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The assessment of sniff nasal inspiratory pressure in patients with Duchenne muscular dystrophy in China

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机构: [a]Department of Neurology and Suzhou Clinical Research Center of Neurological Disease, The Second Affiliated Hospital of Soochow University, China [b]Department of Neurology, General Hospital of Chinese People’s Armed Police force, China
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关键词: Duchenne muscular dystrophy (DMD) Sniff nasal inspiratory pressure (SNIP) Respiratory dysfunction

摘要:
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, 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). Results: The SNIP for DMD-nonsteroid group, DMD-steroid 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 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. 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. (C) 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

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出版当年[2017]版:
大类 | 4 区 医学
小类 | 4 区 临床神经病学
最新[2023]版:
大类 | 4 区 医学
小类 | 4 区 临床神经病学 4 区 儿科
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出版当年[2016]版:
Q4 CLINICAL NEUROLOGY
最新[2023]版:
Q2 PEDIATRICS Q3 PEDIATRICS Q3 CLINICAL NEUROLOGY Q4 CLINICAL NEUROLOGY

影响因子: 最新[2023版] 最新五年平均 出版当年[2016版] 出版当年五年平均 出版前一年[2015版] 出版后一年[2017版]

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第一作者机构: [a]Department of Neurology and Suzhou Clinical Research Center of Neurological Disease, The Second Affiliated Hospital of Soochow University, China [b]Department of Neurology, General Hospital of Chinese People’s Armed Police force, China
通讯作者:
通讯机构: [*1]Department of Neurology, General Hospital of Chinese People’s Armed Police Force, 69 Yongding Road, Haidian District, Beijing, China [*2]Department of Neurology and Suzhou Clinical Research Center of Neurological Disease, The Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Suzhou, China
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