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Exercise interventions may provide important benefits in Prader-Willi syndrome
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Children and adults with Prader-Willi syndrome who participated in long-term exercise interventions experienced increased physical activity level, physical capacity and body coordination, as well as improvements in cardiometabolic markers, particularly when combined with nutrition therapy, according to a systematic review published in Clinical Endocrinology.
“Children, adolescents and adults with [Prader-Willi syndrome] usually present with an impaired physical capacity and low cardiovascular fitness potentially related to their obesity, poor muscle mass and hypotonia,” Javier S. Morales, MSc, a doctoral student and research assistant in the faculty of sports sciences at the European University of Madrid, and colleagues wrote. “Exercise training interventions in individuals with [Prader-Willi syndrome] could represent safe and effective means to improve physical fitness phenotypes in these patients.”
Morales and colleagues analyzed 22 studies reporting on acute physiological and/or performance response (after one exercise bout or during a physical test) or on the long-term effects of physical exercise (after an exercise intervention) in 766 participants (age range, 4 to 44 years), including 356 patients with Prader-Willi syndrome (242 children and adolescents). All but three studies included fewer than 20 participants with Prader-Willi syndrome. Included patients with Prader-Willi syndrome reported prescriptions for a variety of pharmacologica treatments, including insulin and oral antidiabetes medications, growth hormone or testosterone replacement therapy and psychiatric medications.
Acute response to exercise
Ten studies assessed physiologic responses or physical performance measures of patients with Prader-Willi syndrome after completing a variety of exercise tests, including cycle-ergometer, lower-body strength, treadmill or the Bruininks-Oseretsky test to analyze motor proficiency. Eight of the 10 studies were conducted in children or adolescents.
In the five studies assessing cardiorespiratory response, researchers observed peak values of exertional heart rate among patients with Prader-Willi syndrome that were 12% to 22% lower than controls. Researchers also noted a slower heart rate recovery after submaximal exercise in children with Prader-Willi syndrome vs. normal-weight peers, as well as a 12% to 14% higher heart rate during submaximal exercise. Similarly, adults with Prader-Willi syndrome showed lower values of maximal oxygen intake and ventilatory threshold vs. adults with overweight or obesity but without Prader-Willi syndrome. The researchers also observed a lower physical performance in individuals with Prader-Willi syndrome vs. healthy controls.
“Lower values of flexibility and muscle strength were shown in adult [Prader-Willi syndrome] patients compared to normal-weight and overweight/obese age-matched controls,” the researchers wrote. “In children and adolescents with [Prader-Willi syndrome] aged 8 to 16 years, lower levels of motor proficiency were also reported in comparison to children with obesity but without [Prader-Willi syndrome].”
Long-term exercise effects
Researchers assessed the effects of long-term exercise programs (most lasting 6 months) in 12 studies, including six studies conducted in children and adolescents, one study that included adolescents and adults, and five studies with adults only. Interventions included aerobic exercise, resistance training, home-based physical activity interventions or exercise combined with diet or a nutrition education program.
The researchers noted significant benefits after the exercise interventions, particularly a mean 15% improvement in cardiorespiratory fitness across studies as determined by heart rate analyses or predicted maximum oxygen uptake.
In nine studies that assessed the effects of long-term exercise on BMI and body composition, five studies demonstrated reductions in total body weight ranging from 2% to 12% and reductions in BMI ranging from 4% to 7%. However, the remaining studies showed no changes in BMI or body weight, according to researchers. In three of four studies that included a nutrition program component, researchers observed reductions in body weight and BMI.
The researchers reported that more controversy exists regarding training-induced changes in body composition in Prader-Willi syndrome. In six studies assessing muscle mass via DXA measurements or by calf skinfold thickness, three of six studies found a 2% to 5% increase in muscle mass after the exercise interventions, but a reduction or no change was reported in other studies. In five studies evaluating fat mass via the same measurements, only two noted reductions in fat mass, whereas the other three demonstrated no such changes, according to researchers.
“Further, the analysis of a key factor for improving body composition, that is, nutritional intake, showed a higher protein and lower carbohydrate intake in [Prader-Willi syndrome] patients compared to controls when including a nutritional education program for patients’ parents, and no differences were observed between [Prader-Willi syndrome] patients and controls in another report after 24 weeks of home-based [physical activity],” the researchers wrote.
There were no exercise-related adverse events reported during the study, according to researchers.
‘Important health benefits’
The researchers noted that samples sizes across most studies were low, as was the methodologic quality. Additionally, differences between studies in exercise protocols make it difficult to draw conclusions. However, because impaired physical fitness and obesity increase morbidity and mortality risk in Prader-Willi syndrome, the observed benefits are of “major relevance.”
“Additionally, the potential role of exercise interventions for improving movement coordination in children with this syndrome is important in light of the overall association between motor proficiency and [physical activity],” the researchers wrote. “Although more research is needed, physical exercise interventions might thus provide some important health benefits in people with [Prader-Willi syndrome].” – by Regina Schaffer
Disclosures: The authors report no relevant financial disclosures.
Perspective
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Theresa Strong, PhD
Exercise can be a challenge for all of us, but it can be particularly challenging for those with Prader-Willi syndrome, who typically exhibit hypotonia, poor motor planning, decreased muscle mass and reduced stamina. Even those individuals with a BMI in the normal range often have increased adiposity and reduced muscle mass compared with typical individuals. But exercise is critical in this population, as it is one of the few interventions available to combat obesity.
Thus, it is both reassuring and encouraging to families that the meta-analysis by Morales and colleagues suggests significant benefit from long-term exercise programs in this population, with no exercise-related adverse events. Since many of the studies included in this analysis were small and the protocols varied, it is not yet clear if certain regimens or programs are particularly effective in individuals with Prader-Willi syndrome, and this will be important to investigate moving forward. The full benefit of nutritional education or intervention has likely not yet been realized, and understanding optimal nutritional intake in this population is likewise an important goal. Nonetheless, the overall message that long-term exercise programs improve health, especially when coupled with nutritional education, should further incentivize families and health care providers to pursue efforts to encourage exercise and activity in their loved ones with Prader-Willi syndrome.
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