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Prevalence of factor VIII inhibitors among Afghan patients with hemophilia A: a first report.

07:00 EST 1st December 2018 | BioPortfolio

Summary of "Prevalence of factor VIII inhibitors among Afghan patients with hemophilia A: a first report."

: Prevalence of inhibitors in Afghan hemophilia patients has not been reported previously. Our aim was to determine the prevalence of factor VIII inhibitors among hemophilia A patients from the Kabul province of Afghanistan to identify and characterize the pattern of inhibitor formation. Clinical information and blood samples were collected from three hemophilia centers in Kabul, Afghanistan. Plasma samples were obtained from 62 patients with severe (80.5%) and 15 patients with moderate hemophilia A (19.5%) in this cross-sectional study design. All the patients were receiving on-demand treatment. The Nijmegen modification of the Bethesda assay was used to detect inhibitors. Multiplex PCR, inverse-PCR, Multiplex ligation-dependent probe amplification and direct sequencing were performed for genotyping. Inhibitor activity was detected in one out of 15 (6.7%) patients with moderate hemophilia and in six out of 62 (9.7%) with severe disease. Apart from the intron 22 inversion, five different mutations including one missense, two large and two small deletions were detected. This is the first report showing that the prevalence of inhibitors in Afghan hemophilia A patients is much lower than in other populations.

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Journal Details

This article was published in the following journal.

Name: Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis
ISSN: 1473-5733
Pages: 697-700

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Storage-stable blood coagulation factor acting in the intrinsic pathway. Its activated form, IXa, forms a complex with factor VIII and calcium on platelet factor 3 to activate factor X to Xa. Deficiency of factor IX results in HEMOPHILIA B (Christmas Disease).

A non-fibrillar collagen originally found in DESCEMET MEMBRANE. It is expressed in endothelial cell layers and in tissues undergoing active remodeling. It is heterotrimer comprised of alpha1(VIII) and alpha2(VIII) chains.

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