Fujak Albert, Kopschina Carsten, Gras Florian, Forst Raimund, Forst Jürgen
Department of Orthopaedic Surgery, Friedrich-Alexander-University Erlangen-Nuremberg, Germany.
Ortop Traumatol Rehabil. 2010 Sep-Oct;12(5):410-9.
The most significant orthopaedic problem for patients with the intermediate form of spinal muscular atrophy, SMA type II, is the appearance of contractures in addition to progressive scoliosis and pelvic obliquity with increasing loss of sitting stability. This study deals with restrictions of the passive range of motion and the development of contractures in the joints of the upper extremities in these patients.
We followed up 143 patients, 74 female and 69 male, with SMA type II for an average of 5.3 years (± 4.0, 0.2 - 18.7). Their average age at the first examination was 8.4 years (± 6.6, 0.1 - 34.1) and at the last examination 12.3 years (± 6.5, 0.7 - 37.1). The passive range of joint motion was determined using a goniometer. According to Johnson et al. (1992), we calculated the relative contracture index (CI).
The loss of range of motion and the contractures of the joints of the upper extremities (shoulder, elbow and wrist) increased progressively with age. The most marked restriction of motion was in the elbow joint with severe flexion contractures in some cases.
The findings of this study give us more information about the development of contractures of the upper extremities and aim to help to improve the quality of orthopaedic care of patients with SMA type II.
对于中间型脊髓性肌萎缩症（SMA II型）患者而言，最严重的骨科问题是除了进行性脊柱侧弯和骨盆倾斜导致坐位稳定性日益丧失外，还会出现挛缩。本研究探讨了这些患者上肢关节被动活动范围的受限情况以及挛缩的发展。
我们对143例SMA II型患者进行了随访，其中女性74例，男性69例，平均随访5.3年（±4.0，0.2 - 18.7）。他们首次检查时的平均年龄为8.4岁（±6.6，0.1 - 34.1），末次检查时为12.3岁（±6.5，0.7 - 37.1）。使用量角器测定关节的被动活动范围。根据约翰逊等人（1992年）的方法，我们计算了相对挛缩指数（CI）。
上肢关节（肩部、肘部和腕部）的活动范围丧失和挛缩随年龄逐渐增加。运动受限最明显的是肘关节，在某些情况下出现严重的屈曲挛缩。
本研究结果为我们提供了更多关于上肢挛缩发展的信息，旨在帮助改善SMA II型患者的骨科护理质量。
