Elsevier

The Journal of Pediatrics

Volume 153, Issue 6, December 2008, Pages 752-757
The Journal of Pediatrics

Original article
Improvements in Lung Function Outcomes in Children with Cystic Fibrosis are Associated with Better Nutrition, Fewer Chronic Pseudomonas aeruginosa Infections, and Dornase Alfa Use

https://doi.org/10.1016/j.jpeds.2008.07.011Get rights and content

Objective

To compare lung function and nutritional outcomes in cystic fibrosis (CF) for 2 birth cohorts in our CF center.

Study design

Patients with CF born between 1985 and 2000 treated in our CF center before age 5 years were included. The patients were divided into 2 equal birth cohorts for comparison: birth cohort 1 (born between 1985 and 1992) and birth cohort 2 (born between 1993 and 2000). To compare lung function, we used forced expiratory volume in the first second (FEV1)% predicted and FEV1% predicted slope from age 6 to 12 years. We hypothesized that we would find significant improvements in lung function and nutritional outcomes in our patients with CF.

Results

The patients born between 1993 and 2000 (birth cohort 2) had better lung function, a slower rate of decline in lung function, and better nutritional outcomes compared with those born between 1985 and 1992 (birth cohort 1). Factors associated with a slower rate of decline in lung function in both groups were a higher baseline body mass index (BMI)%, a slower BMI% rate of decline, absence of chronic Pseudomonas aeruginosa respiratory infection, and initiation of dornase alfa (Pulmozyme) therapy before age 9 years.

Conclusion

Our results demonstrate dramatically improved lung function and nutritional outcomes in the children with CF in our center. The improvements in lung function outcomes are associated with better nutrition, fewer chronic P aeruginosa infections, and dornase alfa therapy.

Section snippets

Methods

We reviewed our database for lung function and nutritional data for all patients with CF born between 1985 and 2000 treated in our CF center at the Cincinnati Children's Hospital Medical Center before age 5 years. The patients were divided into 2 equal birth cohorts: birth cohort 1 (born between 1985 and 1992) and birth cohort 2 (born between 1993 and 2000).

Lung Function Outcomes

A total of 144 patients with CF born between 1985 and 2000 met the inclusion criteria for comparison of lung function outcomes. There were no significant differences in terms of race, sex, genotype, age of diagnosis, prevalence of pancreatic enzyme supplementation, or prevalence of chronic S aureus infection between birth cohort 1 and birth cohort 2 (Table I). Significant differences were found between the 2 cohorts in the prevalence of chronic P aeruginosa infection (P = .002), the prevalence

Discussion

This longitudinal cohort analysis has demonstrated a statistically significant improvement in the rate of decline in lung function in patients with CF. Studies analyzing the rate of decline in lung function in CF have yielded varied findings. Impaired glucose tolerance and diabetes have been associated with a faster decline in lung function.13, 14 Several studies, including ours, found an association between chronic P aeruginosa respiratory tract infection and a faster decline in lung function.

References (23)

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  • Cited by (62)

    • Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline

      2021, Journal of the Academy of Nutrition and Dietetics
      Citation Excerpt :

      Rationale: Undernutrition is a common concern for individuals with CF due to increased energy expenditure and decreased nutrient absorption.54 In the systematic review of the literature, undernutrition demonstrated a clear longitudinal relationship with lung function decline and mortality.30-53 Participants who maintained a weight, length, weight-for-length, and BMI >50th percentile from infancy and early childhood had higher FEV1% (percent forced expiratory volume in 1 second) predicted values 4 to 16 years later, although there was no added improvement for those who maintained growth parameters >85th percentile compared with >50th percentile (Table).31-46

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    The authors have no conflicts of interest to disclose. There was no sponsor involvement in the design of this study or in the preparation of this manuscript for publication.

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