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)

This study investigated the long-term survival rates of 85 patients with hereditary hemochromatosis. Eighty-five patients with documented hereditary hemochromatosis diagnosed between 1958 and 1989 and followed up at the University Hospital (University of Western Ontario) medical center were retrospectively reviewed for this analysis. The current status of the patient was assessed by interview or written questionnaire completed by the patient or the family physician. Estimates of differences in survival rates were obtained using Kaplan-Meier life-table and Cox regression analysis. Liver histology, clinical features of the disease, and number of venesections were analyzed to determine their relationship to survival. In the course of a mean follow-up interval of 8.1 +/- 6.8 years (range, 0-31 years), there were 17 deaths among the 85 hemochromatosis patients. Patients with cirrhosis at the time of diagnosis were 5.5 times more likely to die than noncirrhotic patients. Patients who were noncirrhotic at the time of diagnosis had an estimated survival that was not significantly different from age- and sex-matched members of the normal population. Diabetes did not increase the risk of death after data were controlled for the presence of cirrhosis. Early diagnosis and treatment of hemochromatosis in the precirrhotic stage can lead to long-term survival similar to that in the general population. The presence of cirrhosis significantly increases mortality and is the major clinical factor affecting survival.
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PMID:Long-term survival analysis in hereditary hemochromatosis. 206 12

To determine the frequency of liver profile abnormalities in hereditary hemochromatosis, we under took a retrospective survey in 100 patients, all of whom had undergone liver biopsy. Liver histology was compared with the biochemical profile, which included aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase, bilirubin and albumin determinations. Mild abnormalities in the AST and ALT levels were seen in more than 65% of patients. Patients with cirrhosis had significantly greater elevations in AST, ALT, and alkaline phosphatase, and a significant decrease in albumin (p less than 0.05). Proband cases had more frequent abnormalities than discovered cases within families. Accordingly, we find that mild abnormalities in the biochemical liver profile are common in hemochromatosis and suggest that patients with an unexplained abnormality in the liver profile should be screened for hemochromatosis with a serum ferritin and transferrin saturation.
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PMID:Biochemical liver profile in hemochromatosis. A survey of 100 patients. 206 47

An autopsy case is described of a primary hemochromatosis in a 33-year-old man which was not diagnosed clinically. The peculiar feature of the disease was the absence of skin discolorations, definite symptoms of diabetes mellitus and a hepatic cirrhosis against the background of pigment cardiomyopathy. It's suggested that the progression of the heart pathology was due to the use of certain antibacterial preparations.
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PMID:[Primary hemochromatosis with cardiac involvement]. 214 39

Primary hemochromatosis is a common genetic disorder that results in inappropriate iron absorption and storage, with progressive damage to target organs. Hepatic cirrhosis and hepatocellular carcinoma are sequelae of hemochromatosis which are potentially preventable. The diagnosis may be suspected prior to target organ damage by appropriate screening tests, and is confirmed by liver biopsy. Three cases of hemochromatosis in the precirrhotic stage of the disease are presented. The pathophysiology, clinical and laboratory features and management are discussed. The high gene frequency in the general population warrants routine screening tests in asymptomatic healthy young adults. Phlebotomy is the indicated treatment for all stages of the disease.
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PMID:Diagnosis and management of precirrhotic hemochromatosis. 215 80

Hepadnaviruses share properties of virion structure, genome structure and replication, epidemiologic behavior, and pathogenic effects, including an association with hepatocellular carcinoma (HCC). Epidemiologic evidence implicating hepadnavirus infection in HCC includes the observation that the geographic distributions of HBV infection and HCC are similar, that the incidence of HCC is much higher in hepadnavirus infected than uninfected hosts, and that viral DNA sequences are integrated in the cellular DNA of most (e.g., 80-90%) but not all hepadnavirus-associated HCC. Cirrhosis further increases the risk of HCC in HBV infected humans. The precise role of hepadnaviruses in development of most HCC is unclear, although the finding of viral integrations within or near protooncogenes in a few cases suggests the possibility that these integrations may play a direct role in these HCC. However, in the great majority of HCC associated with HBV infections, viral integrations are in different cellular DNA sites in different HCC, integrations are not within domains of known protooncogenes, and integrations are not found in some 10-15% hepadnavirus-associated HCC, suggesting that persisting viral sequences are not directly involved in the development of these HCC as viral sequences are for tumors caused by viruses with oncogenes or viruses that act by a "promoter-insertion" mechanism. It is possible, however, that oncogenic mutations could arise via other mutagenic mechanism that may operate in chronic hepatitis B and/or cirrhosis and which do not involve persisting viral integrations. For example, liver regeneration, which is a feature of the cirrhosis associated with chronic HBV infection (and sometimes with chronic hepatitis B) involves proliferation of many cells with HBV integrations, and such integrations have been shown to be unstable and may lead to mutations through post-integration rearrangements of cellular sequences at sites of viral integrations. Viral sequences appear to be lost or deleted at some such sites of rearranged cell DNA. Chronic HBV infection shares pathologic features of liver cell injury and reactive inflammation, liver regeneration, and in man sometimes cirrhosis with other important risk factors for HCC including chronic alcoholic liver disease, chronic non-A, non-B hepatitis, hemochromatosis, and crypogenic cirrhosis, suggesting that this common pathologic process may be carcinogenic by a mechanism that does not depend specifically on the factor which initiates liver cell injury. The pathogenetic role of chronic hepadnavirus infection in such a process would be in causing liver cell injury with reactive inflammation and hepatocyte proliferation (regeneration).(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Hepadnaviruses in cirrhotic liver and hepatocellular carcinoma. 216 15

In the United States, a large percentage of patients with hepatocellular carcinoma are serologically negative for hepatitis B. We conducted a retrospective study to determine the prevalence of hepatitis C antibody in the sera of 59 patients with hepatocellular carcinoma who were HBsAg-negative and had no evidence of alcoholic liver disease, primary biliary cirrhosis, autoimmune hepatitis, hemochromatosis or alpha 1-antitrypsin deficiency. Twenty patients (34%) were hepatitis C antibody-positive and hepatitis B core antibody-negative. All twenty patients had underlying cirrhosis, and seven (35%) had histories of transfusions. Eleven (19%) additional patients were also hepatitis C antibody-positive but were hepatitis B core antibody-positive as well. Twenty-one (36%) patients were both hepatitis C antibody- and hepatitis B core antibody-negative and seven (12%) were hepatitis C antibody-negative but hepatitis B core antibody-positive. The prevalence of hepatitis C antibody was also determined among three other population groups serving as controls and found to be 14% in 28 HbsAg-positive patients with hepatocellular carcinoma, 44% in 76 patients with cryptogenic cirrhosis and 0.5% in 200 consecutive volunteer blood donors. We conclude that hepatitis C antibody is prevalent among patients with hepatocellular carcinoma and may therefore be a common causative agent of this disease. A significant number of patients with and without cirrhosis, negative for hepatitis C antibody and hepatitis B core antibody, remain without a discernible cause for this malignancy. Perhaps a second- or third-generation test will detect hepatitis C antibody in some of these patients.
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PMID:Hepatitis C-associated hepatocellular carcinoma. 165 57

Hemochromatosis, or primary iron overload, is a variably expressed genetic metabolic disorder greatly modified by sex, age, diet, and alcohol consumption. Although a diagnosis has been made at the bedside by careful documentation of the slow resolution of subcutaneous iron pigment, clinical diagnosis is frequently overlooked, and even autopsy may fail to reveal hemochromatosis as the cause for cirrhosis. Genetic linkage studies have confirmed the extremely high prevalence of this disorder. Untreated patients may succumb to sepsis caused by organisms such as Vibrio vulnificus, Yersinia species, and others whose virulence is altered by iron availability.
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PMID:Hemochromatosis and infection: alcohol and iron, oysters and sepsis. 248 33

In both hereditary hemochromatosis and in the various forms of secondary hemochromatosis, there is a pathologic expansion of body iron stores due mainly to an increase in absorption of dietary iron. Excess deposition of iron in the parenchymal tissues of several organs (e.g. liver, heart, pancreas, joints, endocrine glands) results in cell injury and functional insufficiency. In the liver, the major pathological manifestations of chronic iron overload are fibrosis and ultimately cirrhosis. Evidence for hepatotoxicity due to iron has been provided by several clinical studies, however the specific pathophysiologic mechanisms for hepatocellular injury and hepatic fibrosis in chronic iron overload are poorly understood. The postulated mechanisms of liver injury in chronic iron overload include (a) increased lysosomal membrane fragility, perhaps mediated by iron-induced lipid peroxidation, (b) peroxidative damage to mitochondria and microsomes resulting in organelle dysfunction, (c) a direct effect of iron on collagen biosynthesis and (d) a combination of all of the above.
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PMID:Hepatic injury in chronic iron overload. Role of lipid peroxidation. 266 96

We present a patient with idiopathic liver hemochromatosis and mild secondary cirrhosis complicated by Yersinia sepsis and miliary liver abscesses proven by echography and CT.
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PMID:Ultrasound and CT of multifocal liver abscesses caused by Yersinia enterocolitica. 267 27

The contribution of diabetes and cirrhosis to sexual dysfunction and hypogonadism was evaluated by two-way analysis of variance in a group of 30 men with idiopathic hemochromatosis. The prevalence of severe sexual dysfunction was significantly higher in men with hemochromatosis than in a control group matched for prevalence of diabetes and age (P less than 0.001). In both controls and hemochromatosis patients the presence of diabetes was significantly associated with sexual dysfunction (P less than 0.005), but the more severe symptoms in the hemochromatosis patients were related to the additive effects of hypoandrogenism (P less than 0.01). Sexual dysfunction was a common early complaint in hemochromatosis patients, but these symptoms were frequently overlooked, leading to diagnostic delay. Mean testicular volume was a useful measure of gonadal status, being significantly correlated with indices of serum free testosterone (rs = 0.83; P less than 0.01) and LH (rs = 0.71; P less than 0.001). The presence of cirrhosis did not contribute significantly to symptomatology, but had an effect independent of and additive to hypogonadotropic hypogonadism in reducing serum free testosterone (P less than 0.02) and estradiol (P less than 0.002), an effect apparently mediated through central rather than testicular mechanisms. Hypoandrogenism was associated with an increase in serum sex hormone-binding globulin (SHBG) concentrations (P less than 0.005), but cirrhosis also had an independent effect in raising SHBG (P less than 0.005), which could not be accounted for by changes in circulating sex hormone concentrations. Thus, the evaluation of sexual dysfunction or hypogonadism in men with hemochromatosis requires consideration of the effects of both diabetes and cirrhosis. Because of the greater variance in SHBG some estimate of free testosterone rather than total testosterone is preferable.
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PMID:Hypogonadism and sexual dysfunction in hemochromatosis: the effects of cirrhosis and diabetes. 273 93


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