Kara Aslihan, Gurgoze Metin Kaya, Aydin Mustafa, Koc Zehra Pinar
Department of Pediatric Nephrology, Firat University School of Medicine, Elazig, Turkey.
Department of Neonatology, Firat University School of Medicine, Elazig, Turkey.
Pediatr Int. 2018 Aug;60(8):750-754. doi: 10.1111/ped.13612. Epub 2018 Jul 31.
To evaluate the clinical features of patients with multicystic dysplastic kidney (MCDK).
The medical files of children diagnosed with MCDK between January 2008 and November 2015 were retrospectively reviewed. The demographic, clinical, laboratory and radiological data were evaluated.
Of 128 children with MCDK enrolled in the study, 82 (64.1%) were male, and 46 (35.9%) were female (P < 0.05). MCDK were located on left and right sides in 66 (51.6%) and 62 children (48.4%), respectively (P > 0.05). Antenatal diagnosis was present in 64 patients (50%). The mean age at diagnosis was 2.8 ± 2.7 years (range, 0-8 years), and follow-up duration was 4.5 years. Fifteen patients (20.8%) had vesicoureteral reflux. Of these, four underwent endoscopic surgical correction. Other associated urological anomalies were ureteropelvic junction obstruction (n = 6), hypospadias (n = 1), and kidney stones (n = 1). On technetium-99 m dimercaptosuccinic acid scintigraphy, which was performed in all patients, no significant association between grade of reflux and presence of scarring was seen. Hypertension was diagnosed only in one child (0.8%) who required antihypertensive treatment. The prevalence of unilateral undescended testicle in children aged <1 year in the 82 male patients was 4.9%. Seventy-six patients (59.4%) developed compensatory hypertrophy in the contralateral kidney during a 1 year follow-up period. Of the total, only seven children (5.5%) had undergone nephrectomy.
MCDK follows a benign course with relatively few sequelae, and therefore these patients should be closely followed up and conservatively managed.
评估多囊性发育不良肾（MCDK）患者的临床特征。
回顾性分析2008年1月至2015年11月间诊断为MCDK的儿童的病历。对人口统计学、临床、实验室和影像学数据进行评估。
纳入研究的128例MCDK患儿中，82例（64.1%）为男性，46例（35.9%）为女性（P<0.05）。MCDK位于左侧和右侧的分别有66例（51.6%）和62例（48.4%）（P>0.05）。64例患者（50%）为产前诊断。诊断时的平均年龄为2.8±2.7岁（范围0 - 8岁），随访时间为4.5年。15例患者（20.8%）有膀胱输尿管反流。其中4例接受了内镜手术矫正。其他相关泌尿系统异常包括肾盂输尿管连接处梗阻（n = 6）、尿道下裂（n = 1）和肾结石（n = 1）。所有患者均进行了锝-99m二巯基丁二酸闪烁扫描术，未发现反流程度与瘢痕形成之间存在显著关联。仅1例儿童（0.8%）被诊断为高血压，需要进行降压治疗。82例男性患者中，年龄<1岁的儿童单侧隐睾患病率为4.9%。在1年的随访期内，76例患者（59.4%）对侧肾出现代偿性肥大。总共只有7例儿童（5.5%）接受了肾切除术。
MCDK病程呈良性，后遗症相对较少，因此应对这些患者密切随访并采取保守治疗。
