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Venkat V, Ng VL, Magee JC, et al. Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age. Hepatol Commun. 2020;4(12):1824-1834doi: 10.1002/hep4.1602.
Venkat, V., Ng, V. L., Magee, J. C., Ye, W., Hawthorne, K., Harpavat, S., Molleston, J. P., Murray, K. F., Wang, K. S., Soufi, N., Bass, L. M., Alonso, E. M., Bezerra, J. A., Jensen, M. K., Kamath, B. M., Loomes, K. M., Mack, C. L., Rosenthal, P., Shneider, B. L., Squires, R. H., Sokol, R. J., Karpen, S. J. (2020). Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age. Hepatology communications, 4(12), 1824-1834. https://doi.org/10.1002/hep4.1602
Venkat, Veena, et al. "Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age." Hepatology communications vol. 4,12 (2020): 1824-1834. doi: https://doi.org/10.1002/hep4.1602
Venkat V, Ng VL, Magee JC, Ye W, Hawthorne K, Harpavat S, Molleston JP, Murray KF, Wang KS, Soufi N, Bass LM, Alonso EM, Bezerra JA, Jensen MK, Kamath BM, Loomes KM, Mack CL, Rosenthal P, Shneider BL, Squires RH, Sokol RJ, Karpen SJ. Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age. Hepatol Commun. 2020 Oct 03;4(12):1824-1834. doi: 10.1002/hep4.1602. eCollection 2020 Dec. PMID: 33305153; PMCID: PMC7706301.
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Hepatol Commun. 2020 Oct 03;4(12):1824-1834. doi: 10.1002/hep4.1602. eCollection 2020 Dec.

Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age.

Hepatology communications

Veena Venkat, Vicky L Ng, John C Magee, Wen Ye, Kieran Hawthorne, Sanjiv Harpavat, Jean P Molleston, Karen F Murray, Kasper S Wang, Nisreen Soufi, Lee M Bass, Estella M Alonso, Jorge A Bezerra, M Kyle Jensen, Binita M Kamath, Kathleen M Loomes, Cara L Mack, Philip Rosenthal, Benjamin L Shneider, Robert H Squires, Ronald J Sokol, Saul J Karpen,

Affiliations

  1. UPMC Children's Hospital of Pittsburgh Pittsburgh PA.
  2. Hospital for Sick Children University of Toronto Toronto Canada.
  3. University of Michigan Hospitals and Health Centers Ann Arbor MI.
  4. Arbor Research Collaborative for Health Ann Arbor MI.
  5. Texas Children's Hospital Liver Center, Baylor College of Medicine Houston TX.
  6. Indiana University-Riley Hospital for Children Indianapolis IN.
  7. Cleveland Clinic Children's Cleveland OH.
  8. Children's Hospital Los Angeles Los Angeles CA.
  9. Ann & Robert H. Lurie Children's Hospital of Chicago Chicago IL.
  10. Cincinnati Children's Hospital Medical Center Cincinnati OH.
  11. University of Utah School of Medicine Primary Children's Hospital Salt Lake City UT.
  12. The Children's Hospital of Philadelphia Philadelphia PA.
  13. University of Colorado School of Medicine Children's Hospital Colorado Aurora CO.
  14. University of California San Francisco San Francisco CA.
  15. Emory University School of Medicine Atlanta GA.

PMID: 33305153 PMCID: PMC7706301 DOI: 10.1002/hep4.1602

Abstract

Approximately 50% of infants with biliary atresia (BA) undergoing Kasai portoenterostomy show survival with native liver (SNL) at age 2 years. Predictors of disease progression after age 2 years are unknown, despite estimates of 20%-30% undergoing liver transplant (LT) between age 2 and 18 years. We sought to address this knowledge gap by developing prognostic models in participants of the multicenter prospective National Institutes of Health-supported Childhood Liver Disease Research Network. We extracted 14 clinical and biochemical variables at age 2 years to develop two models for future outcomes: 1) LT or death (LTD) and 2) first sentinel event (SE), either new onset ascites, hepatopulmonary syndrome (HPS), or gastrointestinal (GI) bleed. A total of 240 participants, enrolled between 2004 and 2017, were followed until a median age of 5.1 years (range, 2.0-13.3 years). Of these participants, 38 underwent LT (n = 37) or death (n = 1); cumulative incidence, 23.7% (95% confidence interval [CI], 16.2%-32.0%). Twenty-seven experienced either new-onset ascites (n = 13), HPS (n = 1), or GI bleed (n = 14). One participant had ascites and GI bleed concurrently; cumulative incidence, 21.5% (95% CI, 14.2%-29.8%) by age 10 years. The Cox proportional hazard model predicted risk of LTD, using total bilirubin, albumin, platelet count, and history of either ascites or cholangitis (BA LTD model), with a C-index of 0.88 (range, 0.86-0.89). A cause-specific hazard competing risk model predicted SE using platelet count and gamma glutamyltransferase levels (BA SE model) with a C-index of 0.81 (range, 0.80-0.84). Internal model validity was assessed using Harrell's C-index with cross-validation.

© 2020 The Authors. Hepatology Communications published by Wiley Periodicals LLC on behalf of the American Association for the Study of Liver Diseases.

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