Biliary atresia at the dawn of a new century.
Biliary atresia is a unique disorder in pediatric surgery. The incidence is 1 in 3,500 to 20,000 live births, but the disorder accounts for more than half of the patients requiring liver transplantation. After Kasai operation, the 5- and 10-year survival rate without the need of transplantation are 37-48% and 18-28%, respectively. An infectious pathogen, particularly the virus, has long been implicated as the causative agent of biliary atresia, but none has ever been identified. Ductal plate malformation is likely to be important in the pathogenesis of biliary atresia. Earlier diagnosis is associated with a better prognosis and biliary atresia should be considered in the differential diagnosis of prolonged jaundice in any infants older than 2 weeks of age. The experience of the surgeon and surgical decade are determinant in achieving success of the Kasai operation. Postoperative cholangitis affects more than 45% of the patients receiving Kasai operation and the afflicted patients are at risk of cessation of bile flow and exacerbation of preexisting liver cirrhosis. Hepatic fibrosis starts at the moment when biliary atresia develops and the ongoing inflammation will end up with liver cirrhosis and hepatic failure in most patients, including some with adequate bile drainage. The inhibition of liver cirrhosis in mice or rats by gene therapy, such as telomere gene delivery, transduction with the hepatocyte growth factor gene or blockade of TGF-beta signaling, may provide a new strategy to rejuvenate the ailing liver associated with biliary atresia in the future.
Chuang
J H
JH
Department of Pediatric Surgery, Chang Gung Children's Hospital. 123, Ta-Pei Road, Niaosung, Kaohsiung, Taiwan, R.O.C. jhchuang@cgmh.or.tw
Lin
J N
JN
D001656:Biliary Atresia / Q000453:epidemiology / Q000209:etiology / Q000601:surgery*; D002761:Cholangitis / Q000209:etiology; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology; D011183:Postoperative Complications / Q000209:etiology