Prader–Willi syndrome
RTL Couper,
RTL Couper
Department of Paediatrics, University of Adelaide, Women’s and Children’s Hospital, North Adelaide, South Australia, Australia
Search for more papers by this authorRTL Couper,
RTL Couper
Department of Paediatrics, University of Adelaide, Women’s and Children’s Hospital, North Adelaide, South Australia, Australia
Search for more papers by this author
Dr Rtl Couper Senior Lecturer, University of Adelaide Department of Paediatrics, Women’s and Children’s Hospital, 72 King William Road, North Adelaide, SA 5006, Australia. Fax: (08) 8204 7031; email: [email protected]
Abstract
Prader–Willi syndrome is a multi system disorder characterized by neonatal hypotonia, later obesity, hyperphagia and mental retardation. It occurs sporadically, either as a result of microdeletion of chromosome 15p (70%) or as a result of maternal disomy of chromosome 15 (30%). The major problems encountered by parents are those of hyperphagia, food-seeking and obesity, and conduct disorder, particularly tantrums or oppositional behaviour.
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