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822 例产前诊断肾尿路畸形患儿的临床病程。

Clinical course of 822 children with prenatally detected nephrouropathies.

机构信息

Pediatric Nephrology Unit, Department of Pediatrics, National Institute of Science and Technology of Molecular Medicine, Faculty of Medicine, Federal University of Minas Gerais, Belo Horizonte, Brazil.

出版信息

Clin J Am Soc Nephrol. 2012 Mar;7(3):444-51. doi: 10.2215/CJN.03400411. Epub 2012 Jan 19.

Abstract

BACKGROUND AND OBJECTIVES

With the advent of fetal screening ultrasonography, the detection of congenital anomalies of the kidney and urinary tract (CAKUT) in utero has permitted early management of these conditions. This study aims to describe the clinical course of a large cohort of patients with prenatally detected nephrouropathies.

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In this retrospective cohort study, 822 patients were prenatally diagnosed with CAKUT and systematically followed up at a tertiary Renal Unit for a median time of 43 months. Variables included in the analysis were sex, laterality, fetal ultrasonography (isolated versus associated hydronephrosis), and presence/absence of nephrouropathies. The events of interest were urinary tract infection, surgical interventions, hypertension, CKD, and death. Survival analyses were performed to evaluate time until occurrence of the events of interest.

RESULTS

Urinary tract infection occurred in 245 (29.8%) children, with higher risk in females (hazard ratio=1.30, 95% confidence interval=1.02-1.70, P=0.05); 22 patients (2.7%) had hypertension, and 49 (6%) patients developed CKD. The risk of CKD was greater in patients with associated hydronephrosis (hazard ratio=5.20, 95% confidence interval=2.90-9.30, P<0.001). Twelve patients (1.5%) died during follow-up. Death was significantly associated with being born during the first period of the study (hazard ratio=6.00, 95% confidence interval=1.60-22.50, P<0.001), associated hydronephrosis (hazard ratio=9.30, 95% confidence interval=2.90-29.30, P<0.001), and CKD (hazard ratio=170.00, 95% confidence interval=41.00-228.00, P<0.001).

CONCLUSIONS

In our series, the clinical course of prenatally detected CAKUT was heterogeneous, and those infants with associated hydronephrosis at baseline were identified as a high-risk subgroup.

摘要

背景与目的

随着胎儿超声筛查的出现,对先天性肾和尿路异常(CAKUT)的宫内检测已能实现对这些疾病的早期管理。本研究旨在描述一大组经产前诊断患有肾输尿管病变患者的临床病程。

设计、地点、参与者和测量方法:在这项回顾性队列研究中,822 名患者在产前被诊断为 CAKUT,并在一家三级肾脏科进行系统随访,中位随访时间为 43 个月。分析中包括的变量为性别、单侧或双侧病变、胎儿超声(孤立性或伴发肾积水)以及肾输尿管病变的存在/缺失。关注的事件包括尿路感染、手术干预、高血压、CKD 和死亡。生存分析用于评估发生关注事件的时间。

结果

245 名(29.8%)患儿发生尿路感染,女性风险更高(风险比=1.30,95%置信区间=1.02-1.70,P=0.05);22 名(2.7%)患儿发生高血压,49 名(6%)患儿发生 CKD。伴发肾积水的患儿发生 CKD 的风险更高(风险比=5.20,95%置信区间=2.90-9.30,P<0.001)。12 名(1.5%)患儿在随访期间死亡。死亡与出生于研究早期(风险比=6.00,95%置信区间=1.60-22.50,P<0.001)、伴发肾积水(风险比=9.30,95%置信区间=2.90-29.30,P<0.001)和 CKD(风险比=170.00,95%置信区间=41.00-228.00,P<0.001)显著相关。

结论

在我们的系列研究中,经产前诊断的 CAKUT 的临床病程存在异质性,且那些在基线时伴发肾积水的婴儿被确定为高风险亚组。

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