Titlebook: Congenital Bleeding Disorders; Diagnosis and Manage Akbar Dorgalaleh Book 20181st edition Springer International Publishing AG, part of Spr

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https://doi.org/10.1057/9781137388803o confirm the diagnosis. A wide spectrum of mutations has been discovered in ., ., and . encoding the three chains of fibrinogen molecule. Two hotspot mutations are prevalent in afibrinogenemia and dysfibrinogenemia. Management of patients with congenital fibrinogen disorders is challenging and shou

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https://doi.org/10.1057/9781137388803on laboratory tests should be used for diagnose of FII deficiency. FII deficiency is suspected through prolonged prothrombin time (PT) and activated thromboplastin time (aPTT) which was confirmed by a specific FII assay. As no specific prothrombin concentrate is available, prothrombin complex concen

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https://doi.org/10.1057/9781137388803n time (PT) and activate thromboplastin time (aPTT). The diagnosis is confirmed by specific diagnostic tests including FV antigen and activity assays. The only available therapeutic choice for patients with FV deficiency is fresh frozen plasma (FFP): the recommended dose is dependent on the location

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Manning Marable,Vanessa Agard-Joneswhereas for those with more severe hemorrhage, systemic hemostatic agents including platelet concentrates and recombinant human activated factor VII (rFVIIa) are used. Currently, platelet transfusion is the standard treatment, but repeated transfusion can result in allo-immunization and refractorine

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https://doi.org/10.1057/9780230615397more specific laboratory studies such as aggregometry assays, flow cytometry, and molecular analysis can help to precise and timely diagnosis of disorder. BSS treatment includes supportive cares as well as specific treatment of bleeding episodes.