Elsevier

The Journal of Pediatrics

Volume 162, Issue 3, March 2013, Pages 530-535.e1
The Journal of Pediatrics

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

https://doi.org/10.1016/j.jpeds.2012.08.040Get rights and content

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.

Section snippets

Methods

This is a prospective, observational study of patients with CF in the US. Data were extracted from the CFF Patient Registry (“registry”) for patients born between 1989 and 1992. This range was chosen to allow for maximum follow-up of patients born in the era of a modern approach to nutrition management. The registry contains information on over 25 500 patients who receive their care in a CFF-accredited care center; we analyzed data from 3142 patients born during the time period of interest.

Results

The cohort consists of 3142 patients, mostly Caucasian, with a slight male predominance (Table I; available at www.jpeds.com). Most patients were diagnosed by 5 months of age, primarily with gastrointestinal manifestations of disease. The frequency of the F508del allele in the cohort was 72% among those who had a genotype entered in the registry.

Discussion

Several short-term studies have shown associations between various measures of nutritional status in young children and lung function or survival.8, 9 Longer-term studies have demonstrated that either improvement or worsening of nutritional status correlated with either better or worse lung function,10 that short stature was negatively correlated with survival,11 and that children who recovered their birth weight z-scores by age 2 years had better lung function at age 6 years.12 Our lung

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    Funded by Cystic Fibrosis Foundation (BOROWI03CS0 to D.B. and OCONNO01E0 to H.Q.). The authors declare no conflicts of interest.

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