Klaassen Ilka, Neuhaus Thomas J, Mueller-Wiefel Dirk E, Kemper Markus J
Pediatric Nephrology, University Medical Center, Hamburg-Eppendorf, Martinistrasse 52, D-20246 Hamburg, Germany.
Nephrol Dial Transplant. 2007 Feb;22(2):432-9. doi: 10.1093/ndt/gfl591. Epub 2006 Oct 25.
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.
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.
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.
羊水过少胎儿(ROH)的预后通常仍被认为较差。本文描述了两个儿科中心中ROH胎儿的新生儿并发症及长期随访情况。方法:纳入23例胎儿(16例男性,7例女性)作为研究对象。主要疾病包括先天性肾和尿路畸形(n = 16)、常染色体隐性多囊肾病(n = 4)和肾小管发育不全(n = 3)。分析方法为回顾性病历审查。
7例患儿死亡(30%),其中大多数(n = 4,17%)在新生儿期因肺发育不全和肾功能不全死亡。14例患者(61%)出生后需要机械通气,中位时间为4天(范围1 - 60天);11例婴儿伴有气胸。16例存活儿童目前中位年龄为5.7岁(范围0.5 - 14.5岁)时均患有慢性肾脏病(CKD),8例患儿接受保守治疗[中位肾小球滤过率51(范围20 - 78)ml/min/1.73 m²]。8例患者在中位年龄0.3岁(范围2天至8.3岁)时发展为终末期肾病,其中1例患者接受了肾移植。5例需要透析的患者在中位年龄3.5岁(范围2.5 - 4岁)时成功接受了肾移植。7例需要生长激素治疗的儿童生长发育受损。16例患者中有12例(75%)认知和运动发育正常,4例儿童发育延迟,其中2例伴有相关综合征特征。
ROH并非总是与不良预后相关,幸存者的长期预后令人鼓舞。由于CKD导致的新生儿并发症和长期发病率较高,需要对这些儿童进行多学科管理。