{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T22:00:53Z","timestamp":1785448853646,"version":"3.56.0"},"reference-count":10,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2024,4,4]],"date-time":"2024-04-04T00:00:00Z","timestamp":1712188800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2024,4,4]],"date-time":"2024-04-04T00:00:00Z","timestamp":1712188800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JPGN rep."],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Odevixibat, a reversible ileal bile acid transport inhibitor, has been shown to reduce serum bile acids (sBA) and pruritus mostly in children with progressive familial intrahepatic cholestasis (PFIC) 1 and 2 in clinical trials and case reports. There are currently no published case reports or series describing its use in rare variants of cholestatic liver disease.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We describe three children with progressive cholestatic liver disease who developed refractory pruritus, who had a genotypic diagnosis of\n                      <jats:italic>AKR1D1<\/jats:italic>\n                      ,\n                      <jats:italic>ABCB4<\/jats:italic>\n                      variant, and\n                      <jats:italic>PKHD1<\/jats:italic>\n                      and\n                      <jats:italic>PKHD2<\/jats:italic>\n                      variants; all being variants of unknown significance as per the American College of Medical Genetics and Genomics guidelines.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      On Odevixibat there was a significant improvement in sBA (absolute change from baseline: \u2212196 and \u2212393\u2009\u03bcmol\/L) and pruritus in two children with heterozygous\n                      <jats:italic>AKR1D1<\/jats:italic>\n                      and\n                      <jats:italic>ABCB4<\/jats:italic>\n                      mutations. The child with\n                      <jats:italic>ABCB4<\/jats:italic>\n                      variants was found to have features of sclerosing cholangitis along with a diagnosis of Crohn's disease, which represents the first reported usage of Odevixibat in such a case with good response. There was some reported improvement in the third child with\n                      <jats:italic>PKHD1<\/jats:italic>\n                      and\n                      <jats:italic>PKHD2<\/jats:italic>\n                      variants; however, we hypothesize that no sustained improvement could be due to severe and progressive nature of the disease. There were no side effects reported and it was well tolerated in all.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>We suggest that Odevixibat may be used as an adjunctive drug in refractory pruritus and could be started early in the course of disease if clinically and phenotypically indicated.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpr3.12069","type":"journal-article","created":{"date-parts":[[2024,4,4]],"date-time":"2024-04-04T07:19:04Z","timestamp":1712215144000},"page":"296-302","source":"Crossref","is-referenced-by-count":5,"title":["Odevixibat as an adjunctive treatment for refractory pruritus in rare variants of cholestatic liver disease"],"prefix":"10.1002","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8442-3809","authenticated-orcid":false,"given":"Akshat","family":"Goel","sequence":"first","affiliation":[{"name":"Paediatric Liver, GI and Nutrition Centre and MowatLabs King's College Hospital London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bethany","family":"Tucker","sequence":"additional","affiliation":[{"name":"Paediatric Liver, GI and Nutrition Centre and MowatLabs King's College Hospital London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorena Soler","family":"Casale","sequence":"additional","affiliation":[{"name":"Gibraltar Health Authority  Gibraltar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0174-4787","authenticated-orcid":false,"given":"Tassos","family":"Grammatikopoulos","sequence":"additional","affiliation":[{"name":"Paediatric Liver, GI and Nutrition Centre and MowatLabs King's College Hospital London UK"},{"name":"Institute of Liver Studies, Faculty of Life Sciences and Medicine King's College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,4,4]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinre.2018.07.010"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.29019"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-021-01594-y"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2468-1253(22)00093-0"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinre.2021.101751"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/PG9.0000000000000301"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2023.1061535"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinre.2023.102185"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2015.30"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/HC9.0000000000000153"}],"container-title":["JPGN Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpr3.12069","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jpr3.12069","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpr3.12069","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T21:24:19Z","timestamp":1785446659000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jpr3.12069"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,4]]},"references-count":10,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,8]]}},"alternative-id":["10.1002\/jpr3.12069"],"URL":"https:\/\/doi.org\/10.1002\/jpr3.12069","archive":["Portico"],"relation":{},"ISSN":["2691-171X","2691-171X"],"issn-type":[{"value":"2691-171X","type":"print"},{"value":"2691-171X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,4]]}}}