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The aim of this report is to present a new case of MSUD with special emphasis on oral findings and to review the relevant literature.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>A case report of a 4-year-old boy with MSUD was described according to the CARE guidelines for describing case reports. Scoping review of relevant literature was performed, according to the PRISMA-ScR guidelines, by searching PubMed, Medline, Embase, and the grey literature for articles describing dental management and\/or oral manifestations in MSUD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The initial search identified 219 articles, but only 4 met the inclusion criteria. Rampant caries and plaque induced gingivitis were the main oro-dental findings in MSUD. Other oral findings included enamel hypoplasia, skeletal abnormalities, and abnormal oral behaviors. Disease-related factors appeared to play a major role in the development of the observed oral phenotype.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Oral health in MSUD seems to be influenced by the reliance on semi-synthetic diet and associated neurocognitive complications. Tailored oral health promotional interventions should be included in the multidisciplinary management of patients with MSUD.<\/jats:p><\/jats:sec>","DOI":"10.1186\/s12903-024-04135-7","type":"journal-article","created":{"date-parts":[[2024,3,21]],"date-time":"2024-03-21T10:02:02Z","timestamp":1711015322000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["The oral phenotype and dental management in patients with maple syrup urine disease; case report and scoping review"],"prefix":"10.1186","volume":"24","author":[{"given":"Yazan","family":"Hassona","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dua\u2019a","family":"Alqaisi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yara","family":"Flaifl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asma","family":"Alkilani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,3,21]]},"reference":[{"key":"4135_CR1","unstructured":"Strauss KA, Puffenberger EG, Carson VJ, Maple Syrup Urine Disease. 2006 Jan 30 [updated 2020 Apr 23]. 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