Diagnosi e gestione della atresia delle vie biliari

Diagnosis, management and outcome of patients with Biliary Atresia

Daniela Liccardo1, Manila Candusso1, Valerio Nobili1,2

1OCEpatologia, Gastroenterologia,Nutrizione e Trapianti di Fegato Dipartimento Pediatrie Specialistiche Ospedale Pediatrico Bambino Gesù, IRCCS 1,2Dipartimento di Pediatria, Universitàla Sapienza di Roma

Biliary atresia (BA) is a rare, idiopathic, progressive diseaseaffecting the biliary tract leading to end stage and death ifuntreated. It is the main indication to pediatric livertransplant. The current standard treatment of BA issurgical management with initial Kasai portoenterostomywhich aim is to obtain successful bile drainage andpreserve the native liver. The timing of surgery has beenshown to be important for the success of the operationand less than 60 days of life has been used as a cut-off inthe literature. Despite this, about 70% of them at long termoutcome show progression of hepatic fibrosis up to requireliver transplantation, typically within the first two years oflife.

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