Department of Orthopaedic Surgery, Friedrich-Alexander-Universität Erlangen-Nürnberg, Rathsberger Str, 57, D-91054 Erlangen, Germany.
BMC Musculoskelet Disord. 2013 Oct 4;14:283. doi: 10.1186/1471-2474-14-283.
Progressive scoliosis, pelvic obliquity and increasing reduction of pulmonary function are among the most significant problems for patients with SMA type II and SMA type III once they have lost the ability to walk. The aim of this study was to examine and document the development and natural course of scoliosis in patients with spinal muscular atrophy type II and IIIa.
For the purposes of a descriptive clinical study, we observed 126 patients, 99 with SMA II and 27 with SMA IIIa and the data of scoliosis, pelvic obliquity and relative age-dependent inspiratory vital capacity were evaluated.
Scoliosis and pelvic obliquity were regularly observed already in children under 4 years old in the group with SMA II. The severity and progression of both conditions were much more pronounced in the SMA II group than in the IIIa group. There was already a distinct reduction in relative vital capacity in the group of 4- to 6-year-olds with SMA II.
The differences between the two SMA types II and IIIa described in this study should be taken into consideration when developing new treatments and in management of scoliosis in the childhood years of these patients.
对于患有 SMA Ⅱ型和Ⅲ型的患者来说，一旦失去行走能力，脊柱侧凸、骨盆倾斜和肺功能逐渐下降是最严重的问题。本研究的目的是检查和记录 SMA Ⅱ型和Ⅲa 型患者脊柱侧凸的发展和自然病程。
本研究为描述性临床研究，我们观察了 126 例患者，99 例为 SMA Ⅱ型，27 例为 SMA Ⅲa 型，评估了脊柱侧凸、骨盆倾斜和相对年龄依赖性吸气肺活量数据。
在 SMA Ⅱ型组中，4 岁以下的儿童中就经常观察到脊柱侧凸和骨盆倾斜。SMA Ⅱ型组中这两种情况的严重程度和进展程度都明显高于Ⅲa 型组。在 SMA Ⅱ型组中，4 至 6 岁儿童的相对肺活量已经明显降低。
本研究描述的两种 SMA 类型Ⅱ型和Ⅲa 之间的差异，在为这些患者制定新的治疗方案和管理儿童时期脊柱侧凸时应加以考虑。
