منابع مشابه
Rare bleeding disorders: diagnosis and treatment.
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 کاملRare 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...
متن کاملrare bleeding disorders: a narrative review of epidemiology, molecular and clinical presentations, diagnosis and treatment
rare bleeding disorders (rbds) are a heterogeneous group of disorders including different types of coagulation factor deficiencies. the disorders are inherited in an autosomal recessive manner with different frequencies varying from 1:500000 to 1:2000000. patients affected with rbds are presented with a wide spectrum of clinical manifestations ranging from mild to life threatening bleeding diat...
متن کاملDiagnosis and Treatment of Benign Bleeding Disorders
M any common bleeding disorders will be revealed through a careful examination of patient history and a mixing study, according to Ravi Krishnadasan, MD, of the University of Arizona, Tucson, Arizona. Benign bleeding disorders are understood in the context of the two main components of hemostasis: primary and secondary hemostasis. Primary hemostasis is the formation of the platelet plug, which ...
متن کاملDiagnosis and Treatment of von Willebrand Disease and Rare Bleeding Disorders
Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF), represents the most frequent bleeding disorder, mostly inherited as an autosomal ...
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ژورنال
عنوان ژورنال: Russian Journal of Pediatric Hematology and Oncology
سال: 2021
ISSN: 2413-5496,2311-1267
DOI: 10.21682/2311-1267-2020-7-4-71-76