Hepatic fibrosis is the tissue response to any kind of chronic liver injury. Matrix components accumulate extracellularly because of an imbalance in their production, deposition, and degradation. Portal hypertension and impaired liver function are the inevitable consequences.
A congenital form of hepatic fibrosis (CHF) can be distinguished from an acquired form (cirrhosis).
|Autosomal dominant polycystic kidney disease|
Sometimes in ADPKD a CHF may develop as usually observed in ARPKD.
|Autosomal recessive polycystic kidney and hepatic disease 1|
The liver shows the typical signs of CHF in patients with ARPKD.