Mechanisms of obstructive sleep apnea syndrome in infants with micrognathia

项目总结/摘要 我是一名儿科肺病学家，接受过睡眠医学的奖学金培训，有临床和 儿童阻塞性睡眠呼吸暂停综合征（OSAS）的转化研究。我的研究集中在 颅面疾病儿童阻塞性睡眠呼吸暂停综合征的病理生理、后果和治疗。拟议 一个项目旨在确定OSAS的机制，在婴儿与小颌，一组儿童与 下颌发育不全阻塞性睡眠呼吸暂停综合征在儿童中很常见，并与生长障碍和各种 认知和心血管后遗症。患有小颌畸形的婴儿是OSAS的高危人群之一， 但是OSAS在这一人群中的发病机制、OSAS对生长的影响以及结果的可变性 对OSAS手术干预后的情况知之甚少。在目标1中，我们将评估结构贡献 使用上气道MRI对小颌畸形婴儿OSAS的影响以及神经运动对OSAS的影响 评价动态压力-流量关系。Aim 2将使用各种增长评估，包括 静息能量消耗评估和双能X线吸收测定法，以评估不良的原因 增长目的3将确定手术治疗OSAS对颅面结构的影响， 神经运动功能和小颌畸形婴儿的生长。 我的长期目标是成为一名独立的临床/翻译医生-科学家，拥有独特的专业知识， 儿童OSAS的评估和治疗提议的职业 发展奖通过让我获得进一步的培训和经验，促进了这一职业目标， 成像数据分析和生长评估。这与生物统计学课程相辅相成， 儿童医院优秀研究环境中的临床研究设计和管理 费城和宾夕法尼亚大学。我召集了一个特殊的多学科导师 团队我的主要导师Carole Marcus博士是国际公认的儿科临床研究员 有着杰出的导师记录的OSAS。我的共同导师理查德·施瓦布博士是上世纪 OSAS的气道成像这些导师，沿着一个多学科咨询委员会，包括 放射学、整形外科、营养学和生物统计学方面的研究人员将帮助指导我的职业发展 并将提供内容专业知识，以确保拟议的研究取得成功。拟建项目，沿着 通过构成我的职业发展计划的结构化指导、课程和培训， 为我提供必要的技能和经验，以确保他成功地作为一个独立的调查员， 更好地了解小颌畸形儿童OSAS的评估和治疗的独特技能 和其他颅面疾病

PROJECT SUMMARY/ABSTRACT I am a pediatric pulmonologist with fellowship training in sleep medicine and a background in clinical and translational research in pediatric obstructive sleep apnea syndrome (OSAS). My research is focused on the pathophysiology, consequences, and treatment of OSAS in children with craniofacial conditions. The proposed project seeks to determine the mechanisms of OSAS in infants with micrognathia, a group of children with mandibular hypoplasia. OSAS is common in children and is associated with growth failure and a variety of cognitive and cardiovascular sequelae. Infants with micrognathia are one of the highest-risk groups for OSAS, but the mechanisms of OSAS in this population, the effect of OSAS on growth, and the variability in outcomes after surgical intervention for OSAS are poorly understood. In Aim 1, we will assess the structural contributions to OSAS in infants with micrognathia using upper airway MRI and the neuromotor contributions to OSAS by evaluating dynamic pressure-flow relationships. Aim 2 will use a variety of growth assessments, including resting energy expenditure assessment and dual-energy x-ray absorptiometry, to evaluate the causes of poor growth. Aim 3 will determine the impact of surgical treatment for OSAS on the craniofacial structure, neuromotor function, and growth in infants with micrognathia. My long-term goal is to become an independent clinical/translation physician-scientist with unique expertise in the evaluation and management of OSAS in children with craniofacial conditions. The proposed career development award facilitates this career goal by allowing me to obtain further training and experience in imaging data analysis and growth assessment. This is complemented with coursework in biostatistics and clinical study design and management in the outstanding research environment of Children’s Hospital of Philadelphia and the University of Pennsylvania. I have assembled an exceptional multidisciplinary mentorship team. My primary mentor, Dr. Carole Marcus, is an internationally-recognized clinical researcher in pediatric OSAS with an outstanding record of mentorship. My co-mentor, Dr. Richard Schwab, is a pioneer in upper airway imaging in OSAS. These mentors, along with a multidisciplinary Advisory Committee that includes investigators in radiology, plastic surgery, nutrition, and biostatistics, will help guide my career development and will provide content expertise to ensure success of the proposed research. The proposed project, along with the structured mentoring, coursework, and training that comprise the my career development plan, will provide me with the skill and experience necessary to ensure his success as an independent investigator with a unique skill set in better understanding the evaluation and treatment of OSAS in children with micrognathia and other craniofacial conditions.