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类固醇与坐骨承重膝踝足矫形器联合应用可使杜氏肌营养不良症患者的步行时间延长至 20 岁以上——病例报告。

Combination of steroids and ischial weight-bearing knee ankle foot orthoses in Duchenne's muscular dystrophy prolongs ambulation past 20 years of age--a case report.

机构信息

Department of Pediatric Neurology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA.

出版信息

Neuromuscul Disord. 2011 Nov;21(11):800-2. doi: 10.1016/j.nmd.2011.06.006. Epub 2011 Jul 23.

DOI:10.1016/j.nmd.2011.06.006
PMID:21784636
Abstract

Patients with Duchenne muscular dystrophy (DMD) lose ambulation by age 12. Long-term steroids have lengthened ambulation by 2-5 years. Ischial weight-bearing knee ankle foot orthoses prolong ambulation for 2-3 years. We report the outcome of the ambulatory status of a patient with DMD treated with daily steroid therapy and orthoses. This male patient was diagnosed with DMD at age of 2. He has been treated with daily steroids since age 7 years. He lost the ability to arise from the floor and walk up steps at age 14 and lost ambulation by age 16. He was fitted with orthoses at age 16 following surgical correction of his lower extremity contractures and regained independent ambulation. At age 20, he was able to stand independently in his orthoses and take steps with moderate support. We conclude that a combination of daily steroids and orthoses prolongs ambulation beyond that of the natural history DMD.

摘要

杜氏肌营养不良症(DMD)患者在 12 岁左右丧失行走能力。长期使用类固醇可使行走时间延长 2-5 年。坐骨承重膝踝足矫形器可延长行走时间 2-3 年。我们报告了一名接受每日类固醇治疗和矫形器治疗的 DMD 患者的行走状态结果。该男性患者在 2 岁时被诊断为 DMD。自 7 岁起,他每天都接受类固醇治疗。14 岁时,他已无法从地板上站起来并走上台阶,16 岁时已无法行走。他在 16 岁时接受了下肢挛缩手术矫正,并佩戴矫形器,恢复了独立行走能力。20 岁时,他能够在矫形器中独立站立并在适度支撑下行走。我们的结论是,每日类固醇和矫形器的结合可延长行走时间,超过 DMD 的自然病史。

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Lancet Neurol. 2018 Mar;17(3):251-267. doi: 10.1016/S1474-4422(18)30024-3. Epub 2018 Feb 3.
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Assessment of intramuscular lipid and metabolites of the lower leg using magnetic resonance spectroscopy in boys with Duchenne muscular dystrophy.应用磁共振波谱评估杜氏肌营养不良症男童小腿肌肉内的脂质和代谢物。
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