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镰状细胞肾病的病理生理学、临床表现、诊断和治疗的新见解。

New insights on pathophysiology, clinical manifestations, diagnosis, and treatment of sickle cell nephropathy.

机构信息

Post-Graduation Program in Medical Sciences, Department of Internal Medicine, Federal University of Ceará, Rua Vicente Linhares, 1198, CEP 60135-270, Fortaleza, Ceará, Brazil.

出版信息

Ann Hematol. 2011 Dec;90(12):1371-9. doi: 10.1007/s00277-011-1327-8. Epub 2011 Sep 8.

Abstract

Sickle cell nephropathy is one of the main chronic complications of sickle cell disease (SCD), the most common of the hematological hereditary disorders. Several studies have been performed since the first description of SCD 100 years ago to investigate the mechanisms of kidney involvement in this disease. It has been demonstrated that both glomerular and tubular compartments can be damaged as a direct consequence of SCD, including renal function loss, concentration and acidification deficits, and glomerulopathies. This article highlights the aspects of sickle cell nephropathy pathophysiology and clinical manifestations and describes the most recent advances in the diagnosis and treatment of this disorder.

摘要

镰状细胞肾病是镰状细胞病(SCD)的主要慢性并发症之一,SCD 是最常见的血液遗传性疾病。自 100 年前首次描述 SCD 以来,已经进行了多项研究以探讨该疾病中肾脏受累的机制。已经证明,肾小球和肾小管两者都可以作为 SCD 的直接后果而受损,包括肾功能丧失、浓缩和酸化缺陷以及肾小球病变。本文重点介绍镰状细胞肾病的病理生理学和临床表现,并描述了该疾病诊断和治疗的最新进展。

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