Positive Airway Pressure For The Treatment Of The Obstructive Sleep Apnea Syndrome In Children With Down Syndrome

摘要 唐氏综合征(DS)患者由于头面部特征(面中部)易患OSAS 和研究表明，OSAS在这一人群中的患病率明显 与一般发育中的儿童相比有所增加。扁桃体切除术(AT)被认为是一线手术 儿童阻塞性睡眠呼吸暂停综合征的治疗。然而，OSAS在AT后仅能解决一部分DS儿童。事实上, 许多患有DS的儿童由于持续存在而被转介进行气道正压(PAP)治疗 在AT之后，OSAS和PAP似乎是与DS生活在一起的一个重要特征。帕普一直是 被证明在治疗OSAS和改善与OSAS相关的神经行为症状方面非常有效，例如 如生活质量、行为、情绪、日间困倦和学校表现。然而，PAP作为一种治疗方法 因为OSAS在患有DS的儿童中还没有得到很好的研究。因此，我们建议利用健康的睡眠 针对唐氏综合症儿童(HELP-DS，U01HL125295-S1和U01HL123507-S1)的基础设施和 在两个帮助-DS网站(费城和辛辛那提)以及迈阿密大学进行的随机调查 对照试验，测试为期6个月的强化行为干预(INT)以改善PAP依从性与标准 临床护理(CON)。患有DS和OSAS的参与者，年龄6-17.9岁，转介PAP启动 治疗阻塞性睡眠呼吸暂停综合征将包括在内。他们将接受标准化的生活质量、行为、 关注度、与家庭相关的结果、PAP依从性以及基线、6和12的卫生保健利用率 月份。这项严谨的设计和全面的研究将解决最初存在的不确定因素 通过解决关键问题对患有DS和OSAS的儿童进行PAP治疗的管理方法：a) 确定INT与CON在促进PAP黏附方面的有效性，b)阐明哪些因素介导或 DS和OSAS儿童对PAP的适度依从性，c)决定使用PAP对 神经行为和与家庭相关的结果，以及生活质量。

Abstract Individuals with Down syndrome (DS) are predisposed to OSAS due to craniofacial features (midface hypoplasia, glossoptosis) and studies have shown that the prevalence of OSAS in this population is markedly increased compared to that of typically developing children. Adenotonsillectomy (AT) is considered first-line treatment for childhood OSAS. However, OSAS resolves in only a portion of children with DS after AT. In fact, many children with DS are referred for positive airway pressure (PAP) therapy initiation due to persistent OSAS after AT, and PAP appears to be an important feature of the experience of living with DS. PAP has been shown to be highly effective at treating OSAS and improve OSAS-associated neurobehavioral symptoms, such as quality of life, behavior, mood, daytime sleepiness and school performance. However, PAP as a treatment for OSAS has not been well-studied in children with DS. Therefore, we propose to leverage the HEalthy SLeeP for Children with Down Syndrome (HELP-DS, U01HL125295-S1 and U01HL123507-S1) infrastructure and conduct in two HELP-DS sites (Philadelphia and Cincinnati) plus the University of Miami, a randomized controlled trial to test a 6-month intensive behavioral intervention (INT) to improve PAP adherence vs standard clinical care (CON). Participants with DS and OSAS, aged 6-17.9 years, referred to PAP initiation for the treatment of OSAS will be included. They will undergo standardized evaluations of quality of life, behavior, attention, and family-relevant outcomes, PAP adherence, and health care utilization at baseline, 6, and 12 months. This rigorous design and comprehensive study will resolve existing uncertainties on initial management approaches for children with DS and OSAS treated with PAP by addressing critical issues: a) determine the efficacy of INT vs CON in promoting PAP adherence, b) elucidate which factors mediate or moderate adherence to PAP in children with DS and OSAS, c) determine the effect of PAP use on neurobehavioral and family-relevant outcomes, and quality of life.