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梅干腹综合征:当前观点

Prune belly syndrome: current perspectives.

作者信息

Arlen Angela M, Nawaf Cayce, Kirsch Andrew J

机构信息

Yale University School of Medicine, Department of Urology, New Haven, CT 06520, USA.

Emory University, Children's Healthcare of Atlanta, Atlanta, GA 30328, USA.

出版信息

Pediatric Health Med Ther. 2019 Aug 6;10:75-81. doi: 10.2147/PHMT.S188014. eCollection 2019.

Abstract

Prune belly syndrome (PBS) is a rare but morbid congenital disease, classically defined by a triad of cardinal features that includes cryptorchidism, urinary tract dilation and laxity of the abdominal wall musculature. Children often require numerous surgical interventions including bilateral orchidopexy as well as individually tailored urinary tract and abdominal wall reconstruction. Along with the classic features, patients with PBS often experience gastrointestinal, orthopedic, and cardiopulmonary comorbidities.

摘要

梅干腹综合征(PBS)是一种罕见但严重的先天性疾病,典型表现为三联征,包括隐睾、尿路扩张和腹壁肌肉松弛。患儿通常需要多次手术干预,包括双侧睾丸固定术以及根据个体情况定制的尿路和腹壁重建手术。除了典型特征外,PBS患者常伴有胃肠道、骨科和心肺合并症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/38b3/6689549/897f8124b88e/PHMT-10-75-g0001.jpg

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