儿科学营养方面.pdfVIP

Biliary Atresia Karen F. Murray, MD Professor of Pediatrics Director, Hepatobiliary Program Seattle Children’s Biliary Atresia  Incidence: 1/8,000-15,000 live births  Girls boys 1.5:1  The most common cause of surgical obstructive jaundice presenting in the 1st 3 months.  Leading indication for liver transplantation in infants and children (40-50%) Biliary Atresia  Definition  Progressive sclerosing inflammatory process of extrahepatic and intrahepatic bile ducts causing complete biliary obstruction in the first three months of life  Location  Segmental or complete  Most commonly involves complete extrahepatic bile duct extending into the hepatic porta Biliary Atresia: Surgical Anatomy  Type 1 (3%) - common bile duct  Type 2 (6%) – cyst in hilum communicating with intrahepatic bile ducts  Type 3 (19%) – GB, cystic duct and common bile duct patent  Type 4 (72%) – complete extrahepatic atresia Clinical Presentation  Healthy term female, growing well Jaundiced 2 week check-up  Weeks later: jaundiced, hepatomegaly, acholic stools Biliary Atresia: 100% fatal by 3 yrs if treatment unsuccessful Biliary Atresia: Phenotypes Embryonic or “congenital” 10-20%  Prenatal process causing BD injury  Early presentation – born with atretic duct No jaundice free interval  Other congenital anomalies  Polysplenia (BASM) syndrome 8-11%, 3-4% in Asia Biliary Atresia: Phenotypes Perinatal or acquired 80% Post-natal BD injury Viral or toxic insult Frequently have jaundice-free period No other congenital anomalies Biliary Atresia: