Defect size determines survival in infants with congenital diaphragmatic hernia
Defect size determines survival in infants with congenital diaphragmatic hernia
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DOI：
10.1542/peds.2006-3040
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发表时间：
2007-09-01
期刊：
影响因子：
8
通讯作者：
Hirschl, Ronald B.
中科院分区：
文献类型：
--
作者：
Lally, Kevin P.;Lally, Pamela A.;Hirschl, Ronald B.
目标。先天性横隔疝是新生儿死亡的一个重要原因。这项研究的目的是通过使用大型多中心数据集来评估与先天性横隔疝婴儿死亡相关的临床因素。方法：这是一项前瞻性队列研究，研究对象为1995-2004年间在先天性横隔疝研究组第三转诊中心接受护理的所有活产儿。被认为影响死亡的因素包括出生体重、阿普加评分、缺陷大小以及相关的异常。结果：共有8个国家的51个中心提供了3062名活产婴儿的数据。总存活率为69%。538例(18%)患者未接受手术并死亡。缺损的大小是影响预后的最重要因素；与初次修复的婴儿相比，几乎没有横隔膜的婴儿存活率为57%，存活率为95%。与初次修补相比，无发育不良但需要补片修补的婴儿的存活率为79%。结论：横隔膜缺损区的大小似乎是影响先天性横隔疝患儿预后的主要因素。缺损区大小可能是反映肺发育不良程度的替代指标。未来的研究工作应指向准确量化肺发育不良的程度或缺陷大小。然后，实验疗法可以有针对性地前瞻性地识别更有可能受益的高危患者。
OBJECTIVES. Congenital diaphragmatic hernia is a significant cause of neonatal mortality. The objective of this study was to evaluate the clinical factors associated with death in infants with congenital diaphragmatic hernia by using a large multicenter data set.METHODS. This was a prospective cohort study of all liveborn infants with congenital diaphragmatic hernia who were cared for at tertiary referral centers belonging to the Congenital Diaphragmatic Hernia Study Group between 1995 and 2004. Factors thought to influence death included birth weight, Apgar scores, size of defect, and associated anomalies. Survival to hospital discharge, duration of mechanical ventilation, and length of hospital stay were evaluated as end points.RESULTS. A total of 51 centers in 8 countries contributed data on 3062 liveborn infants. The overall survival rate was 69%. Five hundred thirty- eight ( 18%) patients did not undergo an operation and died. The defect size was the most significant factor that affected outcome; infants with a near absence of the diaphragm had a survival rate of 57% compared with infants having a primary repair with a survival rate of 95%. Infants without agenesis but who required a patch for repair had a survival rate of 79% compared with primary repair.CONCLUSIONS. The size of the diaphragmatic defect seems to be the major factor influencing outcome in infants with congenital diaphragmatic hernia. It is likely that the defect size is a surrogate marker for the degree of pulmonary hypoplasia. Future research efforts should be directed to accurately quantitate the degree of pulmonary hypoplasia or defect size antenatally. Experimental therapies can then be targeted to prospectively identify high- risk patients who are more likely to benefit.