Non-invasive quantitation of corneal copper in hepatolenticular degeneration (Wilson's disease).
The corneal copper content was measured by X-ray excitation spectrometry in two controls and in seven patients with Wilson's disease. Patients who were treated irregularly or not treated at all showed a high corneal copper content. Those who were adequately treated had low levels, comparable to the controls. In one case the corneal copper content declined 45%, after a course of dimercaprol. The corneal copper measured showed no correlation with the slit-lamp appearance of the Kayser-Fleischer ring. It is suggested that non-invasive X-ray excitation spectrometry can provide a fast and reliable method for the early diagnosis of Wilson's disease and for the objective evaluation of the efficacy of the treatment of this disease.
D000293:Adolescent; D000328:Adult; D003300:Copper / Q000032:analysis* / Q000378:metabolism; D003315:Cornea / Q000032:analysis*; D004112:Dimercaprol / Q000627:therapeutic use; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D006801:Humans; D008875:Middle Aged; D013050:Spectrometry, Fluorescence / Q000295:instrumentation
D004112:Dimercaprol; D003300:Copper