Aslam M, Watson A R
Children & Young People's Kidney Unit, Nottingham University Hospitals, Nottingham, UK.
Arch Dis Child. 2006 Oct;91(10):820-3. doi: 10.1136/adc.2006.095786. Epub 2006 Jun 5.
To report the long term follow up of children with antenatally detected unilateral multicystic dysplastic kidney (MCDK) with documentation of complications, involution rate with time, and renal function at 10 years.
Data were retrieved from a prospective regional registry of patients with MCDK between 1985 and 2004. Children were followed using a common protocol of investigation with follow up ultrasound scans (USS) at 2 (165 patients), 5 (117 patients), and 10 years (43 patients).
Serial USS showed that 33% of the MCDK kidneys had completely involuted at 2 years of age, 47% at 5 years, and 59% at 10 years. No patients developed hypertension, significant proteinuria, or malignancy, but two developed pelviureteric junction obstruction in the contralateral kidney. Twenty seven of 143 children (19%) had vesicoureteric reflux (VUR) (96% mild to moderate VUR) into the contralateral kidney with no difference in the incidence of urinary tract infections or renal scarring between those with or without VUR. The mean estimated glomerular filtration rate (GFR) was 86.4 ml/min/1.73 m2 (range 48-125) in 31 of 43 patients followed to 10 years.
Conservative management of unilateral MCDK is justified with clinical review and infrequent USS but longer term follow up continues in the 41% still with renal remnants at 10 years and those with impaired GFR. It is suggested that the initial micturating cystogram is deferred unless abnormal USS features are present in the contralateral kidney or ureter.
报告产前检测出的单侧多囊性发育不良肾（MCDK）患儿的长期随访情况，记录并发症、随时间的退化率以及10岁时的肾功能。
数据取自1985年至2004年间一个关于MCDK患者的前瞻性区域登记处。按照通用的调查方案对患儿进行随访，在2岁（165例患者）、5岁（117例患者）和10岁（43例患者）时进行超声随访扫描（USS）。
系列超声检查显示，33%的MCDK肾在2岁时完全退化，47%在5岁时完全退化，59%在十岁时完全退化。没有患者出现高血压、大量蛋白尿或恶性肿瘤，但有两名患者对侧肾出现肾盂输尿管连接处梗阻。143名儿童中有27名（19%）出现膀胱输尿管反流（VUR）（96%为轻度至中度VUR）进入对侧肾，有或无VUR的患者在尿路感染或肾瘢痕形成的发生率上没有差异。在随访至10年的43例患者中，31例的平均估计肾小球滤过率（GFR）为86.4 ml/min/1.73 m²（范围48 - 125）。
对于单侧MCDK，进行临床检查并偶尔进行超声检查的保守治疗是合理的，但对于10岁时仍有肾残余的41%患者以及GFR受损的患者，仍需进行长期随访。建议除非对侧肾或输尿管出现异常超声特征，否则推迟最初的排尿性膀胱尿道造影检查。
