{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:50:35Z","timestamp":1788306635176,"version":"build-2803163510"},"reference-count":14,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,11,18]],"date-time":"2024-11-18T00:00:00Z","timestamp":1731888000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mutations in doublecortin domain\u2010containing protein 2 (<jats:italic>DCDC2<\/jats:italic>) lead to neonatal sclerosing cholangitis (NSC), and portal hypertension (PHTN). The objective of the study was to systematically evaluate PHTN, variceal bleeding, and outcomes of patients with <jats:italic>DCDC2<\/jats:italic>\u2010related NSC. The study included children with homozygous or compound heterozygous variants in <jats:italic>DCDC2<\/jats:italic>. All 14 children with <jats:italic>DCDC2<\/jats:italic>\u2010related NSC had PHTN. Eight (57.1%) developed variceal bleed at a median age of 3 years (range: 1.9\u20135 years). Eleven (78.6%) children with high\u2010risk varices underwent endotherapy. Varices were completely eradicated in three, downstaged to low\u2010risk in five, and there was no response with endotherapy in three. All three children with failure to eradicate\/downstage varices had rebleed, and required listing for liver transplantation (LT). The study shows that children with variants in <jats:italic>DCDC2<\/jats:italic> have a high incidence of variceal bleed at a very young age. Variceal eradication may often be difficult and rebleed rates are high; often necessitating LT.<\/jats:p>","DOI":"10.1002\/jpn3.12414","type":"journal-article","created":{"date-parts":[[2024,11,18]],"date-time":"2024-11-18T09:41:04Z","timestamp":1731922864000},"page":"189-196","source":"Crossref","is-referenced-by-count":2,"title":["Portal hypertension in doublecortin domain\u2010containing protein 2 (DCDC2)\u2010related neonatal sclerosing cholangitis"],"prefix":"10.1002","volume":"80","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-6960-8464","authenticated-orcid":false,"given":"Prabhsaran","family":"Kaur","sequence":"first","affiliation":[{"name":"Department of Pediatric Hepatology Institute of Liver and Biliary Sciences New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6275-7736","authenticated-orcid":false,"given":"Bikrant Bihari","family":"Lal","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hepatology Institute of Liver and Biliary Sciences New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepa","family":"Janakiraman","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Institute of Child Health and Hospital for Children Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2691-3559","authenticated-orcid":false,"given":"Nirmala","family":"Dheivamani","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Institute of Child Health and Hospital for Children Chennai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Snehavardhan","family":"Pandey","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Sahyadri Superspeciality Hospital Pune 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