Denys-Drash综合征(DDS)是一种较为罕见的先天性疾病,1967年和1970年分别由Denys和Drash等首先报道。该疾病主要以肾病综合征为主要表现,同时伴有男性假两性畸形、肾母细胞瘤或两者之一。肾脏病理主要表现为弥漫性系膜硬化,患者早期发病,多在2岁以内,并很快进展至终末期肾病(ESRD)。分子遗传学研究结果证实,主要由Wilms瘤抑制基因(WT1)突变所致。现将我院收治的1例患者报道如下。1病历资料患儿男性,2岁时因左肾母细胞瘤行左肾切除。9岁时 Denys-Drash syndrome (DDS) is a relatively rare congenital disease, first reported by Denys and Drash in 1967 and 1970 respectively. The disease is mainly nephrotic syndrome as the main manifestation, accompanied by male pseudohermaphroditism, Wilms tumor or both. Renal pathology is mainly manifested as diffuse mesangial sclerosis, early onset of patients, mostly within 2 years of age, and rapid progression to end-stage renal disease (ESRD). Results of molecular genetics studies confirm that the mutation is mainly caused by the Wilms tumor suppressor gene (WT1). Now in our hospital admitted to a patient reported as follows. 1 medical records of children with male, 2 years old due to left nephroblastoma left nephrectomy. 9 years old