{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T05:52:24Z","timestamp":1790661144482,"version":"4.1.0"},"reference-count":16,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T00:00:00Z","timestamp":1786665600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:p>Biliary atresia (BA) is the most common cause of cholestasis in infants, and pruritus may be a prominent and debilitating complication. Maralixibat is the first US Food and Drug Administration\u2013approved drug for the treatment of cholestatic pruritus in children with Alagille syndrome and has been subsequently approved for treatment of progressive familial intrahepatic cholestasis as well. Several patients with BA have received maralixibat as part of a compassionate use program. We report the use of maralixibat for the treatment of cholestatic pruritus in BA for six patients. Demographics, past medical history, laboratory markers, and medications were collected. Pruritus was assessed using the Clinician Scratch Scale (CSS) at baseline and last clinical follow-up. All patients reported improved CSS with no increased usage of other antipruritic medications, and the medication was well tolerated.<\/jats:p>","DOI":"10.3389\/fped.2026.1858862","type":"journal-article","created":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T15:54:16Z","timestamp":1786722856000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Real-world use of maralixibat in biliary atresia: a case series"],"prefix":"10.3389","volume":"14","author":[{"given":"Natasha","family":"Dilwali","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Johns Hopkins University","place":["Baltimore, MD, United States"]},{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Columbia University Irving Medical Center","place":["New York, NY, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas B.","family":"Mogul","sequence":"additional","affiliation":[{"name":"Mirum Pharmaceuticals, Inc.","place":["Foster City, CA, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon M.","family":"Vandriel","sequence":"additional","affiliation":[{"name":"Mirum Pharmaceuticals, Inc.","place":["Foster City, CA, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tony","family":"Tokman","sequence":"additional","affiliation":[{"name":"Mirum Pharmaceuticals, Inc.","place":["Foster City, CA, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine A.","family":"Chapin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago","place":["Chicago, IL, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bertrand","family":"Roquelaure","sequence":"additional","affiliation":[{"name":"APHM, Multidisciplinary Pediatrics Department, Timone Children\u2019s Hospital","place":["Marseille, France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rossitsa","family":"Rousseva","sequence":"additional","affiliation":[{"name":"King\u2019s College Hospital","place":["London, United Kingdom"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johanna","family":"Ferreira","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Liver Disease and Nutrition, Cohen Children\u2019s Medical Center","place":["New Hyde Park, NY, United States"]},{"name":"Pediatric Gastroenterology, Hepatology, and Nutrition, University of Miami Miller School of Medicine","place":["Miami, FL, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mercedes","family":"Martinez","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Columbia University Irving Medical Center","place":["New York, NY, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benno","family":"Kohlmaier","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Medizinische Universit\u00e4t Graz","place":["Graz, Austria"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,8,14]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1704","DOI":"10.1016\/s0140-6736(09)60946-6","article-title":"Biliary atresia","volume":"374","author":"Hartley","year":"2009","journal-title":"Lancet"},{"key":"B2","doi-asserted-by":"publisher","first-page":"341","DOI":"10.1016\/j.cld.2022.03.001","article-title":"Biliary atresia in children: update on disease mechanism, 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