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阵发性夜间血红蛋白尿症的肾脏受累谱:三例报告及文献简要综述

Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature.

作者信息

Nair R K, Khaira Ambar, Sharma Alok, Mahajan Sandeep, Dinda Amit K

机构信息

Department of Nephrology, All India Institute of Medical Sciences, Ansari Nagar 110029, New Delhi, India.

出版信息

Int Urol Nephrol. 2008;40(2):471-5. doi: 10.1007/s11255-008-9356-5.

Abstract

Renal involvement in paroxysmal nocturnal hemoglobinuria (PNH) is usually clinically not apparent but, in cases with clinical involvement, varies from reversible acute dysfunction to chronic irreversible damage. We report two cases of acute kidney injury and one case of chronic kidney disease due to PNH. In all three cases a diagnosis of PNH was made after detection of renal dysfunction. The renal involvement was documented on both histology and on imaging studies. Finally, we briefly review the main literature data on renal involvement in PNH.

摘要

阵发性睡眠性血红蛋白尿(PNH)患者的肾脏受累在临床上通常不明显,但在有临床受累的病例中,表现各异,从可逆的急性功能障碍到慢性不可逆损伤。我们报告了两例因PNH导致的急性肾损伤和一例慢性肾病病例。在所有这三例病例中,均在检测到肾功能障碍后确诊为PNH。组织学和影像学研究均证实了肾脏受累情况。最后,我们简要回顾了关于PNH患者肾脏受累的主要文献资料。

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