The Kayser-Fleischer ring during long-term treatment in Wilson's disease (hepatolenticular degeneration). A follow-up study.
The role of Kayser-Fleischer rings is described in 67 patients with Wilson's disease, both in the asymptomatic and symptomatic stage of the disease during life-long therapy, which lasted up to 22 years. The rings were missing in 60% of patients in the presymptomatic stage and in 2% of those in the symptomatic stage at the time of diagnosis. The Kayser-Fleischer rings disappeared in 81% of the patients (completely in 41% and incompletely in 59%). In 2 of the 6 asymptomatic patients the rings did not reabsorb even after therapy of more than 10 years. The fading of Kayser-Fleischer rings seems to be independent not only of the stage of the disease but also of the effectiveness of the decopperizing treatment.
D001480:Basal Ganglia Diseases / Q000209:etiology; D002526:Cerebellar Diseases / Q000209:etiology; D002614:Chelating Agents / Q000627:therapeutic use; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008107:Liver Diseases / Q000209:etiology
D002614:Chelating Agents