Kayser-Fleischer Ring in Wilson's Disease.
- Author:
Hong Bok KIM
1
;
Ji Cheon KIM
;
Young Ja BYUN
Author Information
1. Department of Ophthalmology, Yonsei University, College of Medicine, Seoul, Korea.
- Publication Type:Case Report
- MeSH:
Absorption;
Adolescent;
Ceruloplasmin;
Copper;
Cornea;
Deception;
Fibrosis;
Gastrointestinal Tract;
Hepatolenticular Degeneration*;
Humans;
Liver;
Male;
Membranes;
Metabolism;
Microscopy, Electron;
Nervous System
- From:Journal of the Korean Ophthalmological Society
1979;20(1):129-133
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Wilson's disease is a familial disease characterized by coarsely nodular cirrhosis of the liver, sometimes associated with progressive damage to the nervous system, and the appearance of a coloured ring in the cornea. Wilson's disease is an inborn error of copper metabolism in which the synthesis of ceruloplasmin, with which copper forms a table compound in the blood, is diminished and there is an increased absorption of copper from the gastrointestinal tract together with an increased output in the urine. The Kayser-Fleischer ring is originally described by Kayser(1902) and later by Fleischer(1903). The colours of Kayser-Fleischer ring is seen, varing from rub by red to bright green or an 1lltramarine blue. sometimes interspersed with yellow or smoky shade of brown. The ring starts dose to the limbus as a sharp line just where the endothelial pattern begins to be seen distinctly, extends over a breadth of I to 3mm.. and gradually fades away towards the center of the cornea. Harry et al(1970) described that electron microscopy showed the presence of electron dense deposits of varing size lying mainly in Descmet's membrane and confirmed that the deposits were copper. A 14 year old korean boy with Wilson's disease was found to have Kayser-Fleischer ring in the cornea(ou) and greenish brown pigments on the anterior surface of the lens (ou). In about 16 months after the administration of penicillamine(chelating agent), greenish brown pigments on the anterior surface of the lens (ou) were disappeared remaining Kayser Fleischer ring unchanged.
