PURPOSE OF REVIEW: The purpose of this study is to review both the basic science and clinical advancements in the last 12 months that have furthered our understanding of biliary atresia. RECENT FINDINGS: Early diagnosis and further understanding of the disease process may be the next major step in advancement. Stool color cards have been shown to be an accurate screening tool. Basic science developments have focused on defects in morphogenesis, immunologic dysregulation, and viral infection as the major theories of causes. There have been initial reports of minimally invasive approaches to hepatic portoenterostomy but there has been little comparative study. Postoperative corticosteroid therapy remains an area of debate without definitive data. Early postoperative testing of serum bilirubin levels and hepatobiliary scintigraphy are showing strong correlation with long-term outcomes. The comparison of regions with decentralization policies compared with those with central referral policies is providing a good forum to monitor real-time outcome data. SUMMARY: Biliary atresia continues to represent a major challenge with many unanswered questions. The establishment of multicentered collaboration in both basic science and clinical research interests has been an important step in improving outcomes for this disease.
PURPOSE OF REVIEW: The purpose of this study is to review both the basic science and clinical advancements in the last 12 months that have furthered our understanding of biliary atresia. RECENT FINDINGS: Early diagnosis and further understanding of the disease process may be the next major step in advancement. Stool color cards have been shown to be an accurate screening tool. Basic science developments have focused on defects in morphogenesis, immunologic dysregulation, and viral infection as the major theories of causes. There have been initial reports of minimally invasive approaches to hepatic portoenterostomy but there has been little comparative study. Postoperative corticosteroid therapy remains an area of debate without definitive data. Early postoperative testing of serum bilirubin levels and hepatobiliary scintigraphy are showing strong correlation with long-term outcomes. The comparison of regions with decentralization policies compared with those with central referral policies is providing a good forum to monitor real-time outcome data. SUMMARY:Biliary atresia continues to represent a major challenge with many unanswered questions. The establishment of multicentered collaboration in both basic science and clinical research interests has been an important step in improving outcomes for this disease.