[To know more about the Prader-Willi syndrome. Multidisciplinary support].
نویسندگان
چکیده
Prader-Willi syndrome, induced by a loss of function of paternal genes in the subcentrometric region of the chromosome 15 (q11.2q13), is a complex neurodevelopmental disorder with characteristic obesity resulting from hyperphagia. In addition behavioural disturbancies with obsessive-compulsive features, aggression, temper tantrums included, are relatively frequently seen and they often require psychiatric intervention. In this part of the paper we reviewed the recent data of behavioural phenotype the correlations of phenotype-genotype and possibilities of the multidisciplinary support for the affected persons and theirs families.
منابع مشابه
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 کامل[To know more about the Prader-Willi syndrome. Diagnosis].
Prader-Willi syndrome, induced by a function changes of paternal genes in the subcentrometric region of the chromosome 15 (q11.2q13), is the most common genetic cause of obesity resulting from hyperphagia. Behavioural disturbancies with compulsions in which psychiatric interventions are necessary, are relatively frequently seen. In this paper we reviewed the recent data of the clinical diagnosi...
متن کاملMultidisciplinary care in the intensive care unit for a patient with Prader-Willi syndrome: a dental approach.
Prader-Willi syndrome is a genetic neurobehavioral disease affecting children's development and resulting in obesity, reduced height, hypotonia, endocrine disorders and cognitive deficits, which may impair oral integrity. This study aims to report on a case involving a white male 15-year-old patient with Prader-Willi syndrome whose oral examination revealed bacterial plaque, gingivitis, poor oc...
متن کاملPrader-Willi Syndrome: Clinical Aspects
Prader-Willi Syndrome (PWS) is a complex multisystem genetic disorder that shows great variability, with changing clinical features during a patient's life. The syndrome is due to the loss of expression of several genes encoded on the proximal long arm of chromosome 15 (15q11.2-q13). The complex phenotype is most probably caused by a hypothalamic dysfunction that is responsible for hormonal dys...
متن کاملThree Siblings with Prader-Willi Syndrome: Brief Review of Sleep and Prader-Willi Syndrome
Prader-Willi syndrome (PWS) is a genetic disorder characterized by short stature, mental retardation, hypotonia, functionally deficient gonads, and uncontrolled appetite leading to extreme obesity at an early age. Patients with this condition require multidisciplinary medical care, which facilitates a significant improvement in quality of life. PWS is the first human disorder to be attributed t...
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ورودعنوان ژورنال:
- Psychiatria polska
دوره 43 2 شماره
صفحات -
تاریخ انتشار 2009