There are three main types of extra-hepatic<unk> : * Type I:<unk>  is restricted to the common bile duct. * Type II:<unk>  of the common hepatic duct. * Type III:<unk>  involves the most proximal part of the bile ducts (>95% of all cases). In approximately 10% of cases, other anomalies may be associated with<unk> . The most common of these syndromic forms is<unk> SM and might include heart lesions, polysplenia, situs inversus, absent venae cavae, and a preduodenal portal vein. Progressive cirrhosis is associated with signs and symptoms of portal hypertension, such as esophagogastric varix bleeding, hypersplenism, hepatorenal syndrome, and hepatopulmonary syndrome. In an Egyptian study, abnormally high levels of aflatoxin B1 and to a lesser extent aflatoxin B2 was found in liver tissue and blood of all neonates suffering from<unk> . Aflatoxins may cause extensive damage to the hepatocytes leading to hepatitis and damage to bile ducts causing inflammation, adhesions and final obstruction of bile ducts. The affected neonates have a genetic detoxification defect that does not allow them to detoxify these aflatoxins timely or effectively. The<unk> bies have homozygous deficiency of glutathione S transferase (GST) M1. The aflatoxin damaged liver cells and bile duct cells are removed by neutrophil elastase and by involvement of immune system mediators such as CCL-2 or MCP-1, tumor necrosis factor (TNF), interleukin-6 (IL-6), TGF-beta, endothelin (ET), and nitric oxide (NO). Among these, TGF-beta is the most important pro-fibrogenic cytokine that can be seen in progressive cirrhosis. The cascade of immune involvement to remove damaged hepatocytes and cholangiocytes ushers regeneration. Yet in infants with<unk>  regeneration is defective, and results in cirrhosis, as these infants have disrupted p53 and disrupted GSTPi. p53 and GSTPi are responsible for DNA fidelity at regeneration. Hence, these infants get accelerated cirrhosis and march to portal hypertension.
Biliary atresia
Vanishing bile duct syndrome
Neonatal cholestasis
Congenital hepatic fibrosis
Neonatal jaundice
Progressive familial intrahepatic cholestasis
Biliary disease
Choledochal cysts