Donze Stephany H, de Weerd Al W, van den Bossche Renilde A S, Joosten Koen F M, Hokken-Koelega Anita C S
Dutch Growth Research Foundation, AH Rotterdam, Netherlands.
Department of Pediatrics, Subdivision of Endocrinology, Erasmus University Medical Center/Sophia Children's Hospital, GD Rotterdam, Netherlands.
J Clin Endocrinol Metab. 2019 Sep 1;104(9):3931-3938. doi: 10.1210/jc.2019-00391.
Sleep-related breathing disorders (SRBD) are common in people with Prader-Willi syndrome (PWS). Young adults with PWS benefit from GH continuation after attaining adult height by maintaining the improved body composition obtained during childhood. There are, no studies about the effects of GH on SRBD in young adults with PWS who were treated with GH during childhood.
Investigate the effects of GH vs placebo on SRBD in young adults with PWS who were treated with GH during childhood and had attained adult height.
Two-year, randomized, double-blind, placebo-controlled, crossover study in 27 young adults with PWS, stratified for sex and body mass index.
Dutch PWS Reference Center.
Crossover intervention with GH (0.67 mg/m2/d) and placebo, both over one year.
Apnea hypopnea index (AHI), obstructive apnea index (OAI), central apnea index (CAI), measured by polysomnography.
Compared with placebo, GH did not increase AHI, CAI, or OAI (P > 0.35). The effect of GH vs placebo was neither different between men and women, nor between patients with a deletion or maternal uniparental disomy/imprinting center defect. After two years, there was no difference in AHI, CAI, or OAI compared with baseline (P > 0.18). Two patients (7%) fulfilled the criteria of obstructive sleep apnea regardless of GH or placebo.
GH compared with placebo does not cause a substantial increase in AHI, CAI, or OAI in adults with PWS who were treated with GH during childhood and have attained adult height. Our findings are reassuring and prove that GH can be administered safely.
睡眠相关呼吸障碍（SRBD）在普拉德-威利综合征（PWS）患者中很常见。患有PWS的年轻成年人在达到成人身高后继续使用生长激素（GH），通过维持童年时期获得的改善的身体成分而受益。目前尚无关于GH对童年时期接受GH治疗且已达到成人身高的PWS年轻成年人SRBD影响的研究。
研究GH与安慰剂对童年时期接受GH治疗且已达到成人身高的PWS年轻成年人SRBD的影响。
对27名患有PWS的年轻成年人进行为期两年的随机、双盲、安慰剂对照、交叉研究，按性别和体重指数分层。
荷兰PWS参考中心。
GH（0.67 mg/m²/天）和安慰剂交叉干预，均为期一年。
通过多导睡眠图测量的呼吸暂停低通气指数（AHI）、阻塞性呼吸暂停指数（OAI）、中枢性呼吸暂停指数（CAI）。
与安慰剂相比，GH未增加AHI、CAI或OAI（P>0.35）。GH与安慰剂的效果在男性和女性之间以及有缺失或母系单亲二体/印记中心缺陷的患者之间均无差异。两年后，与基线相比，AHI、CAI或OAI无差异（P>0.18）。两名患者（7%）符合阻塞性睡眠呼吸暂停标准，无论使用GH还是安慰剂。
与安慰剂相比，GH不会使童年时期接受GH治疗且已达到成人身高的PWS成年人的AHI、CAI或OAI大幅增加。我们的研究结果令人放心，并证明GH可以安全使用。
