The oral phenotype and dental management in patients with maple syrup urine disease; case report and scoping review.
BMC Oral Health
; 24(1): 362, 2024 Mar 21.
Article
en En
| MEDLINE
| ID: mdl-38515181
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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Fenotipo
/
Enfermedad de la Orina de Jarabe de Arce
Límite:
Child, preschool
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Humans
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Male
Idioma:
En
Revista:
BMC Oral Health
Asunto de la revista:
ODONTOLOGIA
Año:
2024
Tipo del documento:
Article
País de afiliación:
Jordania