| Abstrakt: |
Biliary atresia (BA) is a leading cause of liver transplantation in children, characterised by neonatal fibroobliterative disease of the extrahepatic biliary tree. BA may be associated with laterality anomalies and congenital malformations that affect prognosis. Clinical findings include jaundice, acholic stools and organomegaly. The differential diagnosis includes several cholestatic diseases. Diagnostic biomarkers such as interleukin-33 and matrix metalloproteinase-7 are promising in the detection of BA. Imaging modalities such as ultrasonography and hepatobiliary scintigraphy aid in the diagnosis. Liver biopsy and intraoperative cholangiography are essential to confirm BA and guide the Kasai procedure, the gold standard intervention. Postoperative markers of successful hepatoportoenterostomy (HPE) include colicky stools, decreased bilirubin, weight gain and decreased pruritus. Patient management includes choleretics (UDCA), nutritional support, prevention of cholangitis and monitoring for complications such as portal hypertension. Liver transplantation may be indicated in certain cases. Timely and accurate diagnosis of biliary atresia is crucial for effective intervention. The Kasai procedure, if performed promptly, may improve outcomes. Continuous monitoring, nutritional support and appropriate management of complications contribute to a better prognosis. In cases where liver transplantation is indicated, preparation should begin, taking into account the patient's age and weight for optimal outcome. [ABSTRACT FROM AUTHOR] |
