Physical activity and maximal oxygen uptake in adults with Prader–Willi syndrome
- 219 Downloads
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.
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.
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.
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.
KeywordsPrader–Willi syndrome VO2 max Aerobic exercise capacity
- VO2 max
Maximal oxygen uptake
Body mass index
Cardiopulmonary exercise testing
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
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 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.
All authors have indicated they have no financial relationships relevant to this article to disclose.
- 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 CrossRefPubMedGoogle 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):57–66Google Scholar
- 11.Bowers RW, Fox EL (1992) Sports Physiology. McGraw-Hill Higher Education, ColumbusGoogle 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 CrossRefGoogle Scholar
- 15.ACSM (2014) ACSM’s guidelines for exercise testing and prescription, Lippincott Williams and WilkinsGoogle Scholar
- 17.Heyward V (2013) Advanced fitness assessment and exercise prescription 7th Edition eBook—Vivian Heyward, Ann Gibson, Human Kinetics, Inc.Google Scholar
- 18.Roberts CG (1992) Motivation in sport and exercise, Human Kinetics PubGoogle Scholar
- 19.Richard MR, Christina MF, Deborah L, Noel R, Kennon MS (1997) Intrinsic motivation and exercise adherence. Int J Sport Psychol 28:335–354Google Scholar