# Cystic Fibrosis and CFTR Modulators: The Impact on Bone Density, Muscle Mass and Strength in Children and Young Adolescents

## TL;DR

This paper reviews how CFTR modulators affect bone density, muscle mass, and strength in children with cystic fibrosis.

## Contribution

The paper provides a narrative review on the variable effects of CFTR modulators on musculoskeletal health in young CF patients.

## Key findings

- CFTR modulators may improve bone mineral density in children with cystic fibrosis.
- The effect of CFTR modulators on muscle mass and strength is inconsistent across studies.
- More research is needed to confirm the observed effects of CFTR modulators on musculoskeletal outcomes.

## Abstract

Background/Objectives: Cystic fibrosis (CF) is a multisystemic and genetic disorder. Mutations in the CF transmembrane conductance regulator (CFTR) gene impair the function of the CFTR protein, leading to various complications in multiple organs, mainly the lungs. Methods: In this article, we have tried to investigate the potential impact of CFTR modulators on bone density, muscle mass, and strength in children and young adolescents with CF by using the existing literature and conducting a narrative (non-systematic) review. Results: It has been demonstrated that CFTR modulators may positively influence bone mineral density. On the other hand, the impact of CFTR modulators on muscle mass and strength seems to vary among studies. Conclusions: Besides the current literature, further studies are needed to validate the existing claims.