Research Notes: Diets for Prader-Willi Syndrome

See also:

• Modified protein-sparing fasting
• Ketogenic diets

Nutr Rev. 1991 Aug.
Parenteral protein-sparing modified fast in an obese adolescent with Prader-Willi syndrome.
Collier SB, Walker WA.
Massachusetts General Hospital, Harvard Medical School, Boston.

A ventilator-dependent, extremely obese adolescent patient with Prader-Willi syndrome was nutritionally managed using a parenteral, protein-sparing modified fast. A solution containing only amino acids, electrolytes, vitamins, and macro- and micro-minerals helped create a ketotic state and facilitate weaning from the ventilator. This case demonstrates an unusual strategy for achieving weight loss and the challenge to provide adequate protein intake while limiting fluid intake.

N Engl J Med. 1977 Apr 7.
Metabolic aspects of a protein-sparing modified fast in the dietary management of Prader-Willi obesity.
Bistrian BR, Blackburn GL, Stanbury JB.

Four adolescents or young adults with the Prader-Willi syndrome (hypotonia, mental retardation, hypogonadism and obesity) received a protein-sparing modified fast consisting of 1.5 g of meat protein per kilogram of ideal body weight and meeting vitamin, mineral and fluid requirements. Evaluation of nitrogen and energy metabolism revealed the development of starvation ketosis and a positive nitrogen balance. Serial whole-body potassium measurements in two patients confirmed preservation of lean tissue despite continuing loss of weight. Clinical diabetes mellitus in two subjects was rapidly ameliorated by the regimen. Short-term weight loss greater than 18 kg occurred in three of the four subjects, and reduced weight persisted during observation periods of 26 to 44 months. This degree of outpatient diet adherence by mentally deficient subjects, who do not normally experience satiety, suggests that hunger is eliminated or at least reduced by modified, protein-sparing fasting.

J Am Diet Assoc. 1976 Apr.
Out-patient dietary management in the Prader-Willi syndrome.
Coplin SS, Hine J, Gormican A.

Dietary adherence of eight children with the Prader-Willi syndrome was studied in the home environment. Weight changes were recorded at two-week intervals, and measured two-week dietary records were completed twice during the study by the parents of seven of the children. An eighth child was similarly followed for three months, and one dietary record was obtained. Calories, protein, fat, and carbohydrate contents were calculated, and related to recorded weight changes to determine which diets were most practical in controlling weight gain. Caloric requirements of children with the Prader-Willi syndrome appear to be much lower than those of healthy, active children of comparable ages. Age, degree of obesity, familial relationships, and probably, composition of the diet influenced the effectiveness of a given diet. Each family designed a diet which took into consideration the family's eating habits, as well as the needs of the Prader-Willi child. Frequent contact with the dietitian enabled each family to try new food preparation ideas. The effectiveness and acceptability of a low caloric, very low-carbohydrate diet should be tested over long periods in Prader-Willi children whose obesity is being managed in a non-institution setting.