Benson G D
Yale J Biol Med. 1979 Jan-Feb;52(1):83-8.
Hepatic copper accumulation is a regular feature of primary biliary cirrhosis (PBC). The levels are directly related to the clinical stage of the disease. Since the copper values in PBC are comparable to Wilson's disease, there is the potential for copper toxicity, although this is speculative since the two diseases differ in the binding, distribution, and intracellular localization of the copper. The involvement of copper toxicity in the progression of PBC is supported by the observation that the highest values occur in association with the hepatic failure that occurs in the advanced stage.Corticosteroid therapy appears to decrease hepatic copper levels in PBC. Although this therapy does not invariably lower the hepatic Cu content in patients with PBC, it does so in many individuals. Therapeutic trials with d-penicillamine are in progress. When results are available they will guide us in the management of individual patients with PBC. In the meantime, dietary copper should be restricted as is done in management of Wilson's disease.
肝铜蓄积是原发性胆汁性肝硬化（PBC）的一个常见特征。其水平与疾病的临床阶段直接相关。由于PBC中的铜值与威尔逊病相当，存在铜毒性的可能性，尽管这只是推测，因为这两种疾病在铜的结合、分布和细胞内定位方面存在差异。晚期发生的肝衰竭伴有最高的铜值，这一观察结果支持了铜毒性在PBC进展中的作用。皮质类固醇疗法似乎可降低PBC患者的肝铜水平。虽然这种疗法并非总能降低PBC患者的肝铜含量，但在许多患者中确实有效。d-青霉胺的治疗试验正在进行中。当有结果时，将指导我们对PBC个体患者的管理。同时，应像治疗威尔逊病一样限制饮食中的铜摄入。
