Changes in vitamins and trace elements after initiation of highly effective CFTR modulator therapy in children and adults with cystic fibrosis – a real-life insight

Dorit Fabricius, Tina Knieling, Noelle Zurmuehl, Leandra Makedon, Joachim Freihorst,Hanna Schmidt,Sebastian Bode

Molecular and Cellular Pediatrics(2024)

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摘要
Highly-effective CFTR-modulator therapy with elexa-/teza-/ivacaftor (ETI) has led to improvements in pulmonary outcomes, sweat chloride, body mass index (BMI) and quality of life in people with cystic fibrosis (CF). Improved uptake of fat-soluble vitamins and micronutrients has been reported for CFTR-modulators but data regarding ETI therapy is lacking. This single-center retrospective study evaluated forced expiratory volume in one second (FEV-1), sweat chloride, BMI, transaminases (AST, ALT), bilirubin, vitamins A, D, E, zinc and selenium in children and adults eligible for ETI. Parameters were assessed before and up to one year after initiation of ETI. 58 patients (median age m = 28 years, SD ± 11.6 years, 51.7
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关键词
Cystic fibrosis,Vitamins,Trace elements,Micronutrients,Highly effective CFTR modulator therapy,Individualized therapy
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