Biliary atresia: Clinical advances and perspectives

Clinics and Research in Hepatology and Gastroenterology
Laure NizeryMuriel Girard

Abstract

Biliary atresia (BA) is a rare and severe inflammatory and obliterative cholangiopathy that affects both extra- and intrahepatic bile ducts. BA symptoms occur shortly after birth with jaundice, pale stools and dark urines. The prognosis of BA has dramatically changed in the last decades: before the Kasai operation most BA patients died, while nowadays with the sequential treatment with Kasai operation±liver transplantation BA patient survival is close to 90%. Early diagnosis is very important since the chances of success of the Kasai procedure decrease with time. The causes of BA remain actually unknown but several mechanisms including genetic and immune dysregulation may probably lead to the obliterative cholangiopathy. Current research focuses on the identification of blood or liver factors linked to the pathogenesis of BA that could become therapeutic targets and avoid the need for liver transplantation. No similar disease leading to total obstruction of the biliary tree exists in older children or adults. But understanding the physiopathology of BA may highlight the mechanisms of other destructive cholangiopathies, such as sclerosing cholangitis.

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Citations

Aug 11, 2018·Journal of Pediatric Gastroenterology and Nutrition·Mauri WittUNKNOWN NeSBAR (Netherlands Study group on Biliary Atresia Registry)
Dec 27, 2018·Current Opinion in Gastroenterology·Muriel Girard, Ganna Panasyuk
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Oct 6, 2020·Mayo Clinic Proceedings·Moira B HilscherJohn E Eaton
Feb 10, 2021·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Sujit K MohantyGreg Tiao

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