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Hepatobiliary lesions in cystic fibrosis

Liver Disorders in Childhood, Page: 349-360
1994
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Book Chapter Description

This chapter discusses the clinical features, management, and diagnosis of hepatobiliary lesions in cystic fibrosis. Cystic fibrosis (CF) is the most common lethal genetic disease in Caucasian populations. It is a generalized disorder of exocrine glands, inherited in an autosomal recessive pattern. The characteristic hepatic lesion in cystic fibrosis is usually termed focal biliary cirrhosis, although it starts as a focal biliary fibrosis without derangement of hepatic architecture or nodular regeneration. There is accumulation in the intrahepatic ducts of amorphous eosinophilic PAS-positive diastase-resistant material, which on electron microscopy is shown to contain lipid and bile pigment and to have a filamentous structure. This material obstructs some intrahepatic bile ducts. The portal tracts draining to these develop edema, chronic inflammatory cell infiltrate, bile duct proliferation, and increased fibrosis. The child with cystic fibrosis is susceptible to all other forms of chronic liver disease in childhood. It is particularly important to consider the possibility of correctable disorders such as Wilson's disease, choledochal cyst, and constrictive pericarditis. The coexistence of autoimmune chronic active hepatitis should also be considered.

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