https://pubmed.ncbi.nlm.nih.gov/40028801/ Biliary atresia
Observational Study

J Pediatr Gastroenterol Nutr. 2025 Mar;80(3):462-470.
doi: 10.1002/jpn3.12451. Epub 2024 Dec 29.

Serum bile acids early after portoenterostomy are predictive for native liver survival and portal hypertension in biliary atresia

Linda Anderson 1, Maria Hukkinen 1, Iiris Nyholm 1 2, Mikko Niemi 3 4 5, Mikko P Pakarinen 1 2 6
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PMID: 40028801

PMCID: PMC11874233

DOI: 10.1002/jpn3.12451
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Abstract

Objectives: To compare the predictive value of serum bile acids on native liver survival (NLS) and portal hypertension (PH) at various time points early after portoenterostomy (PE) in biliary atresia (BA).

Methods: This was a retrospective observational study. Serum bilirubin and bile acid concentrations were defined by enzymatic spectrophotometry 1, 3, and 6 months after PE. After defining optimal bilirubin and bile acids cutoffs by area under the receiver operating characteristic (AUROC) curves, cutoffs were compared with other predictors of NLS and PH in Cox regression.

Results: Out of 56 patients, 42 (75%) achieved clearance of jaundice (COJ, bilirubin <20 µmol/L at 6 months). Both bilirubin and bile acids at 3 and 6 months were accurate predictors of NLS among all patients (AUROC 0.82-0.91, p < 0.001). In COJ patients, bile acids (AUROC 0.82, p = 0.003), but not bilirubin, at 1 month also predicted NLS. Among all patients, the strongest predictors of NLS were bilirubin >18.5 µmol/L and bile acids >150 µmol/L at 3 months, increasing the risk of transplantation/death seven- and eightfold, respectively (p < 0.001 for both). In COJ patients, the strongest predictor of NLS was bile acids >119 µmol/L at 3 months, increasing the risk of transplantation/death 12-fold (p = 0.014). Bile acids and bilirubin at 3 and 6 months predicted PH development in COJ patients with moderate accuracy (AUROC 0.72-0.78, p = 0.004-0.019). Bilirubin >8.5 µmol/L and bile acids >78 µmol/L at 6 months increased PH risk 13-fold (p < 0.001) and 4-fold (p = 0.006).

Conclusions: Serum bile acids offer a simple and useful additional tool to predict PE outcomes in BA, particularly after COJ.