Pulmonary Function Assessment in Patients with Spinal Muscular Atrophy Type II and Type III

第Ⅱ型及第Ⅲ型脊髓肌肉萎縮症病患之肺功能評估

林龍昌(Lung-Chang Lin) ； 鐘育志(Yuh-Jyh Jong)

《Acta Paediatrica Taiwanica》 45卷1期 (2004/02) Pp. 15-18

https://doi.org/10.7097/APT.200402.0015

摘要

本篇主要是針對四十八個脊髓肌肉萎縮症病人，年齡從43到34歲，將這是48個病人依據其不同的疾病分類、性別、最佳運動功能及脊柱側彎的程度分別比較肺功能，脊髓肌肉萎縮症病人根據疾病的嚴重程度、發病年齡分成三個類型-第Ⅰ型、第Ⅱ及第Ⅲ型，此外依據病人上前最佳的運動功能分成四級－需要扶才能坐、能自己坐、需要扶才能走、能自己走。所有的脊髓肌肉萎縮症病人若有肺功能障礙時者屬於限制型肺疾病，當病人的運動功能較好時（Ⅲ,Ⅳ級），他也擁有正常或接近正常的肺功能；反之，當病人運動功能很差時（Ⅰ，Ⅱ級），其肺功能也是最差的。第Ⅲ型的脊髓肌肉萎縮症病人也比第Ⅱ型有較好的肺功能，且當病人的脊柱側彎愈厲害時，肺功能也愈差，但男女病人間肺功能幷無差異，當病人的肺功能變差時，我們必須加强病人的呼吸照護减少呼吸道的感染。

關鍵字

脊髓肌肉萎縮症,肺功能

並列摘要

In this study, w e used a spirometer to measure pulmonary function of 48 patients (4.3 to 34 years old), with type II and type III spinal muscular atrophy (SMA) and compared our findings according to the two SMA types, sex, functional grade, and degree of scoliosis. SMA patients w ere stratified into three groups based on severity and the age at which the symptoms began: type I, type II, and type III SMA patients w ere also classified into four functional grades depending upon whether they were able to 1) sit with support, 2) sit independently, 3) walk with assistance, or 4) walk independently. A restrictive pattern was found in all SMA patients w ho were found to have impaired lung function. The highest functional group had normal or nearly normal pulmonary functions. The low est functional group, how ever, were found to have the worst pulmonary functions. Patients with type III SMA had better lung function than type II. We found a significant inverse relationship between pulmonary function and scoliosis, but no significant difference in pulmonary functioning with respect to gender. As the functional grade decreases, chest care needs to be intensified to prevent respiratory tract infections.

並列關鍵字

spinal muscular atrophy ； pulmonary function