@article{1b8f78db41ae4be0866bf9c1e1f70f28,
title = "Comparison of organoid swelling and in vivo biomarkers of cftr function to determine effects of lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for the f508del mutation",
keywords = "Adolescent, Adult, Aminophenols/pharmacology, Aminopyridines/pharmacology, Benzodioxoles/pharmacology, Child, Chlorides/analysis, Colforsin, Cystic Fibrosis Transmembrane Conductance Regulator/genetics, Cystic Fibrosis/drug therapy, Drug Combinations, Edema, Female, Forced Expiratory Volume, Homozygote, Humans, In Vitro Techniques, Male, Membrane Potentials/physiology, Middle Aged, Nasal Mucosa/physiopathology, Organoids/drug effects, Precision Medicine, Prospective Studies, Quinolones/pharmacology, Rectum/drug effects, Sweat/chemistry, Treatment Outcome, Vital Capacity, Young Adult",
author = "Graeber, \{Simon Y.\} and \{van Mourik\}, Peter and Vonk, \{Annelotte M.\} and Evelien Kruisselbrink and Stephanie Hirtz and \{van der Ent\}, \{Cornelis K.\} and Mall, \{Marcus A.\} and Beekman, \{Jeffrey M.\}",
note = "Funding Information: C.N.S. received salary support from NHLBI (T32 grant HL134632 {\textquotedblleft}Training the Next Generation in Respiratory Science{\textquotedblright}) and has been supported by the ATS ASPIRE fellowship during the conduct of the study. B.A.E. reports grants from the Heart Foundation of Australia during the conduct of the study, grants from the National Health and Medical Research Council, and grants from Apnimed. R.L.O. is funded by the NHLBI. A.M. is funded by the NHLBI. ResMed provided a philanthropic donation to University of California San Diego. Funding Information: Supported in part by grants from the German Federal Ministry of Education and Research (82DZL004A1 to M.A.M.), the Einstein Foundation Berlin (EP-2017-393 to M.A.M.), and grants of the Dutch Cystic Fibrosis Foundation (NCFS, HIT-CF grant) and ZonMW. S.Y.G. is a participant of the Berlin Institute of Health Charit{\'e} Clinician Scientist Program funded by the Charit{\'e} {\textendash} Universit{\"a}tsmedizin Berlin and the Berlin Institute of Health.",
year = "2020",
month = dec,
day = "1",
doi = "10.1164/rccm.202004-1200LE",
language = "English",
volume = "202",
pages = "1589--1592",
journal = "American Journal of Respiratory and Critical Care Medicine",
issn = "1073-449X",
publisher = "Oxford University Press",
number = "11",
}