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)

Serum thiobarbituric acid reactive substances (TBARS), Zn, Cu, and Se concentrations were determined in 47 healthy adults and in patients with diseases, such as renal insufficiency, insulin-dependent diabetes mellitus, chronic pancreatitis, liver cirrhosis, or cancer, in order to clarify the relationship between this indicator of lipid peroxidation and antioxidative trace element status. TBARS levels were higher than control values in all pathological cases, except in cancer patients. Cu levels in patients highly correlated with ferroxidase ceruloplasmin activity (r = 0.86), but were only statistically different from controls in diabetics. Zn levels were lower than normal in dialysis, liver cirrhosis, and cancer patients. Se levels were significantly decreased in all pathological cases. Half of the subjects with liver cirrhosis or renal insufficiency and 3/4 of chronic pancreatitis or cancer patients had an active inflammatory process. Despite intense modifications in determined indicators, no clear correlation could be demonstrated between the different parameters. Basic antioxidative trace element status and inflammation are therefore not major determinants of TBARS levels in normal and in pathological conditions, despite of the frequent association of low serum Zn and mainly low serum Se with high TBARS levels.
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PMID:Lipid peroxidation assessed by serum thiobarbituric acid reactive substances in healthy subjects and in patients with pathologies known to affect trace element status. 777 41

Wilson's disease is an hereditary recessive autosomal disorder which affects around five people per million inhabitants. The primary defect is localized in the liver and the disease is manifested by the accumulation of copper in tissues. The diminution of ceruloplasmin, which until a few years ago was mistakenly thought to be the pathogenetic cause of Wilson's disease, is an epiphenomenon of the underlying metabolic defect characterized by defective copper biliary excretion. There are four stages in the natural history of the disease: 1) an asymptomatic stage of hepatic copper accumulation; 2) dismission and redistribution of copper leading to hepatocellular necrosis and hemolysis; 3) extrahepatic accumulation of copper leading to the onset of cirrhosis and neurological damage; 4) stage of homeostasis following treatment but with possible irreversible neurological damage. Treatment of Wilson's disease takes the form of pharmacological, dietary and surgical therapy. Through the formation of copper and protein metal complexes D-penicillamine impoverishes copper deposits causing the reduction or disappearance of hepatic and neurological symptoms; a small percentage of patients treated develops a nephrotic syndrome requiring the compulsory suspension of the drug. In this case a valid alternative is triethylenetetramine dichlorohydrate (TETA) which provokes increased blood copper during copper diuresis. The response to pharmacological treatment is better the earlier treatment is started and the more regular its administration. Dietary intake of copper must be reduced in parallel avoiding foods with a high copper content. Liver transplant obviously leads to the "resolution" of the underlying metabolic problem in patients who develop fulminating hepatitis with hypercupremia and hemolysis and, of course, in cases of uncompensated cirrhosis which do not respond to chelating therapy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Wilson's disease: physiopathology, therapeutic approach and case report]. 784 48

The activity of antioxidant defense enzymes and lipid peroxidation (LPO) was studied in the liver and blood of 126 patients with hepatobiliary diseases. The activity of superoxide dismutase (SOD) and catalase in the liver appeared inhibited and relevant interactions impaired. Catalase/peroxidase value in hepatic cirrhosis proved minimal. In response to hepatotropic drugs red cell SOD decreased, while glutathione and ceruloplasmin levels became elevated. Blood LPO values were adequate indicators of the disease progression. It is shown that deficient antioxidant defense of the liver in chronic affections contributes to oxygen radical formation which promotes pathological processes in the liver.
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PMID:[The clinical significance of the enzymatic system utilizing active forms of oxygen in chronic liver diseases]. 801 35

Copper contents (Cu) in bodies and serum ceruloplasmin (Cp) were assayed in patients with liver cirrhosis (LC) and hepatocarcinoma (HCC) with atomic absorption and other methods. The results were shown as follows: 1. The mean levels of serum Cp and urine Cu in LC were higher than those of normal (P < 0.05 and 0.01). 2. Serum Cu and Cp levels were consistently high in HCC. Urine Cu level was also elevated and had positive correlation with that of serum Cu (r = 0.567, P < 0.01). 3. Cirrhotic liver Cu content was almost the same as that of pericarcinomatous liver Cu, being higher than that of normal and carcinomatous liver. 4. Hair Cu level in both LC and HCC was apparently lower than that of normal subjects. 5. Serum Cu level in patients with tumor more than 5 cm in size was higher than that in patients with tumor less than 5 cm (P < 0.05). 6. Serum Cu level decreased along with the reduction of tumor size after treatment. 7. Serum Cu and Cp levels may be used as markers for detection of HCC, especially for AFP-negative HCC. Serum Cu estimation is valuable in assessment of the therapeutic effect and prognosis in patients with HCC.
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PMID:[The changes in copper contents and its clinical significance in patients with liver cirrhosis and hepatocarcinoma]. 807 Feb 91

In addition to ten children with Wilson's disease and one with Indian childhood cirrhosis, nine Indian children, aged from 4 to 15 years, with cryptogenic cirrhosis had significant deposits of stainable copper in their hepatocytes. These nine children had normal or elevated serum caeruloplasmin levels, absence of Kayser-Fleischer rings and a history of sibling death owing to liver disease in four cases. Histologically, fatty change was absent from all the biopsies but Mallory's hyaline, pericellular fibrosis and ballooning of hepatocytes were present in some. Since these children did not conform to the accepted clinical or histological definitions of either Indian childhood cirrhosis or Wilson's disease, they were designated as having atypical copper cirrhosis. The relationship of this group of cases to other types of copper cirrhosis is unknown.
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PMID:Atypical copper cirrhosis in Indian children. 853 43

Patients with chronic hepatic disease have higher superoxide dismutase (SOD) activity and lower erythrocytic glutathione levels. There was a decrease in plasma SOD activity in cirrhosis, a feedback between the dismutase and oxidase activities of ceruloplasmin in cholestatic damages to the liver. Drug therapy resulted in positive dynamics in the levels of SOD, glutathione peroxidase, glutathione, ceruloplasmin, which is likely to be associated with the control of the enzymatic mechanisms of antioxidative protection. It is suggested that the enhanced erythrocytic SOD activity in hepatic diseases might trigger free radical oxidation.
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PMID:[Activity of blood antioxidant enzymes in chronic liver damage]. 857 91

A common killer disease of the past, Indian childhood cirrhosis (ICC), which became preventable and treatable in the early 1990s, is now rare. ICC must be clearly distinguished in Indian children from other chronic liver disorders including Wilson disease. Grossly increased hepatic, urinary, and serum copper concentrations are characteristic of ICC. These increased concentrations are easily demonstrated histologically with orcein-rhodanine staining. Environmental ingestion of copper appears to be the most plausible explanation for ICC, as shown by feeding histories, the prevention of ICC is siblings and in the Pune district by a change in feeding vessels, and the dramatic reduction in incidence of ICC throughout India. The nature and role of a second factor in the causation of ICC remains unclear, although an inherited defect in copper metabolism is strongly suspected. ICC, however, does not appear to be a straightforward early onset of Wilson disease because ceruloplasmin is consistently normal and clinical and histologic recovery is maintained in the long term despite withdrawal of D-penicillamine therapy. Descriptions of an ICC-like illness in the West suggest that different mechanisms (environmental, genetic, or both) can lead to the same end-stage liver disease: copper-associated childhood cirrhosis. ICC probably represents a specific form of copper-associated childhood cirrhosis that requires high environmental copper ingestion for its full expression.
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PMID:Present interpretation of the role of copper in Indian childhood cirrhosis. 861 70

We describe a patient who had severe neurologic symptoms, psychiatric abnormalities, and secondary amenorrhea superimposed on a history of hemolytic anemia and micronodular cirrhosis attributed to hemochromatosis. The correct diagnosis of Wilson's disease was delayed until the appearance of Kayser-Fleischer rings and a low serum ceruloplasmin level. Appropriate treatment ameliorated symptoms, and maintenance therapy has been effective in retarding progression. It is essential to consider Wilson's disease in patients with unexplained hepatic, neurologic, and psychiatric dysfunction, because appropriate early medical treatment can prevent further organ damage and reduce the risk of permanent damage to the liver and brain.
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PMID:Wilson's disease. 916 75

End-stage liver disease secondary to cryptogenic cirrhosis is the indication for orthotopic liver transplantation (OLT) in 7% to 14% of recipients. However, there are no reports documenting the outcome of OLT for this indication. The aim of this study was to determine (1) survival and (2) the incidence of histological recurrence of cryptogenic cirrhosis after OLT. Between March 1985 and December 1994, 560 OLTs were performed at our institution. Of these, 39 transplants for cryptogenic cirrhosis were in patients who met the following criteria: antinuclear antibody < 1:40; negative anti-smooth muscle antibody, antimitochondrial antibody, polymerase chain reaction for hepatitis C virus, and hepatitis B surface antigen results; normal ceruloplasmin and alpha-1 antitrypsin phenotype; transferrin saturation < 65%; and liver biopsy specimen not suggestive of hemochromatosis or other known disorders. Histological recurrence was assessed with protocol liver biopsies in all patients who survived longer than 6 months. The mean age of cryptogenic recipients at the time of transplantation was significantly lower (40.6 years; range, 3 to 63 years) than that of noncryptogenic recipients (48.5 years; range, 1-70; P < .03). Median modified Child's-Pugh score was slightly higher for cryptogenic recipients at the time of transplantation (10.0 + 0.08 standard error of mean [SEM]), than for the noncryptogenic recipients (9.0 + 0.03 SEM; P < .02). Actuarial survival was 72% (+ 0.07 SEM) at 1 and 58% (+ 0.08 SEM) at 5 years for cryptogenic recipients compared with 89% at 1 and 80% at 5 years for noncryptogenic recipients. The difference in survival was significant (P < .001) at both 1 and 5 years. Among the 27 cryptogenic recipients surviving more than 6 months (mean follow-up, 5.5 years), 6 have persistent hepatitis histologically without apparent infectious, vascular, biliary, or drug origins. Four patients (15%) had chronic active hepatitis, and 2 (7%) had steatohepatitis. No cases of recurrent cryptogenic cirrhosis were seen. OLT for cryptogenic cirrhosis is associated with a poor outcome compared with other indications, hepatitis of uncertain origin occurred in 22% of cryptogenic recipients surviving longer than 6 months, and no evidence of recurrence of cryptogenic cirrhosis was seen thus far in follow-up.
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PMID:Liver transplantation for cryptogenic cirrhosis. 934 64

Brazil has a young population and areas of endemic mansoni schistosomiasis where Wilson's disease might be easily misdiagnosed in patients erroneously classified as having either the hepatosplenic or the hepatointestinal form of the helminthiasis. Twenty five patients with the "hepatic form" of Wilson's disease (14 males and 11 females) were investigated in Belo Horizonte, MG; the mean age was 13.7 years (3 to 22). Nineteen had hepatomegaly (76%) and nine splenomegaly (36%). Twenty two (88%) had cirrhosis. The Kaiser-Fleisher ring was detected in fifteen (60%). Four (16%) had clear neurological abnormalities. Eleven (44%) had ascitis and/or jaundice. Ninety one point three per cent and 92% had low ceruloplasmin and copper serum levels respectively. Eighty four point two per cent showed an increased 24 hours urinary copper excretion; seven patients in whom hepatic copper was determined had increased values. Six out of nine had at least a ten fold increase in 24 hours urinary copper excretion following penicillamine use ("penicillamine test"). Three out of 19 patients (15.8%) had mansoni schistosoma ova in stools examination, a common prevalence in our population. Their biopsies showed inactive cirrhosis without schistosomiasis-associated alterations. At least fourteen patients (56%) could be misdiagnosed as having hepatointestinal or hepatosplenic schistosomisis when in fact they suffered from Wilson's disease with or without asymptomatic intestinal schistosomiasis, losing the chance of an early treatment. The follow-up time of 22 patients was 52 months (1 to 96); eight (36.3%) died, four from bleeding esphageal varices, three from terminal hepatic failure and one from fulminant liver failure. The majority of the patients, including those who died, had abandomned the use of penicillamine or had taken it irregularly, due mainly to its highly expensive cost. A 17 year old patient underwent a successful liver transplant in 1989.
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PMID:[Wilson's disease ("hepatic form") in a region endemic for schistosomiasis mansoni: clinical presentation of 25 patients]. 971 8


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