{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T07:48:32Z","timestamp":1791877712359,"version":"4.3.4"},"reference-count":10,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T00:00:00Z","timestamp":1791849600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T00:00:00Z","timestamp":1791849600000},"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), caused by ATP8B1 deficiency, is a multisystem disorder with extrahepatic manifestations; however, renal involvement has rarely been described. We report a child with genetically confirmed PFIC1 who underwent living donor liver transplantation (LT) for progressive liver disease. Before transplantation, serum creatinine was normal, but a Fanconi\u2010like proximal tubular dysfunction was characterized by elevated urinary \u03b22\u2010microglobulin, proteinuria, phosphaturia, hypercalciuria, and metabolic acidosis requiring bicarbonate supplementation. Following transplantation, these abnormalities rapidly resolved, supporting the hypothesis of reversible cholestasis\u2010associated tubular injury. This case identifies proximal tubular dysfunction as an underrecognized, potentially reversible manifestation of severe, prolonged cholestasis in PFIC1 and supports systematic evaluation of tubular markers before transplantation.<\/jats:p>","DOI":"10.1002\/jpr3.70258","type":"journal-article","created":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T07:20:44Z","timestamp":1791876044000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Resolution of proximal tubular dysfunction after liver transplantation in ATP8B1 deficiency: A case report"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-2756-8794","authenticated-orcid":false,"given":"Angela","family":"Marino","sequence":"first","affiliation":[{"name":"Department of Pediatric Gastroenterology and Hepatology Cliniques universitaires Saint\u2010Luc, Universit\u00e9 catholique de Louvain Brussels Belgium"},{"name":"European Reference Network on Transplantation in Children"},{"name":"European Reference Network on Rare Liver Diseases"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7354-4741","authenticated-orcid":false,"given":"Nathalie","family":"Godefroid","sequence":"additional","affiliation":[{"name":"Department of Specialized Pediatrics, Pediatric Nephrology, Cliniques universitaires Saint\u2010Luc Universit\u00e9 catholique de Louvain Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"De Magn\u00e9e","sequence":"additional","affiliation":[{"name":"Pediatric Surgery and Transplantation Unit, Cliniques universitaires Saint\u2010Luc Universit\u00e9 catholique de Louvain Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Stephenne","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology and Hepatology Cliniques universitaires Saint\u2010Luc, Universit\u00e9 catholique de Louvain Brussels Belgium"},{"name":"European Reference Network on Transplantation in Children"},{"name":"European Reference Network on Rare Liver Diseases"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,10,13]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1038\/ng0398-219","article-title":"A gene encoding a P\u2010type ATPase mutated in two forms of hereditary cholestasis","volume":"18","author":"Bull LN","year":"1998","journal-title":"Nature Genet"},{"issue":"1","key":"e_1_2_7_3_1","doi-asserted-by":"crossref","first-page":"1","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 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