Diagnosis and treatment of hepatolenticular degeneration

Abstract

Hepatolenticular degeneration (Wilson-Konovalov disease or hepatolenticular disease) is a rare hereditary disease caused by impaired copper metabolism and copper accumulation in the liver and, subsequently, in other organs. The disease primarily affects the liver and the central nervous system (lentil nuclei, cortex and subcortex) and is manifested in brown-green pigmentation around the cornea and in kidney lesions.

Keywords

• болезнь вильсона – коновалова
• церулоплазмин
• гепатоциты
• фосфолипиды
• wilson-konovalov disease
• ceruloplasmin
• hepatocytes
• phospholipids