{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T10:12:32Z","timestamp":1789035152688,"version":"build-2803163510"},"reference-count":31,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,8,8]],"date-time":"2026-08-08T00:00:00Z","timestamp":1786147200000},"content-version":"vor","delay-in-days":38,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Special Care in Dentistry"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    <jats:bold>Aims<\/jats:bold>\n                    : Developmental delay with gastrointestinal, cardiovascular, genitourinary, and skeletal abnormalities (DEGCAGS) syndrome (OMIM #619488) is a rare autosomal recessive disorder caused by pathogenic variants in ZNF699 and characterized by developmental delay and multisystem involvement. Although several systemic and craniofacial manifestations have been described, oral findings have not yet been reported. This case report describes the craniofacial and oral features of a patient with DEGCAGS syndrome, contributing to the expansion of its phenotypic spectrum.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Methods and Results<\/jats:bold>\n                    : A 10\u2010year\u2010old girl with a homozygous pathogenic variant in ZNF699 was referred for dental evaluation. Clinical examination revealed hair thinning, dolichocephaly, retromicrognathia, synophrys, smooth philtrum, thin upper lip, increased overjet, and deep bite. Intraoral findings included generalized spacing, gingivitis, active carious lesions, hypomineralization of primary molars, talon cusps on the maxillary central incisors, erosive tooth wear, and signs consistent with sleep bruxism. Radiographic evaluation revealed taurodontism, particularly in teeth 16 and 26, shortened roots in the mandibular incisors, and increased pericoronal space associated with developing teeth.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Conclusion<\/jats:bold>\n                    : These findings expand the oral phenotype of DEGCAGS syndrome and highlight the importance of dental evaluation in patients with rare genetic disorders. Further reports are needed to better characterize the oral manifestations associated with this condition.\n                  <\/jats:p>","DOI":"10.1111\/scd.70232","type":"journal-article","created":{"date-parts":[[2026,8,8]],"date-time":"2026-08-08T09:51:36Z","timestamp":1786182696000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Expanding the Phenotypic Spectrum of Degcags Syndrome: Novel Craniofacial and Oral Findings"],"prefix":"10.1111","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-4003-7833","authenticated-orcid":false,"given":"Jeferson","family":"Paiva","sequence":"first","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1799-780X","authenticated-orcid":false,"given":"Lucas","family":"Batista\u2010Fontes","sequence":"additional","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jana\u00edna Ta\u00edza Ara\u00fajo de","family":"Jesus","sequence":"additional","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7290-8451","authenticated-orcid":false,"given":"Kelly Fernanda","family":"Molena","sequence":"additional","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raquel Assed Bezerra da","family":"Silva","sequence":"additional","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kranya Victoria","family":"Diaz\u2010Serrano","sequence":"additional","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8559-532X","authenticated-orcid":false,"given":"Francisco Wanderley Garcia de","family":"Paula\u2010Silva","sequence":"additional","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2900-5000","authenticated-orcid":false,"given":"Alexandra Mussolino de","family":"Queiroz","sequence":"additional","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabr\u00edcio Kitazono de","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Department of Pediatric Dentistry School of Dentistry of Ribeir\u00e3o Preto University of S\u00e3o Paulo  Ribeir\u00e3o Preto S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,8]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41436-021-01159-0"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.3390\/genes13020168"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.63111"},{"issue":"13","key":"e_1_2_10_5_1","article-title":"Two Case Report of a New Recognizable Syndrome\u2014DEGCAGS (Developmental delay With Gastrointestinal, Cardiovascular, Genitourinary, and Skeletal Abnormalities)","volume":"102","author":"Santos M.","year":"2023","journal-title":"Medicine"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/mgg3.2329"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41431-024-01702-y"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.31972"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.12659\/AJCR.948733"},{"issue":"37","key":"e_1_2_10_10_1","first-page":"603","article-title":"The CARE guidelines: consesus\u2010based clinical case reporting guideline development","volume":"110","author":"Gagnier J. 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