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)

247 liver transplantations in 210 patients were done between June 1983 and April 1991 in our hospital. In this paper we have analyzed our experience with adult liver transplantation from the view point of quality of life after liver transplant. Indications for liver transplantation were the following: 46 patients with cirrhosis due to non A, non B hepatitis, 29 with alcoholic cirrhosis, 13 with hepatitis B, 34 with primary biliary cirrhosis, 26 with primary sclerosing cholangitis, 29 with acute hepatic necrosis, 10 with liver tumor, 13 with miscellaneous other diseases. Of the 210 patients, 125 are alive to date. One, three and five year survival rates (excluding perioperative death) of all patients were 80.2%, 70.6% and 65.45% respectively. The best rehabilitation is in 73 patients who are full time workers or full time students and in 16 patients who are homemakers. The degree of rehabilitation in these patients is very high: 6 patients died after achieving complete rehabilitation; 11 patients retired from employment after liver transplantation; 8 patients are chronically disabled; 79 patients died before rehabilitation could be achieved. Rehabilitation rates of one and five year survivors are 78% and 84.2% respectively. Quality of life has been satisfactory following liver transplantation.
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PMID:[Quality of life following liver transplantation]. 833 22

To evaluate the diagnostic significance of neutrophil cytoplasmic antibodies in chronic liver diseases, we assessed the prevalence of neutrophil cytoplasmic antibodies in autoimmune liver diseases, in particular in primary sclerosing cholangitis, autoimmune chronic active hepatitis and primary biliary cirrhosis, and we also determined the specificity of perinuclear-pattern neutrophil cytoplasmic antibodies for these autoimmune liver diseases by testing sera from patients with nonautoimmune chronic liver diseases. Neutrophil cytoplasmic antibodies were detected in 79% of sera from patients with primary sclerosing cholangitis (n = 24), in 88% of sera from patients with autoimmune chronic active hepatitis (n = 24) and in 28% of sera from patients with primary biliary cirrhosis (n = 25). The presence of neutrophil cytoplasmic antibodies in these diseases correlated significantly (p < 0.008) with the presence of cirrhosis. Neutrophil cytoplasmic antibodies were not detected in nonautoimmune liver diseases. All neutrophil cytoplasmic antibody-positive sera produced a perinuclear fluorescence pattern on ethanol-fixed granulocytes. On neutrophils fixed with paraformaldehyde, a granular cytoplasmic immunofluorescence pattern was observed, demonstrating the cytoplasmic nature of the antigen or antigens involved. Further characterization studies showed that neutrophil cytoplasmic antibodies in autoimmune liver diseases are not directed against myeloperoxidase, proteinase 3 or elastase, the neutrophil cytoplasmic antibody specificities associated with necrotizing vasculitis, glomerulonephritis or both. On Western blots neutrophil cytoplasmic antibodies in autoimmune liver diseases showed reactivity with either lactoferrin, a 67/66-kD protein combination or a 40-kD polypeptide. Reactivity with either of these proteins was observed in sera from patients with primary sclerosing cholangitis (38%), autoimmune chronic active hepatitis (17%) and primary biliary cirrhosis (20%).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Prevalence and characterization of neutrophil cytoplasmic antibodies in autoimmune liver diseases. 844 14

An association between primary sclerosing cholangitis (PSC) and chronic ulcerative colitis (CUC) is well known in Western countries, but there have been no reports on this association in Japan. We reviewed 163 consecutive CUC patients (91 males and 72 females) diagnosed from 1984 to 1990 at Tokyo Women's Medical College. Abnormal liver function tests were found in 42 patients with CUC (25.8%), but chronic liver disease was only diagnosed in seven patients (4.3%). Among these seven patients, there were four with PSC, one with small-duct PSC, one with transfusion-associated chronic hepatitis and one with Type B liver cirrhosis. No relationship was found between the documented colonic manifestations of CUC and the presence of PSC. The four PSC patients did not have a longer history of CUC at the time of diagnosis of PSC than CUC patients without PSC. At the time of PSC diagnosis, two patients were asymptomatic, one presented with right upper quadrant pain, and the other had fatigue. Three patients were diagnosed as having CUC before the onset of PSC (range 2-13 years), and the other patient had both diseases simultaneously. All four had a good prognosis. Thus PSC was the most common chronic liver disease associated with CUC in our series, and it was present in all our CUC patients with alkaline phosphatase levels exceeding twice the upper limit of normal and mild transaminase elevation.
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PMID:Prevalence of primary sclerosing cholangitis and other liver diseases in Japanese patients with chronic ulcerative colitis. 847 52

Primary sclerosing cholangitis (PSC) is an inflammatory disease of unknown etiology. It affects the biliary system and is characterized by fibrosis and progressive obliteration of extrahepatic and intrahepatic bile ducts. Its frequent coexistence with antibody-mediated diseases, certain human leukocyte antigens, and altered lymphocyte mechanisms suggests a strong autoimmune and genetic etiology. Other conditions such as recurrent cholangitis, acquired immunodeficiency syndrome, previous bile duct surgery, bile duct anomalies, or biliary stone disease may result in similar clinical patterns and radiologic profiles. PSC can be asymptomatic and slowly progressive over years, or it may develop rapidly with an inexorable progression to cirrhosis and portal hypertension. Clinical and biochemical abnormalities may suggest PSC, but it is the characteristic radiological pattern that secures the diagnosis. Once viewed as a rare entity, the widespread use of radiological and endoscopic methods have increased physician awareness. There is no successful medical treatment for PSC. While traditional surgical approaches have been directed at the relief of cholangitis and biliary obstruction, liver transplantation has emerged as definitive therapy for patients with PSC complicated by cirrhosis or recurrent bouts of cholangitis.
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PMID:Primary sclerosing cholangitis. 847 44

Hepatobiliary diseases are frequently associated with ulcerative colitis. The most commonly reported syndromes are primary sclerosing cholangitis, chronic active hepatitis and cirrhosis. Recently, the association of primary biliary cirrhosis and ulcerative colitis has been reported. We present a case of primary biliary cirrhosis in a young man with left-sided ulcerative colitis and review the previous reported cases, noting some unique similarities between them.
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PMID:Primary biliary cirrhosis associated with ulcerative colitis. 850 94

A total of 114 liver transplantations were performed in 106 patients in Norway during 1984-1994. Survival after one year was 65% and after three years 57%. The most frequent causes of death were infections and rejections. The survival rate improved considerably during the period, and after 1990 the 1 year survival was 70%. Approximately 2/3 of the patients return to work or education. Very few patients die later than 12 months after the transplantation. The most frequent indications were primary biliary cirrhosis, metabolic liver disease, primary sclerosing cholangitis, autoimmune cirrhosis and fulminant liver failure. The number of liver transplantations (approximately 4 per million inhabitants) is lower in Norway than in the other Nordic countries. The number should be increased to 7-8 per million inhabitants.
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PMID:[Liver transplantation in Norway. Results after 10 years and 114 transplantations]. 855 29

A retrospective analysis was undertaken to determine if the incidence, timing, and severity of acute and chronic rejection were influenced by the primary disease necessitating transplantation. Of the 875 liver transplantations performed between 1984 and 1992, 768 were primary transplantations and 107 were retransplantations. Among the former, 330 patients that were liver transplant recipients for a chronic liver disease without cancer in the native liver received an ABO-compatible and cross-match-negative graft and were given a cyclosporine- or tacrolimus-based immunosuppression. These included primary biliary cirrhosis (PBC, 66 patients), primary sclerosing cholangitis (PSC, 23 patients), alcoholic cirrhosis (ALC, 21 patients), autoimmune cirrhosis (AIC, 17 patients), hepatitis B virus-induced cirrhosis (HBV-C, 116 patients) and hepatitis C virus-induced cirrhosis (HCV-C, 87 patients). The incidence of acute (48% +/- 3% [SE] at 1 year) and chronic rejection (10% +/- 2% at 3 years) was comparable in patients who have undergone transplantation for PBC, PSC, AIC, and HCV-C. However, the incidence of acute (but not chronic) rejection was significantly lower in patients who have undergone transplantation for ALC (29% at 1 year). This reduced incidence of acute rejection was associated with an increased incidence of bacterial infections. In patients who have undergone transplantation for HBV-C (the majority of whom had received long-term anti-hepatitis B surface antigen [HBs] immunoglobulins), the incidence of both acute (21% at 1 year) and chronic rejection (0% at 3 years) was significantly lower, whereas the incidence of septic complications was comparable with that in the other groups. The incidence of acute rejection in patients who have undergone transplantation for nonviral disease receiving polyclonal human anti-cytomegalovirus (CMV) immunoglobulins was also significantly lower than that of patients who did not receive the immunoglobulins (19% vs. 48% at 3 months; P = .01), and this was identical to that of patients who have undergone transplantation for viral disease receiving polyclonal human anti-HBs immunoglobulins (19% at 3 months). These results show that the risk of rejection is unequal among patients, being lower in patients who have undergone transplantation for ALC (probably as a result of a state of nonspecific hyporesponsiveness) and in patients who have undergone transplantation for HBV-C (possibly as a result of long-term administration of polyclonal human immunoglobulins).
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PMID:Incidence of rejection and infection after liver transplantation as a function of the primary disease: possible influence of alcohol and polyclonal immunoglobulins. 1100 37

Secretin, a gastrointestinal hormone, has been shown to have a potent choleretic effect. Having already obtained some beneficial effects with secretin in patients with intrahepatic cholestasis, we sought to confirm its effects in a double-blind placebo-controlled study in patients with mild jaundice after acute or during chronic hepatitis, where total bilirubin level was in excess of 4.0 mg/dl for 3 days or more. Patients with primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and familiar hyperbilirubinemia were excluded from the study. Ninety-three patients were included in this analysis, but the final evaluation covered 69 of them. No statistically significant differences were found in the reduction of serum bilirubin levels between secretin and placebo groups. As a number of patients with liver cirrhosis had been included, the subjects were subdivided into one group with cholestasis in hepatitis and one with liver cirrhosis. In the subgroup of cirrhotic patients who received secretin, serum levels of AST were significantly increased compared with the placebo group. However, since the choleretic effect of secretin is unique, further studies seem to be warranted.
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PMID:Therapeutic effect of secretin in patients with jaundice; double-blind placebo-controlled multicentric trial. 872 32

A 43-year-old white female underwent orthotopic liver transplantation in 1992 for cirrhosis related to primary sclerosing cholangitis. Pre-transplantation protein S (PS) studies revealed a discrepancy between PS activity and free PS antigen consistent with type II PS deficiency. Since the presence of activated protein C (APC) resistance has been reported to interfere with PS activity assays resulting in an apparent type II PS deficiency, we retrospectively tested a pre-transplantation frozen plasma sample for APC resistance. The sample was found to have an abnormal APC resistance ratio (APCR-R) of 1.71. Follow up testing 2 1/2 years post-transplantation revealed correction of the APC resistance phenotype (normalization of the APCR-R to 2.79). Analysis of DNA extracted from lymphocytes revealed the patient to be heterozygous for the FV mutation associated with APC resistance (FV Leiden). Hereditary APC resistance was confirmed by family studies which revealed the presence of APC resistance and heterozygous FV Leiden in her son. Although the patient's post-transplantation plasma FV is normal, her platelet FV remains heterozygous for FV Leiden. To what extent, if any, platelet FV Leiden in the absence of plasma FV Leiden may contribute to a predisposition to thrombosis is unknown.
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PMID:Phenotypic correction of activated protein C resistance following orthotopic liver transplantation. 884 65

To dispel the common notion that the practice of hepatology in North America largely consists of the care of middle-aged male patients with alcohol-induced liver disease, and, in the process, provide undergraduate and postgraduate students with a clearer picture of what patient profiles might resemble in an urban, hospital-based hepatology practice, 1,226 charts derived from referrals between July 1, 1987, and January 1, 1994, were retrospectively reviewed for the following information: year of referral, age and sex of the patient, practice of the referring physician, status of ongoing care, and principal diagnosis. The results of the study revealed the following: 1) referrals for assessment and care of patients with liver disease are increasing at a rapid rate, 2) the majority of referred patients are between the ages of 25 and 45 years, 3) there is an equal distribution of men and women, 4) referrals are most often from general practitioners (57%) and internists (27%), 5) turnover is common, with 56% of referred patients no longer being followed, and 6) a variety of liver disorders are seen, including acute and chronic viral hepatitis (47%), nonalcoholic steatohepatitis (11%), drug-induced liver disease (6%), alcoholic liver disease (5%), primary biliary cirrhosis (4%), primary sclerosing cholangitis (3%), autoimmune chronic hepatitis (3%), "cryptogenic" cirrhosis (3%), and miscellaneous or undiagnosed causes (20%). Thus, contrary to popular belief, in this urban, hospital-based practice, the majority of liver disease patients are not middle-aged alcoholics, but rather young adults with a variety of hepatobiliary disorders.
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PMID:Clinical hepatology: profile of an urban, hospital-based practice. 914 59


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