RESUMEN
Background: the Cornelia de Lange Syndrome (CDLs) is a rare and complex syndrome characterized, basically, by psychomotor retardation associated with a number of congenital malformations. Aims: this paper reports the case of an 11-year-old female child diagnosed with Cornelia de Lange Syndrome (CdLS) and her successful dental management. Case report: the patient had severe mental retardation, definite negative behavior and the clinical findings included oral and physical changes. The patient's oral hygiene was deficient with the presence of calculus and gingivitis, besides several active caries lesions in permanent and deciduous dental elements. The treatment consisted in guidance for caregivers about oral hygiene and diet, and the dental procedures were performed under general anesthesia. Currently, the patient is accompanied by monthly follow-ups. Conclusions: the lack of knowledge about oral hygiene and cariogenic diets was identified as a one of the reasons for the oral diseases present. Due to the need to care for the other more serious and complex health problems, the oral diseases had evolved faster than usual and thus were difficult to treat and maintain thereafter. Under such conditions, the dentist plays a key role within a multidisciplinary team. From the guidance and knowledge provided in the dental clinic, there was a significant improvement in the life quality of the child and her family
Asunto(s)
Humanos , Femenino , Niño , Síndrome de Cornelia de Lange , Trastornos MentalesRESUMEN
Bone morphogenetic proteins (BMPs) play an important role during the initial process of enamel development and therefore may play a role in caries susceptibility. The purpose of this study was to evaluate the association between the polymorphisms in the BMP2, BMP4 and BMP7 genes and their association with caries experience and primary enamel microhardness characteristics. DNA from buccal cells as well as clinical and demographic information from 1,731 subjects from three different data sets from Brazil were included. Polymorphisms in BMP2, BMP4 and BMP7 were analyzed by real-time polymerase chain reaction from genomic DNA. Association between caries experience, genotype, and allele distribution in both cohorts was evaluated using χ(2) and logistic regression analyses. In the family-based set, the association between caries experience and alleles was tested using the transmission disequilibrium test. In the Rio de Janeiro cohort, microhardness data on 108 exfoliated primary teeth before and after demineralization and remineralization challenges was included. Associations between microhardness values and genotype and allele distribution were evaluated using χ(2) and logistic regression analyses. Differences between caries experience and some risk factors were statistically significant. In the cohort from Nova Friburgo, BMP2 was associated with caries experience in primary dentition during logistic regression analysis (p = 0.023; OR = 2.58; 95% CI 1.13-5.86). There was no association between genotype and allele distribution for BMP polymorphisms and primary enamel microhardness alterations. Our result suggests that BMP2 may be involved in caries experience in primary dentition from a Nova Friburgo cohort.
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Proteína Morfogenética Ósea 2/genética , Índice CPO , Caries Dental/enzimología , Polimorfismo Genético/genética , Diente Primario/enzimología , Adolescente , Proteína Morfogenética Ósea 4/genética , Proteína Morfogenética Ósea 7/genética , Brasil , Niño , Preescolar , Estudios de Cohortes , Caries Dental/genética , Dispositivos para el Autocuidado Bucal/estadística & datos numéricos , Esmalte Dental/anatomía & histología , Conducta Alimentaria , Femenino , Frecuencia de los Genes/genética , Variación Genética/genética , Genotipo , Dureza , Humanos , Lactante , Desequilibrio de Ligamiento/genética , Masculino , Polimorfismo de Nucleótido Simple/genética , Remineralización Dental , Cepillado Dental/estadística & datos numéricos , Adulto JovenRESUMEN
PURPOSE: The purpose of this study was to assess if children with clefts have an increased caries experience. METHODS: Caries data was collected via clinical examination of 115 4- to 21-year-olds with clefts and 230 controls. Cleft type was confirmed through their medical records and fluoride exposure history; oral hygiene habits and dietary history were obtained though a questionnaire. RESULTS: The adherence to the preventive oral health habits (tooth-brushing, use of fluoride, and dietary factors) were not different among groups excluding the use of dental floss. The mean DMFT was 1.20 (±1.8 SD) for the cleft group and 0.90 (±1.8) for the control group. There was no significant difference in the DMFT scores between children with clefts and the control group (P=.16). The mean dmft was 1.68 (±2.1) for the cleft group and 2.61 (±2.9) for the control group. The prevalence of dental caries in primary teeth was significantly lower in the cleft-affected children vs the control group (P=.02). The percentage of caries-free individuals was similar in cleft and control groups (P=.90), and was also similar in different cleft types (P=.67). CONCLUSION: Caries experience in children born with clefts is not higher in comparison to control children.
