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Clinical benefit without sweat chloride response after ETI therapy in an adult with cystic fibrosis bearing the L467F;F508del complex CFTR allele: a case report and the role of AI-assisted chest CT in longitudinal monitoring

Source: Frontiers - Health • Published: 28 Aug 2026, 00:00

Clinical benefit without sweat chloride response after ETI therapy in an adult with cystic fibrosis bearing the L467F;F508del complex CFTR allele: a case report and the role of AI-assisted chest CT in longitudinal monitoring

A longitudinal case report examines discordant clinical improvement despite absent CFTR chloride biomarker response after triple-modulator therapy, plus AI-assisted chest CT monitoring.

Key Takeaways
  • She has pancreatic insufficiency, progressive lung disease, and recurrent pulmonary exacerbations
  • After ETI commencement (at 40.5 years; July 2021) SCC remained unchanged at ∼100 mmol/L
  • BMI increased from 22.0 to 26.8 kg/m2
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