Wilson's disease -Two clinicopathological features with different prognosis-

Abstract

For better understanding the disease entity, we evaluated the two clinico-pathological features, acute and non-acute forms of Wilson's disease. The liver histology of non-acute form was characterized by various combinations of parenchymal fatty change and portal fibrosis. The prognosis of non-acute form was excellent when penicillamine was administered. Patients with acute form died from acute hepatic failure associated with renal failure. The liver pathology specific to the non-acute form was superimposed by cholestasis, coagulative necrosis, submassive collapse, and poor regeneration. The data suggest that diagnosis before acute manifestation is essential in patients with Wilson's disease.