Effects of Gender and Age at Diagnosis on Disease Progression in Long-term Survivors of Cystic Fibrosis

Long-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed with classic manifestations in childhood, and nonclassic phenotypes typically diagnosed as adults. Little is known concerning disease progression and outcomes in these cohorts. Ex...

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Vydáno v:	American journal of respiratory and critical care medicine Ročník 182; číslo 5; s. 614 - 626
Hlavní autoři:	Nick, Jerry A., Chacon, Cathy S., Brayshaw, Sara J., Jones, Marion C., Barboa, Christine M., Clair, Connie G. St, Young, Robert L., Nichols, David P., Janssen, Jennifer S., Huitt, Gwen A., Iseman, Michael D., Daley, Charles L., Taylor-Cousar, Jennifer L., Accurso, Frank J., Saavedra, Milene T., Sontag, Marci K.
Médium:	Journal Article
Jazyk:	angličtina
Vydáno:	United States American Thoracic Society 01.09.2010
Témata:	Adult Adults Age Distribution Age of Onset Aged B. Cystic Fibrosis Colorado - epidemiology Cystic fibrosis Cystic Fibrosis - complications Cystic Fibrosis - diagnosis Cystic Fibrosis - epidemiology Cystic Fibrosis - genetics Cystic Fibrosis Transmembrane Conductance Regulator - genetics Disease Progression Female Gender Genetic testing Humans Lung diseases Male Medical screening Middle age Middle Aged Mortality Mutation Patient Acceptance of Health Care Registries Retrospective Studies Severity of Illness Index Sex Distribution Survivors - statistics & numerical data Colorado United States > US
ISSN:	1073-449X, 1535-4970, 1535-4970
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Shrnutí:	Long-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed with classic manifestations in childhood, and nonclassic phenotypes typically diagnosed as adults. Little is known concerning disease progression and outcomes in these cohorts. Examine effects of age at diagnosis and gender on disease progression, setting of care, response to treatment, and mortality in long-term survivors of CF. Retrospective analysis of the Colorado CF Database (1992-2008), CF Foundation Registry (1992-2007), and Multiple Cause of Death Index (1992-2005). Patients with CF diagnosed in childhood and who survive to age 40 years have more severe CFTR genotypes and phenotypes compared with adult-diagnosed patients. However, past the age of 40 years the rate of FEV(1) decline and death from respiratory complications were not different between these cohorts. Compared with males, childhood-diagnosed females were less likely to reach age 40 years, experienced faster FEV(1) declines, and no survival advantage. Females comprised the majority of adult-diagnosed patients, and demonstrated equal FEV(1) decline and longer survival than males, despite a later age at diagnosis. Most adult-diagnosed patients were not followed at CF centers, and with increasing age a smaller percentage of CF deaths appeared in the Cystic Fibrosis Foundation Registry. However, newly diagnosed adults demonstrated sustained FEV(1) improvement in response to CF center care. For patients with CF older than 40 years, the adult diagnosis correlates with delayed but equally severe pulmonary disease. A gender-associated disadvantage remains for females diagnosed in childhood, but is not present for adult-diagnosed females.
Bibliografie:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 14 content type line 23 This article has an online supplement, which is accessible from this issue's table of contents at www.atsjournals.org Author Disclosure: J.A.N. served on the Board or Advisory Board for Pharmaxis ($5,001–$10,000), and received lecture fees from the France Foundation and MedLearning ($10,001–$50,000). He received grant support from Novartis, Genentech, Altus ($10,001–$50,000), Transave, Boehringer Ingelheim ($50,001–$100,000), the NIH, the CF Foundation, the Gates Foundation, the Karasik Foundation, and the Department of Defense (more than $100,001). C.S.C. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. S.J.B. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. M.C.J. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. C.M.B. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. C.G.S. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. R.L.Y. received grant support from the Cystic Fibrosis Foundation (more than $100,001). D.P.N. received grant support from Gilead Sciences Inc. ($10,001–$50,000), the Cystic Fibrosis Foundation (more than $100,001), and the Department of Defense ($10,001–$50,000). J.S.J. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. G.A.H. served on the Board or Advisory Board for Hill-Rom ($5,001–$10,000) and received lecture fees from Hill-Rom ($10,001–$50,000). She served as an expert witness for Lawson, Dugan, and Murray, Sutter Ins. Services Corp, and Sandberg, Phoenix & von Gontard, P.C. ($1,001–$5,000) and she received grant support from Bayer ($1,001–$5,000). M.D.I. served on the Board for OMNI-Bio and was an expert witness for Flynn, Gaskin, & Bennett, LLP ($5,001–$10,000). C.L.D. served on the Board or Advisory Board for Otsuka and Sanofi-Aventis ($1,001–$5,000). He received lecture fees from Cellestis ($1,001–$5,000) and served as an expert witness for Patterson Belknap Webb and Tyler LLP and May and Buege, Ltd ($1,001–$5,000). He received grant support from Oxford Immunotec, Designer Diagnostics, Gilead, Pharmaxis ($10,001–$50,000), the NIH, and the CDC (more than $100,001). He receives royalties from Bruckmeier Publishing (up to $1,000). J.L.T.-C. served as a consultant and was on the Advisory Board for Genentech ($1,001–$5,000). She received lecture fees from Novartis ($1,001–$5,000) and received grant support from Genentech ($5,001–$10,000), Vertex, Solvay, and Transave ($10,001–$50,000). She has received grant support from the NIH and the CFF ($50,001–$100,000). F.J.A. served on the Advisory Board for Inspire Pharmaceuticals and PTC Therapeutics (up to $1,000). He received lecture fees from Inspire Pharmaceuticals ($1,001–$5,000) and served as an expert witness for Gilead Sciences (up to $1,000). He received grant support from Digestive Care Inc. ($50,001–$100,000), PTC Therapeutics, Gilead Sciences, Vertex Pharmaceuticals (more than $100,001), Altus Pharmaceuticals ($50,001–$100,000), the NIH, and the Cystic Fibrosis Foundation (more than $100,001). He has other financial interests with KaloBios Pharma, Axcan Pharma, Yasoo Health, Inc. ($50,001–$100,000), INO Therapeutics, Inc., and MPEX Pharmaceuticals ($10,001–$50,000). M.T.S. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. M.K.S. has received grant support from Vertex Pharmaceutical ($5,001–$10,000) and the Cystic Fibrosis Foundation (more than $100,001). She served as a consultant and was on the Board or Advisory Board for the Cystic Fibrosis Foundation (up to $1,000). Supported by the CF Foundation, Rebecca Runyon Bryan Chair for Cystic Fibrosis, Max and Yetta Karasik Foundation, and NIH 1R01HL090991. Originally Published in Press as DOI: 10.1164/rccm.201001-0092OC on May 6, 2010
ISSN:	1073-449X 1535-4970 1535-4970
DOI:	10.1164/rccm.201001-0092OC