MON-221 - Growth Characteristics and Determinants of Final Adult Height in Persons with Cystic Fibrosis

Background: Multiple studies demonstrated decreased final adult height (FH) in persons with CF (pwCF). Still, The etiology of impaired growth in children and adolescents with CF has not been fully elucidated, and only few studies investigated the association between mutation severity and FH.

Objective: To characterize longitudinal growth patterns of pwCF, to assess FH in this population, and to identify determinants of FH.

Methods: A retrospective study comprising 147 pwCF (males=88) who have reached FH. Final height was compared to data from the general healthy population in Israel. Potential factors that could affect FH were assessed, including CFTR mutation type, gender, weight, glucocorticoid treatment, exocrine pancreatic insufficiency (PI), CF-related diabetes, liver diseases, Allergic Bronchopulmonary Aspergillosis (ABPA), number of hospitalizations, bacterial colonization, pulmonary function, and pancreatitis events.

Results: FH-SDS of pwCF was -0.46, significantly (p < 0.001) lower compared to the expected in the normal population. When analyzed by gender, the difference in FH was significant in males (172.4±7.6cm vs. 174.8±6.6cm, p< 0.001) but not in females with CF (161.2±6.5cm vs. 162.9±5.9cm, p=0.078). Multivariate analysis showed that CFTR mutation type with minimal tunnel function severity and male gender were the most important predictors of FH-SDS in our cohort. Notably, the association between mutation severity and FH was only significant among males. When examining longitudinal changes in growth, we observed a pattern compatible with delayed puberty, with a gradual decrease in height-SDS from early childhood to adolescence, and an increase in height-SDS from adolescence to FH, resulting in FH-SDS similar to values measured during early childhood. Compatible with this observation, age of menarche in female patients was significantly older compared to reported age in healthy Israeli females (13.7±1.6 years vs. 13.03±1.28 years, p< 0.001). Height-SDS of males was already lower than females during infancy, and the difference persisted until FH was attained.

Conclusions: Males with CF in Israel have a lower FH in comparison to the general population. Predicting factors for lower FH in our cohort were male sex and CFTR mutations with minimal tunnel function. The difference between height outcomes of males and females may be attributed to a greater vulnerability of male pubertal growth to disease severity and nutritional status or to sex-specific endocrine differences.

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