Sclerosing cholangitis in children
Sclerosing cholangitis, also known as fibrous cholangitis, is a cholestatic syndrome caused by inflammation of the intrahepatic and extrahepatic bile ducts, which leads to fibrous degeneration and proliferation of the submucosal and serosal layers of the intrahepatic and extrahepatic bile duct walls, resulting in chronic fibrous stenosis and occlusion of the bile ducts. It can eventually develop into biliary cirrhosis and portal hypertension. In a few cases, the gallbladder and pancreatic duct may be involved, and it may be accompanied by chronic ulcerative colitis or retroperitoneal fibrosis.