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Prader-Willi Syndrome

  • Julia Katz

摘要

Prader-Willi syndrome (PWS) is a multisystem neurodevelopmental disorder caused by the loss of function of paternally expressed genes on chromosome 15 located in the region 15q11.2–q13. Signs and symptoms of PWS in infancy include hypotonia and failure to thrive. In early childhood, severe hyperphagia, obesity, intellectual disability, and behavioral problems including tantrums, skin picking, anxiety, obsessions, compulsions, rigidity, and social impairment become more apparent. While evidence-based psychiatric and psychological treatment options are limited due to a paucity of rigorous trials, many psychotropic and behavioral interventions are used in current clinical practice. This chapter will present the most frequently used interventions and available data thus far, as well as important aspects of the syndrome for psychiatric prescribers to be aware of.