S. Rowe, S. Miller, and E. Sorscher, Cystic Fibrosis, New England Journal of Medicine, vol.352, issue.19, pp.1992-2001, 2005.
DOI : 10.1056/NEJMra043184

J. Yankaskas, B. Marshall, B. Sufian, R. Simon, and D. Rodman, Cystic Fibrosis Adult Care, Chest, vol.125, issue.1, pp.1-39, 2004.
DOI : 10.1378/chest.125.1_suppl.1S

P. Davis and P. Di-sant-'agnese, Assisted Ventilation for Patients With Cystic Fibrosis, JAMA: The Journal of the American Medical Association, vol.239, issue.18, pp.1851-1855, 1978.
DOI : 10.1001/jama.1978.03280450023017

N. Sood, L. Paradowski, and J. Yankaskas, Outcomes of Intensive Care Unit Care in Adults with Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, vol.163, issue.2, pp.335-343, 2001.
DOI : 10.1164/ajrccm.163.2.2003076

H. Vedam, C. Moriarty, P. Torzillo, D. Mcwilliam, and P. Bye, Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit, Journal of Cystic Fibrosis, vol.3, issue.1, pp.8-14, 2004.
DOI : 10.1016/j.jcf.2003.12.003

L. Brochard, J. Mancebo, M. Wysocki, F. Lofaso, G. Conti et al., Noninvasive Ventilation for Acute Exacerbations of Chronic Obstructive Pulmonary Disease, New England Journal of Medicine, vol.333, issue.13, pp.817-839, 1995.
DOI : 10.1056/NEJM199509283331301

E. Girou, C. Brun-buisson, S. Taille, F. Lemaire, and L. Brochard, Secular Trends in Nosocomial Infections and Mortality Associated With Noninvasive Ventilation in Patients With Exacerbation of COPD and Pulmonary Edema, JAMA, vol.290, issue.22, pp.2985-91, 2003.
DOI : 10.1001/jama.290.22.2985

J. Zielenski, Genotype and Phenotype in Cystic Fibrosis, Respiration, vol.67, issue.2, pp.117-150, 2000.
DOI : 10.1159/000029497

L. Gall, J. Lemeshow, S. Saulnier, and F. , A New Simplified Acute Physiology Score (SAPS II) Based on a European/North American Multicenter Study, JAMA: The Journal of the American Medical Association, vol.270, issue.24, pp.2957-63, 1993.
DOI : 10.1001/jama.1993.03510240069035

M. Ellaffi, C. Vinsonneau, J. Coste, D. Hubert, P. Burgel et al., One-year Outcome after Severe Pulmonary Exacerbation in Adults with Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, vol.171, issue.2, pp.158-64, 2005.
DOI : 10.1164/rccm.200405-667OC

D. Annane, P. Aegerter, M. Jars-guincestre, and B. Guidet, Current Epidemiology of Septic Shock, American Journal of Respiratory and Critical Care Medicine, vol.168, issue.2, pp.165-72, 2003.
DOI : 10.1164/rccm.2201087

T. Martin, J. Hovis, J. Costantino, M. Bierman, M. Donahoe et al., A Randomized, Prospective Evaluation of Noninvasive Ventilation for Acute Respiratory Failure, American Journal of Respiratory and Critical Care Medicine, vol.161, issue.3, pp.807-820, 2000.
DOI : 10.1164/ajrccm.161.3.9808143

A. Berlinski, L. Fan, C. Kozinetz, and C. Oermann, Invasive mechanical ventilation for acute respiratory failure in children with cystic fibrosis: Outcome analysis and case-control study, Pediatric Pulmonology, vol.122, issue.4, pp.297-303, 2002.
DOI : 10.1002/ppul.10159

R. Bartz, R. Love, G. Leverson, L. Will, D. Welter et al., Pre-transplant mechanical ventilation and outcome in patients with cystic fibrosis, The Journal of Heart and Lung Transplantation, vol.22, issue.4, pp.433-441, 2003.
DOI : 10.1016/S1053-2498(02)00667-8

E. Kerem, J. Reisman, M. Corey, G. Canny, and H. Levison, Prediction of Mortality in Patients with Cystic Fibrosis, New England Journal of Medicine, vol.326, issue.18, pp.1187-91, 1992.
DOI : 10.1056/NEJM199204303261804

T. Liou, F. Adler, S. Fitzsimmons, B. Cahill, J. Hibbs et al., Predictive 5-Year Survivorship Model of Cystic Fibrosis, American Journal of Epidemiology, vol.153, issue.4, pp.345-52, 2001.
DOI : 10.1093/aje/153.4.345

P. Aurora, A. Wade, P. Whitmore, and B. Whitehead, A model for predicting life expectancy of children with cystic fibrosis, European Respiratory Journal, vol.16, issue.6, pp.1056-60, 2000.
DOI : 10.1034/j.1399-3003.2000.16f06.x

K. Hayllar, S. Williams, A. Wise, S. Pouria, M. Lombard et al., A prognostic model for the prediction of survival in cystic fibrosis, Thorax, vol.52, issue.4, pp.313-320, 1997.
DOI : 10.1136/thx.52.4.313

T. Liou, F. Adler, and D. Huang, Use of Lung Transplantation Survival Models to Refine Patient Selection in Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, vol.171, issue.9, pp.1053-1062, 2005.
DOI : 10.1164/rccm.200407-900OC

J. Yankaskas, G. Mallory, and . Jr, Lung Transplantation in Cystic Fibrosis, Chest, vol.113, issue.1, pp.217-243, 1998.
DOI : 10.1378/chest.113.1.217

C. Milla and W. Warwick, Risk of Death in Cystic Fibrosis Patients With Severely Compromised Lung Function, Chest, vol.113, issue.5, pp.1230-1234, 1998.
DOI : 10.1378/chest.113.5.1230

D. Rosenbluth, K. Wilson, T. Ferkol, and D. Schuster, Lung Function Decline in Cystic Fibrosis Patients and Timing for Lung Transplantation Referral, Chest, vol.126, issue.2, pp.412-421, 2004.
DOI : 10.1378/chest.126.2.412

A. Oikonomou, J. Manavis, P. Karagianni, J. Tsanakas, A. Wells et al., Loss of FEV1 in cystic fibrosis: correlation with HRCT features, European Radiology, vol.12, issue.9
DOI : 10.1007/s00330-002-1340-3