Table 1.
Significant studies on diet in patients with Prader–Willi syndrome
| Reference | Type of study | Subjects | Diet | Control diet | Main results |
|---|---|---|---|---|---|
| Miller et al. [85] | Longitudinal study | Sixty-three children with PWS, aged 2–10 years | Well-balanced, energy-restricted diet (30% fat, 45% carbohydrates and 25% protein, with at least 20 g of fiber/day) | Energy-restricted only diet (10–23% fat, 50–70% carbohydrates and 15–20% protein, with 12 g or less of fiber/day) | Those who followed the well-balanced, energy-restricted diet had lower body fat (19.8% vs 41.9%; p < 0.001) and weight management (BMI SD score 0.3 vs 2.23; p < 0.001) than those who followed the energy intake recommendations but did not alter the macronutrient composition of the diet |
| Irizarry et al. [86] | Longitudinal crossover study | Eight children with PWS, aged 9–18 years | Low-carbohydrate high-fat diet (15% carbohydrate, 65% fat, and 20% protein) | Low-fat high-carbohydrate diet (65% carbohydrate, 15% fat, and 20% protein) | Compared to low-fat high-carbohydrate diet low-carbohydrate high-fat diet: reduced postprandial insulin concentrations (p = 0.02), increased fasting GLP-1 and GIP concentrations and increased postprandial GLP-1 (p < 0.02); reduced fasting ghrelin/GLP‐1 ratio (p = 0.0078); increased FFA and fatty acid oxidation (p < 0.001); reduced fasting TG and TG/HDL ratio (p < 0.01); increased concentrations of branch chain amino acids (p < 0.01) |
| Felix et al. [87] | Clinical feasibility study | Four children with PWS, aged 6–12 years | Modified Atkin Diet (10–15 g of net carbohydrate, protein and fat on individual basis) | n/a | One patient lost 2.9 kg; the others maintained their weight. Positive effects on hyperphagia as well as on behaviors were reported by parents and relatives |
| Bedogni et al. [88] | Retrospective cohort study | Forty-five adults with PWS and obesity, aged 22–30 years | Hypocaloric Mediterranean diet | n/a | The mean weight change was -3.6 ( p = 0.08) Kg at 3 years and − 4.6 (p = 0.02) Kg at 6 years, and that of BMI was -1.7 (p = 0.06) kg/m2 at 3 years and -2.1 (p = 0.02) kg/m2 at 6 years. A decrease of about 2% in fat mass per unit of body mass was observed. A possibly clinically relevant decrease in total and LDL cholesterol was also observed |