Are Bleeding Scores Predicting Severity and Outcome in Hemophilia and Rare Bleeding Disorders?
نویسندگان
چکیده
منابع مشابه
Rare bleeding disorders.
During the haemostatic response, the formation of a primary platelet plug limits bleeding and provides a surface for clotting factors to assemble and become activated. The initial platelet plug is stabilized by fibrin monomers, covalently cross-linked by FXIII, forming a platelets-fibrin thrombus. Defects in platelets as well as inherited deficiencies of coagulation factors including fibrinogen...
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inhibitor development is a lifelong challenge for patients with bleeding disorders who received replacement therapy. most commonly, inhibitor formation was observed in hemophilia a patients but patients with rare bleeding disorders (rbd) especially patients with deficiency of factor xiii (fxiii) and factor v (fv) can develop an inhibitor against exogenous factors. several factors considered as ...
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Despite the worldwide prevalence of rare bleeding disorders (RBDs), knowledge of these conditions and their management is suboptimal; health care professionals often have little diagnostic and treatment experience with variable access to diagnostic modalities required for accurate identification. Therefore, patients often experience morbidity and mortality due to delayed diagnosis. As RBDs repr...
متن کاملBleeding in carriers of hemophilia.
A wide range of factor VIII and IX levels is observed in heterozygous carriers of hemophilia as well as in noncarriers. In female carriers, extreme lyonization may lead to low clotting factor levels. We studied the effect of heterozygous hemophilia carriership on the occurrence of bleeding symptoms. A postal survey was performed among most of the women who were tested for carriership of hemophi...
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Bleeding symptoms are common in healthy children but occasionally may indicate an underlying congenital or acquired bleeding diathesis. The rare bleeding disorders (RBDs) comprise inherited deficiencies of coagulation factors I (congenital fibrinogen deficiencies), II, V, VII, X, XI, and XIII and combined factor deficiencies, most notably of factors V and VIII and of vitamin K–dependent factors...
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ژورنال
عنوان ژورنال: Blood
سال: 2016
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v128.22.4801.4801