Related Experiment Videos
Decrease in urinary excretion of 3-methylhistidine by patients with Duchenne muscular dystrophy during glucocorticoid
1First Department of Internal Medicine, School of Medicine, University of Tokushima, Japan.
Journal of Neurology
|January 1, 1993
Summary
Prednisolone (PSL) treatment improved muscle strength and reduced muscle damage markers in young Duchenne muscular dystrophy patients. PSL also decreased urinary 3-methylhistidine (3-MeH) excretion, indicating reduced muscle protein breakdown.
Area of Science:
- Biochemistry
- Clinical Medicine
- Pharmacology
Background:
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder characterized by muscle degeneration.
- Current treatments for DMD aim to manage symptoms and slow disease progression.
Purpose of the Study:
- To investigate the effects of oral prednisolone (PSL) on muscle function and protein metabolism in adolescent patients with Duchenne muscular dystrophy.
- To assess changes in serum creatine kinase (CK), myoglobin (Mb), and urinary 3-methylhistidine (3-MeH) excretion as indicators of muscle breakdown.
Main Methods:
- Seven patients (aged 10-17 years) with DMD received oral PSL (0.8-1.0 mg/kg/day) for 8 weeks.
- Serial measurements included muscle strength, serum CK and Mb levels, and urinary excretion of 3-MeH and glycine (Gly) relative to creatinine.
Main Results:
- All patients showed improved muscle strength and motor function during PSL treatment.
- Serum CK activity and Mb levels decreased, while urinary 3-MeH excretion reduced to 51-63% of baseline values.
- Urinary 3-MeH to creatinine and 3-MeH to Gly ratios also decreased, suggesting reduced muscle protein catabolism.
Conclusions:
- Prednisolone treatment appears to inhibit proteolysis of muscle contractile proteins in Duchenne muscular dystrophy.
- These findings support the role of PSL in mitigating muscle breakdown in DMD patients.