Survival of people with cystic fibrosis in Australia.


Journal

Scientific reports
ISSN: 2045-2322
Titre abrégé: Sci Rep
Pays: England
ID NLM: 101563288

Informations de publication

Date de publication:
17 11 2022
Historique:
received: 19 09 2022
accepted: 14 11 2022
entrez: 18 11 2022
pubmed: 19 11 2022
medline: 22 11 2022
Statut: epublish

Résumé

Survival statistics, estimated using data from national cystic fibrosis (CF) registries, inform the CF community and monitor disease progression. This study aimed to estimate survival among people with CF in Australia and to identify factors associated with survival. This population-based cohort study used prospectively collected data from 23 Australian CF centres participating in the Australian CF Data Registry (ACFDR) from 2005-2020. Period survival analysis was used to calculate median age of survival estimates for each 5-year window from 2005-2009 until 2016-2020. The overall median survival was estimated using the Kaplan-Meier method. Between 2005-2020 the ACFDR followed 4,601 people with CF, noting 516 (11.2%) deaths including 195 following lung transplantation. Out of the total sample, more than half (52.5%) were male and 395 (8.6%) had undergone lung transplantation. Two thirds of people with CF (66.1%) were diagnosed before six weeks of age or by newborn/prenatal screening. The overall median age of survival was estimated as 54.0 years (95% CI: 51.0-57.04). Estimated median survival increased from 48.9 years (95% CI: 44.7-53.5) for people with CF born in 2005-2009, to 56.3 years (95% CI: 51.2-60.4) for those born in 2016-2020. Factors independently associated with reduced survival include receiving a lung transplant, having low FEV

Identifiants

pubmed: 36396972
doi: 10.1038/s41598-022-24374-4
pii: 10.1038/s41598-022-24374-4
pmc: PMC9672093
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

19748

Informations de copyright

© 2022. The Author(s).

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Auteurs

Rasa Ruseckaite (R)

Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC, Australia. rasa.ruseckaite@monash.edu.

Farhad Salimi (F)

Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC, Australia.

Arul Earnest (A)

Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC, Australia.

Scott C Bell (SC)

Thoracic Medicine, Adult Cystic Fibrosis Centre, The Prince Charles Hospital, Brisbane, QLD, Australia.
Children's Health Research Centre, The University of Queensland, Brisbane, QLD, Australia.
Translational Research Institute, Brisbane, QLD, Australia.

Tonia Douglas (T)

Children's Health Research Centre, The University of Queensland, Brisbane, QLD, Australia.
Faculty of Medicine, Clinical Unit, University of Queensland, Brisbane, QLD, Australia.

Katherine Frayman (K)

Department of Respiratory and Sleep Medicine, Royal Children's Hospital, Melbourne, VIC, Australia.
Respiratory Diseases Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia.

Lucy Keatley (L)

Cystic Fibrosis Service, Department of Respiratory and Sleep Medicine, Westmead Hospital, Sydney, News South Wales, Australia.

Susannah King (S)

Nutrition Department, The Alfred, Melbourne, VIC, Australia.
Discipline of Food, Dietetics and Nutrition, LaTrobe University, Melbourne, VIC, Australia.

Tom Kotsimbos (T)

Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC, Australia.
Department of Respiratory Medicine, The Alfred, Melbourne, VIC, Australia.

Peter G Middleton (PG)

Cystic Fibrosis Service, Department of Respiratory and Sleep Medicine, Westmead Hospital, Sydney, News South Wales, Australia.

Sue Morey (S)

Department of Respiratory Medicine, Sir Charles Gairdner Hospital, Perth, WA, Australia.

Siobhain Mulrennan (S)

Department of Respiratory Medicine, Sir Charles Gairdner Hospital, Perth, WA, Australia.
Institute of Respiratory Health, University of Western Australia, Perth, WA, Australia.

Andre Schultz (A)

Perth Children's Hospital, Perth, WA, Australia.
Wal-Yan Centre for Respiratory Health, Telethon Kids Institute, University of Western Australia, Perth, Australia.

Claire Wainwright (C)

Children's Health Research Centre, The University of Queensland, Brisbane, QLD, Australia.
Cystic Fibrosis Service, Department of Respiratory and Sleep Medicine, Queensland Children's Hospital, Brisbane, QLD, Australia.

Nathan Ward (N)

Physiotherapy/Department of Thoracic Medicine, Royal Adelaide Hospital, Adelaide, South Australia, Australia.

Peter Wark (P)

Immune Health Programme, Hunter Medical Research Institute, University of Newcastle, Newcastle, NSW, Australia.

Susannah Ahern (S)

Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC, Australia.

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