https://pubmed.ncbi.nlm.nih.gov/36131538/ biliary atresia

Hepatology. 2022 Sep 21.
doi: 10.1002/hep.32800. Online ahead of print.

Serum Bile Acids as a New Prognostic Biomarker in Biliary Atresia Following Kasai Portoenterostomy

Sanjiv Harpavat 1, Kieran Hawthorne 2, Kenneth D R Setchell 3, Monica Narvaez Rivas 3, Lisa Henn 2, Charlotte A Beil 2, Saul J Karpen 4, Vicky L Ng 5, Estella M Alonso 6, Jorge A Bezerra 7, Stephen L Guthery 8, Simon Horslen 9 10, Kathy M Loomes 11, Patrick McKiernan 10, John C Magee 12, Robert M Merion 2, Jean P Molleston 13, Philip Rosenthal 14, Richard J Thompson 15, Kasper S Wang 16, Ronald J Sokol 17, Benjamin L Shneider 1, Childhood Liver Disease Research Network (ChiLDReN)
Affiliations expand
PMID: 36131538

DOI: 10.1002/hep.32800

Abstract

Background & aims: In biliary atresia (BA), serum bilirubin is commonly used to predict outcomes after Kasai portoenterostomy (KP). Infants with persistently high levels invariably need liver transplant, but those achieving normalized levels have a less certain disease course. We hypothesized that serum bile acid levels could help predict outcomes in the latter group.

Approach & results: Participants with BA from the Childhood Liver Disease Research Network were included if they had normalized bilirubin levels 6 months post-KP and stored serum samples from the 6-month post-KP clinic visit (n=137). Bile acids were measured from the stored serum samples and used to divide participants into ≤40 μmol/L (n=43) or >40 μmol/L (n=94) groups. At 2 years of age, the ≤40 μmol/L compared with >40 μmol/L group had significantly lower total bilirubin, aspartate aminotransferase, alanine aminotransferase, gamma-glutamyl transferase, bile acids, and spleen size, as well as significantly higher albumin and platelet counts. Furthermore, during 734 person-years of follow-up, those in the ≤40 μmol/L group were significantly less likely to develop splenomegaly, ascites, gastrointestinal bleeding, or clinically evident portal hypertension. The ≤40 μmol/L group had a 10-year cumulative incidence of liver transplant/death of 8.5% (95% confidence interval [CI]: 1.1%-26.1%), compared with 42.9% (95% CI: 28.6%-56.4%) for the >40 μmol/L group (p=0.001).

Conclusions: Serum bile acid levels may be a useful prognostic biomarker for infants achieving normalized bilirubin levels after KP.