论文部分内容阅读
本研究包括20名病人。男11人,女9人。年龄10至62岁。分两组:第一组15名视网膜色素变性病人;第二组4名其他视网膜变性病人(未定型的毯层视网膜变性1例,Stargardt变性1例,斑点状视网膜综合征1例,白点状眼底1例)和1名有视网膜色素变性家族倾向而无视网膜变性的病人。所有病人均测定了血清铜量和血浆中转移铜蛋白量。有9名视网膜色素变性病人测定了尿内铜量。测定结果,所有病人血清铜量均正常,平均值为117.8μg/100ml(正常范围:男74—146,女83—159μg/100ml)。血浆中转移铜蛋白量,除2名病例(此2例转移铜蛋白量升高,1例为0.44g/l,另1例为0.57g/l)外,其余病例均正常,其平均值为0.346g/l(正常为0.18—0.42g/l)。9例测定24小时尿内铜量均正常,平均为43.6μg/24小时(正常为9—80μg/24小时)。
The study included 20 patients. 11 men and 9 women. Ages 10 to 62. Divided into two groups: the first group of 15 patients with retinitis pigmentosa; the second group of 4 other patients with retinal degeneration (untreated blanket retinal degeneration in 1 case, Stargardt degeneration in 1 case, macular retinal syndrome in 1 case, white spots 1 case of fundus) and 1 patient who had a propensity for retinitis pigmentosa without retinal degeneration. All patients were measured serum copper and plasma copper transfer protein. Nine patients with retinitis pigmentosa were assayed for urinary copper levels. The results showed that all patients had normal levels of serum copper with an average of 117.8 μg / 100 ml (normal range: 74-146 males and 83-159 μg females). Except for 2 cases, the amount of copper protein in plasma was normal except for 2 cases (1 case was 0.44g / l in one case and 0.57g / l in another case), and the average value was 0.346 g / l (normally 0.18-0.42 g / l). Nine cases of urinary copper in 24 hours were normal, with an average of 43.6μg / 24 hours (normal 9-80μg / 24 hours).