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CFTR modulator therapy with lumacaftor/ivacaftor alters plasma concentrations of lipid-soluble vitamins A and E in patients with cystic fibrosis

Rationale: Cystic 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 insuf...

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Main Authors: Sommerburg, Olaf (Author) , Hämmerling, Susanne Hedwig (Author) , Schneider, Philipp (Author) , Okun, Jürgen G. (Author) , Langhans, Claus-Dieter (Author) , Leutz-Schmidt, Patricia (Author) , Wielpütz, Mark Oliver (Author) , Siems, Werner (Author) , Gräber, Simon Y. (Author) , Mall, Marcus A. (Author) , Stahl, Mirjam (Author)
Format: Article (Journal)
Language:English
Published: 19 March 2021
In: Antioxidants
Year: 2021, Volume: 10, Issue: 3, Pages: 1-15
ISSN:2076-3921
DOI:10.3390/antiox10030483
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.3390/antiox10030483
Verlag, lizenzpflichtig, Volltext: https://www.mdpi.com/2076-3921/10/3/483
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Author Notes:Olaf Sommerburg, Susanne Hämmerling, S. Philipp Schneider, Jürgen Okun, Claus-Dieter Langhans, Patricia Leutz-Schmidt, Mark O. Wielpütz, Werner Siems, Simon Y. Gräber, Marcus A. Mall and Mirjam Stahl
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Summary:Rationale: Cystic 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. Objectives: To investigate the course of plasma vitamin A and E in patients with CF under LUM/IVA therapy. Methods: Data 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. Results: Patients 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 (p < 0.001). During the same time, plasma vitamin A increased significantly from 1.2 to 1.6 µmol/L (p < 0.05), however, levels above the upper limit of normal were not detected in any of the patients. In contrast, plasma vitamin E as vitamin E/cholesterol ratio decreased moderately over the same time from 6.2 to 5.5 µmol/L (p < 0.01). Conclusions: CFTR 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.
Item Description:Der Artikel erschien im Special Issue "Commemorative issue of antioxidants dedicated to Peter Eckl"
Gesehen am 07.05.2021
Physical Description:Online Resource
ISSN:2076-3921
DOI:10.3390/antiox10030483

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