{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T17:04:31Z","timestamp":1784307871701,"version":"3.55.0"},"reference-count":9,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T00:00:00Z","timestamp":1781740800000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:00:00Z","timestamp":1780272000000},"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":["Clinical Case Reports"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Progressive familial intrahepatic cholestasis type 3 (PFIC\u20103), caused by pathogenic variants in the ABCB4 gene, is a rare inherited cholestatic liver disorder that often presents later in childhood. In some patients, hematologic manifestations may dominate the clinical picture and delay recognition of the underlying liver disease. We report a 4\u2010year\u2010old girl referred for evaluation of anemia, hepatosplenomegaly, and recurrent bleeding. Initial investigation focused on primary hematologic causes. However, imaging revealed portal hypertension with esophageal varices, and genetic testing identified a homozygous pathogenic ABCB4 variant, confirming the diagnosis of PFIC\u20103. This case highlights how chronic cholestatic liver disease may masquerade as a primary hematologic disorder and emphasizes the importance of considering systemic causes in children presenting with anemia, hepatosplenomegaly, and bleeding manifestations. Early recognition of underlying liver disease is essential to facilitate appropriate multidisciplinary management and prevent potentially life\u2010threatening complications.<\/jats:p>","DOI":"10.1002\/ccr3.72961","type":"journal-article","created":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T04:24:12Z","timestamp":1781843052000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Hematologic Masquerader: Progressive Familial Intrahepatic Cholestasis Type 3 Presenting as Anemia, Hepatosplenomegaly, and Recurrent Bleeding in a Child"],"prefix":"10.1002","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-9407-0633","authenticated-orcid":false,"given":"Njood","family":"Alwadei","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology\/Oncology King Khalid University Medical City  Abha Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,18]]},"reference":[{"key":"e_1_2_11_2_1","first-page":"2086","article-title":"Portal Hypertensive Gastropathy","volume":"102","author":"Garcia\u2010Tsao G.","year":"1992","journal-title":"Gastroenterology"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1011170"},{"key":"e_1_2_11_4_1","first-page":"742","article-title":"Coagulation in Liver Disease: A Guide for the Clinician","volume":"7","author":"Northup P. 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F.","year":"2018","journal-title":"New England Journal of Medicine"}],"container-title":["Clinical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ccr3.72961","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ccr3.72961","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ccr3.72961","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T16:20:59Z","timestamp":1784305259000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ccr3.72961"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6]]},"references-count":9,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,6]]}},"alternative-id":["10.1002\/ccr3.72961"],"URL":"https:\/\/doi.org\/10.1002\/ccr3.72961","archive":["Portico"],"relation":{},"ISSN":["2050-0904","2050-0904"],"issn-type":[{"value":"2050-0904","type":"print"},{"value":"2050-0904","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6]]},"assertion":[{"value":"2026-02-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-07","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-18","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e72961"}}