Frommer D, Morris J, Sherlock S, Abrams J, Newman S
Gastroenterology. 1977 Jun;72(6):1331-5.
Three patients, one with cryptogenic cirrhosis, one with active chronic hepatitis and one with neonatal hepatitis, were found to have corneal pigmentation rings indistinguishable from early Kayser-Fleischer rings on slit lamp examination. They did not have the clinical features of Wilson's disease and their serum copper and ceruloplasmin concentrations were normal. Urinary copper excretion rates and hepatic concentrations were only slightly raised but were below the range found in symptomatic Wilson's disease. It is concluded that the Kayser-Fleischer ring would no longer be considered as pathognomonic of Wilson's disease.
三名患者,一名患有隐源性肝硬化,一名患有活动性慢性肝炎,一名患有新生儿肝炎,在裂隙灯检查中发现他们有与早期凯-弗环难以区分的角膜色素沉着环。他们没有威尔逊病的临床特征,血清铜和铜蓝蛋白浓度正常。尿铜排泄率和肝脏铜浓度仅略有升高,但低于有症状的威尔逊病患者的范围。得出的结论是,凯-弗环不再被视为威尔逊病的特征性表现。