Clinical practice: Management of biliary atresia

Biliary atresia is a rare, serious and challenging disease in newborn children. Its aetiology remains unknown. Optimal management at specialist centres with resultant better overall outcomes is achieved through a multidisciplinary team approach. The Kasai portoenterostomy performed early in life rem...

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Bibliographic Details
Published in:European journal of pediatrics 2010-04, Vol.169 (4), p.395-402
Main Authors: Khalil, Basem A., Perera, M. Thamara P. R., Mirza, Darius F.
Format: Article
Language:English
Subjects:
Biliary Atresia - complications
Biliary Atresia - diagnosis
Biliary Atresia - surgery
Biological and medical sciences
Child
Cholangitis - etiology
Drug therapy
General aspects
Humans
Liver Function Tests
Liver, biliary tract, pancreas, portal circulation, spleen
Medical sciences
Medicine
Medicine & Public Health
Pediatrics
Portoenterostomy, Hepatic - methods
Preoperative Care
Review
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Surgery of the digestive system
Transplants & implants
Citations: Items that this one cites
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Description
Summary:Biliary atresia is a rare, serious and challenging disease in newborn children. Its aetiology remains unknown. Optimal management at specialist centres with resultant better overall outcomes is achieved through a multidisciplinary team approach. The Kasai portoenterostomy performed early in life remains the only surgical repair procedure. Two thirds of patients will clear their jaundice after a Kasai procedure, but only about one third will retain their livers after the first decade of life. Failure of this procedure leaves liver transplantation as the only chance for survival, and this disease is the commonest indication for liver transplantation in children. With modern medical care and refinements in surgical techniques, survival after either or both of these procedures is about 90%. Early referral to specialist centres and long-term specialist care remains the key to successful treatment of this condition.
ISSN:0340-6199
1432-1076
DOI:10.1007/s00431-009-1125-7