Dipartimento di Pediatria
*Dipartimento di Scienze Biomorfologiche e Funzionali, Università di Napoli “Federico II”
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Key words: R. Iorio, A.M. Salzano, F. Cirillo, G. Vallone
Biliary atresia (BA) is the most common pediatric cause of cirrhosis, end-stage liver disease and indication for liver transplantation. The clinical presentation is characterized by jaundice with yellow or dark urine and pale stools, which eventually become acholic. BA is difficult to identify during the neonatal period because of the well-nourished appearance of the affected infants. The Kasai portoenterostomy (KP) is the primary treatment for BA and has the greatest likelihood success when performed before the age of 45-60 days. Even with a successful KP, more than 70% of children eventually develop cirrhosis and require liver transplantation before adulthood. So far, no single diagnostic test, such as short of laparotomy, is definitive for diagnosis of BA. The most accurate test for differentiating BA from other forms of cholestasis remains percutanoeus liver biopsy that, however, is pathologist dependent. A number of screening strategies in infants have been studied (measurements of serum conjugated bilirubin and evaluation of stool by stool color card), but so far no convenient means of screening newborns is universally recommended.
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