Odevixibat for treating biliary atresia in children following Kasai hepatoportoenterostomy

Odevixibat is in clinical development for the treatment of children with biliary atresia (BA) following a Kasai hepatoportoenterostomy (HPE). BA is a rare gastrointestinal disorder where either all or a portion of the bile duct on the outside of the liver is absent or destroyed. As a result, bile acids accumulate in the liver causing liver damage. The only currently available treatment option for BA is a Kasai HPE procedure where a surgeon replaces the damaged bile ducts with a portion of the patients small intestine, enabling bile to flow from the liver to the intestine. Although this procedure is typically successful most patients still require a liver transplant later in childhood.