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