[糖皮质激素在治疗杜氏肌营养不良中的作用:我们的经验]。

Anales Espanoles De Pediatria Pub Date : 2002-12-01
S Rafia, S I Pascual-Pascual, M A Martínez-Granero, I Pascual-Castroviejo
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引用次数: 0

摘要

目的评价我科收治的杜氏肌营养不良患者经皮质类固醇治疗后的临床病程,并与未经皮质类固醇治疗的患者进行比较。患者和方法我们对20例诊断为杜氏肌营养不良的儿童患者进行了回顾性研究,他们接受了皮质类固醇治疗:10例接受了地拉法柯治疗,10例拒绝治疗。采用MRC肌肉力量量表和Vignos功能量表评估两组患者的临床病程,并进行比较。结果治疗后患者病情呈进行性恶化。皮质类固醇治疗的患者在肌肉力量和功能表现方面均表现出疾病的稳定。此外,70%的患者肌肉平衡得到改善,但只有2%的患者功能得到改善。类固醇治疗的积极作用平均持续12个月。两组的独立步态丧失发生在相似的年龄(10.3岁vs. 10.5岁)。跟腱手术效果不佳。结论皮质类固醇能稳定临床症状,增强肌力。功能的改善并不显著,包括步态的丧失,可能是因为这种丧失也依赖于关节挛缩的增加。多专业团队之间的良好协调对于取得最佳结果至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Effects of corticosteroids in the management of Duchenne muscular dystrophy: our experience].

Objective To evaluate the clinical course in patients with Duchenne muscular dystrophy admitted to our department who received corticosteroid treatment and to compare their course with that in patients who did not receive corticosteroid treatment.Patients and methodsWe performed a retrospective study of 20 pediatric patients with a diagnosis of Duchenne muscular dystrophy who were offered corticosteroid treatment: 10 patients received deflazacort and 10 refused the treatment. The MRC muscular strength scale and Vignos' functional scale were used to evaluate clinical course, which was compared in both groups.ResultsUntreated patients showed progressive worsening. Corticosteroid-treated patients showed disease stabilization both in muscular strength and functional performance. In addition, muscular balance improved in 70 % of these patients, but only 2 % showed functional improvement. The positive effect of steroid treatment had a mean duration of 12 months. Loss of independent gait occurred at similar ages in both groups (10.3 vs. 10.5 years). The results of Achilles' tendon surgery were poor.ConclusionsCorticosteroids produced clinical stabilization and improved muscular strength. Functional improvement was not significant, including loss of gait, probably because this loss also depends on an increase in joint contracture. Good coordination among multiprofessional teams is essential to achieve optimal results.

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