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

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.