【摘 要】
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Prader-Willi syndrome (PWS) is the most common form of genetic obesity and it occurs in approximately 1 in 25,000 live births.PWS is characterized by neonatal hypotonia, feeding problems and failure t
【机 构】
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Autoimmune Endocrine Diseases Unit Bambino Gesù Children's Hospital-Research Institute Italy
【出 处】
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2013第三届内分泌与代谢大会暨2013第二届糖尿病大会
论文部分内容阅读
Prader-Willi syndrome (PWS) is the most common form of genetic obesity and it occurs in approximately 1 in 25,000 live births.PWS is characterized by neonatal hypotonia, feeding problems and failure to thrive in the neonatal period, followed by severe hyperphagia leading to morbid obesity.Short stature, dysmorphic features, mental retardation, scoliosis, behavioural and psychiatric problems sleep disturbances and hypogonadism are associated.Many of the clinical features are the consequence of a hypothalamic dysfunction.A reduced growth hormone (GH) secretion has been documented in the majority of children with PWS.GH therapy has been approved for these patients and it should be started very precociously.It promotes linear growth and final height and improves body composition, muscle strength, physical agility and behaviour.
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