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The Assessment of Sniff Nasal Inspiratory Pressure in Patients with Duchenne Muscular Dystrophy in China

作者:张淑 等 日期:2018-12-03 浏览量:382

第六届北京罕见病学术大会暨2018京津冀罕见病学术大会征文(47)

Shu ZHANG , Qian-qian MEI, Jing XIN, Hong-ying ZHANG, Shi-wen WU

b Department of neurology, General Hospital of Chinese People's Armed Police force

*Corresponding author:

Shi-wen Wu, M.D., Ph.D.

Department of Neurology, General Hospital of Chinese People’s Armed Police Force, 69 Yongding Road, Haidian District, Beijing, China

Abstract

Objective:  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.

Methods: 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, Steroid DMD group), 145 DMD steroid-naive boys (Age 5.0-13.9, None-Steroid DMD group), and 311 healthy controls (Age 5.0-14.0, Control group).

Results: The SNIP for None-Steroid DMD group, Steroid DMD group and Control group were:56.5 (±14.3) cm H2O,66.4 (±15.5) cm H2O and 78.9 (±21.5) respectively. The SNIP in the None-Steroid DMD group became significantly different from that of the healthy controls since age 7.0-8.9. The significant difference of SNIP between Steroid DMD group and None-Steroid DMD group at age 7.0-10.9. The peak value of SNIP in the None-Steroid DMD group appeared at age 8.7, and decreased dramatically thereafter, while in Steroid DMD group and the Control group peaked at 10.2 years and 12.2 years respectively.

Conclusion: 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.

单位:中国武警总医院神经内科

作者:张淑,梅倩倩,辛婧,张红英,吴士文

通讯作者:吴士文