منابع مشابه
Congenital bleeding disorders.
Both clinical and basic problems related to the congenital bleeding disorders continue to confront hematologists. On the forefront are efforts to bring genetic correction of the more common bleeding disorders such as hemophilia A to the clinic in a safe and accessible manner. A second issue, particularly for patients with hemophilia, is the development of inhibitors-questions of how they arise ...
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 کاملOutcomes of Congenital Bleeding Disorders
Result: Thirty-one patients (48.4%) were diagnosed with hemophilia A,eighteen patients (28.1%) had von Willebrand disease, eleven patients (17.9%) had Hemophilia B and four patients had other factor deficiencies. Elevenpatients with Hemophilia A and one patient with Hemophilia B had severe disease. The prevalence of hepatitis C virus infection was 5%. All patients were negative for hepatitis B ...
متن کاملItalian Registry of Congenital Bleeding Disorders
In Italy, the surveillance of people with bleeding disorders is based on the National Registry of Congenital Coagulopathies (NRCC) managed by the Italian National Institute of Health (Istituto Superiore di Sanità). The NRCC collects epidemiological and therapeutic data from the 54 Hemophilia Treatment Centers, members of the Italian Association of Hemophilia Centres (AICE). The number of people...
متن کاملRare 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...
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ژورنال
عنوان ژورنال: Annals of Translational Medicine
سال: 2018
ISSN: 2305-5839,2305-5847
DOI: 10.21037/atm.2018.08.34