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视网膜色素变性(视网膜毯层变性)病因不明。有人提出无β脂蛋白血症、自体免疫、维生素A结合蛋白异常等有关。Gahlot(1967)等发现患者的血浆铜蓝蛋白含量降低和尿铜排泄增加,认为此病之原发缺陷是铜代谢异常。而美国和丹麦学者未发现此异常。Marmor指出Gahlot来自印度,所研究的是有铜代谢异常的遗传隔离(genetic islIate)患者。作者报告一组病例约1/3患者有铜代谢异常。作者对24例患者的血清铜、血浆铜蓝蛋白及尿铜做了测定,以13例正常人者作对照。结果为:正常人血清铜含量为67.5～150.0μg/100ml,平均为102.4μg/100ml;而患者为75～206.2μg100ml,平均为135.7μg/100ml。较正常者明显为高。正常人血浆铜蓝蛋白为19.8～69.0 mg/
Retinitis pigmentosa (retinal degeneration) etiology unknown. It was suggested that no beta-lipoproteinemia, autoimmune, vitamin A-binding protein abnormalities and so on. Gahlot (1967) found that patients with decreased plasma ceruloplasmin and urinary copper excretion increased, that the disease is the primary defect of copper metabolism. The United States and Danish scholars did not find this anomaly. Marmor pointed out that Gahlot came from India and looked at genetic islIate patients with abnormal copper metabolism. The authors report that about one-third of patients in a group of patients have abnormal copper metabolism. The authors of 24 patients with serum copper, ceruloplasmin and urine copper were measured to 13 normal controls. The results were as follows: normal serum copper content of 67.5 ~ 150.0μg / 100ml, with an average of 102.4μg / 100ml; and patients with 75 ~ 206.2μg100ml, with an average of 135.7μg / 100ml. Obviously higher than normal. Normal human ceruloplasmin 19.8 ~ 69.0 mg /