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Evaluation of Variability in Inhaled Nitric Oxide Use and Pulmonary Hypertension in Patients With Congenital Diaphragmatic Hernia.先天性膈疝患者吸入一氧化氮使用和肺动脉高压的变异性评估。
JAMA Pediatr. 2016 Dec 1;170(12):1188-1194. doi: 10.1001/jamapediatrics.2016.2023.
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Conventional Mechanical Ventilation Versus High-frequency Oscillatory Ventilation for Congenital Diaphragmatic Hernia: A Randomized Clinical Trial (The VICI-trial).先天性膈疝的传统机械通气与高频振荡通气：一项随机临床试验（VICI试验）
Ann Surg. 2016 May;263(5):867-74. doi: 10.1097/SLA.0000000000001533.
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A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns.新生儿先天性膈疝严重程度的临床预测规则。
Pediatrics. 2014 Aug;134(2):e413-9. doi: 10.1542/peds.2013-3367. Epub 2014 Jul 14.
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Extracorporeal Life Support Organization Registry Report 2012.体外生命支持组织登记报告 2012.
ASAIO J. 2013 May-Jun;59(3):202-10. doi: 10.1097/MAT.0b013e3182904a52.
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Hypercapnia and acidosis during open and thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia: results of a pilot randomized controlled trial.先天性膈疝和食管闭锁开放和胸腔镜修复期间的高碳酸血症和酸中毒：一项初步随机对照试验的结果。
Ann Surg. 2013 Dec;258(6):895-900. doi: 10.1097/SLA.0b013e31828fab55.
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Outcomes of congenital diaphragmatic hernia in the modern era of management.先天性膈疝在现代管理时代的结果。
J Pediatr. 2013 Jul;163(1):114-9.e1. doi: 10.1016/j.jpeds.2012.12.036. Epub 2013 Jan 30.
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Minimally invasive repair of congenital diaphragmatic hernia.先天性膈疝的微创修复。
J Pediatr Surg. 2011 Jun;46(6):1158-64. doi: 10.1016/j.jpedsurg.2011.03.050.
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Long term follow-up in high-risk congenital diaphragmatic hernia survivors: patching the diaphragm affects the outcome.高危先天性膈疝幸存者的长期随访：修补膈肌会影响结果。
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Congenital hernia of the diaphragm.先天性膈疝
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先天性膈疝的治疗策略：改变有时会带来礼物。

Treatment Strategies for Congenital Diaphragmatic Hernia: Change Sometimes Comes Bearing Gifts.

作者信息

Morini Francesco, Lally Kevin P, Lally Pamela A, Crisafulli Rosa Maria, Capolupo Irma, Bagolan Pietro

机构信息

Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Department of Pediatric Surgery, McGovern Medical School at the University of Texas Health Science Center at Houston, Children's Memorial Hermann Hospital, Houston, TX, United States.

出版信息

Front Pediatr. 2017 Sep 14;5:195. doi: 10.3389/fped.2017.00195. eCollection 2017.

DOI:10.3389/fped.2017.00195
PMID:28959686
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5603669/
Abstract

OBJECTIVE

To report treatment strategies' evolution and its impact on congenital diaphragmatic hernia (CDH) outcome.

DESIGN

Registry-based cohort study using the CDH Study Group database, 1995-2013.

SETTING

International multicenter database.

PATIENTS

CDH patients entered into the registry. Late presenters or patients with very incomplete data were excluded. Patients were divided into three Eras (1995-2000; 2001-2006; 2007-2013).

MAIN OUTCOME MEASURES

Treatment strategies and outcomes. One-way ANOVA, X2 test, and X2 test for trend were used. A Sydak-adjusted  < 0.0027 was considered significant. Prevalence or mean (SE) are reported.

RESULTS

Patients: 8,603; included: 7,716; Era I: 2,146; Era II: 2,572; Era III: 2,998. From Era I to Era III, significant changes happened. Some severity indicators such as gestational age, prevalence of prenatal diagnosis, and inborn patients significantly worsened. Also, treatment strategies such as the use of prenatal steroids and inhaled nitric oxide, age at operation, prevalence of minimal access surgery, and the use of surfactant significantly changed. Finally, length of hospital stay became significantly longer and survival to discharge slightly but significantly improved, from 67.7 to 71.4% ( for trend 0.0019).

CONCLUSION

Treatment strategies for patients registered since 1995 in the CDH Study Group significantly changed. Survival to discharge slightly but significantly improved.

摘要

目的

报告治疗策略的演变及其对先天性膈疝（CDH）治疗结果的影响。

设计

基于1995 - 2013年CDH研究组数据库的登记队列研究。

设置

国际多中心数据库。

患者

纳入登记的CDH患者。排除就诊较晚或数据极不完整的患者。患者分为三个时期（1995 - 2000年；2001 - 2006年；2007 - 2013年）。

主要观察指标

治疗策略和治疗结果。采用单向方差分析、卡方检验和趋势卡方检验。经西德克校正后P < 0.0027被认为具有统计学意义。报告患病率或均值（标准误）。

结果

患者总数8603例；纳入7716例；时期I：2146例；时期II：2572例；时期III：2998例。从时期I到时期III发生了显著变化。一些严重程度指标如孕周、产前诊断患病率和先天性患者显著变差。同时，治疗策略如产前使用类固醇和吸入一氧化氮、手术年龄、微创手术患病率以及表面活性剂的使用也发生了显著变化。最后，住院时间显著延长，出院生存率略有但显著提高，从67.7%提高到71.4%（趋势P值为0.0019）。

结论

自1995年起在CDH研究组登记的患者治疗策略发生了显著变化。出院生存率略有但显著提高。