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

chronology

An Overview of Hemostasisore blood flow. The fibrinolysis system is one of the most important requirements for clot removal. Procoagulant and anticoagulant elements interacted complicatedly to ensure an effective response to vascular injury that is limited to the injured site.

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松驰

von Willebrand Diseasere available in VWD including VWF/FVIII concentrates, recombinant VWF, desmopressin, and, in some locations, fresh frozen plasma (FFP). With timely diagnosis and appropriate management of the VWD, the burden of the disorder can be alleviated or even eliminated.

细胞

调味品

Congenital Factor II Deficiencyon 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

辩论

incubus

Multiple Coagulation Factor Deficiencyused by defect of a single gene that is implicated in the process or production/activation of multiple factors. The best examples include combined FV and FVIII deficiency (F5F8D) and vitamin K-dependent coagulation factors (VKDCF) deficiency, which have been thoroughly characterized. Isolated combin

乐章

Congenital Factor X Deficiency plasma. Then diagnosis should be confirmed by specific tests including antigenic (FX:Ag) and functional (FX:C) assays. Treatment of FX deficiency is based on replacement therapy by fresh frozen plasma (FFP) and prothrombin complex concentrate (PCC). A single-factor FX concentrate produced from huma

beta-carotene

Congenital Factor XIII Deficiencymmended as the first-line screening test, but a significant number of countries only used clot solubility test. For the treatment of congenital FXIII deficiency, different products are available, but FXIII concentrate is the treatment of choice. For primary prophylaxis, 10–40 IU/kg FXIII concentrate

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Glanzmann Thrombastheniawhereas 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