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)

A double antibody radioimmunoprecipitation test was used to detect anti-human LSP, anti-rabbit LSP, and autoantibodies against the human kidney equivalent of LSP (anti-HKP) in patients' serum. Anti-human LSP was found in 27/62 cases with chronic active liver disease (CALD), 9/16 cases with chronic persistent hepatitis (CPH), and 14/33 patients with acute viral hepatitis (AVH), 2/10 patients with ;inactive' cirrhosis of the liver (Ci), 4/14 patients with alcohol induced liver disease (ALD), 1/7 patients with miscellaneous liver diseases (MLD), and in 6/58 patients with primary non-hepatic autoimmune disease (PNHA). Frequencies of anti-LSP did not depend on HBsAg status. Anti-rabbit LSP was detected in only 9% of patients with AVH as compared with 42% for anti-human LSP. No such difference was observed in the other groups of patients. Anti-HKP was found in 6/62 patients with CALD, 1/7 patients with MLD, and 2/58 patients with PNHA; no anti-HKP occurred in patients with CPH, AVH, ALD, and Ci. The frequency of anti-LSP was not correlated with the presence of non-organ-specific autoantibodies in patients with CALD; furthermore, no correlation with sex-distribution, age, gammaglobulin levels, and SGOT occurred in this group of patients. No correlation existed between anti-LSP and liver membrane autoantibodies detected by indirect immunofluorescence on isolated rabbit hepatocytes (LMA). The reported data show that naturally occurring anti-LSP, characteristic for acute and chronic inflammatory liver diseases, are mostly directed against organ-specific determinants of the LSP complex. It is suggested that the occurrence of antibodies to species-specific determinants of LSP reflects a transient state of autoimmunity. The LMA immunofluorescence test seems to detect antibodies against other liver membrane antigens as well as LSP.
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PMID:Autoantibodies against liver-specific membrane lipoprotein in acute and chronic liver diseases: studies on organ-, species-, and disease-specificity. 745 May 61

Nonalcohol-induced fatty liver is widely believed to be a benign condition with little or no risk of disease progression. There have been occasional reports of progression to cirrhosis but none in the absence of preexisting fibrosis on the index biopsy specimen even when co-existing hepatitis was present (steatohepatitis). From our histological database (1978 to 1985), we identified 161 patients with fatty liver seen at our institution and traced the case notes of 156. One hundred five patients were initially excluded as having an alcohol-induced cause, and the remaining 51 either were seen in the clinic (37) or had died, in which cases copies of their death certificates were obtained (14). A further 7 patients were excluded after clinic attendance gave evidence of alcohol excess and another 4 after review of their initial biopsy showed the presence of fibrosis or steatohepatitis. The apparent cause of the steatosis in the 40 included patients with strictly nonalcohol-induced pure fatty liver was obesity in 12, diabetes in 4 (1 obese patient), and cachexia associated with extrahepatic malignancy in 6. Four of the remaining 19 had serological evidence of an autoimmune disorder, but none of these had any clinical or histological features of autoimmune liver disease. Nine patients had evidence of hyperlipidemia, 3 of whom were also obese. At a median follow-up of 11 years (7 to 16), 12 of 26 living patients had abnormal results of liver blood tests and had repeat liver biopsies performed. None had progressed to steatohepatitis or cirrhosis; 1 obese patient had developed mild fibrosis 9.8 years after her index biopsy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The natural history of nonalcoholic fatty liver: a follow-up study. 748 79

Hypoglycemia in fulminant hepatic failure and hyperinsulinemia in cirrhosis are well-described phenomena. A patient with alcoholic cirrhosis who developed fasting hypoglycemia with an extremely high immunoreactive insulin level and a mildly elevated C-peptide level is reported. An insulinoma was excluded by detailed radiological imaging of the pancreas and by endoscopic ultrasonography. Detection of very high levels of insulin autoantibodies with no prior exposure to exogenous insulin confirmed the diagnosis of insulin autoimmune syndrome. During his hospital course, the patient developed another rare syndrome, acquired inhibitors to factor V, which led to the fatal coagulopathy that resulted in his death. Insulin autoimmune syndrome is the third leading cause of spontaneous hypoglycemia in Japan, where it has been associated with a variety of diseases and drugs. Outside of Japan, only approximately 20 cases have been reported and usually have been found in the context of an underlying autoimmune disorder or prior exposure to sulfhydryl drugs. It is believed that this is the first case reported outside Japan occurring in association with alcoholic liver disease, and the first in the world with coexisting acquired inhibitors to factor V.
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PMID:Insulin autoimmune syndrome as a cause of spontaneous hypoglycemia in alcoholic cirrhosis. 755 52

We report the first series of 9 auxiliary liver transplantations performed as a bridge to recovery in 8 patients with fulminant and subfulminant hepatic failure. Hepatic failure was due to hepatitis A virus (n = 3), hepatitis B virus (n = 1), hepatotoxic drugs (n = 2), autoimmune disease (n = 1), or it was of unknown origin (n = 1). The donor liver was reduced to a left lobe (n = 2), a left liver (n = 4), or a right liver (n = 3), and was implanted in an orthotopic position beside the native liver after it was resected by a left or a right hepatectomy. Conventional immunosuppression was used to prevent rejection. Six patients regained normal consciousness within 2 weeks, without any sequelae. Two patients had persisting encephalopathy due to graft initial dysfunction, one of whom showed portal vein thrombosis, which was successfully cleared. The other one showed hepatic vein stenosis and was retransplanted at day 15. Five of eight patients had to be reoperated because of a surgical complication. Five patients showed rapid regeneration of their native liver, but one died at day 45 from severe herpes virus broncholitis. The auxiliary grafts were removed (n = 3) or left to atrophy by tapering immunosuppression (n = 1). One patient developed cirrhosis of the native liver and died of infectious complications at day 42. The native livers of the two remaining patients are still atrophic, one at 4 months and one at 1 month posttransplant. Finally, 6 of 8 patients are alive with a follow-up of 1 to 17 months. Four of them have permanently stopped their immunosuppressive therapy. Our experience demonstrates that auxiliary orthotopic liver transplantation (1) is feasible in children and adults, using either a left or a right liver graft, (2) is efficient in providing adequate liver function, and (3) gives a real chance to the native liver to regenerate, offering these patients a future free of immunosuppression.
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PMID:Auxiliary liver transplantation for fulminant and subfulminant hepatic failure. 783 43

The differing capacity of subclasses of IgG to bind to protein A and protein G was used in a sequential affinity purification procedure to examine immunoglobulin isotypes and subclasses in autoimmune disease. The utility of the procedure is that affinity-purified fractions containing particular isotypes and subclasses of immunoglobulin can be analyzed for their content of autoantibodies using standard techniques. For each of four autoimmune diseases studied, chronic active hepatitis, Sjogren's syndrome, primary biliary cirrhosis, and rheumatoid arthritis, there were characteristic protein elution profiles and the various disease-specific autoantibodies showed preferential distributions among the isotypes and subclasses. Moreover there was not an absolute correlation between an increased level of a particular subclass and the occurrence of antibodies of that subclass. The occurrence of highly disease-specific immunoglobulin subclass profiles suggests that the hypergammaglobulinemia associated with autoimmunity cannot be attributed entirely to polyclonal B-cell activation. Rather, there are disease-specific alterations in isotype subclass switching which may reflect different cytokine-dependent influences on autoimmune B cells and their products.
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PMID:Subclasses of immunoglobulins and autoantibodies in autoimmune diseases. 844 74

Juvenile Rheumatoid Arthritis (JRA) is a chronic, inflammatory, autoimmune disease of childhood. Methotrexate is an emerging antirheumatic drug in the pediatric population for disease refractory to conventional medications. While observations are encouraging, the toxic side effects can be potentially serious. Toxicity includes gastrointestinal intolerance, ulcerative stomatitis, chemical hepatitis, minor liver fibrosis, infection, hematologic suppression, acute pneumonitis, reversible oligospermia, and cirrhosis. The liver toxicities are of the greatest concern. If proper dosage and monitoring are followed, serious toxic effects can be prevented from occurring.
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PMID:Methotrexate use in juvenile rheumatoid arthritis. 845 Oct 58

The growth in liver transplantations recorded by the Pitt-UNOS Liver Transplant Registry since October 1987 continues as does the net growth of new centers. Characteristics of pediatric recipients in 1994 were compared to those of previous years and no significant differences were found for gender, race or age. The majority of pediatric recipients in 1994 awaited transplantation at home. The most common indication for liver transplantation in children was bilary atresia, though the proportion of recipients with this primary liver disease decreased significantly. Significant increases were noted in the proportions of pediatric recipients with autoimmune disease (though this remains a relatively uncommon indication) and fulminant liver failure. There was a significant decrease in the proportion of children who received ABO-incompatible livers. Many of the characteristics examined for adult recipients changed over time. The proportion of male recipients continued to increase. The mean age of adult recipients continued to increase, likely contributing to the increased prevalence of positive CMV-serology. The proportion of adult recipients awaiting transplantation outside the hospital increased over time. The increase in the proportion of multiorgan transplantations was in large part due to the increased reporting of bone marrow/liver transplants in 1994. Hepatitis non-A, non-B, or C and alcoholic liver disease were the most common reasons for LTX. The proportions of recipients with hepatitis B, fulminant liver failure and malignancies, indications with the poorest survival, all declined significantly. The cumulative probability of surviving (without retransplantation) for 7 years after initial transplantation was 0.70 (0.57) for pediatric recipients. Despite changes in recipient characteristics, the one-year survival for pediatric recipients did not change significantly over time. Significant differences in survival, unadjusted for other factors, were found by age (the youngest recipients had the worst survival), location awaiting transplantation (greater medical intervention just prior to transplantation led to poorer survival), multiorgan transplantation, primary liver disease (survival was worst for recipients transplanted due to malignancies, and best for patients with metabolic diseases), and donor/recipient ABO matching (survival was best for recipients of livers from donors with the same blood type). These results are similar to those previously reported for 4- and 5-year survivals. The cumulative probability of adults surviving (without retransplantation) for 7 years following LTX was 0.59 (0.52). Significant differences in survival, unadjusted for other factors, were found for year of transplantation (recipients in 1994 had better one-year survival than those transplanted in previous years), sex (males had worse survival than females), race (Blacks and Asians had the poorest survivals), age (recipients 50 years of age and older had the poorest survival), location awaiting transplantation (greater medical intervention just prior to transplantation led to poorer survival), multiorgan transplantation (recipients of organs in addition to the liver had worse patient survival than recipients of liver only), and primary liver disease (the best survival was for cirrhosis due to cryptogenic or cholestatic cirrhosis, the poorest survival was for malignancies and hepatitis B). Similar results were also reported previously for 4- and 5-year survivals.
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PMID:An update on liver transplantation in the United States: recipient characteristics and outcome. 879 52

Hepatitis C is becoming the main cause of cirrhosis and primary liver carcinoma. Infection by hepatitis C virus (HCV) generally induces an asymptomatic acute hepatitis. HCV infection becomes chronic in about 80% of cases. In a minority of the subjects, chronic HCV infection is asymptomatic with persistent viremia and normal liver tests. These asymptomatic subjects have minimal liver histologic lesions and a good prognosis. In a majority of the subjects, chronic HCV infection is associated with chronic hepatitis with increased serum transaminases levels. Among the patients with chronic hepatitis, the majority have a mild liver disease with a moderate increase in serum transaminases levels and, at liver histology, minimal lesions; a minority (about 20%) have a more severe liver disease and will develop cirrhosis after 5 to 20 years. In patients with HCV related cirrhosis, the incidence of hepatocellular carcinoma is high (around 5% per year). The factors influencing the evolution of HCV infection are not known. Alcohol is certainly an important factor which increases the risk of development of fibrosis then cirrhosis. Virus related factors, such as genotype and level of replication, might also be important. Autoimmune diseases have been reported in association with hepatitis C. HCV infection is a major cause of mixed cryoglobulinemia associated with vasculitis or glomerulonephritis. A relationship between HCV and auto-immune diseases such as thyroiditis or Gougerot syndrome has been suggested but not demonstrated. HCV infection is frequent in patients with porphyria cutanea tarda; in these patients, HCV related liver disease might trigger the expression of the metabolic disease.
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PMID:[Clinical picture and evolution of hepatitis C]. 899 8

We report the findings from liver biopsies of three patients with polyglandular autoimmune disease type 1. The livers in two patients had histologic features of chronic hepatitis that eventuated in bridging fibrosis and cirrhosis over a period of several years. Nonspecific lobular and portal tract inflammation was present in the liver biopsy of the third patient. Although these patients did not have liver-specific autoantibodies, the liver disease in polyglandular autoimmune disease type 1 possibly represents an expression of autoimmune hepatitis.
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PMID:Liver disease in polyglandular autoimmune disease type one: clinicopathologic study of three patients and review of the literature. 902 59

Primary biliary cirrhosis (PBC) is likely an autoimmune disease that destroys the interlobular bile ducts. Although the term PBC implies cirrhosis, this is not always present. The condition may be entirely silent clinically, save for the hallmark mitochondrial antibodies in serum. The clinical spectrum of PBC ranges from asymptomatic anicteric cholestasis with or without extrahepatic manifestations to severe cholestasis with decompensated cirrhosis. It is uncertain whether or not the course of this disease is universally fatal. Currently, no specific features have been identified which predict progression from asymptomatic to symptomatic disease, although once hyperbilirubinemia is present, a rising level indicates a poor prognosis. The liver-specific complications include pruritus, abdominal pain, xantholasma, and portal hypertension. The latter is often an early feature, as the portal hypertension is presinusoidal in nature and, when present, does not always reflect the presence of cirrhosis. There are many extrahepatic features of PBC, the most common being metabolic, chiefly hypothyroidism and metabolic bone disease. Other common associations are rheumatologic, renal, pulmonary, neuromuscular, and dermatologic. The non-specific yet distressing symptom of fatigue affects up to two-thirds of PBC subjects, but its etiology remains obscure.
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PMID:The clinical expression of primary biliary cirrhosis. 908 8


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