Byler syndrome
| Classification according to ICD-10 | |
|---|---|
| K74.5 | Biliary cirrhosis, unspecified |
| ICD-10 online (WHO version 2019) | |
The Byler syndrome (also progressive familial intrahepatic cholestasis ( PFIC ), or Byler disease called) is a genetic disease of the liver ( autosomal - recessive inherited). The excretion of bile acids is disturbed. A distinction is made between type I and type II.
Treatment consists of replacing fat-soluble vitamins and following a special diet . Only the transplantation of a healthy liver leads to healing. Without a liver transplant, however , the prognosis is poor: only a few children reach the age of ten.
