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Incorporating genetic potential when evaluating stature in children with cystic fibrosis

Zhumin Zhang1, Suzanne M Shoff, Huichuan J Lai

  • 1Department of Nutritional Sciences, University of Wisconsin, Madison, Wisconsin, United States.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|February 9, 2010
PubMed

Insights

Adjusting for genetic potential is crucial for accurately assessing short stature in children with cystic fibrosis (CF). Failure to do so underestimates prevalence and biases lung function correlations.

Area of Science:

  • Pediatric endocrinology
  • Genetics
  • Pulmonology

Background:

  • The Cystic Fibrosis Foundation (CFF) guidelines recommend adjusting for genetic potential when evaluating height in children with CF.
  • Limited data currently support this recommendation.
  • Accurate height assessment is vital for monitoring growth and health outcomes in pediatric CF patients.

Purpose of the Study:

  • To compare different methods of classifying short stature in children with cystic fibrosis (CF).
  • To evaluate the impact of adjusting for genetic potential on short stature prevalence and its association with lung function.

Main Methods:

  • Analysis of data from 3306 children with CF and documented parental heights from the 1986-2005 CFF Patient Registry.
  • Comparison of three classification methods: unadjusted height percentile <10th, Himes adjusted height percentile <10th, and unadjusted height below CFF target lower bound.

Main Results:

  • Children with CF had lower height percentiles than their parents.
  • Himes adjustment significantly altered height percentile classification, particularly in children with short or tall parents.
  • Prevalence of short stature varied substantially based on the classification method used.
  • Himes adjustment revealed a stronger positive association between height percentile and lung function (percent predicted FEV1).

Conclusions:

  • Failure to adjust for genetic potential leads to underestimation of short stature prevalence in children with CF.
  • Unadjusted height assessments can bias the observed relationship between height and lung function.
  • Adjusting for genetic potential provides a more accurate evaluation of growth status in pediatric CF patients.
Abstract

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