Association of Oxygen Therapy with the Natural Disease Progression of Cystic Fibrosis: A Multi-State Model of the European Cystic Fibrosis Society Patient Registry.

cystic fibrosis epidemiology lung transplantation mortality oxygen therapy

Journal

Therapeutics and clinical risk management
ISSN: 1176-6336
Titre abrégé: Ther Clin Risk Manag
Pays: New Zealand
ID NLM: 101253281

Informations de publication

Date de publication:
2023
Historique:
received: 11 10 2022
accepted: 12 02 2023
entrez: 20 3 2023
pubmed: 21 3 2023
medline: 21 3 2023
Statut: epublish

Résumé

Association between dependence on oxygen therapy (OT) and natural disease progression in people with cystic fibrosis (pwCF) has not been estimated yet. The aim of this study is to understand the prognosis for pwCF on OT, evaluating how the transition probabilities from being alive without lung transplantation (LTx) to LTx and to death, and from being alive after LTx to death change in pwCF with and without OT. We used 2008-2017 data from the 35-country European CF Society Patient Registry. A multi-state model was fitted to assess the effects of individual risk factors on transition probabilities. We considered 48,343 pwCF aged from 6 to 50 years. OT (HR 5.78, 95% CI: 5.32-6.29) and abnormal FEV Oxygen therapy is associated with poor survival in pwCF with and without LTx; harmonization of CF care throughout European countries and minimization of the onset of pulmonary gas exchange abnormalities using all available means remains of paramount importance.

Sections du résumé

Background UNASSIGNED
Association between dependence on oxygen therapy (OT) and natural disease progression in people with cystic fibrosis (pwCF) has not been estimated yet. The aim of this study is to understand the prognosis for pwCF on OT, evaluating how the transition probabilities from being alive without lung transplantation (LTx) to LTx and to death, and from being alive after LTx to death change in pwCF with and without OT.
Methods UNASSIGNED
We used 2008-2017 data from the 35-country European CF Society Patient Registry. A multi-state model was fitted to assess the effects of individual risk factors on transition probabilities.
Results UNASSIGNED
We considered 48,343 pwCF aged from 6 to 50 years. OT (HR 5.78, 95% CI: 5.32-6.29) and abnormal FEV
Conclusion UNASSIGNED
Oxygen therapy is associated with poor survival in pwCF with and without LTx; harmonization of CF care throughout European countries and minimization of the onset of pulmonary gas exchange abnormalities using all available means remains of paramount importance.

Identifiants

pubmed: 36935771
doi: 10.2147/TCRM.S391476
pii: 391476
pmc: PMC10022450
doi:

Types de publication

Journal Article

Langues

eng

Pagination

255-267

Informations de copyright

© 2023 Gambazza et al.

Déclaration de conflit d'intérêts

The authors report no conflicts of interest in this work.

Références

Eur Respir J. 2021 Oct 1;58(3):
pubmed: 33678607
J Heart Lung Transplant. 2021 Mar;40(3):201-209
pubmed: 33386232
Presse Med. 2017 Jun;46(6 Pt 2):e87-e95
pubmed: 28554720
Am J Respir Crit Care Med. 2019 Apr 15;199(8):987-995
pubmed: 30321487
Eur Respir J. 2022 Jul 13;60(1):
pubmed: 34949706
Pediatr Pulmonol. 1993 Mar;15(3):187-98
pubmed: 8327283
Chest. 1999 Mar;115(3):782-7
pubmed: 10084492
Am J Respir Crit Care Med. 2017 Apr 1;195(7):912-920
pubmed: 27805836
Chest. 2017 Jun;151(6):1320-1328
pubmed: 28115168
Eur Respir J. 2012 Dec;40(6):1324-43
pubmed: 22743675
Clin Nutr. 2016 Jun;35(3):557-77
pubmed: 27068495
N Engl J Med. 1992 Apr 30;326(18):1187-91
pubmed: 1285737
J Cyst Fibros. 2021 Jan;20(1):68-77
pubmed: 32967799
J Cyst Fibros. 2017 Nov;16(6):702-708
pubmed: 28330773
Ann Intern Med. 2017 Apr 18;166(8):537-546
pubmed: 28288488
N Engl J Med. 2007 Nov 22;357(21):2143-52
pubmed: 18032764
Thorax. 2017 Jun;72(Suppl 1):ii1-ii90
pubmed: 28507176
Orphanet J Rare Dis. 2014 Jun 07;9:81
pubmed: 24908055
Vital Health Stat 11. 2002 May;(246):1-190
pubmed: 12043359
Cochrane Database Syst Rev. 2009 Jan 21;(1):CD003884
pubmed: 19160226
Eur Respir J. 2015 Jul;46(1):133-41
pubmed: 25792639
Curr Opin Pulm Med. 2021 Nov 1;27(6):554-566
pubmed: 34420018
Comput Methods Programs Biomed. 2010 Sep;99(3):261-74
pubmed: 20227129
J Cyst Fibros. 2018 Mar;17(2):218-227
pubmed: 29311001
Eur Respir J. 2022 Mar 3;59(3):
pubmed: 34289977
J Cyst Fibros. 2019 May;18(3):321-333
pubmed: 30926322
Chest. 1998 May;113(5):1230-4
pubmed: 9596299
Clin Chest Med. 2014 Sep;35(3):495-504
pubmed: 25156765
Stat Methods Med Res. 2009 Apr;18(2):195-222
pubmed: 18562394
N Engl J Med. 1992 Dec 17;327(25):1785-8
pubmed: 1435933
Eur Respir J. 2021 Jul 8;58(1):
pubmed: 33361098
BMJ. 2019 Jul 29;366:l4486
pubmed: 31358491
J Cyst Fibros. 2014 May;13(3):319-27
pubmed: 24332996
Am J Respir Crit Care Med. 2018 Mar 1;197(5):e1-e19
pubmed: 29493315
Lung. 2016 Oct;194(5):799-806
pubmed: 27510183

Auteurs

Simone Gambazza (S)

Healthcare Professions Department, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Department of Clinical Sciences and Community Health, Laboratory of Medical Statistics, Biometry and Epidemiology "G. A. Maccacaro", Università degli Studi di Milano, Milan, Italy.

Annalisa Orenti (A)

Department of Clinical Sciences and Community Health, Laboratory of Medical Statistics, Biometry and Epidemiology "G. A. Maccacaro", Università degli Studi di Milano, Milan, Italy.

Giovanna Pizzamiglio (G)

Cystic Fibrosis Center - Adult Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Anna Zolin (A)

Department of Clinical Sciences and Community Health, Laboratory of Medical Statistics, Biometry and Epidemiology "G. A. Maccacaro", Università degli Studi di Milano, Milan, Italy.

Carla Colombo (C)

Cystic Fibrosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milano, Italy.

Dario Laquintana (D)

Healthcare Professions Department, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Federico Ambrogi (F)

Department of Clinical Sciences and Community Health, Laboratory of Medical Statistics, Biometry and Epidemiology "G. A. Maccacaro", Università degli Studi di Milano, Milan, Italy.
Scientific Directorate, IRCCS Policlinico San Donato, San Donato Milanese, MI, Italy.

Classifications MeSH