Cascio S, Paran S, Puri P
Our Lady's Hospital for Sick Children, Crumlin and National Children's Hospital, Dublin, Ireland.
J Urol. 1999 Sep;162(3 Pt 2):1081-3. doi: 10.1016/S0022-5347(01)68074-1.
Unilateral renal agenesis has been noted in 1:1,000 autopsies. Recently an increased incidence of vesicoureteral reflux was reported in patients with a solitary kidney. We determined the incidence of associated renal abnormalities in children with unilateral renal agenesis.
We retrospectively reviewed 46 consecutive cases of unilateral renal agenesis diagnosed at our hospital between January 1985 and February 1998. Patient age at diagnosis ranged from newborn to 12.5 years (mean 2.8 years). There were 24 boys and 22 girls. The left kidney was absent in 27 patients and the right kidney was absent in the remaining 19. A total of 24 patients were evaluated for urinary tract infection and in the other 22 a solitary kidney was found during examination for congenital malformations, enuresis or abdominal pain. The diagnosis was made in all patients by abdominal ultrasound and confirmed by excretory urography, or diethylenetriaminepentaacetic acid or dimercapto-succinic acid scan. A voiding cystourethrogram was performed in 40 patients (87%).
Associated urological anomalies were present in 22 of the 46 patients (48%) with unilateral renal agenesis, including primary vesicoureteral reflux in 13 (28%), ureterovesical junction obstruction in 5 (11%), ureteropelvic junction obstruction in 3 (7%), and ureterovesical and ureteropelvic junction obstruction in 1 (2%). Of the 22 patients 14 (64%) underwent surgical intervention.
Nearly half of the patients with unilateral renal agenesis had associated urological anomalies. Vesicoureteral reflux was the most common associated anomaly and it was usually of high grade. Early recognition and treatment of urological anomalies in a patient with a solitary kidney are imperative to decrease the long-term risk of renal damage.
单侧肾缺如在1000例尸检中约占1例。最近有报道称，孤立肾患者的膀胱输尿管反流发生率有所增加。我们确定了单侧肾缺如儿童相关肾脏异常的发生率。
我们回顾性分析了1985年1月至1998年2月间在我院确诊的46例连续单侧肾缺如病例。诊断时患者年龄从新生儿到12.5岁（平均2.8岁）。其中男孩24例，女孩22例。27例患者左肾缺如，其余19例右肾缺如。共有24例患者因尿路感染接受评估，另外22例在先天性畸形、遗尿或腹痛检查中发现孤立肾。所有患者均通过腹部超声诊断，并经排泄性尿路造影、二乙三胺五乙酸或二巯基琥珀酸扫描证实。40例患者（87%）进行了排尿性膀胱尿道造影。
46例单侧肾缺如患者中有22例（48%）存在相关泌尿系统异常，其中原发性膀胱输尿管反流13例（28%），输尿管膀胱连接部梗阻5例（11%），肾盂输尿管连接部梗阻3例（7%），输尿管膀胱和肾盂输尿管连接部梗阻1例（2%）。22例患者中有14例（64%）接受了手术干预。
近一半的单侧肾缺如患者存在相关泌尿系统异常。膀胱输尿管反流是最常见的相关异常，且通常为重度。对于孤立肾患者，早期识别和治疗泌尿系统异常对于降低肾脏损害的长期风险至关重要。
