PMID: 6981794Jan 1, 1982Paper

Bile duct paucity ("intrahepatic atresia")

Perspectives in Pediatric Pathology
C L Witzleben

Abstract

Reduction of the number of interlobular bile ducts (BDP) has potentially significant physiologic consequences. Most of the disorders currently recognized as BDP involve an easily discernible reduction in duct numbers, but actual duct quantitation needs to be done routinely in the examination of liver biopsies in order to recognize and assay the importance of less marked reduction. Ducts are seldom if ever completely absent, and may not be uniformly reduced throughout the liver, so a wedge biopsy is the only completely satisfactory sample for the morphologic evaluation of duct numbers. In some conditions the number of ducts varies greatly with time. BDP may be only one aspect or manifestation of a disease primarily characterized by other features, or it may be the principal cause of distress and a major feature in defining a disorder. In a few conditions, the mechanism of the paucity has been quite well established as the destruction of previously existing ducts, but at present the basis for duct paucity in most conditions remain obscure. The treatment of BDP is similar in all conditions. Because of the wide range of circumstances in which BDP occurs, the prognosis is highly variable and depends on the underlying condition. In s...Continue Reading

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