Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males

Overview

Journal Am J Respir Crit Care Med

Specialty Critical Care

Date 2019 Dec 17

PMID 31841644

Citations 14

Authors

Katharine E Secunda

Jennifer S Guimbellot

Borko Jovanovic

Sonya L Heltshe

Scott D Sagel

Steven M Rowe

Manu Jain

Affiliations

Soon will be listed here.

Citing Articles

The gut-lung axis in the CFTR modulator era.

Lussac-Sorton F, Charpentier E, Imbert S, Lefranc M, Bui S, Fayon M Front Cell Infect Microbiol. 2023; 13:1271117.

PMID: 37780857 PMC: 10540301. DOI: 10.3389/fcimb.2023.1271117.

Spirometric and anthropometric improvements in response to elexacaftor/tezacaftor/ivacaftor depending on age and lung disease severity.

Schutz K, Pallenberg S, Kontsendorn J, DeLuca D, Sukdolak C, Minso R Front Pharmacol. 2023; 14:1171544.

PMID: 37469865 PMC: 10352657. DOI: 10.3389/fphar.2023.1171544.

Toward a Systematic Assessment of Sex Differences in Cystic Fibrosis.

Gartner C, Fallmann J, Stadler P, Kaiser T, Berkemer S J Pers Med. 2023; 13(6).

PMID: 37373913 PMC: 10304421. DOI: 10.3390/jpm13060924.

Dual CFTR modulator therapy efficacy in the real world: lessons for the future.

Dittrich A, Chuang S ERJ Open Res. 2022; 8(4).

PMID: 36382239 PMC: 9661234. DOI: 10.1183/23120541.00464-2022.

Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study.

Mayer-Hamblett N, Zemanick E, Odem-Davis K, VanDevanter D, Warden M, Rowe S J Cyst Fibros. 2022; 22(1):79-88.

PMID: 35871974 PMC: 10103635. DOI: 10.1016/j.jcf.2022.07.008.

References

Wainwright C, Elborn J, Ramsey B, Marigowda G, Huang X, Cipolli M . Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med. 2015; 373(3):220-31. PMC: 4764353. DOI: 10.1056/NEJMoa1409547. View

Taylor-Cousar J, Munck A, McKone E, van der Ent C, Moeller A, Simard C . Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del. N Engl J Med. 2017; 377(21):2013-2023. DOI: 10.1056/NEJMoa1709846. View

Fidler M, Beusmans J, Panorchan P, Van Goor F . Correlation of sweat chloride and percent predicted FEV in cystic fibrosis patients treated with ivacaftor. J Cyst Fibros. 2016; 16(1):41-44. DOI: 10.1016/j.jcf.2016.10.002. View

Wang Y, Cela E, Gagnon S, Sweezey N . Estrogen aggravates inflammation in Pseudomonas aeruginosa pneumonia in cystic fibrosis mice. Respir Res. 2010; 11:166. PMC: 3006363. DOI: 10.1186/1465-9921-11-166. View

Soldin O, Mattison D . Sex differences in pharmacokinetics and pharmacodynamics. Clin Pharmacokinet. 2009; 48(3):143-57. PMC: 3644551. DOI: 10.2165/00003088-200948030-00001. View

Harness-Brumley C, Elliott A, Rosenbluth D, Raghavan D, Jain R . Gender differences in outcomes of patients with cystic fibrosis. J Womens Health (Larchmt). 2014; 23(12):1012-20. PMC: 4442553. DOI: 10.1089/jwh.2014.4985. View

Rowe S, Heltshe S, Gonska T, Donaldson S, Borowitz D, Gelfond D . Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. Am J Respir Crit Care Med. 2014; 190(2):175-84. PMC: 4226057. DOI: 10.1164/rccm.201404-0703OC. View

Coakley R, Sun H, Clunes L, Rasmussen J, Stackhouse J, Okada S . 17beta-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia. J Clin Invest. 2008; 118(12):4025-35. PMC: 2582929. DOI: 10.1172/JCI33893. View

Chotirmall S, Smith S, Gunaratnam C, Cosgrove S, Dimitrov B, ONeill S . Effect of estrogen on pseudomonas mucoidy and exacerbations in cystic fibrosis. N Engl J Med. 2012; 366(21):1978-86. DOI: 10.1056/NEJMoa1106126. View

10.

Ramsey B, Davies J, McElvaney N, Tullis E, Bell S, Drevinek P . A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011; 365(18):1663-72. PMC: 3230303. DOI: 10.1056/NEJMoa1105185. View