{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T21:09:38Z","timestamp":1790456978895,"version":"4.1.0"},"reference-count":28,"publisher":"S. Karger AG","issue":"4","license":[{"start":{"date-parts":[[2018,11,28]],"date-time":"2018-11-28T00:00:00Z","timestamp":1543363200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2018,11,28]],"date-time":"2018-11-28T00:00:00Z","timestamp":1543363200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Respiration"],"published-print":{"date-parts":[[2019]]},"abstract":"<b><i>Background:<\/i><\/b> In Ehlers-Danlos syndrome (EDS), a group of monogenic disorders affecting connective tissues, obstructive sleep apnoea (OSA) is highly prevalent in adults. The prevalence of OSA in children with EDS is unknown. <b><i>Objectives:<\/i><\/b> This prospective cross-sectional study aimed at determining the prevalence of OSA in paediatric EDS patients. <b><i>Methods:<\/i><\/b> Children with EDS (<i>n<\/i> = 24) were recruited from the Children\u2019s Hospital Zurich and matched to healthy controls. Participants completed home respiratory polygraphy and questionnaires (Sleep-Related Breathing Disorder Scale [SRBD], Epworth Sleepiness Scale [ESS], and Child Health Questionnaire [CHQ]). The American Academy of Sleep Medicine criteria were applied for OSA diagnosis (obstructive apnoea-hypopnoea index [oAHI] \u22651\/h). Conditional logistic regression was used to compare the prevalence of OSA and to adjust for possible confounding. <b><i>Results:<\/i><\/b> OSA was found in 42% of paediatric EDS patients and in 13% of matched controls (OR = 4.5, 95% CI = 0.97\u201320.83, <i>p<\/i> = 0.054). The median oAHI was higher in EDS patients than in controls (0.77\/h, IQR = 0.19\u20131.76, vs. 0.24\/h, IQR = 0.0\u20130.60, <i>p<\/i> &#x3c; 0.001 adjusted for age, sex, and BMI <i>z<\/i>-score). EDS patients had lower scores in most CHQ scales and higher SRBD and ESS scores than controls (0.26, IQR = 0.1\u20130.35, vs. 0.07, IQR = 0\u20130.19, <i>p<\/i> = 0.004); 7 \u00b1 4 vs. 5 \u00b1 4, <i>p<\/i> = 0.033, respectively). <b><i>Conclusion:<\/i><\/b> OSA is a previously underestimated EDS-related complication increasing disease burden.<\/jats:p>","DOI":"10.1159\/000494328","type":"journal-article","created":{"date-parts":[[2018,11,28]],"date-time":"2018-11-28T22:01:24Z","timestamp":1543442484000},"page":"284-291","source":"Crossref","is-referenced-by-count":20,"title":["Obstructive Sleep Apnoea in Children and Adolescents with Ehlers-Danlos Syndrome"],"prefix":"10.1159","volume":"97","author":[{"given":"Anna\u00a0S.","family":"St\u00f6berl","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9017-6143","authenticated-orcid":false,"given":"Thomas","family":"Gaisl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilia","family":"Giunta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noriane\u00a0A.","family":"Sievi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3471-5664","authenticated-orcid":false,"given":"Florian","family":"Singer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"M\u00f6ller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne","family":"Rohrbach","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malcolm","family":"Kohler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2018,11,28]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31460"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2009.08.003"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2016-209560"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2004-0730"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.05.039"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2005.10.020"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201510-2099OC"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.157.4.9704080"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.3345\/kjp.2010.53.10.863"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1378\/chest.123.5.1561"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1177\/019459989110400415"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1378\/chest.119.5.1393"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2003.28"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.159.5.9809079"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-0174"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.5664\/jcsm.2172"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31538"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.5664\/jcsm.4284"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.5665\/sleep.4318"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1001\/archoto.2011.8"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1597\/10-100"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1016\/j.sleep.2016.10.001"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3181eed412"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1016\/j.anclin.2013.10.012"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31539"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejr.2012.12.002"},{"key":"ref27","doi-asserted-by":"publisher","DOI":"10.5665\/sleep.3392"},{"key":"ref28","doi-asserted-by":"publisher","DOI":"10.1007\/s11325-013-0924-2"}],"container-title":["Respiration"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/494328","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/494328","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T17:03:05Z","timestamp":1745427785000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000494328"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11,28]]},"references-count":28,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2019,4,24]]},"published-print":{"date-parts":[[2019,4,29]]}},"URL":"https:\/\/doi.org\/10.1159\/000494328","archive":["Portico"],"relation":{"is-supplemented-by":[{"id-type":"doi","id":"10.6084\/m9.figshare.7392299","asserted-by":"subject"}]},"ISSN":["0025-7931","1423-0356"],"issn-type":[{"value":"0025-7931","type":"print"},{"value":"1423-0356","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,11,28]]}}}