Short-term effects of growth hormone on sleep abnormalities in Prader-Willi syndrome

Short-term effects of growth hormone on sleep abnormalities in Prader-Willi syndrome
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DOI:
10.1210/jc.2005-1279
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发表时间:
2006-02-01
影响因子:
5.8
通讯作者:
Wagner, M
Wagner, M
中科院分区:
医学2区
文献类型:
--
作者:
Miller, J;Silverstein, J;Wagner, M

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背景:GH于2000年被批准用于Prader-Willi综合征(PWS)。在开始GH治疗后不久PWS患者的死亡率提示了对GH恶化睡眠呼吸暂停的关注。目的:我们试图确定GH是否影响PWS患者的睡眠呼吸暂停。设计:25例PWS患者在基线和开始GH治疗后6周进行了夜间多导睡眠图(PSA)。设置:该研究在睡眠实验室中使用标准化程序进行。患者:研究的患者有遗传学证实的PWS.Main Outcome Measures:分析PSA结果的中枢性和阻塞性呼吸暂停/低通气事件的频率和严重程度以及总呼吸暂停/低通气指数。作为一个组,GH改善呼吸暂停/低通气指数平均每小时1.2起事件(P = 0.02),中心事件平均每小时1.7起事件(P < 0.001)。14例患者的阻塞性事件改善,平均每小时1.7起事件。6例患者在GH治疗期间发生阻塞性事件恶化。其中4例患者在第二次PSA时有上呼吸道感染,耳鼻咽喉评价时有扁桃体/腺样体肥大。两个高血清IGF-I水平的患者增加了阻塞events.Conclusions:我们的PWS患者有改善后,短期GH治疗,但32%的睡眠障碍恶化。开始GH后不久,一部分PWS患者在此脆弱窗口期内面临风险。因为很难预测谁会与GH恶化,PWS患者应该有PSA开始GH之前和之后,并应监测睡眠呼吸暂停与上呼吸道感染。如果睡眠呼吸暂停综合征需要GH治疗,则需要进行耳鼻喉科评估。应监测IGF-I水平,目标是生理水平。
Context: GH was approved for Prader-Willi Syndrome (PWS) in 2000. Fatalities in individuals with PWS soon after beginning GH treatment prompted concern about GH worsening sleep apnea.Objective: We sought to determine whether GH affects sleep apnea in individuals with PWS.Design: Twenty-five patients with PWS had overnight polysomnography (PSA) at baseline and 6 wk after starting GH.Setting: The study was conducted in a sleep lab using a standardized procedure.Patients: The patients studied had genetically confirmed PWS.Main Outcome Measures: PSA results were analyzed for frequency and severity of central and obstructive apnea/hypopnea events and total apnea/hypopnea index.Results: As a group, GH improved apnea/hypopnea index by a mean of 1.2 events per hour (P = 0.02) and central events by a median of 1.7 events per hour (P < 0.001). Fourteen patients had improvement in obstructive events by a mean of 1.7 events per hour. Six patients had worsening of obstructive events on GH. Four of these patients had upper respiratory tract infections at the time of the second PSA and had tonsil/adenoid hypertrophy on otorhinolaryngological evaluation. Two patients with high serum IGF-I levels had increased obstructive events.Conclusions: Most of our PWS patients had improvement after short-term GH treatment, but 32% had worsening of sleep disturbance. A subset of PWS patients are at risk during this window of vulnerability shortly after initiation of GH. Because it is difficult to predict who will worsen with GH, patients with PWS should have PSA before and after starting GH and should be monitored for sleep apnea with upper respiratory tract infections. Otorhinolaryngological evaluation is warranted if sleep apnea worsens on GH. IGF-I levels should be monitored, with the goal being physiological levels.