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Article

Heterogeneity of CFTR modulator-induced sweat chloride concentrations in people with cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jul 2024

Zemanick E T, Emerman I, McCreary M, Mayer-Hamblett N, Warden M N, Odem-Davis K, VanDevanter D R, Ren C L, Young J, Konstan M W

Abstract excerpt

BACKGROUND: Sweat chloride (SC) concentrations in people with cystic fibrosis (PwCF) reflect relative CF transmembrane conductance regulator (CFTR) protein function, the primary CF defect. Populations with greater SC concentrations tend to have lesser CFTR function and more severe disease courses. CFTR modulator treatment can improve CFTR function within specific CF genotypes and is commonly associated with...

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Identifiers

PMID: 38360461
DOI: 10.1016/j.jcf.2024.02.001
PMCID: PMC11322419

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