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)

Yersinia pseudotuberculosis is a rare cause of disease in humans, the most common manifestation being mesenteric lymphadenitis accompanied by abdominal pain and fever. A septicemic form of Yersinia pseudotuberculosis infection has been reported only rarely. It is usually seen in patients with underlying disorders such as diabetes, hepatic cirrhosis or iron overload. Fifty-four cases of septicemic infection were found in the literature. The earlier published cases are reviewed, and four cases occurring in Finland during the period February to June 1992 are reported.
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PMID:Report of four cases of Yersinia pseudotuberculosis septicemia and a literature review. 853 31

Iron overload to the liver induces hepatic injury, eventually ending up with liver fibrosis or cirrhosis. Pathogenic mechanisms involved in liver damage are only partially known, but there is evidence for an important role of iron-induced reactive oxygen species. We have, therefore, analyzed the immunohistochemical reactivity for two major free radical scavengers, copper/zinc and manganese superoxide dismutase (Cu/Zn- and Mn-SOD's) in three situations of hepatic iron overload, and compared enzyme patterns with grades of iron deposition, grades of fibrosis, and levels of microphotometrically measured type IV collagen immunoreactivity. Cu/Zn- and Mn-SOD reactivity was detectable in hepatocytes with a heavy and a low iron burden, but Cu/Zn-SOD staining was more intense than that of Mn-SOD in the three groups analysed. There was trend for microphotometrically measured type IV collagen levels to increase with the amount of iron, and increased collagen IV was correlated with higher grades of Cu/Zn-SOD, but not of Mn-SOD, reactivity. The findings suggest that the two SOD's may be differentially expressed in states of hepatic iron overload, and that low expression of the inducible radical scavenger, Mn-SOD, may play a role in chronic iron toxicity.
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PMID:Copper/zinc and manganese superoxide dismutase immunoreactivity in hepatic iron overload diseases. 857 13

Haemochromatosis is a common autosomal recessive disorder of iron metabolism caused by a gene in tight linkage with HLA class I genes. Despite intensive research, the molecular defect and underlying biochemical anomaly are still unknown. Diabetes, a serious complication of haemochromatosis, is frequently associated with cirrhosis which reduces life expectancy. Its development is related to iron excess, directly or through associated liver involvement, although the precise mechanisms of iron toxicity remain unclear. New concepts concerning its pathogenesis include insulin resistance and beta-cell dysfunction which are apparent well before insulin deficiency and can be reversed if iron depletion is promptly initiated. Today, earlier recognition of iron overload through active diagnostic approaches has a direct impact in reducing the frequency of diabetes among hemochromatosis patients. Presymptomatic diagnosis in the general population and among relatives of affected subjects currently relies on the detection of increased iron stores through medical awareness and family screening. Indirect gene diagnosis with serological and molecular markers of the HLA region can be provided for relatives of proven cases. As part of a genetic counselling process, this allows the identification of at-risk subjects before the onset of iron accumulation. Isolation of the gene and identification of the metabolic defect leading to increased iron absorption may have significant implications for future diagnostic procedures and preventive strategies in haemochromatosis.
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PMID:Diabetes and haemochromatosis: current concepts, management and prevention. 858 48

We analyzed data from the first study of iron overload in Africans, conducted between 1925 and 1928, to determine whether this common condition is associated with death from hepatocellular carcinoma and/or tuberculosis. In the original study, necropsies were performed on 714 adult blacks from southern Africa. Hepatic and splenic iron levels were measured semiquantitatively in 604 subjects and one of five iron grades was assigned. We examined death from hepatocellular carcinoma or from tuberculosis and the variables of age, sex, the presence of cirrhosis or other diagnoses that might be influenced by iron status, and tissue iron grades. Nineteen percent of men and 16% of women had the highest grade of hepatic iron. After adjustment for the presence of cirrhosis, hepatic iron grade was the variable most significantly associated with death from hepatocellular carcinoma (P = .021). The odds of death from hepatocellular carcinoma in subjects with the highest grade of hepatic iron was 23.5 (95% confidence interval, 2.1 to 225) times the odds in subjects with the three lowest grades. Splenic iron was the variable most significantly associated with death from tuberculosis (P <.0001). The odds of death from tuberculosis with the highest grade of splenic iron was 16.9 (4.8 to 59.9) times the odds with the two lowest grades. These findings suggest that iron overload in black Africans may be a risk factor for death from hepatocellular carcinoma and for death from tuberculosis.
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PMID:Associations of iron overload in Africa with hepatocellular carcinoma and tuberculosis: Strachan's 1929 thesis revisited. 860 66

Intraperitoneal deferoxamine is a well established treatment for aluminum accumulation syndrome in patients with end-stage renal disease receiving peritoneal dialysis, but the use of intraperitoneal deferoxamine has not been described outside of the setting of chronic renal failure. We present here a case of secondary hemochromatosis, complicated by cirrhosis and cardiomyopathy, in which a chronic peritoneal dialysis catheter was used both to treat ascites and to deliver parenteral deferoxamine for iron overload. Daily urinary iron excretion was similar to that achieved when using standard routes of deferoxamine administration. Over a 2-year period, reversal of both the biochemical indicators and the clinical manifestations of iron overload was accomplished.
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PMID:Long-term intraperitoneal deferoxamine for hemochromatosis. 862 76

The relationship between alcoholism and hereditary hemochromatosis remains controversial. Previous studies have included patients with alcoholic siderosis rather than hereditary hemochromatosis. In this retrospective study, the clinical features, iron status, alcohol history, liver histology, and long-term survival were reviewed in 105 homozygotes for hemochromatosis using rigid diagnostic criteria including an HLA identical sibling with iron overload. Heavy alcohol consumption (>80 g ethanol/day) was found in 15 percent of hemochromatosis patients. Histological features of alcoholic liver disease (Mallory's hyaline bodies, pericentral fibrosis, polymorphonuclear infiltrate, and fatty infiltration) were uncommon in hemochromatosis. Hemochromatosis patients with heavy alcohol consumption had a higher prevalence of cirrhosis than hemochromatosis patients without heavy alcohol consumption. Hepatic iron concentration and hepatic iron index did not significantly differ between these two hemochromatosis groups. Long-term survival was significantly reduced in patients with heavy alcohol consumption (mean follow-up, 9.22 years). This suggests that chronic alcohol consumption has an additive hepatotoxic effect despite the paucity of histological features of alcoholic liver disease.
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PMID:Alcoholism in hereditary hemochromatosis revisited: prevalence and clinical consequences among homozygous siblings. 866 24

The study of 226 cases of hepatocellular carcinoma (HCC) in a homogenous rural Southern African population is based on the assessment of histology, HBV infection, p53 oncoprotein and transforming growth factor alpha (TGFa) expression. Epidemiological and morphological observations were compared to HCC observed in 89 cases from pathological files in Poland and published information from Japan and Italy. Comparatively high number of young patients with HCC in Africa presented high rates of HBV infection, p53 oncoprotein overexpression and high HBsAg/p53 correlation rates. In all patients histological grading of HCC was inversely related to p53 and TGFa expression. No significant differences in histological grading of HCC and patients' mean age were noted between various population groups. The association of hepatic cirrhosis was at least twice as common in non-African patients, whereas iron overload was noted almost exclusively in African patients livers. Signs of HBV infection were lowest in Japanese female patients. The mechanism by which early HBV infection contributes to hepatocarcinogenesis at an early stage of life is confirmed by epidemiological observations in Poland and by the clear association of p53 gene with HBsAg and the age of patients.
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PMID:Hepatocellular carcinoma in young patients: histology, cellular differentiation, HBV infection and oncoprotein p53. 866 53

Iron deposition occurs in parenchymal cells of the liver in two major defects in human subjects (i) in primary iron overload (genetic haemochromatosis) and (ii) secondary to anaemias in which erythropolesis is increased (thalassaemia). Transfusional iron overload results in excessive storage primarily in cells of the reticule endothelial system. The storage patterns in these situations are quite characteristic. Excessive iron storage, particularly in parenchymal cells eventually results in fibrosis and cirrhosis. There is no animal model or iron overload which completely mimics genetics haemochromatosis but dietary iron loading with carbonyl iron or ferrocene does produce excessive parenchymal iron stores in the rat. Such models have been used to study iron toxicity and the action of iron chelators in the effective removal of excessive iron stores.
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PMID:Hepatic iron deposition in human disease and animal models. 874 2

Genetic hemochromatosis is an autosomal recessive disease characterized by increased intestinal iron absorption and consequent tissue iron overload. The hemochromatosis gene has been localized on the short arm of chromosome 6, in close proximity to the HLA locus, but has yet to be identified. Neither the gene product nor the pathogenetic defect have been characterized. Clinical manifestations vary according to the degree of iron overload, ranging from the asymptomatic state to the features of cirrhosis and hepatocellular carcinoma. Early diagnosis remains essential, since the survival of patients without established cirrhosis is comparable to that of the general population. Transferrin saturation and ferritin levels are suggestive of the diagnosis, but measurement of the hepatic iron concentration still remains the gold standard, despite the utilization of computerized tomography and magnetic resonance imaging. Routine phlebotomies constitute the principal therapeutic option, despite the recent preliminary data on oral iron chelators.
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PMID:Genetic hemochromatosis: pathogenesis, diagnosis, and therapy. 890 16

Impaired glucose tolerance(IGT) frequently occurs in patients with liver diseases. Because of the central role of the liver in carbohydrate metabolism, it is generally assumed that impaired hepatic metabolism plays a major role in the pathogenesis of IGT in liver disease. However, recent observation using glucose clamp techniques has demonstrated that the majority of patients with cirrhosis are characterized by peripheral hyperinsulinemia and insulin resistance of muscle tissues. Glucose intolerance in patients with chronic pancreatitis in related to impaired glucose-mediated insulin secretion, which is induced by the loss of B-cell mass in the pancreas. Patients with hemochromatosis have insulin resistance in the precirrhotic stage. The mechanism of insulin resistance, produced by iron overload, remains unknown.
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PMID:[Impaired glucose tolerance in liver and pancreas disease]. 891 34


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