Zhang Shu, Mei Qian-Qian, Xin Jing, Zhang Hong-Ying, Wu Shi-Wen, Liu Chun-Feng
Department of Neurology and Suzhou Clinical Research Center of Neurological Disease, The Second Affiliated Hospital of Soochow University, China; Department of Neurology, General Hospital of Chinese People's Armed Police force, China.
Department of Neurology, General Hospital of Chinese People's Armed Police force, China.
Brain Dev. 2018 May;40(5):391-396. doi: 10.1016/j.braindev.2018.01.008. Epub 2018 Feb 21.
Progressive weakness of respiratory muscles remains one of the leading causes of death among patients with Duchenne muscular dystrophy (DMD). Currently, there are few pulmonary function data among Chinese DMD patients. This study was carried out to evaluate the sniff nasal inspiratory pressure (SNIP) change among a group of Chinese DMD patients, and compare it with the SNIP value of patients with neuromuscular disorders in other countries.
SNIP data were collected in three research groups that consists of 581 subjects: 125 DMD boys who have taken steroid (Age 5.0-13.3, DMD-steroid group), 145 DMD steroid-naive boys (Age 5.0-13.9, DMD-nonsteroid group), and 311 healthy controls (Age 5.0-14.0, Control group).
The SNIP for DMD-nonsteroid group, DMD-steroid group and Control group were: 56.5 (±14.3) cm HO,66.4 (±15.5) cm HO and 78.9 (±21.5) respectively. The SNIP in the DMD-nonsteroid group became significantly different from that of the healthy controls since age 7.0-8.9. The significant difference of SNIP between DMD-steroid group and DMD-nonsteroid group at age 7.0-10.9. The peak value of SNIP in the DMD-nonsteroid group appeared at age 8.7, and decreased dramatically thereafter, while in DMD-steroid group and the Control group peaked at 10.2 years and 12.2 years respectively. There was a bit difference between SNIP in this group and that in previous researches which may be due to geographical distribution and ethnic backgrounds.
This study strengthens the previous findings that SNIP can be used to evaluate respiratory dysfunction during the early stage of young patients with neuromuscular disorders, and demonstrates that steroid is effective in slowing the decrease of SNIP in this group of Chinese DMD boys.
呼吸肌进行性无力仍是杜氏肌营养不良症（DMD）患者的主要死因之一。目前，中国DMD患者的肺功能数据较少。本研究旨在评估一组中国DMD患者的嗅吸鼻吸气压力（SNIP）变化，并将其与其他国家神经肌肉疾病患者的SNIP值进行比较。
收集了三个研究组共581名受试者的SNIP数据：125名服用类固醇的DMD男孩（年龄5.0 - 13.3岁，DMD-类固醇组），145名未服用类固醇的DMD男孩（年龄5.0 - 13.9岁，DMD-非类固醇组），以及311名健康对照者（年龄5.0 - 14.0岁，对照组）。
DMD-非类固醇组、DMD-类固醇组和对照组的SNIP分别为：56.5（±14.3）cmH₂O、66.4（±15.5）cmH₂O和78.9（±21.5）cmH₂O。DMD-非类固醇组的SNIP自7.0 - 8.9岁起与健康对照组有显著差异。DMD-类固醇组和DMD-非类固醇组在7.0 - 10.9岁时SNIP有显著差异。DMD-非类固醇组的SNIP峰值出现在8.7岁，此后急剧下降，而DMD-类固醇组和对照组的峰值分别出现在10.2岁和12.2岁。该组的SNIP与先前研究存在一定差异，这可能归因于地理分布和种族背景。
本研究强化了先前的发现，即SNIP可用于评估神经肌肉疾病年轻患者早期的呼吸功能障碍，并表明类固醇对减缓这组中国DMD男孩的SNIP下降有效。
