Secondary Biliary Cirrhosis

Chronic biliary obstruction does evolve into cirrhosis. The overall appearance is similar to that seen in primary biliary cirrhosis, except for increased bile stasis and a lack of duct degeneration.
Liver involvement is uncommon in children with cystic fibrosis, but increasing survival of these patients has led to an increased number presenting with liver disease. These older children and young adults develop focal biliary cirrhosis, which eventually evolves into portal hypertension (Fig. below). Some cystic fibrosis patients have undergone liver transplantation. Whether screening is useful in cystic fibrosis patients with chronic liver disease prior to
developing cirrhosis remains to be established.
A cirrhotic liver in a 24-year-old man with cystic fibrosis and hepatic failure. The liver has a nodular outline. Splenomegaly and vascular congestion (arrowheads) are secondary to portal hypertension.
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