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)

The authors report the case of a 21 years old man, with sex-linked agammaglobulinemia, who presents a presumed viral hepatitis, followed two years. The evolution, does yet fear a progressive cirrhosis. About this report and analysis of literature, frequency, evolution and pathophysiologic hypothesis, particularly the lymphocyte T role, are also discussed as the problem of hepatitis prevention of these subjects, who receive gammaglobulin injections.
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PMID:[Chronic hepatitis in sex linked agammaglobulinemia. One case (author's transl)]. 8

Patients with agammaglobulinemia are particularly susceptible to chronic enteroviral infections of the central nervous system. Data on 42 patients were obtained by literature review, communications with other physicians, and personal experiences. Thirty-eight patients had congenital immunodeficiencies, most frequently X-linked agammaglobulinemia. Most patients who could be assessed were receiving maintenance therapy with intramuscular gamma-globulin before their enteroviral infection. Seven patients had not been recognized as hypogammaglobulinemic before the onset of infection. The commonest pathogens were echoviruses (37 of 41 cases), especially type 11 (11 cases). Thus far, four patients have had sequential or simultaneous infections with a second enteroviral serotype. Other features of the disease have included weakness, lethargy or coma, headaches, hearing loss, seizures, ataxia, and paresthesias. Some patients have also had nonneurologic manifestations of chronic enteroviral infection, including fever, the dermatomyositis-like syndrome, edema, rashes, and hepatitis. Treatment has consisted primarily of antibody administration, either in intravenous immunoglobulin preparations or in immune plasma. Twelve patients have received intraventricular immunoglobulin through reservoir devices; six of these 12 have improved substantially, as judged by clinical criteria.
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PMID:Chronic enteroviral meningoencephalitis in agammaglobulinemic patients. 329

Human natural immunoglobulin (HunIg) preparation for intravenous use has been used in various diseases. The most typical application of this preparation is agammaglobulinemia. Currently, however, this preparation is being used in the therapy of many other disorders. These include thrombocytopenia, Kawasaki disease, systemic vasculitis, several other disorders of autoimmune origin and systemic inflammation such as sepsis. In some diseases, the clinical improvement following use of HunIg has been dramatic, while in others its effect is not striking. Due to rarity of the side effects, the range of application of HunIg has been recently broadened. Such side effects include transmission of several diseases such as hepatitis and retroviral infections. Before it is recommended for use, however, and primarily due to expense, the efficacy of this drug should be carefully evaluated. The mechanism of action of HunIg is not fully understood. However, it has been suggested that its action may involve blockade of Fc-receptor, an anti-cytokine effect, or inhibition of complement activation. In this review, the mechanism of action of HunIgG and its application in human diseases are discussed.
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PMID:Therapeutic application of intravenous human natural immunoglobulin preparation. 915 42

Hepatitis-associated aplastic anemia (HAA) is characterized by marrow failure developing after acute seronegative hepatitis. A patient with agammaglobulinemia developed HAA in association with HBsAg-negative, hepatitis B virus (HBV) DNA-positive acute hepatitis. Sequence analysis showed several substitutions in the major antigenic determinant of HBsAg, potentially affecting the detection by diagnostic immunoassays. Viral mutants may therefore be implicated as etiologic agents of HBsAg-negative HAA. HBV DNA determination may be necessary to exclude mutant HBV as a cause of HAA, particularly in categories at high risk of mutant selection such as agammaglobulinemic and transplanted patients.
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PMID:Immune-mediated hepatitis-associated aplastic anemia caused by the emergence of a mutant hepatitis B virus undetectable by standard assays. 1731 77