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Sommerburg O, Hämmerling S, Schneider SP, et al. CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis. Antioxidants (Basel). 2021;10(3)doi: 10.3390/antiox10030483.
Sommerburg, O., Hämmerling, S., Schneider, S. P., Okun, J., Langhans, C. D., Leutz-Schmidt, P., Wielpütz, M. O., Siems, W., Gräber, S. Y., Mall, M. A., & Stahl, M. (2021). CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis. Antioxidants (Basel, Switzerland), 10(3), . https://doi.org/10.3390/antiox10030483
Sommerburg, Olaf, et al. "CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis." Antioxidants (Basel, Switzerland) vol. 10,3 (2021). doi: https://doi.org/10.3390/antiox10030483
Sommerburg O, Hämmerling S, Schneider SP, Okun J, Langhans CD, Leutz-Schmidt P, Wielpütz MO, Siems W, Gräber SY, Mall MA, Stahl M. CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis. Antioxidants (Basel). 2021 Mar 19;10(3). doi: 10.3390/antiox10030483. PMID: 33808590; PMCID: PMC8003491.
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Antioxidants (Basel). 2021 Mar 19;10(3). doi: 10.3390/antiox10030483.

CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis.

Antioxidants (Basel, Switzerland)

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

Affiliations

  1. Division of Pediatric Pulmonology & Allergy and Cystic Fibrosis Center, Department of Pediatrics III, University of Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany.
  2. Member of the German Center for Lung Research (DZL), Translational Lung Research Center Heidelberg (TLRC), 69120 Heidelberg, Germany.
  3. Center for Pediatric and Adolescent Medicine, Department of Paediatrics I, Division of Neuropediatrics and Metabolic Medicine and Newborn Screening Center, University Hospital Heidelberg, 69120 Heidelberg, Germany.
  4. Department of Diagnostic and Interventional Radiology, University Hospital of Heidelberg, 69120 Heidelberg, Germany.
  5. Clinics for Prevention and Rehabilitation, 38667 Bad Harzburg, Germany.
  6. Department of Pediatric Pulmonology, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charite -Universitätsmedizin Berlin, 13353 Berlin, Germany.
  7. Berlin Institute of Health (BIH), 10178 Berlin, Germany.
  8. German Center for Lung Research (DZL), Associated Partner Site, 13353 Berlin, Germany.

PMID: 33808590 PMCID: PMC8003491 DOI: 10.3390/antiox10030483

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 (

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.

Keywords: CFTR modulators; cystic fibrosis; hypervitaminosis A; retinol; therapy; vitamin E

References

  1. Adv Med Sci. 2018 Sep;63(2):341-346 - PubMed
  2. J Pediatr. 1999 Nov;135(5):601-10 - PubMed
  3. Pediatr Clin North Am. 2009 Oct;56(5):1035-53 - PubMed
  4. Lancet. 2016 Nov 19;388(10059):2519-2531 - PubMed
  5. Gut. 1986 Jun;27(6):714-8 - PubMed
  6. Curr Opin Clin Nutr Metab Care. 2005 Mar;8(2):133-8 - PubMed
  7. Eur Respir J. 2020 Apr 3;55(4): - PubMed
  8. J Pediatr. 2006 Apr;148(4):556-559 - PubMed
  9. Am J Clin Nutr. 1996 May;63(5):717-21 - PubMed
  10. Am J Respir Crit Care Med. 2018 Jun 1;197(11):1433-1442 - PubMed
  11. Curr Opin Pharmacol. 2017 Jun;34:98-104 - PubMed
  12. J Clin Pharmacol. 1994 Feb;34(2):158-66 - PubMed
  13. Am J Clin Nutr. 2004 Jul;80(1):171-7 - PubMed
  14. N Engl J Med. 2015 Jul 16;373(3):220-31 - PubMed
  15. Pediatr Neurol. 2011 Feb;44(2):150-2 - PubMed
  16. Am J Respir Crit Care Med. 2020 May 15;201(10):1193-1208 - PubMed
  17. Am J Clin Nutr. 2004 Jan;79(1):86-92 - PubMed
  18. J Clin Invest. 1977 Jul;60(1):233-41 - PubMed
  19. Food Nutr Bull. 2003 Dec;24(4 Suppl):S78-90 - PubMed
  20. Lancet Respir Med. 2017 Jul;5(7):557-567 - PubMed
  21. Clin Transl Gastroenterol. 2017 Mar 16;8(3):e81 - PubMed
  22. J Cyst Fibros. 2021 Jan;20(1):e1-e6 - PubMed
  23. J Cyst Fibros. 2019 May;18(3):313-320 - PubMed
  24. J Cyst Fibros. 2019 May;18(3):399-406 - PubMed
  25. Lancet Respir Med. 2017 Feb;5(2):107-118 - PubMed
  26. Nutr Clin Pract. 2014 Apr 17;29(4):491-497 - PubMed
  27. Am J Physiol Lung Cell Mol Physiol. 2007 Feb;292(2):L476-86 - PubMed
  28. J Cyst Fibros. 2008 Mar;7(2):137-41 - PubMed
  29. Lipids Health Dis. 2019 Dec 30;18(1):235 - PubMed
  30. Lancet. 2019 Nov 23;394(10212):1940-1948 - PubMed
  31. Ann Nutr Metab. 2000;44(5-6):207-11 - PubMed
  32. Ann Am Thorac Soc. 2021 Feb 18;: - PubMed
  33. Lancet Respir Med. 2020 Jan;8(1):65-124 - PubMed
  34. Eur Respir J. 2014 Oct;44(4):1042-54 - PubMed
  35. Gastroenterology. 1991 Jun;100(6):1701-9 - PubMed
  36. J Pediatr Gastroenterol Nutr. 2007 Sep;45(3):347-53 - PubMed
  37. Am J Clin Nutr. 2006 Feb;83(2):191-201 - PubMed
  38. J Cyst Fibros. 2020 Nov;19(6):931-933 - PubMed
  39. Clin Chem Lab Med. 2020 Sep 25;58(10):1725-1730 - PubMed
  40. J Pediatr Gastroenterol Nutr. 2005 Feb;40(2):199-201 - PubMed
  41. J Cyst Fibros. 2018 Mar;17(2):153-178 - PubMed
  42. Pediatr Pulmonol. 2007 Mar;42(3):256-62 - PubMed
  43. Clin Nutr. 2016 Jun;35(3):654-9 - PubMed
  44. Clin Nutr. 2013 Oct;32(5):805-10 - PubMed
  45. Eur Respir J. 2005 Jul;26(1):153-61 - PubMed
  46. Mediators Inflamm. 2017;2017:5047403 - PubMed
  47. Expert Opin Ther Targets. 2019 Aug;23(8):711-724 - PubMed
  48. Free Radic Biol Med. 2009 Mar 1;46(5):543-54 - PubMed
  49. Biochem Biophys Res Commun. 2003 Jun 6;305(3):737-40 - PubMed
  50. Respir Res. 2010 May 20;11:61 - PubMed
  51. Nat Rev Dis Primers. 2015 May 14;1:15010 - PubMed
  52. Am J Clin Nutr. 1996 May;63(5):722-8 - PubMed
  53. J Pediatr. 2013 Apr;162(4):808-812.e1 - PubMed
  54. Eur Respir J. 2012 Dec;40(6):1324-43 - PubMed
  55. N Engl J Med. 2010 Nov 18;363(21):1991-2003 - PubMed
  56. Am J Respir Crit Care Med. 2018 Mar 1;197(5):e1-e19 - PubMed
  57. Am J Respir Crit Care Med. 2017 Feb 1;195(3):349-359 - PubMed
  58. J Pediatr. 1989 May;114(5):767-73 - PubMed
  59. Eur J Pediatr. 1996 Apr;155(4):281-5 - PubMed
  60. Clin Nutr. 2016 Jun;35(3):557-77 - PubMed
  61. Am J Respir Crit Care Med. 2017 May 1;195(9):1092-1099 - PubMed
  62. N Engl J Med. 2019 Nov 7;381(19):1809-1819 - PubMed

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