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Peak oxygen uptake and mortality in children with cystic fibrosis

¹ Department of Pediatrics, Dalhousie University, Halifax, Canada
² Department of Biostatistics, University of Rochester, Rochester, NY, USA

Correspondence to:
Correspondence to:
Dr P Pianosi
Respirology Clinic, IWK Health Centre, P O Box 3070, Halifax, Canada B3J 3G9; ppianosi{at}dal.ca

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.

Keywords: cystic fibrosis; exercise; peak oxygen uptake; pulmonary function; survival

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