Atresia biliar extrahepatica pdf

Biliary atresia is initially treated with the kasai procedure, which is a surgical procedure that provides a way for bile to flow out of the liver into the intestine. Some infants, particularly those with the fetal form, also. Biliary atresia is a rare disease of the bile ducts that affects only infants. It is the most frequent surgical cause of cholestatic jaundice in this age group. Ba occurs in approximately 118,000 live births in western europe. Biliary atresia childrens hospital of philadelphia. It has an incidence of one in 10,00015,000 live births in the united states, and a prevalence of one in 16,700 in the british isles. Biliary atresia is a rare disease of the liver and bile ducts that occurs in infants. To improve these results early surgical intervention in all children with extrahepatic biliary atresia is necessary, as are better methods of prophylaxis and treatment of cholangitis. Biliary atresia is a serious liver disorder that occurs before or shortly after birth when a babys bile ducts the tubes that carry bile from the liver become blocked. Atresia bilier adalah penyakit bawaan lahir yang sampai saat ini belum dapat diketahui penyebab pastinya. The body needs bile to aid digestion and carry wastes from the liver out of the body.

Biliary atresia ba is a rare disease characterised by a biliary obstruction of unknown origin that presents in the neonatal period. Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. Biliary atresia childrens liver disease foundation. Perinatal biliary atresia is much more common and does not become evident until 2 to 4 weeks after birth.

Biliary atresia is a rare gastrointestinal disorder characterized by destruction or absence of all or a portion of the bile duct that lies outside the liver extrahepatic bile duct. Biliary atresia nord national organization for rare. Biliary atresia ba is a progressive, idiopathic, fibroobliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. In this procedure, the surgeon removes the damaged and scarred bile duct and connects the intestine to the liver to allow the bile to flow. Bile ducts are pathways that carry a digestive fluid called bile from the liver to the small intestine. This in turn causes scarring fibrosis in the liver.

We studied clinical features, diagnosis studies as well as surgery technique age at the surgey time, anatomopatologic study and their progres. The bile duct is a tube that allows the passage of bile from the liver into the gall bladder and, eventually, the small intestine. Full text get a printable copy pdf file of the complete article 738k, or click on a page image below to browse page by page. Treatment of biliary atresia with special reference to hepatic porto enterostomy and its modifications. In spite of extensive investigation, its etiology has remained poorly understood.

Differential diagnosis of extrahepatic biliary atresia from neonatal hepatitis. The two types of biliary atresia are fetal and perinatal. Biliary atresia is an inflammatory cholangiopathy of infancy that results in progressive fibrosis and obliteration of bile ducts and represents the main indication for liver transplant in young children. Timely surgical intervention kasai procedure may result in significant benefit to these patients.