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

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene resulting in the absence or dysfunction of CFTR protein, an anion channel in epithelial cells lining the respiratory tract, gastrointestinal system, reproductive system, and sweat glands [1]. In the sweat glands, reduced CFTR activity limits reabsorption of chloride, resulting in abnormally high sweat chloride (SC) concentrations. A CF diagnosis is confirmed by elevated SC concentrations ≥ 60 mmol/L [2].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original Article Source Type: research