Byrne E
Department of Neurology, St Vincent's Hospital, Fitzroy, Victoria, Australia.
Aust Paediatr J. 1988;24 Suppl 1:58-61.
Current classifications of mitochondrial cytopathies neglect rigorous assessment of the literature which consists often of single case reports. In addition no attempt is made by many workers to distinguish between primary and secondary respiratory chain defects. Current biochemical classifications also fail to take into account the considerable number of patients who have ragged red fibre myopathies and lactic acidosis but who do not show demonstrable respiratory enzyme defects. For this reason a new approach is advocated with consideration of possible respiratory chain defects, probable primary respiratory chain defects, probable secondary respiratory chain defects, and mitochondrial cytopathies with normal respiratory chain enzyme function in vitro. Existing knowledge is reviewed under these categories to which are added further original observations.
目前线粒体细胞病的分类忽视了对文献的严格评估，而这些文献往往是单个病例报告。此外，许多研究人员并未尝试区分原发性和继发性呼吸链缺陷。目前的生化分类也没有考虑到大量患有破碎红纤维肌病和乳酸性酸中毒但未表现出可证实的呼吸酶缺陷的患者。因此，提倡一种新的方法，考虑可能的呼吸链缺陷、可能的原发性呼吸链缺陷、可能的继发性呼吸链缺陷以及体外呼吸链酶功能正常的线粒体细胞病。在这些类别下对现有知识进行了综述，并补充了进一步的原始观察结果。
