Titlebook: Congenital Bleeding Disorders; Diagnosis and Manage Akbar Dorgalaleh Book 2023Latest edition The Editor(s) (if applicable) and The Author(s

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Cecilia Lwiindi Nedziwe,Oluwaseun Tellarothrombin time (PT) and activated thromboplastin time (aPTT). The diagnosis is confirmed by specific laboratory tests including FV antigen and activity assays. The only available therapeutic choice for patients with FV deficiency is fresh frozen plasma (FFP) in which the recommended dose is depende

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Transnational Actors in Global Governancee limited due to the unavailability of FV concentrates. Patients with F5F8D do not need lifelong hemostatic replacement therapy. Therapy is necessary upon spontaneous or traumatic bleeding, in the perioperative period, during pregnancy and delivery. When planning a family, it is necessary to take in

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https://doi.org/10.1057/978-1-137-48361-4ld bleeds, while in those with more severe hemorrhage, systemic hemostatic agents, including platelet concentrates and recombinant activated factor VII (rFVIIa), are used. Currently, platelet transfusion is the standard treatment, but repeated transfusions can result in alloimmunization and refracto

Armada

An Overview of Hemostasisr injury. This process consists of three main components: the vascular system, cellular components, and non-cellular components. These work together closely to keep the circulatory system in the best condition..Hemostasis is divided into primary and secondary parts. In primary hemostasis, vascular e

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Congenital Bleeding Disorders: Diagnosis and Management mostly in inherited platelet function disorders (IPFDs), to severe life-threatening disorders, notably in factor (F) XIII deficiency. Most of these disorders, which include rare bleeding disorders (RBD) and IPFD, are autosomal recessive disorders. Patients with hemophilia A and B typically have an

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von Willebrand Disease: An Update on Diagnosis and Treatmentis classified into three main types: type 1 and type 3 as (respectively, partial and complete) quantitative deficiency of von Willebrand factor (VWF) and type 2 (with qualitative VWF defects). The bleeding tendency is highly variable, ranging from largely asymptomatic, mainly in mild type 1 VWD, to

SPASM

Hemophilia A: Diagnosis and Management male births, and is caused by a defect or deficiency in coagulation factor VIII (FVIII). Hemophilia A is caused by a variety of mutations in the FVIII gene, the most common of that is intron 22 inversion, which results in severe hemophilia A. The most frequent bleeding sites in hemophilia A patient

Limited

Hemophilia B: Diagnosis and Managementence of ~1 per 30,000 males worldwide, around 3–4 fold less than hemophilia A. Patients with hemophilia B suffer from a bleeding tendency, usually related to the severity of factor deficiency. Recurrent joint bleeds, muscle, and soft-tissue hematoma are frequent in the severe deficiency (FIX <1 U/dL

ALB

Congenital Fibrinogen Disorders, Diagnosis, and Managementype 2 (dysfibrinogenemia and hypodysfibrinogenemia). Classification of hereditary fibrinogen disorders is based not only on the activity (Clauss) and the antigenic fibrinogen levels but on the clinical features and the genotype as well. Diagnosis of dysfibrinogenemia can be challenging, as sensitivi