{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T18:09:52Z","timestamp":1789582192460,"version":"build-2803163510"},"reference-count":93,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2020,7,11]],"date-time":"2020-07-11T00:00:00Z","timestamp":1594425600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2020,7,11]],"date-time":"2020-07-11T00:00:00Z","timestamp":1594425600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"funder":[{"name":"Albireo AB"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Hepatol Int"],"published-print":{"date-parts":[[2020,9]]},"DOI":"10.1007\/s12072-020-10070-w","type":"journal-article","created":{"date-parts":[[2020,7,11]],"date-time":"2020-07-11T11:02:41Z","timestamp":1594465361000},"page":"677-689","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":87,"title":["Ileal bile acid transporter inhibition as an anticholestatic therapeutic target in biliary atresia and other cholestatic disorders"],"prefix":"10.1007","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3379-7592","authenticated-orcid":false,"given":"Saul J.","family":"Karpen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deirdre","family":"Kelly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cara","family":"Mack","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Stein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,7,11]]},"reference":[{"key":"10070_CR1","doi-asserted-by":"crossref","first-page":"e63","DOI":"10.1542\/neo.14-2-e63","volume":"14","author":"AG Feldman","year":"2013","unstructured":"Feldman AG, Sokol RJ. Neonatal cholestasis. NeoReviews 2013;14:e63\u2013e73","journal-title":"NeoReviews"},{"key":"10070_CR2","doi-asserted-by":"crossref","first-page":"1132","DOI":"10.1136\/adc.2006.101451","volume":"92","author":"DA Kelly","year":"2007","unstructured":"Kelly DA, Davenport M. Current management of biliary atresia. Arch Dis Child 2007;92:1132\u20131135","journal-title":"Arch Dis Child"},{"key":"10070_CR3","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1038\/nrgastro.2015.74","volume":"12","author":"A Asai","year":"2015","unstructured":"Asai A, Miethke A, Bezerra JA. Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes. Nat Rev Gastroenterol Hepatol 2015;12:342\u2013352","journal-title":"Nat Rev Gastroenterol Hepatol"},{"key":"10070_CR4","doi-asserted-by":"crossref","first-page":"218","DOI":"10.12688\/f1000research.16732.1","volume":"8","author":"A Wehrman","year":"2019","unstructured":"Wehrman A, Waisbourd-Zinman O, Wells RG. Recent advances in understanding biliary atresia. F1000Research 2019;8:218","journal-title":"F1000Research"},{"key":"10070_CR5","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/j.jpeds.2017.05.006","volume":"187","author":"PC Hopkins","year":"2017","unstructured":"Hopkins PC, Yazigi N, Nylund CM. Incidence of biliary atresia and timing of hepatoportoenterostomy in the United States. J Pediatr 2017;187:253\u2013257","journal-title":"J Pediatr"},{"key":"10070_CR6","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1002\/hep.22182","volume":"47","author":"CH Hsiao","year":"2008","unstructured":"Hsiao CH, Chang MH, Chen HL, Lee HC, Wu TC, Lin CC, et al. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 2008;47:1233\u20131240","journal-title":"Hepatology"},{"key":"10070_CR7","first-page":"646","volume":"1","author":"P Vic","year":"1994","unstructured":"Vic P, Gestas P, Mallet EC, Arnaud JP. Biliary atresia in French Polynesia. Retrospective study of 10 years. Arch Pediatr 1994;1:646\u2013651","journal-title":"Arch Pediatr"},{"key":"10070_CR8","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1186\/s12920-018-0440-0","volume":"11","author":"SR Cameron-Christie","year":"2018","unstructured":"Cameron-Christie SR, Wilde J, Gray A, Tankard R, Bahlo M, Markie D, et al. Genetic investigation into an increased susceptibility to biliary atresia in an extended New Zealand Maori family. BMC Med Genom 2018;11:121","journal-title":"BMC Med Genom"},{"key":"10070_CR9","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/S0140-6736(99)03492-3","volume":"355","author":"PJ McKiernan","year":"2000","unstructured":"McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000;355:25\u201329","journal-title":"Lancet"},{"key":"10070_CR10","doi-asserted-by":"crossref","first-page":"e1663","DOI":"10.1542\/peds.2015-3570","volume":"136","author":"KS Wang","year":"2015","unstructured":"Wang KS. Newborn screening for biliary atresia. Pediatrics 2015;136:e1663\u2013e1669","journal-title":"Pediatrics"},{"key":"10070_CR11","doi-asserted-by":"crossref","first-page":"132089","DOI":"10.5402\/2012\/132089","volume":"2012","author":"BE Wildhaber","year":"2012","unstructured":"Wildhaber BE. Biliary atresia: 50 years after the first kasai. ISRN Surg 2012;2012:132089","journal-title":"ISRN Surg"},{"key":"10070_CR12","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1016\/j.jhep.2016.04.032","volume":"65","author":"HJ Verkade","year":"2016","unstructured":"Verkade HJ, Bezerra JA, Davenport M, Schreiber RA, Mieli-Vergani G, Hulscher JB, et al. Biliary atresia and other cholestatic childhood diseases: advances and future challenges. J Hepatol 2016;65:631\u2013642","journal-title":"J Hepatol"},{"key":"10070_CR13","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1097\/00005176-200307000-00003","volume":"37","author":"RJ Sokol","year":"2003","unstructured":"Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr 2003;37:4\u201321","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10070_CR14","doi-asserted-by":"crossref","first-page":"746","DOI":"10.5858\/arpa.2011-0623-RA","volume":"136","author":"RK Moreira","year":"2012","unstructured":"Moreira RK, Cabral R, Cowles RA, Lobritto SJ. Biliary atresia: a multidisciplinary approach to diagnosis and management. Arch Pathol Lab Med 2012;136:746\u2013760","journal-title":"Arch Pathol Lab Med"},{"key":"10070_CR15","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1002\/cld.592","volume":"8","author":"Y Li","year":"2016","unstructured":"Li Y, Bezerra JA. Novel approaches to the treatment of biliary atresia. Clin Liver Dis 2016;8:145\u2013149","journal-title":"Clin Liver Dis"},{"key":"10070_CR16","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1002\/lt.24640","volume":"23","author":"SS Sundaram","year":"2017","unstructured":"Sundaram SS, Mack CL, Feldman AG, Sokol RJ. Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl 2017;23:96\u2013109","journal-title":"Liver Transpl"},{"key":"10070_CR17","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1002\/hep.20547","volume":"41","author":"P Lykavieris","year":"2005","unstructured":"Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology 2005;41:366\u2013371","journal-title":"Hepatology"},{"key":"10070_CR18","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/S0140-6736(89)90012-3","volume":"1","author":"G Mieli-Vergani","year":"1989","unstructured":"Mieli-Vergani G, Howard ER, Portman B, Mowat AP. Late referral for biliary atresia\u2014missed opportunities for effective surgery. Lancet 1989;1:421\u2013423","journal-title":"Lancet"},{"key":"10070_CR19","doi-asserted-by":"crossref","first-page":"566","DOI":"10.1002\/hep.21790","volume":"46","author":"RJ Sokol","year":"2007","unstructured":"Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop. Hepatology 2007;46:566\u2013581","journal-title":"Hepatology"},{"key":"10070_CR20","doi-asserted-by":"crossref","first-page":"295891","DOI":"10.1155\/2015\/295891","volume":"2015","author":"A Bhalerao","year":"2015","unstructured":"Bhalerao A, Mannu GS. Management of pruritus in chronic liver disease. Dermatol Res Pract 2015;2015:295891","journal-title":"Dermatol Res Pract"},{"key":"10070_CR21","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/bs.apha.2015.04.003","volume":"74","author":"T Li","year":"2015","unstructured":"Li T, Apte U. Bile acid metabolism and signaling in cholestasis, inflammation, and cancer. Adv Pharmacol 2015;74:263\u2013302","journal-title":"Adv Pharmacol"},{"key":"10070_CR22","doi-asserted-by":"crossref","first-page":"2340","DOI":"10.1194\/jlr.R900012-JLR200","volume":"50","author":"PA Dawson","year":"2009","unstructured":"Dawson PA, Lan T, Rao A. Bile acid transporters. J Lipid Res 2009;50:2340\u20132357","journal-title":"J Lipid Res"},{"key":"10070_CR23","doi-asserted-by":"crossref","first-page":"1553","DOI":"10.1194\/jlr.R049437","volume":"55","author":"AF Hofmann","year":"2014","unstructured":"Hofmann AF, Hagey LR. Key discoveries in bile acid chemistry and biology and their clinical applications: history of the last eight decades. J Lipid Res 2014;55:1553\u20131595","journal-title":"J Lipid Res"},{"key":"10070_CR24","doi-asserted-by":"crossref","first-page":"2029","DOI":"10.12688\/f1000research.12449.1","volume":"6","author":"JY Chiang","year":"2017","unstructured":"Chiang JY. Recent advances in understanding bile acid homeostasis. F1000Research 2017;6:2029","journal-title":"F1000Research"},{"key":"10070_CR25","doi-asserted-by":"crossref","first-page":"1983","DOI":"10.1152\/physrev.00054.2017","volume":"98","author":"P Hegyi","year":"2018","unstructured":"Hegyi P, Maleth J, Walters JR, Hofmann AF, Keely SJ. Guts and gall: bile acids in regulation of intestinal epithelial function in health and disease. Physiol Rev 2018;98:1983\u20132023","journal-title":"Physiol Rev"},{"key":"10070_CR26","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.jhep.2017.02.023","volume":"67","author":"NW Orntoft","year":"2017","unstructured":"Orntoft NW, Munk OL, Frisch K, Ott P, Keiding S, Sorensen M. Hepatobiliary transport kinetics of the conjugated bile acid tracer (11)C-CSar quantified in healthy humans and patients by positron emission tomography. J Hepatol 2017;67:321\u2013327","journal-title":"J Hepatol"},{"key":"10070_CR27","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1002\/cphy.c120027","volume":"3","author":"JL Boyer","year":"2013","unstructured":"Boyer JL. Bile formation and secretion. Comp Physiol 2013;3:1035\u20131078","journal-title":"Comp Physiol"},{"key":"10070_CR28","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.phrs.2015.12.007","volume":"104","author":"BL Copple","year":"2016","unstructured":"Copple BL, Li T. Pharmacology of bile acid receptors: Evolution of bile acids from simple detergents to complex signaling molecules. Pharmacol Res 2016;104:9\u201321","journal-title":"Pharmacol Res"},{"key":"10070_CR29","doi-asserted-by":"crossref","first-page":"1340","DOI":"10.1016\/S0021-9258(17)42263-0","volume":"269","author":"MH Wong","year":"1994","unstructured":"Wong MH, Oelkers P, Craddock AL, Dawson PA. Expression cloning and characterization of the hamster ileal sodium-dependent bile acid transporter. J Biol Chem 1994;269:1340\u20131347","journal-title":"J Biol Chem"},{"key":"10070_CR30","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1194\/jlr.R054114","volume":"56","author":"PA Dawson","year":"2015","unstructured":"Dawson PA, Karpen SJ. Intestinal transport and metabolism of bile acids. J Lipid Res 2015;56:1085\u20131099","journal-title":"J Lipid Res"},{"key":"10070_CR31","doi-asserted-by":"crossref","first-page":"1393","DOI":"10.1002\/hep.28991","volume":"65","author":"M Trauner","year":"2017","unstructured":"Trauner M, Fuchs CD, Halilbasic E, Paumgartner G. New therapeutic concepts in bile acid transport and signaling for management of cholestasis. Hepatology 2017;65:1393\u20131404","journal-title":"Hepatology"},{"key":"10070_CR32","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.livres.2017.05.001","volume":"1","author":"JYL Chiang","year":"2017","unstructured":"Chiang JYL. Bile acid metabolism and signaling in liver disease and therapy. Liver Res 2017;1:3\u20139","journal-title":"Liver Res"},{"issue":"4 Pt B","key":"10070_CR33","doi-asserted-by":"crossref","first-page":"1319","DOI":"10.1016\/j.bbadis.2017.08.021","volume":"1864","author":"K Deutschmann","year":"2018","unstructured":"Deutschmann K, Reich M, Klindt C, Droge C, Spomer L, Haussinger D, et al. Bile acid receptors in the biliary tree: TGR5 in physiology and disease. Biochim Biophys Mol Basis Dis 2018;1864(4 Pt B):1319\u20131325","journal-title":"Biochim Biophys Mol Basis Dis."},{"key":"10070_CR34","doi-asserted-by":"crossref","first-page":"1417","DOI":"10.1053\/j.gastro.2014.08.042","volume":"147","author":"T Lieu","year":"2014","unstructured":"Lieu T, Jayaweera G, Zhao P, Poole DP, Jensen D, Grace M, et al. The bile acid receptor TGR5 activates the TRPA1 channel to induce itch in mice. Gastroenterology 2014;147:1417\u20131428","journal-title":"Gastroenterology"},{"key":"10070_CR35","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1016\/j.bbadis.2014.02.007","volume":"1842","author":"AE Kremer","year":"2014","unstructured":"Kremer AE, Feramisco J, Reeh PW, Beuers U, Oude Elferink RP. Receptors, cells and circuits involved in pruritus of systemic disorders. Biochim Biophys Acta 2014;1842:869\u2013892","journal-title":"Biochim Biophys Acta"},{"key":"10070_CR36","doi-asserted-by":"crossref","first-page":"2005","DOI":"10.1002\/hep.29076","volume":"65","author":"Y Wang","year":"2017","unstructured":"Wang Y, Aoki H, Yang J, Peng K, Liu R, Li X, et al. The role of sphingosine 1-phosphate receptor 2 in bile-acid-induced cholangiocyte proliferation and cholestasis-induced liver injury in mice. Hepatology 2017;65:2005\u20132018","journal-title":"Hepatology"},{"key":"10070_CR37","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1093\/toxsci\/kft221","volume":"137","author":"L Luo","year":"2014","unstructured":"Luo L, Schomaker S, Houle C, Aubrecht J, Colangelo JL. Evaluation of serum bile acid profiles as biomarkers of liver injury in rodents. Toxicol Sci 2014;137:12\u201325","journal-title":"Toxicol Sci"},{"key":"10070_CR38","doi-asserted-by":"crossref","first-page":"e0193824","DOI":"10.1371\/journal.pone.0193824","volume":"13","author":"L Luo","year":"2018","unstructured":"Luo L, Aubrecht J, Li D, Warner RL, Johnson KJ, Kenny J, et al. Assessment of serum bile acid profiles as biomarkers of liver injury and liver disease in humans. PLoS One 2018;13:e0193824","journal-title":"PLoS ONE"},{"key":"10070_CR39","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1620\/tjem.185.227","volume":"185","author":"D Abukawa","year":"1998","unstructured":"Abukawa D, Nakagawa M, Iinuma K, Nio M, Ohi R, Goto J. Hepatic and serum bile acid compositions in patients with biliary atresia: a microanalysis using gas chromatography-mass spectrometry with negative ion chemical ionization detection. Tohoku J Exp Med 1998;185:227\u2013237","journal-title":"Tohoku J Exp Med"},{"key":"10070_CR40","doi-asserted-by":"crossref","first-page":"e49270","DOI":"10.1371\/journal.pone.0049270","volume":"7","author":"K Zhou","year":"2012","unstructured":"Zhou K, Lin N, Xiao Y, Wang Y, Wen J, Zou GM, et al. Elevated bile acids in newborns with biliary atresia (BA). PLoS One 2012;7:e49270","journal-title":"PLoS ONE"},{"key":"10070_CR41","doi-asserted-by":"crossref","first-page":"4844","DOI":"10.1021\/acs.jproteome.5b00676","volume":"14","author":"K Zhou","year":"2015","unstructured":"Zhou K, Wang J, Xie G, Zhou Y, Yan W, Pan W, et al. Distinct plasma bile acid profiles of biliary atresia and neonatal hepatitis syndrome. J Proteome Res 2015;14:4844\u20134850","journal-title":"J Proteome Res"},{"issue":"1(suppl)","key":"10070_CR42","first-page":"85A","volume":"68","author":"S Harpavat","year":"2018","unstructured":"Harpavat S, Heubi JE, Karpen SJ, Ng VL, Setchell KDR, Shneider BL, et al. Prognostic value of serum bile acids after kasai portoenterostomy in biliary atresia. Hepatology 2018;68(1(suppl)):85A\u201386A (abstract 137)","journal-title":"Hepatology"},{"key":"10070_CR43","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/j.clinre.2017.08.007","volume":"42","author":"A Thebaut","year":"2018","unstructured":"Thebaut A, Debray D, Gonzales E. An update on the physiopathology and therapeutic management of cholestatic pruritus in children. Clin Res Hepatol Gastroenterol 2018;42:103\u2013109","journal-title":"Clin Res Hepatol Gastroenterol"},{"key":"10070_CR44","doi-asserted-by":"crossref","first-page":"11248","DOI":"10.1038\/ncomms11248","volume":"7","author":"WJ Keune","year":"2016","unstructured":"Keune WJ, Hausmann J, Bolier R, Tolenaars D, Kremer A, Heidebrecht T, et al. Steroid binding to Autotaxin links bile salts and lysophosphatidic acid signalling. Nat Commun 2016;7:11248","journal-title":"Nature Comm"},{"key":"10070_CR45","doi-asserted-by":"crossref","first-page":"89","DOI":"10.3109\/1354750X.2014.994564","volume":"20","author":"W Udomsinprasert","year":"2015","unstructured":"Udomsinprasert W, Honsawek S, Anomasiri W, Chongsrisawat V, Vejchapipat P, Poovorawan Y. Serum autotaxin levels correlate with hepatic dysfunction and severity in postoperative biliary atresia. Biomarkers 2015;20:89\u201394","journal-title":"Biomarkers"},{"issue":"suppl 1","key":"10070_CR46","first-page":"117A","volume":"68","author":"D Van Wessel","year":"2018","unstructured":"Van Wessel D, Thompson RJ, Grammatikopoulos T, Kadaristiana A, Jankowska I, Lipinski P, et al. The natural course of bsep deficiency: results from the global NAPPED-consortium. Hepatology 2018;68(suppl 1):117A\u2013118A (abstract 185)","journal-title":"Hepatology"},{"issue":"1 suppl","key":"10070_CR47","doi-asserted-by":"crossref","first-page":"e121","DOI":"10.1016\/S0618-8278(19)30213-0","volume":"70","author":"D van Wessel","year":"2019","unstructured":"van Wessel D, Thompson R, Grammatikopoulos T, Kadaristiana A, Jankowska I, Lipinski P, et al. Predicting long-term outcome after surgical biliary diversion in Bsep-deficiency patients: results from the NAPPED consortium. J Hepatol 2019;70(1 suppl):e121 (abstract PS-195)","journal-title":"J Hepatol"},{"issue":"suppl 1","key":"10070_CR48","first-page":"688","volume":"68","author":"D van Wessel","year":"2019","unstructured":"van Wessel D, Thompson R, Grammatikopoulos T, Kadaristiana A, Jankowska I, Lipinski P, et al. Factors associated with the natural course of disease in patients with FIC1-deficiency: the NAPPED-consortium. J Pediatr Gastroenterol Nutr 2019;68(suppl 1):688\u2013689 (abstract H-O-007)","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10070_CR49","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1002\/hep.28965","volume":"65","author":"PL Jansen","year":"2017","unstructured":"Jansen PL, Ghallab A, Vartak N, Reif R, Schaap FG, Hampe J, et al. The ascending pathophysiology of cholestatic liver disease. Hepatology 2017;65:722\u2013738","journal-title":"Hepatology"},{"key":"10070_CR50","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.ajpath.2010.11.026","volume":"178","author":"K Allen","year":"2011","unstructured":"Allen K, Jaeschke H, Copple BL. Bile acids induce inflammatory genes in hepatocytes: a novel mechanism of inflammation during obstructive cholestasis. Am J Pathol 2011;178:175\u2013186","journal-title":"Am J Pathol"},{"key":"10070_CR51","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1002\/hep.30213","volume":"69","author":"A Ghallab","year":"2019","unstructured":"Ghallab A, Hofmann U, Sezgin S, Vartak N, Hassan R, Zaza A, et al. Bile microinfarcts in cholestasis are initiated by rupture of the apical hepatocyte membrane and cause shunting of bile to sinusoidal blood. Hepatology 2019;69:666\u2013683","journal-title":"Hepatology"},{"key":"10070_CR52","doi-asserted-by":"crossref","first-page":"1156","DOI":"10.1111\/j.1582-4934.2010.01098.x","volume":"15","author":"L Muchova","year":"2011","unstructured":"Muchova L, Vanova K, Zelenka J, Lenicek M, Petr T, Vejrazka M, et al. Bile acids decrease intracellular bilirubin levels in the cholestatic liver: implications for bile acid-mediated oxidative stress. J Cell Mol Med 2011;15:1156\u20131165","journal-title":"J Cell Mol Med"},{"key":"10070_CR53","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/j.jhep.2017.04.026","volume":"67","author":"P Fickert","year":"2017","unstructured":"Fickert P, Wagner M. Biliary bile acids in hepatobiliary injury\u2014what is the link? J Hepatol 2017;67:619\u2013631","journal-title":"J Hepatol"},{"key":"10070_CR54","first-page":"e90780","volume":"2","author":"SY Cai","year":"2017","unstructured":"Cai SY, Ouyang X, Chen Y, Soroka CJ, Wang J, Mennone A, et al. Bile acids initiate cholestatic liver injury by triggering a hepatocyte-specific inflammatory response. JCI Insight 2017;2:e90780","journal-title":"JCI Insight"},{"key":"10070_CR55","doi-asserted-by":"crossref","first-page":"1869","DOI":"10.3390\/ijms20081869","volume":"20","author":"L Baiocchi","year":"2019","unstructured":"Baiocchi L, Zhou T, Liangpunsakul S, Lenci I, Santopaolo F, Meng F, et al. Dual role of bile acids on the biliary epithelium: friend or foe? Int J Mol Sci 2019;20:1869","journal-title":"Int J Mol Sci"},{"key":"10070_CR56","doi-asserted-by":"crossref","first-page":"2320","DOI":"10.1002\/hep.29585","volume":"67","author":"T Pradhan-Sundd","year":"2018","unstructured":"Pradhan-Sundd T, Zhou L, Vats R, Jiang A, Molina L, Singh S, et al. Dual catenin loss in murine liver causes tight junctional deregulation and progressive intrahepatic cholestasis. Hepatology 2018;67:2320\u20132337","journal-title":"Hepatology"},{"key":"10070_CR57","doi-asserted-by":"crossref","first-page":"69","DOI":"10.3727\/105221619X15469715711907","volume":"19","author":"T Pradhan-Sundd","year":"2019","unstructured":"Pradhan-Sundd T, Monga SP. Blood-bile barrier: morphology, regulation, and pathophysiology. Gene Expr 2019;19:69\u201387","journal-title":"Gene Expr"},{"key":"10070_CR58","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1038\/s41575-019-0125-y","volume":"16","author":"JM Banales","year":"2019","unstructured":"Banales JM, Huebert RC, Karlsen T, Strazzabosco M, LaRusso NF, Gores GJ. Cholangiocyte pathobiology. Nat Rev Gastroenterol Hepatol 2019;16:269\u2013281","journal-title":"Nat Rev Gastroenterol Hepatol"},{"key":"10070_CR59","doi-asserted-by":"crossref","first-page":"1283","DOI":"10.1007\/s00383-017-4158-4","volume":"33","author":"KKY Wong","year":"2017","unstructured":"Wong KKY, Wong CWY. A review of long-term outcome and quality of life of patients after Kasai operation surviving with native livers. Pediatr Surg Int 2017;33:1283\u20131287","journal-title":"Pediatr Surg Int"},{"key":"10070_CR60","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1038\/s41575-019-0132-z","volume":"16","author":"AG Feldman","year":"2019","unstructured":"Feldman AG, Sokol RJ. Neonatal cholestasis: emerging molecular diagnostics and potential novel therapeutics. Nat Rev Gastroenterol Hepatol 2019;16:346\u2013360","journal-title":"Nat Rev Gastroenterol Hepatol"},{"key":"10070_CR61","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.mam.2017.06.001","volume":"56","author":"M Li","year":"2017","unstructured":"Li M, Cai SY, Boyer JL. Mechanisms of bile acid mediated inflammation in the liver. Mol Aspects Med 2017;56:45\u201353","journal-title":"Mol Aspects Med"},{"issue":"4 Pt B","key":"10070_CR62","doi-asserted-by":"crossref","first-page":"1270","DOI":"10.1016\/j.bbadis.2017.07.024","volume":"1864","author":"C Pinto","year":"2018","unstructured":"Pinto C, Giordano DM, Maroni L, Marzioni M. Role of inflammation and proinflammatory cytokines in cholangiocyte pathophysiology. Biochim Biophys Mol Basis Dis 2018;1864(4 Pt B):1270\u20131278","journal-title":"Biochim Biophys Mol Basis Dis."},{"key":"10070_CR63","doi-asserted-by":"crossref","first-page":"705","DOI":"10.12688\/f1000research.8012.1","volume":"5","author":"M Wagner","year":"2016","unstructured":"Wagner M, Trauner M. Recent advances in understanding and managing cholestasis. F1000Research 2016;5:705","journal-title":"F1000Research"},{"key":"10070_CR64","doi-asserted-by":"crossref","first-page":"33920","DOI":"10.1074\/jbc.M306370200","volume":"278","author":"PA Dawson","year":"2003","unstructured":"Dawson PA, Haywood J, Craddock AL, Wilson M, Tietjen M, Kluckman K, et al. Targeted deletion of the ileal bile acid transporter eliminates enterohepatic cycling of bile acids in mice. J Biol Chem 2003;278:33920\u201333927","journal-title":"J Biol Chem"},{"key":"10070_CR65","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/j.jhep.2015.12.007","volume":"64","author":"R Poupon","year":"2016","unstructured":"Poupon R. ASBT inhibitors in cholangiopathies\u2014good for mice, good for men? J Hepatol 2016;64:537\u2013538","journal-title":"J Hepatol"},{"key":"10070_CR66","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1136\/gut.52.9.1239","volume":"52","author":"AF Hofmann","year":"2003","unstructured":"Hofmann AF. Inappropriate ileal conservation of bile acids in cholestatic liver disease: homeostasis gone awry. Gut 2003;52:1239\u20131241","journal-title":"Gut"},{"key":"10070_CR67","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1016\/j.jpedsurg.2011.08.010","volume":"47","author":"N Schukfeh","year":"2012","unstructured":"Schukfeh N, Metzelder ML, Petersen C, Reismann M, Pfister ED, Ure BM, et al. Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive familial intrahepatic cholestasis. J Pediatr Surg 2012;47:501\u2013505","journal-title":"J Pediatr Surg"},{"key":"10070_CR68","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1097\/MPG.0b013e31819a4e3d","volume":"49","author":"H Yang","year":"2009","unstructured":"Yang H, Porte RJ, Verkade HJ, De Langen ZJ, Hulscher JB. Partial external biliary diversion in children with progressive familial intrahepatic cholestasis and Alagille disease. J Pediatr Gastroenterol Nutr 2009;49:216\u2013221","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10070_CR69","doi-asserted-by":"crossref","first-page":"494","DOI":"10.1097\/MPG.0b013e3181df99d5","volume":"51","author":"H Arnell","year":"2010","unstructured":"Arnell H, Papadogiannakis N, Zemack H, Knisely AS, Nemeth A, Fischler B. Follow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversion. J Pediatr Gastroenterol Nutr 2010;51:494\u2013499","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10070_CR70","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1111\/apt.13457","volume":"43","author":"H Graffner","year":"2016","unstructured":"Graffner H, Gillberg PG, Rikner L, Marschall HU. The ileal bile acid transporter inhibitor A4250 decreases serum bile acids by interrupting the enterohepatic circulation. Aliment Pharmacol Ther 2016;43:303\u2013310","journal-title":"Aliment Pharmacol Ther"},{"key":"10070_CR71","doi-asserted-by":"crossref","first-page":"931","DOI":"10.3389\/fphar.2018.00931","volume":"9","author":"S Al-Dury","year":"2018","unstructured":"Al-Dury S, Marschall HU. Ileal bile acid transporter inhibition for the treatment of chronic constipation, cholestatic pruritus, and NASH. Front Pharmacol 2018;9:931","journal-title":"Front Pharmacol"},{"key":"10070_CR72","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1016\/j.jhep.2015.10.024","volume":"64","author":"A Baghdasaryan","year":"2016","unstructured":"Baghdasaryan A, Fuchs CD, Osterreicher CH, Lemberger UJ, Halilbasic E, Pahlman I, et al. Inhibition of intestinal bile acid absorption improves cholestatic liver and bile duct injury in a mouse model of sclerosing cholangitis. J Hepatol 2016;64:674\u2013681","journal-title":"J Hepatol"},{"key":"10070_CR73","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1002\/hep.27973","volume":"63","author":"AG Miethke","year":"2016","unstructured":"Miethke AG, Zhang W, Simmons J, Taylor AE, Shi T, Shanmukhappa SK, et al. Pharmacological inhibition of apical sodium-dependent bile acid transporter changes bile composition and blocks progression of sclerosing cholangitis in multidrug resistance 2 knockout mice. Hepatology 2016;63:512\u2013523","journal-title":"Hepatology"},{"key":"10070_CR74","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.1016\/S0140-6736(17)30319-7","volume":"389","author":"VS Hegade","year":"2017","unstructured":"Hegade VS, Kendrick SF, Dobbins RL, Miller SR, Thompson D, Richards D, et al. Effect of ileal bile acid transporter inhibitor GSK2330672 on pruritus in primary biliary cholangitis: a double-blind, randomised, placebo-controlled, crossover, phase 2a study. Lancet 2017;389:1114\u20131123","journal-title":"Lancet"},{"issue":"suppl 2","key":"10070_CR75","first-page":"S168","volume":"65","author":"E Sturm","year":"2017","unstructured":"Sturm E, Baumann U, Lacaille F, Gonzales E, Arnell H, Fischler B, et al. The ileal bile acid transport inhibitor A4250 reduced pruritus and serum bile acid levels in children with cholestatic liver disease and pruritus: final results from a multiple-dose, open-label, multinational study. J Pediatr Gastroenterol Nutr 2017;65(suppl 2):S168\u2013S169 (abstract 371)","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"suppl 1","key":"10070_CR76","first-page":"646A","volume":"66","author":"E Sturm","year":"2017","unstructured":"Sturm E, Baumann U, Lacaille F, Gonzales E, Arnell H, Fischler B, et al. The ileal bile acid transport inhibitor A4250 reduced pruritus and serum bile acid levels in children with cholestatic liver disease and pruritus: final results from a multiple-dose, open-label, multinational study. Hepatology 2017;66(suppl 1):646A\u2013647A (abstract 1200)","journal-title":"Hepatology"},{"issue":"suppl 1","key":"10070_CR77","doi-asserted-by":"crossref","first-page":"S632","DOI":"10.1016\/S0168-8278(18)31521-6","volume":"69","author":"E Gonzales","year":"2018","unstructured":"Gonzales E, Sturm E, Baumann U, Lacaille F, Arnell H, Fischler B, et al. Correlation of autotaxin levels, serum bile acids, and pruritus in a multiple-dose, open-label, multinational study of the ileal bile acid transport inhibitor A4250. J Hepatol 2018;69(suppl 1):S632 (abstract SAT-060)","journal-title":"J Hepatol"},{"issue":"suppl 1","key":"10070_CR78","doi-asserted-by":"crossref","first-page":"e120","DOI":"10.1016\/S0618-8278(19)30212-9","volume":"70","author":"U Baumann","year":"2019","unstructured":"Baumann U, Sturm E, Lacaille F, Gonzales E, Arnell H, Fischler B, et al. Effects of the ileal bile acid transport inhibitor A4250 on serum bile acids, pruritus and sleep in patients with Alagille syndrome: phase 2 study results. J Hepatol 2019;70(suppl 1):e120 (abstract PS-194)","journal-title":"J Hepatol"},{"issue":"suppl 1","key":"10070_CR79","doi-asserted-by":"crossref","first-page":"e411","DOI":"10.1016\/S0618-8278(19)30810-2","volume":"70","author":"U Baumann","year":"2019","unstructured":"Baumann U, Sturm E, Lacaille F, Gonzales E, Arnell H, Fischler B, et al. Effects of the ileal bile acid transport inhibitor A4250 on pruritus and serum bile acids in patients with biliary atresia: phase 2 study results. J Hepatol 2019;70(suppl 1):e411 (abstract FRI-060)","journal-title":"J Hepatol"},{"issue":"suppl 2","key":"10070_CR80","first-page":"S57","volume":"65","author":"B Kamath","year":"2017","unstructured":"Kamath B, Shneider B, Spino C, Magee J, Whitington P, Setchell K, et al. Unraveling the relationship between itching, scratch scales and biomarkers in children with Alagille syndrome. J Pediatr Gastroenterol Nutr 2017;65(suppl 2):S57\u2013S58 (abstract 143)","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"suppl 1","key":"10070_CR81","first-page":"653A","volume":"66","author":"BM Kamath","year":"2017","unstructured":"Kamath BM, Shneider BL, Spino C, Magee JC, Whitington PF, Setchell KD, et al. Unraveling the relationship between itching, scratch scales and biomarkers in children with Alagille syndrome. Hepatology 2017;66(suppl 1):653A\u2013654A (abstract 1210)","journal-title":"Hepatology"},{"key":"10070_CR82","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1002\/hep4.1244","volume":"2","author":"BL Shneider","year":"2018","unstructured":"Shneider BL, Spino C, Kamath BM, Magee JC, Bass LM, Setchell KD, et al. Placebo-controlled randomized trial of an intestinal bile salt transport inhibitor for pruritus in Alagille syndrome. Hepatol Commun 2018;2:1184\u20131198","journal-title":"Hepatol Commun"},{"issue":"suppl 1","key":"10070_CR83","doi-asserted-by":"crossref","first-page":"e119","DOI":"10.1016\/S0618-8278(19)30211-7","volume":"70","author":"E Gonzales","year":"2019","unstructured":"Gonzales E, Sturm E, Stormon M, Sokal E, Hardikar W, Lacaille F, et al. Phase 2 open-label study with a placebo-controlled drug withdrawal period of the apical sodium-dependent bile acid transporter inhibitor maralixibat in children with Alagille syndrome: 48-week interim efficacy analysis. J Hepatol 2019;70(suppl 1):e119\u2013e120 (abstract PS-193)","journal-title":"J Hepatol"},{"issue":"Suppl 1","key":"10070_CR84","first-page":"57A","volume":"66","author":"R Thompson","year":"2017","unstructured":"Thompson R, Kelly D, McClean P, Miethke A, Soufi N, Rivet C, et al. Phase 2 open-label efficacy and safety study of the apical sodium-dependent bile acid transporter inhibitor maralixibat in children with progressive familial intrahepatic cholestasis: 48 week interim efficacy analysis. Hepatology 2017;66(Suppl 1):57A","journal-title":"Hepatology"},{"issue":"suppl 1","key":"10070_CR85","doi-asserted-by":"crossref","first-page":"e131","DOI":"10.1016\/S0618-8278(19)30232-4","volume":"70","author":"R Thompson","year":"2019","unstructured":"Thompson R, Kelly D, Rajwal S, Miethke A, Soufi N, Rivet C, et al. Growth analysis in children with progressive familial intrahepatic cholestasis treated with the apical sodium-dependent bile acid transporter inhibitor maralixibat. J Hepatol 2019;70(suppl 1):e131\u2013e132 (abstract LBO-08)","journal-title":"J Hepatol"},{"key":"10070_CR86","doi-asserted-by":"crossref","unstructured":"Baumann U, Sturm E, Lacaille F, Gonzales E, Arnell H, Fischler B, et al. Effects of the ileal bile acid transport inhibitor A4250 on pruritus and serum bile acids in patients with biliary atresia: phase 2 study results [poster]. Annual Meeting of the European Association for the Study of the Liver; April 10\u201314, 2019; Vienna, Austria","DOI":"10.1016\/S0618-8278(19)30810-2"},{"key":"10070_CR87","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1002\/hep4.1332","volume":"3","author":"S Kim","year":"2019","unstructured":"Kim S, Moore J, Alonso E, Bednarek J, Bezerra JA, Goodhue C, et al. Correlation of immune markers with outcomes in biliary atresia following intravenous immunoglobulin therapy. Hepatol Commun 2019;3:685\u2013696","journal-title":"Hepatol Comm"},{"key":"10070_CR88","unstructured":"Albireo reports Q1 2020 financial results and provides business update [press release]. Boston, MA: Albireo; Available from: https:\/\/ir.albireopharma.com\/news-releases\/news-release-details\/albireo-reports-q1-2020-financial-results-and-provides-business. Accessed 7 May 2020"},{"key":"10070_CR89","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1038\/pr.2014.46","volume":"76","author":"EK Fenner","year":"2014","unstructured":"Fenner EK, Boguniewicz J, Tucker RM, Sokol RJ, Mack CL. High-dose IgG therapy mitigates bile duct-targeted inflammation and obstruction in a mouse model of biliary atresia. Pediatr Res 2014;76:72\u201380","journal-title":"Pediatr Res"},{"key":"10070_CR90","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1007\/s00383-018-4240-6","volume":"34","author":"D Li","year":"2018","unstructured":"Li D, Wang P, He Y, Jiao C, Zhuansun D, Wei N, et al. Intravenous immunoglobulin for the treatment of intractable cholangitis after Kasai portoenterostomy in biliary atresia patients. Pediatr Surg Int 2018;34:399\u2013404","journal-title":"Pediatr Surg Int"},{"key":"10070_CR91","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1097\/MPG.0000000000002256","volume":"68","author":"CL Mack","year":"2019","unstructured":"Mack CL, Spino C, Alonso EM, Bezerra JA, Moore J, Goodhue C, et al. A phase I\/IIa trial of intravenous immunoglobulin following portoenterostomy in biliary atresia. J Pediatr Gastroenterol Nutr 2019;68:495\u2013501","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10070_CR92","doi-asserted-by":"crossref","first-page":"1750","DOI":"10.1001\/jama.2014.2623","volume":"311","author":"JA Bezerra","year":"2014","unstructured":"Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA 2014;311:1750\u20131759","journal-title":"JAMA"},{"issue":"2 suppl","key":"10070_CR93","doi-asserted-by":"crossref","first-page":"S294","DOI":"10.1016\/S0168-8278(16)00371-8","volume":"64","author":"S Karpen","year":"2016","unstructured":"Karpen S, Eliot L, Scholz B, Joshi MM, Merrigan MM, Sciacca C, et al. A multicenter, randomized, open label, single- and multiple-dose, dose finding study and open-label extension to assess the effects of obeticholic acid in pediatric patients with biliary atresia. J Hepatol 2016;64(2 suppl):S294 (abstract THU-487)","journal-title":"J Hepatol"}],"container-title":["Hepatology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12072-020-10070-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12072-020-10070-w\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12072-020-10070-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,10]],"date-time":"2021-07-10T19:57:09Z","timestamp":1625947029000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12072-020-10070-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,7,11]]},"references-count":93,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2020,9]]}},"alternative-id":["10070"],"URL":"https:\/\/doi.org\/10.1007\/s12072-020-10070-w","relation":{},"ISSN":["1936-0533","1936-0541"],"issn-type":[{"value":"1936-0533","type":"print"},{"value":"1936-0541","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,7,11]]},"assertion":[{"value":"20 April 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 June 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 July 2020","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"Saul J. Karpen is a consultant for Albireo, Intercept, LogicBio, Mirum, and Spruce Biosciences. Deirdre Kelly is a consultant for Albireo, and her unit receives research funding for clinical trials from Albireo, Intercept, and Mirum. Cara Mack is a consultant for Albireo. Philip Stein is an employee of Albireo Pharma, Inc.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"This article does not contain any studies with human participants or animals performed by any of the authors.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}]}}