Prader-Willi Syndrome
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چکیده
Prader-Willi syndrome is a neurogenetic disorder characterized by hypotonia and feeding difficulties in infancy, followed by hyperphagia, hypogonadism, mental retardation, and short stature. It was the first recognized microdeletion syndrome identified with high-resolution chromosome analysis, the first recognized human genomic imprinting disorder, and the first recognized disorder resulting from uniparental disomy. The incidence of Prader-Willi syndrome is approximately 1/10,000 to 1/15,000 individuals.
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Dental Management of Patients with Prader Willi Syndrome
Prader–Willi syndrome (PWS) is a genetic disorder which occurs with a frequency of about one in 10,000–30,000 live newborns. Both males and females, and all races are equally affected. PWS is a complex disorder with multiple disabilities, and the main defect is found in the hypothalamus. Child with PWS at the age between 2 and 3 years becomes constantly hungry and if the diet is not controlled,...
متن کاملStrategies and correlates of jigsaw puzzle and visuospatial performance by persons with Prader-Willi syndrome.
Some individuals with Prader-Willi syndrome exhibit strengths in solving jigsaw puzzles. We compared visuospatial ability and jigsaw puzzle performance and strategies of 26 persons with Prader-Willi syndrome and 26 MA-matched typically developing controls. Individuals with Prader-Willi syndrome relied on piece shape. Those in the control group used a different, picture-focused strategy. Individ...
متن کاملExperience of severe desaturation during anesthetic induction period in an obese adult patient with Prader-Willi syndrome -A case report-
Prader-Willi syndrome is characterized by infantile hypotonia, childhood-onset obesity, short stature, mental retardation, hyperphagia, hypogonadism. After infantile hypotonia phase, patient is prone to morbid obesity due to hyperphagia. Complications associated with morbid obesity are recognized as the main risk factors for death the lifespan of patients with Prader-Willi syndrome. We experien...
متن کاملAre jigsaw puzzle skills 'spared' in persons with Prader-Willi syndrome?
BACKGROUND This three-part study examines previous clinical impressions that people with Prader-Willi syndrome have unusual jigsaw puzzle and word search skills. RESULTS Children with Prader-Willi syndrome showed relative strengths on standardized visual-spatial tasks (Object Assembly, Triangles, VMI) in that their scores were significantly higher than age- and IQ-matched peers with mixed men...
متن کاملTryptophan for the treatment of excessive daytime sleepiness in Prader-Willi syndrome.
An 8-year old girl with Prader-Willi Syndrome presenting with excessive daytime sleepiness improved following treatment with tryptophan; possibly by consolidation of her fragmented sleep. Improvement was recorded on a follow-up sleep study, one year after initiating treatment with tryptophan. We conclude that tryptophan may be an useful medication for excessive sleepiness in children with Prade...
متن کاملPsychiatric Comorbidity in Prader-Willi Syndrome A Case Series
Prader-Willi syndrome is a genetic disorder. Psychiatric comorbidity can occur in persons with Prader-Willi syndrome varying from emotional and behavioural disorders characterised by impulsiveness, temper tantrums, with occurrence of other psychiatric disorders in the form of obsessive compulsive disorder, mood disorders and psychotic illnesses. We present 3 patients with Prader-Willi syndrome ...
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