{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T08:11:39Z","timestamp":1792656699526,"version":"4.5.0"},"reference-count":45,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2010,11,16]],"date-time":"2010-11-16T00:00:00Z","timestamp":1289865600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dental Traumatology"],"published-print":{"date-parts":[[2010,12]]},"abstract":"<jats:p><jats:bold>Abstract\u2002\u2013\u2002<\/jats:bold> Lesch\u2013Nyhan syndrome (LNS) is an X\u2010linked disorder originating from deficiency of the enzyme hypoxanthine guanine phosphoribosyl transferase. It is characterized by neurological manifestations, including the dramatic symptom of compulsive self\u2010mutilation, which results in destruction of oral and perioral tissues. Several drug trials have been administered to improve the severe self\u2010destructive behaviour, with questionable effectiveness. Invasive treatment approaches, such as extraction of teeth and orthognathic surgery, have been suggested with variable success. A conservative treatment with an intraoral appliance serving to prevent oral and peri\u2010oral self\u2010injury is presented in this report. The patient was a 14\u2010year\u2010old boy demonstrating the typical LNS behaviour, including compulsive self\u2010biting, significant loss of lip and tongue tissue, spasticity and involuntary movements. An acrylic maxillary appliance was designed and constructed with an occlusal plate raising the bite. The appliance was retained by two Adams\u2019 clasps on the first premolars, along with three ball clasps between the incisors. Fabrication, insertion, and maintenance were uncomplicated and non\u2010stressful to the patient. Periodic recall over 3\u2010year period has confirmed the effective healing of the oral lesions and a high level of tolerance of the appliance.<\/jats:p>","DOI":"10.1111\/j.1600-9657.2010.00930.x","type":"journal-article","created":{"date-parts":[[2010,11,16]],"date-time":"2010-11-16T09:48:07Z","timestamp":1289900887000},"page":"496-500","source":"Crossref","is-referenced-by-count":26,"title":["Effective treatment of self\u2010injurious oral trauma in Lesch\u2013Nyhan syndrome: a case report"],"prefix":"10.1111","volume":"26","author":[{"given":"Aristidis","family":"Arhakis","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikolaos","family":"Topouzelis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleni","family":"Kotsiomiti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikolaos","family":"Kotsanos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,11,16]]},"reference":[{"key":"e_1_2_4_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(64)90104-4"},{"key":"e_1_2_4_3_2","doi-asserted-by":"publisher","DOI":"10.1093\/jpepsy\/1.3.51"},{"key":"e_1_2_4_4_2","first-page":"403","article-title":"Lesch\u2013Nyhan Syndrome: report of three cases in one family","volume":"45","author":"Shoptaw 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