If left untreated, Biliary Atresia progression to end-stage cirrhosis usually occurs within one year
Jaundice is common in newborns. However, for up to one in every 5000 newborns, Jaundice may never go away and children develop Biliary Atresia (BA) (Hartley et al., 2009). The cause of Biliary Atresia is not entirely clear, both developmental defects or perinatal infections can cause obstructions of the extrahepatic bile ducts and result in rapid liver failure in early infancy (Lakshminarayanan and Davenport, 2016). Surgical removal of the duct remnants followed by hepatoportoenterostomy (Kasai's procedure) is required after diagnosis, (Hopkins et al., 2017). If left untreated, Biliary Atresia progression to end-stage cirrhosis usually occurs within one year. Following the Kasai's operation, approximately 50% of the Biliary Atresia patients will require liver transplantation within the first 2 years of life, while the remainder children surviving with the native livers will suffer long-term complications including repeated cholangitis, portal hypertension, esophageal variceal bleeding, and hepatic osteodystrophy. In the laboratory, we study how immune defects leads to Biliary Atresia development, and develop methods to treat children with Biliary Atresia.