Linear Growth in Children With Cystic Fibrosis in the Netherlands Born Between 1997–2004; Results of a Multicenter Cohort Analysis

Abstract

Objectives: Short stature has been associated with reduced life expectancy in people with Cystic Fibrosis (pwCF). We aimed to evaluate linear growth and final height in a Dutch cohort of children with CF, diagnosed in early childhood and now aged ≥ 18 years and identify risk factors for impaired linear growth. Methods: A multicenter longitudinal retrospective cohort study was performed in pwCF born between 1997 and 2004, before implementation of newborn screening (NBS). Anthropometric measurements and CF-related risk factors for poor growth (pulmonary infections, malnutrition, CF-Related Diabetes [CFRD], CF-related liver disease [CFLD]) were obtained annually from ages 0.5 to 10 years and biannually from ages 10 to 18. Measurements were converted to Height-For-Age-For-Target-Height (HFA-TH) Z-scores. Differences in HFA-TH Z-scores between pwCF and healthy standards, and risk factors associated with linear growth were analyzed. Results: A total of 128 pwCF (60 males) were included. Most patients did not receive modulator-therapy during pubertal growth. In boys, mean HFA-TH Z-scores at age 18 years (HFA-TH18) were lower in comparison to healthy standards (−0.66 [0.96], p < 0.001). In girls at age 18, a normal mean HFA-TH z-score was found (−0.18 [0.78]). Development of CFRD and a greater change in BMI Z-scores between 0.5 and 6 years of age (ΔBMI0.5–6) were associated with lower HFA-TH18 Z-scores in boys. In both sexes, pulmonary function and BMI Z-scores were positively associated with linear growth. Conclusion: Boys with CF may have impaired final height, especially those with CFRD or a ΔBMI0.5–6. Glucose metabolism and nutritional status should be monitored closely in pwCF, as these factors may contribute to impaired linear growth.