Restrictive Cardiomyopathy in a Patient with Extrahepatic Biliary Atresia

Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia.

The most commonly associated anomalies in patients with extrahepatic biliary atresia are cardiovascular, digestive and splenic defects. Of the cardiovascular anomalies, there are very few reports of biliary atresia with cardiomyopathy. We report the first case of a child with extrahepatic biliary atresia and restrictive cardiomyopathy. The patient was a 13-month-old boy diagnosed with extrahepa...

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

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...