Growth hormone improves body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome: A controlled study
Growth hormone improves body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome: A controlled study
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DOI：
10.1016/s0022-3476(99)70418-x
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发表时间：
1999-02-01
影响因子：
5.1
通讯作者：
Allen, DB
中科院分区：
文献类型：
--
作者：
Carrel, AL;Myers, SE;Allen, DB
背景资料：患有Prader-Willi综合征（PWS）的儿童的肥胖和肌张力减退伴随着异常的身体组成和类似于生长发育缺陷状态的能量消耗减少。PWS的下丘脑功能障碍通常包括生长激素（GH）分泌减少，这表明外源性GH治疗可能具有治疗作用。目的和方法：经过6个月的观察，以确定基线生长率，并与使用12个月的随机对照研究设计，生长激素治疗的影响在54名PWS儿童中评估了（1 mg/m2/d）对生长、身体成分、力量和敏捷性、肺功能、静息能量消耗（REE）和脂肪利用的影响（n = 35治疗组和n = 19对照组）。采用双能X线吸收法测量体脂百分比和骨密度。间接量热法被用来确定REE和计算respiratory efficients.Results：刺激水平的CH在可乐定测试的反应是低的，在所有患者（峰值，2.0 ng/mL）。12个月后，CH治疗的受试者显示身高速度Z评分显著增加（平均值，-1.0 +/- 1.7至4.6 +/- 2.9; P < .001），体脂百分比降低（平均46.3% +/- 8.4%至38.3% +/- 10.7%; P <0.001），并改善呼吸肌功能、体力和敏捷性（仰卧起坐、举重、跑步速度和协调性）。GH治疗期间呼吸衰竭发生显著下降（0.81 ~ 0.77 P <0.001），但总REE未发生变化。结论：CH治疗PWS儿童加速生长，降低体脂百分比，增加脂肪氧化，但总REE未显著增加。呼吸肌力量，体力和敏捷性也有所改善，这表明GH治疗可能有助于减少PWS儿童所经历的一些身体残疾。
Background: Obesity and hypotonia in children with Prader-Willi syndrome (PWS) are accompanied by abnormal body composition and diminished energy expenditure resembling a growth hormone-deficient state. Hypothalamic dysfunction in PWS often includes decreased growth hormone (GH) secretion, suggesting a possible therapeutic role for exogenous GH treatment.Objectives and methods: After 6 months of observation to determine baseline growth rate, and with the use of a 12-month randomized controlled study design, the effects of GH treatment (1 mg/m(2)/d) on growth, body composition, strength and agility, pulmonary function, resting energy expenditure (REE), and fat utilization were assessed in 54 children with PWS (n = 35 treatment and n = 19 control). Percent body fat and bone mineral density were measured by dual x-ray absorptiometry. Indirect calorimetry was used to determine REE and to calculate respiratory quotients.Results: Stimulated levels of CH in response to clonidine testing were low in all patients (peak, 2.0 ng/mL). After 12 months, CH-treated subjects showed significantly increased height velocity Z scores (mean, -1.0 +/- 1.7 to 4.6 +/- 2.9; P < .001), decreased percent body fat (mean, 46.3% +/- 8.4% to 38.3% +/- 10.7%; P < .001), and improved respiratory muscle function, physical strength, and agility (sit-ups, weight-lifts, running speed, and coordination). A significant decline in respiratory quotients occurred during GH therapy (0.81 to 0.77 P < .001), but total REE did not change.Conclusions: CH treatment of children with PWS accelerated growth, decreased percent body fat, and increased fat oxidation but did not significantly increase total REE. Improvements in respiratory muscle strength, physical strength, and agility also occurred, suggesting that GH treatment may have value in reducing some physical disabilities experienced by children with PWS.