Beck Jennifer, Weinberg Jan, Hamnegård Carl-Hugo, Spahija Jadranka, Olofson Jan, Grimby Gunnar, Sinderby Christer
Department of Newborn and Developmental Pediatrics, Sunnybrook and Women's College Health Sciences Center, Women's College Compus, No. 440-76 Grenville Street, Toronto, Ont., Canada M5S 1B2.
Neuromuscul Disord. 2006 Mar;16(3):161-7. doi: 10.1016/j.nmd.2006.01.003. Epub 2006 Feb 20.
The aim of this study was to assess diaphragm electrical activation and diaphragm strength in patients with advanced Duchenne muscular dystrophy during resting conditions. Eight patients with advanced Duchenne muscular dystrophy (age of 25 +/- 2 years) were studied during tidal breathing, maximal inspiratory capacity, maximal sniff inhalations, and magnetic stimulation of the phrenic nerves. Six patients were prescribed home mechanical ventilation (five non-invasive and one tracheotomy). Transdiaphragmatic pressure and diaphragm electrical activation were measured using an esophageal catheter. During tidal breathing (tidal volume 198 +/- 83 ml, breathing frequency 25 +/- 7), inspiratory diaphragm electrical activation was clearly detectable in seven out of eight patients and was 12 +/- 7 times above the noise level, and represented 45 +/- 19% of the maximum diaphragm electrical activation. Mean inspiratory transdiaphragmatic pressure during tidal breathing was 1.5 +/- 1.2 cmH2O, and during maximal sniff was 7.6 +/- 3.6 cmH2O. Twitch transdiaphragmatic pressure deflections could not be detected. This study shows that despite near complete loss of diaphragm strength in advanced Duchenne muscular dystrophy, diaphragm electrical activation measured with an esophageal electrode array remains clearly detectable in all but one patient.
本研究旨在评估晚期杜氏肌营养不良症患者在静息状态下的膈肌电激活情况和膈肌力量。对8例晚期杜氏肌营养不良症患者（年龄25±2岁）进行了潮式呼吸、最大吸气容量、最大嗅吸以及膈神经磁刺激研究。6例患者接受了家庭机械通气治疗（5例无创通气，1例气管切开）。使用食管导管测量跨膈压和膈肌电激活情况。在潮式呼吸时（潮气量198±83 ml，呼吸频率25±7），8例患者中有7例可清晰检测到吸气时的膈肌电激活，其强度比噪声水平高12±7倍，占膈肌最大电激活的45±19%。潮式呼吸时平均吸气跨膈压为1.5±1.2 cmH₂O，最大嗅吸时为7.6±3.6 cmH₂O。未检测到颤搐跨膈压偏转。本研究表明，尽管晚期杜氏肌营养不良症患者的膈肌力量几乎完全丧失，但除1例患者外，使用食管电极阵列测量的膈肌电激活在其他所有患者中仍可清晰检测到。
