{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T04:58:44Z","timestamp":1785473924042,"version":"3.56.0"},"reference-count":24,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T00:00:00Z","timestamp":1785283200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background\/Objectives: Self-injurious behavior (SIB) is one of the most disabling manifestations of Lesch\u2013Nyhan disease (LND), frequently involving the oral cavity and severely affecting patients\u2019 quality of life and caregiver burden. Evidence regarding the impact of dental interventions on patient- and caregiver-reported outcomes remains limited. This study investigated the characteristics of SIB in patients with LND and explored the perceived effectiveness, tolerability, and psychosocial impact of intraoral devices and dental extractions. Methods: A questionnaire-based observational study was conducted among patients with LND. A purpose-built 32-item questionnaire, developed by dentists with expertise in Special Care Dentistry at Mauriziano Umberto I Hospital (Turin, Italy), was administered to patients attending the Pediatric Dentistry and Orthodontics Unit at IRCCS Istituto Giannina Gaslini (Genoa, Italy). The questionnaire assessed demographic and clinical characteristics, SIB features, dental management strategies, treatment-related complications, and patient- and caregiver-reported outcomes. Results: Twenty-four questionnaires were analyzed. SIB was reported in 21 patients (87.5%). Fingers (80%) and lips (70%) were the most frequently affected sites, with multiple anatomical sites involved in 80% of patients. Intraoral devices were used in 14 patients (66.7%), whereas dental extractions were performed in 4 (19%). Among patients treated with intraoral devices, 78.6% reported increased reassurance and protection. Device-related complications occurred in three patients and were mainly periodontal. All patients and caregivers reported relief following dental extraction, although SIB persisted in 50% of cases. Conclusions: SIB is highly prevalent among individuals with LND and commonly affects multiple anatomical sites. Intraoral devices are the most frequently adopted treatment strategy and are generally well tolerated, whereas dental extractions were associated with substantial perceived relief but do not necessarily eliminate SIB. Patient- and caregiver-reported outcomes should be considered when evaluating treatment effectiveness.<\/jats:p>","DOI":"10.3390\/children13081000","type":"journal-article","created":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T08:16:52Z","timestamp":1785313012000},"page":"1000","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Dental Management of Self-Injurious Behavior in Lesch\u2013Nyhan Disease: A Patient- and Caregiver-Reported Outcome Study"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9745-0750","authenticated-orcid":false,"given":"Claudia","family":"Capurro","sequence":"first","affiliation":[{"name":"Pediatric Dentistry and Orthodontics Unit, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9193-1622","authenticated-orcid":false,"given":"Stefano","family":"Parodi","sequence":"additional","affiliation":[{"name":"Epidemiology and Biostatistics Unit, Scientific Directorate, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6751-4397","authenticated-orcid":false,"given":"Simone","family":"Buttiglieri","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Mauriziano Umberto I Hospital, 10128 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-4825-5143","authenticated-orcid":false,"given":"Giulia","family":"Romanelli","sequence":"additional","affiliation":[{"name":"Pediatric Dentistry and Orthodontics Unit, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caterina","family":"Del Buono","sequence":"additional","affiliation":[{"name":"Pediatric Dentistry and Orthodontics Unit, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1294-2930","authenticated-orcid":false,"given":"Nicola","family":"Laffi","sequence":"additional","affiliation":[{"name":"Pediatric Dentistry and Orthodontics Unit, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,7,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1186\/1750-1172-2-48","article-title":"Hypoxanthine-guanine phosophoribosyltransferase (HPRT) deficiency: Lesch-Nyhan syndrome","volume":"2","author":"Torres","year":"2007","journal-title":"Orphanet J. 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