The Association Between Meconium Ileus and Nutritional and Pulmonary Outcomes in Children With Cystic Fibrosis
Don B. Sanders, Yash Shah, Jorge M. Gonzales Cordova, Jessica L. Saunders, Clement L. Ren, Arthur H. OworaABSTRACT
Background
Meconium ileus (MI) affects up to 20% of newborns with cystic fibrosis (CF). We compared clinical outcomes among children with CF in the US CF Foundation Patient Registry (CFFPR) by meconium ileus diagnosis and severity.
Methods
This was a retrospective cohort study of children with CF with and without MI in the CFFPR born between 2005 and 2017. Growth and pulmonary outcomes were compared by MI severity using linear mixed models.
Results
10,008 children with CF met inclusion criteria, including 1558 (15.6%) with MI. A higher proportion of children with MI had Class I–III CFTR mutations, infections with Pseudomonas aeruginosa and methicillin‐resistant Staphylococcus aureus (MRSA), and nutritional interventions. Among children with MI, 450 (28.8%) of children with MI had a gastrostomy tube (MI‐GT). 142 (9.1%) had a perforation and 346 (22.2%) had no perforation; 620 (39.8%) did not have perforation status recorded and were excluded from analyses. Growth z‐scores through age 2 years were significantly lower in the MI‐GT group compared to other MI and non‐MI categories. Percentage predicted forced expiratory volume in 1 s (ppFEV1) was also significantly lower in the MI‐GT group compared to other groups. Differences in growth and ppFEV1 became less pronounced with increasing age (age x MI category interaction p < 0.01).
Conclusions
Children with CF and more severe MI, characterized by g‐tube placement, had decreased growth and pulmonary function. Gastrostomy tube placement may indicate a higher risk of poor health outcomes and could serve as a marker of MI severity, warranting attention to prevent subsequent adverse outcomes.