Better Nutritional Status in Early Childhood Is Associated with Improved Clinical Outcomes and Survival in Patients with Cystic Fibrosis

Abstract

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. Prospective, observational study using data from the Cystic Fibrosis Foundation Registry (US) for patients born between 1989 and 1992 (n=3142). 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. 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.