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Relationship between clinical parameters and linear growth in children with cystic fibrosis

Pamela J Byard1

  • 1Department of Pediatrics, Case Western Reserve University, Rainbow Babies and Childrens Hospital, Cleveland, Ohio 44106.

American Journal of Human Biology : the Official Journal of the Human Biology Council
|June 10, 2017
PubMed

Insights

Cystic fibrosis (CF) patients often show growth retardation, with delayed adolescent growth spurts continuing into early adulthood. Pancreatic enzyme sufficiency and lung health show some impact on growth, but explain limited variation.

Area of Science:

  • Pediatric Endocrinology
  • Pulmonology
  • Genetics

Background:

  • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, including linear growth.
  • Growth retardation is a common complication in CF patients, impacting their overall health and development.

Purpose of the Study:

  • To analyze longitudinal height data in a large cohort of cystic fibrosis patients.
  • To identify factors influencing growth patterns, including pancreatic enzyme status and pulmonary function.

Main Methods:

  • Analysis of mixed longitudinal height data from 1,170 cystic fibrosis patients.
  • Comparison of height percentiles and growth increments against NCHS standards.
  • Correlation analysis between pulmonary status (lung X-rays) and height growth.

Main Results:

  • CF patients exhibit growth retardation, with median heights below the 25th percentile until late adolescence.
  • Delayed adolescent growth spurts are observed, with some patients continuing to grow into early adulthood.
  • Pancreatic-enzyme-sufficient patients showed higher height-for-age percentiles after age 9, though not statistically significant.
  • Pulmonary status correlated with height increment peaks in adolescence, with significant negative correlation after age 16.

Conclusions:

  • Growth retardation and delayed adolescent growth spurts are characteristic of cystic fibrosis.
  • Pancreatic enzyme status and pulmonary disease influence linear growth in CF, particularly during adolescence.
  • These factors explain only a small proportion of the variability in growth percentiles among CF patients.

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