Nashef L, Lane R J
Regional Neurosciences Centre, Charing Cross Hospital, London.
J Neurol Neurosurg Psychiatry. 1989 Sep;52(9):1090-4. doi: 10.1136/jnnp.52.9.1090.
A simple test is described for identifying patients with abnormalities of muscle energy metabolism secondary to mitochondrial dysfunction, based on the venous lactate response to exercise at 90% of predicted work rate at the anaerobic threshold. The test was standardised for age, weight and sex of subjects, and was abnormal in all cases of mitochondrial cytopathy tested, with a false positive rate of 7% in a control population. The test was abnormal in two cases of mitochondrial disease in which muscle biopsy was normal or showed only non-specific changes.
本文描述了一种简单的测试方法，用于识别继发于线粒体功能障碍的肌肉能量代谢异常患者，该方法基于在无氧阈值时按预测工作率的90%进行运动后静脉血乳酸反应。该测试针对受试者的年龄、体重和性别进行了标准化，在所有测试的线粒体细胞病病例中均表现异常，在对照人群中的假阳性率为7%。在两例线粒体疾病患者中，该测试结果异常，而其肌肉活检结果正常或仅显示非特异性变化。
