Abstract
Biliary atresia (BA) is a rare newborn liver disease with significant morbidity and mortality, especially if not recognized and treated early in life. It is the most common cause of liver-related death in children and the leading indication for liver transplantation in the pediatric population. Timely intervention with a Kasai portoenterostomy (KPE) can significantly improve prognosis. Delayed disease recognition, late patient referral, and untimely surgery remains a worldwide problem. This article will focus on biliary atresia from a global public health perspective, including disease epidemiology, current national screening programs, and their impact on outcome, as well as new and novel BA screening initiatives. Policy challenges for the implementation of BA screening programs will also be discussed, highlighting examples from the North American, European, and Asian experience.
Highlights
Biliary atresia (BA) is a rare orphan newborn liver disease that results from an idiopathic progressive fibrosclerosing obliteration of large bile ducts [1,2]
The current standard of care for BA is sequential surgery with an initial Kasai hepato-portoenterostomy (KPE), in which the obstructed bile duct is resected and a loop of the small bowel is brought to the porta hepatis of the liver to restore bile flow, followed by liver transplantation for those in whom the KP fails or who progress to cirrhosis and liver failure at a later pediatric age or into adulthood
The aims of this article are to provide up-to-date knowledge of the epidemiology of biliary atresia, to focus on the current clinically applicable BA screening strategies implemented in large populations or national programs, and to describe the challenges of BA screening in the context of public health policies
Summary
Biliary atresia (BA) is a rare orphan newborn liver disease that results from an idiopathic progressive fibrosclerosing obliteration of large bile ducts [1,2]. The condition is recognized as one of the most rapidly progressive liver diseases known to man. It is the leading cause of liver-related death in children and the foremost indication for liver transplantation in the pediatric population. The current standard of care for BA is sequential surgery with an initial Kasai hepato-portoenterostomy (KPE), in which the obstructed bile duct is resected and a loop of the small bowel is brought to the porta hepatis of the liver to restore bile flow, followed by liver transplantation for those in whom the KP fails or who progress to cirrhosis and liver failure at a later pediatric age or into adulthood.
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