FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 327:1785-1788 December 17, 1992 Number 25 Next

	Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

Abstract

BACKGROUND. Previous studies have shown female sex, impaired pulmonary function, older age, malnutrition, and colonization of the respiratory tract with Pseudomonas cepacia to be associated with a poor prognosis in patients with cystic fibrosis. We sought to determine the prognostic value of exercise testing in addition to the other prognostic factors. METHODS. A total of 109 patients with cystic fibrosis, 7 to 35 years old, underwent pulmonary-function and exercise testing in the late 1970s. They were followed for eight years to determine the factors associated with subsequent mortality. Survival rates were calculated with standard life-table methods. Cox proportional-hazards regression models were used to determine crude relative risks of mortality and relative risks adjusted for age, sex, body-mass index, forced expiratory volume in one second (FEV1) end-tidal partial pressure of carbon dioxide (PCO2) at peak exercise, and oxygen consumption at peak exercise (VO2 peak). RESULTS. Patients with the highest levels of aerobic fitness (VO2 peak, > or = 82 percent of predicted) had a survival rate of 83 percent at eight years, as compared with rates of 51 percent and 28 percent for patients with middle (VO2 peak, 59 to 81 percent of predicted) and lowest (VO2 peak, < or = 58 percent of predicted) levels of fitness, respectively. After adjustment for other risk factors, patients with higher levels of aerobic fitness were more than three times as likely to survive than patients with lower levels of fitness. Colonization with P. cepacia was associated with a risk of dying that was increased fivefold. Age, sex, body-mass index, FEV1, and end-tidal PCO2 at peak exercise were not independently correlated with mortality. CONCLUSIONS. Higher levels of aerobic fitness in patients with cystic fibrosis are associated with a significantly lower risk of dying. Although better aerobic fitness may simply be a marker for less severe illness, measurement of VO2 peak appears to be valuable for predicting prognosis. Further research is warranted to determine whether improving aerobic fitness through exercise programs will result in a better prognosis.

Source Information

Department of Pediatrics, University of Pittsburgh, PA.

This article has been cited by other articles:

• Barry, P. J., Waterhouse, D. F., Reilly, C. M., McKenna, T. J., McKone, E. F., Gallagher, C. G. (2008). Androgens, Exercise Capacity, and Muscle Function in Cystic Fibrosis. Chest 134: 1258-1264 [Abstract] [Full Text]  
• Young, A C, Wilson, J W, Kotsimbos, T C, Naughton, M T (2008). Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis. Thorax 63: 72-77 [Abstract] [Full Text]  
• Schnabel, D., Grasemann, C., Staab, D., Wollmann, H., Ratjen, F., for the German Cystic Fibrosis Growth Hormone Stud, (2007). A Multicenter, Randomized, Double-Blind, Placebo-Controlled Trial to Evaluate the Metabolic and Respiratory Effects of Growth Hormone in Children With Cystic Fibrosis. Pediatrics 119: e1230-e1238 [Abstract] [Full Text]  
• Mayer-Hamblett, N., Aitken, M. L., Accurso, F. J., Kronmal, R. A., Konstan, M. W., Burns, J. L., Sagel, S. D., Ramsey, B. W. (2007). Association between Pulmonary Function and Sputum Biomarkers in Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 175: 822-828 [Abstract] [Full Text]  
• Serisier, D. J., Coates, A. D., Bowler, S. D. (2007). Effect of Albuterol on Maximal Exercise Capacity in Cystic Fibrosis. Chest 131: 1181-1187 [Abstract] [Full Text]  
• Palange, P., Ward, S. A., Carlsen, K-H., Casaburi, R., Gallagher, C. G., Gosselink, R., O'Donnell, D. E., Puente-Maestu, L., Schols, A. M., Singh, S., Whipp, B. J. (2007). Recommendations on the use of exercise testing in clinical practice. Eur Respir J 29: 185-209 [Abstract] [Full Text]  
• Decramer, M., Gosselink, R. (2006). Physical activity in patients with cystic fibrosis: a new variable in the health-status equation unravelled?. Eur Respir J 28: 678-679 [Full Text]  
• Hebestreit, H., Kieser, S., Rudiger, S., Schenk, T., Junge, S., Hebestreit, A., Ballmann, M., Posselt, H-G., Kriemler, S. (2006). Physical activity is independently related to aerobic capacity in cystic fibrosis. Eur Respir J 28: 734-739 [Abstract] [Full Text]  
• Dodd, J. D., Barry, S. C., Barry, R. B. M., Gallagher, C. G., Skehan, S. J., Masterson, J. B. (2006). Thin-Section CT in Patients with Cystic Fibrosis: Correlation with Peak Exercise Capacity and Body Mass Index.. Radiology 240: 236-245 [Abstract] [Full Text]  
• Belkin, R. A., Henig, N. R., Singer, L. G., Chaparro, C., Rubenstein, R. C., Xie, S. X., Yee, J. Y., Kotloff, R. M., Lipson, D. A., Bunin, G. R. (2006). Risk Factors for Death of Patients with Cystic Fibrosis Awaiting Lung Transplantation. Am. J. Respir. Crit. Care Med. 173: 659-666 [Abstract] [Full Text]  
• McKone, E. F., Barry, S. C., FitzGerald, M. X., Gallagher, C. G. (2005). Role of arterial hypoxemia and pulmonary mechanics in exercise limitation in adults with cystic fibrosis. J. Appl. Physiol. 99: 1012-1018 [Abstract] [Full Text]  
• Javadpour, S M, Selvadurai, H, Wilkes, D L, Schneiderman-Walker, J, Coates, A L (2005). Does carbon dioxide retention during exercise predict a more rapid decline in FEV1 in cystic fibrosis?. Arch. Dis. Child. 90: 792-795 [Abstract] [Full Text]  
• Dodd, M E, Prasad, S A (2005). Physiotherapy management of cystic fibrosis. Chronic Respiratory Disease 2: 139-149 [Abstract]  
• Pianosi, P, LeBlanc, J, Almudevar, A (2005). Peak oxygen uptake and mortality in children with cystic fibrosis. Thorax 60: 50-54 [Abstract] [Full Text]  
• Moorcroft, A J, Dodd, M E, Morris, J, Webb, A K (2004). Individualised unsupervised exercise training in adults with cystic fibrosis: a 1 year randomised controlled trial. Thorax 59: 1074-1080 [Abstract] [Full Text]  
• Edwards, E.A., Narang, I., Li, A., Hansell, D.M., Rosenthal, M., Bush, A. (2004). HRCT lung abnormalities are not a surrogate for exercise limitation in bronchiectasis. Eur Respir J 24: 538-544 [Abstract] [Full Text]  
• Orenstein, D. M., Hovell, M. F., Mulvihill, M., Keating, K. K., Hofstetter, C. R., Kelsey, S., Morris, K., Nixon, P. A. (2004). Strength vs Aerobic Training in Children With Cystic Fibrosis: A Randomized Controlled Trial. Chest 126: 1204-1214 [Abstract] [Full Text]  
• Selvadurai, H C, Blimkie, C J, Cooper, P J, Mellis, C M, Van Asperen, P P (2004). Gender differences in habitual activity in children with cystic fibrosis. Arch. Dis. Child. 89: 928-933 [Abstract] [Full Text]  
• Enright, S., Chatham, K., Ionescu, A. A., Unnithan, V. B., Shale, D. J. (2004). Inspiratory Muscle Training Improves Lung Function and Exercise Capacity in Adults With Cystic Fibrosis. Chest 126: 405-411 [Abstract] [Full Text]  
• Klijn, P. H. C., Oudshoorn, A., van der Ent, C. K., van der Net, J., Kimpen, J. L., Helders, P. J. M. (2004). Effects of Anaerobic Training in Children With Cystic Fibrosis: A Randomized Controlled Study. Chest 125: 1299-1305 [Abstract] [Full Text]  
• Divangahi, M., Matecki, S., Dudley, R. W. R., Tuck, S. A., Bao, W., Radzioch, D., Comtois, A. S., Petrof, B. J. (2004). Preferential Diaphragmatic Weakness during Sustained Pseudomonas aeruginosa Lung Infection. Am. J. Respir. Crit. Care Med. 169: 679-686 [Abstract] [Full Text]  
• Selvadurai, H. C., Allen, J., Sachinwalla, T., Macauley, J., Blimkie, C. J., Van Asperen, P. P. (2003). Muscle Function and Resting Energy Expenditure in Female Athletes with Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 168: 1476-1480 [Abstract] [Full Text]  
• Klijn, P. H. C., van der Net, J., Kimpen, J. L., Helders, P. J. M., van der Ent, C. K. (2003). Longitudinal Determinants of Peak Aerobic Performance in Children With Cystic Fibrosis. Chest 124: 2215-2219 [Abstract] [Full Text]  
• Schibler, A, von der Heiden, R, Birrer, P, Mullis, P E (2003). Prospective randomised treatment with recombinant human growth hormone in cystic fibrosis. Arch. Dis. Child. 88: 1078-1081 [Abstract] [Full Text]  
• Sexauer, W. P., Cheng, H.-K., Fiel, S. B. (2003). Utility of the Breathing Reserve Index at the Anaerobic Threshold in Determining Ventilatory-Limited Exercise in Adult Cystic Fibrosis Patients. Chest 124: 1469-1475 [Abstract] [Full Text]  
• (2003). ATS/ACCP Statement on Cardiopulmonary Exercise Testing. Am. J. Respir. Crit. Care Med. 167: 211-277 [Full Text]  
• McKone, E.F., Barry, S.C., FitzGerald, M.X., Gallagher, C.G. (2002). The role of supplemental oxygen during submaximal exercise in patients with cystic fibrosis. Eur Respir J 20: 134-142 [Abstract] [Full Text]  
• Tantisira, K. G., Systrom, D. M., Ginns, L. C. (2002). An Elevated Breathing Reserve Index at the Lactate Threshold Is a Predictor of Mortality in Patients with Cystic Fibrosis Awaiting Lung Transplantation. Am. J. Respir. Crit. Care Med. 165: 1629-1633 [Abstract] [Full Text]  
• Thin, A. G., Linnane, S. J., McKone, E. F., Freaney, R., FitzGerald, M. X., Gallagher, C. G., McLoughlin, P. (2002). Use of the Gas Exchange Threshold to Noninvasively Determine the Lactate Threshold in Patients With Cystic Fibrosis*. Chest 121: 1761-1770 [Abstract] [Full Text]  
• SELVADURAI, H. C., MCKAY, K. O., BLIMKIE, C. J., COOPER, P. J., MELLIS, C. M., VAN ASPEREN, P. P. (2002). The Relationship between Genotype and Exercise Tolerance in Children with Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 165: 762-765 [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]  
• Karila, C., de Blic, J., Waernessyckle, S., Benoist, M.-R., Scheinmann, P. (2001). Cardiopulmonary Exercise Testing in Children : An Individualized Protocol for Workload Increase. Chest 120: 81-87 [Abstract] [Full Text]  
• MOSER, C., TIRAKITSOONTORN, P., NUSSBAUM, E., NEWCOMB, R., COOPER, D. M. (2000). Muscle Size and Cardiorespiratory Response to Exercise in Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 162: 1823-1827 [Abstract] [Full Text]  
• Bradley, J., Howard, J., Wallace, E., Elborn, S. (2000). Reliability, Repeatability, and Sensitivity of the Modified Shuttle Test in Adult Cystic Fibrosis. Chest 117: 1666-1671 [Abstract] [Full Text]  
• McKone, E. F., Barry, S. C., FitzGerald, M. X., Gallagher, C. G. (1999). Reproducibility of Maximal Exercise Ergometer Testing in Patients With Cystic Fibrosis. Chest 116: 363-368 [Abstract] [Full Text]  
• de MEER, K., GULMANS, V. A. M., van der LAAG, J. (1999). Peripheral Muscle Weakness and Exercise Capacity in Children with Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 159: 748-754 [Abstract] [Full Text]  
• SHAH, A. R., GOZAL, D., KEENS, T. G. (1998). Determinants of Aerobic and Anaerobic Exercise Performance in Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 157: 1145-1150 [Abstract] [Full Text]  
• Shah, A. R., Keens, T. G., Gozal, D. (1997). Effect of supplemental oxygen on supramaximal exercise performance and recovery in cystic fibrosis. J. Appl. Physiol. 83: 1641-1647 [Abstract] [Full Text]  
• ALISON, J. A., REGNIS, J. A., DONNELLY, P. M., ADAMS, R. D., SUTTON, J. R., BYE, P. T. P. (1997). Evaluation of Supported Upper Limb Exercise Capacity in Patients with Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 156: 1541-1548 [Abstract] [Full Text]  
• Pegues, D. A., Schidlow, D. V., Tablan, O. C., Carson, L. A., Clark, N. C., Jarvis, W. R. (1994). Possible Nosocomial Transmission of Pseudomonas cepacia in Patients With Cystic Fibrosis. Arch Pediatr Adolesc Med 148: 805-812 [Abstract]