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

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.

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Fig. 1

Abbreviations

PWS:

Prader–Willi syndrome

VO2 max:

Maximal oxygen uptake

OC:

Overweight controls

BMI:

Body mass index

GH:

Growth hormone

CPET:

Cardiopulmonary exercise testing

References

  1. Cassidy SB, Schwartz S, Miller JL, Driscoll DJ (2012) Prader–Willi syndrome. Genet Med 14:10–26. doi:10.1038/gim.0b013e31822bead0

    CAS  Article  PubMed  Google Scholar 

  2. Grolla E, Andrighetto G, Parmigiani P, Hladnik U, Ferrari G, Bernardelle R, Lago MD, Albarello A, Baschirotto G, Filippi G, Lovato R, Dolcetta D (2011) Specific treatment of Prader–Willi syndrome through cyclical rehabilitation programmes. Disabil Rehabil 33:1837–1847. doi:10.3109/09638288.2010.549288

    Article  PubMed  Google Scholar 

  3. Castner DM, Tucker JM, Wilson KS, Rubin DA (2014) Patterns of habitual physical activity in youth with and without Prader–Willi Syndrome. Res Dev Disabil 35:3081–3088. doi:10.1016/j.ridd.2014.07.035

    Article  PubMed  Google Scholar 

  4. Butler MG, Theodoro MF, Bittel DC, Donnelly JE (2007) Energy expenditure and physical activity in Prader–Willi syndrome: comparison with obese subjects. Am J Med Genet A 143a:449–459. doi:10.1002/ajmg.a.31507

    Article  PubMed  PubMed Central  Google Scholar 

  5. Rubin DA, Mouttapa M, Weiss JW, Barrera-Ng A (2012) Physical activity in children with Prader–Willi syndrome: a parents’ perspective. Californian J Health Promot 10(S1):57–66

    Google Scholar 

  6. Nordstrøm M, Hansen BH, Paus B, Kolset SO (2013) Accelerometer-determined physical activity and walking capacity in persons with Down syndrome, Williams syndrome and Prader–Willi syndrome. Res Dev Disabil 34:4395–4403. doi:10.1016/j.ridd.2013.09.021

    Article  PubMed  Google Scholar 

  7. Silverthorn KH, Hornak JE (1993) Beneficial effects of exercise on aerobic capacity and body composition in adults with Prader–Willi syndrome. Am J Ment Retard 97:654–658

    CAS  PubMed  Google Scholar 

  8. Messersmith NV, Slifer KJ, Pulbrook-Vetter V, Bellipanni K (2008) Interdisciplinary behavioral intervention for life-threatening obesity in an adolescent with Prader–Willi syndrome—a case report. J Dev Behav Pediatr 29:129–134

    Article  Google Scholar 

  9. Bekx MT, Carrel AL, Shriver TC, Li Z, Allen DB (2003) Decreased EE is caused by abnormal body composition in infants with Prader–Willi Syndrome. J Pediatr. doi:10.1067/S0022-3476(03)00386-X

    Article  PubMed  Google Scholar 

  10. Rankovic G, Mutavdzic V, Toskic D, Preljevic A, Kocic M, Nedin Rankovic G, Damjanovic N (2010) Aerobic capacity as an indicator in different kinds of sports. Bosn J Basic Med Sci 10:44–48

    Article  Google Scholar 

  11. Bowers RW, Fox EL (1992) Sports Physiology. McGraw-Hill Higher Education, Columbus

  12. Tabata I, Nishimura K, Kouzaki M, Hirai Y, Ogita F, Miyachi M, Yamamoto K (1996) Effects of moderate-intensity endurance and high-intensity intermittent training on anaerobic capacity and VO2max. Med Sci Sports Exerc 28:1327–1330

    CAS  Article  Google Scholar 

  13. Garber CE, Blissmer B, Deschenes MR, Franklin BA, Lamonte MJ, Lee IM, Nieman DC, Swain DP (2011) American College of Sports Medicine position stand. Quantity and quality of exercise for developing and maintaining cardiorespiratory, musculoskeletal, and neuromotor fitness in apparently healthy adults: guidance for prescribing exercise. Med Sci Sports Exerc 43:1334–1359. doi:10.1249/MSS.0b013e318213fefb

    Article  PubMed  Google Scholar 

  14. Uth N, Sorensen H, Overgaard K, Pedersen PK (2004) Estimation of VO2max from the ratio between HRmax and HRrest—the Heart Rate Ratio Method. Eur J Appl Physiol 91:111–115. doi:10.1007/s00421-003-0988-y

    Article  PubMed  Google Scholar 

  15. ACSM (2014) ACSM’s guidelines for exercise testing and prescription, Lippincott Williams and Wilkins

  16. Larsson I, Henning B, Lindroos AK, Naslund I, Sjostrom CD, Sjostrom L (2006) Optimized predictions of absolute and relative amounts of body fat from weight, height, other anthropometric predictors, and age. Am J Clin Nutr 83:252–259. doi:10.1038/oby.2005.82

    CAS  Article  PubMed  Google Scholar 

  17. Heyward V (2013) Advanced fitness assessment and exercise prescription 7th Edition eBook—Vivian Heyward, Ann Gibson, Human Kinetics, Inc.

  18. Roberts CG (1992) Motivation in sport and exercise, Human Kinetics Pub

  19. Richard MR, Christina MF, Deborah L, Noel R, Kennon MS (1997) Intrinsic motivation and exercise adherence. Int J Sport Psychol 28:335–354

    Google Scholar 

  20. Martin K, Kaltenmark T, Lewallen A, Smith C, Yoshida A (2007) Clinical characteristics of hypotonia: a survey of pediatric physical and occupational therapists. Pediatr Phys Ther 19:217–226. doi:10.1097/PEP.0b013e3180f62bb0

    Article  PubMed  Google Scholar 

  21. Parker AW, James B (1985) Age changes in the flexibility of Down’s syndrome children. J Ment Defic Res 29(Pt 3):207–218

    PubMed  Google Scholar 

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Acknowledgements

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

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Correspondence to Itai Gross.

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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.

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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.

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

Additional information

I. Gross and H. J. Hirsch contributed equally to this work.

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Gross, I., Hirsch, H.J., Constantini, N. et al. Physical activity and maximal oxygen uptake in adults with Prader–Willi syndrome. Eat Weight Disord 23, 615–620 (2018). https://doi.org/10.1007/s40519-016-0356-7

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Keywords

  • Prader–Willi syndrome
  • VO2 max
  • Aerobic exercise capacity