{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T05:35:19Z","timestamp":1791264919906,"version":"4.1.0"},"reference-count":8,"publisher":"American Academy of Pediatrics (AAP)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2010,9,1]]},"abstract":"<jats:p>The mechanisms that determine organ identity along the digestive tract in humans are poorly understood. Here we describe the rare case of a young patient who presented with congenital gastric-type heteroplasia in the midjejunum. The lesions, located along the antimesenteric midline of the gut, were made of histologically and functionally normal gastric epithelium without inflammation or in situ\/invasive carcinoma. They resembled the anatomy of the lesions developing in the mouse gut as a result of haploinsufficiency of the Cdx2 homeobox gene. The lesions were devoid of CDX2 but without mutation in the coding sequence or in a cis-regulatory element required for intestine-specific expression. Combining these data with the CDX2 expression pattern established from human embryos and cases of Meckel diverticula, we propose a scenario for this patient's presentation, in which CDX2 was missing at the site of ventral closure during gut morphogenesis, with subsequent default differentiation into gastric instead of intestinal tissue. Altogether, these observations argue in favor of a pivotal role played by CDX2 in determining intestinal identity during human embryonic development, as previously shown experimentally in mice.<\/jats:p>","DOI":"10.1542\/peds.2009-3512","type":"journal-article","created":{"date-parts":[[2010,8,3]],"date-time":"2010-08-03T03:27:41Z","timestamp":1280806061000},"page":"e723-e727","source":"Crossref","is-referenced-by-count":10,"title":["<i>CDX2<\/i> in Congenital Gut Gastric-Type Heteroplasia and Intestinal-Type Meckel Diverticula"],"prefix":"10.1542","volume":"126","author":[{"given":"Elisabeth","family":"Martin","sequence":"first","affiliation":[{"name":"INSERM, U682, Strasbourg, France;"},{"name":"Universit\u00e9 de Strasbourg, Strasbourg, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Vanier","sequence":"additional","affiliation":[{"name":"INSERM, U682, Strasbourg, France;"},{"name":"Universit\u00e9 de Strasbourg, Strasbourg, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Tavian","sequence":"additional","affiliation":[{"name":"INSERM, U682, Strasbourg, France;"},{"name":"Universit\u00e9 de Strasbourg, Strasbourg, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Guerin","sequence":"additional","affiliation":[{"name":"INSERM, U682, Strasbourg, France;"},{"name":"Universit\u00e9 de Strasbourg, Strasbourg, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Domon-Dell","sequence":"additional","affiliation":[{"name":"INSERM, U682, Strasbourg, France;"},{"name":"Universit\u00e9 de Strasbourg, Strasbourg, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Duluc","sequence":"additional","affiliation":[{"name":"INSERM, U682, Strasbourg, France;"},{"name":"Universit\u00e9 de Strasbourg, Strasbourg, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Gross","sequence":"additional","affiliation":[{"name":"INSERM, U682, Strasbourg, France;"},{"name":"Universit\u00e9 de Strasbourg, Strasbourg, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Rowland","sequence":"additional","affiliation":[{"name":"Departments of Pathology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunghoon","family":"Kim","sequence":"additional","affiliation":[{"name":"Pediatric Surgery, Children's Hospital & Research Center Oakland, Oakland, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-No\u00ebl","family":"Freund","sequence":"additional","affiliation":[{"name":"INSERM, U682, Strasbourg, France;"},{"name":"Universit\u00e9 de Strasbourg, Strasbourg, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2010,9,1]]},"reference":[{"issue":"3","key":"2021121009452589700_B1","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1007\/s100249910036","article-title":"Extensive gastric heterotopia of the small intestine resulting in massive gastrointestinal bleeding, bowel perforation, and death: report of a case and review of the literature","volume":"3","author":"Lambert","year":"2000","journal-title":"Pediatr Dev Pathol"},{"issue":"13","key":"2021121009452589700_B2","doi-asserted-by":"crossref","first-page":"7318","DOI":"10.1073\/pnas.96.13.7318","article-title":"Reprogramming of intestinal differentiation and intercalary regeneration in Cdx2 mutant mice","volume":"96","author":"Beck","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"4","key":"2021121009452589700_B3","doi-asserted-by":"crossref","first-page":"588","DOI":"10.1016\/j.devcel.2009.02.010","article-title":"Establishment of intestinal identity and epithelial-mesenchymal signaling by Cdx2","volume":"16","author":"Gao","year":"2009","journal-title":"Dev Cell"},{"issue":"4","key":"2021121009452589700_B4","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1053\/j.gastro.2008.06.045","article-title":"Multiple regulatory regions control the complex expression pattern of the mouse Cdx2 homeobox gene","volume":"135","author":"Benahmed","year":"2008","journal-title":"Gastroenterology"},{"key":"2021121009452589700_B5","first-page":"413","article-title":"Analysis of hematopoietic development during human embryonic ontogenesis","volume":"105","author":"Tavian","year":"2005","journal-title":"Methods Mol Med"},{"issue":"2","key":"2021121009452589700_B6","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1016\/S0006-291X(02)00480-1","article-title":"Conversion of gastric mucosa to intestinal metaplasia in Cdx2-expressing transgenic mice","volume":"294","author":"Mutoh","year":"2002","journal-title":"Biochem Biophys Res Commun"},{"issue":"1","key":"2021121009452589700_B7","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1136\/gut.2004.042770","article-title":"Pericryptal fibroblast sheath in intestinal metaplasia and gastric carcinoma","volume":"54","author":"Mutoh","year":"2005","journal-title":"Gut"},{"issue":"3","key":"2021121009452589700_B8","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1042\/BJ20091177","article-title":"Direct repression of Sonic Hedgehog expression in the stomach by Cdx2 leads to intestinal transformation","volume":"427","author":"Mutoh","year":"2010","journal-title":"Biochem J"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/126\/3\/e723\/846619\/zpe0091000e723.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/126\/3\/e723\/846619\/zpe0091000e723.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T21:44:49Z","timestamp":1639172689000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/126\/3\/e723\/66210\/CDX2-in-Congenital-Gut-Gastric-Type-Heteroplasia"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,9,1]]},"references-count":8,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2010,9,1]]},"published-print":{"date-parts":[[2010,9,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2009-3512","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,9,1]]}}}