UPPER AND LOWER AIRWAY COMPROMISE IN THE APERT SYNDROME
UPPER AND LOWER AIRWAY COMPROMISE IN THE APERT SYNDROME
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DOI：
10.1002/ajmg.1320440121
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发表时间：
1992-09-01
期刊：
影响因子：
--
通讯作者：
KREIBORG, S
中科院分区：
文献类型：
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作者：
COHEN, MM;KREIBORG, S
上呼吸道和下呼吸道损伤可能是导致某些Apert综合征患者早期死亡的原因。我们报告二个和审查六例完全或部分软骨套异常的气管。可能的机制包括气管狭窄和/或缺乏气管扩张性，这可能导致呼吸效率低下、无法清除分泌物和/或增加气管抽吸引起表面损伤的可能性。上呼吸道损害，包括阻塞性睡眠呼吸暂停和肺心病，可能是由于减少鼻咽和口咽的尺寸在Apert颅面配置。
Both upper and lower airway compromise may be responsible for early death in some patients with the Apert syndrome. We report on two and review six cases with complete or partial cartilage sleeve abnormalities of the trachea. Possible mechanisms include tracheal stenosis and/or lack of tracheal distensibility which may result in respiratory inefficiency, inability to clear secretions, and/or increased liability to surface injury from tracheal suctioning. Upper airway compromise, consisting of obstructive sleep apnea and cor pulmonale, may result from reduced nasopharyngeal and oropharyngeal dimensions in the Apert craniofacial configuration.