摘要：

This chapter discusses the clinical feature, pathology, and management of familial and genetic structural abnormalities of the liver and biliary system, such as infantile polycystic disease (IPCD) of the kidneys and liver and congenital hepatic fibrosis. IPCD of the kidneys and liver is characterized by renal and hepatic enlargement because of replacement of the renal parenchyma by cysts and fibrous tissue, and periportal hepatic fibrosis with dilatation of the bile ducts. The disorder is inherited in an autosomal recessive fashion. It has thus been distinguished from the adult type of polycystic disease, which is inherited in an autosomal dominant fashion, usually with a high degree of penetrance. In IPCD, treatment is thus directed at controlling infection and congestive cardiac failure in infancy, symptomatic treatment of disturbances in fluid electrolyte balance induced by renal failure, and systemic hypertension. Congenital hepatic fibrosis is a condition defined pathologically by the presence within the liver of bands of fibrous tissue, which often contain linear or circular spaces lined by bile duct cells. Treatment consists of antibiotics to control cholangitis, but it is rarely possible to produce complete sterility of the bile. As there is no mechanical obstruction, surgery is contraindicated unless there is very clear evidence of secondary bile duct obstruction because of calculi.

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