Publications

Cellular crosstalk mediated by TGF-β drives epithelial-mesenchymal transition in patient-derived multi-compartment biliary organoids

Ayabe H, DePasquale EA, Amarachintha SP, Mourya R, Li W, Nalluri S, Fox SR, Konishi K, Shivakumar P, Bezerra JA 2025 Dec Nature communications 16

Increased serum GM-CSF at diagnosis of biliary atresia is associated with improved biliary drainage

Author Collaboration ft, Taylor SA, Harpavat S, Gromer KD, Andreev V, Loomes KM, Bezerra JA, Jarasvaraparn C, Wang K, Horslen S, Rosenthal P, Teckman J, Valentino PL, Ng VL, Karpen SJ, Sokol RJ, Alonso EM, Mack CL 2025 Pediatric Research

Diagnostic accuracy of serum matrix metalloproteinase-7 as a biomarker of biliary atresia in a large North American cohort

Author Collaboration TC, Pandurangi S, Mourya R, Nalluri S, Fei L, Dong S, Harpavat S, Guthery SL, Molleston JP, Rosenthal P, Sokol RJ, Wang KS, Ng V, Alonso EM, Hsu EK, Karpen SJ, Loomes KM, Magee JC, Shneider BL, Horslen SP, Teckman JH, Bezerra JA 2024 Jul Hepatology 80 152-162

Plasticity between type 2 innate lymphoid cell subsets and amphiregulin expression regulates epithelial repair in biliary atresia

Russi AE, Shivakumar P, Luo Z, Bezerra JA 2023 Oct Hepatology 78 1035-1049

Customized Postoperative Therapy Improves Bile Drainage in Biliary Atresia: A Single Center Preliminary Report

Pandurangi S, Kim S, Asai A, Bondoc A, Balistreri W, Campbell K, Miethke A, Peters A, Rogers M, Taylor A, Attia SL, Gibbons T, Mullapudi B, Sheridan R, Tiao G, Bezerra JA 2023 Aug Journal of Pediatric Surgery 58 1483-1488

Recent Increase in Incidence of Severe Acute Hepatitis of Unknown Etiology in Children is Associated with Infection with Adenovirus and Other Nonhepatotropic Viruses

Peters AL, Kim S, Mourya R, Asai A, Taylor A, Rogers M, Campbell K, Fei L, Miethke A, Balistreri WF, Bezerra JA 2023 Aug Journal of Pediatrics 259

Clinical spectrum and genetic causes of mitochondrial hepatopathy phenotype in children

Author Collaboration tC, Squires JE, Miethke AG, Alexander Valencia C, Hawthorne K, Henn L, Van Hove JL, Squires RH, Bove K, Horslen S, Kohli R, Molleston JP, Romero R, Alonso EM, Bezerra JA, Guthery SL, Hsu E, Karpen SJ, Loomes KM, Ng VL, Rosenthal P, Mysore K, Wang KS, Friederich MW, Magee JC, Sokol RJ 2023 Jun Hepatology Communications 7

Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy

Harpavat S, Hawthorne K, Setchell KD, Rivas MN, Henn L, Beil CA, Karpen SJ, Ng VL, Alonso EM, Bezerra JA, Guthery SL, Horslen S, Loomes KM, McKiernan P, Magee JC, Merion RM, Molleston JP, Rosenthal P, Thompson RJ, Wang KS, Sokol RJ, Shneider BL 2023 Mar Hepatology 77 862-873

Serum biomarkers correlated with liver stiffness assessed in a multicenter study of pediatric cholestatic liver disease

Author Collaboration TC, Leung DH, Devaraj S, Goodrich NP, Chen X, Rajapakshe D, Ye W, Andreev V, Minard CG, Guffey D, Molleston JP, Bass LM, Karpen SJ, Kamath BM, Wang KS, Sundaram SS, Rosenthal P, McKiernan P, Loomes KM, Jensen MK, Horslen SP, Bezerra JA, Magee JC, Merion RM, Sokol RJ, Shneider BL, Alonso E, Bass L, Kelly S, Riordan M, Melin-Aldana H, Bezerra J, Bove K, Heubi J, Miethke A, Tiao G, Denlinger J, Chapman E, Sokol R, Feldman A, Mack C, Narkewicz M, Suchy F, Sundaram SS, Van Hove J, Garcia B, Kauma M, Kocher K, Steinbeiss M, Lovell M, Loomes KM 2023 Feb Hepatology 77 530-545

Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency

Author Collaboration tN, Felzen A, van Wessel DB, Gonzales E, Thompson RJ, Jankowska I, Shneider BL, Sokal E, Grammatikopoulos T, Kadaristiana A, Jacquemin E, Spraul A, Lipiński P, Czubkowski P, Rock N, Shagrani M, Broering D, Nicastro E, Kelly D, Nebbia G, Arnell H, Fischler B, Hulscher JB, Serranti D, Arikan C, Polat E, Debray D, Lacaille F, Goncalves C, Hierro L, Muñoz Bartolo G, Mozer-Glassberg Y, Azaz A, Brecelj J, Dezsőfi A, Calvo PL, Grabhorn E, Hartleif S, van der Woerd WJ, Kamath BM, Wang JS, Li L, Durmaz Ö, Kerkar N, Jørgensen MH, Fischer R, Jimenez-Rivera C, Alam S, Cananzi M, Laverdure N, Bezerra JA 2023 Feb JHEP Reports 5

Maternal regulation of biliary disease in neonates via gut microbial metabolites

Jee JJ, Yang L, Shivakumar P, Xu Pp, Mourya R, Thanekar U, Yu P, Zhu Y, Pan Y, Wang H, Duan X, Ye Y, Wang B, Jin Z, Liu Y, Cao Z, Watanabe-Chailland M, Romick-Rosendale LE, Wagner M, Fei L, Luo Z, Ollberding NJ, Tang St, Bezerra JA 2022 Dec Nature communications 13

Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia

Author Collaboration C, Bass LM, Ye W, Hawthorne K, Leung DH, Murray KF, Molleston JP, Romero R, Karpen S, Rosenthal P, Loomes KM, Wang KS, Squires RH, Miethke A, Ng VL, Horslen S, Kyle Jensen M, Sokol RJ, Magee JC, Shneider BL, Alonso E, Bass L, Kelly S, Riordan M, Melin-Aldana H, Bezerra J, Bove K, Heubi J, Tiao G, Denlinger J, Chapman E, Sokol R, Feldman A, Mack C, Narkewicz M, Suchy F, Sundaram S, Van Hove J, Garcia B, Kauma M, Kocher K, Steinbeiss M, Lovell M, Loomes K, Piccoli D, Rand E, Russo P, Spinner N, Erlichman J, Stalford S, Pakstis D 2022 Sep Hepatology 76 712-726

Use of funded multicenter prospective longitudinal databases to inform clinical trials in rare diseases—Examination of cholestatic liver disease in Alagille syndrome

Author Collaboration ft, Shneider BL, Kamath BM, Magee JC, Goodrich NP, Loomes KM, Ye W, Spino C, Alonso EM, Molleston JP, Bezerra JA, Wang KS, Karpen SJ, Horslen SP, Guthery SL, Rosenthal P, Squires RH, Sokol RJ 2022 Aug Hepatology Communications 6 1910-1921

Serum Proteomics Uncovers Biomarkers of Clinical Portal Hypertension in Children With Biliary Atresia

Osborn J, Mourya R, Thanekar U, Su W, Fei L, Shivakumar P, Bezerra JA 2022 May Hepatology Communications 6 995-1004

The Liver Biopsy in Neonatal Cholestasis: Just a Cherry on Top?

Russi AE, Pandurangi S, Bezerra JA 2022 Mar Clinical Liver Disease 19 111-113

Biliary organoids uncover delayed epithelial development and barrier function in biliary atresia

Amarachintha SP, Mourya R, Ayabe H, Yang L, Luo Z, Li X, Thanekar U, Shivakumar P, Bezerra JA 2022 Jan Hepatology 75 89-103

The 2020 Nobel Prize for Medicine or Physiology for the Discovery of Hepatitis C Virus: A Triumph of Curiosity and Persistence

Ghany MG, Lok AS, Dienstag JL, Feinstone SM, Hoofnagle JH, Jake Liang T, Seeff LB, Cohen DE, Bezerra JA, Chung RT 2021 Nov Hepatology 74 2813-2823

Presentation and Outcomes of Infants With Idiopathic Cholestasis: A Multicenter Prospective Study

Author Collaboration tC, Hertel PM, Hawthorne K, Kim S, Finegold MJ, Shneider BL, Squires JE, Gupta NA, Bull LN, Murray KF, Kerkar N, Ng VL, Molleston JP, Bezerra JA, Loomes KM, Taylor SA, Schwarz KB, Turmelle YP, Rosenthal P, Magee JC, Sokol RJ 2021 Oct Journal of pediatric gastroenterology and nutrition 73 478-484

High Mobility Group Box 1 Release by Cholangiocytes Governs Biliary Atresia Pathogenesis and Correlates With Increases in Afflicted Infants

Mohanty SK, Donnelly B, Temple H, Ortiz-Perez A, Mowery S, Lobeck I, Dupree P, Poling HM, McNeal M, Mourya R, Jenkins T, Bansal R, Bezerra J, Tiao G 2021 Aug Hepatology 74 864-878

A single-cell view of biliary atresia

Russi AE, Bezerra JA 2021 Apr Nature Reviews Gastroenterology and Hepatology 18 219-220

Autoimmune Hepatitis: Predictors of Native Liver Survival in Children and Adolescents

Author Collaboration BG, Porta G, de Carvalho E, Santos JL, Gama J, Bezerra JA, Borges CV, Seixas RB, Ferreira A, Miura IK, Silveira TR, Silva LR, Fagundes ED, de Carvalho Trevizoli I, Brandão MA, Sawamura R, Vieira SM, Melere M, Ferreira CD, Porta A, Ferreira CT, Pugliese RP, Danesi VL, Marsilac M, Valadares MA, Vasconcelos JR, Meneses DG, de Paula M, Maia J 2021 Feb Journal of Pediatrics 229 95-101.e3

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

Author Collaboration ft, 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 2020 Dec Hepatology Communications 4 1824-1834

Longitudinal Outcomes in Young Patients with Alpha-1-Antitrypsin Deficiency with Native Liver Reveal that Neonatal Cholestasis is a Poor Predictor of Future Portal Hypertension

Author Collaboration C(, Teckman J, Rosenthal P, Hawthorne K, Spino C, Bass LM, Murray KF, Kerkar N, Magee JC, Karpen S, Heubi JE, Molleston JP, Squires RH, Kamath BM, Guthery SL, Loomes KM, Sherker AH, Sokol RJ, Alonso E, Bass L, Kelly S, Riordan M, Melin-Aldana H, Bezerra J, Bove K, Heubi J, Miethke A, Tiao G, Denlinger J, Chapman E, Sokol R, Feldman A, Mack C, Narkewicz M, Suchy F, Sundaram S, Van Hove J, Garcia B, Kauma M, Kocher K, Steinbeiss M, Lovell M, Loomes K, Piccoli D, Rand E, Russo P, Spinner N, Erlichman J, Stalford S, Pakstis D, King S 2020 Dec Journal of Pediatrics 227 81-86.e4

Nonfasted Liver Stiffness Correlates with Liver Disease Parameters and Portal Hypertension in Pediatric Cholestatic Liver Disease

Author Collaboration ft, Shneider BL, Goodrich NP, Ye W, Sawyers C, Molleston JP, Merion RM, Leung DH, Karpen SJ, Kamath BM, Cavallo L, Wang K, Teckman JH, Squires JE, Sundaram SS, Rosenthal P, Romero R, Murray KF, Loomes KM, Jensen MK, Bezerra JA, Bass LM, Sokol RJ, Magee JC 2020 Nov Hepatology Communications 4 1694-1707

Natural Course of Pediatric Portal Hypertension

Kassam AF, Goddard GR, Johnston ME, Cortez AR, Trout AT, Jenkins TM, Miethke AG, Campbell KM, Bezerra JA, Balistreri WF, Nathan JD, Alonso MH, Tiao GM, Bondoc AJ 2020 Sep Hepatology Communications 4 1346-1352

Clinical Best Practice Advice for Hepatology and Liver Transplant Providers During the COVID-19 Pandemic: AASLD Expert Panel Consensus Statement

Fix OK, Hameed B, Fontana RJ, Kwok RM, McGuire BM, Mulligan DC, Pratt DS, Russo MW, Schilsky ML, Verna EC, Loomba R, Cohen DE, Bezerra JA, Reddy KR, Chung RT 2020 Jul Hepatology 72 287-304

Regulation of bile duct epithelial injury by hepatic CD71+ erythroid cells

Yang L, Shivakumar P, Kinder J, Way SS, Donnelly B, Mourya R, Luo Z, Bezerra JA 2020 Jun JCI Insight 5

Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes

DiPaola F, Trout AT, Walther AE, Gupta A, Sheridan R, Campbell KM, Tiao G, Bezerra JA, Bove KE, Patel M, Nathan JD 2020 Apr Digestive Diseases and Sciences 65 1239-1251

Neurodevelopmental Outcomes in Preschool and School Aged Children with Biliary Atresia and Their Native Liver

Squires JE, Ng VL, Hawthorne K, Henn LL, Sorensen LG, Fredericks EM, Alonso EM, Murray KF, Loomes KM, Karpen SJ, Cavallo LA, Molleston JP, Bezerra JA, Rosenthal P, Squires RH, Wang KS, Schwarz KB, Arnon R, Magee JC, Sokol RJ 2020 Jan Journal of pediatric gastroenterology and nutrition 70 79-86

Gene Expression Signatures Associated With Survival Times of Pediatric Patients With Biliary Atresia Identify Potential Therapeutic Agents

Luo Z, Shivakumar P, Mourya R, Gutta S, Bezerra JA 2019 Oct Gastroenterology 157 1138-1152.e14

Identification of Polycystic Kidney Disease 1 Like 1 Gene Variants in Children With Biliary Atresia Splenic Malformation Syndrome

Author Collaboration ft, Berauer JP, Mezina AI, Okou DT, Sabo A, Muzny DM, Gibbs RA, Hegde MR, Chopra P, Cutler DJ, Perlmutter DH, Bull LN, Thompson RJ, Loomes KM, Spinner NB, Rajagopalan R, Guthery SL, Moore B, Yandell M, Harpavat S, Magee JC, Kamath BM, Molleston JP, Bezerra JA, Murray KF, Alonso EM, Rosenthal P, Squires RH, Wang KS, Finegold MJ, Russo P, Sherker AH, Sokol RJ, Karpen SJ 2019 Sep Hepatology 70 899-910

Prospective Assessment of Ultrasound Shear Wave Elastography for Discriminating Biliary Atresia from other Causes of Neonatal Cholestasis

Dillman JR, DiPaola FW, Smith SJ, Barth RA, Asai A, Lam S, Campbell KM, Bezerra JA, Tiao GM, Trout AT 2019 Sep Journal of Pediatrics 212 60-65.e3

Visualizing structures in confocal microscopy datasets through clusterization: A case study on bile ducts

Beltran LA, Cruz CU, Dos Santos JL, Shivakumar P, Bezerra J, Dal Sasso Freitas CM 2019 Jun 405-410

Correlation of Immune Markers With Outcomes in Biliary Atresia Following Intravenous Immunoglobulin Therapy

Author Collaboration TC, Kim S, Moore J, Alonso E, Bednarek J, Bezerra JA, Goodhue C, Karpen SJ, Loomes KM, Magee JC, Ng VL, Sherker AH, Smith C, Spino C, Venkat V, Wang K, Sokol RJ, Mack CL 2019 May Hepatology Communications 3 685-696

A Phase I/IIa Trial of Intravenous Immunoglobulin Following Portoenterostomy in Biliary Atresia

Author Collaboration TC, Mack CL, Spino C, Alonso EM, Bezerra JA, Moore J, Goodhue C, Ng VL, Karpen SJ, Venkat V, Loomes KM, Wang K, Sherker AH, Magee JC, Sokol RJ 2019 Apr Journal of pediatric gastroenterology and nutrition 68 495-501

Single cell RNA sequencing reveals regional heterogeneity of hepatobiliary innate lymphoid cells in a tissue-enriched fashion

Peters AL, Luo Z, Li J, Mourya R, Wang Y, Dexheimer P, Shivakumar P, Aronow B, Bezerra JA 2019 Apr PloS one 14

Diagnostic Accuracy of Serum Matrix Metalloproteinase-7 for Biliary Atresia

Yang L, Zhou Y, Xu Pp, Mourya R, Lei Hy, Cao Gq, Xiong Xl, Xu H, Duan Xf, Wang N, Fei L, Chang Xp, Zhang X, Jiang M, Bezerra JA, Tang St 2018 Dec Hepatology 68 2069-2077

Regulation of epithelial injury and bile duct obstruction by NLRP3, IL-1R1 in experimental biliary atresia

Yang L, Mizuochi T, Shivakumar P, Mourya R, Luo Z, Gutta S, Bezerra JA 2018 Nov Journal of Hepatology 69 1136-1144

Impact of Steroid Therapy on Early Growth in Infants with Biliary Atresia: The Multicenter Steroids in Biliary Atresia Randomized Trial

Author Collaboration CN, Alonso EM, Ye W, Hawthorne K, Venkat V, Loomes KM, Mack CL, Hertel PM, Karpen SJ, Kerkar N, Molleston JP, Murray KF, Romero R, Rosenthal P, Schwarz KB, Shneider BL, Suchy FJ, Turmelle YP, Wang KS, Sherker AH, Sokol RJ, Bezerra JA, Magee JC 2018 Nov Journal of Pediatrics 202 179-185.e4

Biliary Atresia: Clinical and Research Challenges for the Twenty-First Century

Bezerra JA, Wells RG, Mack CL, Karpen SJ, Hoofnagle JH, Doo E, Sokol RJ 2018 Sep Hepatology 68 1163-1173

Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study

Author Collaboration CL, Ng VL, Sorensen LG, Alonso EM, Fredericks EM, Ye W, Moore J, Karpen SJ, Shneider BL, Molleston JP, Bezerra JA, Murray KF, Loomes KM, Rosenthal P, Squires RH, Wang K, Arnon R, Schwarz KB, Turmelle YP, Haber BH, Sherker AH, Magee JC, Sokol RJ, Hertel PM, Harpavat S, Brandt ML, Leung DH, Karnsakul W, Torrance R, Hall S, Doo E, Hoofnagle JH, Whitington P, Bass L, Miethke AG, Heubi JE, Setchell K, Bove KE, Tiao G, Mack CL, Narkewicz MR, Feldman AG, Sundaram SS, Suchy FJ, Karrer FM, Lovell M, Van Hove JL, Rand EB, Squires JE, Venkat VL, Sindhi R 2018 May Journal of Pediatrics 196 139-147.e3

Gene-disease associations identify a connectome with shared molecular pathways in human cholangiopathies

Luo Z, Jegga AG, Bezerra JA 2018 Feb Hepatology 67 676-689

A novel pkhd1 mutation interacts with the nonobese diabetic genetic background to cause autoimmune cholangitis

Huang W, Rainbow DB, Wu Y, Adams D, Shivakumar P, Kottyan L, Karns R, Aronow B, Bezerra J, Gershwin ME, Peterson LB, Wicker LS, Ridgway WM 2018 Jan Journal of Immunology 200 147-162

Large-scale proteomics identifies MMP-7 as a sentinel of epithelial injury and of biliary atresia

Lertudomphonwanit C, Mourya R, Fei L, Zhang Y, Gutta S, Yang L, Bove KE, Shivakumar P, Bezerra JA 2017 Nov Science translational medicine 9

Cxcr2 signaling and the microbiome suppress inflammation, bile duct injury, and the phenotype of experimental biliary atresia

Jee J, Mourya R, Shivakumar P, Fei L, Wagner M, Bezerra JA 2017 Aug PloS one 12

Initial assessment of the infant with neonatal cholestasis-Is this biliary atresia?

Author Collaboration CL, Shneider BL, Moore J, Kerkar N, Magee JC, Ye W, Karpen SJ, Kamath BM, Molleston JP, Bezerra JA, Murray KF, Loomes KM, Whitington PF, Rosenthal P, Squires RH, Guthery SL, Arnon R, Schwarz KB, Turmelle YP, Sherker AH, Sokol RJ, Hertel PM, Alonso EM, Fredericks EM, Haber BH, Wang KS, Sorensen LG, Ng VL, Bass L, Lin H, Goodrich NP, Hawthorne K, Heubi JE, Sheridan R, Fei L, Teckman J, Spino CA 2017 May PloS one 12

Paracrine signals regulate human liver organoid maturation from induced pluripotent stem cells

Asai A, Aihara E, Watson C, Mourya R, Mizuochi T, Shivakumar P, Phelan K, Mayhew C, Helmrath M, Takebe T, Wells J, Bezerra JA 2017 Mar Development (Cambridge) 144 1056-1064

Pediatric Cholestatic Syndromes

Squires JE, Balistreri WF, Bezerra JA 2017 Jan 976-994

Preferential TNFα signaling via TNFR2 regulates epithelial injury and duct obstruction in experimental biliary atresia

Shivakumar P, Mizuochi T, Mourya R, Gutta S, Yang L, Luo Z, Bezerra JA 2017 JCI Insight 2

Novel approaches to the treatment of biliary atresia

Li Y, Bezerra JA 2016 Dec Clinical Liver Disease 8 145-149

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

Verkade HJ, Bezerra JA, Davenport M, Schreiber RA, Mieli-Vergani G, Hulscher JB, Sokol RJ, Kelly DA, Ure B, Whitington PF, Samyn M, Petersen C 2016 Sep Journal of Hepatology 65 631-642

MDR3 mutation analysis: A step closer to precision medicine

Bezerra JA 2016 May Hepatology 63 1421-1423

Increased frequency of double and triple heterozygous gene variants in children with intrahepatic cholestasis

Goldschmidt ML, Mourya R, Connor J, Dexheimer P, Karns R, Miethke A, Sheridan R, Zhang K, Bezerra JA 2016 Apr Hepatology Research 46 306-311

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

Author Collaboration CL, Shneider BL, Magee JC, Karpen SJ, Rand EB, Narkewicz MR, Bass LM, Schwarz K, Whitington PF, Bezerra JA, Kerkar N, Haber B, Rosenthal P, Turmelle YP, Molleston JP, Murray KF, Ng VL, Wang KS, Romero R, Squires RH, Arnon R, Sherker AH, Moore J, Ye W, Sokol RJ 2016 Mar Journal of Pediatrics 170 211-217.e2

Pathogenesis of biliary atresia: Defining biology to understand clinical phenotypes

Asai A, Miethke A, Bezerra JA 2015 Jun Nature Reviews Gastroenterology and Hepatology 12 342-352

Natural killer cells promote long-term hepatobiliary inflammation in a low-dose rotavirus model of experimental biliary atresia

Squires JE, Shivakumar P, Mourya R, Bessho K, Walters S, Bezerra JA 2015 May PloS one 10

IL-33 facilitates oncogene-induced cholangiocarcinoma in mice by an interleukin-6-sensitive mechanism

Yamada D, Rizvi S, Razumilava N, Bronk SF, Davila JI, Champion MD, Borad MJ, Bezerra JA, Chen X, Gores GJ 2015 May Hepatology 61 1627-1642

Reply

Mourya R, Bessho K, Shivakumar P, Bezerra JA 2015 Feb Hepatology 61 732-733

Transplantation for Cholestatic Liver Disease in Children

Ibrahim SH, Bezerra JA, Balistreri WF 2015 Jan 288-304

Total serum bilirubin predicts fat-soluble vitamin deficiency better than serum bile acids in infants with biliary atresia

Author Collaboration CL, Venkat VL, Shneider BL, Magee JC, Turmelle Y, Arnon R, Bezerra JA, Hertel PM, Karpen SJ, Kerkar N, Loomes KM, Molleston J, Murray KF, Ng VL, Raghunathan T, Rosenthal P, Schwartz K, Sherker AH, Sokol RJ, Teckman J, Wang K, Whitington PF, Heubi JE 2014 Dec Journal of pediatric gastroenterology and nutrition 59 702-707

Biliary repair and carcinogenesis are mediated by IL-33-dependent cholangiocyte proliferation

Li J, Razumilava N, Gores GJ, Walters S, Mizuochi T, Mourya R, Bessho K, Wang YH, Glaser SS, Shivakumar P, Bezerra JA 2014 Jul Journal of Clinical Investigation 124 3241-3251

Gene expression signature for biliary atresia and a role for interleukin-8 in pathogenesis of experimental disease

Bessho K, Mourya R, Shivakumar P, Walters S, Magee JC, Rao M, Jegga AG, Bezerra JA 2014 Jul Hepatology 60 211-223

Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia the start randomized clinical trial

Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, Erlichman J, Haber B, Hertel PM, Karpen SJ, Kerkar N, Loomes KM, Molleston JP, Murray KF, Romero R, Schwarz KB, Shepherd R, Suchy FJ, Turmelle YP, Whitington PF, Moore J, Sherker AH, Robuck PR, Sokol RJ 2014 May JAMA 311 1750-1759

Perforin and granzymes work in synergy to mediate cholangiocyte injury in experimental biliary atresia

Shivakumar P, Mourya R, Bezerra JA 2014 Feb Journal of Hepatology 60 370-376

Replication of a GWAS signal in a Caucasian population implicates ADD3 in susceptibility to biliary atresia

Tsai EA, Grochowski CM, Loomes KM, Bessho K, Hakonarson H, Bezerra JA, Russo PA, Haber BA, Spinner NB, Devoto M 2014 Feb Human genetics 133 235-243

Extrahepatic anomalies in infants with biliary atresia: Results of a Large prospective North American multicenter study

Schwarz KB, Haber BH, Rosenthal P, Mack CL, Moore J, Bove K, Bezerra JA, Karpen SJ, Kerkar N, Shneider BL, Turmelle YP, Whitington PF, Molleston JP, Murray KF, Ng VL, Romero R, Wang KS, Sokol RJ, Magee JC 2013 Nov Hepatology 58 1724-1731

Integrative genomics identifies candidate microRNAs for pathogenesis of experimental biliary atresia

Bessho K, Shanmukhappa K, Sheridan R, Shivakumar P, Mourya R, Walters S, Kaimal V, Dilbone E, Jegga AG, Bezerra JA 2013 Oct BMC Systems Biology 7

Identification of intramural epithelial networks linked to peribiliary glands that express progenitor cell markers and proliferate after injury in mice

Dipaola F, Shivakumar P, Pfister J, Walters S, Sabla G, Bezerra JA 2013 Oct Hepatology 58 1486-1496

Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia

Shneider BL, Magee JC, Bezerra JA, Haber B, Karpen SJ, Raghunathan T, Rosenthal P, Schwarz K, Suchy FJ, Kerkar N, Turmelle Y, Whitington PF, Robuck PR, Sokol RJ 2012 Sep Pediatrics 130 e607-e614

50 years ago in the journal of pediatrics: Persistent jaundice in infancy

Dipaola F, Bezerra JA 2012 Jul Journal of Pediatrics 161 164

The beneficial impact of revision of kasai portoenterostomy for biliary atresia: An institutional study

Bondoc AJ, Taylor JA, Alonso MH, Nathan JD, Wang Y, Balistreri WF, Bezerra JA, Ryckman FC, Tiao GM 2012 Mar Annals of surgery 255 570-576

Th2 signals induce epithelial injury in mice and are compatible with the biliary atresia phenotype

Li J, Bessho K, Shivakumar P, Mourya R, Mohanty SK, Dos Santos JL, Miura IK, Porta G, Bezerra JA 2011 Nov Journal of Clinical Investigation 121 4244-4256

Dendritic cells regulate natural killer cell activation and epithelial injury in experimental biliary atresia

Saxena V, Shivakumar P, Sabla G, Mourya R, Chougnet C, Bezerra JA 2011 Sep Science translational medicine 3

Biliary Atresia: Will blocking inflammation tame the disease?

Bessho K, Bezerra JA 2011 Feb Annual review of medicine 62 171-185

Biliary atresia and other disorders of the extrahepatic bile ducts

Balistreri WF, Bezerra JA, Ryckman FC 2011 Jan 155-176

Lampe1: An ENU-germline mutation causing spontaneous hepatosteatosis identified through targeted exon-enrichment and next-generation sequencing

Sheridan R, Lampe K, Shanmukhappa SK, Putnam P, Keddache M, Divanovic S, Bezerra J, Hoebe K 2011 PloS one 6

Biliary atresia in Brazil: Where we are and where we are going

Bezerra JA 2010 Nov Jornal de Pediatria 86 445-447

Analysis of gene mutations in children with cholestasis of undefined etiology

Matte U, Mourya R, Miethke A, Liu C, Kauffmann G, Moyer K, Zhang K, Bezerra JA 2010 Oct Journal of pediatric gastroenterology and nutrition 51 488-493

Analysis of gene mutations in children with cholestasis of undefined etiology

Matte U, Mourya R, Miethke A, Liu C, Kauffmann G, Moyer K, Zhang K, Bezerra JA 2010 Oct Journal of pediatric gastroenterology and nutrition 51 488-493

Advances in biliary atresia: From patient care to research

Santos JL, Carvalho E, Bezerra JA 2010 Jun Brazilian Journal of Medical and Biological Research 43 522-527

Staging of biliary atresia at diagnosis by molecular profiling of the liver

Moyer K, Kaimal V, Pacheco C, Mourya R, Xu H, Shivakumar P, Chakraborty R, Rao M, Magee JC, Bove K, Aronow BJ, Jegga AG, Bezerra JA 2010 May Genome Medicine 2

Macrophages are targeted by rotavirus in experimental biliary atresia and induce neutrophil chemotaxis by Mip2/Cxcl2

Mohanty SK, Ivantes CA, Mourya R, Pacheco C, Bezerra JA 2010 Apr Pediatric Research 67 345-351

Cholestatic liver disease in children

Santos JL, Choquette M, Bezerra JA 2010 Feb Current gastroenterology reports 12 30-39

Neonatal NK cells target the mouse duct epithelium via Nkg2d and drive tissue-specific injury in experimental biliary atresia

Shivakumar P, Sabla GE, Whitington P, Chougnet CA, Bezerra JA 2009 Aug Journal of Clinical Investigation 119 2281-2290

Plasmin-mediated proteolysis is required for hepatocyte growth factor activation during liver repair

Shanmukhappa K, Matte U, Degen JL, Bezerra JA 2009 May Journal of Biological Chemistry 284 12917-12923

Cholestasis: Current issues and plan for the future

Haber B, Ferreira CT, Aw M, Bezerra J, Sturm E, Thompson R, D'Agostino D, McKiernan P 2008 Aug Journal of pediatric gastroenterology and nutrition 47 220-224

Temporal-spatial activation of apoptosis and epithelial injury in murine experimental biliary atresia

Erickson N, Mohanty SK, Shivakumar P, Sabla G, Chakraborty R, Bezerra JA 2008 May Hepatology 47 1567-1577

Growth failure and outcomes in infants with biliary atresia: A report from the biliary atresia research consortium

DeRusso PA, Ye W, Shepherd R, Haber BA, Shneider BL, Whitington PF, Schwarz KB, Bezerra JA, Rosenthal P, Karpen S, Squires RH, Magee JC, Robuck PR, Sokol RJ 2007 Nov Hepatology 46 1632-1638

Screening and outcomes in biliary atresia: Summary of a national institutes of health workshop

Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH 2007 Aug Hepatology 46 566-581

Reply

Shneider BL, Bezerra JA, Sokol RJ, Whitington PF 2007 Jun Journal of Pediatrics 150 e89-e90

Effector Role of Neonatal Hepatic CD8+ Lymphocytes in Epithelial Injury and Autoimmunity in Experimental Biliary Atresia

Shivakumar P, Sabla G, Mohanty S, McNeal M, Ward R, Stringer K, Caldwell C, Chougnet C, Bezerra JA 2007 Jun Gastroenterology 133 268-277

Extrahepatic biliary atresia: Current concepts and future directions

De Carvalho E, Pontes Ivantes CA, Bezerra JA 2007 Mar Jornal de Pediatria 83 105-120

Effect of rotavirus strain on the murine model of biliary atresia

Allen SR, Jafri M, Donnelly B, McNeal M, Witte D, Bezerra J, Ward R, Tiao GM 2007 Feb Journal of virology 81 1671-1679

Biliary atresia and other disorders of the extrahepatic bile ducts

Balistreri WF, Balistreri WF, Bezerra JA, Bezerra JA, Ryckman FC, Ryckman FC 2007 Jan 247-269

Novel Resequencing Chip Customized to Diagnose Mutations in Patients With Inherited Syndromes of Intrahepatic Cholestasis

Liu C, Aronow BJ, Jegga AG, Wang N, Miethke A, Mourya R, Bezerra JA 2007 Jan Gastroenterology 132 119-126

High prevalence of α-1-antitrypsin heterozygosity in children with chronic liver disease

Campbell KM, Arya G, Ryckman FC, Alonso M, Tiao G, Balistreri WF, Bezerra JA 2007 Jan Journal of pediatric gastroenterology and nutrition 44 99-103

Urokinase-type plasminogen activator supports liver repair independent of its cellular receptor

Shanmukhappa K, Sabla GE, Degen JL, Bezerra JA 2006 Nov BMC Gastroenterology 6

Pancreas Recovery Following Cerulein-Induced Pancreatitis Is Impaired in Plasminogen-Deficient Mice

Lugea A, Nan L, French SW, Bezerra JA, Gukovskaya AS, Pandol SJ 2006 Sep Gastroenterology 131 885-899

The next challenge in pediatric cholestasis: Deciphering the pathogenesis of biliary atresia

Bezerra JA 2006 Jul Journal of pediatric gastroenterology and nutrition 43 S23-S29

Biliary atresia - Translational research on key molecular processes regulating biliary injury and obstruction

Bezerra JA 2006 May Chang Gung Medical Journal 29 222-230

Loss of interleukin-12 modifies the pro-inflammatory response but does not prevent duct obstruction in experimental biliary atresia

Mohanty SK, Shivakumar P, Sabla G, Bezerra JA 2006 Apr BMC Gastroenterology 6

A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000

Shneider BL, Brown MB, Haber B, Whitington PF, Schwarz K, Squires R, Bezerra J, Shepherd R, Rosenthal P, Hoofnagle JH, Sokol RJ 2006 Apr Journal of Pediatrics 148 467-467.e

Whatever happened to "neonatal hepatitis"?

Balistreri WF, Bezerra JA 2006 Feb Clinics in Liver Disease 10 27-53

Pediatric hepatology: Progress in deciphering mechanisms and advancing therapy

Bezerra JA, Balistreri WF 2006 Feb Clinics in Liver Disease 10 ix-xii

Genetic modifiers of cholestatic liver disease: An evolving field

Bezerra J, Shneider BL 2006 Jan Journal of pediatric gastroenterology and nutrition 42 7-8

Potential etiologies of biliary atresia

Bezerra JA 2005 Oct Pediatric Transplantation 9 646-651

Analysis of the biliary transcriptome in experimental biliary atresia

Carvalho E, Liu C, Shivakumar P, Sabla G, Aronow B, Bezerra JA 2005 Aug Gastroenterology 129 713-717

Hepatic to pancreatic switch defines a role for hemostatic factors in cellular plasticity in mice

Shanmukhappa K, Mourya R, Sabla GE, Degen JL, Bezerra JA 2005 Jul Proceedings of the National Academy of Sciences of the United States of America 102 10182-10187

Intrahepatic cholestasis: Summary of an American Association for the Study of Liver Diseases Single-Topic Conference

Balistreri WF, Bezerra JA, Jansen P, Karpen SJ, Shneider BL, Suchy FJ 2005 Jul Hepatology 42 222-235

A challenge on the use of the words Embryonic and Perinatal in the context of biliary atresia [3] (multiple letters)

Davenport M, Bezerra JA, Sokol RJ 2005 Feb Hepatology 41 403-404

Transplantation for Cholestatic Liver Disease in the Pediatric Patient

Choe BH, Bezerra JA, Balistreri WF 2005 Jan 303-321

Liver transplantation in children younger than 1 year-the Cincinnati experience

Tiao GM, Alonso M, Bezerra J, Yazigi N, Heubi J, Balistreri W, Bucuvalas J, Ryckman F 2005 Jan Journal of Pediatric Surgery 40 268-273

Obstruction of extrahepatic bile ducts by lymphocytes is regulated by IFN-γ in experimental biliary atresia

Shivakumar P, Campbell KM, Sabla GE, Miethke A, Tiao G, McNeal MM, Ward RL, Beserra JA 2004 Aug Journal of Clinical Investigation 114 322-329

Biliary atresia and Th1 function: Linking lymphocytes and bile ducts

Shivakumar P, Bezerra JA 2004 Jul Pediatric Research 56 9-10

Coordinate Expression of Regulatory Genes Differentiates Embryonic and Perinatal Forms of Biliary Atresia

Zhang DY, Sabla G, Shivakumar P, Tiao G, Sokol RJ, Mack C, Shneider BL, Aronow B, Bezerra JA 2004 Apr Hepatology 39 954-962

Transcriptional reprogramming in murine liver defines the physiologic consequences of biliary obstruction

Campbell KM, Sabla GE, Bezerra JA 2004 Jan Journal of Hepatology 40 14-23

Gene expression profiling in biliary atresia [6] (multiple letters)

Smith MJ, Coffey JC, Wang JH, Cotter TG, Redmond HP, Bezerra JA, Aronow BJ 2003 Mar Lancet 361 971-972

Plasminogen directs the pleiotropic effects of uPA in liver injury and repair

Currier AR, Sabla G, Locaputo S, Melin-Aldana H, Degen JL, Bezerra JA 2003 Mar American Journal of Physiology - Gastrointestinal and Liver Physiology 284 G508-G515

Genetic induction of proinflammatory immunity in children with biliary atresia

Bezerra JA, Tiao G, Ryckman FC, Alonso M, Sabla GE, Shneider B, Sokol RJ, Aronow BJ 2002 Nov Lancet 360 1653-1659

Independent and overlapping transcriptional activation during liver development and regeneration in mice

Kelley-Loughnane N, Sabla GE, Ley-Ebert C, Aronow BJ, Bezerra JA 2002 Hepatology 35 525-534

Hepatocytes and endothelial cells: Joining forces to conquer development

Bezerra JA 2002 Pediatric Research 51 413

Novel genes and functional relationships in the adult mouse gastrointestinal tract identified by microarray analysis

Bates MD, Erwin CR, Philip SL, Wiginton D, Bezerra JA, Schatzman LC, Jegga AG, Ley-Ebert C, Williams SS, Steinbrecher KA, Warner BW, Cohen MB, Aronow BJ 2002 Gastroenterology 122 1467-1482

Plasminogen deficiency leads to impaired lobular reorganization and matrix accumulation after chronic liver injury

Pohl JF, Melin-Aldana H, Sabla G, Degen JL, Bezerra JA 2001 Dec American Journal of Pathology 159 2179-2186

Genetic manipulation of fibrinogen and fibrinolysis in mice

Degen JL, Drew AF, Palumbo JS, Kombrinck KW, Bezerra JA, Danton MJ, Holmbäck K, Suh TT 2001 Annals of the New York Academy of Sciences 936 276-290

Plasminogen deficiency results in poor clearance of non-fibrin matrix and persistent activation of hepatic stellate cells after an acute injury

Ng VL, Sabla GE, Melin-Aldana H, Kelley-Loughnane N, Degen JL, Bezerra JA 2001 Journal of Hepatology 35 781-789

Cholestatic syndromes of infancy and childhood

Bezerra JA, Balistreri WF 2001 Seminars in Gastrointestinal Disease 12 54-65

Plasminogen activators direct reorganization of the liver lobule after acute injury

Bezerra JA, Currier AR, Melin-Aldana H, Sabla G, Bugge TH, Kombrinck KW, Degen JL 2001 American Journal of Pathology 158 921-929

Long-term outcome after partial external biliary diversion for intractable pruritus in patients with intrahepatic cholestasis

Ng VL, Ryckman FC, Porta G, Miura IK, De Carvalho E, Servidoni MF, Bezerra JA, Balistreri WF 2000 Feb Journal of pediatric gastroenterology and nutrition 30 152-156

Hepatocyte transplantation: Advancing biology and treating children

Ng VL, Alonso M, Bezerra JA 2000 Clinics in Liver Disease 4 929-945

The unique nature of the pediatric liver

Bezerra JA, Balistreri WF 2000 Clinics in Liver Disease 4 xi-xv

Plasminogen deficiency leads to impaired remodeling after a toxic injury to the liver

Bezerra JA, Bugge TH, Melin-Aldana H, Sabla G, Kombrinck KW, Witte DP, Degen JL 1999 Dec Proceedings of the National Academy of Sciences of the United States of America 96 15143-15148

Zonal regulation of gene expression during liver regeneration of urokinase transgenic mice

Locaputo S, Carrick TL, Bezerra JA 1999 Hepatology 29 1106-1113

Image of the month

Rudolph JA, Bezerra J, Alonso M 1999 Gastroenterology 116 788

Pylephlebitis

Pohl JF, Murarka P, Farrell MK, Bezerra J 1999 Journal of Pediatrics 135 529

Intrahepatic cholestasis: Order out of chaos

Bezerra JA, Balistreri WE 1999 Gastroenterology 117 1496-1498

Molecular and enzymatic analyses of lysosomal acid lipase in cholesteryl ester storage disease

Du H, Sheriff S, Bezerra J, Leonova T, Grabowski GA 1998 Jun Molecular genetics and metabolism 64 126-134

Biological effects of targeted inactivation of hepatocyte growth factor- like protein in mice

Bezerra JA, Carrick TL, Degen JL, Witte D, Degen SJ 1998 Mar Journal of Clinical Investigation 101 1175-1183

Hepatobiliary disorders of childhood pathophysiology, clinical features and treatment: Foreword

Bezerra JA, Balistreri WF 1998 Seminars in liver disease 18 193

Liver development: A paradigm for hepatobiliary disease in later life

Bezerra JA 1998 Seminars in liver disease 18 203-216

Differential expression of liver growth factors in tyrosinemic transgenic livers: 47

Bezerra JA, Carrick TL, Pavlus JE, Grompe M 1997 Oct Journal of pediatric gastroenterology and nutrition 25 454

Effects of protein malnutrition on liver cytochrome P450s

Lee PC, Struve MF, Bezerra JA, Duncan B 1997 Oct Nutrition Research 17 1577-1587

Expansion of transplanted hepatocytes during liver regeneration

Yazigi NA, Carrick TL, Bucuvalas JC, Schmidt CS, Balistreri WF, Bezerra JA 1997 Sep Transplantation 64 816-820

Hepatocyte growth factor (HGF) mrna expression is increased in diseased liver and decreased in regenerating liver nodules in urokinase transgenic mice

Carrick TL, Chen T, Bezerra JA 1996 Oct Journal of pediatric gastroenterology and nutrition 23 351

Progress in pediatric hepatology

Bezerra JA, Balistreri WF 1996 Current Opinion in Gastroenterology 12 237-245

Hepatocyte transplantation: 'only the strong survive'

Bezerra JA, Balistreri WF 1996 Hepatology 24 1321-1323

Use of a single solution for oral rehydration and maintenance therapy of infants with diarrhea and mild to moderate dehydration

Cohen MB, Mezoff AG, Laney DW, Bezerra JA, Beane BM, Drazner D, Baker R, Moran JR 1995 Pediatrics 95 639-645

Increased expression of mRNA for hepatocyte growth factor-like protein during liver regeneration and inflammation

Bezerra JA, Laney DW, Degen SJ 1994 Aug Biochemical and Biophysical Research Communications 203 666-673

Upregulation of Escherichia coli heat-stable enterotoxin receptor in regenerating rat liver

Laney DW, Bezerra JA, Kosiba JL, Degen SJ, Cohen MB 1994 American Journal of Physiology - Gastrointestinal and Liver Physiology 266 G899-G906

Hepatocyte‐specific expression of the mouse hepatocyte growth factor–like protein

Bezerra JA, Witte DP, Arono BJ, Friezner SJ 1993 Aug Hepatology 18 394-399

Are hepatocyte growth factor‐like protein and macrophage stimulating protein the same protein?

Bezerra JA, Han S, Danton MJ, Friezner Degen SJ 1993 Apr Protein Science 2 666-668

A one-month-old infant who had a 'double bubble'

Bezerra JA, Duncan B, Anderson F 1992 Hospital Practice 27 255+257-258

Treatment of infants with acute diarrhea: What's recommended and what's practiced

Bezerra JA, Stathos TH, Duncan B, Gaines JA, Udall JN 1992 Pediatrics 90 1-4

Age-related changes in sucrase and lactase activity in the small intestine of 3- and 10-week-old obese mice (C57bl/6jobob)

Flores CA, Bezerra J, Bustamante SA, Goda T, Macdonal P, Kaplan ML, Koldovsky O 1990 Jun Journal of the American College of Nutrition 9 255-260

Dietary management of acute diarrhea: Fast or feed?

Bezerra JA, Duncan B, Udall J 1990 International Pediatrics 5 30-36

Intestinal permeability to intact lactose in newborns and adults

Bezerra JA, Thompson SH, Morse M, Koldovský O, Udall JN 1990 Neonatology 58 334-342

Diagnosis and management of acute diarrhea in children

Bezerra JA, Cohen MB 1990 Current opinion in pediatrics 2 1134-1140

Assessment of lactose absorption by measurement of urinary galactose

Grant JD, Bezerra JA, Thompson SH, Lemen RJ, Koldovsky O, Udall JN 1989 Oct Gastroenterology 97 895-899

Effect of diet on intestinal and pancreatic enzyme activities in the pig

Flores CA, Brannon PM, Bustamante SA, Bezerra J, Butler KT, Goda T, Koldovsky O 1988 Journal of pediatric gastroenterology and nutrition 7 914-921

Chronic effects of an α-glucosidase inhibitor (Bay o 1248) on intestinal disaccharidase activity in normal and diabetic mice

Lee SM, Bustamante S, Flores C, Bezerra J, Goda T, Koldovský O 1987 Journal of Pharmacology and Experimental Therapeutics 240 132-137

Effect of a high-dextrose diet on sucrase and lactase activity in jejunum of obese mice (C57bL/6J obob)

Flores CA, Bezerra J, Goda T, Bustamante S, MaCdonald MP, Kaplan M, Koldovsky O 1986 Jan Journal of the American College of Nutrition 5 565-575

Effect of a high dextrose diet on sucrase and lactase activity in jejunum of obese mice (C57 BL/6J-ob)

Koldovsky O, Bezerra J, Bustamante S 1985 Federation Proceedings 44 No. 1161

Postweaning changes of jejunal protein and activity of disaccharidases in obese mice and lean littermates

Goda T, Bezerra J, Bustamante S 1985 Federation Proceedings 44 No.4407

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