Kousidis Grigorios, Thomas David F M, Morgan Henry, Haider Nadeem, Subramaniam Ramnath, Feather Sally
Departments of Paediatric Urology and Paediatric Nephrology, St James's University Hospital, Leeds, UK.
BJU Int. 2008 Sep;102(8):1020-4. doi: 10.1111/j.1464-410X.2008.07745.x. Epub 2008 May 15.
To document the functional outcome of patients with prenatally detected posterior urethral valves (PUV) in the second decade of life, and to evaluate the possible impact of prenatal diagnosis on the long-term outcome of this condition.
We analysed the functional outcome of 25 patients with prenatally detected PUV born between 1984 and 1996, whose mean (range) age at follow-up was 17.7 (10-23) years. The findings were compared with those in 17 patients (mean age 16.1 years) who had presented clinically to our unit during the same period. The duration of follow-up in both groups was >or=10 years. Late outcomes were also compared with published data for PUV. Outcome measures included; death, incidence of end-stage renal failure (ESRF), age at transplantation and the most the recently available plasma creatinine level in untransplanted patients. We also examined any possible association between functional outcome and early predictors, including nadir plasma creatinine level at <1 year and vesico-ureteric reflux (VUR).
Three patients died (12%), two as neonates and one aged 3 years. Of five patients who had been shunted in utero, four died or developed early-onset renal failure. In the 23 prenatally detected patients who survived the neonatal period, four (17%) had a renal transplant at a mean (range) age of 6.5 (3.0-12.0) years. Of 19 patients with prenatally detected PUV who had not been transplanted in the first 12 years of life, only one (5%) developed new-onset ESRF at 10.0-23.4 years whilst 11 (58%) of these patients had normal creatinine values. In the untransplanted patients there was a statistically significant correlation between age and plasma creatinine level, but no correlation between late functional outcome and nadir creatinine in the first year of life, or bilateral VUR.
Prenatal diagnosis had little impact on mortality or ESRF in the first decade of life. This appears to be largely predetermined by renal dysplasia and the severity of intrauterine obstruction. However, the functional outcome of patients with prenatally detected PUV aged 10-23 years was considerably better than published long-term data and the outcome of clinically presenting patients in our study. These findings suggest that the long-term prognosis of PUV of intermediate severity might be improved by prenatal diagnosis.
记录产前检测出后尿道瓣膜（PUV）的患者在第二个十年的功能转归，并评估产前诊断对该疾病长期转归的可能影响。
我们分析了1984年至1996年间出生的25例产前检测出PUV的患者的功能转归，其随访时的平均（范围）年龄为17.7（10 - 23）岁。将这些结果与同期临床就诊于我们科室的17例患者（平均年龄16.1岁）的结果进行比较。两组的随访时间均≥10年。晚期转归也与已发表的PUV数据进行比较。转归指标包括：死亡、终末期肾衰竭（ESRF）的发生率、移植时的年龄以及未移植患者最近可获得的血浆肌酐水平。我们还研究了功能转归与早期预测因素之间的任何可能关联，包括1岁以内的最低血浆肌酐水平和膀胱输尿管反流（VUR）。
3例患者死亡（12%），2例为新生儿，1例3岁。在5例宫内接受分流术的患者中，4例死亡或发生早发性肾衰竭。在23例度过新生儿期存活的产前检测出的患者中，4例（17%）在平均（范围）年龄6.5（3.0 - 12.0）岁时接受了肾移植。在19例产前检测出PUV且在生命的前12年未接受移植的患者中，只有1例（5%）在10.0 - 23.4岁时出现新发ESRF，而这些患者中有11例（58%）肌酐值正常。在未移植患者中，年龄与血浆肌酐水平之间存在统计学显著相关性，但生命第一年的最低肌酐水平或双侧VUR与晚期功能转归之间无相关性。
产前诊断对生命第一个十年的死亡率或ESRF影响不大。这似乎很大程度上由肾发育不良和宫内梗阻的严重程度预先决定。然而，产前检测出PUV且年龄在10 - 23岁的患者的功能转归明显优于已发表的长期数据以及我们研究中临床就诊患者的转归。这些发现表明，产前诊断可能改善中度严重程度PUV的长期预后。
