{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:16:50Z","timestamp":1788221810793,"version":"build-2803163510"},"reference-count":14,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2021,1,20]],"date-time":"2021-01-20T00:00:00Z","timestamp":1611100800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:p>Dubin-Johnson syndrome (DJS) is a rare autosomal recessive disorder that typically manifests in young adulthood as jaundice with conjugated hyperbilirubinemia. We report a case presenting as neonatal cholestasis with the unexpected histologic finding of paucity of interlobular bile ducts, a feature that is not typically seen in DJS. The diagnosis was confirmed by absent canalicular multidrug-resistance-associated protein 2 (MRP2) immunohistochemical staining on liver biopsy tissue and molecular genetic testing that demonstrated heterozygous mutations in the ATP-Binding Cassette Subfamily C Member 2 ( ABCC2) gene, including a novel missense mutation. This report describes a case of DJS with atypical clinicopathologic findings and suggests that DJS should be considered in patients with neonatal cholestasis and bile duct paucity.<\/jats:p>","DOI":"10.1177\/1093526620980577","type":"journal-article","created":{"date-parts":[[2021,1,20]],"date-time":"2021-01-20T08:44:24Z","timestamp":1611132264000},"page":"154-158","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":5,"title":["A Case of Dubin-Johnson Syndrome Presenting as Neonatal Cholestasis With Paucity of Interlobular Bile Ducts"],"prefix":"10.1177","volume":"24","author":[{"given":"Kara L","family":"Chan","sequence":"first","affiliation":[{"name":"Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natasha","family":"Varughese","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, UT Southwestern Medical Center\/Children\u2019s Medical Center, Dallas, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia M","family":"Jones","sequence":"additional","affiliation":[{"name":"Department of Pathology, UT Southwestern Medical Center\/Children\u2019s Medical Center, Dallas, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David L","family":"Zwick","sequence":"additional","affiliation":[{"name":"Department of Pathology, UT Southwestern Medical Center\/Children\u2019s Medical Center, Dallas, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2128-2006","authenticated-orcid":false,"given":"Veena","family":"Rajaram","sequence":"additional","affiliation":[{"name":"Department of Pathology, UT Southwestern Medical Center\/Children\u2019s Medical Center, Dallas, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, UT Southwestern Medical Center\/Children\u2019s Medical Center, Dallas, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charina M","family":"Ramirez","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, UT Southwestern Medical Center\/Children\u2019s Medical Center, Dallas, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2021,1,20]]},"reference":[{"key":"bibr1-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1203\/01.pdr.0000203093.10908.bb"},{"key":"bibr2-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-195409000-00001"},{"key":"bibr3-1093526620980577","first-page":"119","volume-title":"Schiff\u2019s Disease of the Liver","author":"Wolkoff AW","year":"2018"},{"key":"bibr4-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-7020-6697-9.00003-0"},{"key":"bibr5-1093526620980577","first-page":"43","volume":"3","author":"Gotze T","year":"2015","journal-title":"Front Pediatr"},{"key":"bibr6-1093526620980577","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2011-0623-RA"},{"key":"bibr7-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2017.12.058"},{"key":"bibr8-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0676.2001.021004247.x"},{"key":"bibr9-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1186\/1476-5926-10-6"},{"key":"bibr10-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-018-3181-3"},{"key":"bibr11-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840100105"},{"key":"bibr12-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1038\/ejhg.2011.181"},{"key":"bibr13-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1136\/adc.65.8.898"},{"key":"bibr14-1093526620980577","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2005.02.030"}],"container-title":["Pediatric and Developmental Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/1093526620980577","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/1093526620980577","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/1093526620980577","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T04:07:07Z","timestamp":1777694827000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/1093526620980577"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,20]]},"references-count":14,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2021,4]]}},"alternative-id":["10.1177\/1093526620980577"],"URL":"https:\/\/doi.org\/10.1177\/1093526620980577","relation":{},"ISSN":["1093-5266","1615-5742"],"issn-type":[{"value":"1093-5266","type":"print"},{"value":"1615-5742","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1,20]]}}}