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A short clinical overview of Prader–Willi syndrome
Author(s) -
McAllister C. J.,
Whittington J. E.
Publication year - 2011
Publication title -
clinical obesity
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.64
H-Index - 12
eISSN - 1758-8111
pISSN - 1758-8103
DOI - 10.1111/j.1758-8111.2011.00022.x
Subject(s) - overeating , medicine , imprinting (psychology) , obesity , genetic disorder , eating disorders , weight management , starvation , psychiatry , weight loss , endocrinology , genetics , pathology , disease , biology , gene
Summary Prader–Willi syndrome (PWS) is a multifaceted developmental disorder most commonly associated with extreme hyperphagia and life‐threatening obesity. PWS is a genetic disorder of imprinting with almost all cases occurring spontaneously. Behavioural and imaging studies have shown that obesity in PWS arises from overeating driven by a faulty satiety mechanism which manifests as an almost permanent state similar to starvation. With no available treatments, management of the eating behaviour is the only option and has two main strategies: restrict access to food and distract thoughts from food. In this mini review, which we have aimed at clinicians, we outline the main aspects of PWS including genetics, development of the eating behaviour and best practice approaches to management.