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单侧肾缺如

Unilateral renal agenesis.

作者信息

Robson W L, Leung A K, Rogers R C

机构信息

Department of Pediatrics, Faculty of Medicine, University of South Carolina School of Medicine, Columbia, USA.

出版信息

Adv Pediatr. 1995;42:575-92.

PMID:8540439
Abstract

Unilateral renal agenesis is a relatively common congenital urinary malformation that is usually diagnosed during fetal ultrasonography. Some cases of URA may represent involution of a previous MCDK. Unilateral renal agenesis may be an isolated congenital malformation or may be associated with chromosomal abnormalities or a variety of nonchromosomal syndromes including the VACTERL and MURCS associations. Congenital cardiac malformations are the most common malformations associated with URA. Girls with URA should have a pelvic ultrasound to look for abnormalities in the müllerian structures. Vesicoureteral reflux is the most common abnormality noted in the contralateral kidney. The prognosis of individuals with isolated URA is good. There are reports of hypertension and renal insufficiency in long-term studies of patients with a single kidney, and lifetime follow-up is recommended.

摘要

单侧肾缺如是一种相对常见的先天性泌尿系统畸形,通常在胎儿超声检查时被诊断出来。一些单侧肾缺如的病例可能代表之前的多囊性发育不良肾的退化。单侧肾缺如可能是一种孤立的先天性畸形,也可能与染色体异常或多种非染色体综合征有关,包括VACTERL和MURCS联合征。先天性心脏畸形是与单侧肾缺如相关的最常见畸形。患有单侧肾缺如的女孩应进行盆腔超声检查,以寻找苗勒氏结构的异常。膀胱输尿管反流是对侧肾脏最常见的异常情况。孤立性单侧肾缺如患者的预后良好。在对单肾患者的长期研究中有高血压和肾功能不全的报告,因此建议进行终身随访。

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