Antenatal oligohydramnios of renal origin: long-term outcome
Antenatal oligohydramnios of renal origin: long-term outcome
复制标题
肾源性产前少尿：长期结果
DOI：
10.1093/ndt/gfl591
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发表时间：
2007-02-01
影响因子：
6.1
通讯作者：
Kemper, Markus J.
中科院分区：
文献类型：
--
作者：
Klaassen, Ilka;Neuhaus, Thomas J.;Kemper, Markus J.
背景。肾性羊水过少（ROH）胎儿的预后通常仍被认为很差。本文描述了两个儿科中心的新生儿并发症和长期随访的胎儿与ROH。23例胎儿（男16例，女7例）作为患者。原发疾病包括先天性肾脏和尿路异常（n = 16）、常染色体隐性多囊肾病（n = 4）和肾小管发育不良（n = 3）。分析包括回顾性图表回顾。7例患儿死亡（30%），其中多数（n = 4,17 %）发生在新生儿期，死因是肺发育不全和肾功能不全。14名患者（61%）需要产后机械通气，时间中位数为4天（范围1-60天）；11名婴儿并发气胸。所有存活的16名儿童均患有慢性肾脏疾病（CKD），目前中位年龄为5.7岁（范围0.5-14.5），其中8名患者进行了保守治疗[中位肾小球滤过率51（范围20-78）ml/min/1.73 m(2)]。8例患者中位年龄为0.3岁（2天至8.3岁）达到终末期肾病，其中1例患者进行了先发制人的肾移植。5例需要透析的患者在中位年龄3.5岁（范围2.5-4岁）时成功进行了肾移植。需要生长激素治疗的7名儿童生长受损。16例患者中有12例（75%）认知和运动发育正常，4例患儿出现发育迟缓，其中2例伴有相关综合征特征。ROH并不总是与预后不良相关，幸存者的长期预后令人鼓舞。由于CKD引起的新生儿并发症和长期发病率高，需要对这些儿童进行多学科管理。
Background. Prognosis of fetuses with renal oligohydramnios (ROH) is often still regarded as poor. Neonatal complications and the long-term follow-up of fetuses with ROH in two pediatric centres are described.Method. 23 fetuses (16 males, 7 females) were included as patients. Primary diseases included congenital anomalies of the kidney and urinary tract (n = 16), autosomal recessive polycystic kidney disease (n = 4) and renal tubular dysgenesis (n = 3). The analysis includes retrospective chart review.Results. Seven children died (30%), the majority (n = 4, 17%) within the neonatal period due to pulmonary hypoplasia and renal insufficiency. Fourteen patients (61%) required postnatal mechanical ventilation for a median of 4 (range 1-60) days; 11 infants had an associated pneumothorax. All 16 surviving children have chronic kidney disease (CKD) at a current median age of 5.7 years (range 0.5-14.5), managed conservatively in eight patients [median glomerular filtration rate 51 (range 20-78) ml/min/1.73 m(2)]. Eight patients reached end-stage renal disease at a median age of 0.3 years (range 2 days to 8.3 years), including one patient with pre-emptive kidney transplantation. Five of the patients requiring dialysis underwent successful renal transplantation at a median age of 3.5 years (range 2.5-4). Growth was impaired in seven children requiring growth hormone treatment. Cognitive and motor development was normal in 12 (75%) of the 16 patients and showed a delay in four children, including two with associated syndromal features.Conclusion. ROH is not always associated with a poor prognosis and long-term outcome in survivors is encouraging. The high incidence of neonatal complications and long-term morbidity due to CKD requires a multidisciplinary management of these children.