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Article

CFTR pharmacological modulators: A great advance in cystic fibrosis management.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie - 1 Jan 2023

Foucaud P, Mercier J C

Abstract excerpt

Cystic fibrosis is a severe monogenic disease that affects around 7400 patients in France. More than 2100 mutations in the cystic fibrosis conductance transmembrane regulator (CFTR), the gene encoding for an epithelial ion channel that normally transports chloride and bicarbonate, lead to mucus dehydration and impaired bronchial clearance. Systematic neonatal screening in France since 2002 has enabled early...

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Identifiers

PMID: 36509624
DOI: 10.1016/j.arcped.2022.11.019

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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