Aim of the study: Delayed presentation of biliary atresia (BA) may preclude Kasai portoenterostomy (KPE) owing to advanced fibrosis and perceived futility. Primary liver transplantation (PLT) becomes the only treatment option. These infants, waiting for transplant, give us insight into the natural history of BA.
Methods: We identified infants listed for PLT over a 19-year period (1999-2017). Serial biochemistry (arbitrarily at d150 and d225 for analysis) and Ishak liver fibrosis scores were documented. Adverse outcome measures were variceal bleeding and death. Appropriate statistical tests were used with a P-value <0.05 regarded as significant. Data are quoted as median(IQR).
Main Results: We identified 13 infants (4 male), aged 156 (109-157) days at presentation. 10 (77%) underwent LT at 279(239-410) days. Two died of syndromic anomalies aged 62d and 378d. One patient is awaiting transplant (at >d300). 3 had variceal bleeding aged 172d, 264d and 297d.
Serial biochemistry showed a relative “steady state” and only modestly rising bilirubin (P=0.003) [<350 µmol/L in 8/13(61%)] (FIG.1). Whereas both GGT(P=0.004) (FIG.1) and platelet count declined dramatically [273(206-401) to 129(90-228);P=0.001] and albumin remained static 33(29-39)and 32(31-34);P=0.5. Ishak fibrosis score at diagnosis and at transplant was ≥5/6 in 4/11(36%) and 9/10 (90%) respectively.