Radley H G, De Luca A, Lynch G S, Grounds M D
School of Anatomy and Human Biology, University of Western Australia, Crawley, Australia.
Int J Biochem Cell Biol. 2007;39(3):469-77. doi: 10.1016/j.biocel.2006.09.009. Epub 2006 Oct 10.
Duchenne muscular dystrophy is a lethal X-linked muscle disease resulting from a defect in the muscle membrane protein dystrophin. The absence of dystrophin leads to muscle membrane fragility, muscle death (necrosis) and eventual replacement of skeletal muscle by fat and fibrous connective tissue. Extensive muscle wasting and respiratory failure results in premature death often by the early 20s. This short review evaluates drug and nutritional interventions designed to reduce the severity of muscular dystrophy, while awaiting the outcome of research into therapies to correct the fundamental gene defect. Combinations of dietary supplementation with amino-acids such as creatine, specific anti-inflammatory drugs and perhaps drugs that target ion channels might have immediate realistic clinical benefits although rigorous research is required to determine optimal combinations of such interventions.
杜氏肌营养不良症是一种致命的X连锁肌肉疾病，由肌肉膜蛋白抗肌萎缩蛋白缺陷引起。抗肌萎缩蛋白的缺失导致肌肉膜脆弱、肌肉死亡（坏死），最终骨骼肌被脂肪和纤维结缔组织替代。广泛的肌肉萎缩和呼吸衰竭通常导致在20岁出头时过早死亡。这篇简短的综述评估了旨在减轻肌营养不良症严重程度的药物和营养干预措施，同时等待纠正基本基因缺陷疗法的研究结果。补充诸如肌酸等氨基酸的饮食、特定的抗炎药物以及可能针对离子通道的药物联合使用，可能会立即产生切实的临床益处，不过需要进行严格研究以确定此类干预措施的最佳组合。
