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Previous Volume 326:1187-1191 April 30, 1992 Number 18 Next

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Abstract

BACKGROUND. The majority of patients with cystic fibrosis die in early adulthood of lung disease. Lung transplantation is a treatment option for patients with advanced pulmonary disease, although the waiting period for organs may be as long as two years. Our purpose was to determine whether the risk of death due to respiratory failure could be predicted one or two years in advance on the basis of pulmonary function, blood gas levels, and nutritional status. METHODS. The study cohort consisted of 673 patients followed between 1977 and 1989. In each patient, pulmonary function, blood gas levels, nutritional status, and vital status were assessed between 1977 and 1987. Cox proportional-hazards regression analysis was used to compute the relative risk of death within one or two years after particular measurements. The effects of age and sex on mortality were also included in the analysis. RESULTS. One hundred ninety patients (28 percent) died during the study period. Overall, patients with a forced expiratory volume in one second (FEV1) less than 30 percent of the predicted value, a partial pressure of arterial oxygen below 55 mm Hg, or a partial pressure of arterial carbon dioxide above 50 mm Hg had two-year mortality rates above 50 percent. Among the laboratory measurements, the FEV1 was the most significant predictor of mortality, but age and sex were also significant in predicting risk. After adjustment for age and sex, the relative risk of death within two years was 2.0 (95 percent confidence interval, 1.9 to 2.2) for each decrement in the FEV1 of 10 percent below the predicted value. Among patients with the same FEV1, the relative risk of death was 2.0 (95 percent confidence interval, 1.5 to 2.6) in patients 10 years younger than other patients, and 2.2 (1.6 to 3.1) in female patients as compared with male patients. CONCLUSIONS. Patients with cystic fibrosis should be considered candidates for lung transplantation when the FEV1 falls below 30 percent of the predicted value. Female patients and younger patients may need to be considered for transplantation at an earlier stage.

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Department of Pediatrics, Hospital for Sick Children, Toronto, Canada.

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• Liou, T. G., Adler, F. R., Cahill, B. C., FitzSimmons, S. C., Huang, D., Hibbs, J. R., Marshall, B. C. (2001). Survival Effect of Lung Transplantation Among Patients With Cystic Fibrosis. JAMA 286: 2683-2689 [Abstract] [Full Text]  
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• COREY, M (2001). Modelling survival in cystic fibrosis. Thorax 56: 743-743 [Full Text]  
• Sharma, R, Florea, V G, Bolger, A P, Doehner, W, Florea, N D, Coats, A J S, Hodson, M E, Anker, S D, Henein, M Y (2001). Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax 56: 746-750 [Abstract] [Full Text]  
• Aurora, P., Prasad, S.A., Balfour-Lynn, I.M., Slade, G., Whitehead, B., Dinwiddie, R. (2001). Exercise tolerance in children with cystic fibrosis undergoing lung transplantation assessment. Eur Respir J 18: 293-297 [Abstract] [Full Text]  
• Navarro, J., Rainisio, M., Harms, H.K., Hodson, M.E., Koch, C., Mastella, G., Strandvik, B., McKenzie, S.G. (2001). Factors associated with poor pulmonary function: cross-sectional analysis of data from the ERCF. Eur Respir J 18: 298-305 [Abstract] [Full Text]  
• SCHECHTER, M. S., SHELTON, B. J., MARGOLIS, P. A., FITZSIMMONS, S. C. (2001). The Association of Socioeconomic Status with Outcomes in Cystic Fibrosis Patients in the United States. Am. J. Respir. Crit. Care Med. 163: 1331-1337 [Abstract] [Full Text]  
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• Liou, T. G., Adler, F. R., FitzSimmons, S. C., Cahill, B. C., Hibbs, J. R., Marshall, B. C. (2001). Predictive 5-Year Survivorship Model of Cystic Fibrosis. Am J Epidemiol 153: 345-352 [Abstract] [Full Text]  
• MILROSS, M. A., PIPER, A. J., NORMAN, M., BECKER, H. F., WILLSON, G. N., GRUNSTEIN, R. R., SULLIVAN, C. E., BYE, P. T. P. (2001). Low-flow Oxygen and Bilevel Ventilatory Support . Effects on Ventilation during Sleep in Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 163: 129-134 [Abstract] [Full Text]  
• Farrell, P. M., Kosorok, M. R., Rock, M. J., Laxova, A., Zeng, L., Lai, H.-C., Hoffman, G., Laessig, R. H., Splaingard, M. L., the Wisconsin Cystic Fibrosis Neonatal Screening S, (2001). Early Diagnosis of Cystic Fibrosis Through Neonatal Screening Prevents Severe Malnutrition and Improves Long-Term Growth. Pediatrics 107: 1-13 [Abstract] [Full Text]  
• CIABATTONI, G., DAVI, G., COLLURA, M., IAPICHINO, L., PARDO, F., GANCI, A., ROMAGNOLI, R., MACLOUF, J., PATRONO, C. (2000). In Vivo Lipid Peroxidation and Platelet Activation in Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 162: 1195-1201 [Abstract] [Full Text]  
• VIZZA, C. D., YUSEN, R. D., LYNCH, J. P., FEDELE, F., ALEXANDER PATTERSON, G., TRULOCK, E. P. (2000). Outcome of Patients with Cystic Fibrosis Awaiting Lung Transplantation. Am. J. Respir. Crit. Care Med. 162: 819-825 [Abstract] [Full Text]  
• Stettler, N., Kawchak, D. A, Boyle, L. L, Propert, K. J, Scanlin, T. F, Stallings, V. A, Zemel, B. S (2000). Prospective evaluation of growth, nutritional status, and body composition in children with cystic fibrosis. Am. J. Clin. Nutr. 72: 407-413 [Abstract] [Full Text]  
• Pier, G. B. (2000). Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections. Proc. Natl. Acad. Sci. USA 97: 8822-8828 [Abstract] [Full Text]  
• Mitchell, I., Nakielna, E., Tullis, E., Adair, C. (2000). Cystic Fibrosis : End-Stage Care in Canada. Chest 118: 80-84 [Abstract] [Full Text]  
• Gilljam, M., Antoniou, M., Shin, J., Dupuis, A., Corey, M., Tullis, D. E. (2000). Pregnancy in Cystic Fibrosis : Fetal and Maternal Outcome. Chest 118: 85-91 [Abstract] [Full Text]  
• Lanuza, D. M., Lefaiver, C., Mc Cabe, M., Farcas, G. A., Garrity, E. Jr. (2000). Prospective Study of Functional Status and Quality of Life Before and After Lung Transplantation. Chest 118: 115-122 [Abstract] [Full Text]  
• COHEN, A. H., SWEET, S. C., MENDELOFF, E., MALLORY, G. B. Jr., HUDDLESTON, C. B., KRAUS, M., KELLY, M., HAYASHI, R., DEBAUN, M. R. (2000). High Incidence of Posttransplant Lymphoproliferative Disease in Pediatric Patients with Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 161: 1252-1255 [Abstract] [Full Text]  
• Lai, H.-C., FitzSimmons, S. C., Allen, D. B., Kosorok, M. R., Rosenstein, B. J., Campbell, P. W., Farrell, P. M. (2000). Risk of Persistent Growth Impairment after Alternate-Day Prednisone Treatment in Children with Cystic Fibrosis. NEJM 342: 851-859 [Abstract] [Full Text]  
• Wiebe, K., Wahlers, Th., Harringer, W., Hardt, H.v.d., Fabel, H., Haverich, A. (1999). Lung transplantation for cystic fibrosis - a single center experience over 8 years. Eur. J. Cardiothorac. Surg. 14: 191-196 [Abstract] [Full Text]  
• Rubin, B. K. (1999). Emerging Therapies for Cystic Fibrosis Lung Disease. Chest 115: 1120-1126 [Full Text]  
• Lai, H.-C., Corey, M., FitzSimmons, S., Kosorok, M. R, Farrell, P. M (1999). Comparison of growth status of patients with cystic fibrosis between the United States and Canada. Am. J. Clin. Nutr. 69: 531-538 [Abstract] [Full Text]  
• Doershuk, C. F., Stern, R. C. (1999). Timing of Referral for Lung Transplantation for Cystic Fibrosis: Overemphasis on FEV1 May Adversely Affect Overall Survival. Chest 115: 782-787 [Abstract] [Full Text]  
• Ramsey, B. W., Pepe, M. S., Quan, J. M., Otto, K. L., Montgomery, A. B., Williams-Warren, J., Vasiljev-K, M., Borowitz, D., Bowman, C. M., Marshall, B. C., Marshall, S., Smith, A. L., The Cystic Fibrosis Inhaled Tobramycin Study Group, (1999). Intermittent Administration of Inhaled Tobramycin in Patients with Cystic Fibrosis. NEJM 340: 23-30 [Abstract] [Full Text]  
• Egan, T. M., Detterbeck, F. C., Mill, M. R., Gott, K. K., Rea, J. B., McSweeney, J., Aris, R. M., Paradowski, L. J. (1998). Lung transplantation for cystic fibrosis: effective and durable therapy in a high-risk group. Ann. Thorac. Surg. 66: 337-346 [Abstract] [Full Text]  
• (1998). International Guidelines for the Selection of Lung Transplant Candidates. Am. J. Respir. Crit. Care Med. 158: 335-339 [Full Text]  
• Mahadeva, R., Webb, K., Westerbeek, R. C, Carroll, N. R, Dodd, M. E, Bilton, D., Lomas, D. A, Dodge, J A, Egger, M., Low, N., Smith, G. D., Lindblom, B., Herrmann, B. (1998). Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional study • Commentary: Management in paediatric and adult cystic fibrosis centres improves clinical outcome • Screening for chlamydial infections and the risk of ectopic pregnancy in a county in Sweden: ecological analysis. BMJ 316: 1771-1780 [Abstract] [Full Text]  
• DONOVAN, D. S. Jr., PAPADOPOULOS, A., STARON, R. B., ADDESSO, V., SCHULMAN, L., MCGREGOR, C., COSMAN, F., LINDSAY, R. L., SHANE, E. (1998). Bone Mass and Vitamin D Deficiency in Adults with Advanced Cystic Fibrosis Lung Disease. Am. J. Respir. Crit. Care Med. 157: 1892-1899 [Abstract] [Full Text]  
• Santamaria, F., Grillo, G., Guidi, G., Rotondo, A., Raia, V., de Ritis, G., Sarnelli, P., Caterino, M., Greco, L. (1998). Cystic Fibrosis: When Should High-resolution Computed Tomography of the Chest Be Obtained?. Pediatrics 101: 908-913 [Abstract] [Full Text]  
• Mendeloff, E. N., Huddleston, C. B., Mallory, G. B., Trulock, E. P., Cohen, A. H., Sweet, S. C., Lynch, J., Sundaresan, S., Cooper, J. D., Patterson, G. A. (1998). Pediatric And Adult Lung Transplantation For Cystic Fibrosis. J. Thorac. Cardiovasc. Surg. 115: 404-414 [Abstract] [Full Text]  
• Pier, G. B., Grout, M., Zaidi, T. S. (1997). Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung. Proc. Natl. Acad. Sci. USA 94: 12088-12093 [Abstract] [Full Text]  
• Gan, K.-H., Veeze, H. J., van den Ouweland, A., Halley, D., Scheffer, H., van der Hout, A., Overbeek, S. E., de Jongste, J. C., Bakker, W., Heijerman, H. (1995). A Cystic Fibrosis Mutation Associated with Mild Lung Disease. NEJM 333: 95-99 [Abstract] [Full Text]  
• Konstan, M. W., Byard, P. J., Hoppel, C. L., Davis, P. B. (1995). Effect of High-Dose Ibuprofen in Patients with Cystic Fibrosis. NEJM 332: 848-854 [Abstract] [Full Text]  
• Egan, T. M., Detterbeck, F. C., Mill, M. R., Paradowski, L. J., Lackner, R. P., Ogden, W. D., Yankaskas, J. R., Westerman, J. H., Thompson, J. T., Weiner, M. A., Cairns, E. L., Wilcox, B. R. (1995). Improved results of lung transplantation for patients with cystic fibrosis. J. Thorac. Cardiovasc. Surg. 109: 224-235 [Abstract] [Full Text]  
• Davis, P. B. (1994). Evolution of Therapy for Cystic Fibrosis. NEJM 331: 672-673 [Full Text]  
• Webb, A K, David, T J (1994). Clinical management of children and adults with cystic fibrosis. BMJ 308: 459-462 [Full Text]  
• Cooper, J. D., Patterson, G. A., Trulock, E. P., Group, t. W. U. L. T. (1994). Results of single and bilateral lung transplantation in 131 consecutive recipients. J. Thorac. Cardiovasc. Surg. 107: 460-471 [Abstract] [Full Text]