普拉德-威利综合征成年患者的生长激素治疗
Growth Hormone Therapy in Adults with Prader-Willi Syndrome.
作者信息
Vogt Karen S, Emerick Jill E
机构信息
Division of Endocrinology, Department of Pediatrics, Walter Reed National Military Medical Center, Bethesda, 20889, MD, USA.
出版信息
Diseases. 2015 Apr 16;3(2):56-67. doi: 10.3390/diseases3020056.
Abstract
Prader-Willi syndrome (PWS) is characterized by hyperphagia, obesity if food intake is not strictly controlled, abnormal body composition with decreased lean body mass and increased fat mass, decreased basal metabolic rate, short stature, low muscle tone, cognitive disability, and hypogonadism. In addition to improvements in linear growth, the benefits of growth hormone therapy on body composition and motor function in children with PWS are well established. Evidence is now emerging on the benefits of growth hormone therapy in adults with PWS. This review summarizes the current literature on growth hormone status and the use of growth hormone therapy in adults with PWS. The benefits of growth hormone therapy on body composition, muscle strength, exercise capacity, certain measures of sleep-disordered breathing, metabolic parameters, quality of life, and cognition are covered in detail along with potential adverse effects and guidelines for initiating and monitoring therapy.
摘要
普拉德-威利综合征（PWS）的特征包括食欲亢进、若不严格控制食物摄入量则会出现肥胖、身体成分异常（瘦体重减少而脂肪量增加）、基础代谢率降低、身材矮小、肌张力低下、认知障碍和性腺功能减退。除了线性生长得到改善外，生长激素治疗对PWS患儿身体成分和运动功能的益处已得到充分证实。目前，关于生长激素治疗对成年PWS患者的益处也有了新的证据。本综述总结了目前关于成年PWS患者生长激素状态及生长激素治疗应用的文献。详细阐述了生长激素治疗对身体成分、肌肉力量、运动能力、某些睡眠呼吸紊乱指标、代谢参数、生活质量和认知的益处，以及潜在的不良反应和启动及监测治疗的指南。
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