1980 Volume 21 Issue 10 Pages 1366-1378
A post-mortem case of "Absence of the Intrahepatic Bile ducts" of a female infant of 1.5 years of age was presented.
The Jaundice was noticed on the third day of life and persisted until death. "Congenital Extrahepatic Biliary Atresia" Was diagnosed clinically.
Wedge biopsy specimen of the liver at 2.5 months of age revealed the presence of interlobular bile ducts in half of 14 portal triads examined and destructive or degenerative changes in different degrees of the bile ducts (i.e disarrangement of the epithelium and nuclei, degeneration, scarring and disappearing of the bile ducts). Liver cells showed ballooning degeneration with occasional giant cell transformation. Neither fibrosis or proliferation of bile ducts was observed.
At autopsy, the extrahepatic biliary system was patent though hypoplastic. Intrahepatic bile ducts was completely absent; any trace of the bile duct could not be seen in all liver sections examined. Hepatic fibrosis and cholestasis were slight to moderate in centrilobular areas. However, there was no evidence of periportal fibrosis or proliferation of bile buct.
These findings are apparently different from liver change of "Congenital Extrahepatic Biliary Atresia", and compatible with those of "Alagine Syndrome" and/or "Paucity of the Intrahepatic Bile Ducts".
Intrauterine infection was suggested as its etiology.
This report seems to be the first autopsy report of the disease in Japan.
