Abstract
The article presents the structure of diseases that led to the need for liver sector transplantation in children living in the Novosibirsk region. The paper provides a detailed description of progressive familial intrahepatic cholestasis, principles of modern diagnosis and treatment. A description of the medical history of family members and, in particular, a clinical case in a young child with type 2 PFIC (Byler's syndrome) is given.
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