Kawai H, Adachi K, Nishida Y, Inui T, Kimura C, Saito S
First Department of Internal Medicine, School of Medicine, University of Tokushima, Japan.
J Neurol. 1993;240(3):181-6. doi: 10.1007/BF00857525.
Seven patients, aged 10-17 years, with Duchenne muscular dystrophy were treated orally with prednisolone (PSL) at a dose of 0.8-1.0 mg/kg per day for 8 weeks. During the treatment their muscle strength, serum creatine kinase (CK) activity, serum levels of myoglobin (Mb), and urinary excretion of 3-methylhistidine (3-MeH) and glycine (Gly) were measured serially. In all the patients, the motor function or muscle strength improved, and the serum CK activity and Mb level decreased during PSL treatment. Urinary excretion of 3-MeH, a unique constituent of muscle contractile proteins, decreased to 51-63% of the baseline value in weeks 6-9 after the start of PSL administration, and returned to the baseline level in week 12. The ratios of 3-MeH to creatinine and to Gly also decreased during the treatment. Urinary excretion of Gly, which is ubiquitous in all tissues including muscle, did not decrease during the treatment. These findings suggest that PSL inhibits proteolysis of muscle contractile protein.
7名年龄在10至17岁之间的杜氏肌营养不良症患者口服泼尼松龙（PSL），剂量为每日0.8至1.0毫克/千克，持续8周。治疗期间，连续测量他们的肌肉力量、血清肌酸激酶（CK）活性、血清肌红蛋白（Mb）水平以及3-甲基组氨酸（3-MeH）和甘氨酸（Gly）的尿排泄量。在所有患者中，PSL治疗期间运动功能或肌肉力量得到改善，血清CK活性和Mb水平下降。肌肉收缩蛋白的独特成分3-MeH的尿排泄量在PSL给药开始后的第6至9周降至基线值的51%至63%，并在第12周恢复到基线水平。治疗期间，3-MeH与肌酐和与Gly的比率也下降。在包括肌肉在内的所有组织中普遍存在的Gly的尿排泄量在治疗期间没有下降。这些发现表明PSL抑制肌肉收缩蛋白的蛋白水解。
