In biliary atresia (BA), gamma-glutamyl transferase (GGT) levels are generally increased, however, normal GGT levels have been seen. This group of “normal GGT” BA has not been characterized, nor has the predictive significance of GGT level on BA outcomes been established. The goal of this study was to report the results of a single-center Australian cohort of babies with BA and to evaluate the influence of GGT level at presentation on outcomes in BA. Infants diagnosed with BA between 1991 and 2017 were studied retrospectively. Survival with native liver, liver transplantation (LT), and death was specified as outcomes. Patients were divided into normal and high GGT groups based on a mean of three consecutive GGT readings obtained before to Kasai portoenterostomy (KPE). The two groups were compared in terms of baseline characteristics, age at operation, clearing of jaundice (COJ), and outcomes. KPE was performed on 131 babies at a median age of 61 days. At a median follow-up of 14.2 years, 35% of patients were still living with their original liver, 55% underwent LT, and 11% died before the transplant. GGT was normal in 12.3% of the patients. The age at KPE and time to COJ were comparable in the normal and high GGT groups. The normal GGT group had a shorter period between KPE and LT, received LT at a younger age, and had a worse transplant-free survival rate than the high GGT group.

At the time of diagnosis, 12.3% of babies with BA had normal GGT levels. Low GGT levels at presentation in BA were linked to a worse outcome.