{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T18:09:13Z","timestamp":1792001353129,"version":"4.4.0"},"reference-count":42,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2021,7,13]],"date-time":"2021-07-13T00:00:00Z","timestamp":1626134400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Hepatology"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>Mutations in ATPase phospholipid transporting 8B1 (ATP8B1) can lead to familial intrahepatic cholestasis type 1 (FIC1) deficiency, or progressive familial intrahepatic cholestasis type 1. The rarity of FIC1 deficiency has largely prevented a detailed analysis of its natural history, effects of predicted protein truncating mutations (PPTMs), and possible associations of serum bile acid (sBA) concentrations and surgical biliary diversion (SBD) with long\u2010term outcome. We aimed to provide insights by using the largest genetically defined cohort of patients with FIC1 deficiency to date.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Approach and Results<\/jats:title>\n                    <jats:p>\n                      This multicenter, combined retrospective and prospective study included 130 patients with compound heterozygous or homozygous predicted pathogenic\n                      <jats:italic toggle=\"yes\">ATP8B1<\/jats:italic>\n                      variants. Patients were categorized according to the number of PPTMs (i.e., splice site, frameshift due to deletion or insertion, nonsense, duplication), FIC1\u2010A (n\u00a0=\u00a067; no PPTMs), FIC1\u2010B (n\u00a0=\u00a029; one PPTM), or FIC1\u2010C (n\u00a0=\u00a034; two PPTMs). Survival analysis showed an overall native liver survival (NLS) of 44% at age 18 years. NLS was comparable among FIC1\u2010A, FIC1\u2010B, and FIC1\u2010C (% NLS at age 10 years: 67%, 41%, and 59%, respectively;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0=\u00a00.12), despite FIC1\u2010C undergoing SBD less often (% SBD at age 10 years: 65%, 57%, and 45%, respectively;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0=\u00a00.03). sBAs at presentation were negatively associated with NLS (NLS at age 10 years, sBAs\u00a0&lt;\u00a0194\u00a0\u00b5mol\/L: 49% vs. sBAs\u00a0\u2265\u00a0194\u00a0\u00b5mol\/L: 15%;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0=\u00a00.03). SBD decreased sBAs (230 [125\u2010282] to 74 [11\u2010177] \u03bcmol\/L;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0=\u00a00.005). SBD (HR 0.55, 95% CI 0.28\u20101.03,\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0=\u00a00.06) and post\u2010SBD sBA concentrations\u00a0&lt;\u00a065 \u03bcmol\/L (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0=\u00a00.05) tended to be associated with improved NLS.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Less than half of patients with FIC1 deficiency reach adulthood with native liver. The number of PPTMs did not associate with the natural history or prognosis of FIC1 deficiency. sBA concentrations at initial presentation and after SBD provide limited prognostic information on long\u2010term NLS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hep.31787","type":"journal-article","created":{"date-parts":[[2021,3,5]],"date-time":"2021-03-05T05:38:50Z","timestamp":1614922730000},"page":"892-906","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":66,"title":["Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency"],"prefix":"10.1097","volume":"74","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0730-0201","authenticated-orcid":false,"given":"Daan B.E.","family":"van Wessel","sequence":"first","affiliation":[{"name":"Pediatric Gastroenterology and Hepatology,University Medical Center Groningen,University of Groningen,Groningen,the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J.","family":"Thompson","sequence":"additional","affiliation":[{"name":"Institute of Liver Studies,King\u2019s College Hospital,London,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1546-3193","authenticated-orcid":false,"given":"Emmanuel","family":"Gonzales","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irena","family":"Jankowska","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics,the Children\u2019s Memorial Health Institute,Warsaw,Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin L.","family":"Shneider","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition,Department of Pediatrics,Baylor College of Medicine,Houston,TX,USA"},{"name":"Childhood Liver Disease Research Network (ChiLDReN)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5597-4708","authenticated-orcid":false,"given":"Etienne","family":"Sokal","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Cliniques St. Luc,Universit\u00e9 Catholique de Louvain,Brussels,Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tassos","family":"Grammatikopoulos","sequence":"additional","affiliation":[{"name":"Institute of Liver Studies,King\u2019s College Hospital,London,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agustina","family":"Kadaristiana","sequence":"additional","affiliation":[{"name":"Institute of Liver Studies,King\u2019s College Hospital,London,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuel","family":"Jacquemin","sequence":"additional","affiliation":[{"name":"INSERM,UMR\u2010S 1193,Universit\u00e9 Paris\u2010Saclay,Orsay,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Spraul","sequence":"additional","affiliation":[{"name":"INSERM,UMR\u2010S 1193,Universit\u00e9 Paris\u2010Saclay,Orsay,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patryk","family":"Lipi\u0144ski","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics,the Children\u2019s Memorial Health Institute,Warsaw,Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piotr","family":"Czubkowski","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics,the Children\u2019s Memorial Health Institute,Warsaw,Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Rock","sequence":"additional","affiliation":[{"name":"Cliniques St. Luc,Universit\u00e9 Catholique de Louvain,Brussels,Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammad","family":"Shagrani","sequence":"additional","affiliation":[{"name":"Department of Liver & SB Transplant & Hepatobiliary\u2010Pancreatic Surgery,King Faisal Specialist Hospital & Research Center,Riyadh,Saudi Arabia"},{"name":"College of Medicine,Alfaisal University,Riyadh,Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dieter","family":"Broering","sequence":"additional","affiliation":[{"name":"Department of Liver & SB Transplant & Hepatobiliary\u2010Pancreatic Surgery,King Faisal Specialist Hospital & Research Center,Riyadh,Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Talal","family":"Algoufi","sequence":"additional","affiliation":[{"name":"Department of Liver & SB Transplant & Hepatobiliary\u2010Pancreatic Surgery,King Faisal Specialist Hospital & Research Center,Riyadh,Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nejat","family":"Mazhar","sequence":"additional","affiliation":[{"name":"Department of Liver & SB Transplant & Hepatobiliary\u2010Pancreatic Surgery,King Faisal Specialist Hospital & Research Center,Riyadh,Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Nicastro","sequence":"additional","affiliation":[{"name":"Pediatric Hepatology, Gastroenterology and Transplantation,Ospedale Papa Giovanni XXIII,Bergamo,Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deirdre","family":"Kelly","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Liver Unit,Birmingham Women\u2019s and Children\u2019s Hospital,University of Birmingham,Birmingham,United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriella","family":"Nebbia","sequence":"additional","affiliation":[{"name":"Servizio Di Epatologia e Nutrizione Pediatrica,Fondazione Irccs Ca\u2019 Granda Ospedale Maggiore Policlinico,Milano,Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrik","family":"Arnell","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Pediatric Digestive Diseases,Astrid Lindgren Children\u2019s Hospital,CLINTEC,Karolinska Institutet,Karolinska University Hospital,Stockholm,Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bj\u00f6rn","family":"Fischler","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Pediatric Digestive Diseases,Astrid Lindgren Children\u2019s Hospital,CLINTEC,Karolinska Institutet,Karolinska University Hospital,Stockholm,Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan B.F.","family":"Hulscher","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Pediatric Surgery,University Medical Center Groningen,Groningen,the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniele","family":"Serranti","sequence":"additional","affiliation":[{"name":"Pediatric and Liver Unit,Meyer Children\u2019s University Hospital of Florence,Florence,Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cigdem","family":"Arikan","sequence":"additional","affiliation":[{"name":"Pediatric GI and Hepatology Liver Transplantation Center,Kuttam System in Liver Medicine,Koc University School of Medicine,Istanbul,Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Debray","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Lacaille","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Goncalves","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Coimbra University Hospital Center,Coimbra,Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Loreto","family":"Hierro","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Pediatric Liver Service,La Paz University Hospital,Madrid,Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gema","family":"Mu\u00f1oz Bartolo","sequence":"additional","affiliation":[{"name":"European Reference Network on 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Ljubljana,Ljubljana,Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antal","family":"Dezso\u030bfi","sequence":"additional","affiliation":[{"name":"First Department of Pediatrics,Semmelweis University,Budapest,Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pier","family":"Luigi Calvo","sequence":"additional","affiliation":[{"name":"Pediatic Gastroenterology Unit,Regina Margherita Children\u2019s Hospital,Azienda Ospedaliera Citta\u0300 Della Salute e Della Scienza University Hospital,Torino,Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorothee","family":"Krebs\u2010Schmitt","sequence":"additional","affiliation":[{"name":"Klinik F\u00fcr Kinder\u2010Und Jugendmedizin,Universit\u00e4tsklinikum Hamburg Eppendorf,Hamburg,Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2608-5087","authenticated-orcid":false,"given":"Steffen","family":"Hartleif","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"University Children\u2019s Hospital T\u03c5\u0308bingen,T\u03c5\u0308bingen,Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy L.","family":"van der Woerd","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, Hepatology and Nutrition,Wilhelmina Children\u2019s Hospital,University Medical Center Utrecht,Utrecht,the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian\u2010She","family":"Wang","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital of Fudan University,Shanghai,China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li\u2010ting","family":"Li","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital of Fudan University,Shanghai,China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d6zlem","family":"Durmaz","sequence":"additional","affiliation":[{"name":"Istanbul Faculty of Medicine,Istanbul University,Istanbul,Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nanda","family":"Kerkar","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, Hepatology and Nutrition,University of Rochester Medical Center,Rochester,NY,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne","family":"H\u00f8rby J\u00f8rgensen","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Pediatric and Adolescent Department,Department of Pediatrics and Adolescent Medicine,Rigshospitalet Copenhagen University Hospital,Copenhagen,Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan","family":"Fischer","sequence":"additional","affiliation":[{"name":"Section of Hepatology and Transplant Medicine,Children\u2019s Mercy Hospital,Kansas City,MO,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Jimenez\u2010Rivera","sequence":"additional","affiliation":[{"name":"Department of Pediatrics,Children\u2019s Hospital of Eastern Ontario,University of Ottawa,Ottawa,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seema","family":"Alam","sequence":"additional","affiliation":[{"name":"Pediatric Hepatology,Institute of Liver and Biliary Sciences,New Delhi,India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mara","family":"Cananzi","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Pediatric Gastroenterology and Hepatology,University Hospital of Padova,Padova,Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"No\u00e9mie","family":"Laverdure","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Service de Gastroent\u00e9rologie, H\u00e9patologie et Nutrition P\u00e9diatriques,Hospices Civils de Lyon,H\u00f4pital Femme M\u00e8re Enfant,Lyon,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Targa Ferreira","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology,Hospital da Crian\u00e7a Santo Ant\u00f4nio,Porto Allegre,Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felipe","family":"Ordonez","sequence":"additional","affiliation":[{"name":"Fundaci\u00f3n Cardioinfantil Instituto de Cardiologia,Pediatric Gastroenterology and Hepatology,Bogot\u00e1,Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heng","family":"Wang","sequence":"additional","affiliation":[{"name":"DDC Clinic Center for Special Needs Children,Middlefield,OH,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valerie","family":"Sency","sequence":"additional","affiliation":[{"name":"DDC Clinic Center for Special Needs Children,Middlefield,OH,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyung","family":"Mo Kim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics,Asan Medical Center Children\u2019s Hospital,Seoul,South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4074-5838","authenticated-orcid":false,"given":"Huey\u2010Ling","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition,National Taiwan University Children\u2019s Hospital,Taipei,Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Hepatology,Bras\u00edlia Children\u2019s Hospital,Brasilia,Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Fabre","sequence":"additional","affiliation":[{"name":"INSERM,MMG,Aix Marseille University,Marseille,France"},{"name":"Serveice de P\u00e9diatrie Multidisciplinaire,Timone Enfant,Marseille,France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jesus","family":"Quintero Bernabeu","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases"},{"name":"Pediatric Hepatology and Liver Transplant Unit,Barcelona,Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estella M.","family":"Alonso","sequence":"additional","affiliation":[{"name":"Childhood Liver Disease Research Network (ChiLDReN)"},{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition,Ann & Robert H. Lurie Children\u2019s Hospital,Chicago,IL,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald J.","family":"Sokol","sequence":"additional","affiliation":[{"name":"Childhood Liver Disease Research Network (ChiLDReN)"},{"name":"Section of Pediatric Gastroenterology, Hepatology and Nutrition,Department of Pediatrics,Children\u2019s Hospital Colorado,University of Colorado School of Medicine,Aurora,CO,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederick J.","family":"Suchy","sequence":"additional","affiliation":[{"name":"Childhood Liver Disease Research Network (ChiLDReN)"},{"name":"Icahn School of Medicine at Mount Sinai,Mount Sinai Kravis Children\u2019s Hospital,New York,NY,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen M.","family":"Loomes","sequence":"additional","affiliation":[{"name":"Childhood Liver Disease Research Network (ChiLDReN)"},{"name":"Division of Gastroenterology, Hepatology and Nutrition,Children\u2019s Hospital of Philadelphia,Philadelphia,PA,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick J.","family":"McKiernan","sequence":"additional","affiliation":[{"name":"Childhood Liver Disease Research Network (ChiLDReN)"},{"name":"Department of Pediatric Gastroenterology and Hepatology,University of Pittsburgh Medical Center Children\u2019s Hospital of Pittsburgh,Pittsburgh,PA,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Rosenthal","sequence":"additional","affiliation":[{"name":"Childhood Liver Disease Research Network (ChiLDReN)"},{"name":"Department of Pediatrics and Surgery,UCSF Benioff Children\u2019s Hospital,University of California San Francisco School of Medicine,San Francisco,CA,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yumirle","family":"Turmelle","sequence":"additional","affiliation":[{"name":"Childhood Liver Disease Research Network 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Toronto,Toronto,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Rogalidou","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology & Hepatology,First Pediatrics Department,University of Athens,Agia Sofia Children\u2019s Hospital,Athens,Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wikrom W.","family":"Karnsakul","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Nutrition, and Hepatology,Department of Pediatrics,Johns Hopkins University School of Medicine,Baltimore,MD,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bettina","family":"Hansen","sequence":"additional","affiliation":[{"name":"Toronto Center for Liver Disease,University Health Network,Toronto,Canada"},{"name":"IHPME,University of Toronto,Toronto,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henkjan J.","family":"Verkade","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Hepatology,University Medical Center Groningen,University of Groningen,Groningen,the Netherlands"},{"name":"European Reference Network on Hepatological Diseases"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"on behalf of Natural Course and Prognosis of PFIC and Effect of Biliary Diversion Consortium","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2021,7,13]]},"reference":[{"key":"hep31787-bib-0001-20250824","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1016\/S0925-4439(01)00103-X","article-title":"Genetic defects in hepatobiliary transport","volume":"1586","year":"2002","journal-title":"Biochim Biophys Acta"},{"key":"hep31787-bib-0002-20250824","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1016\/j.cld.2018.06.003","article-title":"Progressive 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