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Possible Defect in the Bile Secretory Apparatus in Arteriohepatic Dysplasia (Alagille's Syndrome): A Review with Observations on the Ultrastructure of Liver
Author(s) -
ValenciaMayoral Pedro,
Weber James,
Cutz Ernest,
Edwards Ver D.,
Phillips M. James
Publication year - 1984
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.1840040422
Subject(s) - cholestasis , alagille syndrome , bone canaliculus , ultrastructure , pathology , golgi apparatus , dysplasia , intrahepatic bile ducts , biology , liver biopsy , medicine , biopsy , bile duct , microbiology and biotechnology , endoplasmic reticulum
Ultrastructural observations on 12 liver biopsies from 10 patients with arteriohepatic dysplasia syndrome (Alagille's syndrome) are reported. The electron microscopic changes in the liver in this condition are different from those seen in other forms of chronic intra‐ and extrahepatic cholestasis. In particular, the bile canalicular and pericanalicular changes classically observed in cholestasis are infrequently seen. When compared with other forms of intrahepatic cholestasis including syndromes associated with paucity of intrahepatic bile ducts, the ultrastructural changes in Alagille's syndrome appear to be distinctive. Bile pigment retention is found in the cytoplasm especially in lysosomes and in vesicles of the outer convex face of the Golgi apparatus (cis‐Golgi), but rarely in bile canaliculi or the immediate pericanalicular region. These results suggest a block in the Golgi apparatus or in the pericanalicular cytoplasm.