Respiratory complications of achondroplasia.
Respiratory complications of achondroplasia.
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软骨发育不全的呼吸系统并发症。
DOI：
10.1016/s0022-3476(83)80180-2
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
D. Brown
中科院分区：
文献类型：
--
作者：
D. Stokes;J. Phillips;C. Leonard;J. Dorst;S. Kopits;J. Trojak;D. Brown
9例软骨发育不全患者在3年内出现了严重的呼吸系统并发症。八人有睡眠呼吸障碍，其中五人有阻塞性睡眠呼吸暂停，其中两人需要气管切开术。在7例明显低氧血症的患者中，5例有肺心病和复发性肺浸润的临床证据。2例患者死亡，1例尸检发现枕骨大孔水平髓质受压，1例呼吸和心力衰竭。对我们患者的适当治疗取决于对导致呼吸系统并发症的机制的认识，包括（1）胸部畸形，（2）上气道阻塞和睡眠呼吸障碍，（3）神经系统并发症，（4）并发慢性肺部疾病，如哮喘。
Nine patients with achondroplasia who were seen over a three-year period developed significant respiratory complications. Eight had sleep-disordered breathing, including obtructive sleep apnea in five, for which two required tracheostomy. Of the seven patients with significant hypoxemia, five had clinical evidence of cor pulmonale and recurrent pulmonary infiltrates. Two patients died, one with autopsy findings of compression of the medulla at the level of the foramen magnum and one with respiratory and cardiac failure. Appropriate therapy for our patients depended on recognition of the mechanisms that led to the respiratory complications, including (1) chest deformity, (2) upper airway obstruction and sleep-disordered breathing, (3) neurologic complications, and (4) coincidental chronic pulmonary conditions such as asthma.