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

Rho, Joo Heon; Jeng, Ji Won; Hahn, Young Ho

Case Report

Journal of the Korean Ophthalmological Society 1997;38(3):504-510.
Published online January 1, 2001.

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

Joo Heon Rho, Ji Won Jeng, Young Ho Hahn

Department of Ophthalmology, Kosin Medical College, Pusan, Korea.

Kayser-Fleischer ring을 동반한 Wilson씨 병 2례

노주헌(Joo Heon Rho),정지원(Ji Won Jeng),한영호(Young Ho Hahn)

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.

Key Words: D-penicillamine;Kayser-Fleischer ring;Wilson's disease