Byler syndrome
Classification according to ICD-10 | |
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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.