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)

Twenty-four children and adolescents who have been receiving home treatment for haemophilia A and B, and were followed up for a median period of five years, have been assessed for physical activity, social adjustment, range of joint movement and infection with hepatitis viruses. They were treated with cryoprecipitate from 1972 to 1977, and since then with factor-VIII concentrates. The average dose of factor VIII was 20 units/kg body mass. It was found that there was near normal range of physical activity and school performance, and, in virtually all families, near normal family function could be preserved. Approximately one-third of the patients showed impairment of the normal range of joint movement in flexion and extension. Although there was no clinical evidence of liver disease, elevated aspartate aminotransferase (AST) levels were found in 14 patients. Evidence of past, or present, infection with hepatitis B was found in 19 patients, and of infection with hepatitis A in seven patients. Home treatment is associated with a reduced level of disability from haemophilia, but transfusion therapy continues to be associated with a high rate of liver function abnormalities, probably of infectious origin.
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PMID:Home treatment of haemophilia. A follow-up study. 679 76

Ten cases are reported of short incubation (one to four weeks) non-A, non-B hepatitis occurring after infusion of various preparations of factor VIII concentrates into patients with coagulation disorders. Five patients were symptomatic and, in all, serum transaminase levels were increased for at least six months. These cases of chronic hepatitis exhibited none of the features of autoimmune chronic hepatitis: autoantibodies were negative and serum immunoglobulins were normal. Hepatic histology confirmed acute hepatitis in two cases biopsied early in the illness, and chronic active hepatitis (three) of chronic persistent hepatitis (two) in five cases studied later. Lobular inflammation was a prominent feature in all cases. Other features not commonly associated with type A or B hepatitis included fatty change and damaged bile ducts.
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PMID:Short incubation non-A, non-B hepatitis transmitted by factor VIII concentrates in patients with congenital coagulation disorders. 679 93

A retrospective survey on clinical hepatitis in patients with bleeding disorders was performed. Nine episodes of hepatitis non-A, non-B occurred in 8 out of 20 patients (40%) with mild hemophilia A or von Willebrand's disease, who had been treated with commercial factor VIII concentrates. Only two episodes of hepatitis B occurred during the study period. The non-A, non-B attack rate after the first treatment was 40% with factor VIII concentrate obtained from large plasma pools (= 2,000 donors) including professional plasma donors as compared to 8% after treatment with factor VIII concentrate obtained from smaller (100-250 donors) plasma pools from Scandinavian donors.
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PMID:Acute hepatitis non-A, non-B following administration of factor VIII concentrates. 680 Jan 31

Non-A, non-B hepatitis was transmitted to eight chimpanzees by intravenous inoculation with antihemophilic materials (factor VIII) that had been implicated in transmission of the disease, with acute-phase liver homogenate from an infected chimpanzee, with acute-phase from an infected chimpanzee, or with chronic-phase plasma from infected chimpanzees. All eight animals developed elevated alanine aminotransferase activity, and all demonstrated unique hepatocyte cytoplasmic tubules at some time during the acute phase of disease. The temporal patterns for tubule appearance in hepatocyte cytoplasm, however, were highly variable, even between chimpanzees given similar inocula. Convoluted membranous structures were found occasionally in early or pre-acute-phase liver biopsy specimens. Aggregates of microtubules were also found in hepatocyte cytoplasm in some acute-phase biopsy specimens. No disease-specific nuclear changes or structures, including clusters or crystalline arrays of virus-like particles, were found in any of the serial liver biopsy specimens from chimpanzees with experimental non-A, non-B hepatitis.
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PMID:Temporal patterns of ultrastructural alterations in hepatocytes of chimpanzees with experimental non-A, non-B hepatitis. 680 2

The efficacy of combined beta-propiolactone/ultraviolet irradiation (betaPL/UV) for inactivation of hepatitis B virus in labile blood derivatives has been reviewed. The initial evaluations of these procedures were hampered by inadequate process control that resulted in excessive protein denaturation; furthermore, adequate evaluation of process efficacy for virus inactivation was prevented by the absence of titered hepatitis virus stocks, the lack of an animal model, and the failure to carry out controlled trials. Finally, it was not appreciated that the power of these procedures lay especially in their use in combination. These deficits have now been remedied. To permit quantitation of process efficacy, a regression analysis of the relation between virus dose and incubation period in chimpanzees has been carried out. This has provided a means of estimating virus titer and determining the accuracy of such estimates. The most recent data suggest that betaPL/UV can reduce the titer of hepatitis B virus about 10 million fold (10(-7)). The process efficacy for betaPL/UV followed by the special adsorption procedures used in preparation of a stabilized human serum containing most human serum proteins except for factor VIII, the factor IX complex, fibrinogen, and the lipoproteins was estimated as a 10(8)-fold reduction in virus titer. This degree of virus inactivation should be more than sufficient to sterilize the amounts of hepatitis B virus that could be expected in pooled human plasma that has been screened for hepatitis B surface antigen. Preliminary data also suggest that the betaPL/UV procedure effectively inactivates non-A, non-B hepatitis virus(es).
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PMID:beta-propiolactone/ultraviolet irradiation: a review of its effectiveness for inactivation of viruses in blood derivatives. 682 13

The hepatitis risk of protein fractions derived from pooled human plasma is eliminated by beta-propiolactone treatment and UV irradiation. The combination of this sterilization procedure with an adsorption and elution procedure leads to coagulation factor concentrates such as fibrinogen, factor VIII and factor IX concentrate and a stabilized serum preparation for intravenous use. This technology is carried out mostly at room temperature, without alcohol and minimum pollution. It seems, especially for developing countries, an attractive alternative to the widely used alcohol fractionation.
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PMID:Fractionation of cold-sterilized plasma. A new concept in production of non-infectious plasma proteins. 689 Mar 53

Von Willebrand's disease causes spontaneous bleeding from mucocutaneous surfaces and excessive blood loss after surgery. Some women with the disease have less spontaneous hemorrhage and show improved coagulation while taking oral contraceptives (OCs) or while pregnant; estrogens are believed responsible for these improvements. 3 women are described who had previously received infusions of either fresh frozen plasma or cryoprecipitate to stop postoperative hemorrhage, and exhibited prolonged bleeding times, abnormal platelet retention results, and decreased plasma levels of factor VIII-related coagulant, factor VIII-related antigen, and factor VIII-related von Willebrand factor. However while taking estrogens they experienced cessation of spontaneous bleeding episodes and exhibited normal coagulation test results; all observed less epistaxis and spontaneous bruising during pregnancy. Continuing estrogen therapy, each woman underwent major surgery with no abnormal bleeding an no blood component therapy. 2 other women with type 1 von Willebrand's disease also exhibited improved hemostasis while taking OCs. It is unclear how estrogens stimulate synthesis of the various factor VIII components and for certain women with von Willebrand's disease certain estrogens may improve hemostasis more effectively than others. Estrogens might obviate the need for cryoprecipitate or fresh frozen plasma to assure hemostasis, thereby eliminating the threat of transfusion-induced hepatitis and reducing surgical costs.
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PMID:Estrogens and surgery in women with von Willebrand's disease. 698 97

Liver biopsies were performed in 5 boys aged between 2 and 9 years with severe classical haemophilia who had persistently abnormal liver function tests. Abnormal histology was present in all; 4 had chronic persistent hepatitis and the fifth chronic aggressive hepatitis with early cirrhosis. Evidence of previous hepatitis B infection was present in one patient, 3 had antibodies to hepatitis, A, and 2 had subnormal levels of alpha-1-antitrypsin. Haemobilia occurred as a late complication of biopsy in one. The significance of these findings in young boys is discussed, as is the role of exposure to factor VIII containing blood products. It is concluded that cryoprecipitate should be used in preference to large pool factor VIII concentrates in children with haemophilia.
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PMID:Liver disease complicating severe haemophilia in childhood. 743 4

An assessment of the immune status of both HIV-positive and HIV-negative haemophiliacs showed hypo-responsive and anergic patients in both groups. In HIV-positive patients, the immune suppression, in addition to HIV itself, may be caused by other viral infections such as all types of hepatitis as well as by factor VIII concentrates. The introduction of new technologies for preparing factor VIII concentrates led to the question of whether higher purity is associated with a protective effect on the immune system, which might improve the clinical course of HIV-positive patients. Factor VIII from two manufacturing processes was investigated, one prepared using an ion-exchange S/D step, and the other utilizing a monoclonal antibody step. It is concluded that changing the product, from the monoclonal antibody to the ion-exchange and vice versa in the HIV-negative patients does not cause a change in the immune profiles.
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PMID:Prospective clinical trial of high-purity factor VIII preparations in haemophiliacs. 749 75

Inherited coagulation protein deficiencies associated with bleeding diatheses may present with spontaneous bleeding early in life, or may not be recognized until the development of hemorrhage after trauma or surgery. Diagnostic evaluation with coagulation screening tests, followed by confirmation with coagulation factor assays, is essential for appropriate management. For moderate-to-severe hemophilia, treatment includes coagulation factor replacement with purified, plasma-derived coagulation factor, or in the case of hemophilia A, factor VIII concentrate produced with recombinant techniques. Increased use of pharmacologic agents such as desmopressin acetate for patients with mild hemophilia A or type 1 von Willebrand's disease has allowed physicians to treat patients without the risk of infectious complications from plasma-derived factor concentrates. In addition to the management of the inherited bleeding disorders, patients may also require management of human immunodeficiency virus infection, hepatitis, and coagulation factor inhibitors. Issues for the coming years will include continued work to ensure product safety, the role of prophylactic treatment to prevent longterm disabilities, and the application of gene therapy to the management of bleeding disorders.
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PMID:Treatment of inherited coagulation disorders. 887 17


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