亲属肾移植治疗Denys-Drash综合征一例

Male child, born on October 15, 2004 in a local hospital in Jiading, Shanghai by cesarean section. After birth, hypospadias and external genitalia malformation were found and transferred to the Children's Hospital affiliated to Fudan University for treatment. In October 2005, a chromosome examination showed 46, XY, and a laparoscopic left testicular pull-down and fixation operation was performed. In April 2006, the child had eyelid edema and a lot of urine foam. The urine routine showed positive protein (+++). A bilateral kidney biopsy showed focal global and segmental sclerosing glomerulopathy. Hormone pulse therapy was given with poor effect and was considered hormone-resistant. In March 2009, the child's urine volume gradually decreased and urine foam still existed. The creatinine was 500 μmol/L. In June, a peritoneal dialysis tube was placed. After one peritoneal dialysis, the lumen was blocked with wound exudate and the peritoneal dialysis tube was removed. 200...

患儿男,2004年10月15日出生于上海市嘉定当地医院,剖腹产,出生后发现尿道下裂外生殖器畸形,转至复旦大学附属儿科医院就诊.2005年10月行染色体检查为46,XY,行腹腔镜左侧睾丸下拉固定术.2006年4月患儿出现眼睑浮肿,尿泡沫多,查尿常规蛋白阳性(+++).双侧肾脏穿刺活检为局灶性球性及节段硬化性肾小球病.给予激素冲击治疗,效果不佳,考虑为激素耐药型.2009年3月开始患儿尿量逐渐减少,尿泡沫仍存在,肌酐500 μmol/L,6月给予置腹透管,腹膜透析1次后管腔堵塞伴伤口渗液,予拔出腹透管.200