Physiological predictors of cardiorespiratory fitness in children and adolescents with cystic fibrosis without ventilatory limitation

Author:

Burghard Marcella12ORCID,Takken Tim3,Nap-van der Vlist Merel M.4,Nijhof Sanne L.4,van der Ent C. Kors25,Heijerman Harry G.M.26,Hulzebos H.J. Erik32

Affiliation:

1. Child Development, Exercise, and Physical Literacy Center, Wilhelmina Children’s Hospital, University Medical Center Utrecht, P.O. Box 85090, 3508 EA Utrecht, The Netherlands

2. Cystic Fibrosis Center Utrecht, University Medical Center Utrecht, Utrecht, The Netherlands

3. Child Development, Exercise, and Physical Literacy Center, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

4. Department of Social Pediatrics, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

5. Department of Pediatric Pulmonology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

6. Division Heart and Lung, Department of Pulmonology, Cystic Fibrosis Center Utrecht, University Medical Center Utrecht, Utrecht, The Netherlands

Abstract

Objectives: [1] To investigate the cardiorespiratory fitness (CRF) levels in children and adolescents with cystic fibrosis (CF) with no ventilatory limitation (ventilatory reserve ⩾ 15%) during exercise, and [2] to assess which physiological factors are related to CRF. Methods: A cross-sectional study design was used in 8- to 18-year-old children and adolescents with CF. Cardiopulmonary exercise testing was used to determine peak oxygen uptake normalized to body weight as a measure of CRF. Patients were defined as having ‘low CRF’ when CRF was less than 82%predicted. Physiological predictors used in this study were body mass index z-score, P. Aeruginosa lung infection, impaired glucose tolerance (IGT) including CF-related diabetes, CF-related liver disease, sweat chloride concentration, and self-reported physical activity. Backward likelihood ratio (LR) logistic regression analysis was used. Results: Sixty children and adolescents (51.7% boys) with a median age of 15.3 years (25th–75th percentile: 12.9–17.0 years) and a mean percentage predicted forced expiratory volume in 1 second of 88.5% (±16.9) participated. Mean percentage predicted CRF (ppVO2peak/kg) was 81.4% (±12.4, range: 51%–105%). Thirty-three patients (55.0%) were classified as having ‘low CRF’. The final model that best predicted low CRF included IGT ( p = 0.085; Exp(B) = 6.770) and P. Aeruginosa lung infection (p = 0.095; Exp(B) = 3.945). This model was able to explain between 26.7% and 35.6% of variance. Conclusions: CRF is reduced in over half of children and adolescents with CF with normal ventilatory reserve. Glucose intolerance and P. Aeruginosa lung infection seem to be associated to low CRF in children and adolescents with CF.

Publisher

SAGE Publications

Subject

Pharmacology (medical),Pulmonary and Respiratory Medicine

Reference68 articles.

1. Alexander BM, Petren EK, Rizvi S, et al. Cystic Fibrosis Foundation Patient Registry 2014 Annual Data Report Bethesda, Maryland ©2015 Cystic Fibrosis Foundation, https://prod-test.cff.org/2014_CFF_Annual_Data_Report_to_the_Center_Directors.pdf/

2. Quality of life in patients with cystic fibrosis

3. Cardiopulmonary Exercise Testing Provides Additional Prognostic Information in Cystic Fibrosis

4. The Prognostic Value of Exercise Testing in Patients with Cystic Fibrosis

5. Relationship between FEV1 and peak oxygen uptake in children with cystic fibrosis

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