Department of Renal Medicine, King's College London, UK.
Br J Haematol. 2011 Nov;155(3):287-97. doi: 10.1111/j.1365-2141.2011.08853.x. Epub 2011 Sep 9.
Despite its apparently simple molecular aetiology, sickle cell disease (SCD) has long been known to have a remarkably variable clinical course, with complications involving many organs including the kidneys. Whilst many affected individuals show no evidence of renal involvement into late adulthood, others develop renal dysfunction in childhood or early adult life with a significant proportion eventually requiring renal replacement therapy. This review explores the pathophysiology and clinical manifestations of sickle cell nephropathy (SCN) and discusses how each complication can be investigated, monitored and managed in the outpatient setting. We summarize current knowledge of genetic modulation of sickle-related renal dysfunction. We outline the evidence for various treatment options and discuss others for which little evidence currently exists.
尽管镰状细胞病 (SCD) 的分子病因学看似简单，但长期以来，人们已经知道其临床表现变化极大，涉及包括肾脏在内的许多器官的并发症。虽然许多受影响的个体在成年后期没有肾脏受累的证据，但其他人在儿童或成年早期会出现肾功能障碍，其中很大一部分最终需要肾脏替代治疗。这篇综述探讨了镰状细胞肾病 (SCN) 的病理生理学和临床表现，并讨论了如何在门诊环境中对每个并发症进行检查、监测和管理。我们总结了目前对与镰状细胞相关的肾功能障碍的遗传调节的认识。我们概述了各种治疗选择的证据，并讨论了其他目前证据很少的治疗选择。
