[Wilson's disease]

Abstract

Wilson's disease, also called hepatolenticular degeneration, is a serious inherited disorder of copper metabolism. The disease needs a particular attention because it is estimated that about half of the patients are never diagnosed. Beside, once the diagnosis is made, Wilson's disease can be effectively treated. After the epidemiological, pathogenetical and clinical data, authors present the diagnostical aspects of the disease, and the various approaches to the treatment of the symptomatic or presymptomatic patients and of the pregnant patients. Also reported are two cases of Wilson's disease.