Abstract
Background: Single measurements of peak oxygen uptake (V·O2) have been shown to predict mortality in patients with cystic fibrosis (CF) although no longitudinal study of serial measurements has been reported in children. A study was undertaken to determine whether the initial, final, or the rate of fall of forced expiratory volume in 1 second (FEV1) or peak V·O2 was a better predictor of mortality.
Methods: Twenty eight children aged 8–17 years with CF performed annual pulmonary function and maximal exercise tests over a 5 year period to determine FEV1 and peak V·O2, magnitude of their change over time, and survival over the subsequent 7–8 years. Analysis was done using Kaplan-Meier curves and Cox proportional hazard model.
Results: Peak V·O2 fell during the observation period in 70% of the patients, with a mean annual decline of 2.1 ml/min/kg. Initial peak V·O2 was not predictive of mortality but rate of decline and final peak V·O2 of the series were significant predictors. Patients with peak V·O2 less than 32 ml/min/kg exhibited a dramatic increase in mortality, in contrast to those whose peak V·O2 exceeded 45 ml/min/kg, none of whom died. The first, last, and rate of decline in FEV1 over time were all significant predictors of mortality.
Conclusions: Higher peak V·O2 is a marker for longer survival in CF patients.
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Selected References
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- Boucher G. P., Lands L. C., Hay J. A., Hornby L. Activity levels and the relationship to lung function and nutritional status in children with cystic fibrosis. Am J Phys Med Rehabil. 1997 Jul-Aug;76(4):311–315. doi: 10.1097/00002060-199707000-00010. [DOI] [PubMed] [Google Scholar]
- Britto M. T., Garrett J. M., Konrad T. R., Majure J. M., Leigh M. W. Comparison of physical activity in adolescents with cystic fibrosis versus age-matched controls. Pediatr Pulmonol. 2000 Aug;30(2):86–91. doi: 10.1002/1099-0496(200008)30:2<86::aid-ppul2>3.0.co;2-z. [DOI] [PubMed] [Google Scholar]
- Cerny F. J., Pullano T. P., Cropp G. J. Cardiorespiratory adaptations to exercise in cystic fibrosis. Am Rev Respir Dis. 1982 Aug;126(2):217–220. doi: 10.1164/arrd.1982.126.2.217. [DOI] [PubMed] [Google Scholar]
- Corey M., Edwards L., Levison H., Knowles M. Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis. J Pediatr. 1997 Dec;131(6):809–814. doi: 10.1016/s0022-3476(97)70025-8. [DOI] [PubMed] [Google Scholar]
- Cropp G. J., Pullano T. P., Cerny F. J., Nathanson I. T. Exercise tolerance and cardiorespiratory adjustments at peak work capacity in cystic fibrosis. Am Rev Respir Dis. 1982 Aug;126(2):211–216. doi: 10.1164/arrd.1982.126.2.211. [DOI] [PubMed] [Google Scholar]
- Godfrey S., Mearns M. Pulmonary function and response to exercise in cystic fibrosis. Arch Dis Child. 1971 Apr;46(246):144–151. doi: 10.1136/adc.46.246.144. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Knudson R. J., Lebowitz M. D., Holberg C. J., Burrows B. Changes in the normal maximal expiratory flow-volume curve with growth and aging. Am Rev Respir Dis. 1983 Jun;127(6):725–734. doi: 10.1164/arrd.1983.127.6.725. [DOI] [PubMed] [Google Scholar]
- Lands L. C., Heigenhauser G. J., Jones N. L. Analysis of factors limiting maximal exercise performance in cystic fibrosis. Clin Sci (Lond) 1992 Oct;83(4):391–397. doi: 10.1042/cs0830391. [DOI] [PubMed] [Google Scholar]
- Marcotte J. E., Canny G. J., Grisdale R., Desmond K., Corey M., Zinman R., Levison H., Coates A. L. Effects of nutritional status on exercise performance in advanced cystic fibrosis. Chest. 1986 Sep;90(3):375–379. doi: 10.1378/chest.90.3.375. [DOI] [PubMed] [Google Scholar]
- Marcotte J. E., Grisdale R. K., Levison H., Coates A. L., Canny G. J. Multiple factors limit exercise capacity in cystic fibrosis. Pediatr Pulmonol. 1986 Sep-Oct;2(5):274–281. doi: 10.1002/ppul.1950020505. [DOI] [PubMed] [Google Scholar]
- Moorcroft A. J., Dodd M. E., Webb A. K. Exercise testing and prognosis in adult cystic fibrosis. Thorax. 1997 Mar;52(3):291–293. doi: 10.1136/thx.52.3.291. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Moorcroft A. J., Dodd M. E., Webb A. K. Long-term change in exercise capacity, body mass, and pulmonary function in adults with cystic fibrosis. Chest. 1997 Feb;111(2):338–343. doi: 10.1016/s0012-3692(15)52531-2. [DOI] [PubMed] [Google Scholar]
- Nixon P. A., Orenstein D. M., Kelsey S. F., Doershuk C. F. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med. 1992 Dec 17;327(25):1785–1788. doi: 10.1056/NEJM199212173272504. [DOI] [PubMed] [Google Scholar]
- Nixon P. A., Orenstein D. M., Kelsey S. F. Habitual physical activity in children and adolescents with cystic fibrosis. Med Sci Sports Exerc. 2001 Jan;33(1):30–35. doi: 10.1097/00005768-200101000-00006. [DOI] [PubMed] [Google Scholar]
- Oga Toru, Nishimura Koichi, Tsukino Mitsuhiro, Sato Susumu, Hajiro Takashi. Analysis of the factors related to mortality in chronic obstructive pulmonary disease: role of exercise capacity and health status. Am J Respir Crit Care Med. 2002 Nov 21;167(4):544–549. doi: 10.1164/rccm.200206-583OC. [DOI] [PubMed] [Google Scholar]
- Rogan Mark P., Taggart Clifford C., Greene Catherine M., Murphy Philip G., O'Neill Shane J., McElvaney Noel G. Loss of microbicidal activity and increased formation of biofilm due to decreased lactoferrin activity in patients with cystic fibrosis. J Infect Dis. 2004 Aug 26;190(7):1245–1253. doi: 10.1086/423821. [DOI] [PubMed] [Google Scholar]
- Santis G., Osborne L., Knight R. A., Hodson M. E. Linked marker haplotypes and the delta F508 mutation in adults with mild pulmonary disease and cystic fibrosis. Lancet. 1990 Jun 16;335(8703):1426–1429. doi: 10.1016/0140-6736(90)91448-j. [DOI] [PubMed] [Google Scholar]
- Selvadurai Hiran C., McKay Karen O., Blimkie Cameron J., Cooper Peter J., Mellis Craig M., Van Asperen Peter P. The relationship between genotype and exercise tolerance in children with cystic fibrosis. Am J Respir Crit Care Med. 2002 Mar 15;165(6):762–765. doi: 10.1164/ajrccm.165.6.2104036. [DOI] [PubMed] [Google Scholar]
- Sharma R., Florea V. G., Bolger A. P., Doehner W., Florea N. D., Coats A. J., Hodson M. E., Anker S. D., Henein M. Y. Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax. 2001 Oct;56(10):746–750. doi: 10.1136/thorax.56.10.746. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Stanghelle J. K., Skyberg D., Haanaes O. C. Eight-year follow-up of pulmonary function and oxygen uptake during exercise in 16-year-old males with cystic fibrosis. Acta Paediatr. 1992 Jun-Jul;81(6-7):527–531. doi: 10.1111/j.1651-2227.1992.tb12288.x. [DOI] [PubMed] [Google Scholar]
- Welsman J. R., Armstrong N. Daily physical activity and blood lactate indices of aerobic fitness in children. Br J Sports Med. 1992 Dec;26(4):228–232. doi: 10.1136/bjsm.26.4.228. [DOI] [PMC free article] [PubMed] [Google Scholar]