[强直性肌营养不良和杜氏肌营养不良低氧血症时的鼻扇动]

[Nasal flaring during hypoxemia in myotonic dystrophy and duchenne muscular dystrophy].

作者信息

Suzuki Mikiya, Oya Yasushi, Murakami Yoshitake, Ogawa Masafumi, Kawai Mitsuru

机构信息

Department of Neurology, National Center Hospital of Neurology and Psychiatry.

出版信息

Rinsho Shinkeigaku. 2009 May;49(5):278-80. doi: 10.5692/clinicalneurol.49.278.

DOI:10.5692/clinicalneurol.49.278

PMID:19594107

Abstract

We investigated the relationship between nasal flaring and SpO2 in 19 patients with Duchenne muscular dystrophy (DMD) and 26 patients with myotonic dystrophy (DM1). In DMD patients, nasal flaring was observed when SpO2 was lower than 96%, while it was not seen even at 82% of SpO2 in DM1. None of the DM1 patients could perform voluntary nasal flaring. Nasal flaring is a useful indicator of hypoxemia in DMD but not in DM1. It remains to be elucidated whether the lack of nasal flaring in DM1 patients is due to abnormal respiratory central mechanism or nasal muscle weakness.

摘要

我们研究了19例杜氏肌营养不良症（DMD）患者和26例强直性肌营养不良症（DM1）患者的鼻翼扇动与血氧饱和度（SpO2）之间的关系。在DMD患者中，当SpO2低于96%时可观察到鼻翼扇动，而在DM1患者中，即使SpO2低至82%也未观察到鼻翼扇动。没有一名DM1患者能够自主进行鼻翼扇动。鼻翼扇动是DMD患者低氧血症的一个有用指标，但在DM1患者中并非如此。DM1患者缺乏鼻翼扇动是由于呼吸中枢机制异常还是鼻肌无力，仍有待阐明。