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Biliary atresia, a fibro-obliterating biliary disease of infants, remains the most common indication for pediatric liver transplantation because of the rapid progression of liver injury. To identify predictive biomarkers of disease progression and to elucidate the pathophysiology of liver injury in biliary atresia, we profiled serum bile acids and studied their liver metabolism after Kasai portoenterostomy. Accumulation and altered composition of circulating bile acids predicted progression of liver disease and need for liver transplantation. Patients with poor prognosis showed low bile acid synthesis and abnormal liver expression of fibroblast growth factor 19.
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Nyholm et al. (2025) studied this question.
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