Quantitative Liver Fibrosis Using Collagen Hybridizing Peptide to Predict Native Liver Survival in Biliary Atresia: A Pilot Study.

BACKGROUND/RATIONALE Biliary atresia (BA) is a cholangiopathy characterized by bile flow obstruction due to destruction of the biliary tree. Without surgical correction with Kasai portoenterostomy (KPE), BA leads to death or liver transplant (LTx). Early-onset, progressive liver fibrosis is a defining characteristic of BA. Collagen hybridizing peptide (CHP) is a synthetic peptide which binds to denatured collagen strands allowing quantification of fibrosis. This technique has not been used on human liver tissue. The aim of this pilot study was to evaluate the utility of CHP as a measurement of quantitative fibrosis to allow earlier survival with native liver (SNL) prognostication. RESULTS We identified 21 patients with wedge liver biopsies available, of which 14 required LTx. No deaths occurred. Patients requiring LTx tended to be female with a significantly different mean bilirubin (p = 0.002), albumin (p = 0.001) and ALT (p = 0.03) at 3-months post-KPE. By 1-year post-KPE, 50% of patients in the high-CHP intensity group required LTx versus 27% in the low-CHP. Overall, fibrosis as quantified by CHP at time of KPE was associated with more than three-times the risk of requiring LTx by 4-years of age (HR 3.6, 95%CI 1.15-10.93, p = 0.03). When controlling for gender and TB > 2 mg/dL and albumin at 3-months post-KPE, it predicted nearly seven times the risk of LTx (HR 6.89, 95%CI 1.38-34.32, p = 0.02). CONCLUSION Our results suggest that quantitative assessment of fibrosis at the time of KPE holds promise as an earlier predictor of LTx requirement in BA. A larger study is justified to assess quantitative fibrosis as a BA prognostic tool.