J Korean Ophthalmol Soc.  1997 Mar;38(3):504-510.

Two Cases of Wilson's Disease Associated with Kayser-Fleicher Ring

Affiliations
  • 1Department of Ophthalmology, Kosin Medical College, Pusan, Korea.

Abstract

Wilson's disease is an inborn error of copper metabolism which may be associated with hepatic cirrhosis and progressive degeneration of the central nervous system. The most common ophthalmologic finding in Wilson's disease is the Kayser-Fleischer ring. Other much less common physical signs include neurologic signs, endocrinologic abnormality. The Kayser-Fleischer ring occurs in the corneal periphery and is usually yellow- brown color. The Kayser-Fleischer ring.copper deposition at the level of the posteior position of Descemet's membran. The authours have recently experienced two cases of wilson's disease. One case. a 20-year-old girl, has Kayser-Fleischer rings in both eyes, amenorrhea, chronic active hepatitis and the other case, a 21-years-old girl, has dense yellow-green colored Kayser-Fleischer rings in both eyes, palilalia, and family relationship. Both cases have been treated with D-penicillaine and low copper diet. After treatment, clinical manifestation have been improved markedly at former case and the other cae is steady stage, but the Kayse-Fleischer rings have not disappeared yet in both cases.

Keyword

D-penicillamine; Kayser-Fleischer ring; Wilson's disease

MeSH Terms

Amenorrhea
Central Nervous System
Copper
Diet
Family Relations
Female
Hepatitis, Chronic
Hepatolenticular Degeneration*
Humans
Liver Cirrhosis
Metabolism
Neurologic Manifestations
Penicillamine
Young Adult
Copper
Penicillamine
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