Pediatric Nephrology Unit, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, 14 Weizman St, 64239, Tel Aviv, Israel.
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Pediatr Nephrol. 2023 Aug;38(8):2711-2717. doi: 10.1007/s00467-023-05889-y. Epub 2023 Feb 6.
Exstrophy-epispadias complex (EEC) is a complex malformation of the lower abdominal wall, bladder, and pelvic floor, which necessitates multiple successive reconstruction procedures. Surgical and infectious complications are frequent. Our aim was to evaluate kidney function in these patients.
This cross-sectional study included patients with EEC, followed since birth in a pediatric urology clinic, who underwent nephrological evaluation (blood pressure (BP) measurement and blood and urine chemistries) and imaging studies (urinary tract ultrasound and DMSA kidney scan) during 2017-2020.
Forty-three patients (29 males), median age 9 years (interquartile range 6-19), were included. Eleven (26%) used clean intermittent catheterization (CIC) for bladder drainage. At least one sign of kidney injury was identified in 32 (74%) patients; elevated BP, decreased kidney function (estimated glomerular filtration rate (eGFR) < 90 ml/min/1.73 m), and proteinuria/albuminuria were detected in 29%, 12%, and 36% of patients, respectively. Urinary tract dilatation (UTD) was found in 13 (37%) ultrasound examinations. Parenchymal kidney defects were suspected in 46% and 61% of ultrasound and DMSA scintigraphy, respectively. UTD was significantly associated with DMSA-proven kidney defects (p = 0.043) and with elevated BP, 39% vs. 20% in those without UTD. Decreased eGFR and elevated BP were less frequent among patients on CIC than among patients who voided spontaneously: 10% vs. 14% and 18% vs. 36%, respectively. Recurrent UTIs/bacteriuria and nephro/cystolithiasis were reported by 44% and 29% patients, respectively.
The high rate of signs of kidney injury in pediatric patients with EEC dictates early-onset long-term kidney function monitoring by joint pediatric urological and nephrological teams. A higher resolution version of the Graphical abstract is available as Supplementary information.
脐膨出-尿道上裂复合畸形（EEC）是一种腹壁、膀胱和盆底的复杂畸形，需要多次连续的重建手术。手术和感染并发症较为常见。我们的目的是评估这些患者的肾功能。
这是一项横断面研究，纳入了在小儿泌尿科诊所自出生起即接受随访的 EEC 患者，这些患者在 2017 年至 2020 年期间接受了肾脏评估（血压（BP）测量、血液和尿液化学分析）和影像学研究（尿路超声和 DMSA 肾脏扫描）。
共纳入 43 例患者（29 名男性），中位年龄 9 岁（四分位距 6-19 岁）。11 例（26%）患者采用间歇性清洁导尿（CIC）进行膀胱引流。32 例（74%）患者至少存在一项肾脏损伤表现；29%、12%和 36%的患者分别存在血压升高、肾功能下降（估算肾小球滤过率（eGFR）＜90ml/min/1.73m）和蛋白尿/白蛋白尿。13 例（37%）超声检查发现尿路扩张（UTD）。超声和 DMSA 闪烁显像分别提示 46%和 61%的患者存在实质肾脏缺陷。UTD 与 DMSA 证实的肾脏缺陷显著相关（p=0.043），UTD 患者的血压升高发生率（39%）高于无 UTD 患者（20%）。接受 CIC 的患者中，eGFR 下降和血压升高的发生率低于自发排尿者：分别为 10%和 18%，20%和 36%。分别有 44%和 29%的患者报告有反复尿路感染/菌尿和肾/胱结石。
EEC 患儿中肾脏损伤的发生率较高，需要小儿泌尿科和肾病科联合团队进行早期、长期的肾功能监测。本文提供了一个高分辨率的图表摘要。
