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Physical activity and maximal oxygen uptake in adults with Prader–Willi syndrome

  • Itai Gross
  • Harry J. Hirsch
  • Naama Constantini
  • Shachar Nice
  • Yehuda Pollak
  • Larry Genstil
  • Talia Eldar-Geva
  • Varda Gross Tsur
Original Article
  • 219 Downloads

Abstract

Background

Prader–Willi Syndrome (PWS) is the most common genetic syndrome causing life-threatening obesity. Strict adherence to a low-calorie diet and regular physical activity are needed to prevent weight gain. Direct measurement of maximal oxygen uptake (VO2 max), the “gold standard” for assessing aerobic exercise capacity, has not been previously described in PWS.

Objectives

Assess aerobic capacity by direct measurement of VO2 max in adults with PWS, and in age and BMI-matched controls (OC), and compare the results with values obtained by indirect prediction methods.

Methods and patients

Seventeen individuals (12 males) age: 19–35 (28.6 ± 4.9) years, BMI: 19.4–38.1 (27.8 ± 5) kg/m2 with genetically confirmed PWS who exercise daily, and 32 matched OC (22 males) age: 19–36 (29.3 ± 5.2) years, BMI: 21.1–48.1 (26.3 ± 4.9) kg/m2. All completed a medical questionnaire and performed strength and flexibility tests. VO2 max was determined by measuring oxygen consumption during a graded exercise test on a treadmill.

Results

VO2 max (24.6 ± 3.4 vs 46.5 ± 12.2 ml/kg/min, p < 0.001) and ventilatory threshold (20 ± 2 and 36.2 ± 10.5 ml/kg/min, p < 0.001), maximal strength of both hands (36 ± 4 vs 91.4 ± 21.2 kg, p < 0.001), and flexibility (15.2 ± 9.5 vs 26 ± 11.1 cm, p = 0.001) were all significantly lower for PWS compared to OC. Predicted estimates and direct measurements of VO2 max were almost identical for the OC group (p = 0.995), for the PWS group, both methods for estimating VO2 max gave values which were significantly greater (p < 0.001) than results obtained by direct measurements.

Conclusions

Aerobic capacity, assessed by direct measurement of VO2 max, is significantly lower in PWS adults, even in those who exercise daily, compared to OCs. Indirect estimates of VO2 max are accurate for OC, but unreliable in PWS. Direct measurement of VO2 should be used for designing personal training programs and in clinical studies of exercise in PWS.

Keywords

Prader–Willi syndrome VO2 max Aerobic exercise capacity 

Abbreviations

PWS

Prader–Willi syndrome

VO2 max

Maximal oxygen uptake

OC

Overweight controls

BMI

Body mass index

GH

Growth hormone

CPET

Cardiopulmonary exercise testing

Notes

Acknowledgements

We are grateful to Nava Badichi for administrative assistance in coordinating this study and to the precipitants and their families.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study. Additional informed consent was obtained from all individual participants for whom identifying information is included in this article.

Financial disclosure

All authors have indicated they have no financial relationships relevant to this article to disclose.

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Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  • Itai Gross
    • 1
  • Harry J. Hirsch
    • 2
    • 3
  • Naama Constantini
    • 4
  • Shachar Nice
    • 4
  • Yehuda Pollak
    • 5
  • Larry Genstil
    • 2
    • 3
  • Talia Eldar-Geva
    • 2
    • 6
    • 7
  • Varda Gross Tsur
    • 2
    • 3
    • 6
  1. 1.Department of PediatricsHadassah Medical CenterJerusalemIsrael
  2. 2.Israel Multidisciplinary PraderWilli Syndrome Clinic, Neuropediatric Unit, Department of PediatricsShaare Zedek Medical CenterJerusalemIsrael
  3. 3.Neuropediatric Unit, Department of PediatricsShaare Zedek Medical CenterJerusalemIsrael
  4. 4.Sports Medicine Center, Department of Orthopedic Surgery, Hadassah Medical CenterHebrew University Medical CenterJerusalemIsrael
  5. 5.The School of EducationHebrew UniversityJerusalemIsrael
  6. 6.The Hebrew University Faculty of MedicineJerusalemIsrael
  7. 7.Reproductive Endocrinology and Genetics Unit, Department of Obstetrics and GynecologyShaare Zedek Medical CenterJerusalemIsrael

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