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女性梅干腹综合征:一种由腹部肌肉组织缺陷以及泌尿系统和生殖系统异常组成的三联征。

Prune belly syndrome in females: a triad of abdominal musculature deficiency and anomalies of the urinary and genital systems.

作者信息

Reinberg Y, Shapiro E, Manivel J C, Manley C B, Pettinato G, Gonzalez R

机构信息

Department of Urologic Surgery (Section of Pediatric Urology, University of Minnesota Hospital and Clinics, Minneapolis 55455.

出版信息

J Pediatr. 1991 Mar;118(3):395-8. doi: 10.1016/s0022-3476(05)82153-5.

Abstract

We describe seven female patients with deficient abdominal wall musculature and urinary tract and genital anomalies that represent the female equivalent of the prune belly syndrome. Urethral atresia, uterine duplication, and anorectal anomalies occurred frequently. The perinatal mortality rate was high; of the four surviving patients, renal failure developed in two and renal transplantation was required. The analysis of these cases suggests that urethral obstruction is an important factor contributing to the development of the prune belly syndrome in females.

摘要

我们描述了7例腹壁肌肉组织、泌尿道和生殖器官存在缺陷的女性患者,这些表现相当于女性的梅干腹综合征。尿道闭锁、子宫重复畸形和肛门直肠畸形很常见。围产期死亡率很高;4例存活患者中,2例出现肾衰竭,需要进行肾移植。对这些病例的分析表明,尿道梗阻是导致女性梅干腹综合征发生的一个重要因素。

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