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后尿道瓣膜症的预后:产前诊断能在多大程度上改善其预后?

Outcome of posterior urethral valves: to what extent is it improved by prenatal diagnosis?

作者信息

El-Ghoneimi A, Desgrippes A, Luton D, Macher M A, Guibourdenche J, Garel C, Muller F, Vuillard E, Lottmann H, Nessmann C, Oury J F, Aigrain Y

机构信息

Department of Pediatric Urology, Hôpital Robert Debré, Paris, France.

出版信息

J Urol. 1999 Sep;162(3 Pt 1):849-53. doi: 10.1097/00005392-199909010-00076.

DOI:10.1097/00005392-199909010-00076
PMID:10458394
Abstract

PURPOSE

To assess the impact of prenatal diagnosis and evaluation on the outcome of posterior urethral valves we studied all cases of valves detected prenatally, including cases of pregnancy termination due to posterior urethral valves.

MATERIALS AND METHODS

Between 1989 and 1996, 30 neonates with prenatally detected posterior urethral valves were treated at our hospital. The prenatal parameters analyzed were age of gestation at diagnosis, ultrasonographic appearance of renal parenchyma and amniotic fluid volume. Fetal urine was analyzed in 9 cases. We reviewed the outcome of 10 neonates treated for posterior urethral valves which were not diagnosed prenatally during the same period.

RESULTS

Of the 30 neonatal survivors 6 (20%) had renal failure, including end stage renal disease in 2, after a mean followup of 4 years. Renal failure developed in 2 of 5 cases detected before 24 weeks of gestation, in 1 of 6 with oligohydramnios and in 2 of 5 with abnormal parenchymal renal ultrasound. Normal parenchymal ultrasound and amniotic volume could not predict for good outcome. Renal failure developed in 2 of 7 cases predicted by fetal urinalysis as good prognosis and in 1 of 2 cases predicted as poor prognosis. Pregnancy was terminated for posterior urethral valves in 5 cases based on prenatal criteria of severe renal impairment. Considering these cases as poor outcome, the rate of poor prognosis increased from 20 to 31%. Among the 10 neonates without a prenatal diagnosis of posterior urethral valves renal failure developed in 2 (20%), including end stage renal disease in 1.

CONCLUSIONS

When negative parameters were absent and/or fetal urine predicted good outcome there were no cases of end stage renal disease in early infancy, which was a significant help in parent counseling. The predictive value of the currently available prenatal parameters needs to be updated with larger series specifically dealing with posterior urethral valves. According to the current data, the outcome of posterior urethral valves is not yet significantly improved by prenatal diagnosis.

摘要

目的

为评估产前诊断及评估对后尿道瓣膜症预后的影响,我们研究了所有产前检测出瓣膜症的病例,包括因后尿道瓣膜症而终止妊娠的病例。

材料与方法

1989年至1996年间,我院治疗了30例产前检测出后尿道瓣膜症的新生儿。分析的产前参数包括诊断时的孕周、肾实质的超声表现及羊水量。9例分析了胎儿尿液。我们回顾了同期10例未产前诊断出后尿道瓣膜症而接受治疗的新生儿的预后情况。

结果

30例新生儿幸存者中,平均随访4年后,6例(20%)出现肾衰竭,其中2例为终末期肾病。妊娠24周前检测出的5例中有2例出现肾衰竭,羊水过少的6例中有1例出现肾衰竭,肾实质超声异常的5例中有2例出现肾衰竭。肾实质超声及羊水量正常并不能预测良好预后。胎儿尿液分析预测预后良好的7例中有2例出现肾衰竭,预测预后不良的2例中有1例出现肾衰竭。基于严重肾功能损害的产前标准,5例因后尿道瓣膜症而终止妊娠。将这些病例视为不良预后,不良预后率从20%升至31%。10例未产前诊断出后尿道瓣膜症的新生儿中,2例(20%)出现肾衰竭,其中1例为终末期肾病。

结论

当不存在负面参数和/或胎儿尿液预测预后良好时,婴儿早期无终末期肾病病例,这对向家长咨询有很大帮助。现有产前参数的预测价值需要通过专门针对后尿道瓣膜症的更大样本系列进行更新。根据目前的数据,产前诊断尚未显著改善后尿道瓣膜症的预后。

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