Does Gamma-Glutamyl Transpeptidase Serum Level Play a Role as a Prognostic Marker in Biliary Atresia, an Immune-Mediated Cholangiopathy in Children?

<b>Background</b>: Biliary atresia (BA) is a rare, immune-mediated cholangiopathy in children and a leading cause of neonatal cholestasis and pediatric liver transplantation (LT). Gamma-glutamyl transpeptidase (GGT) is commonly elevated in BA and is used as a diagnostic marker; however, recent studies have suggested that a subset of BA patients present with normal or low GGT levels, potentially indicating a more severe disease course. <b>Methods</b>: This retrospective study evaluated the prognostic value of serum GGT levels in 47 children diagnosed with BA at a single center over 15 years. Patients were stratified by GGT levels at diagnosis, and outcomes were compared, including survival with native liver, need for LT, and mortality. GGT thresholds of 200 U/L and 300 U/L were used to define normal and elevated levels. <b>Results</b>: The study found that 12% of patients had normal or low GGT at diagnosis. Still, there were no statistically significant differences in age at diagnosis, severity of liver fibrosis, age at Kasai portoenterostomy (KPE), or overall outcomes between low and high GGT groups. Although patients with lower GGT tended to require LT at a younger age, this difference was not significant. Receiver operating characteristic (ROC) curve analysis showed that GGT levels at diagnosis, before, and after KPE were not reliable predictors of outcome. <b>Conclusions</b>: The findings contrast with some previous reports suggesting that low GGT is associated with a worse prognosis. Low GGT level alone should not delay diagnosis or surgical intervention in suspected BA. Early referral for KPE remains critical, and patients with low GGT may benefit from earlier LT evaluation. Larger, multicenter studies are needed to clarify the prognostic role of GGT in BA.