{"id":"https://openalex.org/W4229870262","doi":"https://doi.org/10.1016/s0168-8278(20)30757-1","title":"Serum bile acid control in long-term maralixibat-treated patients is associated with native liver survival in children with progressive familial intrahepatic cholestasis due to bile salt export pump deficiency","display_name":"Serum bile acid control in long-term maralixibat-treated patients is associated with native liver survival in children with progressive familial intrahepatic cholestasis due to bile salt export pump deficiency","publication_year":2020,"publication_date":"2020-08-01","ids":{"openalex":"https://openalex.org/W4229870262","doi":"https://doi.org/10.1016/s0168-8278(20)30757-1"},"language":"en","primary_location":{"id":"doi:10.1016/s0168-8278(20)30757-1","doi":"https://doi.org/10.1016/s0168-8278(20)30757-1","is_oa":false,"landing_page_url":"https://doi.org/10.1016/s0168-8278(20)30757-1","pdf_url":null,"source":{"id":"https://openalex.org/S83651209","display_name":"Journal of Hepatology","issn_l":"0168-8278","issn":["0168-8278","1600-0641"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Hepatology","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5002550781","display_name":"Richard J. Thompson","orcid":"https://orcid.org/0000-0001-5652-0150","observed_orcids":["https://orcid.org/0000-0001-5652-0150"]},"institutions":[{"id":"https://openalex.org/I183935753","display_name":"King's College London","ror":"https://ror.org/0220mzb33","country_code":"GB","type":"education","lineage":["https://openalex.org/I124357947","https://openalex.org/I183935753"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Richard Thompson","raw_affiliation_strings":["King's College London ,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"King's College London ,","institution_ids":["https://openalex.org/I183935753"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5006405927","display_name":"Deirdre Anne Kelly","orcid":"https://orcid.org/0000-0001-9776-4354","observed_orcids":["https://orcid.org/0000-0001-9776-4354","https://orcid.org/0009-0007-8098-7966"]},"institutions":[{"id":"https://openalex.org/I4210099987","display_name":"Birmingham Women's Hospital","ror":"https://ror.org/00xe5zs60","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I4210099987","https://openalex.org/I4210155225"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Deirdre Kelly","raw_affiliation_strings":["Birmingham Women's and Children's Hospital , Liver Unit , Birmingham , United Kingdom;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Birmingham Women's and Children's Hospital , Liver Unit , Birmingham , United Kingdom;","institution_ids":["https://openalex.org/I4210099987"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5017669989","display_name":"Alexander Gerhard Miethke","orcid":"https://orcid.org/0000-0003-1395-9475","observed_orcids":["https://orcid.org/0000-0003-1395-9475"]},"institutions":[{"id":"https://openalex.org/I1285204247","display_name":"Cincinnati Children's Hospital Medical Center","ror":"https://ror.org/01hcyya48","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1285204247"]},{"id":"https://openalex.org/I63135867","display_name":"University of Cincinnati","ror":"https://ror.org/01e3m7079","country_code":"US","type":"education","lineage":["https://openalex.org/I63135867"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Alexander Miethke","raw_affiliation_strings":["Cincinnati Children's Hospital Medical Center , Division of Gastroenterology, Hepatology and Nutrition , Cincinnati , United States;","University of Cincinnati College of Medicine , Department of Pediatrics , Cincinnati , United States;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Cincinnati Children's Hospital Medical Center , Division of Gastroenterology, Hepatology and Nutrition , Cincinnati , United States;","institution_ids":["https://openalex.org/I1285204247"]},{"raw_affiliation_string":"University of Cincinnati College of Medicine , Department of Pediatrics , Cincinnati , United States;","institution_ids":["https://openalex.org/I63135867"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5050509894","display_name":"Sanjay R. Rajwal","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2799390153","display_name":"Leeds Teaching Hospitals NHS Trust","ror":"https://ror.org/00v4dac24","country_code":"GB","type":"healthcare","lineage":["https://openalex.org/I2799390153"]}],"countries":["GB"],"is_corresponding":false,"raw_author_name":"Sanjay Rajwal","raw_affiliation_strings":["NHS Trust , Leeds Teaching Hospitals , Leeds , United Kingdom;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"NHS Trust , Leeds Teaching Hospitals , Leeds , United Kingdom;","institution_ids":["https://openalex.org/I2799390153"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5070423382","display_name":"Nisreen Soufi","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1174212","display_name":"University of Southern California","ror":"https://ror.org/03taz7m60","country_code":"US","type":"education","lineage":["https://openalex.org/I1174212"]},{"id":"https://openalex.org/I1336910626","display_name":"Children's Hospital Los Angeles","ror":"https://ror.org/00412ts95","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1336910626"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Nisreen Soufi","raw_affiliation_strings":["Children's Hospital Los Angeles , Division of Gastroenterology,","Keck School of Medicine , University of Southern California , Department of Pediatrics , Los Angeles , United States;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Children's Hospital Los Angeles , Division of Gastroenterology,","institution_ids":["https://openalex.org/I1336910626"]},{"raw_affiliation_string":"Keck School of Medicine , University of Southern California , Department of Pediatrics , Los Angeles , United States;","institution_ids":["https://openalex.org/I1174212"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5075399641","display_name":"Irena Jankowska","orcid":"https://orcid.org/0000-0001-6847-9570","observed_orcids":["https://orcid.org/0000-0001-6847-9570"]},"institutions":[{"id":"https://openalex.org/I4210114383","display_name":"Children's Memorial Health Institute","ror":"https://ror.org/020atbp69","country_code":"PL","type":"healthcare","lineage":["https://openalex.org/I4210114383"]}],"countries":["PL"],"is_corresponding":false,"raw_author_name":"Irena Jankowska","raw_affiliation_strings":["Children's Memorial Health Institute , Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics , Warsaw , Poland;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Children's Memorial Health Institute , Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics , Warsaw , Poland;","institution_ids":["https://openalex.org/I4210114383"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5027984281","display_name":"Cara L. Mack","orcid":"https://orcid.org/0000-0002-5760-3836","observed_orcids":["https://orcid.org/0000-0002-5760-3836"]},"institutions":[{"id":"https://openalex.org/I2802236040","display_name":"University of Colorado System","ror":"https://ror.org/00jc20583","country_code":"US","type":"education","lineage":["https://openalex.org/I2802236040"]},{"id":"https://openalex.org/I4210096275","display_name":"Children's Hospital Colorado","ror":"https://ror.org/00mj9k629","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210096275"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Cara Mack","raw_affiliation_strings":["Children's Hospital Colorado , University of Colorado"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Children's Hospital Colorado , University of Colorado","institution_ids":["https://openalex.org/I2802236040","https://openalex.org/I4210096275"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5081268394","display_name":"Alain Lachaux","orcid":"https://orcid.org/0000-0003-3852-788X","observed_orcids":["https://orcid.org/0000-0003-3852-788X"]},"institutions":[{"id":"https://openalex.org/I4210085940","display_name":"H\u00f4pital Femme M\u00e8re Enfant","ror":"https://ror.org/006yspz11","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I4210085940","https://openalex.org/I4210100596"]},{"id":"https://openalex.org/I4210100596","display_name":"Hospices Civils de Lyon","ror":"https://ror.org/01502ca60","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I4210100596"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Alain Lachaux","raw_affiliation_strings":["H\u00f4pital Femme Mere Enfant , Hospices Civils de Lyon , H\u00e9patologie ,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"H\u00f4pital Femme Mere Enfant , Hospices Civils de Lyon , H\u00e9patologie ,","institution_ids":["https://openalex.org/I4210085940","https://openalex.org/I4210100596"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5012240572","display_name":"Thomas Jaecklin","orcid":"https://orcid.org/0000-0002-8704-2419","observed_orcids":["https://orcid.org/0000-0002-8704-2419"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Thomas Jaecklin","raw_affiliation_strings":["Mirum Pharmaceuticals , Basel , Switzerland;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Mirum Pharmaceuticals , Basel , Switzerland;","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5014809151","display_name":"Pamela Vig","orcid":"https://orcid.org/0009-0000-4226-5435","observed_orcids":["https://orcid.org/0009-0000-4226-5435"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Pamela Vig","raw_affiliation_strings":["Hepatology and Nutrition , Los Angeles , United States;","Mirum Pharmaceuticals , Foster City , United States;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Hepatology and Nutrition , Los Angeles , United States;","institution_ids":[]},{"raw_affiliation_string":"Mirum Pharmaceuticals , Foster City , United States;","institution_ids":[]}]},{"author_position":"middle","author":{"id":null,"display_name":"Andrew Wardle","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Andrew Wardle","raw_affiliation_strings":["Mirum Pharmaceuticals , Foster City , United States;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Mirum Pharmaceuticals , Foster City , United States;","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5107881446","display_name":"Robert H. Squires","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I170201317","display_name":"University of Pittsburgh","ror":"https://ror.org/01an3r305","country_code":"US","type":"education","lineage":["https://openalex.org/I170201317"]},{"id":"https://openalex.org/I4210130600","display_name":"Children's Hospital of Pittsburgh","ror":"https://ror.org/03763ep67","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210098814","https://openalex.org/I4210130600","https://openalex.org/I4210134769"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Robert Squires","raw_affiliation_strings":["Children's Hospital of Pittsburgh , University of Pittsburgh","University of Pittsburgh School of Medicine , Department of Pediatrics , Pittsburgh , United States;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Children's Hospital of Pittsburgh , University of Pittsburgh","institution_ids":["https://openalex.org/I170201317","https://openalex.org/I4210130600"]},{"raw_affiliation_string":"University of Pittsburgh School of Medicine , Department of Pediatrics , Pittsburgh , United States;","institution_ids":["https://openalex.org/I170201317"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5063054175","display_name":"Kathleen M. Loomes","orcid":"https://orcid.org/0000-0002-1539-6672","observed_orcids":["https://orcid.org/0000-0002-1539-6672"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Kathleen Loomes","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":4,"institutions_distinct_count":14,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":{"value":3000,"currency":"USD","value_usd":3000},"apc_paid":null,"fwci":0.6721,"has_fulltext":true,"cited_by_count":5,"citation_normalized_percentile":{"value":0.70965225,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":95},"biblio":{"volume":"73","issue":null,"first_page":"S120","last_page":"S120"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T12460","display_name":"Pediatric Hepatobiliary Diseases and Treatments","score":0.7304999828338623,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T12460","display_name":"Pediatric Hepatobiliary Diseases and Treatments","score":0.7304999828338623,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10570","display_name":"Drug Transport and Resistance Mechanisms","score":0.25780001282691956,"subfield":{"id":"https://openalex.org/subfields/2730","display_name":"Oncology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10999","display_name":"Organ Transplantation Techniques and Outcomes","score":0.0010999999940395355,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/maralixibat","display_name":"maralixibat","score":0.9890000224113464},{"id":"https://openalex.org/keywords/serum-bile-acids","display_name":"serum bile acids","score":0.8019999861717224},{"id":"https://openalex.org/keywords/progressive-familial-intrahepatic-cholestasis","display_name":"progressive familial intrahepatic cholestasis","score":0.9330000281333923},{"id":"https://openalex.org/keywords/native-liver-survival","display_name":"native liver survival","score":0.8370000123977661},{"id":"https://openalex.org/keywords/children","display_name":"children","score":0.6620000004768372},{"id":"https://openalex.org/keywords/long-term-treatment","display_name":"long-term treatment","score":0.597000002861023},{"id":"https://openalex.org/keywords/cholestatic-liver-disease","display_name":"cholestatic liver disease","score":0.640999972820282}],"concepts":[{"id":"https://openalex.org/C2910800957","wikidata":"https://www.wikidata.org/wiki/Q15315873","display_name":"Bile Salt Export Pump","level":4,"score":0.867922306060791},{"id":"https://openalex.org/C2778593092","wikidata":"https://www.wikidata.org/wiki/Q1075923","display_name":"Cholestasis","level":2,"score":0.7744479179382324},{"id":"https://openalex.org/C2778132995","wikidata":"https://www.wikidata.org/wiki/Q1018534","display_name":"Progressive familial intrahepatic cholestasis","level":4,"score":0.7399550676345825},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.7073624134063721},{"id":"https://openalex.org/C2779399885","wikidata":"https://www.wikidata.org/wiki/Q413288","display_name":"Bile acid","level":2,"score":0.6921400427818298},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.6261324286460876},{"id":"https://openalex.org/C2776371256","wikidata":"https://www.wikidata.org/wiki/Q12370","display_name":"Salt (chemistry)","level":2,"score":0.5431801676750183},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.49787425994873047},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.2648809552192688},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.18265920877456665},{"id":"https://openalex.org/C2779609443","wikidata":"https://www.wikidata.org/wiki/Q1368191","display_name":"Liver transplantation","level":3,"score":0.11530545353889465},{"id":"https://openalex.org/C149011108","wikidata":"https://www.wikidata.org/wiki/Q652985","display_name":"Transporter","level":3,"score":0.0},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.0},{"id":"https://openalex.org/C147789679","wikidata":"https://www.wikidata.org/wiki/Q11372","display_name":"Physical chemistry","level":1,"score":0.0},{"id":"https://openalex.org/C2911091166","wikidata":"https://www.wikidata.org/wiki/Q106419912","display_name":"Transplantation","level":2,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1016/s0168-8278(20)30757-1","doi":"https://doi.org/10.1016/s0168-8278(20)30757-1","is_oa":false,"landing_page_url":"https://doi.org/10.1016/s0168-8278(20)30757-1","pdf_url":null,"source":{"id":"https://openalex.org/S83651209","display_name":"Journal of Hepatology","issn_l":"0168-8278","issn":["0168-8278","1600-0641"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Hepatology","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"score":1.0,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W4399560574","https://openalex.org/W2939293921","https://openalex.org/W4384947149","https://openalex.org/W7002088585","https://openalex.org/W2938746505","https://openalex.org/W7030277933","https://openalex.org/W4414218197","https://openalex.org/W4229048487","https://openalex.org/W4414218107","https://openalex.org/W4384941910"],"abstract_inverted_index":null,"counts_by_year":[{"year":2026,"cited_by_count":1},{"year":2025,"cited_by_count":1},{"year":2021,"cited_by_count":2},{"year":2020,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2022-05-11T00:00:00"}