Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 46-year-old patient with the typical biochemical, histological and cholangiographic findings of primary sclerosing cholangitis (PSC) whose clinical and laboratory findings would also qualify her for the diagnosis of definite autoimmune hepatitis (AIH), according to the aggregate score of the International Autoimmune Hepatitis Group. We suggest that this patient may represent an example of the overlapping syndrome of PSC and AIH.
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PMID:Overlap syndrome of primary sclerosing cholangitis and autoimmune hepatitis. 1124 24

Autoimmune hepatitis (AIH) is characterised by the presence of periportal hepatitis coupled with the presence of autoantibodies in the serum. We report our experience with 10 cases (females--8, males--2) who presented to the rheumatology clinic with either articular or extra-articular manifestations. Three patients (1 SLE, 1 Sjogren's and 1 RA), satisfied the criteria for an underlying rheumatic disease (secondary AIH) while, others had primary AIH. Median duration of hepatic involvement was 6 months and the varied presentations were noted. Articular disease ranged from arthralgias, palindromic arthritis to persistent non-erosive/non-deforming arthritis (Jaccoud's arthritis). Autoimmune thrombocytopenia was seen in 2 and autoimmune hypo and hyperthyroidism were seen in 3 patients each. Anti-nuclear antibody was positive in 9/10 (6 with speckled pattern and 3 with homogenous pattern) and anti-mitochondrial antibodies were positive in three. Hepatitis C virus (HCV) markers were positive in 1, who probably had viral hepatitis with dominant autoimmune features. All have been started on steroids (5 patients--1 mg/kg dose, 1 patient--0.5 mg/kg dose and 3 patients--0.25 mg/kg dose). The HCV positive patient was on a low dose steroid (0.25 mg/kg) and interferron treatment was contemplated before she was lost to follow up. Four patients are also on azathioprine in the dose of 2 mg/kg/day. Of the 6 patients who are under regular follow up, the liver parameters have normalised in 5 and one showed hypoalbuminaemia with normal enzyme levels at the last follow up.
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PMID:The spectrum of chronic autoimmune hepatitis. 1127 83

To determine the frequency, risk factors, and consequences of recurrent autoimmune hepatitis after liver transplantation, 41 patients with type 1 disease were monitored after surgery in accordance with a surveillance protocol. Tacrolimus or cyclosporine plus prednisone were administered to each patient, and liver biopsy examinations were performed at least annually according to protocol. Corticosteroid therapy was ultimately discontinued in only 2 patients. Recurrent disease was defined as the presence of lymphoplasmacytic infiltrates in liver tissue in the absence of other causes of allograft dysfunction. Autoimmune hepatitis recurred in 7 patients (17%), and the mean time to recurrence was 4.6 +/- 1 years. Recurrence was asymptomatic in 4 of 7 patients and detected only by surveillance liver biopsy assessment in 2 patients. Histological changes were mild, and there was no progression to cirrhosis during 4.9 +/- 0.9 years of observation. Five-year patient (86% v. 82%; P =.9) and graft (86% v. 67%; P =.5) survival rates were not statistically different between patients with and without recurrent disease. HLA-DR3 or HLA-DR4 occurred more commonly in patients with than without recurrence (100% v. 40%; P =.008) and healthy subjects (100% v. 49%; P =.01). Recurrent disease was unrelated to donor HLA status. In conclusion, recurrence after transplantation for type 1 autoimmune hepatitis is common. Its mild manifestations and favorable prognosis may reflect early detection by a surveillance protocol and/or continuous corticosteroid treatment. HLA-DR3- or HLA-DR4-positive recipients are at risk for recurrence regardless of donor HLA status.
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PMID:Recurrent autoimmune hepatitis after orthotopic liver transplantation. 1130 89

Autoimmune hepatitis (AIH) is a necro-inflammatory disease that, untreated, carries a 3-year mortality rate of approximately 50%. In this last of a series of three articles, current knowledge regarding AIH is reviewed. The mode of presentation of AIH is variable: insidious onset with few if any symptoms, presenting with symptoms indistinguishable from that of any acute viral hepatitis, or onset with fulminate hepatitis. In AIH, hepatocytes become injured by various agents (such as a viral infection) and become antigenic, leading to a self-perpetuating antigen-antibody response with subsequent chronic liver disease. AIH is the only type of hepatitis that is responsive to corticosteroids. Occasionally, AIH develops after liver transplantation.
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PMID:Autoimmune hepatitis. 1131 82

Autoimmune hepatitis (AIH) in humans arises spontaneously in genetically susceptible individuals and is associated with the presence of Th1 cells in the liver. The understanding of AIH has advanced more slowly than that of other organ-specific autoimmune diseases, however, largely because of the lack of an appropriate animal model. We now describe a new mouse model characterized by spontaneous development of necroinflammatory hepatitis that is restricted by genetic background. Mice deficient in the immunomodulatory cytokine TGF-beta1 were extensively back-bred to the BALB/c background. The BALB/c background dramatically modified the phenotype of TGF-beta1(-/-) mice: specifically, BALB/c-TGF-beta1(-/-) mice developed a lethal necroinflammatory hepatitis that was not observed in TGF-beta1(-/-) mice on a different genetic background. BALB/c background TGF-beta1(-/-) livers contained large numbers of activated CD4(+) T cells that produced large quantities of IFN-gamma, but little IL-4, identifying them as Th1 cells. BALB/c background TGF-beta1(-/-)/IFN-gamma(-/-) double knockout mice, generated by cross-breeding, did not develop necroinflammatory hepatitis, demonstrating that IFN-gamma is mechanistically required for its pathogenesis. This represents the first murine model of hepatitis that develops spontaneously, is restricted by genetic background, and is dependent upon the Th1 cytokine IFN-gamma, and that thus recapitulates these important aspects of AIH.
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PMID:Genetic regulation of autoimmune disease: BALB/c background TGF-beta 1-deficient mice develop necroinflammatory IFN-gamma-dependent hepatitis. 1134 67

Autoimmune hepatitis (AIH), a chronic T-cell-mediated liver injury, is treated with corticosteroids with or without Azathioprine. Corticosteroids are not universally effective and have serious side effects. Cyclosporin A was effective in refractory cases. To assess efficacy and safety of Cyclosporin A (Neoral) in induction of remission in AIH patients this study was performed. Nineteen consenting AIH patients (nine treatment-naive) were treated with cyclosporin A in an open label trial and followed for 26 weeks. Liver biopsy was done and hepatitis activity index (HAI) determined at the beginning and end of treatment. Four patients did not complete the study for various reasons. Mean AST and ALT levels decreased from 948.7 +/- 103.5 and 454.8 +/- 354 to 100.6 +/- 111.8 and 78.5 +/- 40.3 (P < 0.03, P < 0.001) respectively. HAI decreased from 15.2 +/- 3.16 to 7.14 +/- 4.01 (P < 0.005). Serum creatinine did not change significantly. In conclusion, low-dose cyclosporin A appears to be safe and effective even in treatment-naive autoimmune hepatitis patients. Randomized controlled trials are warranted.
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PMID:Cyclosporin A is a promising alternative to corticosteroids in autoimmune hepatitis. 1141 11

Autoimmune hepatitis (AIH) is a chronic liver disease of unknown etiology. The incidence of AIH keeps rising, most probably because of increasing availability of the diagnostic tools permitting thorough differential diagnosis amongst liver diseases presenting histologically as chronic active hepatitis. In this review we focus on clinical aspects of autoimmune hepatitis and discuss pathogenesis, clinical features and treatment of AIH.
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PMID:[Autoimmune hepatitis: pathogenesis, clinical features and treatment]. 1147 61

Autoimmune hepatitis is one of the causes of chronic progressive liver disease in childhood. Here we report 14 cases with clinical findings, therapeutic management and prognosis, in order to define the course of the disease. Diagnosis of autoimmune hepatitis was done with the presence of at least one of these autoantibodies; antinuclear antibody, smooth muscle antibody, liver-kidney microsomal type 1 antibody, and perinuclear antineutrophilic cytoplasmic antibody. Patients were seen every 3 to 6 months. After doing a complete physical examination, biochemical parameters and autoantibodies determined at each visit. Mean age at diagnosis was 10.9 +/- 2.6 years (range, 7-15.5 years) and female to male ratio was 1:3. Thirteen patients had jaundice and all had high levels of ALT, AST and gammaglobulin. Hepatomegaly was found in 71.4% and splenomegaly in 64.3% of the patients. All patients were classified as type 1 autoimmune hepatitis. Liver biopsies revealed severe active hepatitis with mononuclear cell infiltration in portal areas, piecemeal necrosis. Drug therapy consisted of prednisone (2 mg/kg/day) per oral at the beginning, and addition of azathioprine (1.5 mg/kg/day) per oral at the 3rd-6th month with slow tapering of prednisone in 12 children. Both drugs were started together to two patients. Follow-up period was 30.7 +/- 15.6 months (range, 12-72 months). Sustained normalization of ALT could not be obtained with tapering doses of prednisone alone. Decrease in ALT levels did not correlate with disappearance of serum autoantibodies. None of the patients showed decompensation of liver disease. Azathioprine administration is necessary to decrease prednisone dose and to maintain a sustained normal transaminase values.
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PMID:Autoimmune hepatitis. 1156 49

The number of patients with autoimmune hepatitis with histological features of acute hepatitis (AIH-AH) has been increasing recently. Here, the clinical features of patients with AIH-AH have been compared with those of patients with AIH with histological findings of chronic hepatitis (AIH-CH) and liver cirrhosis (AIH-LC). The levels of total serum bilirubin (P<0.05) and serum transaminases (P<0.05) were significantly higher in patients with AIH-AH than in patients with AIH-CH and AIH-LC. However, the serum levels of gamma-globulin (P<0.05) and immunoglobulin G (P<0.05) were significantly lower in AIH-AH than in patients with AIH-CH and AIH-LC. The aggregate score according to the criteria of the International Autoimmune Hepatitis Group in 1999 was also significantly lower in AIH-AH patients than in patients with AIH-CH and AIH-LC (P<0.05). Eleven patients with AIH-AH were treated with corticosteroids, however, the clinical response was insignificant in three patients. In summary, it is difficult to diagnose of patients with AIH-AH using the criteria of the International AIH scoring system. We wish that this scoring system would be modified in the near future.
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PMID:Clinical characteristics of autoimmune hepatitis with histological features of acute hepatitis. 1167 6

Autoimmune hepatitis is associated with genes located in the major histocompatibility complex. The search for genes at other loci that may play a role in disease susceptibility and/or severity is an area of active investigation in autoimmune liver diseases. Genes for glutathione-S-transferases, enzymes that are widely distributed and collectively metabolize carcinogens, pollutants, drugs, and a broad spectrum of harmful, foreign compounds have been associated with liver disease. The objective of this study was to search for a relationship between the glutathione-S-transferase Ml null genotype and autoimmune hepatitis using polymerase chain reaction analysis. The findings indicate that the frequency of the null genotype is not increased in patients with autoimmune hepatitis when compared to control subjects. These results coupled with similar ones in primary biliary cirrhosis do not support a role for this mutation in autoimmune liver disease.
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PMID:Glutathione-S-Transferase M1 null genotype in autoimmune hepatitis. 1168 May 79


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