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)

The efficacy and viral safety of a pasteurized, immunoaffinity-purified procoagulant factor VIII protein (FVIII:C; Monoclate-P) was studied in two multicentre, prospective, open-label trials in 30 previously untreated patients, 18 with severe (< 1% FVIII:C activity), and 12 with moderate (1% to 5% FVIII:C activity) haemophilia A. Clinical assessments, performed at screening and regularly thereafter for 6 to > 24 months (maximum 34 months), showed that none of 24 assessable patients acquired illnesses consistent with monitored transfusion-transmissible diseases. No patients acquired hepatitis B surface antigen, or antibodies against hepatitis B core antigen, hepatitis C, or human immunodeficiency virus. Likewise, no patients acquired treatment-related hepatitis A antibodies or sustained elevations of alanine aminotransferase levels. The safety profile for Monoclate-P is brought about by a multi-step safety system that incorporates viral inactivation (through a combination of immunoaffinity chromatography and pasteurization) plus donor screening, plasma testing, and quality assurance. The inhibitor development rate (13% low titre, 10% high titre) was similar to that reported in the literature for other FVIII concentrates (24% to 52%). The most frequently reported adverse events were related to typical infant and childhood diseases. Monoclate-P was effective in all patients treated according to protocol, except in two, who developed inhibitors.
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PMID:Viral safety of a pasteurized, monoclonal antibody-purified factor VIII concentrate in previously untreated haemophilia A patients. 1126 Feb 73

A comprehensive survey concerning the Shiraz Hemophilia Society and the associated haemophilia treatment centre was undertaken in April 2002 to collect data on demographics, signs and symptoms in the southern Iranian population with haemophilia and allied disorders. The total number of patients with coagulation disorders was 367. Haemophilia A (factor [F] VIII deficiency) was found in 271, 39 had haemophilia B (FIX deficiency) and 24 had von Willebrand disease. The rare coagulation disorders (n = 33) included 11 patients with FX deficiency; 10 with FVII; six with FXIII; two with afibrinogenaemia; two with FXI; one with combined FVIII and FV; and one with combined FVII, FVIII and FIX deficiency. The prevalence was 6.64 per 100,000 inhabitants. The most common symptoms were haemarthrosis, haematomas and epistaxis. None of the patients were human immunodeficiency virus positive but 47 (15%) were hepatitis C virus positive and two (0.7%) were hepatitis B positive, so that the rate of transfusion-transmitted infections was lower compared with other populations.
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PMID:Inherited coagulation disorders in southern Iran. 1241 Jun 41

One of the largest therapeutic problem during the continuous treatment of the patients with Hemophilia A and B, are viral infections as Hepatitis B and C, and HIV, and the other infective diseases, which can be transmitted by the transfusion of blood products. The aim of this study is to analyze the complications of the hemophiliacs in Kosovo which have been treated with fresh frozen plasma, cryoprecipitate and concentrated products of FVIII and FIX. We have tested 75 patients with hemophilia A or B and there were used enzyme immunoassay test-Elisa method for the following: anti-HCV, HBsAg, HIV and TPHA.The serological data showed that HCV infection was positive in 29 cases or 38,7%, whereas infection with HBV and HIV were present in a smaller percentage of the patients (2,7% HBV and 1,4% for HIV). HCV infection was present only in 9,5% of the cases of the age group under 18 years. Infected hemophiliacs with one or two infective agents were found in 34,7%, respectively 4%. Infection with T. pallidum was present at none of the examined patients with hemophilia. HCV infection was higher in severe forms of hemophilia B (44,4%), compared with severe form of hemophilia A (30%).Based on our results, despite the infrequent application of FVIII and FIX concentrates, and other anti hemophilic preparations used in treating hemophilia patients, the number of infected hemophiliacs with blood-transmittable infectious agents was substantially high, especially with hepatitis C virus.
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PMID:Transfusion-transmitted infections in haemophilia patients. 2000 91

The current standard of care treatment for severe hemophilia A and B (SHA and SHB) is the prophylactic intravenous replacement of coagulation factor VIII or IX (FVIII/FIX) to prevent spontaneous bleeding. Persons with hemophilia without prophylactic treatment receive therapy in case of bleeding, i.e., on demand. To assess treatment patterns, utilization of products, and bleeding outcomes in a real-world cohort of persons with SHA and SHB, defined as FVIII or FIX activity < 1%, data was retrospectively collected from hemophilia-specific patient diaries used for home treatment, medical records, and entries into the Austrian Hemophilia Registry from the year 2012 to 2017. Fifty-three male persons with SHA (n = 47) and SHB (n = 6) were included; 26 with SHA and 5 with SHB were on prophylaxis, 8 and 1 switched therapy regimen, and 13 and 0 received on-demand therapy. Persons on prophylaxis used a mean factor FVIII or FIX dose of 71.7 and 40.1 IU/kg/week. Median (IQR) annualized bleeding rates (ABR) in SHA were 28.0 (23.4-31.3) in the on-demand, 4.9 (1.6-13.5) in the prophylaxis group, and 3.0 (2.0-6.8) in the prophylactic group of SHB. Three persons with SHA had zero bleeds during the observation period. On-demand therapy and hepatitis B and C were associated with higher ABR but not age, weight, and HIV positivity. Bleeding rates and the proportion of on-demand therapy in persons with hemophilia were high in our real-world cohort. Further improvement is needed, which might be facilitated with the advent of factor products with extended half-life or non-factor therapies.
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PMID:Treatment patterns and bleeding outcomes in persons with severe hemophilia A and B in a real-world setting. 3291 14


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