腺切除术成功治疗普拉德-威利综合征儿童阻塞性睡眠呼吸暂停
Ross Rosen1, Jamil Hayden2, Abdul Saltagi2
1Department of Otolaryngology-Head and Neck Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.
International journal of pediatric otorhinolaryngology
|March 16, 2025
概括
腺切除术在治疗普拉德-威利综合征 (PWS) 患者的儿科阻塞性睡眠呼吸暂停 (OSA) 方面不太成功. 建议对患有OSA的PWS患者提供多学科护理.
科学领域:
- 儿科遗传学 儿科遗传学
- 睡眠医学 睡眠医学
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
背景情况:
- 普拉德-威利综合征 (PWS) 是一种罕见的遗传疾病,与儿科阻塞性睡眠呼吸暂停 (OSA) 的风险增加有关.
- 在PWS患者中,复杂的病理生理学,包括肥胖和面异常,可能会使OSA治疗复杂化.
- 标准的第一线治疗,腺切除术,在这个人群中可能只有有限的成功.
研究的目的:
- 评估腺切除术在治疗患有普拉德-威利综合征 (PWS) 的儿童阻塞性睡眠呼吸暂停 (OSA) 的有效性.
- 为了比较PWS患者和对照组之间的手术成功率.
- 确定与PWS患者OSA风险增加相关的医疗干预措施.
主要方法:
- 在TrinetX数据库的追溯查询中,确定了患有PWS和OSA诊断的儿科患者 (≤18岁).
- 分析了手术干预 (腺切除术,切除术,腺切除术) 的情况.
- 主要终点是手术后6个月OSA诊断的解决;没有PWS的对照被用于比较.
主要成果:
- 在2163名PWS患者中,1035人患有OSA. 在PWS患者中,手术成功率为39.0%,明显低于对照组 (79.6%,p < 0.001).
- 即使与人口统计和肥胖相匹配,成功率仍然较低 (36.8%与82.1%,p < 0.001).
- 使用生长激素和丸激素与增加的OSA风险有关 (RR分别为1.43和1.39,p <0.001).
结论:
- 腺切除术表明,在治疗PWS患者的儿科OSA中,成功率明显较低.
- 患有OSA的PWS患者可能会从多学科的方法中受益,以管理他们的病情.
- 需要进一步的研究,以建立最佳的护理实践PWS患者与OSA.
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