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胎儿下尿路梗阻的定义、诊断和管理:ERKNet CAKUT-梗阻性尿路病工作组的共识。

Definition, diagnosis and management of fetal lower urinary tract obstruction: consensus of the ERKNet CAKUT-Obstructive Uropathy Work Group.

机构信息

Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy.

Fetal Medicine and Surgery Service, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

出版信息

Nat Rev Urol. 2022 May;19(5):295-303. doi: 10.1038/s41585-022-00563-8. Epub 2022 Feb 8.

Abstract

Fetal lower urinary tract obstruction (LUTO) is associated with high mortality and postnatal morbidity caused by lung hypoplasia and impaired kidney function. Specific diagnostic features that can guide clinical approach and decisions are lacking; thus, the European Reference Network for Rare Kidney Diseases established a work group to develop recommendations regarding the clinical definition, diagnosis and management of prenatally detected LUTO. The work group recommends the use of antero-posterior diameter of renal pelvis as the most reliable parameter for suspecting obstructive uropathies and for suspecting prenatal LUTO in the presence of fetal megacystis. Regarding prenatal and postnatal prognosis of fetuses with LUTO, the risk of fetal and neonatal death depends on the presence of oligohydramnios or anhydramnios before 20 weeks' gestation, whereas the risk of kidney replacement therapy cannot be reliably foreseen before birth. Parents of fetuses with LUTO must be referred to a tertiary obstetric centre with multidisciplinary expertise in prenatal and postnatal management of obstructive uropathies, and vesico-amniotic shunt placement should be offered in selected instances, as it increases perinatal survival of fetuses with LUTO.

摘要

胎儿下尿路梗阻(LUTO)与肺发育不全和肾功能受损导致的高死亡率和产后发病率有关。缺乏可以指导临床方法和决策的特定诊断特征;因此,欧洲罕见肾脏疾病参考网络成立了一个工作组,制定关于产前检测到的 LUTO 的临床定义、诊断和管理的建议。工作组建议使用肾盂前后直径作为怀疑梗阻性尿路疾病和怀疑存在胎儿巨膀胱时怀疑产前 LUTO 的最可靠参数。关于 LUTO 胎儿的产前和产后预后,胎儿和新生儿死亡的风险取决于 20 周妊娠前是否存在羊水过少或无羊水,而在出生前无法可靠预测接受肾脏替代治疗的风险。LUTO 胎儿的父母必须转介到具有产前和产后梗阻性尿路疾病管理专业知识的三级产科中心,并且应在选定的情况下提供膀胱羊膜分流术,因为它可以提高 LUTO 胎儿的围产期存活率。

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