congenital hepatic fibrosis वाक्य

Congenital Hepatic Fibrosis ( CHF ) is hallmark of COACH syndrome.
Congenital hepatic fibrosis usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life.
Embryogenically, congenital hepatic fibrosis is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive hepatocytes, which differentiate into cholangiocytes.
After reviewing 46 cases of Caroli disease before 1990, it was found that 21.7 % of the cases were the result of an intraheptic cyst or non-obstructive biliary tree dilation, 34.7 % were linked with congenital hepatic fibrosis, 13 % were isolated choledochal cystic dilation, and the remaining 24.6 % had a combination of all three.