Gordon N
Booth Hall Children's Hospital, Blackley, Manchester, England.
Brain Dev. 1987;9(6):571-5. doi: 10.1016/s0387-7604(87)80087-6.
The different types of adrenoleukodystrophy are considered with their clinical and biochemical features, particularly the excess of very-long-chain fatty acids. Then other conditions which show this latter finding are described, including the Zellweger cerebrohepatorenal syndrome, hyperpipecolic acidemia and Refsum disease. The role of peroxisomes is discussed and the different ways in which their functions can be disordered. The possibilities of treating these diseases is at the moment limited but examples are given of research already carried out in this field.
本文探讨了不同类型的肾上腺脑白质营养不良及其临床和生化特征，尤其是超长链脂肪酸的过量情况。接着描述了出现超长链脂肪酸过量这一现象的其他病症，包括泽尔韦格脑肝肾综合征、高哌可酸血症和雷夫叙姆病。文中讨论了过氧化物酶体的作用以及其功能可能出现紊乱的不同方式。目前治疗这些疾病的可能性有限，但文中给出了该领域已开展的研究实例。
