Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia

• Type of Material: Article
• Authors: Benjamin L. Shneider, Baylor College of Medicine; John C. Magee, University of Michigan; Saul Karpen, Emory University; Elizabeth B. Rand, Childrens Hospital of Philadelphia; Michael R. Narkewicz, University of Colorado; Lee M. Bass, Ann & Robert H Lurie Childrens Hospital Chicago; Kathleen Schwarz, Johns Hopkins University School of Medicine; Peter F. Whitington, Ann & Robert H Lurie Childrens Hospital Chicago; Jorge A. Bezerra, Cincinnati Children’s Hospital; Nanda Kerkar, University of Southern California; Barbara Haber, Merck Sharp & Dohme Corporation; Philip Rosenthal, University of California San Francisco; Yumirle P. Turmelle, Washington University; Jean P. Molleston, Indiana School of Medicine; Karen F. Murray, University of Washington; Vicky L. Ng, Hospital for Sick Children; Kasper S. Wang, Children's Hospital; Rene Romero, Emory University; Robert H. Squires, Childrens Hospital Pittsburgh; Ronen Arnon, Mt Sinai Hospital; Averell H. Sherker, NIDDK; Jeffrey Moore, University of Michigan; Wen Ye, University of Michigan; Ronald J. Sokol, University Colorado
• Language: English
• Date: 2016-03-01
• Publisher: Elsevier
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2016 Elsevier Inc.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.jpeds.2015.11.058
• Title of Journal or Parent Work: Journal of Pediatrics
• ISSN: 0022-3476
• Volume: 170
• Start Page: 211
• End Page: +
• Grant/Funding Information: Funded by the National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases (U01DK103149 [to B.S.]; U01DK062456 [to J.M.]; U01DK062470 and UL1TR000454 [to S.K. and R.R.]; U01DK062481 [to E.R. and B.H.]; U01DK062453 and UL1TR001082 [to M.N. and R.S.]; U01DK062436 and UL1TR000150 [to L.B. and P.W.]; U01DK062503 and UL1TR000424 [to K.S.]; U01DK062497 and UL1TR000077 [to J.B.]; U01DK062445 [to N.K. and R.A.]; U01DK062500 and UL1TR000004 [to P.R.]; U01DK062452 and UL1TR000448 [Y.T.]; U01DK084536 and UL1TR001108 [to J.M.]; U01DK084575, UL1TR000423, and UL1RR025014 [to K.M.]; U01DK103135 [to V.N.]; U01DK084538 and UL1TR000130 [to K.W.]; and U01DK062466 and UL1TR000005 [to R.S.]).
• Keywords: Science & Technology; Life Sciences & Biomedicine; CHILDREN; VARICES; EXPERIENCE; SURGERY; SURVIVAL; RISK; KASAIS OPERATION; INFANTS; MANAGEMENT; PORTOENTEROSTOMY; Pediatrics
• Research Categories: Health Sciences, Nutrition; Health Sciences, General

Abstract

Objectives 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. Trial registration ClinicalTrials.gov: NCT00061828 and NCT00294684.