Brewer G J, Hill G M, Dick R D, Nostrant T T, Sams J S, Wells J J, Prasad A S
J Lab Clin Med. 1987 May;109(5):526-31.
Twelve patients with Wilson's disease, most of whom had received intensive treatment with penicillamine, were given zinc therapy as their sole medication for copper control. Serial liver biopsies were performed during a 12- to 20-month follow-up period to determine whether hepatic copper reaccumulates during zinc therapy. Mean baseline liver copper concentration was 255 micrograms/gm dry weight, whereas the mean value after therapy was 239 micrograms. No patient demonstrated hepatic reaccumulation of copper during zinc therapy. Copper balance, 24-hour urinary copper excretion, and nonceruloplasmin plasma copper concentration all indicated good copper control during zinc therapy. Hepatic zinc concentration increased twofold to threefold over baseline values but no toxicity was seen. Hepatic zinc concentrations appeared to reach a plateau after 12 to 18 months of zinc therapy. We conclude that oral zinc as the sole maintenance therapy in patients with Wilson's disease prevents hepatic reaccumulation of copper.
12例威尔逊病患者，其中大多数曾接受青霉胺强化治疗，现给予锌剂作为控制铜的唯一药物治疗。在12至20个月的随访期内进行系列肝脏活检，以确定锌剂治疗期间肝脏铜是否会重新蓄积。平均基线肝脏铜浓度为255微克/克干重，而治疗后的平均值为239微克。在锌剂治疗期间，没有患者出现肝脏铜重新蓄积。铜平衡、24小时尿铜排泄量和非铜蓝蛋白血浆铜浓度均表明锌剂治疗期间铜得到了良好控制。肝脏锌浓度比基线值增加了两倍至三倍，但未观察到毒性反应。锌剂治疗12至18个月后，肝脏锌浓度似乎达到了平台期。我们得出结论，口服锌剂作为威尔逊病患者的唯一维持治疗可防止肝脏铜重新蓄积。
