Improved survival at low lung function in cystic fibrosis: Cohort study from 1990 to 2007

BMJ

Volumn 342, Issue 7797, 2011, Pages 586-

  George, P M ^a   Banya, W ^a   Pareek, N ^a   Bilton, D ^a,b   Cullinan, P ^b   Hodson, M E ^a,b   Simmonds, N J ^a  

^a ROYAL BROMPTON HOSPITAL   (United Kingdom)

^b NATIONAL HEART AND LUNG INSTITUTE   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIBIOTIC AGENT; DEOXYRIBONUCLEASE; RECOMBINANT HUMAN DEOXYRIBONUCLEASE; UNCLASSIFIED DRUG; RECOMBINANT PROTEIN;

ADULT; ARTICLE; BODY MASS; COHORT ANALYSIS; CYSTIC FIBROSIS; FEMALE; FORCED EXPIRATORY VOLUME; HUMAN; LONG TERM CARE; LUNG INSUFFICIENCY; MAJOR CLINICAL STUDY; MALE; MORTALITY; OXYGEN THERAPY; PRIORITY JOURNAL; PROGNOSIS; RISK ASSESSMENT; RISK REDUCTION; SURVIVAL; SURVIVAL TIME; TREATMENT OUTCOME; KAPLAN MEIER METHOD; PATHOPHYSIOLOGY; PHYSIOLOGY; RISK FACTOR;

ADULT; BODY MASS INDEX; CYSTIC FIBROSIS; DEOXYRIBONUCLEASES; FEMALE; FORCED EXPIRATORY VOLUME; HUMANS; KAPLAN-MEIER ESTIMATE; MALE; RECOMBINANT PROTEINS; RISK FACTORS;

EID: 79952522208     PISSN: None     EISSN: 17561833     Source Type: Journal    
DOI: 10.1136/bmj.d1008     Document Type: Article

References (28)

1. Elborn JS, Shale DJ, Britton JR. Cystic fibrosis: current survival and population estimates to the year 2000. Thorax 1991;46:881-5.
2. Hodson ME, Simmonds NJ, Warwick WJ, Tullis E, Castellani C, Assael B, et al. An international/multicentre report on patients with cystic fibrosis (CF) over the age of 40 years. J Cyst Fibros 2008;7:537-42.
3. Kerem E, Reisman J, Corey M, Canny GJ, Levison H. Prediction of mortality in patients with cystic fibrosis.N Engl J Med 1992;326:1187-91.
4. Orens JB, Estenne M, Arcasoy S, Conte JV, Corris P, Egan JJ, et al. International guidelines for the selection of lung transplant candidates: 2006 update - a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2006;25:745-55.
5. Liou TG, Adler FR, Cahill BC, Fitzsimmons SC, Huang D, Hibbs JR, et al. Survival effect of lung transplantation among patients with cystic fibrosis. JAMA 2001;286:2683-9. (Pubitemid 33135074)
6. Corey M, Farewell V. Determinants of mortality from cystic fibrosis in Canada, 1970-1989. Am J Epidemiol 1996;143:1007-17.
7. Steinkamp G, Wiedemann B. Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project. Thorax 2002;57:596-601. (Pubitemid 34761761)
8. Ramsey BW, Dorkin HL, Eisenberg JD, Gibson RL, Harwood IR, Kravitz RM, et al. Efficacy of aerosolized tobramycin in patients with cystic fibrosis. N Engl J Med 1993;328:1740-6. (Pubitemid 23170123)
9. Fuchs HJ, Borowitz DS, Christiansen DH, Morris EM, Nash ML, Ramsey BW, et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N Engl J Med 1994;331:637-42. (Pubitemid 24276863)
10. Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006;354:229-40. (Pubitemid 43113028)
11. Saiman L, Marshall BC, Mayer-Hamblett N, Burns JL, Quittner AL, Cibene DA, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 2003;290:1749-56. (Pubitemid 37430625)
12. Shah PL, Scott SF, Fuchs HJ, Geddes DM, Hodson ME. Medium term treatment of stable stage cystic fibrosis with recombinant human DNase I. Thorax 1995;50:333-8.
13. Madden BP, Kariyawasam H, Siddiqi AJ, Machin A, Pryor JA, Hodson ME. Noninvasive ventilation in cystic fibrosis patients with acute or chronic respiratory failure. Eur Respir J 2002;19:310-3. (Pubitemid 34162833)
14. Mahadeva R, Webb K, Westerbeek RC, Carroll NR, Dodd ME, Bilton D, et al. Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional study. BMJ 1998;316:1771-5. (Pubitemid 28269150)
15. Milla CE, Warwick WJ. Risk of death in cystic fibrosis patients with severely compromised lung function. Chest 1998;113:1230-4. (Pubitemid 28223809)
16. Doershuk CF, Stern RC. Timing of referral for lung transplantation for cystic fibrosis: overemphasis on FEV1 may adversely affect overall survival. Chest 1999;115:782-7. (Pubitemid 29129961)
17. Gibson GJ. Standardised lung function testing. Eur Respir J 1993;6:155-7. (Pubitemid 23074648)
18. UK Cystic Fibrosis Trust Nutrition Working Group. Nutritional management of cystic fibrosis. Cystic Fibrosis Trust, 2002.
19. Paul K, Rietschel E, Ballmann M, Griese M, Worlitzsch D, Shute J, et al. Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis. Am J Respir Crit Care Med 2004;169:719-25. (Pubitemid 38338924)
20. Jones AP, Wallis C. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev 2010;3:CD001127.
21. Quan JM, Tiddens HA, Sy JP, McKenzie SG, Montgomery MD, Robinson PJ, et al. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr 2001;139:813-20. (Pubitemid 34014460)
22. Mischler EH, Parrell S, Farrell PM, Odell GB. Comparison of effectiveness of pancreatic enzyme preparations in cystic fibrosis. Am J Dis Child 1982;136:1060-3. (Pubitemid 13243773)
23. Emerson J, Rosenfeld M, McNamara S, Ramsey B, Gibson RL. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol 2002;34:91-100. (Pubitemid 34809519)
24. Finkelstein SM, Wielinski CL, Elliott GR, Warwick WJ, Barbosa J, Wu SC, et al. Diabetes mellitus associated with cystic fibrosis. J Pediatr 1988;112:373-7. (Pubitemid 18079818)
25. Milla CE, Billings J,Moran A. Diabetes is associated with dramatically decreased survival in female but not male subjects with cystic fibrosis. Diabetes Care 2005;28:2141-4. (Pubitemid 41242458)
26. Rosenfeld M, Davis R, Fitz Simmons S, Pepe M, Ramsey B. Gender gap in cystic fibrosis mortality. Am J Epidemiol 1997;145:794-803. (Pubitemid 27188516)
27. Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database Syst Rev 2009;2:CD001506.
28. Calverley PM, Anderson JA, Celli B, Ferguson GT, Jenkins C, Jones PW, et al. Salmeterol and fluticasone propionate and survival in chronic obstructive pulmonary disease. N Engl J Med 2007;356:775-89. (Pubitemid 46294610)