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

J Pediatr. 2013 Mar;162(3):530-535.e1. doi: 10.1016/j.jpeds.2012.08.040. Epub 2012 Oct 11.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Body Height
  • Body Weight
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / mortality
  • Cystic Fibrosis / physiopathology
  • Female
  • Glucose Intolerance
  • Humans
  • Lung / physiopathology*
  • Male
  • Nutritional Status*
  • Prospective Studies
  • Respiratory Function Tests
  • Survival Analysis