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先天性膈疝:4 家大容量中心 10 年生存、体外膜肺氧合和胎儿镜气管内封堵评估。

Congenital Diaphragmatic Hernia: 10-Year Evaluation of Survival, Extracorporeal Membrane Oxygenation, and Foetoscopic Endotracheal Occlusion in Four High-Volume Centres.

机构信息

Intensive Care and Department of Paediatric Surgery, Erasmus Medical Centre-Sophia Children's Hospital, Rotterdam, The Netherlands.

出版信息

Neonatology. 2018;113(1):63-68. doi: 10.1159/000480451. Epub 2017 Oct 28.

DOI:10.1159/000480451
PMID:29080897
Abstract

BACKGROUND

Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly with significant mortality.

OBJECTIVES

The aim of this study was to determine if there were trends in survival over the last decade and to compare patient populations, treatment options, and survival rates between 4 high-volume centres, and hence determine which factors were associated with survival.

METHODS

In 4 high-volume CDH centres from the CDH EURO Consortium, data from all CDH patients born between 2004 and 2013 were analysed. The predictive value of variables known at birth and the influence of centre-specific treatments (extracorporeal membrane oxygenation, ECMO, and foetoscopic endotracheal occlusion, FETO) on survival were evaluated in multivariable logistic regression analyses.

RESULTS

Nine hundred and seventy-five patients were included in the analysis, of whom 274 (28.1%) died. ECMO was performed in 259 patients, of whom 81 (31.3%) died. One hundred and forty-five patients (14.9%) underwent FETO, and from those 76 patients (52.4%) survived. Survival differed significantly between years (p = 0.006) and between the 4 centres (p < 0.001). In the multivariable logistic regression analysis, lung-to-head ratio, gestational age at birth, ECMO, centre of birth, and year of birth were significantly associated with survival, whereas FETO was not.

CONCLUSIONS

The patient populations were different between centres, which influenced outcomes. There was a significant variability in survival over time and between centres, which should be taken into consideration in the planning of future trials.

摘要

背景

先天性膈疝(CDH)是一种严重的先天性畸形,死亡率很高。

目的

本研究旨在确定过去十年中生存率是否存在趋势,并比较 4 个大容量中心的患者人群、治疗选择和生存率,从而确定哪些因素与生存率相关。

方法

在 CDH EURO 联盟的 4 个大容量 CDH 中心,分析了 2004 年至 2013 年期间所有出生的 CDH 患者的数据。在多变量逻辑回归分析中,评估了出生时已知变量的预测值以及中心特异性治疗(体外膜氧合、ECMO 和胎儿镜气管内闭塞、FETO)对生存率的影响。

结果

共纳入 975 例患者,其中 274 例(28.1%)死亡。259 例患者接受 ECMO 治疗,其中 81 例(31.3%)死亡。145 例患者(14.9%)接受 FETO 治疗,其中 76 例(52.4%)存活。生存率在不同年份(p=0.006)和 4 个中心之间(p<0.001)差异有统计学意义。在多变量逻辑回归分析中,肺与头比、出生时的胎龄、ECMO、出生中心和出生年份与生存率显著相关,而 FETO 则不然。

结论

各中心的患者人群不同,影响了结果。生存率随时间和中心的变化有显著差异,在未来的试验规划中应予以考虑。

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