Department of Pediatrics, University of Tennessee Health Science Center and Le Bonheur Children's Hospital, Memphis, TN 38105; Children's Foundation Research Institute (CFRI), Le Bonheur Children's Hospital, Memphis, TN 38105; Pediatric Medicine Department, St. Jude Children's Research Hospital, Memphis, TN 38105.
Johns Hopkins All Children's Hospital, St. Petersburg, FL 33702.
Crit Rev Oncog. 2022;27(2):35-49. doi: 10.1615/CritRevOncog.2022042857.
Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that affects both fetal development and postnatal tissue growth, resulting in altered brain structures and a tumor predisposition syndrome. Although every organ system is affected by the disease, kidney involvement is a leading cause of death in adults with TSC. Over the past decade, significant progress has been made in understanding the renal disease. This review focuses on the cystic and solid renal lesions in TSC, including their pathobiology and treatment.
结节性硬化症（TSC）是一种常染色体显性遗传疾病，影响胎儿发育和出生后组织生长，导致脑结构改变和肿瘤易患综合征。尽管每个器官系统都受到该疾病的影响，但肾脏受累是 TSC 成人患者死亡的主要原因。在过去的十年中，人们对肾脏疾病的认识取得了重大进展。本综述重点介绍 TSC 的囊性和实性肾脏病变，包括其发病机制和治疗。
