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‐TH 18 ) 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 (ΔBMI 0.5–6 ) were associated with lower HFA‐TH 18 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 ΔBMI 0.5–6 . Glucose metabolism and nutritional status should be monitored closely in pwCF, as these factors may contribute to impaired linear growth.
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