Orliaguet O, Pépin J L, Veale D, Kelkel E, Pinel N, Lévy P
Dept of Respiratory Medicine, CHU de Grenoble, France.
Eur Respir J. 1999 May;13(5):1195-7. doi: 10.1034/j.1399-3003.1999.13e42.x.
A 42-yr-old male with Hunter's syndrome presented with severe obstructive sleep apnoea syndrome (OSAS) and daytime respiratory failure. Continuous positive airway pressure (CPAP) therapy was initially ineffective and produced acute respiratory distress. Extensive Hunter's disease infiltration of the upper airway with a myxoma was confirmed. Following surgery to remove the myxoma at the level of the vocal cords, CPAP therapy was highly effective and well tolerated. This report demonstrates the necessity of evaluating fully the upper airway in patients with unusual variants of OSAS, particularly where the disease is not adequately controlled by CPAP.
一名42岁患有亨特综合征的男性患者出现了严重的阻塞性睡眠呼吸暂停综合征（OSAS）和白天呼吸衰竭。持续气道正压通气（CPAP）治疗最初无效，并引发了急性呼吸窘迫。经证实，患者上气道存在广泛的亨特病浸润并伴有黏液瘤。在声带水平切除黏液瘤的手术后，CPAP治疗效果显著且耐受性良好。本报告表明，对于OSAS异常变体的患者，尤其是CPAP治疗无法充分控制病情的患者，全面评估上气道非常必要。
