Nihilism in the 1990s: The true mortality of congenital diaphragmatic hernia
Nihilism in the 1990s: The true mortality of congenital diaphragmatic hernia
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DOI：
10.1542/peds.112.3.532
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发表时间：
2003-09-01
期刊：
影响因子：
8
通讯作者：
Jaffray, B
中科院分区：
文献类型：
--
作者：
Stege, G;Fenton, A;Jaffray, B
Objective.报告的先天性腹股沟疝（CDH）生存率不允许病例选择偏倚。本研究报告了11年来在地理上确定的人群中CDH的发病率，并评估了避免病例选择时新疗法（高频振荡通气、体外膜肺氧合、吸入一氧化氮和延迟手术）对生存率的影响。对来自区域病例登记处的病例进行回顾性分析，即北方地区先天性畸形调查。共发现185例病例。死亡率为62%，在研究期间没有显著变化。死亡率不受新疗法的影响。选择性终止率与存活率呈显著负相关。额外异常的存在使死亡率增加至79%。当完成病例确定时，CDH的死亡率不受新疗法的影响。存活率主要取决于产前终止妊娠率和相关畸形的发生率。CDH生存率提高的报告应谨慎解释，因为结局的变化更可能由病例选择伪影解释。
Objective. Reported survival in congenital diaphragmatic hernia (CDH) fails to allow for case selection bias. This study reports the incidence of CDH in a geographically defined population over 11 years and assesses the effect of new therapies (high-frequency oscillatory ventilation, extracorporeal membrane oxygenation, inhaled nitric oxide, and delayed surgery) on survival when case selection is avoided.Methods. A retrospective review of cases from a regional case registry, the Northern Region Congenital Anomaly Survey, was conducted.Results. A total of 185 cases were identified. Mortality was 62% and did not vary significantly during the study period. Mortality was unaffected by the introduction of new therapies. There was a significant inverse correlation between the rate of elective termination and survival of live borns. The presence of an additional anomaly increased mortality to 79%.Conclusions. The mortality of CDH when complete case ascertainment is achieved is unaffected by new therapies. The survival rate is principally determined by the rate of antenatal termination and the incidence of associated anomalies. Reports of improved survival of CDH should be interpreted with caution, as variations in outcome are more likely to be explained by case selection artifact.