Cases reported "Hepatitis"

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1/40. Fulminant hepatitis is significantly increased in hepatitis B carriers after allogeneic bone marrow transplantation.

    BACKGROUND: bone marrow transplantation (BMT) is effective treatment for many hematologic disease, but performed in a population with a high endemic hepatitis B virus carrier rate, the incidence of liver function impairment and fulminant hepatitis (FH) is expected to be raised. methods: Forty-three hepatitis b virus carriers received high-dose chemotherapy and BMT, 32 patients received an allogeneic graft, and 11 patients autologous marrow. Acute graft-versus-host disease prophylaxis consisted of methotrexate on day 1, 3, 6, and 11 and cyclosporine for 6 months. RESULTS: After a median follow-up period of 68 months (range: 1-11.5 years), 26 (81.3%) allogeneic BMT patients developed impaired liver function (LF), 5 progressed to FH on day 93, 169, 170, 180, and 468, respectively, and died after an average of 13.8 days (range: 1-45 days). Whereas only 4 (36.4%) autologous BMT patients developed impaired LF, and none FH. Impaired LF (P=0.026, chi-square), and FH (odds ratio=12.86, P=0.009 for coefficient) were significantly related to an allogeneic marrow graft, and the timing of liver function impairment coincided with cyclosporine withdrawal. Hepatitis B surface antigen (HbsAg) disappeared from the serum in 4/14 (28.6%) patients receiving a marrow graft from an HbsAg donor. HbsAg was not detected in the serum after BMT in 2/11 (18.2%) autologous BMT patients. CONCLUSIONS: hepatitis b virus carriers receiving a marrow graft from an HbsAg donor have a significantly increased risk of FH.
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2/40. Report of a fatal case of dengue infection with hepatitis: demonstration of dengue antigens in hepatocytes and liver apoptosis.

    A fatal case of dengue (DEN) infection associated with a spleen rupture and with hepatitis is reported here. Microscopic studies showed numerous areas of spleen rupture with hematomas and revealed necrotic foci in liver samples obtained at autopsy. Although hepatitis was reported in several cases of DEN fever, the mechanism of liver injury remains poorly understood. In this case, immunohistochemistry showed that DEN viral antigens were mostly detected in hepatocytes surrounding the necrotic foci. By in situ detection of dna fragmentation, apoptotic hepatocytes were found to be colocated with DEN virus-infected hepatocytes. These findings suggest that hepatocytes are the major sites of DEN virus replication in the liver and that DEN virus induces apoptosis of hepatocytes in vivo.
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3/40. alpha1-Antitrypsin deficiency and liver disease in children.

    This report describes the clinical, biochemical, and hepatic morphologic findings in ten children with severe serum alpha1-antitrypsin deficiency. Genetic protease inhibitor (Pi) phenotyping, using acid-starch gel and crossed antigen-antibody electrophoresis, demonstrated Pi phenotype ZZ in all our cases. In eight patients, manifestations of liver disease appeared during the first year of life. The case reports show that alpha1-antitrypsin deficiency should be suspected in any child with neonatal hepatitis, unexplained hepatomegaly or splenomegaly, or cirrhosis. In our report, one infant is normal at age 6 months, and one infant had progressive hepatic damage that culminated in liver failure and death at age 6 months. The variable clinical course and prognosis for infants with severe alpha1-antitrypsin deficiency is well illustrated by these two infants.
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4/40. Hepato-splenic lymphoma: a rare entity mimicking acute hepatitis: a case report.

    We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented with markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic, autoimmune and metabolic liver diseases were excluded. Bone marrow biopsy showed an intracapillary infiltration of t-lymphocytes with no evidence of lipid storage disease. Because of a progressive spleen enlargement, splenectomy was performed. Histological examination showed lymphomatous intrasinuses invasion of the spleen. Immunohistochemical investigation revealed the T phenotype of the neoplastic cells: CD45 , CD45RO , CD3 , CD4-, CD8-, TIA1-. About 50 % of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatosplenic T cell lymphoma was done. The patient was treated with chemotherapy, which induced a complete remission. Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, thrombocytopenic purpura and blast in the peripheral blood. In spite of autologous bone marrow transplantation, he died twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic T-cell lymphoma should be considered in the differential diagnosis of a patient whose clinical course is atypical for acute hepatic dysfunction.
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5/40. Acute immune complex disease associated with hepatitis. Etiopathogenic and immunopathologic studies of the renal lesion.

    Immune deposit glomerulonephritis has been associated with hepatitis B antigenemia. Immune complexes of this antigen and its antibody have been implicated in the pathogenesis of the renal disease. A boy had acute immune complex disease with glomerulitis in which cryoprecipitable complexes of HbsAg and its antibody were isolated from serum. HbsAg was concentrated in the cryoprecipitate and localized in a granular pattern along the glomerular basement membrane in association with immunoglobulins. Glomerular fixed antibody was eluted and shown to be directed against HbsAg. The level of antibody activity to HbsAg was higher in the eluate than the serum, suggesting immunopathogenic specificity of the antibody. The study demonstrates that the nephritis was mediated by immune complexes of HbsAg and its antibody, and the presence of immunoglobulin on the kidney did not represent trapping from the circulation.
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6/40. Chronic active Epstein-Barr virus infection in an adult.

    We report a rare adult case of chronic active Epstein-Barr virus (EBV) infection. A 54-year-old woman was admitted to our hospital with intermittent fever, weight loss, hepatosplenomegaly, pancytopenia and liver disturbance. In serological tests for EBV, anti-virus capsid antigen (VCA)-IgG antibody and anti-early antigen (EA)-IgG antibody were markedly elevated and anti-EBV nuclear antigen (EBNA) antibody was negative. EBV genome was detected in the bone marrow nucleated cells and peripheral lymphocytes by Southern blot hybridization. The patient developed left facial edema, bilateral breast tumor and pneumonia. She died one year after admission in spite of the administration of prednisolone, interferon and acyclovir.
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7/40. autoantibodies and the spectrum of sjogren's syndrome.

    In studies reported recently, the sera of patients with sjogren's syndrome were found to contain precipitating antibodies to nuclear antigens that can be identified by immunodiffusion analysis. These precipitating autoantibodies have been termed SS-A and SS-B antibodies. We show that identification of these autoantibodies helped in establishing the diagnosis of sjogren's syndrome in 12 of 30 patients in whom the diagnosis had not been considered at the time of the physician's initial examination. The reasons for this were related to lack of spontaneous complaints of keratoconjunctivitis sicca and xerostomia and prominence of symptoms associated with arthritis, myalgia, pulmonary fibrosis, and cardiac disease. This study re-emphasizes the importance of multisystem disease in sjogren's syndrome and shows that specific serologic assays for autoantibodies aided in diagnosis.
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8/40. Autoimmune enteric leiomyositis: a rare cause of chronic intestinal pseudo-obstruction with specific morphological features.

    Autoimmune enteric leiomyositis is an extraordinary rare cause of acquired chronic intestinal pseudo-obstruction in children. We report a 5-year-old girl who developed chronic intestinal pseudo-obstruction 3 years after an autoimmune hepatitis. Mucosal biopsies of the upper gastrointestinal tract and colon showed minimal inflammatory changes. On full-thickness biopsies of the small intestine, a dense lymphocytic infiltrate of the muscularis propria was seen, mainly consisting of cytotoxic T lymphocytes. Smooth muscle fibers were degenerated and diminished, but the myenteric plexus was intact. The coexistence of an autoimmune hepatitis in our case indicates an expansion of autoreactive T cells to homologous self-antigens. It is of practical importance for histopathological diagnosis that inflammation in autoimmune enteric leiomyositis affects the muscularis propria of the small intestine, whereas mucosa and submucosa do not show severe inflammatory changes. Therefore, correct diagnosis may be missed in peroral and peranal mucosal biopsies, but full-thickness biopsies are required.
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9/40. Atypical American visceral leishmaniasis caused by disseminated Leishmania amazonensis infection presenting with hepatitis and adenopathy.

    Leishmania amazonensis is widely recognised as a cause of cutaneous leishmaniasis in latin america, but it can also disseminate to produce atypical visceral leishmaniasis with hepatitis and lymphadenopathy. The patient, an 8-year-old Brazilian boy, presented with a febrile illness and hepatosplenomegaly, elevated liver enzymes and generalised adenopathy. Serological tests using antigens of L. chagasi, the typical cause of visceral leishmaniasis in latin america, were inconclusive. Leishmania amazonensis was eventually isolated in a culture of a lymph node. The patient recovered fully after treatment with meglumine antimoniate. As this case illustrates, L. amazonensis produces a spectrum of disease that includes atypical American visceral leishmaniasis with evidence of hepatocellular injury and generalised lymphadenopathy.
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10/40. Identification of a novel single-stranded DNA fragment associated with human hepatitis.

    By performing nonspecific polymerase chain reaction followed by elimination of chromosome-derived sequences, foreign DNA fragments were obtained from the serum of a patient with non-A-E hepatitis. One of the sequences, named NV-F, contained a partial open reading frame and was detected in 17 (24.6%) of 69 patients with non-A-E hepatitis, including 1 with fulminant hepatitis (vs. in 5 [2.8%] of 180 healthy individuals). A peptide was synthesized accordingly, to detect serum anti-NV-F antibody, which was found in 49 (75.4%) of 65 patients positive for NV-F. This DNA fragment was sensitive to S1 nuclease digestion. cesium chloride gradient analysis revealed that the NV-F-associated particles had buoyant densities of 1.33-1.39 and 1.22-1.25 g/mL. Immunofluorescence analysis revealed that the novel antigen was present in the hepatocytes of patients infected with NV-F. In conclusion, we have identified a novel single-stranded DNA fragment derived from a virus-like agent associated with human hepatitis.
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