{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T08:16:03Z","timestamp":1788164163294,"version":"build-2803163510"},"reference-count":10,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T00:00:00Z","timestamp":1788134400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T00:00:00Z","timestamp":1788134400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JPGN rep."],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Progressive Familial Intrahepatic Cholestasis type 1 (PFIC1) is a multisystem disorder. Although liver transplant (LT) resolves the hepatic disease, post\u2010LT complications may occur, including severe enteropathy and graft steatosis caused by impaired bile acids handling by the native intestine. Surgical biliary diversion (SBD) is commonly used to alleviate these complications but may result in long\u2010term morbidity, such as fat\u2010soluble vitamin deficiency and essential fatty acids malabsorption. We report a PFIC1 patient who developed post\u2010LT protein\u2010losing enteropathy, initially managed with SBD. While effective, SBD led to severe fat malabsorption requiring intravenous lipid supplementation. After initiation of ileal bile acid transport inhibitor (IBATi) therapy, SBD was surgically reversed without recurrence of enteropathy. This case highlights IBATi as a potential alternative to SBD and as a strategy to reverse prior surgical interventions and improve quality of life.<\/jats:p>","DOI":"10.1002\/jpr3.70236","type":"journal-article","created":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T07:34:32Z","timestamp":1788161672000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Reversal of surgical biliary diversion with ileal bile acid transport inhibitors: A new chapter in progressive familiar intrahepatic cholestasis type 1 management?"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2884-9685","authenticated-orcid":false,"given":"Teresa","family":"Botelho","sequence":"first","affiliation":[{"name":"Pediatric Liver Transplant and Hepatology Unit Unidade Local de Sa\u00fade de Coimbra Coimbra Portugal"},{"name":"Pediatric Gastroenterology and Hepatology, Department of Pediatrics Cliniques Universitaires Saint\u2010Luc Brussels Belgium"},{"name":"Member of the European Reference Network Rare Liver"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Jannone","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Hepatology, Department of Pediatrics Cliniques Universitaires Saint\u2010Luc Brussels Belgium"},{"name":"Member of the European Reference Network Rare Liver"},{"name":"Member of the European Reference Networt TransplantChild"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Tambucci","sequence":"additional","affiliation":[{"name":"Member of the European Reference Network Rare Liver"},{"name":"Member of the European Reference Networt TransplantChild"},{"name":"Pediatric Surgery and Transplantation Unit, Department of Surgery Cliniques Universitaires Saint\u2010Luc Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"de Magnee","sequence":"additional","affiliation":[{"name":"Member of the European Reference Network Rare Liver"},{"name":"Member of the European Reference Networt TransplantChild"},{"name":"Pediatric Surgery and Transplantation Unit, Department of Surgery Cliniques Universitaires Saint\u2010Luc Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Scheers","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Hepatology, Department of Pediatrics Cliniques Universitaires Saint\u2010Luc Brussels Belgium"},{"name":"Member of the European Reference Network Rare Liver"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Etienne","family":"Sokal","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Hepatology, Department of Pediatrics Cliniques Universitaires Saint\u2010Luc Brussels Belgium"},{"name":"Member of the European Reference Network Rare Liver"},{"name":"Member of the European Reference Networt TransplantChild"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Stephenne","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Hepatology, Department of Pediatrics Cliniques Universitaires Saint\u2010Luc Brussels Belgium"},{"name":"Member of the European Reference Network Rare Liver"},{"name":"Member of the European Reference Networt TransplantChild"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,31]]},"reference":[{"issue":"1","key":"e_1_2_7_2_1","doi-asserted-by":"crossref","DOI":"10.1186\/1750-1172-4-1","article-title":"Progressive familial intrahepatic cholestasis","volume":"4","author":"Davit\u2010Spraul A","year":"2009","journal-title":"Orphanet J Rare Dis"},{"issue":"5","key":"e_1_2_7_3_1","doi-asserted-by":"crossref","first-page":"E177","DOI":"10.1111\/j.1399-3046.2011.01514.x","article-title":"Recovery of graft steatosis and protein\u2010losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child","volume":"16","author":"Nicastro E","year":"2012","journal-title":"Pediatr Transplant"},{"issue":"19","key":"e_1_2_7_4_1","article-title":"Management and outcomes after liver transplantation for progressive familial intrahepatic cholestasis: a systematic review and meta\u2010analysis","volume":"7","author":"Kavallar AM","year":"2023","journal-title":"Hepatol Commun"},{"issue":"2","key":"e_1_2_7_5_1","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1002\/hep.31787","article-title":"Impact of genotype, serum bile acids, and surgical biliary diversion on native liver survival in FIC1 deficiency","volume":"74","author":"Wessel DBE","year":"2021","journal-title":"Hepatology"},{"issue":"8","key":"e_1_2_7_6_1","doi-asserted-by":"crossref","first-page":"1812","DOI":"10.1111\/liv.14553","article-title":"Potential of ileal bile acid transporter inhibition as a therapeutic target in Alagille syndrome and progressive familial intrahepatic cholestasis","volume":"40","author":"Kamath BM","year":"2020","journal-title":"Liver Int"},{"issue":"5","key":"e_1_2_7_7_1","doi-asserted-by":"crossref","DOI":"10.3390\/children9050669","article-title":"Ileal bile acid transporter inhibition reduces post\u2010transplant diarrhea and growth failure in FIC1 disease\u2014a case report","volume":"9","author":"Ohlendorf J","year":"2022","journal-title":"Children"},{"issue":"5","key":"e_1_2_7_8_1","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1111\/j.1399-3046.2008.00878.x","article-title":"Liver retransplantation with external biliary diversion for progressive familial intrahepatic cholestasis type 1: a case report","volume":"13","author":"Usui M","year":"2009","journal-title":"Pediatr Transplant"},{"issue":"4","key":"e_1_2_7_9_1","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1002\/jpn3.12462","article-title":"Recent advances in the management of pediatric cholestatic liver diseases","volume":"80","author":"Mysore KR","year":"2025","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"10","key":"e_1_2_7_10_1","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1007\/s00431-020-03646-z","article-title":"Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II","volume":"179","author":"Jannone G","year":"2020","journal-title":"Eur J Pediatr"},{"issue":"6","key":"e_1_2_7_11_1","doi-asserted-by":"crossref","first-page":"1410","DOI":"10.1002\/jpn3.70227","article-title":"Odevixibat after liver transplant in patients with progressive familial intrahepatic cholestasis type 1: a case series","volume":"81","author":"Vogel G","year":"2025","journal-title":"J Pediatr Gastroenterol Nutr"}],"container-title":["JPGN 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