PubMed HealthSearch

PubMed · 1345477

Biliary atresia.

Abstract

Although biliary atresia is characterized by luminal obstruction of the extrahepatic bile ducts, the etiology and the pathophysiology of the liver are still controversial. The prognosis of biliary atresia has been improved after the introduction of Kasai's hepatic portoenterostomy, but there are still many problems to be solved in the treatment of this disease. Successful results of hepatic portoenterostomy depend on early diagnosis and operation, adequate operative technique, prevention of postoperative cholangitis, and precise postoperative management. However, we are on the verge of a new era in the therapy of biliary atresia combining portoenterostomy with liver transplantation.

Explore related subjects

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

R Ohi, M Ibrahim. 1992. Biliary atresia.. https://pubmed.ncbi.nlm.nih.gov/1345477/

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

Recurrence of extrahepatic biliary atresia in two half sibs.

Extrahepatic biliary atresia (EHBA) usually is a sporadic disorder. Familial cases, including occurrence in sibs and twins, have been reported. We report the first recurrence of EHBA in two half sibs born to a common father and unrelated mothers and suggest the possibility of gonadal mosaicism for a new dominant mutation.

Biliary Atresia

Hepatic transplantation in children using reduced size allografts.

The shortage of paediatric liver donors has led to the use of reduced size hepatic allografts. Between July 1987 and July 1990, 30 reduced size orthotopic liver transplantations were performed in 24 children aged between 3 months and 7 years. All patients were in advanced chronic or acute liver failure and were considered unlikely to survive for long enough for a size-matched donor to become available. The most common indication was biliary atresia. The median intraoperative blood loss was 75 (range 13-1015) ml kg-1. Nine patients have died and seven have undergone retransplantation, four successfully. Seven patients had portal vein hypoplasia with a high graft failure rate due to ischaemic infarction. There was significant morbidity from biliary tract complications, leading to further operations in four cases. The 1-year actuarial survival rate was 62 per cent.

Biliary Atresia

Hepatitis B vaccine response before and after transplantation in 55 extrahepatic biliary atresia children.

Fifty-five children with cholestatic cirrhosis due to extrahepatic biliary atresia received a course of hepatitis B vaccine. Forty-seven received a plasma-derived vaccine and eight a recombinant vaccine. Antibody determination was evaluated before and after liver transplantation in 30 patients. Twenty-five additional patients had antibody determination after transplantation only. Protection against hepatitis B was observed in 73.3% of the children evaluated prior to transplantation. One to 15 months after transplantation, 54.6% of all children studied showed protective levels of anti HBs. We conclude that hepatitis B vaccination is efficient in inducing immunity in the majority of children with cholestatic cirrhosis. Some patients will loose immunity under immunosuppression, but the protection rate remains higher than reported for patients vaccinated after transplantation.

Biliary Atresia