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肾脏和泌尿系统先天性异常的肾脏结局:一项单中心回顾性研究。

Renal outcome of congenital anomalies of the kidney and urinary tract system: a single-center retrospective study.

作者信息

Renda Rahime

机构信息

Department of Pediatric Nephrology, Antalya Research and Education Hospital, Antalya, Turkey -

出版信息

Minerva Urol Nefrol. 2018 Apr;70(2):218-225. doi: 10.23736/S0393-2249.17.03034-X. Epub 2017 Nov 21.

Abstract

BACKGROUND

Congenital anomalies of the kidney and urinary tract (CAKUT) have been defined in 20% to 50% of all fetal anomalies. The aim of this study was to determine the demographic and clinical characteristics of such patients, as well as to describe the outcome and risk factors that affect the prognosis.

METHODS

The analysis investigated retrospective data from 303 patients diagnosed with CAKUT between January 2015 and April 2017. Demographic data, clinical history, diagnosis, investigations, disease outcomes, and treatment procedures were evaluated.

RESULTS

A total of 303 children were included (126 females, 177 males). The median age was 3.0 years (range 0.01-17), and the median follow-up time was 1.65 years (range 0.02-12). The most commonly diagnosed anomalies of CAKUT were hydronephrosis (N.=139; isolated HN, N.=58), followed by renal parenchymal malformations (N.=109), collecting system anomalies (N.=56), migration and fusion anomalies (N.=53), and abnormalities of the bladder and urethra (N.=27). In 56 cases with isolated hydronephrosis, ultrasound normalized at a median age of 0.9 years (range 0.02-6). Surgery was performed on 44 children, and anomalies persisted in 201 children without any need for intervention. Poor outcome was defined in patients with renal parenchymal malformations and patients with abnormalities of the bladder and urethra. For 28 children (9.3%) who developed chronic kidney disease, the most common diagnoses were vesicoureteral reflux and neurogenic bladder.

CONCLUSIONS

Children with isolated hydronephrosis had a good prognosis. The risk factors for poor outcome were postnatal bilateral anomalies, a need for surgery, and persisting anomalies with impaired renal function.

摘要

背景

先天性肾和尿路畸形(CAKUT)在所有胎儿畸形中占20%至50%。本研究的目的是确定此类患者的人口统计学和临床特征,并描述影响预后的结局和危险因素。

方法

该分析调查了2015年1月至2017年4月期间诊断为CAKUT的303例患者的回顾性数据。评估了人口统计学数据、临床病史、诊断、检查、疾病结局和治疗程序。

结果

共纳入303名儿童(126名女性,177名男性)。中位年龄为3.0岁(范围0.01 - 17岁),中位随访时间为1.65年(范围0.02 - 12年)。CAKUT最常诊断的畸形是肾积水(n = 139;孤立性肾积水,n = 58),其次是肾实质畸形(n = 109)、集合系统畸形(n = 56)、迁移和融合畸形(n = 53)以及膀胱和尿道异常(n = 27)。在56例孤立性肾积水病例中,超声在中位年龄0.9岁(范围0.02 - 6岁)时恢复正常。44名儿童接受了手术,201名儿童的畸形持续存在且无需任何干预。肾实质畸形患者和膀胱及尿道异常患者的结局较差。在28名(9.3%)患慢性肾脏病的儿童中,最常见的诊断是膀胱输尿管反流和神经源性膀胱。

结论

孤立性肾积水患儿预后良好。结局不良的危险因素是出生后双侧畸形、需要手术以及肾功能受损的持续性畸形。

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