Ekici Barış, Ergül Yakup, Tatlı Burak, Bilir Feride, Binboğa Fatih, Süleyman Ayşe, Tamay Zeynep, Calışkan Mine, Güler Nermin
Department of Pediatric Neurology, Istanbul Medical Faculty, Istanbul, Turkey.
Ann Indian Acad Neurol. 2011 Jul;14(3):182-4. doi: 10.4103/0972-2327.85889.
The aim of this work was to assess the respiratory functions of ambulatory Duchenne patients and to propose an earlier time period for intervention.
Lung functions and North Star Ambulatory Assessment (NSAA) scores of Duchenne patients were evaluated simultaneously.
Thirty ambulatory Duchenne patients were included in this study. NSAA scores of the patients were directly correlated with arm abduction, arm adduction, and shoulder flexion strengths. Forced expiratory volume in 1 second percent predicted and forced vital capacity (FVC) percent predicted correlated inversely to age and to the NSAA score. Twelve of 13 patients with FVC values lower than 80% of predicted had NSAA scores below 24 points. None of the patients who were younger than 7 years had FVC values lower than 80% of predicted.
Annual spirometry is necessary for Duchenne patients older than 6 years regardless of the ambulatory status.
本研究旨在评估门诊杜氏肌营养不良症患者的呼吸功能，并提出更早的干预时间段。
同时评估杜氏肌营养不良症患者的肺功能和北极星门诊评估（NSAA）评分。
本研究纳入了30例门诊杜氏肌营养不良症患者。患者的NSAA评分与手臂外展、手臂内收和肩部屈曲力量直接相关。一秒用力呼气容积占预计值百分比和用力肺活量（FVC）占预计值百分比与年龄和NSAA评分呈负相关。13例FVC值低于预计值80%的患者中有12例NSAA评分低于24分。7岁以下的患者中没有FVC值低于预计值80%的。
无论门诊状态如何，6岁以上的杜氏肌营养不良症患者每年都需要进行肺活量测定。
