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URL Address
<a href="http://doi.org/10.1016/j.jcf.2008.06.003" target="_blank" rel="noreferrer">http://doi.org/10.1016/j.jcf.2008.06.003</a>
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Title
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An international/multicentre report on patients with cystic fibrosis (CF) over the age of 40 years
Publisher
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Journal Of Cystic Fibrosis
Date
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2008
Subject
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Female; Humans; Male; Survival Rate; Cohort Studies; Adult; Canada; Aged; Middle Aged; Minnesota; Longevity; Italy; Adolescent Transitions; retrospective studies; Age Distribution; Life Expectancy; Sex Distribution; Cystic Fibrosis/complications/epidemiology/physiopathology; London
Creator
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Hodson ME; Simmonds NJ; Warwick WJ; Tullis E; Castellani C; Assael B; Dodge JA; Corey M; International Study Of Aging In Cystic Fibrosis
Description
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BACKGROUND: The lifespan of patients with cystic fibrosis (CF) is increasing significantly. The objective of this international pilot study was to study the characteristics of these long-term survivors. METHODS: Four centres with large CF clinics from London (UK), Minneapolis (USA), Toronto (Canada) and Verona (Italy) identified 366 patients who had survived 40 years and longer. RESULTS: At all centres males survived longer than females. There were more pancreatic sufficient patients in Verona (60%) and Toronto (40%) than in London (16%) and Minneapolis (21%). The percentage of DeltaF508 homozygous patients varied between 47% in London and 45% in Minneapolis to only 26% in Toronto and 9% in Verona. Average FEV(1) and BMI values of the surviving population appeared to stabilise after 40 years of age. FEV(1) was on average 12% higher in patients who were pancreatic sufficient (p > 0.0001). There was no difference in survival between the centres. The overall median survival after the age of 40 was 13 years. The estimated annual death rate was approximately 3.4% from the age of 40-60 years. CONCLUSIONS: Significant numbers of patients are now surviving to 40 years or more, and it is hoped that an in-depth study of these patients may identify the factors contributing to longer survival.
2008
Identifier
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<a href="http://doi.org/10.1016/j.jcf.2008.06.003" target="_blank" rel="noreferrer">10.1016/j.jcf.2008.06.003</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Type
The nature or genre of the resource
Journal Article
2008
Adolescent Transitions
Adult
Age Distribution
Aged
Assael B
Backlog
Canada
Castellani C
Cohort Studies
Corey M
Cystic Fibrosis/complications/epidemiology/physiopathology
Dodge JA
Female
Hodson ME
Humans
International Study Of Aging In Cystic Fibrosis
Italy
Journal Article
Journal of Cystic Fibrosis
Life Expectancy
London
Longevity
Male
Middle Aged
Minnesota
Retrospective Studies
Sex Distribution
Simmonds NJ
Survival Rate
Tullis E
Warwick WJ