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有或无胎儿干预的 LUTO 婴儿的长期肾脏和神经发育结局。

Long-term renal and neurodevelopmental outcome in infants with LUTO, with and without fetal intervention.

机构信息

Clinical Lecturer in Fetal Medicine, School of Clinical and Experimental Medicine, University of Birmingham, c/o Birmingham Women's NHS Foundation Trust, Edgbaston, Birmingham, B15 2TG, United Kingdom.

出版信息

Early Hum Dev. 2011 Sep;87(9):607-10. doi: 10.1016/j.earlhumdev.2011.07.004. Epub 2011 Aug 12.

Abstract

Congenital lower urinary tract obstruction (LUTO) is a heterogeneous group of pathologies, the most common being posterior urethral valves (PUV) or urethral atresia. The bladder neck obstruction in utero leads to a spectrum of disease including mild oligohydramnios with normal renal function to a picture of severe oligohydramnios associated with chronic obstructive macro/microcystic renal parenchymal disease leading to chronic renal impairment. These anomalies may be isolated or complex; the latter being associated with other structural or chromosomal abnormalities. If isolated, the congenital bladder neck obstruction may be amenable to in-utero therapy. In a significant proportion of babies affected by LUTO there is severe oligohydramnios (occurring before 20 weeks gestation) and associated with pulmonary hypoplasia, a scenario almost always associated with perinatal death. For those babies that survive the perinatal period there is a significant risk of renal impairment, often necessitating renal dialysis or transplantation in childhood. In addition, there may be other morbidities such as chronic filling anomalies of the bladder that may require treatment.

摘要

先天性下尿路梗阻(LUTO)是一组异质性的病理,最常见的是后尿道瓣膜(PUV)或尿道闭锁。在子宫内膀胱颈部梗阻导致一系列疾病,包括轻度羊水过少伴肾功能正常,到严重羊水过少伴慢性梗阻性巨/微囊状肾实质疾病,导致慢性肾功能损害。这些异常可能是孤立的或复杂的;后者与其他结构或染色体异常有关。如果孤立,先天性膀胱颈部梗阻可能在子宫内治疗。在相当一部分患有 LUTO 的婴儿中,存在严重的羊水过少(发生在 20 周妊娠前)并伴有肺发育不良,这种情况几乎总是与围产儿死亡相关。对于那些在围产期幸存的婴儿,存在显著的肾功能损害风险,通常需要在儿童期进行肾透析或移植。此外,可能存在其他并发症,如膀胱的慢性充盈异常,可能需要治疗。

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