How long does a hepatomegaly live?

Patients with hepatomegaly, diagnosed early and treated with timely copper expulsion, usually do not affect their survival and quality of life, while a few patients with severe disease have a poor prognosis. Hepatomegaly is a hereditary disorder of copper metabolism resulting in cirrhosis and a degenerative disease of the brain with a predominantly basal nucleus. Patients mainly present with chorea-like and tachycardia-like movements of the limbs, motor retardation, swallowing difficulties; emotional disturbances and behavioral abnormalities; liver damage manifested as chronic liver disease syndrome, such as lethargy, lack of appetite, and pain in the liver area; as well as ocular K-F rings, skin pigmentation, and renal function impairment. If detected early and given standardized treatment, they can generally survive for a long time. However, if timely treatment is not given and serious complications occur, such as cirrhosis, hepatic encephalopathy, gastrointestinal hemorrhage, or even brain herniation, survival can be significantly shortened, and death may even occur within weeks to months. The principles of treatment for patients with hepatomegaly are low copper diet, reduction of copper absorption and increase of copper elimination with drugs, and the earlier the treatment, the better.