{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T00:23:45Z","timestamp":1787271825664,"version":"build-2736575974"},"reference-count":13,"publisher":"Georg Thieme Verlag KG","funder":[{"name":"Else Kr\u00f6ner-Fresenius Foundation"},{"DOI":"10.13039\/100020228","name":"Hamburgischen Stiftung f\u00fcr Wissenschaften, Entwicklung und Kultur Helmut und Hannelore Greve","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100020228","id-type":"DOI","asserted-by":"crossref"}]},{"name":"YAEL Foundation"},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"crossref","award":["493624519,CRC1700, Grant No 530990199"],"award-info":[{"award-number":["493624519,CRC1700, Grant No 530990199"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Z Gastroenterol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>X-linked hyper IgM-syndrome caused by CD40L deficiency is a rare primary immunodeficiency. Affected male individuals who reach adulthood often develop sclerosing cholangitis. Here, we report the case of a 33-year-old patient with long-standing solitary pre-papillary bile duct stenosis but without further signs of sclerosing cholangitis, who developed distal cholangiocarcinoma. Given the increased risk of malignancy in these patients, this case highlights, that individuals with CD40L deficiency should be screened for the presence of cholangiopathy and be closely monitored for any signs of disease progression, development of hepatobiliary dysplasia or malignancy.<\/jats:p>","DOI":"10.1055\/a-2922-7771","type":"journal-article","created":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T23:35:37Z","timestamp":1787268937000},"source":"Crossref","is-referenced-by-count":0,"title":["Cholangiocarcinoma in a 33-year-old male with CD40L deficiency and pre-papillary bile duct stenosis"],"prefix":"10.1055","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7248-7266","authenticated-orcid":false,"given":"Britta F.","family":"Zecher","sequence":"additional","affiliation":[{"name":"University Medical Center Hamburg-Eppendorf, Germany","place":["Hamburg"],"department":["I. Department of Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guido","family":"Schachschal","sequence":"additional","affiliation":[{"name":"University Medical Center Hamburg-Eppendorf, Germany","place":["Hamburg"],"department":["Department of Interdisciplinary Endoscopy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabel","family":"Molwitz","sequence":"additional","affiliation":[{"name":"University Medical Center Hamburg-Eppendorf, Germany","place":["Hamburg"],"department":["Department of Diagnostic and Interventional Radiology and Nuclear Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Till Sebastian","family":"Clauditz","sequence":"additional","affiliation":[{"name":"University Medical Center Hamburg-Eppendorf, Germany","place":["Hamburg"],"department":["Institute of Pathology"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ansgar W.","family":"Lohse","sequence":"additional","affiliation":[{"name":"University Medical Center Hamburg-Eppendorf, Germany","place":["Hamburg"],"department":["I. Department of Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Schramm","sequence":"additional","affiliation":[{"name":"University Medical Center Hamburg-Eppendorf, Germany","place":["Hamburg"],"department":["I. Department of Medicine"]},{"name":"University Medical Center Hamburg-Eppendorf, Germany","place":["Hamburg"],"department":["Martin Zeitz Center for Rare Diseases"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2026,8,20]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"2694","DOI":"10.1182\/blood.V99.8.2694","article-title":"Autoimmunity in human primary immunodeficiency diseases","volume":"99","author":"PD Arkwright","year":"2002","journal-title":"Blood"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"977","DOI":"10.4049\/jimmunol.158.2.977","article-title":"Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM","volume":"158","author":"AR Hayward","year":"1997","journal-title":"J Immunol"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1097\/01.md.0000100046.06009.b0","article-title":"The X-linked 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