vitalwiki

Первинний склерозуючий холангіт

ORPHA:171· ICD-10 K83.0· Primary sclerosing cholangitis

Визначення(English summary)

Primary sclerosing cholangitis (PSC) is a rare, slowly progressive liver disease characterized by inflammation and destruction of the intra- and/or extra-hepatic bile ducts that lead to cholestasis, liver fibrosis, liver cirrhosis and ultimately liver failure.

Поширеність
1-9 / 100 000
Успадкування
Multigenic/multifactorial
Вік початку
Adolescent, Adult, Childhood, Elderly