{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T12:30:33Z","timestamp":1792672233998,"version":"4.5.0"},"reference-count":30,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,2,2]],"date-time":"2021-02-02T00:00:00Z","timestamp":1612224000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Paed Dentistry"],"published-print":{"date-parts":[[2021,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Chromosome 22q11.2 deletion syndrome (22q11.2DS) is a multifaceted syndrome with a variable phenotype. Few studies have described the associated dental characteristics and their relationship with medical co\u2010morbidities; and no Australian data exist.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To determine the clinical manifestations and correlations between oral and medical conditions in children with 22q11.2DS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design<\/jats:title><jats:p>A retrospective observational study. Children genetically diagnosed with 22q11.2DS at the Royal Children's Hospital Melbourne were selected; their medical and dental characteristics were collated and analysed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The study population (n\u00a0=\u00a057; mean age 11.5\u00a0years, range 2\u201027\u00a0years) experienced a range of medical conditions involving multiple medical systems; of whom 44 (77.2%) had caries experience, 7 (12.3%) developmentally missing teeth, and 31 (54.4%) developmental defects of enamel (DDE). Smaller proportions of primary teeth were affected by DDE in children with congenital heart disease (2.2% vs 9.7%;<jats:italic>P<\/jats:italic>\u00a0=\u00a0.02), and cardiac surgery (0.2% vs 9%;<jats:italic>P<\/jats:italic>\u00a0=\u00a0.001). Conversely, children with hypoparathyroidism (n\u00a0=\u00a02) had significantly higher proportions of primary teeth affected by DDE (27.5% vs 4%;<jats:italic>P<\/jats:italic>\u00a0=\u00a0.02).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Significant associations existed between medical conditions (congenital heart disease, history of cardiac surgery, and hypoparathyroidism) and primary dentition DDE in children with 22q11.2 DS.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ipd.12755","type":"journal-article","created":{"date-parts":[[2020,11,22]],"date-time":"2020-11-22T04:41:04Z","timestamp":1606020064000},"page":"682-690","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Medical and dental characteristics of children with chromosome 22q11.2 deletion syndrome at the Royal Children's Hospital, Melbourne"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2673-9079","authenticated-orcid":false,"given":"Deborah H.","family":"Wong","sequence":"first","affiliation":[{"name":"Paediatric Dentistry Melbourne Dental School The University of Melbourne Melbourne Vic. Australia"},{"name":"The Royal Children's Hospital Melbourne Melbourne Vic. Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4576-4894","authenticated-orcid":false,"given":"Sadna","family":"Rajan","sequence":"additional","affiliation":[{"name":"Paediatric Dentistry Melbourne Dental School The University of Melbourne Melbourne Vic. Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kerrod B.","family":"Hallett","sequence":"additional","affiliation":[{"name":"The Royal Children's Hospital Melbourne Melbourne Vic. Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4570-0620","authenticated-orcid":false,"given":"David J.","family":"Manton","sequence":"additional","affiliation":[{"name":"Paediatric Dentistry Melbourne Dental School The University of Melbourne Melbourne Vic. Australia"},{"name":"The Royal Children's Hospital Melbourne Melbourne Vic. Australia"},{"name":"Centrum voor Tandheelkunde en Mondzorgkunde Universitair Medisch Centrum Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,2,2]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.03.007"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.34.10.798"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.35.9.789-a"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.79.4.348"},{"key":"e_1_2_9_6_1","first-page":"1","article-title":"All\u2010cause mortality and survival in adults with 22q11. 2 deletion syndrome","author":"Van L","year":"2019","journal-title":"Genet 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