Ezel Çelakil Mehtap, Ekinci Zelal, Bozkaya Yücel Burcu, Mutlu Nazım, Günlemez Ayla, Bek Kenan
Department of Pediatric Nephrology, Faculty of Medicine, Kocaeli University, İzmit, Turkey -
Department of Pediatric Nephrology, Faculty of Medicine, Kocaeli University, İzmit, Turkey.
Minerva Urol Nefrol. 2019 Dec;71(6):651-656. doi: 10.23736/S0393-2249.19.03272-7. Epub 2019 Feb 14.
Posterior urethral valve (PUV) is the most serious form of congenital anomalies of kidney and urinary tract (CAKUT) in boys with significant risk of progression to chronic kidney disease (CKD). We present our long-term results in children with PUV.
Retrospective chart review of 113 children with PUV followed within the years of 1996-2018 was performed. Clinical, laboratory and epidemiologic parameters were analyzed for their impact on renal outcome.
The median age of diagnosis was 1.00 month (1.00-132.00) and the median follow-up period was 70 months (60.00-216.00). Antenatal diagnosis was present in 33 patients (51.5%) mainly with bilateral hydronephrosis and oligohydramnios. The most common postnatal presentation was recurrent urinary tract infection (UTI) in 14 cases (21.9%) and incontinence in three cases (4.7%). Vesicoureteral-reflux (VUR) was present in 31 cases (48.4%). All patients had surgery and urinary diversion was needed in 18 (28.2%). Varying stages of chronic kidney disease (CKD) developed in 23 cases (35.9%) and rise in serum creatinine was especially prominent after the 4th year of follow-up. Of 23 CKD patients, seven (10.9%) were in ESRD and on dialysis. Mortality occurred in one (1.5%) patient. Hypertension, proteinuria and high initial serum creatinine (>1.28 mg/dL) were statistically significant risk factors for CKD, as expected. Surprisingly VUR and UTI did not show such a significant impact on CKD development. Antenatal detection was with significantly less risk for CKD.
Our results confirm that PUV has a considerable risk for CKD development. Antenatal diagnosis, management of proteinuria and hypertension may modify this progression. But already injured kidneys still have a potential risk. The need for further research to evaluate the impact of any intervention on long term renal outcome is obvious.
后尿道瓣膜（PUV）是男性先天性肾脏和尿路畸形（CAKUT）最严重的形式，进展为慢性肾脏病（CKD）的风险很大。我们展示了我们对患有PUV儿童的长期研究结果。
对1996年至2018年期间随访的113例患有PUV的儿童进行回顾性病历审查。分析临床、实验室和流行病学参数对肾脏结局的影响。
诊断的中位年龄为1.00个月（1.00 - 132.00），中位随访期为70个月（60.00 - 216.00）。33例（51.5%）患者为产前诊断，主要表现为双侧肾积水和羊水过少。最常见的产后表现是14例（21.9%）反复尿路感染（UTI）和3例（4.7%）尿失禁。31例（48.4%）存在膀胱输尿管反流（VUR）。所有患者均接受了手术，18例（28.2%）需要尿流改道。23例（35.9%）出现了不同阶段的慢性肾脏病（CKD），血清肌酐升高在随访第4年后尤为明显。在23例CKD患者中，7例（10.9%）进入终末期肾病（ESRD）并接受透析。1例（1.5%）患者死亡。正如预期的那样，高血压、蛋白尿和初始血清肌酐高（>1.28 mg/dL）是CKD的统计学显著危险因素。令人惊讶的是，VUR和UTI对CKD发展没有如此显著的影响。产前检测CKD的风险显著较低。
我们的结果证实PUV有发展为CKD的相当大风险。产前诊断、蛋白尿和高血压的管理可能会改变这种进展。但已经受损的肾脏仍有潜在风险。显然需要进一步研究以评估任何干预措施对长期肾脏结局的影响。
