A reduced‐energy intake, well‐balanced diet improves weight control in children with Prader‐Willi syndrome

Abstract

Background Children with Prader-Willi syndrome (PWS) have a predictable pattern of weight gain, with obesity beginning in early childhood and worsening as they get older and hyperphagia increases. Data on the most effective dietary modifications are scant and primarily anecdotal. As part of a longitudinal study investigating the natural history of PWS, we evaluated the effect of a well-balanced, energy-restricted diet on body composition and weight in young children with PWS. Methods Sixty-three children, aged 2–10 years, with genetically proven PWS participated in the present study. These children had measurements of body composition by dual-energy X-ray absorptiometry and resting energy expenditure (REE), as well as a 3-day diet history analysis both before and after intervention. Energy calculations were based on the individual's REE, with the recommendation that the macronutrients of the diet consist of 30% fat, 45% carbohydrates and 25% protein, with at least 20 g of fibre per day. Results Thirty-three families adhered to our dietary recommendations for both energy intake and macronutrient distribution. Those 33 children had lower body fat (19.8% versus 41.9%; P < 0.001) and weight management (body mass index SD score 0.3 versus 2.23; P < 0.001) than those whose parents followed the energy intake recommendations but did not alter the macronutrient composition of the diet. Those who followed our recommendations also had a lower respiratory quotient (0.84 versus 0.95; P = 0.002). Conclusions Our recommendation for an energy-restricted diet with a well-balanced macronutrient composition and fibre intake improves both weight and body composition in children with PWS compared to a simple energy-restricted diet.

Endocrine manifestations and management of Prader-Willi syndrome

“…Interestingly, parents rarely reported that their children experienced hyperphagia or food cravings, suggesting a learned behavioral component of the hyperphagia [18]. A diet with carbohydrate content as low as 45% of total calories may have more favorable effects on body composition and fat utilization [19]. Prescribed daily physical activity is also important and contributes to improved body composition and REE [20].…”

The prevalence of metabolic risk factors of atherosclerotic cardiovascular disease in Williams syndrome, Prader–Willi syndrome, and Down syndrome

“…The large proportion with abdominal obesity that was observed supports the need for lifelong strategies to reduce morbid obesity in the group with PWS. Controlled energy-restricted diets are essential, and macronutrient-balanced diets combined with rich intakes of fibre have shown better body weight outcomes compared to simple energy-restricted diets (Miller, Lynn, Shuster, & Driscoll, 2013).…”

Prader-Willi syndrome and hyperphagia: an update

“…The authors concluded that: activities have to be widely mixed with appropriate rest times, since fatigue and stress can provoke temper tantrums; definitive progress was achieved following the introduction of psychomotor activity and music therapy; groups of more than ten patients would be hard to manage due to the complicated social dynamics; and activities were performed at a lower speed than normal and with frequent interruptions (Grolla et al, 2011). Miller et al (2013) also recommended daily moderate exercise to the 63 individuals with PWS who participated in their study, but paid particular attention to the effects of a balanced diet in PWS. They calculated individuals 0 resting energy expenditure and recommended 33 children to consume a well-balanced diet consisting of 30 per cent fat, 45 per cent carbohydrates and 25 per cent protein, and the remaining 30 participants were placed on a low-fat restricted energy diet.…”