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梅干腹综合征

Prune Belly Syndrome

作者信息

Pomajzl AJ, Sankararaman Senthilkumar

机构信息

Creighton University School of Medicine

University Hospitals Rainbow Babies & Children’s Hospital, Cleveland

Abstract

Prune belly syndrome, also referred to as Eagle-Barrett syndrome or the triad syndrome, is a rare congenital disorder characterized by the triad of deficient abdominal musculature, cryptorchidism, and urinary tract abnormalities. Children born with this condition present on a broad spectrum ranging from incompatibility with life to aging normally and having children of their own. The severity of renal dysplasia mostly determines the survival and prognosis among the survivors. Perinatal mortality ranges between 10 to 25% in contemporary studies and directly correlates to the severity of pulmonary hypoplasia as a result of oligohydramnios from reduced fetal urine production from renal dysplasia and urinary tract abnormalities leading to Potter sequence.

摘要

梅干腹综合征,也称为伊格尔 - 巴雷特综合征或三联征综合征,是一种罕见的先天性疾病,其特征为腹部肌肉组织发育不全、隐睾症和泌尿系统异常三联征。患有这种疾病的儿童表现范围广泛,从无法存活到正常衰老并育有自己的子女。肾发育不良的严重程度在很大程度上决定了幸存者的生存和预后。当代研究中围产期死亡率在10%至25%之间,并且与肺发育不全的严重程度直接相关,肺发育不全是由于肾发育不良和泌尿系统异常导致胎儿尿液生成减少,进而羊水过少,最终导致波特序列征。

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