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Published on: August 3, 2021
Early life growth trajectories in cystic fibrosis are associated with lung function at age six
Kevin J Psoter1, Kimberly M Dickinson2, Kristin A Riekert3
1Division of General Pediatrics, Department of Pediatrics, Johns Hopkins University, Baltimore, MD.
Insights
Children with cystic fibrosis (CF) show distinct growth patterns. Meeting CF Foundation (CFF) nutritional guidelines is linked to better lung function (FEV1pp) by age six.
Area of Science:
- Pediatric pulmonology
- Growth and development in chronic disease
- Biostatistics and epidemiology
Background:
- Higher growth percentiles correlate with improved lung function in cystic fibrosis (CF).
- The CF Foundation (CFF) developed nutritional guidelines to promote optimal growth in children with CF.
Purpose of the Study:
- To characterize early childhood growth trajectories in children with CF.
- To assess the association between growth trajectories and lung function at age six.
- To identify factors influencing these growth trajectories.
Main Methods:
- Retrospective cohort study of 6,809 children with CF (born 2000-2011) from the US CFF Patient Registry.
- Group-based trajectory modeling of annualized growth parameters before age six.
- Linear regression to compare FEV1 percent predicted (FEV1pp) at age six between trajectory groups.
- Multinomial logistic regression to identify factors associated with trajectory membership.
Main Results:
- Six distinct growth trajectories were identified: 'always high', 'gradual decliner', 'rapid decliner', 'rapid riser', 'gradual riser', and 'always low'.
- The 'Always High' trajectory group exhibited the highest FEV1pp at age six.
- The 'Always Low' trajectory group showed approximately 10% lower FEV1pp compared to the 'Always High' group.
- Sex, ethnicity, newborn screening, and pancreatic function were associated with trajectory group membership.
Conclusions:
- Distinct early life growth trajectories exist in children with CF.
- Growth trajectories aligning with CFF nutritional guidelines are associated with better lung function at age six.
- Continued partnership between CF care teams and families is crucial for interventions supporting optimal growth and improved lung function.
Background:
Higher growth percentiles are associated with more favorable lung function in cystic fibrosis (CF), prompting the creation of CF Foundation (CFF) nutritional guidelines.
Objectives:
To describe early childhood growth trajectories within CF, to determine if growth trajectories are associated with differences in lung function at age six, and to identify factors that differ between trajectory groups.
Methods:
Retrospective cohort study of children diagnosed with CF and born 2000-2011 using the US CFF Patient Registry. Annualized growth parameters prior to age six were included in group-based trajectory modeling to identify unique early life growth trajectories. FEV1 percent predicted (FEV1pp) at age six was compared between trajectory groups using linear regression. Factors associated with group membership were identified using multinomial logistic regression.
Results:
6,809 children met inclusion criteria. Six discrete growth trajectories were identified, including three groups that began with growth parameters >50th percentile, termed: "always high", "gradual decliner", "rapid decliner", and three which began with growth parameters <50th percentile, termed: "rapid riser", "gradual riser", "always low". FEV1pp at age six was highest for the Always High trajectory. The Always Low trajectory was nearly 10% lower than the Always High trajectory. Sex, ethnicity, newborn screening and pancreatic function were associated with trajectory class membership.
Conclusions:
Distinct early life growth trajectories were identified within CF. Trajectories that met CFF nutritional guideline recommendations were associated with higher FEV1pp at age six. CF care teams should continue to partner with families to encourage interventions to support optimal growth to improve lung function in CF.
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