The nutritional status of children with cystic fibrosis

Alexia J Murphy1, Helen M Buntain, Claire E Wainwright

  • 1Department of Respiratory Medicine, Royal Children's Hospital, Brisbane, Australia. alexia.murphy@uq.edu.au

Insights

Children with cystic fibrosis (CF) generally maintain good nutritional status, but it declines with age, particularly in males. Ongoing nutritional intervention is crucial throughout life for individuals with CF.

Area of Science:

  • Pediatric Nutrition
  • Cystic Fibrosis Research
  • Body Composition Analysis

Background:

  • Nutritional intervention is vital for children with cystic fibrosis (CF).
  • Advances in knowledge over the past decade should ideally improve pediatric CF nutritional status.
  • Evaluating current nutritional status in this population is essential.

Purpose of the Study:

  • To assess the nutritional status of children with CF using cross-sectional and longitudinal methods.
  • To identify trends in nutritional status over a two-year period.
  • To determine if nutritional status has improved compared to previous expectations.

Main Methods:

  • Measured body cell mass adjusted for gender and size (BCM/Htp) in 64 children with CF.
  • Expressed BCM/Htp as a Z-score to represent nutritional status.
  • Conducted longitudinal analysis over two years to track changes.

Main Results:

  • Mean BCM/Htp Z-score was 0.54 (sd 1.21) cross-sectionally; only 4% were sub-optimally nourished initially.
  • Longitudinal analysis showed a significant decrease in mean BCM/Htp Z-score over two years.
  • The decline in nutritional status was significant only in males and became more pronounced with age.

Conclusions:

  • Children with CF are generally well-nourished with current treatment support.
  • Nutritional status tends to decrease with age, especially in males.
  • Continuous nutritional intervention is necessary throughout the lifespan of CF patients to manage age-related changes.

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