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阵发性睡眠性血红蛋白尿症患者主动脉瓣狭窄行主动脉瓣旁路术。

Aortic valve bypass for aortic stenosis in a patient with paroxysmal nocturnal hemoglobinuria.

机构信息

Department of Cardiac Surgery, University of Maryland Medical Center, Baltimore, Maryland, USA.

出版信息

Ann Thorac Surg. 2010 Jul;90(1):279-81. doi: 10.1016/j.athoracsur.2009.11.047.

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired bone marrow disorder characterized by hemolytic anemia, neutropenia, thrombocytopenia, hemoglobinuria, and venous thrombosis. Cardiopulmonary bypass-induced complement activation may be associated with exacerbation of hemolysis in patients with PNH undergoing cardiac operations. We describe a 77-year-old man with PNH and critical symptomatic aortic stenosis who underwent successful aortic valve bypass operation without cardiopulmonary bypass as an alternative to conventional aortic valve replacement.

摘要

阵发性睡眠性血红蛋白尿症(PNH)是一种罕见的获得性骨髓疾病,其特征为溶血性贫血、中性粒细胞减少症、血小板减少症、血红蛋白尿和静脉血栓形成。体外循环引起的补体激活可能与 PNH 患者心脏手术后溶血加重有关。我们描述了一名 77 岁男性,患有 PNH 和严重症状性主动脉瓣狭窄,成功地进行了主动脉瓣旁路手术,而不是传统的主动脉瓣置换术。

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