Haymond M W, Strobel K E, DeVivo D C
Neurology. 1978 Dec;28(12):1224-31. doi: 10.1212/wnl.28.12.1224.
Carbohydrate homeostasis was evaluated in nine patients with Duchenne muscular dystrophy (DMD), and in six age-matched normal boys. Mild carbohydrate intolerance was observed in the DMD patients during an oral glucose tolerance test. Only minor differences were observed between the DMD patients and the normal subjects during an oral glucose tolerance test. Only minor differnences were observed between the DMD patients and the normal subjects during a 30-hour period of caloric deprivation. Despite obvious muscle wasting and significant hypoalaninemia, the DMD group was resistant to fasting hypoglycemia. Plasma glutamine concentrations were the same in both groups under all study conditions. Dueterium-labeled glucose and alanine flux studies documented normal glucose inflow and outflow rates and decreased alanine inflow and outflow rates in the DMD group. The 40 percent reduction in alanine turnover, however, was less than expected for a group of patients with an estimated 67 percent reduction in muscle mass. These observations suggest that there may be accelerated protein degradation in the muscles of DMD patients.
对9名杜氏肌营养不良症（DMD）患者和6名年龄匹配的正常男孩进行了碳水化合物稳态评估。在口服葡萄糖耐量试验期间，DMD患者出现轻度糖耐量异常。在口服葡萄糖耐量试验期间，DMD患者与正常受试者之间仅观察到微小差异。在30小时的热量剥夺期间，DMD患者与正常受试者之间也仅观察到微小差异。尽管存在明显的肌肉萎缩和显著的低丙氨酸血症，但DMD组对空腹低血糖具有抵抗性。在所有研究条件下，两组的血浆谷氨酰胺浓度相同。氘标记的葡萄糖和丙氨酸通量研究记录了DMD组正常的葡萄糖流入和流出速率以及降低的丙氨酸流入和流出速率。然而，丙氨酸周转率降低40%，低于预计肌肉量减少67%的一组患者的预期值。这些观察结果表明，DMD患者的肌肉中可能存在加速的蛋白质降解。
