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Using matched comparators as a reference, the overall hazard ratio (HR) for death was 1.33 (95% confidence interval [CI] 0.70\u20132.55) in ED patients, and particularly high in males with hypohidrosis (HR 3.77, 95% CI 1.57\u20139.03) and individuals diagnosed before age 18 (HR 6.53, 95% CI 1.84\u201323.13). ED was associated with an increased risk of hospital-diagnosed infections before (odds ratio [OR] 2.27, 95% CI 1.81\u20132.85) and after (HR 2.06, 95% CI 1.74\u20132.45) diagnosis, varying across subtypes. Sensitivity analyses supported these findings, e.g., using antimicrobial prescriptions to identify infections. An association between hypohidrosis and previous hyperthermia (OR 7.11, 95% CI 3.26\u201315.51) diminished after diagnosis (HR 1.38, 95% CI 0.54\u20133.53). This study found an increased mortality risk in males with hypohidrosis and those diagnosed in childhood, and infection and hyperthermia risks depending on ED subtype. These data enhance understanding of ED\u2019s clinical course, informing patient management and counselling.<\/jats:p>","DOI":"10.2340\/actadv.v105.43101","type":"journal-article","created":{"date-parts":[[2025,6,18]],"date-time":"2025-06-18T12:28:59Z","timestamp":1750249739000},"page":"adv43101","source":"Crossref","is-referenced-by-count":0,"title":["Risk of Death, Infections, and Hyperthermia in Ectodermal Dysplasias: A Nationwide Study"],"prefix":"10.2340","volume":"105","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4852-0297","authenticated-orcid":false,"given":"Laura Krogh","family":"Herlin","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5527-0698","authenticated-orcid":false,"given":"Sigr\u00fan A.J.","family":"Schmidt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1853-9704","authenticated-orcid":false,"given":"Trine H.","family":"Mogensen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6310-4208","authenticated-orcid":false,"given":"Mette","family":"Sommerlund","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1183","published-online":{"date-parts":[[2025,6,18]]},"reference":[{"key":"115398","doi-asserted-by":"crossref","unstructured":"Wright JT, Fete M, Schneider H, Zinser M, Koster MI, Clarke AJ, et al. 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