Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV
Adult
Aminophenols
/ therapeutic use
Biomarkers
/ analysis
Body Mass Index
Child
Chloride Channel Agonists
/ therapeutic use
Cystic Fibrosis
/ genetics
Cystic Fibrosis Transmembrane Conductance Regulator
/ genetics
Drug Monitoring
/ methods
Female
Forced Expiratory Volume
/ drug effects
Humans
Male
Mutation
Quinolones
/ therapeutic use
Respiratory Function Tests
/ methods
Retrospective Studies
Sweat
/ chemistry
WAP Four-Disulfide Core Domain Protein 2
/ analysis
BMI
Biomarker
Cystic fibrosis
FEV(1)
HE4
Ivacaftor
Sweat chloride
Journal
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
ISSN: 1873-5010
Titre abrégé: J Cyst Fibros
Pays: Netherlands
ID NLM: 101128966
Informations de publication
Date de publication:
03 2019
03 2019
Historique:
received:
22
05
2018
revised:
28
08
2018
accepted:
28
08
2018
pubmed:
1
10
2018
medline:
17
7
2020
entrez:
1
10
2018
Statut:
ppublish
Résumé
We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy. In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: 12 and Irish: 19 cases) were enrolled carrying at least one Class III CFTR CF-causing mutation (p.Gly551Asp) and being treated with CFTR potentiator ivacaftor. Plasma HE4 was measured by immunoassay before treatment (baseline) and 1-6 months after commencement of ivacaftor, and were correlated with FEV After 1 month of therapy, HE4 levels were significantly lower than at baseline and remained decreased up to 6 months. A significant inverse correlation between absolute and delta values of HE4 and FEV This study shows that plasma HE4 levels inversely correlate with lung function improvement in CF patients receiving ivacaftor. Overall, this potential biomarker may be of value for routine clinical and laboratory follow-up of CFTR modulating therapy.
Sections du résumé
BACKGROUND
We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy.
METHODS
In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: 12 and Irish: 19 cases) were enrolled carrying at least one Class III CFTR CF-causing mutation (p.Gly551Asp) and being treated with CFTR potentiator ivacaftor. Plasma HE4 was measured by immunoassay before treatment (baseline) and 1-6 months after commencement of ivacaftor, and were correlated with FEV
RESULTS
After 1 month of therapy, HE4 levels were significantly lower than at baseline and remained decreased up to 6 months. A significant inverse correlation between absolute and delta values of HE4 and FEV
CONCLUSIONS
This study shows that plasma HE4 levels inversely correlate with lung function improvement in CF patients receiving ivacaftor. Overall, this potential biomarker may be of value for routine clinical and laboratory follow-up of CFTR modulating therapy.
Identifiants
pubmed: 30268371
pii: S1569-1993(18)30795-1
doi: 10.1016/j.jcf.2018.08.013
pii:
doi:
Substances chimiques
Aminophenols
0
Biomarkers
0
Chloride Channel Agonists
0
Quinolones
0
WAP Four-Disulfide Core Domain Protein 2
0
WFDC2 protein, human
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
ivacaftor
1Y740ILL1Z
Types de publication
Clinical Study
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
271-277Informations de copyright
Copyright © 2018 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.