Levison John, Halliday Robert, Holland Andrew J A, Walker Karen, Williams Gary, Shi Edward, Badawi Nadia
Department of Neonatology, The Children's Hospital at Westmead, Sydney, NSW 2145, Australia.
J Pediatr Surg. 2006 Jun;41(6):1049-53. doi: 10.1016/j.jpedsurg.2006.01.073.
The aim of the study was to describe the incidence and survival of infants born with congenital diaphragmatic hernia (CDH) in the state of New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.
A population-based cohort study of all infants inclusive up to 1 month of age diagnosed with CDH in NSW and the ACT between 1992 and 2001 was conducted. Data sources were the NSW and ACT Neonatal Intensive Care Data Collection (Neonatal Intensive Care Units Study), NSW Birth Defects Register, Population Health Research Centre of ACT Health, and NSW Midwives Data Collection. Individual risk factors for mortality were assessed using the chi(2) test with P < .05 being considered statistically significant. Multivariate analysis was performed using logistic regression to adjust for potential confounding variables.
From the databases used, the incidence of CDH in NSW and the ACT was 1 per 3800 births. Of 242 infants identified with CDH, 8% underwent termination of pregnancy, 10% were stillborn after 20 weeks' gestation, and 82% were liveborn. Most liveborn infants (70%) were delivered at term with a 64% survival, whereas 30% were preterm with a 35% survival. For liveborn infants, the overall preoperative mortality was 35% with 56% surviving to discharge. Logistic regression identified a low 5-minute Apgar score, prematurity, and air leak as independent risk factors for mortality.
This population-based study of CDH provides us with baseline data for our states. Mortality is high in preterm infants and in the preoperative period. Avoiding preterm delivery and improving preoperative stabilization are the measures most likely to improve survival.
本研究旨在描述澳大利亚新南威尔士州（NSW）和澳大利亚首都直辖区（ACT）先天性膈疝（CDH）患儿的发病率及生存率。
对1992年至2001年间在NSW和ACT诊断为CDH的所有1月龄及以内婴儿进行基于人群的队列研究。数据来源为NSW和ACT新生儿重症监护数据收集（新生儿重症监护病房研究）、NSW出生缺陷登记册、ACT卫生局人口健康研究中心以及NSW助产士数据收集。使用卡方检验评估个体死亡风险因素，P <.05被认为具有统计学意义。采用逻辑回归进行多变量分析以调整潜在的混杂变量。
根据所使用的数据库，NSW和ACT的CDH发病率为每3800例出生中有1例。在242例确诊为CDH的婴儿中，8%接受了妊娠终止，10%在妊娠20周后死产，82%为活产。大多数活产婴儿（70%）足月分娩，生存率为64%，而30%为早产，生存率为35%。对于活产婴儿，术前总体死亡率为35%，56%存活至出院。逻辑回归确定5分钟阿氏评分低、早产和气漏是死亡的独立风险因素。
这项基于人群的CDH研究为我们所在的州提供了基线数据。早产儿和术前死亡率较高。避免早产和改善术前稳定状态是最有可能提高生存率的措施。
