Sickle cell syndromes, including sickle cell disease (SCD) and sickle cell trait, are associated with multiple kidney abnormalities. Young patients with SCD have elevated effective renal plasma flow and glomerular filtration rates, which decrease to normal ranges in young adulthood and subnormal levels with advancing age. The pathophysiology of SCD-related nephropathy is multifactorial — oxidative stress, hyperfiltration and glomerular hypertension are all contributing factors. Albuminuria, which is an early clinical manifestation of glomerular damage, is common in individuals with SCD. Kidney function declines more rapidly in individuals with SCD than in those with sickle cell trait or in healthy individuals. Multiple genetic modifiers, including APOL1, HMOX1, HBA1 and HBA2 variants are also implicated in the development and progression of SCD-related nephropathy. Chronic kidney disease and rapid decline in estimated glomerular filtration rate are associated with increased mortality in adults with SCD. Renin–angiotensin–aldosterone system inhibitors are the standard of care treatment for albuminuria in SCD, despite a lack of controlled studies demonstrating their long-term efficacy. Multiple studies of novel therapeutic agents are ongoing, and patients with SCD and kidney failure should be evaluated for kidney transplantation. Given the high prevalence and severe consequences of kidney disease, additional studies are needed to elucidate the pathophysiology, natural history and treatment of SCD-related nephropathy.

镰状细胞综合征，包括镰状细胞病 (SCD) 和镰状细胞性状，与多种肾脏异常相关。患有 SCD 的年轻患者的有效肾血浆流量和肾小球滤过率升高，在青年时期降至正常范围，并随着年龄的增长而低于正常水平。 SCD 相关肾病的病理生理学是多因素的——氧化应激、高滤过和肾小球高血压都是促成因素。蛋白尿是肾小球损伤的早期临床表现，在 SCD 患者中很常见。与镰状细胞性状或健康个体相比，SCD 个体的肾功能下降得更快。多种基因修饰因子，包括APOL1 、 HMOX1 、 HBA1和HBA2变体，也与 SCD 相关肾病的发生和进展有关。慢性肾脏病和估计肾小球滤过率的快速下降与成人 SCD 死亡率增加相关。肾素-血管紧张素-醛固酮系统抑制剂是 SCD 蛋白尿的护理治疗标准，尽管缺乏对照研究证明其长期疗效。新型治疗药物的多项研究正在进行中，患有 SCD 和肾衰竭的患者应接受肾移植评估。鉴于肾脏疾病的高患病率和严重后果，需要进行更多研究来阐明 SCD 相关肾病的病理生理学、自然史和治疗。