593 UNIQUE HEPATIC PATHOLOGY IN CHILDREN WITH BOTH EXTRAHEPATIC BILIARY ATRESIA (EHBA) AND ARTERIOHEPATIC DYSPLASIA (AHD)

Congenital Extrahepatic Biliary Atresia

Case 1. The elder of two sibs, this boy was born on May 21, 1965 to unrelated healthy parents aged 29 (mother) and 30 years (father). Birthweight was 2820 g.; delivery was by forceps. His mother had 'influenza' between the fifth and sixth months of the pregnancy which had been otherwise normal. There was no previous foetal loss. Four generations of the family, comprising, in addition to the par...

Extrahepatic Biliary Atresia

[Biliary atresia].

Extrahepatic biliary atresia (EHBA) is an inflammatory fibrosing process affecting the extrahepatic and intrahepatic biliary tree resulting in fibrous obliteration of the extrahepatic biliary tract, ductopenia of intrahepatic bile ducts, and biliary cirrhosis. EHBA is divided in a fetal, prenatal or embryonic, and a more common, perinatal form. The symptoms of the fetal form start shortly after...

Aflatoxins in Infants with Extrahepatic Biliary Atresia

Background: Aflatoxin B 1 induced hepatitis was reported to be associated with characteristic features of centrizonal scarring, hepatic venous occlusion, ductular proliferation and cholestasis, focal syncytial giant cell giant cell transformation of hepatocytes, and pericelluar fibrosis which is congruent to biopsy findings in extra hepatic biliary atresia (EHBA). Aim of Work: Is to study aflat...

Prognosis of extrahepatic biliary atresia.

We carried out a retrospective investigation of the 89 patients with extrahepatic biliary atresia born in The Netherlands during a 10 year period. Of these 89 patients 10 had a diagnostic laparotomy only. Eight patients had an anastomosis between the proximal bile duct and the intestine, and the remaining 71 had hepatic portoenterostomies. Bile drainage was re-established in 46 (65%). After suc...