Dastur D K, Manghani D K
Clin Exp Neurol. 1979;16:1-8.
A brief account is given of some aspects of Wilson's disease in India, studied during the periods 1959 to 1967 and 1970 to 1978, with emphasis on disordered parameters of copper metabolism. Among the latter, serum copper oxidase (representing caeruloplasmin) was found to be the most constant laboratory evidence of Wilson's disease. It wad drastically lowered in all patients in both periods (25 and 44 patients respectively), when compared to its level in the parents and siblings of the patients, in other neurological patients and in normal subjects serving as controls. The other constantly involved parameter was direct-reacting serum copper which was highly significantly elevated in the patients compared to the other groups. Most of the patients presented with a predominantly neurological form of the disease, smaller groups presenting with predominantly osseomuscular or hepatic forms. The mean age of patients in both periods was approximately 13 years.
本文简要介绍了1959年至1967年以及1970年至1978年期间在印度对威尔逊氏病某些方面的研究，重点是铜代谢紊乱参数。在这些参数中，血清铜氧化酶（代表血浆铜蓝蛋白）被发现是威尔逊氏病最稳定的实验室证据。与患者的父母、兄弟姐妹、其他神经疾病患者以及作为对照的正常受试者相比，在这两个时期的所有患者中（分别为25例和44例），该酶水平均大幅降低。另一个经常出现异常的参数是直接反应血清铜，与其他组相比，患者的该参数显著升高。大多数患者主要表现为神经型疾病，少数患者主要表现为骨肌肉型或肝型疾病。两个时期患者的平均年龄约为13岁。
