Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019163 (hepatitis B)
 38,309 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ninety-five patients with Hemophilia A, B and von Willebrand's disease followed over a three year period were evaluated for liver disease. Half of the patients were treated episodically and half received additional prophylactic treatment. Fifty-five per cent have significant transaminitis (elevated SGOT-SGPT), 42 per cent have biochemical evidence of chronic active liver disease and two per cent have subacute and active cirrhosis. There is an annual attack rate of Hepatitis B disease of 3.5 per cent and Hepatitis B antibody titres are present in 84 per cent. This asymptomatic liver disease requires close monitoring for clinical significance.
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PMID:Liver dysfunction in patients with hemophilia A, B, and von Willebrand's disease. 30 96

A multicenter prospective study was carried out to evaluate whether a vapor-heated factor VIII concentrate transmitted blood-borne viral infections over a surveillance period of 15 months. Thirty-five patients with hemophilia and von Willebrand disease who had never received any blood components were treated. Twenty-eight were analyzed and found not to have non-A, non-B hepatitis. Sera from 20 of these 28 patients were also tested for the antibody to the hepatitis C virus. None had sero-converted during the follow-up period. None of the patients analyzed developed markers of the hepatitis B virus (n = 17) or the human immunodeficiency virus (n = 31). This vapor-heated factor VIII concentrate carries a low risk of transmitting hepatitis and human immunodeficiency virus infection.
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PMID:Low risk of viral infection after administration of vapor-heated factor VIII concentrate. International Investigator Group. 131 76

Hemophilia A and von Willebrand's disease are hereditary disorders associated with qualitative and quantitative abnormalities of clotting factor VIII. A major clinical feature is excessive or abnormal bleeding often necessitating the use of transfusions of pooled blood products to achieve hemostasis. Exposure to blood products places the recipient at risk for infection by the hepatitis B virus or the human immunodeficiency virus. A synthetic analog of arginine vasopressin, 1-desamino-8-D-arginine vasopressin, has been shown to increase the plasma levels of factor VIII coagulant activity and von Willebrand's factor, and clinically to improve abnormal bleeding, obviating the need to use blood products.
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PMID:DDAVP in the treatment of bleeding disorders. 304 85

Factor VIII deficient plasma was made from pooled, HIV antibody and hepatitis B antigen screened, normal human plasma by cryoprecipitation and immuno-depletion, using three different monoclonal antibodies bound to Sepharose columns, in series. These monoclonal antibodies are specific respectively for von Willebrand factor, factor VIII heavy chain and factor VIII light chain. The immunodepleted plasma contained less than 0.002 u/ml factor VIII coagulation activity (VIII:C) less than 0.0001 u/ml von Willebrand factor antigen and 1-2 g/l fibrinogen, while the levels of other clotting factors were unchanged. This immunodepleted plasma was compared with commercial factor VIII deficient plasma obtained from a severe haemophilia A patient as substrate in the one-stage factor VIII assay. Plasmas obtained from 20 normal subjects and 28 patients with von Willebrand's disease or haemophilia A were assayed for VIII:C using the two substrates. The results were very highly correlated (r = 0.96). The columns have high capacity and can be regenerated at least 10 times. Large-scale production of a substrate for factor VIII assays free of virus contamination is now feasible.
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PMID:Production of factor VIII deficient plasma by immunodepletion using three monoclonal antibodies. 311 89

Liver function was studied in 139 of 291 haemophiliacs known to a single Regional Haemophilia Centre including patients with classical haemophilia, Christmas disease and von Willebrand's disease. In 57 patients, six-monthly liver function tests over a five year period were also available. Thirty-nine of the 139 patients had had jaundice or hepatitis and 56 had a positive test for HBsAb in the blood although few of these had had an identifiable clinical illness. Fifty-eight haemophiliacs had elevated serum aminotransferases at the time of study, but the five year review revealed only six patients who had had persistently abnormal results, although none had clinically evident liver disease. Liver dysfunction was unrelated to a history of hepatitis, to a positive HBsAb test, or to age, type of haemophilia, factor level or frequency of factor replacement treatment. Abnormalities of liver function in haemophilia appear to be unrelated to past or present hepatitis B infection in most cases and may not be related to any single transmitted infectious agent.
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PMID:Liver dysfunction in haemophilia. 373 67

We suspected a patient attending our Haemophilia Centre had developed Acquired Immunodeficiency Syndrome (AIDS) and therefore immunological evaluation was performed on 43 patients with haemophilia and von Willebrand's disease attending the Centre. The index patient died of Pneumocytis carinii pneumonia. Thirty-one patients had either abnormal T cell subsets or helper/suppressor ratios. Thirty-two patients had hypergammaglobulinaemia. There was no direct correlation between these immunological abnormalities and the total amount or type of treatment received. T cell abnormalities were not confined to the 13 patients who had received the same batches of concentrate as the index case. The index case simultaneously contracted hepatitis B. He was the only patient to receive a large amount of the suspect batches of concentrate, not previously immune to hepatitis B.
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PMID:AIDS surveillance in haemophilia. 391 59

Seventy patients with Haemophilia A, B, von Willebrand's disease and Factor V deficiency had their liver functions studied. Twenty-five patients (36%) were found to have significant "transaminitis" (elevated SGPT/SGOT). Nine patients (13%) had positive Hepatitis B surface antigen (HBsAg). The incidence of Hepatitis B surface antibody (anti-HBs) in the study group was 74%. All patients were asymptomatic at the time of study. This asymptomatic liver dysfunction will require close monitoring for clinical significance.
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PMID:Liver dysfunction in haemophilia A, B and other hereditary haemorrhagic disorders. 633 8

Sera from 63 patients with haemophilia A, 21 with haemophilia B and 29 with von Willebrand's disease were screened for the presence of circulating immune complexes (CICs), serological markers of hepatitis A and B virus, autoantibodies and factor VIII or factor IX inhibitors. CICs were detected by the 125J Clq binding assay (ClqBA), the solid phase conglutinin assay (KgBSP) and the solid phase Clq assay (ClqSP). The incidence of CICs detected by the ClqBA and the ClqSP methods in haemophiliacs and in von Willebrand patients was higher than that observed in normal subjects, while the prevalence of CICs detected by the KgBSP method was not. The presence of CICs was not correlated with patient age, severity of disease, presence of hepatitis B virus serological markers, abnormal liver function tests or factor VIII inhibitors. A significant connection was demonstrated between CICs detected by the ClqBA method and replacement therapy when the dose administered over 1 year was over 20 000 U of factor VIII or IX concentrates. The high proportion of CICs in von Willebrand's disease, not connected with the replacement therapy or the presence of serological markers of hepatitis virus, is in agreement with the possibility that immune complexes may be related to the disease itself and independent, at least in part, of exogenous agents.
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PMID:Circulating immune complexes in haemophilia and von Willebrand's disease. 660 14

The epidemiology of viral hepatitis and liver function were studied in a retrospective survey of 69 patients with moderate and severe hemophilia A and B, and with severe von Willebrand's disease. Forty-nine patients were on prophylactic self-therapy and 20 on episodic treatment by medical personnel. Serologic markers of viral hepatitis (HBsAg, anti-HBs, anti-HBc, anti-HAV, and in some cases HBeAg and anti-HBe) and liver function tests (ASAT, ALAT, IgG) were followed for up to 12 years. There was a history of clinical hepatitis in 19%, and 96% showed some serologic evidence of exposure to hepatitis B virus. Only one patient was a HBsAg carrier. The prevalence of elevated ASAT and/or ALAT was 65% and the incidence 96%. In 68% of the patients there had been a transaminase elevation for more than 6 months. The clinical picture, serologic markers or liver function tests showed no significant difference between the types of hemophilia, amounts and modes of therapy, or age groups. The chronic hepatitis seen in our hemophiliacs seemed to be a slowly or non-progressive disease.
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PMID:Hepatitis, epidemiology and liver function in hemophiliacs in Sweden. 661 Feb 81

Circulating immune complexes (ICs), assayed by the L1210 enzyme-linked immunoassay, were detected in 48% of patients with hemophilia. A, 50% of patients with von Willebrand's disease, and in none of our patients with hemophilia B. Eighty-five % of the hemophilia A and B patients had mild to moderate disease with only one patient demonstrating a circulating inhibitor. No correlation was found between IC levels and hepatitis B infection, SGOT, disease severity, total quantity of factor VIII or IX infused, time interval from list infusion, or rheumatoid factor positivity. Although the nature of the ICs is not known, the similarity of IC levels between hemophilia A and von Willebrand's disease is discussed with regard to antibodies generated to non-procoagulant portions of the factor VIII molecule.
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PMID:Immune complexes in hemophilia. 679 22


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