Original articleImprovements in Lung Function Outcomes in Children with Cystic Fibrosis are Associated with Better Nutrition, Fewer Chronic Pseudomonas aeruginosa Infections, and Dornase Alfa Use
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.
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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 DieteticsCitation Excerpt :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 When monitoring clients, RDNs must use clinical judgment to determine whether a child with z scores below this cutoff is actually at nutrition risk or they are stable on a lower growth curve and assessment is not needed.
Seasonal variation of lung function in cystic fibrosis: Longitudinal modeling to compare a Midwest US cohort to international populations
2021, Science of the Total EnvironmentEvaluating the impact of 2006 Australasian Clinical Practice Guidelines for nutrition in children with cystic fibrosis in Australia
2018, Respiratory MedicineCitation Excerpt :Age, sex, pancreatic status, dornase alfa (Pulmozyme) therapy, number of ever-colonised positive sample of Pseudomonas aeruginosa and presence of a G551D mutation were considered as potential confounders in this study. Dornase alfa (Pulmozyme) was included to the analysis as a possible confounder because of its mucolytic therapy use, which might be an indication of a more severe lung disease and could potentially result in poorer nutritional outcomes in children undertaking this therapy [26]. Dornase alfa (Pulmozyme) was listed on the Australian Pharmaceutical Benefits Scheme in 1996 and recommended for children age of six years or older [27].
Association of growth and nutritional parameters with pulmonary function in cystic fibrosis: a literature review
2016, Revista Paulista de Pediatria
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.