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血友病的进展:实验方面与治疗

Advances in hemophilia: experimental aspects and therapy.

作者信息

Rodriguez Nidra I, Hoots W Keith

机构信息

Division of Pediatrics, Hematology Section, The University of Texas Health Science Center, 6411 Fannin, Houston, TX 77030, USA.

出版信息

Pediatr Clin North Am. 2008 Apr;55(2):357-76, viii. doi: 10.1016/j.pcl.2008.01.010.

Abstract

This article describes recent clinical and research advances in hemophilia therapy. Different prophylactic regimens for the management of severe hemophilia are described along with the use of adjuvant treatment options to achieve hemostasis. The safety and efficacy of radionuclide synovectomy with phosphorus 32-sulfur colloid to treat existing joint arthropathy also are described. The development of inhibitors to factor VIII or IX remains a challenge for hemophilia care and recent approaches to achieve immune tolerance induction are discussed. Finally, recent advances in hemophilia are mentioned, including the role of iron, inflammation, and angiogenesis in the pathogenesis of hemophilic arthopathy.

摘要

本文介绍了血友病治疗领域近期的临床和研究进展。文中描述了用于重度血友病管理的不同预防方案,以及使用辅助治疗手段实现止血的情况。还介绍了用磷32 - 硫胶体进行放射性核素滑膜切除术治疗现有关节病的安全性和有效性。针对血友病治疗而言,凝血因子VIII或IX抑制物的产生仍然是一项挑战,文中讨论了近期实现免疫耐受诱导的方法。最后,提及了血友病领域的最新进展,包括铁、炎症和血管生成在血友病性关节病发病机制中的作用。

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