Department of Development and Regeneration, Katholieke Universiteit Leuven, Leuven, Belgium.
Center for Molecular and Vascular Biology, Department of Cardiovascular Sciences, Katholieke Universiteit Leuven, Leuven, Belgium.
Pediatr Nephrol. 2022 Jun;37(6):1231-1243. doi: 10.1007/s00467-021-05126-4. Epub 2021 May 29.
The life expectancy of individuals with sickle cell disease has increased over the years, majorly due to an overall improvement in diagnosis and medical care. Nevertheless, this improved longevity has resulted in an increased prevalence of chronic complications such as sickle cell nephropathy (SCN), which poses a challenge to the medical care of the patient, shortening the lifespan of patients by 20-30 years. Clinical presentation of SCN is age-dependent, with kidney dysfunction slowly beginning to develop from childhood, progressing to chronic kidney disease and kidney failure during the third and fourth decades of life. This review explores the epidemiology, pathology, pathophysiology, clinical presentation, and management of SCN by focusing on the pediatric population. It also discusses the factors that can modify SCN susceptibility.
近年来,镰状细胞病患者的预期寿命有所增加,主要是由于诊断和医疗护理的整体改善。然而,这种寿命的延长导致了慢性并发症(如镰状细胞肾病[SCN])的患病率增加,这给患者的医疗带来了挑战,使患者的寿命缩短了 20-30 年。SCN 的临床表现与年龄有关,肾功能障碍从儿童期开始缓慢发展,在第三和第四个十年发展为慢性肾脏病和肾衰竭。本综述通过关注儿科人群,探讨了 SCN 的流行病学、病理学、病理生理学、临床表现和治疗方法。它还讨论了可以改变 SCN 易感性的因素。