Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis

•The association between BMI and FEV1 in CF differs significantly by pancreatic status (PS vs PI).•Higher BMI was associated with a smaller slope of FEV1 increase in PS-CF.•In PS-CF, BMI about the 85th percentile may be associated with decreasing FEV1.•The decline in FEV1 with age is minimal in PS-CF and does not differ by BMI. There is a strong association between nutrition and long-term FEV1 in cystic fibrosis (CF), but studies have been driven by data from subjects with pancreatic insufficiency (PI-CF). We thus evaluated the association between body mass index (BMI) and FEV1 percent-predicted (FEV1pp) in children with pancreatic sufficiency (PS-CF) and contrasted it with the association in PI-CF. We utilized data from the CF Foundation Patient Registry. The cohort included children born 1995–2010, diagnosed