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 porphyrin metabolism of 100 patients with porphyria and 351 of their relatives has been studied. Thin layer chromatography of methyl esters of the urinary porphyrin was undertaken in sixty-six patients with different types of porphyria, and forty-five relatives, seventeen patients with hepatic cirrhosis, three patients with lead poisoning and twenty normal control subjects. This investigation was also made on the stools of thirty-six patients with porphyria, and then of their relatives. Countercurrent analysis of the bile of nine selected patients with porphyria was also undertaken. The results provide some evidence that symptomatic hepatic porphyria may be familial. Thin layer chromatography was decisive in the characterization of a new type of porphyria described recently by the authors (hepato-erythrocytic porphyria). The counter-current examination of the bile showed the absence of the 'S 411' porphyrin in all the nine cases investigated.
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PMID:Thin layer chromatography and counter-current analysis in porphyrias. 119 36

Our investigations in 134 patients showed corresponding to literature porphyria cutanea tarda (PCT) diagnosed by biochemical methods not to be a paraneoplastic dermatosis (but one possible exception acquainted). Relations between PCT and extrahepatic non-porphyrin producing tumours are improbable. Nevertheless but extremely seldom an irregular urinary porphyrin excretion associated with cutaneous changes of hepatic porphyria should lead to the presumption of a porphyrin producing hepatoma. PCT lasting for decades apparently presents a higher frequency of hepatocellular carcinoma in patients suffering from liver cirrhosis than in cirrhotics without PCT. It is supposed that this possible progredience of liver disease in PCT into hepatocellular carcinoma may be prevented by chloroquine phosphate therapy.
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PMID:[Does paraneoplastic porphyria cutanea tarda exist?]. 337 Dec 36

In addition to a previous paper [7] a survey is given of acute viral hepatitis, drug-induced liver-disease, chronic hepatitis, cirrhosis of the liver, hyperbilirubinemia, hepatic porphyria, and obstructive jaundice as hepatobiliary diseases independent of pregnancy. Finally, some questions of treatment of pregnant women suffering from liver disease are stressed.
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PMID:[Liver diseases and pregnancy. II: Liver diseases without causal relation to pregnancy--general principles for treatment of patients with liver disease]. 393 79

Out of 147 patients with chronic hepatic diseases, chronic persistent hepatitis, chronic active hepatitis, hepatic cirrhosis, alcoholic lesions of the liver, biliary hepatic cirrhosis and Gilbert syndrome were registered in 26, 35, 27, 43, 8 and 8 patients, respectively. Urinary and fecal porphyrins were measured spectrophotometrically. Disturbances in porphyrin metabolism were diagnosed in 76 patients (51.7%). Four different biochemical syndromes were identified: 1) a symptomatic rise of fecal porphyrins only, 2) secondary coproporphyrinuria, 3) secondary coproporphyrinuria in combination with high fecal protoporphyrin, 4) biochemical syndrome of chronic latent hepatic porphyria. These syndromes were not strictly specific, but secondary coproporphyrinuria occurred significantly more often in chronic active hepatitis and biliary cirrhosis. High symptomatic fecal porphyrins were characteristic for alcoholic affections, and latent hepatic porphyria was indicative of hepatic cirrhosis. Disturbed porphyrin metabolism arises in more severe hepatic lesions and runs in association with more rapid development of hepatocellular insufficiency. Probable pathochemical mechanism and diagnostic value of the above impairment are discussed.
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PMID:[Porphyrin metabolism in chronic liver diseases]. 816 Jan 36

Alcohol has an porphyrinogenic action and can cause a disturbance of porphyrin metabolism in healthy people as well as lead to a biochemical and clinical manifestation of acute and chronic hepatic porphyrias, especially acute intermittent porphyria and porphyria cutanea tarda. After excessive consumption of alcohol a temporary, clinically asymptomatic secondary hepatic coproporphyrinuria in man can be observed, which can become persistent in cases of alcohol-induced liver damage. Nowadays alcohol-liver-porphyrinuria syndrome is the first to be mentioned in secondary hepatic disturbances of porphyrin metabolism. In people with a genetic lack of uroporphyrinogen-decarboxylase alcohol is able to transform an asymptomatic coproporphyrinuria into a chronic hepatic porphyria or porphyria cutanea tarda. From experimental and clinical studies the conclusion can be drawn that alcohol inhibits the enzymes delta-aminolevulinic-acid-dehydratase (synonym: porphobilinogen-synthase), uroporphyrinogen-decarboxylase and coproporphyrinogen-oxidase and induces delta-aminolevulinic-acid-synthase in the liver. Abstinence of alcohol is a therapeutically and prophylactically important measurement in all types of hepatic porphyrias. For clinical experience follows that in cases with chronic consumption of alcohol, fatty liver, alcohol induced hepatitis and liver cirrhosis porphyrin studies in urine should be made to notice a hepatic porphyria in the latent phase very early. When dealing with abdominal and cutaneous symptoms in clinical context with consumption of alcohol one has to exclude hepatic porphyria differential diagnostically.
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PMID:[Hepatic porphyrias and alcohol]. 1042 Jul 23

The incidence of hepatocellular carcinoma (HCC) shows marked variation worldwide but the magnitude of this tumor is reflected by the occurrence of at least 1 million new cases annually and the uniformly dismal outlook with median survivals of <25 months after resection and <6 months with symptomatic treatment. The strikingly uneven distribution of this tumor parallels the prevalence of hepatitis B infection with rising incidence in western countries attributed to hepatitis C infection. Chronic hepatitis and cirrhosis constitute the major preneoplastic conditions in the majority of HCCs and may be related to other etiologic agents such as environmental chemical carcinogens including nitrites, hydrocarbons, solvents, organochlorine pesticides, and the chemicals in processed foods, cleaning agents, cosmetics and pharmaceuticals, as well as plant toxins such as anatoxins produced by fungi that cause spoilage of grain and food in the tropics. Genetic diseases such as genetic hematochromatosis, Wilson's disease, alpha-1-antitrypsin deficiency, and the inborn errors of metabolism including hereditary tyrosinemia and hepatic porphyria, are known to be associated with HCC. Numerous genetic alterations and the modulation of DNA methylation are recognized in HCC and it is likely that these genetic and epigenetic changes combine with factors involved in chronic hepatocyte destruction and regeneration to result in neoplastic growth and multiple molecular pathways may be involved in the production of subsets of hepatocellular tumors.
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PMID:Epidemiology and carcinogenesis of hepatocellular carcinoma. 1833 56