Occasionally, the cyst drains intermittently and jaundice and pain occur sporadically. This disorder is treatable by surgery if addressed early, before scarring and infection in the liver are present. Paucity of the intrahepatic bile ducts Paucity of bile ducts is characterized by a decreased number of small bile channels inside the liver. This pathological appearance can be seen in a number of abnormalities, including genetic diseases and infections. It may be seen as part of a constellation of malformations known as Alagille syndrome.
Medical management is usually successful.
Genetic and metabolic diseases Hundreds have been described. Abnormal accumulation of byproducts of metabolism or inadequate production of important compounds cause liver dysfunction. This disease is characterized by production of a poorly functioning protein. Most patients with this disease develop emphysema in adulthood. This is one of the most common genetic diseases in this country.