{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T17:09:19Z","timestamp":1773767359181,"version":"3.50.1"},"reference-count":42,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T00:00:00Z","timestamp":1659052800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100011666","name":"Ehlers-Danlos Society","doi-asserted-by":"publisher","award":["2019.02.PRO05"],"award-info":[{"award-number":["2019.02.PRO05"]}],"id":[{"id":"10.13039\/100011666","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Clinic Periodontology"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Periodontal Ehlers\u2010Danlos syndrome (pEDS) is a monogenic type of Ehlers\u2010Danlos syndrome characterized by periodontal destruction at a young age. The present study aimed to document the oral phenotype of pEDS based on prospective clinical investigations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and Methods<\/jats:title><jats:p>Thirty\u2010five adult individuals from 13 families with a clinically and genetically confirmed diagnosis of pEDS underwent a systematic oral assessment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Periodontitis stage 3 or 4 or edentulism due to periodontal destruction were diagnosed in 94% of the individuals. First permanent tooth loss was reported at the age of 21.5\u2009years (median; range 13\u201343\u2009years). Deep periodontal pockets were infrequent, with 94% measuring &lt;4\u2009mm. However, there was increased clinical attachment loss (CAL) averaging 8\u2009mm (range 4\u201313\u2009mm), and the probability of being edentate between the age of 35 and 44\u2009years was 28\u201347% compared with less than 0.25% of the general population. Radiographic anomalous findings were only found in a portion of subjects and consisted of fused roots of maxillary second molars (81%), root hypoplasia (57%), taurodontism (26%) and tooth rotation of premolars (67%). As such, radiographic findings are not considered common characteristics of pEDS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Characteristic oral traits of pEDS in adults are severe CAL with shallow probing depths and marked gingival recession. This is complemented by a lack of attached gingiva. These indications need to be paralleled by genetic analyses to diagnose pEDS unambiguously.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jcpe.13698","type":"journal-article","created":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T16:12:46Z","timestamp":1659111166000},"page":"1244-1252","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Oral characteristics in adult individuals with periodontal <scp>Ehlers\u2010Danlos<\/scp> syndrome"],"prefix":"10.1111","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3584-7650","authenticated-orcid":false,"given":"Ulrike","family":"Lepperdinger","sequence":"first","affiliation":[{"name":"Department of Operative and Restorative Dentistry Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chloe","family":"Angwin","sequence":"additional","affiliation":[{"name":"National Ehlers Danlos Syndrome Service London North West University Healthcare NHS Trust  London UK"},{"name":"Department of Metabolism, Digestion and Reproduction Section of Genetics and Genomics, Imperial College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8779-5157","authenticated-orcid":false,"given":"Di","family":"Milnes","sequence":"additional","affiliation":[{"name":"Genetic Health Queensland Royal Brisbane and Women's Hospital  Herston Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Glenda","family":"Sobey","sequence":"additional","affiliation":[{"name":"Ehlers Danlos Syndrome National Diagnostic Service Sheffield Children's Hospital  Sheffield UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Neeti","family":"Ghali","sequence":"additional","affiliation":[{"name":"National Ehlers Danlos Syndrome Service London North West University Healthcare NHS Trust  London UK"},{"name":"Department of Metabolism, Digestion and Reproduction Section of Genetics and Genomics, Imperial College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Diana","family":"Johnson","sequence":"additional","affiliation":[{"name":"Ehlers Danlos Syndrome National Diagnostic Service Sheffield Children's Hospital  Sheffield UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela F.","family":"Brady","sequence":"additional","affiliation":[{"name":"National Ehlers Danlos Syndrome Service London North West University Healthcare NHS Trust  London UK"},{"name":"Department of Metabolism, Digestion and Reproduction Section of Genetics and Genomics, Imperial College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tammy","family":"Kammin","sequence":"additional","affiliation":[{"name":"Ehlers Danlos Syndrome National Diagnostic Service Sheffield Children's Hospital  Sheffield UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica M.","family":"Bowen","sequence":"additional","affiliation":[{"name":"Ehlers Danlos Syndrome National Diagnostic Service Sheffield Children's Hospital  Sheffield UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rebekka","family":"Gr\u00f6bner","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8244-5200","authenticated-orcid":false,"given":"Pernilla","family":"Lundberg","sequence":"additional","affiliation":[{"name":"Department of Molecular Periodontology Ume\u00e5 University  Ume\u00e5 Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James","family":"Scott","sequence":"additional","affiliation":[{"name":"Academic Unit of Restorative Dentistry Sheffield University  Sheffield UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes","family":"Zschocke","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fleur S.","family":"van Dijk","sequence":"additional","affiliation":[{"name":"National Ehlers Danlos Syndrome Service London North West University Healthcare NHS Trust  London UK"},{"name":"Department of Metabolism, Digestion and Reproduction Section of Genetics and Genomics, Imperial College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6169-6812","authenticated-orcid":false,"given":"Ines","family":"Kapferer\u2010Seebacher","sequence":"additional","affiliation":[{"name":"Department of Operative and Restorative Dentistry Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,7,29]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.archoralbio.2012.12.008"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1080\/000163502753472014"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/10556656221086174"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.02962"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0104808"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/jcpe.12935"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.3290\/j.qi.a45263"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.13972"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjopen-2011-000705"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/ced.12911"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.02537"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chom.2011.10.006"},{"issue":"3","key":"e_1_2_12_14_1","first-page":"115","article-title":"Prevalence of short root anomaly in a group of Puerto Ricans","volume":"40","author":"Herrera C.","year":"2021","journal-title":"Puerto Rico Health Sciences Journal"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0722.1973.tb00600.x"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.oooo.2021.01.023"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.14134"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/jcpe.12807"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41436-020-00985-y"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2016.08.019"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10048-018-0560-x"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.5005\/jp-journals-10005-1986"},{"key":"e_1_2_12_23_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12903-017-0352-y"},{"issue":"1","key":"e_1_2_12_24_1","first-page":"7","article-title":"Periodontal risk assessment (PRA) for patients in supportive periodontal therapy (SPT)","volume":"1","author":"Lang N. P.","year":"2003","journal-title":"Oral Health & Preventive Dentistry"},{"key":"e_1_2_12_25_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1400569"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.matbio.2018.04.013"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-020-0194-9"},{"key":"e_1_2_12_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31552"},{"key":"e_1_2_12_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.joen.2018.12.016"},{"key":"e_1_2_12_30_1","volume-title":"Heritable disorders of connective tissue","author":"McKusick V. A.","year":"1972"},{"key":"e_1_2_12_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.16372"},{"key":"e_1_2_12_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-051X.1989.tb01609.x"},{"key":"e_1_2_12_33_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0714.1992.tb01031.x"},{"key":"e_1_2_12_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/ipd.12502"},{"key":"e_1_2_12_35_1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/469505"},{"key":"e_1_2_12_36_1","doi-asserted-by":"publisher","DOI":"10.5734\/JGM.2019.16.1.43"},{"issue":"3","key":"e_1_2_12_37_1","first-page":"85","article-title":"A new variant of Ehlers\u2010Danlos syndrome: An autosomal dominant disorder of fragile skin, abnormal scarring, and generalized periodontitis","volume":"13","author":"Stewart R. E.","year":"1977","journal-title":"Birth Defects Original Article Series"},{"key":"e_1_2_12_38_1","doi-asserted-by":"publisher","DOI":"10.3390\/biom11020149"},{"key":"e_1_2_12_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/jper.10239"},{"key":"e_1_2_12_40_1","doi-asserted-by":"publisher","DOI":"10.1126\/scisignal.2000697"},{"key":"e_1_2_12_41_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12903-016-0266-0"},{"key":"e_1_2_12_42_1","doi-asserted-by":"publisher","DOI":"10.1111\/jcpe.12988"},{"key":"e_1_2_12_43_1","doi-asserted-by":"publisher","DOI":"10.1111\/odi.13266"}],"container-title":["Journal of Clinical Periodontology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jcpe.13698","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/jcpe.13698","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jcpe.13698","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,21]],"date-time":"2025-04-21T01:01:04Z","timestamp":1745197264000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jcpe.13698"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,29]]},"references-count":42,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2022,12]]}},"alternative-id":["10.1111\/jcpe.13698"],"URL":"https:\/\/doi.org\/10.1111\/jcpe.13698","archive":["Portico"],"relation":{},"ISSN":["0303-6979","1600-051X"],"issn-type":[{"value":"0303-6979","type":"print"},{"value":"1600-051X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,7,29]]},"assertion":[{"value":"2022-01-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-07-06","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-07-29","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}