Tuberous sclerosis complex(TSC)is a rare autosomal dominant genetic disorder caused by mutations in the TSC1 or TSC2 genes.The disease can cause multiple organ lesions.The most common renal le-sions are renal angiomyolipoma(RAML).In recent years,China has made progresss in the understanding of TSC-RAML,so that the standardization of the diagnosis and management of TSC-RAML have improved.Efforts have been taken in the studies of the pathogenesis and treatment of TSC-RAML.This article reviews the diagno-sis,therapy,and research development of TSC-RAML in China.
关键词
结节性硬化症/肾血管平滑肌脂肪瘤/诊疗进展
Key words
tuberous sclerosis complex/renal angiomyolipoma/progress of diagnosis and treatment
维普
万方数据