BOB Ped Surg 2023 - Maria Soledad Jara Valdivia, CIPESUR - Presentation

Biliary atresia leads to liver cirrhosis without early treatment

The diagnosis of BA can be challenging as its histopathologic features overlap with those of other pediatric cholestatic liver diseases

The most helpful hallmark of BA on histology is portal fibrosis and bile duct proliferation

A similar pattern to BA can be found in other causes of cholestasis such as familiar intrahepatic cholestasis, neonatal hepatitis, and Alagille syndrome

During cirrhosis, biliary epithelial structure express immunohistochemical markers of immaturity such as CD56

639 biopsies were performed between 2013 and 2019

Final groups were distributed as: group BA equals 30 patients, group NC equals 28 patients, group C no BA equals 31

CD56 plus had sensitivity of 74% for BA versus 16% and 9% for other groups

CD56 plus had specificity of 87.7% for BA versus 61% and 50% for other groups

The PPV was 76% for BA versus 18% and 9% for other groups

The NPV was 80% for BA versus 57% and 50% for other groups

The AUC was 0.81

The labeling quantile from 6 to 25% had a higher sensitivity and adequate specificity

CD56 in more than 5% of the ductular epithelial cells has a sensitivity of 74.2% versus 16.1% and 9.68% in non-BA groups

CD56 has specificity of 87.7% and LHR+ of 6.04 compared to 0.42 and 0.20 in non BA group to diagnose biliary atresia

An NPV of 86.2% shows that if CD56 is negative, biliary atresia is unlikely

CD56 IHC stain is helpful as a complementary test in liver biopsies to rule out biliary atresia as an etiology for neonates presenting with cholestasis