Liu Jocelyn M K, Okamura Carol S, Bogan Daniel J, Bogan Janet R, Childers Martin K, Kornegay Joe N
Dalton Cardiovascular Research Center, University of Missouri-Columbia, Columbia, Missouri, USA.
Muscle Nerve. 2004 Dec;30(6):767-73. doi: 10.1002/mus.20154.
Glucocorticoid use may provide short-term functional improvement in boys with Duchenne muscular dystrophy (DMD). We report functional and histopathologic changes following a 4-month course of daily oral prednisone in a canine model of DMD, termed golden retriever muscular dystrophy (GRMD). Muscle extension forces in GRMD dogs treated daily with 1 and 2 mg/kg prednisone measured 2.349 +/- 0.92 and 3.486 +/- 0.67 N/kg, respectively, compared to 1.927 +/- 0.63 N/kg in untreated GRMD controls (p < 0.05 for 2 mg/kg group); GRMD muscle flexion forces measured 0.435 +/- 0.13 and 0.303 +/- 0.08 N/kg, respectively, compared to 0.527 +/- 0.01 N/kg in untreated GRMD controls (p < 0.05 for both groups). Although cranial sartorius hypertrophy and tibiotarsal joint angles also tended to improve, myofiber calcification increased and fetal myosin expression decreased following prednisone. Thus, functional data indicate benefit but histopathologic changes following prednisone treatment in GRMD suggest possible deleterious consequences.
糖皮质激素的使用可能会使杜氏肌营养不良症(DMD)男孩获得短期功能改善。我们报告了在杜氏肌营养不良症的犬模型——金毛寻回犬肌营养不良症(GRMD)中,每日口服泼尼松4个月疗程后的功能和组织病理学变化。每日接受1毫克/千克和2毫克/千克泼尼松治疗的GRMD犬的肌肉伸展力分别为2.349±0.92和3.486±0.67牛/千克,而未治疗的GRMD对照组为1.927±0.63牛/千克(2毫克/千克组p<0.05);GRMD犬的肌肉屈曲力分别为0.435±0.13和0.303±0.08牛/千克,而未治疗的GRMD对照组为0.527±0.01牛/千克(两组p<0.05)。尽管缝匠肌头部肥大和胫跗关节角度也有改善趋势,但泼尼松治疗后肌纤维钙化增加,胎儿型肌球蛋白表达减少。因此,功能数据显示有益,但GRMD犬接受泼尼松治疗后的组织病理学变化提示可能存在有害后果。