ArticleAntioxidants (Basel, Switzerland)2021
CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis.
Article in Antioxidants (Basel, Switzerland), 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 35 citations in OpenAlex.
- Review
- Evaluation of the effect of CFTR modulator therapy on lipid profiles in children.Pediatric research · 2026Article
- Role of elexacaftor-tezacaftor-ivacaftor therapy on fecal elastase-1 and fat-soluble vitamins in children with cystic fibrosis.European journal of pediatrics · 2026Article
- Redox Imbalance in Cystic Fibrosis: The Multifaceted Role of Oxidative Stress.Pharmaceuticals (Basel, Switzerland) · 2025Review
- Effect of cystic fibrosis modulator therapies on serum levels of fat-soluble vitamins.JPGN reports · 2025Article
- Mapping the oxidative landscape in cystic fibrosis: methodological frontiers and application.Frontiers in pharmacology · 2025Review
- Impaired intestinal free fatty acid transport followed by chylomicron malformation, not pancreatic insufficiency, cause metabolic defects in cystic fibrosis.Journal of lipid research · 2024Article
- Vitamin D status and variable responses to supplements depend in part on genetic factors in adults with cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024Article
- 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.Molecular and cellular pediatrics · 2024Article
- Article
- The impact of elexacaftor/tezacaftor/ivacaftor on fat-soluble vitamin levels in people with cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023Article
- Evolving Nutritional Needs in Cystic Fibrosis.Life (Basel, Switzerland) · 2023Review
- Elexacaftor - Tezacaftor - Ivacaftor treatment improves systemic infection parameters andHeliyon · 2023Article
- Nutritional Status and Circulating Levels of Fat-Soluble Vitamins in Cystic Fibrosis Patients: A Cohort Study and Evaluation of the Effect of CFTR Modulators.Children (Basel, Switzerland) · 2023Article
- Nutritional Care in Children with Cystic Fibrosis.Nutrients · 2023Review
- Nutritional impact of CFTR modulators in children with cystic fibrosis.Frontiers in pediatrics · 2023Article
- Cystic Fibrosis Patients with F508del/Minimal Function Genotype: Laboratory and Nutritional Evaluations after One Year of Elexacaftor/Tezacaftor/Ivacaftor Treatment.Journal of clinical medicine · 2022Article
- Update on the management of vitamins and minerals in cystic fibrosis.Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition · 2022Review
- Peter Eckl: Research on the Pro-/Antioxidant Balance.Antioxidants (Basel, Switzerland) · 2022Article
- Might Routine Vitamin A Monitoring in Cystic Fibrosis Patients Reduce Virus-Mediated Lung Pathology?Frontiers in immunology · 2021Article
Corrections and comments
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
Abstract
rationaleCystic fibrosis (CF), caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, leads to impaired pancreatic function and therefore reduced intestinal absorption of lipids and fat-soluble vitamins especially in patients with CF developing pancreatic insufficiency (PI). Previous studies showed that CFTR modulator therapy with lumacaftor-ivacaftor (LUM/IVA) in Phe508del-homozygous patients with CF results in improvement of pulmonary disease and thriving. However, the effects of LUM/IVA on plasma concentration of the lipid soluble vitamins A and E remain unknown.
objectivesTo investigate the course of plasma vitamin A and E in patients with CF under LUM/IVA therapy.
methodsData from annual follow-up examinations of patients with CF were obtained to assess clinical outcomes including pulmonary function status, body mass index (BMI), and clinical chemistry as well as fat-soluble vitamins in Phe508del-homozygous CF patients before initiation and during LUM/IVA therapy.
resultsPatients with CF receiving LUM/IVA improved substantially, including improvement in pulmonary inflammation, associated with a decrease in blood immunoglobulin G (IgG) from 9.4 to 8.2 g/L after two years (
conclusionsCFTR modulator therapy with LUM/IVA alters concentrations of vitamins A and vitamin E in plasma. The increase of vitamin A must be monitored critically to avoid hypervitaminosis A in patients with CF.
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