PSYC 391 Lecture 9: Psyc 391 - Week 9 Prader Willi Syndrome

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A rare genetic disorder (1 in 15,000 live births) Prader willi syndrome: seven genes (or some subset thereof on chromosome 15 (q 11-15) deleted or unexpressed, normally one copy is expressed and the other silent (maternal usually silent, father"s is (cid:373)utated a(cid:374)d (cid:373)other"s is sile(cid:374)t i(cid:374) pws. If (cid:373)other is the pro(cid:271)le(cid:373)ati(cid:272) o(cid:374)e results i(cid:374) a(cid:374)gel(cid:373)a(cid:374)"s sy(cid:374)dro(cid:373)e. Dysmorphic features (child and adult: almond shaped eyes, narrow temples, strabismus (cross-eyed, then upper lip. Inability to satiate-excessive food seeking: mild intellectual impairments, skin picking-compulsive behaviour, short-stature, sexually immature puberty delayed or incomplete. Infancy: failure to thrive, maybe related to sever hypotnia, childhood, tendency toward obesity, overeating, preoccupation with food, adulthood, risk of obesity increases, co-morbid psychiatric problems, mortality related to health risks. Subtypes: possible subtypes, obesity and obsession with food, obsession with food as a topic of interest but obesity can be controlled, drugs and surgical treatments to suppress hunger are not effective.

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