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先天性肾脏和尿路异常的产前诊断后的结局

Outcome after prenatal diagnosis of congenital anomalies of the kidney and urinary tract.

作者信息

Nef Samuel, Neuhaus Thomas J, Spartà Giuseppina, Weitz Marcus, Buder Kathrin, Wisser Josef, Gobet Rita, Willi Ulrich, Laube Guido F

机构信息

University Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032, Zurich, Switzerland.

Children's Hospital Lucerne, Lucerne, Switzerland.

出版信息

Eur J Pediatr. 2016 May;175(5):667-76. doi: 10.1007/s00431-015-2687-1. Epub 2016 Jan 25.

Abstract

UNLABELLED

Congenital anomalies of the kidney and urinary tract are common findings on fetal ultrasound. The aim of this prospective observational study was to describe outcome and risk factors in 115 patients born 1995-2001. All prenatally diagnosed children were stratified into low- and high-risk group and followed postnatally clinically and by imaging at defined endpoints. Risk factors were evaluated using odds ratios. Neonatal diagnosis included pelvi-ureteric junction obstruction (n = 33), vesicoureteral reflux (n = 27), solitary mild pelvic dilatation (postnatal anteroposterior diameter 5-10 mm; n = 25), and further diagnosis as primary obstructive megaureter, unilateral multicystic dysplastic kidney, renal dysplasia and posterior urethral valves. In 38 children with prenatal isolated hydronephrosis, ultrasound normalized at median age of 1.2 years (range 0.1-9). Surgery was performed in 34 children at median age of 0.4 years (0.1-10.8). Persistent renal anomalies without surgery were present in 43 children and followed in 36 for median time of 16 years (12.2-18). Oligohydramnios and postnatal bilateral anomalies were significantly associated with surgery and impaired renal function.

CONCLUSION

The majority of children had a favourable postnatal outcome, in particular children with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis. Oligohydramnios and postnatal bilateral anomalies were risk factors for non-favourable outcome.

WHAT IS KNOWN

• In congenital anomalies of the kidney and urinary tract significantly poorer outcome is known in patients with bilateral renal hypoplasia or solitary kidney associated with posterior urethral valves. • Other factors as proteinuria and vesicoureteral reflux were associated with a higher risk of progression to chronic renal failure in these patients. What is New: • Unlike other studies giving us above-mentioned information, we included all patients with any kind of prenatally diagnosed congenital anomalies of the kidney and urinary tract. Our study shows long-term follow up (median 16 years, range 12.2-18 years), especially in patients not needing surgery, but with persistent anomalies. • During postnatal long-term follow up (median 2.2 years, range 0.1-18 years) one third each showed normalization, need of surgery or persistence of anomalies without need of surgery. Our study revealed a good prognosis in the majority of these children, in particular with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis, and revealed oligohydramnios and postnatal bilateral anomalies as risk factors for a non-favourable outcome, defined as need of surgery, persistent anomalies with impaired renal function, end stage renal failure or death.

摘要

未标注

先天性肾和尿路异常是胎儿超声检查中常见的发现。这项前瞻性观察性研究的目的是描述1995年至2001年出生的115例患者的结局及危险因素。所有产前诊断的儿童被分为低风险组和高风险组,并在出生后进行临床随访及在特定时间点进行影像学检查。使用比值比评估危险因素。新生儿诊断包括肾盂输尿管连接处梗阻(n = 33)、膀胱输尿管反流(n = 27)、孤立性轻度肾盂扩张(出生后前后径5 - 10毫米;n = 25),以及进一步诊断为原发性梗阻性巨输尿管、单侧多囊性发育不良肾、肾发育不良和后尿道瓣膜。在38例产前孤立性肾积水患儿中,超声在中位年龄1.2岁时恢复正常(范围0.1 - 9岁)。34例患儿在中位年龄0.4岁时接受了手术(0.1 - 10.8岁)。43例患儿存在持续性肾异常且未接受手术,并对其中36例进行了中位时间为16年的随访(12.2 - 18年)。羊水过少和出生后双侧异常与手术及肾功能受损显著相关。

结论

大多数儿童出生后结局良好,尤其是产前低风险的儿童,即孤立性单侧或双侧肾积水。羊水过少和出生后双侧异常是不良结局的危险因素。

已知信息

• 在先天性肾和尿路异常中,双侧肾发育不全或与后尿道瓣膜相关的孤立肾患者的结局明显较差。• 蛋白尿和膀胱输尿管反流等其他因素与这些患者进展为慢性肾衰竭的较高风险相关。新发现:• 与其他提供上述信息的研究不同,我们纳入了所有产前诊断为任何类型先天性肾和尿路异常的患者。我们研究显示了长期随访(中位时间16年,范围12.2 - 18年),特别是在不需要手术但存在持续性异常的患者中。• 在出生后长期随访(中位时间2.2年,范围0.1 - 18年)中,各有三分之一的患儿分别显示正常、需要手术或存在无需手术的持续性异常。我们的研究显示这些儿童中的大多数预后良好,尤其是产前低风险的儿童,即孤立性单侧或双侧肾积水,并显示羊水过少和出生后双侧异常是不良结局的危险因素,不良结局定义为需要手术、存在肾功能受损的持续性异常、终末期肾衰竭或死亡。

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