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Abstract：
OBJECTIVE: The literature about oral manifestations and dental management in maple syrup urine disease (MSUD) is sparse. 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
目的：关于枫糖浆尿病（MSUD）的口腔表现和牙齿管理的文献很少。本报告的目的是介绍一个新的MSUD病例，特别强调口头发现，并回顾相关文献。
方法：根据描述病例报告的CARE指南，描述了一例4岁男孩MSUD的病例报告。对相关文献进行了范围回顾，根据PRISMA-ScR指南,通过搜索PubMed,Medline,Embase,以及描述MSUD中牙科管理和/或口腔表现的文章的灰色文献。
结果：最初的搜索确定了219篇文章，但只有4人符合纳入标准。大量龋齿和牙菌斑引起的牙龈炎是MSUD的主要口腔发现。其他口腔发现包括釉质发育不全，骨骼异常,和异常的口头行为。疾病相关因素似乎在观察到的口腔表型的发展中起主要作用。
结论：MSUD的口腔健康似乎受到半合成饮食依赖和相关神经认知并发症的影响。量身定制的口腔健康促进干预措施应包括在MSUD患者的多学科管理中。
方法：根据描述病例报告的CARE指南，描述了一例4岁男孩MSUD的病例报告。对相关文献进行了范围回顾，根据PRISMA-ScR指南,通过搜索PubMed,Medline,Embase,以及描述MSUD中牙科管理和/或口腔表现的文章的灰色文献。
结果：最初的搜索确定了219篇文章，但只有4人符合纳入标准。大量龋齿和牙菌斑引起的牙龈炎是MSUD的主要口腔发现。其他口腔发现包括釉质发育不全，骨骼异常,和异常的口头行为。疾病相关因素似乎在观察到的口腔表型的发展中起主要作用。
结论：MSUD的口腔健康似乎受到半合成饮食依赖和相关神经认知并发症的影响。量身定制的口腔健康促进干预措施应包括在MSUD患者的多学科管理中。
