Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis

Objectives: To evaluate the relationship between nutritional status early in life and the timing and velocity of height growth, lung function, complications of cystic fibrosis, and survival. Study design: Prospective, observational study using data from the Cystic Fibrosis Foundation Registry (US) for patients born between 1989 and 1992 (n = 3142). Results: Weight-for-age percentile (WAP) at 4 years of age was positively associated with height-for-age percentiles throughout childhood. Age 4 years WAP >10% was associated with better lung function from 6-18 years of age. In boys and girls with current WAP >50%, peak pubertal height velocities approximated but remained lower than that of the healthy reference population. By age 18 years, patients with an age 4 years WAP >50% suffered fewer acute pulmonary exacerbations, spent fewer days in the hospital, and had lower rates of impaired glucose tolerance or diabetes. Patients attaining higher age 4 years WAP and height-for-age percentiles had a survival advantage throughout childhood. Conclusion: For the population studied, greater weight at age 4 years is associated with greater height, better pulmonary function, fewer complications of cystic fibrosis, and better survival through age 18 years. Furthermore, greater weight-for-age in the peripubertal period is associated on average with improved tempo and timing of pubertal height growth.