{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T12:38:06Z","timestamp":1789562286604,"version":"build-2803163510"},"reference-count":28,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T00:00:00Z","timestamp":1751846400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background &amp; Aims<\/jats:title>\n                    <jats:p>\n                      In episodic intrahepatic cholestasis (IC; known historically as benign recurrent intrahepatic cholestasis), intermittent cholestasis is typically followed by periods of remission. During cholestatic episodes, symptoms can include jaundice, fatigue, abdominal pain, diarrhoea, and severe pruritus that can interfere with patients' lives. Here, we report clinical features and response to odevixibat, an ileal bile acid transporter inhibitor, in patients with episodic IC associated with biallelic mutations in\n                      <jats:italic>ATP8B1<\/jats:italic>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Clinical information before and after odevixibat initiation was collected.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Six patients diagnosed with episodic IC were included. During cholestatic episodes before odevixibat, all patients had high serum bile acids and severe pruritus associated with sleep and\/or mood disturbances that affected patients' quality of life. Physicians initiated odevixibat (dose range: 28\u2013120\u2009\u03bcg\/kg\/day) either during a cholestatic relapse (5\/6 patients) or prophylactically (1 patient), resulting in marked symptom improvement in 4\/6 (67%) patients and partial improvement in 2\/6 (33%); rapid reductions in serum bile acids were reported in 3\/6 patients (50%). All patients had improved pruritus and resumed daily activities. Subsequently, patients either stopped treatment after the disappearance of cholestasis or continued treatment. Patients had further cholestatic episodes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      In this case series, most patients with episodic IC associated with biallelic mutations in\n                      <jats:italic>ATP8B1<\/jats:italic>\n                      treated with odevixibat during a relapse of cholestasis had clinical improvement, including reductions in serum bile acids and positive impacts on pruritus and quality of life. Treatment did not appear to have a preventive effect on future episodes. More studies are needed to confirm these findings.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/liv.70216","type":"journal-article","created":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T09:13:35Z","timestamp":1751879615000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Odevixibat for Episodic Intrahepatic Cholestasis due to Biallelic Mutations in\n                    <scp>\n                      <i>ATP8B1<\/i>\n                    <\/scp>\n                    : A Case Series"],"prefix":"10.1111","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0363-5565","authenticated-orcid":false,"given":"Angelo","family":"Di Giorgio","sequence":"first","affiliation":[{"name":"Paediatric Hepatology, Gastroenterology, and Transplantation ASST Hospital Papa Giovanni XXIII  Bergamo Italy"},{"name":"Department of Medicine University of Udine  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans","family":"Blokzijl","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology University of Groningen, University Medical Center Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuel","family":"Gonzal\u00e8s","sequence":"additional","affiliation":[{"name":"H\u00e9patologie et Transplantation H\u00e9patique P\u00e9diatriques, Centre de R\u00e9f\u00e9rence de L'atr\u00e9sie Des Voies Biliaires et Des Cholestases G\u00e9n\u00e9tiques, FSMR FILFOIE, ERN RARE LIVER H\u00f4pital Bic\u00eatre, AP\u2010HP, Universit\u00e9 Paris\u2010Saclay, H\u00e9patinov, Inserm U 1193  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdalena","family":"Salcedo","sequence":"additional","affiliation":[{"name":"Hepatology and Liver Transplantation Unit CIBEREhd Hospital General Universitario Gregorio Mara\u00f1\u00f3n, Universidad Complutense  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy L.","family":"van der Woerd","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Wilhelmina Children's Hospital, University Medical Center Utrecht  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Clemson","sequence":"additional","affiliation":[{"name":"Ipsen  Cambridge Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lionel","family":"Thevathasan","sequence":"additional","affiliation":[{"name":"Ipsen  Cambridge Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Velichka","family":"Valcheva","sequence":"additional","affiliation":[{"name":"Ipsen  Cambridge Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Valzasina","sequence":"additional","affiliation":[{"name":"Ipsen  Cambridge Massachusetts USA"}],"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"}]}],"member":"311","published-online":{"date-parts":[[2025,7,7]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00535-015-1110-z"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.32437"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2019.10.006"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1667299"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0168-8278(01)00299-9"},{"key":"e_1_2_12_7_1","first-page":"95","article-title":"Progressive Familial Intrahepatic Cholestasis: Diagnosis, Management, and Treatment","volume":"10","author":"Gunaydin M.","year":"2018","journal-title":"Hepatology, Medicine and Policy"},{"key":"e_1_2_12_8_1","unstructured":"\u201cClinical Synopsis Table: Cholestasis Progressive Familial Intrahepatic \u201d(2024) 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