{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T14:24:18Z","timestamp":1784903058449,"version":"3.55.0"},"reference-count":14,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2025,6,6]],"date-time":"2025-06-06T00:00:00Z","timestamp":1749168000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,6,6]],"date-time":"2025-06-06T00:00:00Z","timestamp":1749168000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"name":"Department of Medicine, G\u00f8dstrup regional hospital"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Sage Open Medical Case Reports"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:p>\n                    Caroli\u2019s syndrome is a rare autosomal recessive disorder characterized by segmental cystic dilation of the intrahepatic bile ducts and congenital hepatic fibrosis. It is associated with autosomal recessive polycystic kidney disease. Here, we report a 63-year-old male known with polycystic kidney disease, who was admitted to our hospital with intermittent fever, accompanied by weight loss. Magnetic resonance cholangiopancreatography suggested the presence of Caroli\u2019s syndrome, characterized by cystic dilation of the intrahepatic proximal bile ducts on both the right and left lobes of the liver. Shear wave elastography revealed the presence of mild liver fibrosis. The patient recovered with broad-spectrum antibiotics. Genetic testing found no evidence of variation in the\n                    <jats:italic toggle=\"yes\">PKHD1<\/jats:italic>\n                    gene. However, a heterozygous deletion of the\n                    <jats:italic toggle=\"yes\">PKD1<\/jats:italic>\n                    gene was identified, a deletion that has not previously been linked to disease, and therefore the patient\u2019s family was offered genetic analysis. The patient has since remained free of cholangitis and icterus and continues to be followed in the nephrology department.\n                  <\/jats:p>","DOI":"10.1177\/2050313x251346657","type":"journal-article","created":{"date-parts":[[2025,6,23]],"date-time":"2025-06-23T12:57:08Z","timestamp":1750683428000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Cholangitis resembling Caroli\u2019s syndrome in a patient with autosomal dominant polycystic kidney disease: Case report"],"prefix":"10.1177","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-7503-364X","authenticated-orcid":false,"given":"Maha Arkan","family":"Khudhair","sequence":"first","affiliation":[{"name":"Department of Medicine, G\u00f8dstrup Regional Hospital, Herning, Central Denmark Region, Denmark"},{"name":"Department of Gastroenterology and Hepatology, Aalborg University Hospital, North Denmark Region, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rikke","family":"Christensen","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics, Aarhus University Hospital, Central Denmark Region, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Skakkeb\u00e6k","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics, Aarhus University Hospital, Central Denmark Region, Denmark"},{"name":"Department of Clinical Medicine, Aarhus University Hospital, Central Denmark Region, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jakob Grue","family":"Graverholt","sequence":"additional","affiliation":[{"name":"Department of Radiology and Scanning, G\u00f8dstrup Regional Hospital, Herning, Central Denmark Region, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anders Bergh","family":"L\u00f8drup","sequence":"additional","affiliation":[{"name":"Clinic of Gastroenterology and Hepatology, Medical Department, G\u00f8dstrup Regional Hospital, Herning, Central Denmark Region, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2025,6,6]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13000-021-01142-y"},{"issue":"10","key":"e_1_3_2_3_2","article-title":"Caroli\u2019s syndrome: an early presentation","volume":"12","author":"Acevedo E","year":"2020","unstructured":"Acevedo E, La\u00ednez SS, C\u00e1ceres Cano PA, et al. Caroli\u2019s syndrome: an early presentation. Cureus 2020; 12(10): e11029.","journal-title":"Cureus"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/hpb.12330"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v13.i13.1930"},{"key":"e_1_3_2_6_2","unstructured":"Online Mendelian Inheritance in Man (Internet) https:\/\/omim.org\/entry\/173900"},{"key":"e_1_3_2_7_2","unstructured":"The Human Gene Mutation Database version 2023.1 (Internet) https:\/\/www.hgmd.cf.ac.uk\/"},{"key":"e_1_3_2_8_2","unstructured":"The ADPKD Variant Database (Internet) https:\/\/pkdb.mayo.edu\/welcome"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1997.300"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.55.6818"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12884-021-03768-8"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.179.4.1791053"},{"issue":"5","key":"e_1_3_2_13_2","first-page":"434","article-title":"Choledochal cysts: part 1 of 3: classification and pathogenesis","volume":"52","author":"Singham J","year":"2009","unstructured":"Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 1 of 3: classification and pathogenesis. Can J Surg 2009; 52(5): 434\u2013440.","journal-title":"Can J Surg"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.165.2.7618550"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.radcr.2024.09.080"}],"container-title":["Sage Open Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/2050313X251346657","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/2050313X251346657","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/2050313X251346657","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T05:19:34Z","timestamp":1767676774000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/2050313X251346657"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,6]]},"references-count":14,"alternative-id":["10.1177\/2050313X251346657"],"URL":"https:\/\/doi.org\/10.1177\/2050313x251346657","relation":{},"ISSN":["2050-313X","2050-313X"],"issn-type":[{"value":"2050-313X","type":"print"},{"value":"2050-313X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,6]]},"article-number":"2050313X251346657"}}