NDLI: The oral phenotype and dental management in patients with maple syrup urine disease; case report and scoping review

Content Provider	Springer Nature : BioMed Central
Author	Hassona, Yazan Alqaisi, Dua’a Flaifl, Yara Alkilani, Asma
Abstract	Background and objectives 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. Method 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. Conclusion 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.
Related Links	https://bmcoralhealth.biomedcentral.com/counter/pdf/10.1186/s12903-024-04135-7.pdf
Ending Page	8
Page Count	8
Starting Page	1
File Format	HTM / HTML
ISSN	14726831
DOI	10.1186/s12903-024-04135-7
Journal	BMC Oral Health
Issue Number	1
Volume Number	24
Language	English
Publisher	BioMed Central
Publisher Date	2024-03-21
Access Restriction	Open
Subject Keyword	Dentistry Oral and Maxillofacial Surgery Maple syrup Branched chain amino acids Ketonuria Oral Anesthesia
Content Type	Text
Resource Type	Case study
Subject	Dentistry
Journal Impact Factor	2.6/2023
5-Year Journal Impact Factor	3.2/2023

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1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
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