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.
Abstract

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