Normal Gamma Glutamyl Transferase Levels at Presentation Predict Poor Outcome in Biliary Atresia.

OBJECTIVES Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of 'normal GGT' BA has not been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to 1) describe outcomes of a single-centre Australian cohort of infants with biliary atresia and 2) assess the impact of GGT level at presentation on outcomes in BA. METHODS Infants diagnosed with BA between 1991 - 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT) and death. Patients were categorised into normal (<200IU/L) or high GGT groups based on a mean of three consecutive GGT values done prior to Kasai portoenterostomy (KPE). Baseline parameters, age at surgery, clearance of jaundice and outcomes were compared between the two groups. RESULTS 113 infants underwent KPE at median 61 (30-149) days. At a median follow-up of 14.2 (0.9-26.3) years, 35% (39/113) patients were surviving with native liver, 55% (62/113) underwent LT and 11% (12/113) died pre-transplant. 12.3% (14/113) patients had normal GGT. Age at KPE and time to clearance of jaundice were similar between normal and high GGT groups. Normal GGT group had shorter time from KPE to LT (11 vs 18months, p = 0.02), underwent LT at a younger age (14 vs 20months, p = 0.04) and had poorer transplant-free survival (p = 0.04) than high GGT group. CONCLUSION 12.3% of infants with BA had normal GGT levels at diagnosis. Low GGT levels at presentation in biliary atresia was associated with a poorer outcome.