Síndrome de hipomineralización incisivo-molar / No disponible

No disponible

Immediate Desensitization in Teeth Affected by Amelogenesis Imperfecta

Abstract The aim of this paper was to describe a clinical case of immediate dental desensitization using a self-etch adhesive system in an adolescent patient diagnosed with amelogenesis imperfecta (AI). AI was associated with severe tooth sensitivity, treated by the application of a universal adhesive system for desensitization of the teeth affected by AI. Reduction of tooth sensitivity was assessed using a visual analog scale during all reevaluations. The technique was effective for reducing tooth sensitivity. It was concluded that the adhesive system for tooth desensitization had an immediate effect and maintained its effectiveness during a 12-month follow-up period.

Resumo O objetivo deste estudo foi descrever uma técnica de dessensibilização dental imediata, usando o adesivo universal em paciente adolescente diagnosticado com amelogênese imperfeita. O paciente afetado pela amelogênese imperfeita apresentava dentes extremamente sensíveis, e foi submetido a aplicações de adesivo universal para dessensibilizar os dentes afetados por amelogênese imperfeita. A redução da sensibilidade dentária foi avaliada pela escala analógica visual durante todas as reavaliações. A técnica foi efetiva na redução da sensibilidade dental. Concluiu-se que o uso do sistema adesivo foi eficaz na imediata dessensibilidazação dentária e mantida após doze meses de avaliação.

Immediate Desensitization in Teeth Affected by Amelogenesis Imperfecta.

The aim of this paper was to describe a clinical case of immediate dental desensitization using a self-etch adhesive system in an adolescent patient diagnosed with amelogenesis imperfecta (AI). AI was associated with severe tooth sensitivity, treated by the application of a universal adhesive system for desensitization of the teeth affected by AI. Reduction of tooth sensitivity was assessed using a visual analog scale during all reevaluations. The technique was effective for reducing tooth sensitivity. It was concluded that the adhesive system for tooth desensitization had an immediate effect and maintained its effectiveness during a 12-month follow-up period.

Endoplasmic reticulum stress in amelogenesis imperfecta and phenotypic rescue using 4-phenylbutyrate.

Inherited diseases caused by genetic mutations can arise due to loss of protein function. Alternatively, mutated proteins may mis-fold, impairing endoplasmic reticulum (ER) trafficking, causing ER stress and triggering the unfolded protein response (UPR). The UPR attempts to restore proteostasis but if unsuccessful drives affected cells towards apoptosis. Previously, we reported that in mice, the p.Tyr64His mutation in the enamel extracellular matrix (EEM) protein amelogenin disrupts the secretory pathway in the enamel-forming ameloblasts, resulting in eruption of malformed tooth enamel that phenocopies human amelogenesis imperfecta (AI). Defective amelogenin post-secretory self-assembly and processing within the developing EEM has been suggested to underlie the pathogenesis of X chromosome-linked AI. Here, we challenge this concept by showing that AI pathogenesis associated with the p.Tyr64His amelogenin mutation involves ameloblast apoptosis induced by ER stress. Furthermore, we show that 4-phenylbutyrate can rescue the enamel phenotype in affected female mice by promoting cell survival over apoptosis such that they are able to complete enamel formation despite the presence of the mutation, offering a potential therapeutic option for patients with this form of AI and emphasizing the importance of ER stress in the pathogenesis of this inherited conformational disease.

Hallazgos radiograficos e histologicos en pacientes odontologicos con terapia anticonvulsivante: informe de un caso / Radiographic and histologic findings in odontologic patients with anticonvulsivant therapy: report of a case

Enzimas hidroliticas como hexosaminasas, B-glucoronidasas, galactosidasas presentes en encias inflamadas e hiperplasicas producen alteraciones en el metabolismo celular, alterando el desarrollo adecuado de las raices dentarias, asi como alteraciones en le crecimiento gingival. Se estudia paciente masculino con 17 anos de edad, con cuadro clinico de retardo mental y crisis convulsivas, toma difenil hidantoina 100 mg. 3 dosis diarias, clinicamente el agrandamiento gingival no es el tipico en esta clase de patologia, se diagnostica amelogenesis imperfecta, radiograficamente se observan atipias morfologicas en las raices e histologicamente hay actividad proliferativa tipo hiperplasico en las papilas del conjutivo, vacuolizacion intracitoplasmatica e infiltrado inflamatorio. La severidad del cuadro clinico no esta asociada ni a la dosis ni al timepo de ingesta, es asi que la droga produce una interaccion entre droga-huesped y antigenos de la placa bacteriana. La alta cantidad de hidroxiprolina en el fluido gingival demuestra una actividad fibroblastica como la degradacion del colageno y el desarrollo dentario se vera afectado como se observa clinica y radiograficamente en este caso. Se necesitan estudios bioquimicos-bacteriologicos en pacientes que estan siendo sometidos a farmacoterapia con difenil hidantoina.