation of PBC in two of them. Wedged hepatic venous pressure was increased in all the patients with portal hypertension whether regenerative nodules were present or absent. Portacaval shunt was performed in five patients and was well tolerated in three of them. It is concluded that (a) portal hypertension is common in PBC; (b) the intrahepatic block is of the so-called postsinusoidal type, even in patients without regenerative nodules; (c) gastro-intestinal bleeding due to portal hypertension occurs in about half of the patients and may be the first manifestation of PBC; (d) portacaval shunt seems to be indicated when gastro-intestinal bleeding occurs in earlier stage of the disease.
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PMID:Portal hypertension and primary biliary cirrhosis. 108 67

Selective hepatic arteriography and umbilical portography in a patient with idiopathic sclerosing cholangitis revealed uneven distribution of blood flow through the liver, the majority of the contrast media being demonstrated in the central portions of the liver. Mild portal hypertension was present. The stenotic biliary tree offered no unusual resistance to the passage of dye from the main biliary radicals into the duodenum. T tube drainage dramatically relieved pruritus, but clinical improvement with weight gain, disappearance of jaundice and reduction of portal pressure did not occur until after the administration of steroids. After one year of steroid therapy, liver biopsy showed a reduction in the periportal inflammatory reaction and lessening of the diffuse stenosis of the biliary tree. The relationship of these findings to current pathophysiologic hypotheses concerning sclerosing cholangitis is discussed.
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PMID:Idiopathic sclerosing cholangitis. Pathophysiologic observations. 115 21

Ursodeoxycholic acid (UDCA) was administered to 10 patients diagnosed as having primary biliary cirrhosis (PBC) after liver biopsy. Eight patients were anicteric, and two were icteric cases. One patient was in stage I, seven were in stage II, one in stage I-III, and one in stage III-IV of Scheuer's classification. Six hundred milligrams of UDCA were administered orally after meals three times daily to all of the patients for more than 1 yr. The period of UDCA administration ranged from 6 to 41 months. The major findings are as follows: 1) in six out of seven patients with pruritus, itching disappeared 1 month after administration of UDCA; 2) both serum alkaline phosphatase and gamma-glutamyltranspeptidase levels began decreasing significantly the first month after the onset of UDCA treatment, and continued decreasing throughout the treatment; 3) GOT and GPT levels also decreased significantly during the administration of UDCA, compared with before-treatment levels; 4) in one icteric patient with portal hypertension, although serum biliary enzyme levels improved after treatment, serum bilirubin level got worse, and the patient died of esophageal variceal hemorrhage. In another icteric case, biliary and bilirubin levels improved slightly after treatment; 5) antimitochondrial antibody titer decreased in four cases, but IgM levels and other immunological parameters were not changed; 6) serum UDCA increased significantly during UDCA treatment; in particular, glyco-UDCA occupied up to 40% of the total bile acid and CDC decreased to 25%; 7) portal inflammation activity decreased in all five patients who had undergone follow-up liver biopsy, more than 1 yr after UDCA administration--bridging fibrosis decreased in three cases; and 8) no side effects were observed in any of the cases. Although large-scale, randomized, controlled, double-blind tests are necessary, it is speculated that the long-term administration of UDCA is a safe and effective treatment for the improvement of biliary enzyme levels and pruritus in anicteric PBC.
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PMID:Improvement of biliary enzyme levels and itching as a result of long-term administration of ursodeoxycholic acid in primary biliary cirrhosis. 196 12

Primary biliary cirrhosis is a chronic cholestatic disease which usually affects middle-aged women and is characterized by portal vein inflammation and by segmental and focal necrosis of small intrahepatic bile ducts. The prevalence of the disease is estimated at 8 to 12 cases for 100,000 inhabitants. Genetic, infectious and/or immunological factors acting together may be responsible for small bile duct destruction. The main consequence of this destruction is cholestasis. As in all types of mechanical cholestasis, so-called lobular lesions such a fibrosis or even cirrhosis may then develop. Clinically, primary biliary cirrhosis evolves in three phases: (1) a preclinical asymptomatic phase where the disease is revealed by the accidental discovery of antimitochondrial antibodies or of a moderate rise in gammaglutamyltranspeptidase or serum alkaline phosphatase activity; (2) a clinical phase, usually lasting 5 to 10 years, characterized by fatigue, pruritus and later, clinical signs directly related to the hepatic lesions; (3) a terminal phase marked by major cholestasis with lesions of fibrosis or cirrhosis and sometimes ascites and portal hypertension responsible for gastrointestinal haemorrhages. In the last few years the prognosis of primary biliary cirrhosis has been considerably improved by the introduction and development of liver transplantation (the first choice treatment in the terminal phase) and by the introduction of ursodeoxycholic acid.
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PMID:[Primary biliary cirrhosis]. 206 16

Primary biliary cirrhosis is a progressive disease of the liver characterized by the immunologic destruction of bile ducts; effective therapy is lacking. We therefore evaluated the safety and efficacy of low-dose cyclosporine in 29 patients with primary biliary cirrhosis without evidence of damage to the lobular architecture (precirrhotic disease) or portal hypertension. The patients were randomly assigned to receive either cyclosporine (4 mg per kilogram of body weight per day) or placebo. After one year 17 of the 19 patients assigned to cyclosporine had improvement or stability in their degree of fatigue, and 18 in their degree of pruritus. In contrast, among the 10 patients assigned to placebo, fatigue increased in 4 (P less than 0.06) and pruritus worsened in 6 (P less than 0.001). Those assigned to cyclosporine also had significant decreases in serum levels of bilirubin, alanine aminotransferase, alkaline phosphatase, gamma globulin, and the titer of antimitochondrial antibodies. For the 20 patients who have completed two years in the study, liver biopsies (coded specimens) showed evidence of histologic progression in only 1 of 13 patients in the cyclosporine group, as compared with 5 of 7 in the placebo group (P less than 0.003). No patient has permanently discontinued cyclosporine because of side effects; however, signs of nephrotoxicity developed in 12 of 19, and 9 of 19 had increased blood pressure. We conclude that in patients with precirrhotic primary biliary cirrhosis, immunosuppressive therapy with cyclosporine is promising and deserves further evaluation.
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PMID:A controlled trial of cyclosporine in the treatment of primary biliary cirrhosis. 221 26

Four women aged 35 to 57 years (m: 49.2) with primary biliary cirrhosis and intractable pruritus had an estimated median survival time according to Christensen between 6 and 50 months (m: 27). They were enrolled in a therapeutic trial associating plasma exchange (PE) and immunosuppressive drugs. During the first 2 months, they received prednisone 15 mg/day, cyclophosphamide 2 mg/kg BW/day and 28 PE (60 ml/kg BW). Pruritus disappeared rapidly. After 2 months, mean levels of bilirubin, alkaline phosphatases, IgM and anti-mitochondrial antibodies dropped by 27 p. 100, 47 p. 100, 50 p. 100 and 85 p. 100 respectively, whereas amino-transferase and gamma-glutamyl-transpeptidase activities were unaltered. Two patients then received supportive therapy only: one was lost to follow-up after one year, the other died 50 months later from liver failure. The third patient received PE only when intractable pruritus reappeared: anti-mitochondrial antibodies, IgM and alkaline phosphatases remained below initial values for 38 months, until successful liver transplantation was performed. The fourth patient was treated on a long term basis by PE twice a month, prednisone 10 mg/day for 3 years and cyclophosphamide 1 mg/kgBW/day for one year only. Her initial estimated survival time was 6 months, but 3 years later she developed portal hypertension; anti-mitochondrial antibody titer was between 0 and 1/80, alkaline phosphatase levels reduced by 80 p. 100 to 70 p. 100; bilirubin level up by 50 p. 100. The mean survival time for the 4 patients exceeded 34 months, results better than those obtained with other kinds of therapy.
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PMID:[Primary biliary cirrhosis: therapeutic trial using plasma exchange and immunosuppression. Preliminary results]. 324 87


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