Shneider, Benjamin L.Magee, John C.Karpen, Saul J.Rand, Elizabeth B.Narkewicz, Michael R.Bass, Lee M.Schwarz, KathleenWhitington, Peter F.Bezerra, Jorge A.Kerkar, NandaHaber, BarbaraRosenthal, PhilipTurmelle, Yumirle P.Molleston, Jean P.Murray, Karen F.Nguyen, Vicky L.Wang, Kasper S.Romero, ReneSquires, Robert H.Arnon, RonenSherker, Averell H.Moore, JeffreyYe, WenSokol, Ronald J.2017-07-262017-07-262016-03Shneider, B. L., Magee, J. C., Karpen, S. J., Rand, E. B., Narkewicz, M. R., Bass, L. M., … Sokol, R. J. (2016). Total Serum Bilirubin within Three Months of Hepatoportoenterostomy Predicts Short-term Outcomes in Biliary Atresia. The Journal of Pediatrics, 170, 211–217.e2. http://doi.org/10.1016/j.jpeds.2015.11.058https://hdl.handle.net/1805/13596OBJECTIVES: To prospectively assess the value of serum total bilirubin (TB) within 3 months of hepatoportoenterostomy (HPE) in infants with biliary atresia as a biomarker predictive of clinical sequelae of liver disease in the first 2 years of life. STUDY DESIGN: Infants with biliary atresia undergoing HPE between June 2004 and January 2011 were enrolled in a prospective, multicenter study. Complications were monitored until 2 years of age or the earliest of liver transplantation (LT), death, or study withdrawal. TB below 2 mg/dL (34.2 μM) at any time in the first 3 months (TB <2.0, all others TB ≥ 2) after HPE was examined as a biomarker, using Kaplan-Meier survival and logistic regression. RESULTS: Fifty percent (68/137) of infants had TB < 2.0 in the first 3 months after HPE. Transplant-free survival at 2 years was significantly higher in the TB < 2.0 group vs TB ≥ 2 (86% vs 20%, P < .0001). Infants with TB ≥ 2 had diminished weight gain (P < .0001), greater probability of developing ascites (OR 6.4, 95% CI 2.9-14.1, P < .0001), hypoalbuminemia (OR 7.6, 95% CI 3.2-17.7, P < .0001), coagulopathy (OR 10.8, 95% CI 3.1-38.2, P = .0002), LT (OR 12.4, 95% CI 5.3-28.7, P < .0001), or LT or death (OR 16.8, 95% CI 7.2-39.2, P < .0001). CONCLUSIONS: Infants whose TB does not fall below 2.0 mg/dL within 3 months of HPE were at high risk for early disease progression, suggesting they should be considered for LT in a timely fashion. Interventions increasing the likelihood of achieving TB <2.0 mg/dL within 3 months of HPE may enhance early outcomes.en-USPublisher PolicyCholestasisBileLiverInfantHealth outcomesTransplantCirrhosisAscitesVaricesArticle