Oral manifestations of Smith-Lemli-Opitz syndrome: a paediatric case report.
Eur J Paediatr Dent
; 9(4 Suppl): 19-22, 2008 Dec.
Article
en En
| MEDLINE
| ID: mdl-19886368
ABSTRACT
BACKGROUND:
The Smith-Lemli-Opitz Syndrome (SLOS) is an autosomal recessive genetic disorder, characterised by multiple congenital malformations, dysmorphic facial features and mental retardation. SLOS is caused by a genetically inherited deficiency of the enzyme 7-dehydrocholesterol delta7 reductase (7-DHC reductase), the catalyst involved in the final step of cholesterol biosynthesis, with the consequence of an increased serum levels of 7-DHC and generalised cholesterol deficiency. CASE REPORT A 5-year-old female child was referred to the Department of Paediatric Dentistry of the University of Palermo for caries, gingivitis and malocclusion. The medical history revealed the diagnosis of SLOS, confirmed by a biochemical analysis 5 weeks after birth. The child exhibited several dysmorphic craniofacial features, typical of SLOS. Dental treatment, including oral prophylaxis, was performed without sedation. Instructions on proper oral self-care and dental disease prevention were provided to the mother of the patient.CONCLUSION:
Suggestions regarding comprehensive dental care may be important to properly treat children with SLOS in the dental office.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Síndrome de Smith-Lemli-Opitz
/
Mordida Abierta
/
Caries Dental
/
Gingivitis
Tipo de estudio:
Observational_studies
/
Prognostic_studies
Límite:
Child, preschool
/
Female
/
Humans
Idioma:
En
Revista:
Eur J Paediatr Dent
Asunto de la revista:
ODONTOLOGIA
/
PEDIATRIA
Año:
2008
Tipo del documento:
Article
País de afiliación:
Italia