Long-Term Reduction of Liver Fibrosis Surrogates in Syndromic Biliary Atresia.

AIM: Biliary atresia (BA) has a known association with other congenital anomalies and specifically the Biliary Atresia Splenic Malformation syndrome (BASM). Although initial response to the Kasai portoenterostomy (KPE) appear similar, it seems that they have less liver fibrosis in the longer term. We aimed to test this hypothesis.

METHODS

Single-centre prospective database with a 2:1 ratio of contemporaneous controls. We used native liver survivors (at 3+ yrs) as the principal comparison. Three surrogate markers of liver fibrosis were used: AST-to-Platelet-index (APRi), Varices Prediction Rule (VPR) and endoscopic presence of clinically significant varices (grade 2 or 3). [N.B. higher APRI and lower VPR are predictive of liver fibrosis]. Data were quoted as median (IQR where applicable), and non-parametric comparisons were used throughout. P≤0.05 was regarded as significant.

RESULTS

During the period 1990 - 2024 there were 154 infants with Syndromic BA (BASM, n= 95 and Non-BASM, n= 59) and Isolated BA (n=252), and of these 55 (35.7%), and 104 (41.3%) were 3+ year native liver survivors. There was no significant difference in age at KPE between the 3 groups and both the APRi was lower in the BASM than both, Non-BASM and IBA (0.63 vs 0.76 vs. 0.72, P<0.01) and VPR was higher in the BASM than IBA (23 vs. 18, P =0.02) respectively. In the 3+ year native liver survivors' group, APRi was lower in both BASM and Non-BASM groups compared to IBA (0.78 vs. 0.45 vs 1.03, P<0.05) and VPR higher 10.6 vs. 10.9 vs 8.1 (P < 0.05). Clinically significant varices were less prevalent in the syndromic groups [2 (3.6%) vs. 22 (21.1%), P=0.002].

CONCLUSION

There is prima facie clinical and biomarker evidence for reduced portal hypertension and presumably liver fibrosis in both syndromic BA groups, compared to those with Isolated BA. This might suggest that the ongoing pathobiology post-KPE in such syndromic groups is different to that of isolated BA.