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Status: Bibliographieeintrag

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Verfasst von:Sommerburg, Olaf [VerfasserIn]   i
 Hämmerling, Susanne Hedwig [VerfasserIn]   i
 Schneider, Philipp [VerfasserIn]   i
 Okun, Jürgen G. [VerfasserIn]   i
 Langhans, Claus-Dieter [VerfasserIn]   i
 Leutz-Schmidt, Patricia [VerfasserIn]   i
 Wielpütz, Mark Oliver [VerfasserIn]   i
 Siems, Werner [VerfasserIn]   i
 Gräber, Simon Y. [VerfasserIn]   i
 Mall, Marcus A. [VerfasserIn]   i
 Stahl, Mirjam [VerfasserIn]   i
Titel:CFTR modulator therapy with lumacaftor/ivacaftor alters plasma concentrations of lipid-soluble vitamins A and E in patients with cystic fibrosis
Verf.angabe: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
E-Jahr:2021
Jahr:19 March 2021
Umfang:15 S.
Teil:volume:10
 year:2021
 number:3
 elocationid:483
 pages:1-15
 extent:15
Fussnoten:Der Artikel erschien im Special Issue "Commemorative issue of antioxidants dedicated to Peter Eckl" ; Gesehen am 07.05.2021
Titel Quelle:Enthalten in: Antioxidants
Ort Quelle:Basel : MDPI, 2013
Jahr Quelle:2021
Band/Heft Quelle:10(2021), 3, Artikel-ID 483, Seite 1-15
ISSN Quelle:2076-3921
Abstract: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.
DOI:doi:10.3390/antiox10030483
URL:Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.

Volltext ; Verlag: https://doi.org/10.3390/antiox10030483
 Volltext: https://www.mdpi.com/2076-3921/10/3/483
 DOI: https://doi.org/10.3390/antiox10030483
Datenträger:Online-Ressource
Sprache:eng
Sach-SW:CFTR modulators
 cystic fibrosis
 hypervitaminosis A
 retinol
 therapy
 vitamin E
K10plus-PPN:1757486623
Verknüpfungen:→ Zeitschrift

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