{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T18:09:11Z","timestamp":1792001351590,"version":"4.4.0"},"reference-count":29,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,10,5]],"date-time":"2025-10-05T00:00:00Z","timestamp":1759622400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Patients with progressive familial intrahepatic cholestasis type 1 (PFIC1) who have undergone liver transplantation (LT) may have unmet needs and impacts on daily life due to post\u2010LT complications, including diarrhea and hepatic steatosis. Here, we describe the effects of the ileal bile acid transporter inhibitor odevixibat on diarrhea and hepatic steatosis in a cohort of patients with PFIC1 post\u2010LT.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Treating physicians from six centers retrospectively collected data through July 2023 on patients with PFIC1 who received odevixibat post\u2010LT. Data collected included demographics, medical history, and symptom presentation, characteristics of diarrhea, and liver imaging and\/or histopathology.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Overall, nine male patients with PFIC1 (seven aged &lt;18 years at initial completion of the case report form) were included. In most patients, the primary indication for odevixibat treatment was diarrhea and\/or steatosis post\u2010LT. Odevixibat was initiated at a daily dose of 30\u2212120\u2009\u00b5g\/kg (median exposure: 13 months). All patients had post\u2010LT diarrhea, which was generally associated with negative impacts on daily life (e.g., ability to attend school, needing to wear diapers due to fecal urgency). After odevixibat initiation, most patients had improved diarrhea and positive impacts on daily life. Among five patients with post\u2010LT steatosis and data available before and after odevixibat initiation, steatosis appeared to improve in three and did not change in two.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Overall, the majority of patients with PFIC1 post\u2010LT complications in this case series experienced improvements in diarrhea and daily activities with odevixibat. Treatment with odevixibat following LT also appeared to reduce steatosis in some patients. Further studies, particularly those with a prospective design, are needed to confirm these findings.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpn3.70227","type":"journal-article","created":{"date-parts":[[2025,10,5]],"date-time":"2025-10-05T08:00:31Z","timestamp":1759651231000},"page":"1410-1421","source":"Crossref","is-referenced-by-count":4,"title":["Odevixibat after liver transplant in patients with progressive familial intrahepatic cholestasis type 1: A case series"],"prefix":"10.1002","volume":"81","author":[{"given":"Georg\u2010Friedrich","family":"Vogel","sequence":"first","affiliation":[{"name":"Department of Paediatrics I Medical University of Innsbruck Innsbruck Austria"},{"name":"Institute of Cell Biology Medical University of Innsbruck Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Kathemann","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Transplant Medicine, University Children's Hospital Essen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Pietrobattista","sequence":"additional","affiliation":[{"name":"Hepatology and Liver Transplantation Unit, Bambino Ges\u00f9 Children's Hospital IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Maggiore","sequence":"additional","affiliation":[{"name":"Hepatology and Liver Transplantation Unit, Bambino Ges\u00f9 Children's Hospital IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denise","family":"Aldrian","sequence":"additional","affiliation":[{"name":"Department of Paediatrics I Medical University of Innsbruck Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Sciveres","sequence":"additional","affiliation":[{"name":"Hepatology and Liver Transplantation Unit, Bambino Ges\u00f9 Children's Hospital IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henkjan J.","family":"Verkade","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology\/Hepatology, Department of Pediatrics University of Groningen, Beatrix Children's Hospital\/University Medical Center Groningen Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Etienne","family":"Sokal","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Catholique de Louvain, Cliniques St Luc Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Jannone","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Catholique de Louvain, Cliniques St Luc Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdalena","family":"Salcedo","sequence":"additional","affiliation":[{"name":"Hepatology and Liver Transplantation Unit, CIBER\u2010Ehd Hospital General Universitario Gregorio Mara\u00f1\u00f3n Universidad Complutense Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Rauschkolb","sequence":"additional","affiliation":[{"name":"Ipsen Pharma GmBH Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christof","family":"Maucksch","sequence":"additional","affiliation":[{"name":"Ipsen Pharma GmBH Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Velichka","family":"Valcheva","sequence":"additional","affiliation":[{"name":"Ipsen Ireland Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7624-2968","authenticated-orcid":false,"given":"Elke","family":"Lainka","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Transplant Medicine, University Children's Hospital Essen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,10,5]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cld.2018.06.003"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1203\/01.PDR.0000145564.06791.D1"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2001.27663"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jceh.2013.10.005"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.2147\/HMER.S137209"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1053\/jlts.2002.34384"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00268-010-0869-6"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.21686"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep4.1168"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.14108"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.3390\/children9050669"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002983"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jceh.2021.03.008"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.14600"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.14701\/ahbps.21-114"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2011.01514.x"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2007.00722.x"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0168-8278(03)00286-1"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002990"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.3390\/children9121964"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/HC9.0000000000000286"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.13184"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.12659\/AJCR.932374"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12072-020-10070-w"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000001493"},{"key":"e_1_2_8_27_1","unstructured":"Bylvay [Package Insert]. Ipsen Biopharmaceuticals Inc.;2025."},{"key":"e_1_2_8_28_1","unstructured":"Bylvay [Summary of Product Characteristics]. Ipsen Pharma;2024."},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.4103\/1319-3767.80387"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.3390\/medicina57080854"}],"container-title":["Journal of Pediatric Gastroenterology and Nutrition"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpn3.70227","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,1]],"date-time":"2025-12-01T17:44:37Z","timestamp":1764611077000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jpn3.70227"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,5]]},"references-count":29,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2025,12]]}},"alternative-id":["10.1002\/jpn3.70227"],"URL":"https:\/\/doi.org\/10.1002\/jpn3.70227","archive":["Portico"],"relation":{},"ISSN":["0277-2116","1536-4801"],"issn-type":[{"value":"0277-2116","type":"print"},{"value":"1536-4801","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,5]]}}}