Brasil Santos Dante, Vaugier Isabelle, Boussaïd Ghilas, Orlikowski David, Prigent Hélène, Lofaso Frédéric
INSERM U 1179-Université de Versailles Saint Quentin en Yvelines, Versailles, France.
Centro de Fisioterapia e Reabilitação-Hospital Universitário de Brasília, Universidade de Brasília, Brasilia, Brazil.
Respir Care. 2016 Nov;61(11):1530-1535. doi: 10.4187/respcare.04703. Epub 2016 Oct 18.
Duchenne muscular dystrophy (DMD) is a sex-linked genetic disorder in which progressive impairment of skeletal muscle function eventually leads to severe respiratory failure requiring continuous noninvasive ventilation (NIV) at home. A current focus of debate is whether NIV may slow the decline in respiratory function or, on the contrary, worsen respiratory function when started early. Our objective here was to describe the effects of NIV on vital capacity (VC) and maximum respiratory pressures in DMD.
We analyzed retrospective data from 71 subjects with DMD, including VC, maximum static respiratory pressures, and sniff nasal inspiratory pressure before and after NIV initiation. The declines in these variables from the highest value to the most recent value were computed.
Although respiratory function continued to deteriorate over time, NIV introduction was followed by significant slowing in the annual rates of decline in VC (from 4.28 to 1.36 percent predicted), maximum inspiratory pressure (from 2.77 to 1.48 cm HO), and maximum expiratory pressure (from 2.00 to 1.00 cm HO). NIV had no effect on sniff nasal inspiratory pressure.
Introducing NIV in subjects with DMD was followed by slowing of the declines in VC and in maximum static inspiratory and expiratory pressures.
杜氏肌营养不良症（DMD）是一种X连锁遗传性疾病，其中骨骼肌功能的进行性损害最终导致严重呼吸衰竭，需要在家中持续进行无创通气（NIV）。当前争论的一个焦点是，NIV在早期启动时是会减缓呼吸功能的下降，还是相反会使呼吸功能恶化。我们在此的目的是描述NIV对DMD患者肺活量（VC）和最大呼吸压力的影响。
我们分析了71例DMD患者的回顾性数据，包括NIV启动前后的VC、最大静态呼吸压力和嗅吸鼻吸气压力。计算这些变量从最高值到最近值的下降情况。
尽管呼吸功能随时间持续恶化，但引入NIV后，VC的年下降率（从预测值的4.28%降至1.36%）、最大吸气压力（从2.77 cm H₂O降至1.48 cm H₂O）和最大呼气压力（从2.00 cm H₂O降至1.00 cm H₂O）均显著减缓。NIV对嗅吸鼻吸气压力没有影响。
在DMD患者中引入NIV后，VC以及最大静态吸气和呼气压力的下降减缓。
