التفاصيل البيبلوغرافية
| العنوان: |
CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis |
| المؤلفون: |
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, Mirjam Stahl |
| المصدر: |
Antioxidants, Vol 10, Iss 3, p 483 (2021) |
| بيانات النشر: |
MDPI AG, 2021. |
| سنة النشر: |
2021 |
| المجموعة: |
LCC:Therapeutics. Pharmacology |
| مصطلحات موضوعية: |
cystic fibrosis, CFTR modulators, therapy, retinol, vitamin E, hypervitaminosis A, Therapeutics. Pharmacology, RM1-950 |
| الوصف: |
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. |
| نوع الوثيقة: |
article |
| وصف الملف: |
electronic resource |
| اللغة: |
English |
| تدمد: |
2076-3921 |
| Relation: |
https://www.mdpi.com/2076-3921/10/3/483; https://doaj.org/toc/2076-3921 |
| DOI: |
10.3390/antiox10030483 |
| URL الوصول: |
https://doaj.org/article/be35b711a4b64940872bfe40649354f3 |
| رقم الانضمام: |
edsdoj.be35b711a4b64940872bfe40649354f3 |
| قاعدة البيانات: |
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