Kayser-Fleischer rings (KF rings) are seen in

Wilson's disease (hepatolenticular degeneration) is a rare but impoant autosomal recessive disorder of copper metabolism caused by a variety of mutations in the ATP7B gene on chromosome 13. Total body copper is increased, with excess copper deposited in, and causing damage to, several organs. Kayser-Fleischer (KF) rings are a common ophthalmologic finding in patients with Wilson disease. Initially thought to be due to the accumulation of silver, they were first demonstrated to contain copper in 1934. KF rings are seen in most of the patients with neurologic involvement from Wilson disease. These rings are caused by deposition of excess copper on the inner surface of the cornea in the Descemet membrane. A slit lamp examination is mandatory to make a diagnosis of KF rings paicularly in the early stages unless the rings are visible to the naked eye in conditions of severe copper overload. Kayser-Fleischer rings do not cause any impairment of vision but disappear with treatment and reappear with disease progression. KF rings not specific to Wilson disease alone, they are also seen in other chronic cholestatic disorders such as primary biliary cholangitis and children with neonatal cholestasis. Ref - Davidsons internal medicine 23e p896 , Pubmed.com