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Body composition in children with cystic fibrosis treated with CFTR modulators versus modulator naïve individuals

Carolyn Dress1, Elizabeth Hente1, Md M Hossain1,2,3

  • 1Division of Pulmonary Medicine , Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Pediatric Pulmonology
|December 20, 2023
PubMed
Abstract

No abstract available in PubMed .

Keywords:
BMIbody compositionchildren with CFmodulator therapy

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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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