<p>Dietary Management for Adolescents with Prader–Willi Syndrome</p>

Abstract

Prader-Willi syndrome (PWS) is a complex, multisystem neurodevelopmental disorder affecting approximately 1 in 25,000 live births. PWS is caused by absence of expression of paternally inherited imprinted genes on chromosome 15q11-q13. The syndrome typically occurs due to one of three genetic mechanisms: paternal deletion of involved genes, maternal uniparental disomy, or imprinting center defects. These genetic anomalies lead to well-described clinical phenotype that includes hypotonia, hypothalamic dysfunction, social and behavioral issues, life-threatening hyperphagia, and elevated probability of obesity. Adolescents with PWS are at the highest risk for development of life-threatening obesity due to increased access to food, decreased physical activity, and hyperphagia. Currently, the only treatment for the hyperphagia is environmental control, including locked kitchens and continuous supervision of the affected individual. Caloric intake must be restricted to prevent obesity, which subsequently increases the hunger drive even more. Research and clinical practice have demonstrated that increasing physical activity along with insuring a wellbalanced, nutritionally dense diet can improve overall weight control in adolescents with PWS.

Long‐term weight control in adults with Prader‐Willi syndrome living in residential hostels

“…Typical daily caloric intakes range from 800 to 1,500 kcal, with the higher amount of daily calories offered to those who maintain a BMI below 23 kg/m 2 . Individual caloric requirements are calculated according to height as recommended by current guidelines, namely 7–8 kcal/cm for weight loss and 10–14 kcal/cm for weight maintenance (Hoffman et al, 1992; Miller & Tan, 2020; Pereira et al, 2009). Typical diet recommendations include 40%–45% carbohydrates, 25%–30% protein, and 30% fats.…”

“…The study population includes 34 individuals living in one of two hostels specifically dedicated for the care of individuals with PWS and 23 living at home with their families (Table 3) Typical daily caloric intakes range from 800 to 1,500 kcal, with the higher amount of daily calories offered to those who maintain a BMI below 23 kg/m 2 . Individual caloric requirements are calculated according to height as recommended by current guidelines, namely 7-8 kcal/cm for weight loss and 10-14 kcal/cm for weight maintenance (Hoffman et al, 1992;Miller & Tan, 2020;Pereira et al, 2009).…”

Prader–Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach

“…A relevant aspect in the management of the patients with PWS is the adherence to a diet starting from the first months of life to favor a regular body growth and, subsequently, to cope with the incipient hyperphagia and to prevent or treat the excess of weight throughout the life span [6,7,89]. Therefore, the nutritionist represents together with the endocrinologist the key figures in the management of patients with PWS, since a close nutritional follow-up, adapted to the patient's life stages and in agreement with the caregivers is essential.…”

Body weight, behaviours of concern, and social contact in adults and adolescents with Prader-Willi syndrome in full-time care services: Findings from pooled international archival data

“…Such meticulous control of the food environment, such as locked food access and continuous supervision, amounts to the only ‘treatment’ or ‘management’ currently available for hyperphagia [ 9 ]. When describing their own aspirations, people with PWS typically identify control of the food environment as being fundamental to the type of care they wish to receive [ 10 ].…”