What is another word for Hepatic Form of Wilson Disease?
Pronunciation: [hɛpˈatɪk fˈɔːm ɒv wˈɪlsən dɪzˈiːz] (IPA) 
The hepatic form of Wilson disease, also known as hepatic copper toxicosis, is a rare genetic disorder characterized by copper buildup in the liver. Synonymous terms reflecting this condition include Wilson disease with predominantly hepatic presentation and hepatic Wilson disease. This condition stems from a mutation in the ATP7B gene, responsible for the production of a copper-transporting protein. Consequently, copper accumulates in liver cells, leading to hepatocyte damage and clinical manifestations such as jaundice, hepatomegaly, and liver failure. Proper diagnosis and treatment with copper-chelating agents such as D-penicillamine or trientine are crucial to prevent long-term complications and preserve liver function. Regular monitoring of copper levels and liver function is paramount to managing this form of Wilson disease effectively.
