Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males.
作者信息
Secunda Katharine E, Guimbellot Jennifer S, Jovanovic Borko, Heltshe Sonya L, Sagel Scott D, Rowe Steven M, Jain Manu
机构信息
Northwestern University Feinberg School of MedicineChicago, Illinois.
University of Alabama at BirminghamBirmingham, Alabama.
出版信息
Am J Respir Crit Care Med. 2020 Apr 15;201(8):996-998. doi: 10.1164/rccm.201909-1845LE.
Abstract
摘要
相似文献
1
Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males.
Am J Respir Crit Care Med. 2020 Apr 15;201(8):996-998. doi: 10.1164/rccm.201909-1845LE.
2
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
N Engl J Med. 2018 Oct 25;379(17):1612-1620. doi: 10.1056/NEJMoa1807120. Epub 2018 Oct 18.
3
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
N Engl J Med. 2018 Oct 25;379(17):1599-1611. doi: 10.1056/NEJMoa1807119. Epub 2018 Oct 18.
4
Effectivenesss of ivacaftor in severe cystic fibrosis patients and non-G551D gating mutations.
Pediatr Pulmonol. 2019 Sep;54(9):1398-1403. doi: 10.1002/ppul.24424. Epub 2019 Jun 25.
5
Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study.
Lancet Respir Med. 2016 Feb;4(2):107-15. doi: 10.1016/S2213-2600(15)00545-7. Epub 2016 Jan 21.
6
Cystic Fibrosis-related Diabetes Is Associated with Worse Lung Function Trajectory despite Ivacaftor Use.
Am J Respir Crit Care Med. 2021 Dec 1;204(11):1343-1345. doi: 10.1164/rccm.202104-1060LE.
7
Sweat chloride is not a useful marker of clinical response to Ivacaftor.
Thorax. 2014 Jun;69(6):586-7. doi: 10.1136/thoraxjnl-2013-204532. Epub 2013 Nov 20.
8
Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience.
Respir Med. 2017 Oct;131:225-228. doi: 10.1016/j.rmed.2017.08.026. Epub 2017 Sep 1.
9
Lumacaftor/Ivacaftor Treatment of Patients with Cystic Fibrosis Heterozygous for F508del-CFTR.
Ann Am Thorac Soc. 2017 Feb;14(2):213-219. doi: 10.1513/AnnalsATS.201609-689OC.
10
Ivacaftor in a G551D homozygote with cystic fibrosis.
N Engl J Med. 2013 Sep 26;369(13):1280-2. doi: 10.1056/NEJMc1213681.
引用本文的文献
1
Remote analysis and management of sweat biomarkers using a wearable microfluidic sticker in adult cystic fibrosis patients.
Proc Natl Acad Sci U S A. 2025 Aug 19;122(33):e2506137122. doi: 10.1073/pnas.2506137122. Epub 2025 Aug 12.
2
Sex disparities in cystic fibrosis in the era of highly effective modulator treatment.
BMC Pulm Med. 2025 May 2;25(1):212. doi: 10.1186/s12890-025-03621-0.
3
The gut-lung axis in the CFTR modulator era.
Front Cell Infect Microbiol. 2023 Sep 15;13:1271117. doi: 10.3389/fcimb.2023.1271117. eCollection 2023.
4
Spirometric and anthropometric improvements in response to elexacaftor/tezacaftor/ivacaftor depending on age and lung disease severity.
Front Pharmacol. 2023 Jul 4;14:1171544. doi: 10.3389/fphar.2023.1171544. eCollection 2023.
5
Toward a Systematic Assessment of Sex Differences in Cystic Fibrosis.
J Pers Med. 2023 May 31;13(6):924. doi: 10.3390/jpm13060924.
6
Dual CFTR modulator therapy efficacy in the real world: lessons for the future.
ERJ Open Res. 2022 Nov 14;8(4). doi: 10.1183/23120541.00464-2022. eCollection 2022 Oct.
7
Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study.
J Cyst Fibros. 2023 Jan;22(1):79-88. doi: 10.1016/j.jcf.2022.07.008. Epub 2022 Jul 21.
8
Prediction of Real-World Long-Term Outcomes of People with CF Homozygous for the F508del Mutation Treated with CFTR Modulators.
J Pers Med. 2021 Dec 16;11(12):1376. doi: 10.3390/jpm11121376.
9
Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial.
Am J Respir Crit Care Med. 2022 Mar 1;205(5):529-539. doi: 10.1164/rccm.202108-1986OC.
10
Update in Pediatrics 2020.
Am J Respir Crit Care Med. 2021 Aug 1;204(3):274-284. doi: 10.1164/rccm.202103-0605UP.
本文引用的文献
1
Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.
N Engl J Med. 2017 Nov 23;377(21):2013-2023. doi: 10.1056/NEJMoa1709846. Epub 2017 Nov 3.
2
Correlation of sweat chloride and percent predicted FEV in cystic fibrosis patients treated with ivacaftor.
J Cyst Fibros. 2017 Jan;16(1):41-44. doi: 10.1016/j.jcf.2016.10.002. Epub 2016 Oct 20.
3
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
N Engl J Med. 2015 Jul 16;373(3):220-31. doi: 10.1056/NEJMoa1409547. Epub 2015 May 17.
4
Gender differences in outcomes of patients with cystic fibrosis.
J Womens Health (Larchmt). 2014 Dec;23(12):1012-20. doi: 10.1089/jwh.2014.4985.
5
Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.
Am J Respir Crit Care Med. 2014 Jul 15;190(2):175-84. doi: 10.1164/rccm.201404-0703OC.
6
Effect of estrogen on pseudomonas mucoidy and exacerbations in cystic fibrosis.
N Engl J Med. 2012 May 24;366(21):1978-86. doi: 10.1056/NEJMoa1106126. Epub 2012 May 20.
7
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.
N Engl J Med. 2011 Nov 3;365(18):1663-72. doi: 10.1056/NEJMoa1105185.
8
Estrogen aggravates inflammation in Pseudomonas aeruginosa pneumonia in cystic fibrosis mice.
Respir Res. 2010 Nov 30;11(1):166. doi: 10.1186/1465-9921-11-166.
9
Sex differences in pharmacokinetics and pharmacodynamics.
Clin Pharmacokinet. 2009;48(3):143-57. doi: 10.2165/00003088-200948030-00001.
10
17beta-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia.
J Clin Invest. 2008 Dec;118(12):4025-35. doi: 10.1172/JCI33893. Epub 2008 Nov 20.
Zotero 插件