Fleming C R, Dickson E R, Wahner H W, Hollenhorst R W, McCall J T
Ann Intern Med. 1977 Mar;86(3):285-8. doi: 10.7326/0003-4819-86-3-285.
Kayser-Fleischer rings are pigmented corneal rings at the limbus of the cornea in Descemet's membrane that have been deemed pathognomonic of Wilson's disease. However, we have observed four exceptions in patients with non-Wilsonian liver disease. Three patients had primary biliary cirrhosis and one patient had chronic aggressive hepatitis with cirrhosis. Pigmented corneal rings were seen only by slit-lamp examination. Hepatic, serum, and urinary copper and serum ceruloplasmin levels were significantly elevated in the patients with primary biliary cirrhosis. Radiocopper (64Cu or 67Cu) studies in patients with primary biliary cirrhosis showed plasma disappearance curves which allowed a clear distinction from Wilson's disease in that all three patients with primary biliary cirrhosis showed a secondary rise in radiocopper that presumably represented copper incorporation into ceruloplasmin. In one patient, in whom 64Cu in ceruloplasmin was studied specifically, incorporation was found to be normal.
凯泽-弗莱舍尔环是角膜缘处位于后弹力层的色素沉着角膜环，被认为是威尔逊病的特征性表现。然而，我们在非威尔逊病性肝病患者中观察到了4例例外情况。3例患者患有原发性胆汁性肝硬化，1例患者患有慢性侵袭性肝炎伴肝硬化。色素沉着角膜环仅通过裂隙灯检查才能看到。原发性胆汁性肝硬化患者的肝脏、血清和尿液铜以及血清铜蓝蛋白水平显著升高。对原发性胆汁性肝硬化患者进行的放射性铜（64Cu或67Cu）研究显示，血浆消失曲线与威尔逊病有明显区别，因为所有3例原发性胆汁性肝硬化患者的放射性铜均出现二次升高，这可能代表铜掺入铜蓝蛋白。在1例专门研究铜蓝蛋白中64Cu的患者中，发现掺入情况正常。
