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Management of obesity in Prader–Willi syndrome

Nature Clinical Practice Endocrinology & Metabolism volume 2, pages 592–593 (2006)Cite this article

Prader–Willi syndrome (PWS) is considered the most common genetic cause of morbid obesity and has a birth incidence of 1 in 25,000–30,000.1,2 PWS is characterized by infantile hypotonia, failure to thrive, hypogonadism, small hands and feet, short stature, intellectual challenges, behavioral problems, food foraging, and hyperphagia leading to early childhood obesity.1 Gastric rupture is also a reported complication in PWS.3 Individuals with PWS often have a significant preoccupation with food, experience constant hunger, and display food-seeking behavior. During the first stage of PWS (onset at birth), poor suckling and feeding difficulties require specific dietary intervention for weight gain; however, during the second stage (onset 2–4 years of age) an insatiable appetite develops, which is followed by rapid weight gain. Without intervention, obesity occurs, and this is the most significant health problem of PWS—obesity and its complications can potentially be life-threatening. In this Viewpoint, I will discuss strategies to treat obesity in this complex disorder, outline medical and surgical options, then concentrate on nutritional and behavioral management of energy expenditure.

Energy expenditure components are estimated by a variety of methods but include resting metabolism (accounting for ∼60% of energy expenditure), thermic effects of food (energy required to consume and digest food; ∼10% of energy expenditure), and physical activity (spontaneous or planned exercise). Growth hormone deficiency is recognized as a key component of PWS and replacement therapy at a young age can have beneficial effects on height and body composition by expanding lean mass. The increase in lean mass raises the metabolic rate and increases physical activity and energy expenditure, which leads to a decreased fat mass and weight loss. Similar improvements in body composition and quality of life have been noted in adults with PWS who were treated with growth hormone.4

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  1. the William R Brown/Missouri Chair in Medical Genetics, Chief of the Section of Medical Genetics and Molecular Medicine, Children's Mercy Hospitals and Clinics, and Professor of Pediatrics, University of Missouri–Kansas City School of Medicine, Kansas City, MO, USA

    Merlin G Butler

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  1. Merlin G Butler
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Correspondence to Merlin G Butler.

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Butler, M. Management of obesity in Prader–Willi syndrome. Nat Rev Endocrinol 2, 592–593 (2006). https://doi.org/10.1038/ncpendmet0320

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