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

A 19-year-old woman presented with clinical manifestations of sudden, fulminant thrombotic thrombocytopenic purpura associated with autoimmune hepatitis and autoimmune thrombocytopenic purpura. Although thrombotic thrombocytopenic purpura may, rarely, be associated with systemic lupus erythematosus and other autoimmune diseases, to our knowledge, the syndrome has never been described in association with autoimmune hepatitis. In this patient, too, the etiology of thrombotic thrombocytopenic purpura associated with autoimmune disease remains elusive. The patient was treated with corticosteroid, which brought about no improvement in her condition, and she died of multiorgan failure. Diagnosis is challenging, but prompt diagnosis is necessary because thrombotic thrombocytopenic purpura is a life-threatening syndrome whose prognosis has been improved significantly by early plasmapheresis treatment.
 ...
PMID:Thrombotic thrombocytopenic purpura in autoimmune hepatitis. 1151 38

In children, a type of graft dysfunction associated with autoimmune features has been described. We have identified 7 adult liver-transplant (LT) recipients from a series of over 1,000 consecutive transplant recipients who presented between 0.3 years and 7.2 years following transplantation with characteristic symptoms, autoantibody profiles, and histologic findings of autoimmune disease. The indications for transplantation were Ecstasy overdose, alcohol-related cirrhosis, primary sclerosing cholangitis (PSC) (2), primary biliary cirrhosis (PBC), hepatitis C cirrhosis, and cryptogenic cirrhosis. Two patterns of de novo autoantibody development were noted; anti-liver-kidney-microsome (LKM) antibody development at high titer in association with an aspartate transaminase (AST) > 500 and antinuclear (ANA) and antismooth muscle (AMA) antibody development at titers >1/80 with lower AST levels. All cases had elevated IgG. Liver biopsies showed changes of an autoimmune-type hepatitis with portal and periportal hepatitis in association with a marked infiltrate of plasma cells, lymphocytes, and bridging collapse. Two patients lost their grafts because of the disease. Patients were treated with reintroduction of steroids and azathioprine in cases in which it had been withdrawn. Major histocompatibility class I and II mismatching did not incur risk. Eight of 12 liver allografts were acquired from either DRB*0301- or DRB*0401-positive donors, and 4 recipients were DRB*0301-positive. This series illustrates that both symptoms and histologic findings of graft dysfunction compatible with autoimmune hepatitis (AIH) exist in adult LT recipients. Graft loss may be a consequence. This entity may represent a specific type of rejection that should currently be classified as "graft dysfunction mimicking autoimmune hepatitis."
 ...
PMID:Graft dysfunction mimicking autoimmune hepatitis following liver transplantation in adults. 1152 30

TT virus is a recently discovered virus, of which the pathogenetic potential is still uncertain. The present paper describes the histopathological features of a case of TT virus-related acute recurrent hepatitis. The patient is a 28-year-old woman with no history of drug or alcohol abuse, presenting with repeated episodes of hypertransaminasemia evidenced during the last 4 years. No other markers of viral or autoimmune disease were found. On histological analysis, the liver parenchyma showed a preserved architecture. The histological features were those of a mild acute hepatitis. The clinicopathological findings suggest th
 ...
PMID:TT virus-related acute recurrent hepatitis. Histological features of a case and review of the literature. 1178 46

Graft dysfunction associated with autoimmune phenomena has been recently described in liver transplant recipients without previous autoimmune disease. However, the natural history, diagnostic criteria, and definitive therapeutic approach of de novo autoimmune hepatitis (de novo AIH) are poorly understood. We report 12 cases of de novo AIH 27.9 +/- 24.5 months after liver transplantation: the outcome of 7 patients treated with steroids is compared with a group of 5 nontreated patients. Nontreated patients lost the graft after 5.8 +/- 2.6 months from de novo AIH onset. All treated patients were alive after 48.4 +/- 14 (29-65) months from de novo AIH onset, and none of them lost the graft. However, 5 patients relapsed in relation to steroid tapering. All patients presented an atypical antiliver/kidney cytosolic autoantibody, associated to classical autoantibodies in 10 cases. Histological study showed several degrees of lobular necrosis and inflammatory infiltrate. HLA antigen frequencies and matching were compared with 2 control groups (16 orthotopic liver transplantation [LTX] patients without de novo AIH and 929 healthy blood donors); de novo AIH patients showed a higher prevalence of HLA-DR3 (54.5% vs. 25.9%, P =.04) than healthy controls, which was not observed in LTX patients without de novo AIH. In conclusion, this new disease should be included in the differential diagnosis of unexplained graft dysfunction. In addition, treatment with steroids results in a dramatically improved outcome. However, maintenance therapy is usually required.
 ...
PMID:Response to steroids in de novo autoimmune hepatitis after liver transplantation. 1182 8

Viral etiology of hepatitis is routinely proved by standard immunological tests detecting specific antibodies. However, identification of specific antibodies cannot always be conclusive. Since specific hepatitis C virus (HCV) antibodies may appear after some months of the infection, identification of HCV RNA and/or hepatitis G virus (HGV) RNA should clarify the etiology of hepatitis. The aim of this study was to diagnose etiologically unknown hepatitis by a reverse transcription-polymerase chain reaction (RT-PCR) testing of the presence of HCV RNA and HGV RNA. The study involved 33 children with histologically proved hepatitis. The presence of HCV and any signs of autoimmune disease were not observed at the beginning of the follow-up study. During 2.5 years of the follow-up HCV-RNA was found in the blood and liver biopsies in 17 patients. Eight of them became HCV antibodies-positive during the follow-up. None of them eliminated the virus from the blood during the follow-up. In two other patients HCV-RNA was found only in the liver. HGV infection in all cryptogenic patients was excluded by PCR testing. Identification of HCV RNA RT-PCR allowed to diagnose 19 out of 33 (57.6%) patients with cryptogenic hepatitis. The etiology of the hepatitis in remaining 12 patients has to be established.
 ...
PMID:Significance of molecular identification of hepatitis C virus RNA in diagnosis of cryptogenic hepatitis in children. 1188 34

We prospectively collected data on 1,429 liver transplant recipients between December 1984 and December 1998. Fifty-five patients (3.8%; 10 men, 45 women; median age, 44.5 +/- 13 [SD] years) with autoimmune hepatitis (AIH) underwent orthotopic liver transplantation (OLT). Transplant recipients with AIH were younger, more likely to be women, and had a greater likelihood of rejection in the first 3, 6, and 12 months. There was no difference in patient survival or graft survival. There were 11 biopsy-proven recurrences (1 man, 10 women) of AIH after OLT. Almost half the episodes occurred within the first year after OLT. No patient required re-OLT because of recurrent disease. AIH has an incidence of 4% and a recurrence rate of 20% in OLT. Transplant recipients are more likely to be young women and have an increased incidence of acute cellular rejection (ACR) during the first post-OLT year. Recurrence should be suspected in those with abnormal liver function test results in the absence of ACR, especially during the first year after OLT. We cannot establish with certainty whether the observed process represents recurrence of the original autoimmune disease, an alloimmune phenomenon, or allograft dysfunction mimicking AIH.
 ...
PMID:Incidence and recurrence of autoimmune/alloimmune hepatitis in liver transplant recipients. 1203 82

The role of apoptpsis in the occurrence of massive or submassive liver cell death was investigated. Of liver tissues removed from 49 autopsy cases of acute hepatic failure, 26 cases were fulminant hepatic failure (FHF), 13 were subacute hepatic failure (SAHF) and the remaining 10 were acute-on-chronic hepatic failure (AoCHF). Eleven cases were associated with HBV infection, 2 with HCV, 5 with non-A non-B hepatitis, 4 with drug medication and 4 with autoimmune disorder, while the etiology of 23 cases remained unknown. Examinations by the TUNEL method and immunohistochemistry using anti-Fas, -Bax, and -Bcl-2 antibodies were conducted. Most of the FHF and AoCHF cases and half of the SAHF cases showed massive or submassive hepatic lobular injury histopathologically. TUNEL positive cells were observed frequently, and the grade of lobular injury showed significant correlation with the frequency of TUNEL-positive liver cells. Bax and Fas were expressed on the remaining liver cells, while Bcl-2 was seen on the infiltrating lymphocytes. The frequency of TUNEL-positive liver cells showed a significant correlation with Bax expression and the grades of inflammatory cell infiltration, but a poor correlation with Fas and Bcl-2 expression. The results of the present study suggested that apoptosis plays an important role in massive and submassive hepatic cell death. Fas and Bcl-2 might not be involved in the apoptosis of liver cells, but the findings suggested that Bax might play a role in inducing liver cell apoptosis, though the mechanism could not be explained.
 ...
PMID:Apoptosis in acute hepatic failure: histopathological study of human liver tissue using the tunel method and immunohistochemistry. 1216 Feb 29

Our experience regarding serum soluble interleukin-2 receptor (sIL-2R) measurement as a marker of lymphocyte activation consists of patients with autoimmune disease: 37 with systemic lupus erythematosus (SLE), 23 with autoimmune hepatitis (AIH), 74 with inflammatory bowel disease and six with Wegener's granulomatosis (WG). The influence of immunosuppressive therapy has also been assessed. Serum sIL-2R in SLE is significantly higher than in healthy controls and good correlation is found between sIL-2R and disease activity. Severity of kidney inflammation in lupus nephritis can be reflected by the increased excretion of sIL-2R. It was found that sIL-2R level significantly falls when the disease becomes clinically inactive after immunosuppressive therapy, but in many cases (up to 50%) it does not reach normal levels. The last finding suggests that lymphocyte activation may still be present even though the disease is considered inactive under clinical criteria and support the need of prolonged immunosuppression after the first signs of remission. In AIH the serum levels of sIL-2R are elevated in all patients with active disease; all cases with "highly active" disease have significantly higher concentrations than patients with "mild activity". A good correlation has been demonstrated between elevated serum sIL-2R values and anti-asialoglycoprotein receptor (ASGPR) titer (the specific marker of AIH). The follow-up study showed a significant decrease of both sIL-2R levels and anti-ASGPR titer after 3-9 month immunosuppressive therapy. The findings support that sIL-2R and anti-ASGPR titer could serve as reliable humoral markers for disease-specific activity. Compared with inactive ulcerative colitis (UC) and Crohn's disease (CD), significantly higher levels of sIL-2R were present in the serum of patients with active disease, and in inactive disease than in healthy age-matched controls. Methotrexate (MTX) therapy of patients with refractory UC resulted in sIL-2R decrease at the end of therapeutic period (20 i.m. injections of once a week 25 mg), good responders showing > 50% decrease even at 5-7 weeks of treatment. Serum sIL-2R is elevated in all six patients with WG. Contrary to anti-neutrophil cytoplasmic antibodies (ANCA), sIL-2R remains elevated above cut-off for normal range, despite clinical improvement following immunosuppressive treatment. The last observation suggests that serum sIL-2R is not a good measure of the disease activity and argue for the need of longer immunosuppressive therapy just after the first days of clinical remission.
 ...
PMID:Serum soluble IL-2 receptor as a marker of lymphocyte activation in some autoimmune diseases. Effect of immunosuppressive therapy. 1216 73

Fas antigen (Fas) is a cell surface receptor molecule introducing apoptosis-inducing signals into Fas-bearing cells by stimulation with Fas ligand or agonistic anti-Fas monoclonal antibodies. Fas system has been implicated in the regulation of homeostasis of peripheral T and B lymphocytes including elimination of autoreactive cells, and in the exclusion of tumor and virus-infected cells. Fas system, however, also plays a role in the mechanisms responsible for tissue disruption in tissue-specific autoimmune disease and fulminant hepatitis. In this review, I describe how we prepared the original anti-human Fas monoclonal antibody with associated cell-killing activity, and I propose here a strategy of therapeutic use of a novel anti-Fas monoclonal antibody for autoimmune and other diseases.
 ...
PMID:Death receptor Fas and autoimmune disease: from the original generation to therapeutic application of agonistic anti-Fas monoclonal antibody. 1222 May 52

Autoimmune diseases, especially autoimmune thyroid disease, frequently develop after delivery due to the immune rebound mechanism. Most cases involve transient dysfunction of affected organs. We examined three patients who developed liver dysfunction after delivery. They were all diagnosed with definite or probable autoimmune hepatitis using the scoring system of the International Autoimmune Hepatitis Group. Moreover, all of them had anti-CYP2D6 antibodies detected by a sensitive radioligand assay. Our findings strongly suggest that liver dysfunction is induced by postpartum autoimmune hepatitis, and clinicians should be aware of this disease.
 ...
PMID:Development of liver dysfunction after delivery is possibly due to postpartum autoimmune hepatitis. A report of three cases. 1236 9


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>