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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

19 Indian children with liver disease were studied. 5 in whom a clinical and histological diagnosis of Indian Childhood Cirrhosis was made had massive orcein-staining deposits in liver cells. The hepatic copper content in these 5 cases was strikingly high (1389 microgram/g dry tissue, range 1045--2303) the normal range being 15--55 microgram/g. Of the other 14 cases, only 2 had hepatic copper levels above normal (170 and 262 microgram/g.) This high hepatic copper concentration may be caused by excessive copper ingestion or an abnormality of copper metabolism.
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PMID:Increased hepatic copper concentration in Indian childhood cirrhosis. 8 75

Morphological, histochemical, and chemical study of three necropsy specimens of liver in the terminal stage of Indian Childhood Cirrhosis revealed a strikingly high copper content. it is proposed that excess accumulation of copper in the cytoplasm of hepatocytes disturbs the microtubular system, causing hydropic swelling and the formation of Mallory's hyalin. Copper and copper-binding protein showed topographical association with Mallory's hyalin. Diffuse cytoplasmic staining and the lysosomal copper distribution also suggested that copper had a cytotoxic effect. The pattern of copper distribution in Indian Childhood Cirrhosis differs from that in Wilson's disease and in prolonged cholestasis with excessive hepatic copper deposition, indicating a different mechanism of the copper accumulation.
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PMID:Cytoplasmic copper and its toxic effects. Studies in Indian childhood cirrhosis. 8 76

A histochemical study was carried out on orcein-positive hepatocellular material in 111 cases, 27 of which were stained positively by orcein. Orcein-positive material was very frequently found in protracted viral hepatitis and in chronic active hepatitis, as well as in other liver diseases with or without cholestasis; it was absent in liver cirrhosis. In all the cases considered the orcein-positive material was mainly formed of proteins, rich in amino acids with sulfhydryl and disulphide radicals, tryptophan and, to a lesser extent, of NH2 alpha amino acids. The orcein-positive substance was apparently carbohydrate-free. The presence of copper was also confirmed.
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PMID:Histochemical study of orcein-positive hepatocellular material in paraffin sections of liver biopsy samples in the course of various liver diseases. 9 Nov 88

The onset of spontaneous hemolytic jaundice in a young subject should lead to the search for Wilson's disease when clinical examination reveals cirrhosis. This hemolysis may evolve in the form of severe jaundice to a stage where the cirrhosis remains usually latent or well tolerated. The intervention of a toxic, allergic of infective factor liable to produce a hepatic lesion which frees a dose of copper sufficient to trigger off hemolysis, is discussed. The mechanism of the latter, that of the coagulation disorders observed, liver cell failure and widespread intravascular coagulation, are analysed in this paper and compared with data in the literature. The dramatic character of the case indicates that it is necessary to treat as a routine with penicillamine all homozygous forms of Wilson's disease.
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PMID:[Severe hemolytic jaundice and Wilson's disease]. 19 22

Histological study of 69 cases of cirrhosis, 9 of severe generalised hepatic fibrosis, and 19 of hepatocellular carcinoma showed an association with alcohol, hepatitis B surface antigen (HBsAg) or a1-antitrypsin bodies in, respectively, 41 (cirrhosis), 5 (fibrosis), and 9 (carcinoma). Eight of the cirrhotic cases and two of the carcinoma cases had double associations, HBsAg being present in all. Torcein and aldehyde fuchsin staining gave both false positive and false negative results when compared with immunofluorescence and immunoperoxidase methods for HBsAg. Large amounts of copper were found in four cirrhotic livers, and moderate amounts in 13: the diagnostic value of copper staining is questioned.
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PMID:Aetiology of cirrhosis, hepatic fibrosis, and hepatocellular carcinoma. 19 27

The enzyme collagen proline hydroxylase has been measured in liver biopsies from fourteen patients with primary biliary cirrhosis. The activity was elevated in ten of the patients, but not to the degree previously found in patients with active cirrhosis. There was no correlation between the enzyme activity and the liver copper concentration, which was elevated in all except one of those measured. This suggests that excessive collagen synthesis in PBC is not directly related to the high liver copper concentrations.
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PMID:Hepatic collagen proline hydroxylase activity in primary biliary cirrhosis. 20 46

In connection with 6 cases of Wilson's disease, the authors recall the main features of this hereditary metabolic disorder at late onset (usually the second decade), treatable with a chelating agent, when diagnosed at an early stage. Wilson's disease is first of all a liver disease and the authors emphasize the fact that cirrhosis is usually present when neurologic symptoms, revealing the disease in 5 cases, appear, even if there is no clinical or biological evidence for liver disease. In one instance hemolytic anemia and chronic active hepatitis were observed at clinical onset. Copper metabolism usually gives the key for diagnosis but its interpretation may be difficult, a normal serum ceruleoplasmin level being found in two patients and evaluated at 6% in the literature. This fact brings up the puzzling question of the pathogesis of the disease. Wilson's disease is not a simple ceruleoplasmin synthesis defect, but a lysosomal disease responsible for the lack of copper biliary excretion. This is pointed out by histochemical studies using a special rubeanic acid preparation (revealing copper deposit on the biliary side of the hepatic cell), and by electron microscopy showing lysosomal dystrophy.
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PMID:[Wilson's disease. A clinical and pathological study on 6 cases (author's transl)]. 22 95

Four cases of hepatic angiosarcoma are reported with a review of 99 other cases in the English literature. Angiosarcoma of the liver is associated with chronic exposure to thorotrast, vinyl chloride, arsenicals, radium and possibly copper and with chronic idiopathic hemochromatosis. Although 40% of patients have hepatic fibrosis or cirrhosis at autopsy, the nature of the association between chronic liver disease and hepatic angiosarcoma is unknown. The clinical presentation of hepatic angiosarcoma is nonspecific with abdominal pain, weakness and weight loss common complaints and with hepatomegaly, ascites and jaundice common findings. Liver function tests are usually abnormal but there is no one liver function test or set of tests specific for the tumor. The occurrence of thrombocytopenia and disseminated intravascular coagulation is characteristic of hepatic angiosarcoma and may be related to local consumption of clotting factors and formed blood elements in the tumor. Catastrophic intraabdominal bleeding is also characteristic and occurs in one-fourth of all cases. This complication is likely related to the high incidence of clotting abnormalities and the vascular nature of the neoplasm. Selective hepatic arteriogram and open liver biopsy are the foundations of diagnostic evaluation. Percutaneous liver biopsy should be avoided. Failure to appreciate the possibility of hepatic angiosarcoma in the proper clinical setting, leading to blind percutaneous biopsy, may result in failure to make the diagnosis at the cost of significant morbidity and mortality. Survival of patients with hepatic angiosarcoma is brief; only 3% live longer than 2 years. Treatment of the tumor to date is empirical. There are probably a few patients who might benefit from radical surgery with curative intent. For all others chemotherapy is indicated. Adriamycin is active against hepatic angiosarcoma, but optimal dose and mode of administration require further investigation. Further study is also required to delineate the cause of hepatic angiosarcoma in the 60% of cases without definite epidemiologic association.
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PMID:The clinical features of hepatic angiosarcoma: a report of four cases and a review of the English literature. 36 8

The levels of cupriuresis before and after DL-Penicillamine have been investigated in 168 cases. The mean copper excretion before Penicillamine in chronic activ liver disease, chronic persistant hepatitis, cirrhosis and in transitional cases of aggressiv chronic hepatitis and primary biliary cirrhosis ranged from 29 gamma to 48 gamma/24 hr.; however, in some cases the daily copper excretion exceeds 100 gamma, as well in subjects with liver disease as in normals too. After ingesting 900 mg DL-Penicillamine the mean values of cupriuresis ranged from 500 gamma to 600 gamma/24 hr. Abnormal results were found in about 15% of those subjects with liver diseases; in only two of 20 cases with hypercupruria after Penicillamine Wilson's Disease was established.
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PMID:[Spontaneous and DL-penicillamine-induced renal copper excretion in liver diseases (author's transl)]. 43 70

In a study of 90 Bedlington Terriers, 68 had a defect that resulted in the accumulation of toxic excesses of copper in the liver. Concentrations of copper were 5 to 50 times that of clinically normal mongrel dogs. The bulk of this excess copper was sequestered in lysosomes. When copper concentrations exceeded 2,000 micrograms/g dry liver, progressive signs of functional and morphologic disturbance appeared as focal hepatitis, chronic active hepatitis, and ultimately cirrhosis. The disorder, which appears to be inherited, could only be diagnosed by liver biopsy. It was latent for many years in some dogs but led early in life to acute or chronic hepatic disease and death in others.
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PMID:Clinical, morphologic, and chemical studies on copper toxicosis of Bedlington Terriers. 50 Apr 53


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