Rate of improvement of CF life expectancy exceeds that of general population-Observational death registration study

Journal of Cystic Fibrosis

Volumn 13, Issue 4, 2014, Pages 410-415

  Hurley, Matthew N ^a   McKeever, Tricia M ^b   Prayle, Andrew P ^a   Fogarty, Andrew W ^b   Smyth, Alan R ^a  

^a UNIVERSITY OF NOTTINGHAM   (United Kingdom)

^b NOTTINGHAM CITY HOSPITAL   (United Kingdom)

Author keywords

Cystic fibrosis; Healthcare; Mortality; Survival

Indexed keywords

ADULT; AGE; ARTICLE; CAUSE OF DEATH; CYSTIC FIBROSIS; DYING; FEMALE; HUMAN; LIFE EXPECTANCY; MAJOR CLINICAL STUDY; MALE; MORTALITY; OBSERVATIONAL STUDY; REGISTER; SEX DIFFERENCE; SURVIVAL; UNITED KINGDOM; UNITED STATES; ADOLESCENT; AGE DISTRIBUTION; CHILD; CLINICAL TRIAL; EPIDEMIOLOGY; HEALTH SURVEY; INFANT; MIDDLE AGED; MULTICENTER STUDY; PRESCHOOL CHILD; PROCEDURES; RETROSPECTIVE STUDY; SURVIVAL RATE; TRENDS; YOUNG ADULT;

ADOLESCENT; ADULT; AGE DISTRIBUTION; AGE FACTORS; CAUSE OF DEATH; CHILD; CHILD, PRESCHOOL; CYSTIC FIBROSIS; ENGLAND; FEMALE; HUMANS; INFANT; LIFE EXPECTANCY; MALE; MIDDLE AGED; POPULATION SURVEILLANCE; REGISTRIES; RETROSPECTIVE STUDIES; SURVIVAL RATE; UNITED STATES; WALES; YOUNG ADULT;

EID: 84902314504     PISSN: 15691993     EISSN: 18735010     Source Type: Journal    
DOI: 10.1016/j.jcf.2013.12.002     Document Type: Article

References (30)

1. UK CF Registry Annual data report 2010 2012.
2. Cystic Fibrosis Foundation Patient Registry 2010 annual data report 2011.
3. Cystic Fibrosis Foundation What you need to know 06/07/12; Available from. http://www.cff.org/AboutCF/.
4. Dodge J.A., Lewis P.A., Stanton M., Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J 2007, 29(3):522-526.
5. Dodge J.A., Morison S., Lewis P.A., Coles E.C., Geddes D., Russell G., et al. Incidence, population, and survival of cystic fibrosis in the UK, 1968-95. Arch Dis Child 1997, 77(6):493-496.
6. Pritchard C., Wallace M.S. Comparing the USA, UK and 17 Western countries' efficiency and effectiveness in reducing mortality. JRSM Short Reports 2011, 2(7).
7. Slieker M.G., Uiterwaal C.S.P.M., Sinaasappel M., Heijerman H.G.M., van der Laag J., van der Ent C.K. Birth prevalence and survival in cystic fibrosis: a national cohort study in the Netherlands. Chest 2005, 128(4):2309-2315.
8. Mahadeva R., Dodge J.A., Webb K., Westerbeek R.C., Carroll N.R., Dodd M.E., et al. Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional studyCommentary: management in paediatric and adult cystic fibrosis centres improves clinical outcome. BMJ 1998, 316(7147):1771-1775.
9. Bobadilla J.L., Macek M., Fine J.P., Farrell P.M. Cystic fibrosis: a worldwide analysis of CFTR mutations-correlation with incidence data and application to screening. Hum Mutat 2002, 19(6):575-606.
10. Walsh A.C., Rault G., Li Z., Scotet V., Dugueperoux I., Ferec C., et al. Pulmonary outcome differences in U.S. and French cystic fibrosis cohorts diagnosed through newborn screening. J Cyst Fibros 2010, 9(1):44-50.
11. Fogarty A., Hubbard R., Britton J. International comparison of median age at death from cystic fibrosis. Chest 2000, 117(6):1656-1660. [Epub 2000/06/20].
12. Massie J., Curnow L., Gaffney L., Carlin J., Francis I. Declining prevalence of cystic fibrosis since the introduction of newborn screening. Arch Dis Child 2010, 95(7):531-533.
13. Commission on Social Determinants of Health Closing the gap in a generation. Health equity through action on the social determinants of health 2008.
14. O'Connor G.T., Quinton H.B., Kneeland T., Kahn R., Lever T., Maddock J., et al. Median household income and mortality rate in cystic fibrosis. Pediatrics 2003, 111(4):e333-e339.
15. Barr H.L., Britton J., Smyth A.R., Fogarty A.W. Association between socioeconomic status, sex, and age at death from cystic fibrosis in England and Wales (1959 to 2008): cross sectional study. BMJ 2011, 343.
16. Courtney J.M., Bradley J., McCaughan J., O'Connor T.M., Shortt C., Bredin C.P., et al. Predictors of mortality in adults with cystic fibrosis. Pediatr Pulmonol 2007, 42(6):525-532.
17. Kerem E., Reisman J., Corey M., Canny G.J., Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med 1992, 326(18):1187-1191.
18. Chamnan P., Shine B.S.F., Haworth C.S., Bilton D., Adler A.I. Diabetes as a determinant of mortality in cystic fibrosis. Diabetes Care 2010, 33(2):311-316.
19. Verma N., Bush A., Buchdahl R. Is there still a gender gap in cystic fibrosis?. Chest 2005, 128(4):2824-2834.
20. Fogarty A.W., Britton J., Clayton A., Smyth A. Are measures of body habitus associated with mortality in cystic fibrosis?. Chest 2012, 142(3):712-717.
21. Corey M., McLaughlin F.J., Williams M., Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 1988, 41(6):583-591. [Epub 1988/01/01].
22. Li Z., Kosorok M.R., Farrell P.M., Laxova A., West S.E.H., Green C.G., et al. Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis. JAMA 2005, 293(5):581-588.
23. Chotirmall S.H., Smith S.G., Gunaratnam C., Cosgrove S., Dimitrov B.D., O'Neill S.J., et al. Effect of estrogen on Pseudomonas mucoidy and exacerbations in cystic fibrosis. New Engl J Med 2012, 366(21):1978-1986.
24. Boudaya M., Fredj S.H., Haj R.B., Khrouf M., Bouker A., Halouani L., et al. Cystic fibrosis transmembrane conductance regulator mutations and polymorphisms associated with congenital bilateral absence of vas deferens in a restricted group of patients from North Africa. Ann Hum Biol 2012, 39(1):76-79.
25. Nick J.A., Chacon C.S., Brayshaw S.J., Jones M.C., Barboa C.M., Clair C.G.S., et al. Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis. Am J Respir Crit Care Med 2010, 182(5):614-626.
26. Buzzetti R., Salvatore D., Baldo E., Forneris M.P., Lucidi V., Manunza D., et al. An overview of international literature from cystic fibrosis registries: 1. Mortality and survival studies in cystic fibrosis. J Cyst Fibros 2009, 8(4):229-237.
27. Simmonds N.J., D'Souza L., Roughton M., Alton E.W.F.W., Davies J.C., Hodson M.E. Cystic fibrosis and survival to 40years: a study of cystic fibrosis transmembrane conductance regulator function. Eur Respir J 2011, 37(5):1076-1082.
28. Ramsey B.W., Davies J., McElvaney N.G., Tullis E., Bell S.C., Drevinek P., et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011, 365(18):1663-1672. [Epub 2011/11/04].
29. Fibrosis Foundation Cystic Research milestones [13.12.12]. http://www.cff.org/research/researchmilestones.
30. Super M. Milestones in cystic fibrosis. Br Med Bull 1992, 48(4):717-737. [Epub 1992/10/01].