Most Short Children with Cystic Fibrosis Do Not Catch Up by Adulthood.

Margaret P Marks,Bonnie W Ramsey,Sonya L Heltshe,Lucas R Hoffman,Michael S Stalvey,Arthur Baines

doi:10.3390/nu13124414

Abstract

Poor linear growth is common in children with cystic fibrosis (CF) and predicts pulmonary status and mortality. Growth impairment develops in infancy, prior to pulmonary decline and despite aggressive nutritional measures. We hypothesized that growth restriction during early childhood in CF is associated with reduced adult height. We used the Cystic Fibrosis Foundation (CFF) patient registry to identify CF adults between 2011 and 2015 (ages 18–19 y, n = 3655) and had height for age (HFA) records between ages 2 and 4 y. We found that only 26% CF adults were ≥median HFA and 25% were <10th percentile. Between 2 and 4 years, those with height < 10th percentile had increased odds of being <10th percentile in adulthood compared to children ≥ 10th percentile (OR = 7.7). Of HFA measured between the 10th and 25th percentiles at ages 2–4, 58% were <25th percentile as adults. Only 13% between the 10th and 25th percentile HFA at age 2–4 years were >50th percentile as adults. Maximum height between ages 2 and 4 highly correlated with adult height. These results demonstrate that low early childhood CF height correlates with height in adulthood. Since linear growth correlates with lung growth, identifying both risk factors and interventions for growth failure (nutritional support, confounders of clinical care, and potential endocrine involvement) could lead to improved overall health.

Highlights

Childhood Height for Age Is Associated with Adult Height
Among the children with height for age (HFA)% < 10 in all three years between 2 and 4 years, 67% were short as adults (
We found that early-life growth impairments of preschool children with Cystic fibrosis (CF) correlated with stunting in early adulthood

Summary

Introduction

Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CFTR encodes a chloride channel expressed in a variety of epithelial tissues, and mutations in this gene impair protein activity and/or expression [1]. CFTR protein dysfunction affects multiple organ systems and unequivocally contributes to growth deficits and progressive lung disease from birth and throughout infancy and childhood [2]. The association between nutrition and pulmonary outcomes in CF is well established, when using weight-for-length (WFL) and body mass index (BMI) as key nutritional indices [3,4]

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