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多囊性发育不良肾患儿的临床病程及转归

Clinical course and outcome for children with multicystic dysplastic kidneys.

作者信息

Feldenberg L R, Siegel N J

机构信息

Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut 06520-8064, USA.

出版信息

Pediatr Nephrol. 2000 Oct;14(12):1098-101. doi: 10.1007/s004670000391.

Abstract

The purpose of this study was to evaluate the clinical course and outcome for children with multicystic dysplastic kidney (MCDK) disease and to non-invasively predict which of these patients are at significant risk for developing urinary tract infection (UTI) and renal insufficiency. Patients were divided, on the basis of postnatal physical examination and renal ultrasonography, into simple or complex MCDK. Simple MCDK was defined as unilateral renal dysplasia without additional genitourinary (GU) abnormalities. Complex MCDK included patients with bilateral renal dysplasia or unilateral renal dysplasia with other GU abnormalities. The designation as simple or complex MCDK was independent of reflux, since routine voiding cystourethrography (VCUG) was not performed. The charts of all patients with the diagnosis of MCDK disease seen from August 1995 to March 1999 at Yale University School of Medicine were examined to determine: (1) if UTI had occurred and (2) the level of renal function at last follow-up. Thirty-five patients were evaluated: 28 (80%) patients had unilateral MCDK, 7 (20%) were bilateral, and 14 (40%) had associated GU anomalies. Overall, 21 patients had unilateral MCDK without GU abnormalities (simple MCDK), while 14 had complex MCDK. The final outcome for patients with simple MCDK was quite good, with normal renal function and compensatory hypertrophy of the contralateral kidney in all patients. Although the patients with simple MCDK did not have routine VCUG or prophylactic antibiotics, the development of UTI was infrequent, damage to the contralateral kidney did not occur, and renal function was well preserved. In contrast, patients with bilateral disease or associated GU anomalies had a higher incidence of UTI and progression to renal failure. Complex MCDK was associated with a worse outcome (50% chronic renal insufficiency or failure).

摘要

本研究的目的是评估多囊性发育不良肾(MCDK)疾病患儿的临床病程及预后,并以非侵入性方式预测哪些患儿有发生尿路感染(UTI)和肾功能不全的重大风险。根据出生后体格检查和肾脏超声检查结果,将患者分为单纯性或复杂性MCDK。单纯性MCDK定义为单侧肾发育不良且无其他泌尿生殖系统(GU)异常。复杂性MCDK包括双侧肾发育不良患者或伴有其他GU异常的单侧肾发育不良患者。由于未进行常规排尿性膀胱尿道造影(VCUG),单纯性或复杂性MCDK的分类与反流无关。对1995年8月至1999年3月在耶鲁大学医学院就诊的所有诊断为MCDK疾病的患者病历进行检查,以确定:(1)是否发生UTI以及(2)末次随访时的肾功能水平。共评估了35例患者:28例(80%)为单侧MCDK,7例(20%)为双侧,14例(40%)伴有GU异常。总体而言,21例患者为无GU异常的单侧MCDK(单纯性MCDK),14例为复杂性MCDK。单纯性MCDK患者的最终预后相当好,所有患者肾功能正常且对侧肾代偿性肥大。尽管单纯性MCDK患者未进行常规VCUG或预防性使用抗生素,但UTI的发生并不常见,对侧肾未出现损害,肾功能得到良好保留。相比之下,双侧疾病或伴有GU异常的患者UTI发生率较高且进展为肾衰竭。复杂性MCDK与较差的预后相关(50%慢性肾功能不全或肾衰竭)。

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