Puberty and Growth in Cystic Fibrosis: A Review
Nellie Hani1, Marissa Kilberg2, Sara Bartz3
1Department of Pediatric Endocrinology, University of Michigan, Ann Arbor, Michigan.
Summary
Cystic fibrosis (CF) historically causes delayed puberty and growth. New modulator therapies may alter these outcomes, but their full impact on height is still being studied.
Area of Science:
- Pediatric Endocrinology
- Pulmonology
- Genetics
Background:
- Cystic fibrosis (CF) is a chronic disease historically linked to delayed puberty and impaired growth.
- Potential mechanisms include malnutrition, inflammation, growth hormone axis alterations, corticosteroid use, and gonadotropin signaling changes.
- Previous studies indicate delayed puberty, reduced peak height velocity, and lower final adult height in people with CF (pwCF).
Purpose of the Study:
- To review the impact of highly effective modulator therapies (HEMT) on growth and puberty trajectories in youth with CF.
- To understand the evolving understanding of growth and puberty in the context of modern CF treatment.
Main Methods:
- Literature review of studies examining growth and puberty in pwCF.
- Analysis of early data on the effects of HEMT on growth parameters.
Main Results:
- The full impact of HEMT on height outcomes in pwCF is not yet fully understood.
- Early research is investigating changes in growth and puberty following HEMT initiation.
Conclusions:
- The long-term effects of HEMT on growth and pubertal development in cystic fibrosis require further investigation.
- Understanding these changes is crucial for optimizing long-term health outcomes in pwCF.
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