Khirani Sonia, Dabaj Ivana, Amaddeo Alessandro, Olmo Arroyo Jorge, Ropers Jacques, Tirolien Stéphane, Coudert Véronique, Estournet Brigitte, Fauroux Brigitte, Quijano-Roy Susana
ASV Santé, Gennevilliers, France; AP-HP, Hôpital Necker-Enfants Malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.
AP-HP, Hôpital Raymond Poincaré, Hôpitaux Universitaires Paris-Ile-de-France Ouest, Pôle pédiatrique, Service de Pédiatrie, Garches, France; Centre de Référence Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye (GNMH), Garches, France; Réseau National de Maladies Neuromusculaires FILNEMUS, Garches, France.
Pediatr Neurol. 2017 Aug;73:78-87.e1. doi: 10.1016/j.pediatrneurol.2017.04.013. Epub 2017 Apr 20.
Oral salbutamol has shown clinical benefits in spinal muscular atrophy (SMA). We studied its effect on the respiratory muscle strength in children with different types of SMA.
Lung and respiratory muscle functions were assessed in children receiving daily oral salbutamol for at least one year. The respiratory data of age-matched SMA II historical control subjects were compared with data of SMA II patients receiving salbutamol.
Seven children (6.4 ± 2.0 years old, range four to ten; one SMA I, five SMA II, and one SMA III) treated with salbutamol (duration 23 ± 8 months) were assessed. Maximal static inspiratory pressure, sniff nasal inspiratory pressure, and slow vital capacity were significantly better in the salbutamol-treated SMA II group compared with control subjects (P < 0.05).
Long-term oral salbutamol showed benefits in respiratory function in children with SMA and appeared to increase the strength of the inspiratory muscles in a small cohort of SMA II patients.
口服沙丁胺醇已在脊髓性肌萎缩症（SMA）中显示出临床益处。我们研究了其对不同类型SMA儿童呼吸肌力量的影响。
对每日口服沙丁胺醇至少一年的儿童进行肺和呼吸肌功能评估。将年龄匹配的SMA II历史对照受试者的呼吸数据与接受沙丁胺醇治疗的SMA II患者的数据进行比较。
评估了7名接受沙丁胺醇治疗（疗程23±8个月）的儿童（6.4±2.0岁，范围4至10岁；1例SMA I，5例SMA II，1例SMA III）。与对照受试者相比，沙丁胺醇治疗的SMA II组的最大静态吸气压力、嗅鼻吸气压力和慢肺活量明显更好（P<0.05）。
长期口服沙丁胺醇对SMA儿童的呼吸功能有益，并且似乎在一小群SMA II患者中增加了吸气肌的力量。
