Girls born with congenital diaphragmatic hernia (CDH), a rare birth defect that affects approximately one in 3600 babies in the United States, are slightly less likely to survive than their male counterparts with the condition, a study published in the Journal of Pediatrics has found.
The retrospective cohort study used demographic, clinical, and outcomes data from the Congenital Diaphragmatic Hernia Study Group registry to investigate the role of biological sex for babies with CDH.
The study included more than 7200 newborns at 105 hospitals across 17 countries from January 2007 to December 2018.
The primary outcomes were 30-day, 60-day, and in-hospital mortality.
The secondary outcomes included weight gain during admission, feeding, and oxygen status at 30 days and at discharge.
After controlling for markers of disease severity, girls with CDH had a 32% increased risk for death at 30-days (adjusted hazard ratio, 1.32; P = .02).
Girls had lower survival rates at 30-days (77.3%) compared with boys (80.1%).
The disparity remained at 60 days, with the survival rate for girls with CDH (73.5%) modestly lower than that for boys (77.2%), and also at discharge — 70.2% compared with 74.2%, respectively.
The survival differences between boys and girls primarily affected babies who did not undergo extracorporeal life support cannulation; in this group, the survival rate for girls was 77.5% compared with 82.1% for boys.
Girls with CDH weighed less at birth (2.8 kg) than boys with the condition (3 kg); birth weight is a predictor of mortality risk for babies with CDH, according to the authors .
“Although racial and ethnic outcome disparities have been documented in CDH, disparities between males and females are not well known,” Shaun Kunisaki, MD, MSc, a senior author of the study and professor of surgery at Johns Hopkins University in Baltimore, said in a press release about the findings. “It is really important to understand if those disparities exist, because it may change how we can better manage these patients.”
The authors include seven from the Division of General Pediatric Surgery and the Eudowood Division of Pediatric Respiratory Sciences at Johns Hopkins University School of Medicine. Two authors are from the Department of Pediatric Surgery at the University of Texas McGovern Medical School and Children’s Memorial Hermann Hospital, both in Houston.
The authors point to potential challenges with data collection, including diagnosis miscoding and missing data. They acknowledge that MRI fetal lung volumes, degree of liver herniation, and emerging markers of socioeconomic status were not available in the database. The study also could not examine long-term outcomes because data were limited to in-hospital variables.
The authors report no financial conflicts of interest.
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