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Aerobic physical capacity and health-related quality of life in children with sickle cell disease

Pediatric Research (2024)Cite this article

Abstract

Background

Aerobic fitness is a predictor of cardiovascular health which correlates with health-related quality of life in the general population. The aim is to evaluate the aerobic capacity by cardiopulmonary exercise test (CPET) in children with sickle cell disease in comparison with healthy matched controls.

Methods

Controlled cross-sectional study.

Results

A total of 72 children (24 with sickle cell disease and 48 healthy controls), aged 6–17 years old were enrolled. Children with sickle cell disease had a poor aerobic capacity, with median VO2max Z-score values significantly lower than matched controls (−3.55[−4.68; −2.02] vs. 0.25[−0.22; 0.66], P < 0.01, respectively), and a high proportion of 92% children affected by an impaired aerobic capacity (VO2max Z-score < −1.64). The VO2max decrease was associated with the level of anemia, the existence of a homozygote HbS/S mutation, restrictive lung disease and health-related quality of life.

Conclusion

Aerobic capacity is poor in children with sickle cell disease. VO2max decrease is associated with the level of anemia, the existence of a homozygote HbS/S mutation, lung function, and health-related quality of life. These results represent a signal in favor of early initiation of cardiac rehabilitation in patients with sickle cell disease.

Clinical trials

NCT05995743.

Impact

  • Aerobic fitness is a predictor of cardiovascular health which correlates with health-related quality of life in the general population.

  • Aerobic capacity (VO2max) is poor in children with sickle cell disease, despite the absence of any pattern of heart failure.

  • VO2max decrease was associated with the level of anemia, the existence of a homozygote HbS/S mutation, restrictive lung disease, and health-related quality of life.

  • These results are in favor of early initiation of cardiac rehabilitation in children with sickle cell disease.

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Fig. 1: VO2max Z-score in the sickle cell disease group and in matched controls.
Fig. 2: Correlation between aerobic capacity and HRQoL in the sickle cell disease group.
Fig. 3: Predictors of impaired aerobic capacity in children and adolescents with sickle cell disease.

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Data availability

The datasets used and/or analyzed during the current study are available from the corresponding author upon reasonable request.

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Author information

Authors and Affiliations

  1. Department of Pediatric and Congenital Cardiology, M3C Regional Reference CHD Centre, Montpellier University Hospital, 371 Avenue du Doyen Giraud, 34295, Montpellier, France

    Corentin Laurent-Lacroix, Marie Vincenti, Gregoire De La Villeon & Sophie Guillaumont

  2. PhyMedExp, CNRS, INSERM, University of Montpellier, 371 Avenue du Doyen Giraud, 34295, Montpellier, France

    Marie Vincenti, Stefan Matecki & Arthur Gavotto

  3. Pediatric Functional Exploration Laboratory, Physiology Department, Montpellier University Hospital, 371 Avenue du Doyen Giraud, 34295, Montpellier, France

    Stefan Matecki, Anne Requirand & Johan Moreau

  4. Pediatric Department, Montpellier University Hospital, 371 Avenue du Doyen Giraud, 34295, Montpellier, France

    Perrine Mahé & Muriel Lalande

  5. Reference Center on Rare Red Cell Disorders, Montpellier University Hospital, 34000, Montpellier, France

    Perrine Mahé & Muriel Lalande

  6. Clinical Research and Epidemiology Unit, Montpellier University Hospital, 34000, Montpellier, France

    Lionel Moulis & Marie-Christine Picot

  7. Pediatric Cardiology and Rehabilitation Unit, St-Pierre Institute, 371 Avenue de l’Évêché de Maguelone, 34250, Palavas-Les-Flots, France

    Gregoire De La Villeon & Sophie Guillaumont

  8. Department of Pediatric Pneumology, Montpellier University Hospital, 371 Avenue du Doyen Giraud, 34295, Montpellier, France

    Johan Moreau

  9. Clinical Investigation Centre, INSERM-CIC 1411, University of Montpellier, 34000, Montpellier, France

    Marie-Christine Picot

  10. Department of Pediatric and Congenital Cardiology, M3C National Reference Centre, Bordeaux University Hospital, 1 Avenue Magellan, 33604, Pessac, France

    Pascal Amedro

  11. IHU Liryc, Electrophysiology and Heart Modelling Institute, INSERM 1045, Bordeaux University Foundation, Avenue du Haut Lévêque, 33600, Pessac, France

    Pascal Amedro

  12. Pediatric Intensive Care Unit, Arnaud de Villeneuve Hospital, Montpellier University Hospital, 371 Avenue du Doyen Giraud, 34295, Montpellier, France

    Arthur Gavotto

Authors
  1. Corentin Laurent-Lacroix
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Contributions

Laurent-Lacroix and Vincenti contributed to the study’s conception and design. Material preparation, data collection and analysis were performed by Laurent-Lacroix, Vincenti, Matecki, Moulis, Amedro, and Gavotto. The first draft of the manuscript was written by Laurent-Lacroix, Vincenti, Amedro, and Gavotto and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Arthur Gavotto.

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The authors declare no competing interests.

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The study was conducted in compliance with the Good Clinical Practices protocol and Declaration of Helsinki principles. It was approved by our institutional review board (IRB-MTP_2022_04_202201102) and registered on Clinicaltrials.gov (NCT05995743). Informed consent was obtained from all parents or legal guardians.

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Laurent-Lacroix, C., Vincenti, M., Matecki, S. et al. Aerobic physical capacity and health-related quality of life in children with sickle cell disease. Pediatr Res (2024). https://doi.org/10.1038/s41390-024-03143-1

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  • DOI: https://doi.org/10.1038/s41390-024-03143-1

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