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一例梅干腹综合征病例。

A case of prune belly syndrome.

作者信息

Xu Wei, Wu Hui, Wang Dong-Xuan, Mu Zhi-Hong

机构信息

Department of Neonatology, The First Hospital of Jilin University, Changchun 130021, People's Republic of China.

Department of Neonatology, The First Hospital of Jilin University, Changchun 130021, People's Republic of China.

出版信息

Pediatr Neonatol. 2015 Jun;56(3):193-6. doi: 10.1016/j.pedneo.2013.03.014. Epub 2013 Apr 29.

Abstract

Prune belly syndrome (PBS) is a rare congenital disorder characterized by deficient abdominal wall muscles, urinary tract malformation, and, in males, cryptorchidism. We present a case of PBS in China. The patient was a newborn baby boy who had wrinkled, "prune-like" abdominal skin, bilateral cryptorchidism, and urinary system malformation, complicated with hypoplasia of the lung and branch of the coronary artery-right ventricular fistula. His kidney function was inadequate. The patient subsequently died at age 28 days due to septicemia from a severe urinary tract infection.

摘要

梅干腹综合征(PBS)是一种罕见的先天性疾病,其特征为腹壁肌肉缺损、泌尿系统畸形,男性患者还伴有隐睾症。我们在中国报告了一例梅干腹综合征病例。该患者为一名男婴,其腹部皮肤呈皱缩的“梅干样”,双侧隐睾,泌尿系统畸形,并伴有肺发育不全和冠状动脉-右心室瘘。他的肾功能不全。该患者随后在28日龄时因严重尿路感染引发的败血症死亡。

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