Background Biliary atresia (BA) is among the commonest indications for liver transplantation (LT) in children. We examined whether serum matrix metalloproteinase‐7 (MMP‐7) is useful for diagnosis of BA in Japanese infants, and whether serum MMP‐7 concentrations before and after Kasai portoenterostomy (KP) predicted LT within a year. Methods Subjects under 6 months old at eight pediatric centers in Japan were enrolled retrospectively, including patients with cholestasis and normal controls (NC) without liver disease. Patients with cholestasis were divided into groups representing BA versus cholestasis from other causes (non‐BA). Serum samples were collected from patients with BA at diagnosis and 1 and 4 weeks after KP, as well as from non‐BA and NC. Results Serum MMP‐7 concentrations were significantly higher in BA at diagnosis (median, 89.1 ng/ml) than in non‐BA (11.0; p < 0.001) or NC (10.3; p < 0.001). Receiver operating characteristic (ROC) analysis of MMP‐7 for BA versus non‐BA yielded an area under the ROC curve of 0.99 (95% confidence interval, 0.96–1.00). An optimal cut‐off value of 18.6 ng/ml for serum MMP‐7 in diagnosing BA demonstrated sensitivity and specificity of 100% and 90%, respectively. Serum MMP‐7 before and 1 week and 4 weeks after KP did not differ significantly between BA requiring only KP and BA requiring LT after KP. Conclusion Serum MMP‐7 is a useful marker for diagnosis of BA in Japanese infants, but it could not predict LT within a year.

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few reports describe oxysterols in healthy children or in children with liver disease. We aimed to determine whether developmental changes in urinary and serum oxysterols occur during childhood, and to assess whether oxysterols might be biomarkers for pediatric liver disease. Healthy children enrolled as subjects (36 and 35 for urine and serum analysis, respectively) included neonates, infants, preschoolers, and school-age children, studied along with 14 healthy adults and 8 children with liver disease. We quantitated 7 oxysterols including 4β-, 20(S)-, 22(S)-, 22(R)-, 24(S)-, 25-, and 27-hydroxycholesterol using liquid chromatography/electrospray ionization-tandem mass spectrometry. Urinary total oxysterols were significantly greater in neonates than in infants (P < 0.05), preschoolers (P < 0.001), school-age children (P < 0.001), or adults (P < 0.001), declining with age. Serum total oxysterols in neonates were significantly lower than in infants (P < 0.05), preschoolers (P < 0.001), school-age children (P < 0.05), or adults (P < 0.01). Compared with healthy children, total oxysterols and 24(S)-hydroxycholesterol in liver disease were significantly increased in both urine (P < 0.001 and P < 0.001, respectively) and serum (P < 0.001 and P < 0.05, respectively). Oxysterols in liver disease, particularly 24(S)-hydroxycholesterol, were greater in urine than serum. Oxysterols change developmentally and might serve as a biomarker for pediatric liver disease. to our knowledge, this is the first such report.