Silverstone B Z, Nawratzki I, Berson D, Yanko L
Department of Ophthalmology, Shaare Zedek Medical Center, Jerusalem, Israel.
Metab Pediatr Syst Ophthalmol (1985). 1986;9(1):589-91.
Abnormalities in the zinc and copper metabolism have been described in some pigmentary retinopathies. In these eye disorders changes in the Retinal Pigment Epithelium are a common finding. Albinism is a manifestation of a metabolic disorder with abnormalities of pigment formation which also occurs in this mentioned retinal layer. Recently, we described abnormalities in the metabolism of these metals in black albino patients. In them, elevated values of ceruloplasmin were found. In the present study we performed a similar work among Caucasians. When both the Caucasian albino and control groups were compared, no statistical difference in the ceruloplasmin, serum zinc and copper and their concentration in urine was present. However, in 7 patients of the Caucasian albino group the serum zinc was distinctly elevated. The mean concentration of ceruloplasmin was high as well. No definitive correlation in regard to the positivity or negativity of tyrosinase was observed. Our findings may support our previous observations regarding albinism as a possible primary abnormality of zinc and copper metabolism.
在一些色素性视网膜病变中，锌和铜代谢异常已有相关描述。在这些眼部疾病中，视网膜色素上皮的变化是常见现象。白化病是一种色素形成异常的代谢紊乱表现，同样也发生在上述视网膜层。最近，我们描述了黑种白化病患者中这些金属的代谢异常。在他们当中，发现血浆铜蓝蛋白值升高。在本研究中，我们在白种人中开展了类似工作。当对白种白化病患者组和对照组进行比较时，血浆铜蓝蛋白、血清锌和铜及其尿中浓度没有统计学差异。然而，在白种白化病患者组的7名患者中，血清锌明显升高。血浆铜蓝蛋白的平均浓度也很高。未观察到酪氨酸酶阳性或阴性的明确相关性。我们的发现可能支持我们之前关于白化病可能是锌和铜代谢原发性异常的观察结果。
