Syndrome de Bernard-SoulierR. FuzierPrise en charge des maladies rares en anesthésie et analgésie obstétricales
⏩Bernard-Soulier syndrome (BSS) also known as Hemorrhagiparous thrombocytic dystrophy, is a hereditary bleeding disorder affecting the megakaryocyte/platelet lineage and characterized by bleeding tendency, giant blood platelets and low platelet counts.⠀...
The pathophysiology of Bernard-Soulier syndrome (BSS) and TTP both involve platelet GPIb. BSS is usually an inherited autosomal recessive (rarely autosomal dominant) condition characterized by prolonged bleeding time and macrothrombocytopenia.8鈥 10 BSS is often associated with epistaxis, gingival and ...
bernard-souliersyndrome网络巨大血小板综合征网络释义 1. 巨大血小板综合征 1、巨大血小板综合征(Bernard-Souliersyndrome)为常染色体隐性遗传,生后数日即可发病,临床多见中度出血征。其基本 …www.baike.com|基于5个网页© 2025 Microsoft 隐私声明和 Cookie 法律声明 广告 帮助 反馈...
In nearly all cases of Bernard-Soulier syndrome, it is inherited from both the mother and the father. There are very rare situations where the same blood disorder may appear as part of another illness and only a few situations where only one parent can pass the disease along. The gene that...
Bernard-Soulier Syndrome is an autosomal recessively transmitted disorder characterized by the absence of glycoprotein Ib/IX (GPIb/IX), which binds von Willebrand Factor (vWF), resulting in defective...Lambert M. SurhoneMariam T. TennoeSusan F. Henssonow...
Bernard-Soulier syndrome (BSS), also known as Hemorrhagiparous thrombocytic dystrophy, is a hereditary bleeding disorder affecting the megakaryocyte/platelet lineage and characterized by bleeding tendency, giant blood platelets and low platelet counts. This syndrome is extremely rare as only ~100 cases...
Bernard-Soulier syndrome (BSS), also known as Hemorrhagiparous Thrombolytic Dystrophy, is an extremely rare inherited blood clotting disorder characterized by giant platelet cells, thrombocytopenia, and prolonged bleeding time. In 1948, Jean-Bernard and Jean-Pierre Soulier described the first male patien...
(bĕr-năr′sool-yā′) [Jean A. Bernard, Fr. hematologist, 1907-2006; Jean-Pierre Soulier, Fr. hematologist, 1915-2003] An autosomal recessive bleeding disorder marked by an inherited deficiency of a platelet glycoprotein. The platelets are large. Bleeding results from defective adhesion of...
Bernard-Soulier syndrome (BSS) is a rare autosomal bleeding disorder characterized clinically by prolonged skin bleeding time, normal clot retraction and thrombocytopenia with large and morphologically abnormal platelets, and biochemically by the absence of platelet membrane glycoproteins (GP)Ib, V and IX...