Dentinogenesis imperfecta type 1-Shield’s classification (1973): a rare case report and literature review

Dentinogenesis imperfecta (DI) is an autosomal dominant disorder that affects the dentin development. It is characterized by the presence of opalescent dentin, with dusky blue to brownish discoloration of the teeth. This condition is genetically and clinically heterogeneous; DI may affect only the teeth or it may be associated with the osteogenesis imperfect (OI). When both are associated there are various degrees of clinical presentation from mild form with less significant signs to severe form such as death in infancy. The most familiar classification system of this hereditary disorder is Shields classification which is of 3 types namely, type I, DI associated with OI, type II is not associated with OI. Type III which is also known as ‘Brandywine type’ DI. This case report describes a rare case of 32-year-old female patient who showed the characteristic features of Shield’s classification type I with penetrance of defect through 4 generations.

Tratamiento integral de una adolescente con dentinogénesis imperfecta tipo I / Comprehensive treatment of an adolescent with type I dentinogenesis imperfecta

La dentinogénesis imperfecta (DI) es un desorden hereditario de carácter autosómico dominante, que se origina durante la etapa de histodiferenciación en el desarrollo dental y altera la formación de la denti-na. Se considera una displasia dentinaria que puede afectar ambas denticiones con una incidencia de 1 en 6000 a 8000 nacimientos. El tratamiento del pa-ciente con DI es complejo y multidisciplinario, supone un desafío para el odontólogo, ya que por lo general están involucradas todas las piezas dentarias y afec-ta no solo la salud buco dental sino el aspecto emo-cional y psicológico de los pacientes. Objetivo: des-cribir el tratamiento integral y rehabilitador realiza-do en una paciente adolescente con diagnóstico de DI tipo I. Relato del caso: Paciente de sexo femenino de 14 años, que concurrió en demanda de atención a la Cátedra de Odontología Integral Niños de la FOU-BA derivada del Hospital "Prof. Dr. Juan P. Garrahan" con diagnóstico de osteogénesis imperfecta tipo III (OI). Nunca recibió atención odontológica y el motivo de consulta fue la apariencia estética de sus piezas dentarias. Se realizó el examen clínico y radiográfico arrojando el diagnóstico de DI tipo I asociada a OI. Conclusión: El tratamiento rehabilitador de la DI tipo I en los pacientes en crecimiento y desarrollo debe estar dirigido a intervenir de manera integral y tem-prana para resolver la apariencia estética y funcio-nal, evitar las repercusiones sociales y emocionales y acompañar a los pacientes y sus familias (AU)

Dentinogenesis imperfecta (DI) is an autosomal dominant inherited disorder that originates during the histodifferentiation stage of tooth development and alters dentin formation. It is considered a den-tin dysplasia that can affect both dentitions with an incidence of 1 in 6000 to 8000 births. The treatment of patients with DI is complex and multidisciplinary, it is a challenge for the dentist, since in general all the teeth are involved and it affects not only oral health but also the emotional and psychological aspect of the patients. Objective: To describe the comprehen-sive and rehabilitative treatment carried out in an adolescent patient with a diagnosis of DI type I. Case report: A 14-year-old female patient, who required dental attention at the Department of Pediatric Den-tistry of FOUBA and was referred from the Hospital "Prof. Dr. Juan P. Garrahan" with a diagnosis of os-teogenesis imperfecta type III (OI). The patient never received dental care and the reason for consultation was esthetic appearance of her teeth. A clinical and radiographic examination was performed, resulting in a diagnosis of DI type I associated with OI. Conclu-sion: Rehabilitative treatment of DI in growing and developing patients will be aimed at early and com-prehensive intervention to resolve esthetic and func-tional appearance, avoid social and emotional reper-cussions and accompany patients and their families (AU)

Comparative evaluation of the effects of three hydraulic calcium silicate cements on odontoblastic differentiation of human dental pulp stem cells: an in vitro study

Abstract Objective The study aimed to compare the response of human dental pulp stem cells (hDPSCs) towards three hydraulic calcium silicate cements (HCSCs) by measuring cytotoxicity and expression of dentinogenic genes. Methodology Dental pulps of five impacted mandibular third molars were extirpated as a source for hDPSCs. Next to culturing, hDPSCs were subjected to fluorescence-activated cell sorting after the third passage to validate stemness of the cells. Human DPSCs were exposed to diluted supernatants of OrthoMTA (OMTA), Biodentine (BD) and Calcium-Enriched Mixture (CEM) at concentrations 10, 25, 50 and 100% at the first, third and fifth day of culture. Then, cells were exposed to 10% concentrations supernatant of HCSCs to determine DSPP and DMP1 gene expression, using a quantitative polymerase-chain reaction. Data were analyzed using one-way and three-way ANOVA, followed by Tukey post hoc statistical tests. Results Optimal cell proliferation was observed in all groups, regardless of concentration and time-point. HCSC supernatants were non-cytotoxic to hDPSCs at all three time-points, except for 100% Biodentine on day five. On day seven, OMTA group significantly upregulated the expression of DSPP and DMP1 genes. On day 14, expression of DMP1 and DSPP genes were significantly higher in BD and OMTA groups, respectively. Conclusion Biodentine significantly upregulated DMP1 gene expression over 14 days, whereas CEM was associated with only minimal expression of DSPP and DMP1 .

Caracterización microscópica de la dentina de dientes temporales / Microscopic characterization of the dentin of temporary teeth

La dentina se compone de un mineral de fosfato de calcio identificado como dahllita, que se dispone en pequeños cristales de hidroxiapatita carbonatada con dimensiones de 36 × 25 × 4 nm, y por una fase orgánica cuyo principal componente es el colágeno tipo 1 en 90%, que se orienta en forma de malla. Esta conformación corresponde a los dientes permanentes. Dentro de las estructuras, encontramos túbulos dentinarios que miden, aproximadamente, entre 0.5-1 µm de diámetro en la periferia y hasta 3-5 µm cerca de la pulpa. En el presente estudio, realizado en dentina de dientes temporales, el lumen de dichos túbulos es más grande cuando se encuentra cerca de la pulpa dental. Asimismo, se encontraron cambios elementales importantes de acuerdo con las diferentes profundidades en las que se observó, encontrando un aumento en el peso porcentual de carbono cuando se encuentra a mayor profundidad, lo que indica una composición orgánica mayor en la dentina pulpar. En estudios de dientes permanentes esta composición es disminuida y con mayor concentración en la dentina cercana a la unión amelodentinaria. En dentina de dientes temporales se encontraron diferencias en el recuento de túbulos dentinarios por mm2, comparado a la dentina de dientes permanentes, donde el número de túbulos no varía mucho (AU)

Dentin is composed of a calcium phosphate mineral identified as dahllite, which is arranged in small crystals of carbonated hydroxyapatite with dimensions of 36 × 25 × 4 nm, and by an organic phase whose main component is type l collagen in 90%, which is oriented in the form of a mesh. This conformation corresponds to permanent teeth. Within the structures, we find dentin tubules that measure approximately 0.5-1 µm in diameter at the periphery and up to 3-5 µm near the pulp. In the present study, carried out in dentin of primary teeth, the lumen of these tubules is larger when it is close to the dental pulp. Likewise, important elemental changes were found according to the different depths in which it was observed, finding an increase in the percentage weight of carbon when it is at a greater depth, indicating a greater organic composition in the pulp dentin. In studies of permanent teeth, this composition is decreased and with a higher concentration in the dentin near the amelodentinal junction. In dentin of primary teeth, differences were found in the count of dentin tubules per mm2, compared to dentin of permanent teeth, where the number of tubules did not vary much (AU)

Tratamiento integral y control a 12 años de paciente con Dentinogénesis Imperfecta tipo I / Manejo clínico e seguimento de 12 anos em paciente com Dentinogênese Imperfeita tipo I / Clinical management and 12 years follow up in a patient with Dentinogenesis Imperfecta type I

La Dentinogénesis Imperfecta es una anomalía dentaria determinada genéticamente y caracterizada clínicamente por una apariencia ámbar opalescente de la dentina. Se presenta la resolución clínica, con seguimiento y control a 12 años de un paciente de 3 años de edad al momento de la consulta, con diagnóstico de Dentinogénesis Imperfecta tipo I asociada a Osteogénesis Imperfecta tipo IB. La identificación temprana de esta entidad y el tratamiento oportuno y multidisciplinario, contribuyen a mejorar el pronóstico de la misma.

Dentinogênese Imperfeita é uma anormalidade dentária geneticamente determinada, caracterizada clinicamente pela aparência opalescente e translúcida da dentina. Manejo clínico e seguimento de 12 anos são relatados, em um paciente de 3 anos com Dentinogênese Imperfeita tipo I associado à Osteogenesis Imperfecta tipo IB. O diagnóstico precoce.

Dentinogenesis Imperfecta is a geneti-cally determinated dental abnormality, characterized clinically by opalescent and translucent appearance of the den-tin. Clinical management and a 12 years follow up are reported, in a 3 years old patient with Dentinogenesis Imperfecta type I associated with Osteogenesis Im-perfecta type IB. The earlier diagnosis and the opportune and multidisciplinary treatment, led to improve the prognosis.

Dentinogénesis imperfecta tipo II: Reporte de caso de dos pacientes de un mismo grupo familiar / Dentinogenesis imperfecta type II: Case report of two patients from the same family group

Resumen Introducción: La dentinogenesis imperfecta (DI) se ha definido como una alteración hereditaria de carácter autosómico dominante, que se origina durante la etapa de histodiferenciación en el desarrollo dental, es un tipo de displasia del tejido dentinario que afecta la estructura de la dentina de una o ambas denticiones. Las complicaciones de la DI tiene un fuerte impacto en la calidad de vida de los pacientes ya que la parte funcional, estética y fonética se suelen encontrar afectadas y representan un reto importante para el tratante. El diagnóstico precoz y el tratamiento adecuado son necesarios para lograr mejores resultados funcionales y estéticos, minimizar las deficiencias nutricionales y trastornos psicosociales, permitiendo así mejorarla calidad de vida de la persona. Objetivo: Determinar el tipo de dentinogenesis, la relación familiar, y las características clínicas de cada paciente. Métodos: Acuden a la clínica de Odontología de la Universidad Católica de Cuenca, dos Hermanos procedentes de la ciudad de Cuenca-Ecuador, de las edades de 5 y 6 años respectivamente, por presentar múltiples lesiones cariosas, se puede evidencia destrucción generalizada del remanente coronario y pérdida prematura de las piezas dentales. Después de realizar el diagnóstico clínico y radiográfico, historia familiar, se estableció el diagnóstico de dentinogenesis imperfecta tipo II. Conclusión: es de gran importancia el diagnóstico oportuno y temprano de la dentinogenesis imperfecta para un tratamiento adecuado, debido a que la DI, afecto de mayormente a la dentición temporal, es fundamental las visitas al odontólogo, ya que este podrá diagnosticar tempranamente la patología y evitar grandes daños.

Abstract Introduction: Dentinogenesis imperfecta (DI) has been defined as a hereditary alteration of autosomal dominant character, which originates during the stage of histodifferentiation in dental development, is a type of dysplasia of dentinal tissue that affects the dentine structure of one or both dentitions. The complications of ID have a strong impact on the quality of life of patients since the functional, aesthetic and phonetic part are usually affected and represent an important challenge for the trafficker. Early diagnosis and adequate treatment are necessary to achieve better functional and aesthetic results, minimize nutritional deficiencies and psychosocial disorders, thus improving the quality of life of the person. Objective: To determine the type of dentinogenesis, the family relationship, and the clinical characteristics of each patient. Methods: Two brothers coming from the city of Cuenca-Ecuador, ages 5 and 6 years old, come to the Odontology clinic of the Catholic University of Cuenca, respectively, for presenting multiple carious lesions. Generalized destruction of the coronary remnant and premature loss of teeth. After performing the clinical and radiographic diagnosis, family history, the diagnosis of dentinogenesis imperfecta type II was established. Conclusion: the timely and early diagnosis of dentinogenesisimperfecta is of great importance for an adequate treatment, because the ID, affected mainly by the primary dentition, is fundamental visits to the dentist, since this will be able to diagnose the pathology at an early stage. Avoid big damages.

Dentinogénesis imperfecta tipo II. Manejo estomatológico / Dentinogenesis imperfecta-type II dentistry management

RESUMEN Introducción: la dentinogénesis imperfecta (DI) es una alteración hereditaria que se origina en la etapa de histodiferenciación durante la odontogénesis, y constituye una forma de displasia mesodérmica localizada, caracterizada por una expresa alteración de las proteínas dentinarias. Presentación del caso: paciente masculino de 6 años de edad, que acude a consulta de la Clínica Docente "Antonio Briones Montoto" en diciembre de 2019, remitido por el servicio de genética. Se estableció el diagnóstico de dentinogénesis imperfecta tipo II de carácter autosómico dominante sin predilección de sexo. Se rehabilitaron los dientes temporales con coronas metálicas, para evitar una mayor abrasión de los dientes, devolver la función masticatoria al paciente y restituir la dimensión vertical perdida. Se aplicó terapia Láser con flúor en primeros molares permanentes presentes e incisivos centrales inferiores para aumentar la resistencia del esmalte. Conclusiones: la dentinogénesis imperfecta tipo II afecta la primera dentición y puede aparecer en la permanente; con base en la valoración genética, características clínicas y radiográficas de esta alteración, resulta necesario establecer un correcto diagnóstico para comenzar el tratamiento más oportuno y seguir la evolución del paciente.

ABSTRACT Introduction: Dentinogenesis Imperfecta (DI) is a hereditary alteration that originates in the stage of histodifferentiation during odontogenesis; it represents a type of localized mesodermal dysplasia, characterized by an expressed alteration of dentin proteins. Case Report: a 6-year-old-male patient came to Antonio Briones Montoto Teaching Dentistry Clinic on December 2019; he was referred by genetics service. The diagnosis of dentinogenesis imperfecta-type II was established, of autosomal dominant character without sex predominance. Temporary teeth were rehabilitated with metal crowns, to avoid further abrasion of the teeth, performing a restoration of the masticatory function and replacement of the lost vertical dimension. Laser therapy with fluoride was applied to the present first permanent molars and lower central incisors to increase the resistance of the enamel. Conclusions: imperfect dentinogenesis type II affects the first dentition and may appear in the permanent one; based on genetic evaluation, clinical and radiological characteristics of this alteration, it is necessary to establish a precise diagnosis in order to start a timely treatment and follow-up of the patient.

Diagnóstico diferencial e conduta clínica de um cisto folicular inflamatório mandibular / Differential diagnosis and management of inflammatory follicular cyst in mandibular anterior area

Este artigo descreve a dificuldade em diagnosticar um cisto folicular inflamatório na área anterior da mandíbula em um menino com dentinogênese imperfeita tipo I (DI-1). Um menino de 6 anos de idade, com DI-1, procurou tratamento devido ao comprometimento estético. O exame radiográfico revelou uma lesão periapical envolvendo os dentes decíduos incisivo central e lateral direitos. Esses dentes foram extraídos sem intercorrências. Após três meses, a criança se queixou de dor em um edema de cor azulada na mesma área. O diagnóstico diferencial foi de cisto folicular e a lesão foi acompanhada. Como os sinais e sintomas persistiram, o tratamento de escolha foi a descompressão da lesão, seguido por irrigação abundante e curetagem das paredes da lesão. O acompanhamento clínico e radiográfico, mostrou, após 6 meses, remissão da lesão, reparo ósseo, e erupção ativa dos incisivos permanentes. As características incomuns deste caso, fizeram com que o diagnóstico de cisto folicular inflamatório fosse dificultado.

This article reports the difficulties in diagnosing an inflammatory follicular cyst in the mandibular anterior area of a boy with type 1 dentinogenesis imperfecta (DI-1). A 6-year-old boy, with DI-1, sought treatment due to esthetic complaints. The radiographic examination revealed a periapical lesion involving the right primary central and lateral incisors. These teeth were extracted with no complications. After three months, the boy complained of pain in a blue-black edema in the same area. The differential diagnosis was of follicular cyst and the lesion was followed-up. As the signs and symptoms persisted, the treatment of choice was to decompress the lesion, followed by copious irrigation, and lesion's wall curettage. After six months, the clinical and radiographic follow-up showed lesion remission, bone repair, and active eruption of permanent incisors. The uncommon characteristics of the case make the diagnosis of inflammatory follicular cyst difficult.

Evaluation of dentinogenesis inducer biomaterials: an in vivo study

Abstract When exposure of the pulp to external environment occurs, reparative dentinogenesis can be induced by direct pulp capping to maintain pulp tissue vitality and function. These clinical situations require the use of materials that induce dentin repair and, subsequently, formation of a mineralized tissue. Objective: This work aims to assess the effect of tricalcium silicate cements and mineral trioxide aggregate cements, including repairing dentin formation and inflammatory reactions over time after pulp exposure in Wistar rats. Methodology: These two biomaterials were compared with positive control groups (open cavity with pulp tissue exposure) and negative control groups (no intervention). The evaluations were performed in three stages; three, seven and twenty-one days, and consisted of an imaging (nuclear medicine) and histological evaluation (H&E staining, immunohistochemistry and Alizarin Red S). Results: The therapeutic effect of these biomaterials was confirmed. Nuclear medicine evaluation demonstrated that the uptake of 99mTc-Hydroxymethylene diphosphonate (HMDP) showed no significant differences between the different experimental groups and the control, revealing the non-occurrence of differences in the phosphocalcium metabolism. The histological study demonstrated that in mineral trioxide aggregate therapies, the presence of moderate inflammatory infiltration was found after three days, decreasing during follow-ups. The formation of mineralized tissue was only verified at 21 days of follow-up. The tricalcium silicate therapies demonstrated the presence of a slight inflammatory infiltration on the third day, increasing throughout the follow-up. The formation of mineralized tissue was observed in the seventh follow-up day, increasing over time. Conclusions: The mineral trioxide aggregate (WhiteProRoot®MTA) and tricalcium silicate (Biodentine™) present slight and reversible inflammatory signs in the pulp tissue, with the formation of mineralized tissue. However, the exacerbated induction of mineralized tissue formation with the tricalcium silicate biomaterial may lead to the formation of pulp calcifications

A histological continuum between dentinogenesis imperfecta and dentin dysplasia: A case report with literature review

Dentinogenesis Imperfecta and dentin dysplasia are genetic oral diseases inherited in a simple autosomal dominant mode, with high penetrance and a low mutation rate. Both of them are present with bulbous crowns, marked cervical constrictions, severe attritions, few periapical radiolucencies, and premature tooth loss. The diagnosis is based on family history, and detailed clinical examination, while genetic diagnosis may become useful in the future once sufficient disease-causing mutations have been discovered. Here, we present a case with overlapping features of both dentinogenesis imperfecta and dentin dysplasia asserting both the anomalies to be part of the same continuum of the genetic event.

Má oclusão em crianças e adolescentes com osteogênese imperfeita / Malocclusion in children and adolescents with Osteogenesis Imperfecta

A Osteogênese Imperfeita (OI) é uma doença genética que afeta o tecido conjuntivo e é causada por defeitos na síntese de colágeno tipo I. As alterações na produção do colágeno podem causar anomalias nos ossos da face, resultando em um crescimento craniofacial anormal e má-formação dos dentes e arcos dentários. Dentre as más oclusões, indivíduos com OI apresentam alta prevalência de mordida cruzada, mordida aberta e má oclusão de Classe III. Este estudo objetivou comparar os fatores associados com as más oclusões em crianças e adolescentes com OI e sem OI. Foi realizado um estudo transversal, pareado, com 39 indivíduos com OI e 39 sem OI, na faixa etária de três a 17 anos, e seus pais/responsáveis. A coleta de dados ocorreu por meio de um questionário direcionado aos pais/responsáveis e exame bucal das crianças e adolescentes atendidos nos setores de Ortopedia e Pediatria do Hospital das Clínicas da Universidade Federal de Minas Gerais (UFMG), em Belo Horizonte, região sudeste do Brasil. O instrumento continha itens sobre as características individuais, econômicas, hábitos comportamentais e história médica-odontológica dos filhos. O exame clínico identificou o tipo de respiração das crianças/adolescentes e a presença de anomalias dentárias, apinhamento dentário, má oclusão (mordida aberta anterior e mordida cruzada anterior/posterior) e dentinogênese imperfeita (DI). A fim de garantir a confiabilidade dos dados, foi realizado um treinamento teórico e a calibração prática dos examinadores. O treinamento teórico foi conduzido por meio de leitura sobre o tema e imagens em slides. Após essa etapa, seguindo um padrão ouro diagnóstico, foi realizada a calibração prática. Os valores kappa obtidos para cada condição examinada variaram de 0,82 a 0,96. A confiabilidade interna foi garantida por meio do testereteste do instrumento, que apresentou valores kappa entre 0,81 e 1,00. Em seguida foi realizado o estudo piloto com 5 pares de pais/responsáveis e crianças/adolescentes com OI e 5 sem OI. Após essas fases foi iniciado o estudo principal. Esta pesquisa foi aprovada pelo Comitê de Ética em Pesquisa da UFMG. Os dados foram analisados por meio do software Statistical Package for Social Science - SPSS® (versão 21.0). A média de idade das crianças/adolescentes foi de 7,9 anos (±4,5). Ao comparar o grupo de crianças/adolescentes com OI e sem OI verificou-se uma associação estatisticamente significativa entre ter OI e ser diagnosticado com anomalias dentárias (dentes conóides, dentes fusionados/ geminados, ausência clínica, rotação e microdontia) (p=0,001) e apinhamento dentário (p=0,001). A presença de OI foi estatisticamente associada com mordida aberta anterior (p=0,043), mordida cruzada anterior (p=0,045) e mordida cruzada posterior (p=0,004). No grupo com OI, a prevalência de mordida cruzada anterior foi associada com o uso de bifosfonatos (p=0,036) e com a presença de DI (p=0,004). A presença de mordida cruzada posterior foi associada com a presença de DI (p=0,006). A mordida aberta anterior não foi estatisticamente associada com as variáveis independentes (p>0,05). Concluiu-se que a prevalência de anomalias dentárias e de má oclusão foi maior entre as crianças/adolescentes com OI. No grupo com OI, ter DI foi associado a prevalência de mordida cruzada anterior e posterior entre as crianças/adolescentes. Fazer uso de bifosfonatos foi associado a mordida cruzada anterior.

Osteogenesis Imperfecta (OI) is a rare genetic disorder which affects connective tissue and it is caused by defects in type I collagen structure or synthesis. Alterations in collagen production might cause abnormal craniofacial growth and malformation of the teeth and dental arches. Among the malocclusions, individuals with OI have a high prevalence of crossbite, open bite and Class III malocclusion. This study aimed to compare the prevalence of malocclusion in children/adolescents with OI and without OI and to identify its associated factors. A cross-sectional study was performed of 39 individuals with OI and 39 without OI, aged from 3 to 17 years, and their parents/guardians. Children and adolescents with OI and without OI were matched by sex and age. They are treated in the Orthopedic and Pediatric outpatient clinics of a university hospital, in Belo Horizonte, southeastern Brazil. The instrument contained items on the individual, economic and oral-medical characteristics of the children. The clinical examination identified the type of breathing of the children / adolescents and the presence of dental anomalies, dental crowding, malocclusion (anterior open bite and anterior / posterior crossbite) and imperfect dentinogenesis (DI). In order to guarantee the reliability of the data, a theoretical training and the practical calibration of the examiners were carried out. Theoretical training was conducted through reading on the subject and images on slides. After this step, following a gold standard diagnosis, the practical calibration was performed. The kappa values obtained for each condition examined ranged from 0.82 to 0.96. The internal reliability was guaranteed by the test-retest of the instrument, which presented kappa values between 0.81 and 1.00. Then, the pilot study was carried out with 5 pairs of parents / guardians and children / adolescents with OI and 5 pairs without OI. After these phases the main study was started. This study was approved for the Research Ethics Committee of the Federal University of Minas Gerais. The mean age of children/adolescents was 7.9 years (± 4.5). When comparing the OI group and without OI, there was a statistically significant association between having OI and being diagnosed with dental anomalies (conoid teeth, fused/geminated teeth, clinical absence, rotation and microdontia) (p=0.001) and dental crowding (p=0.001). The presence of OI was statistically associated with anterior open bite (p=0.043), anterior crossbite (p=0.045) and posterior crossbite (p=0.004). In the OI group, the prevalence of anterior crossbite was associated with the use of bisphosphonates (p=0.036) and with the presence of ID (p=0.004). The presence of posterior crossbite was associated with the presence of ID (p=0.006). The anterior open bite was not statistically associated with the independent variables (p>0.05). It was concluded that the prevalence of dental anomalies and malocclusion was higher among children / adolescents with OI. In the group with OI, having DI was associated with the prevalence of anterior and posterior crossbite among children / adolescents. Using bisphosphonates was associated with anterior crossbite.

Restauraciones adhesivas en casos de dentinogénesis imperfecta: Reporte de un caso

La Dentinogénesis Imperfecta es un desorden genético autosómico dominante caracterizado por la alteración de la estructura normal de la dentina. Esta mutación se asocia a la alteración de la sialofosfoproteína dentinaria y a su desarrollo, lo que origina una malformación en la matriz orgánica de la dentina. El objetivo de este artículo es presentar una alternativa de tratamiento en niños pequeños, con manejo de conducta, utilizando restauraciones adhesivas. Se reporta a una niña de 3 años y 11 meses que acudió a consulta al servicio de Odontopediatría en el año 2016, quien presentaba al examen clínico múltiples alteraciones en cuanto a forma y coloración de los dientes y pérdida de tejido dentario sobre todo en el sector anterior, con disminución de la dimensión vertical. Radiográficamente, se evidenció la disminución de las cámaras pulpares, así como, estructuras óseas normales. Se dio como diagnóstico Dentinogénesis Imperfecta tipo II, ya que la paciente no presentaba osteogénesis imperfecta. El tratamiento consistió en profilaxis, topicación de flúor barniz en todas las piezas, Restauraciones de resina compuesta en piezas 54 (v), 64(v) y 74(v), coronas celuloides en piezas anterosuperiores (52, 51, 61 y 62) y se le instruyó a la madre sobre medidas de prevención en casa para evitar futuras lesiones de caries y mantenimiento de las restauraciones.

Efeito de flavonoides sobre microrganismos de interesse endodôntico e sua influência na expressão de marcadores de mineralização / Effect of flavonoids on endodontic microorganisms and their influence on the expression of mineralization markers

O tratamento endodôntico de dentes permanentes jovens representa um grande desafio clínico devido a presença de paredes dentinárias finas, divergentes ou paralelas e ápice aberto, o que dificulta a desinfecção e a execução dos procedimentos convencionais. Terapias de regeneração endodôntica envolvem o uso de materiais capazes de promover uma desinfecção eficaz sem causar citotoxicidade, além de induzir a diferenciação de células-tronco ou bioestimular células remanescentes do tecido pulpar mesmo após a injúria. Nesse contexto, os flavonoides, polifenóis presentes em frutas e vegetais, poderiam ser agentes interessantes para o tratamento endodôntico de dentes imaturos devido a sua amplitude terapêutica. Dessa forma, o objetivo do presente trabalho foi avaliar o efeito antimicrobiano, citotóxico e indutor de mineralização de flavonoides com finalidade de aplicação endodôntica. Este trabalho de tese foi dividido em três capítulos. O capítulo 1 avaliou a toxicidade dos flavonoides taxifolina, crisina, pinocembrina e galangina sobre fibroblastos pelo ensaio de MTT, a atividade antimicrobiana pela determinação da concentração inibitória e bactericida mínima, e a ação antibiofilme do flavonoide com melhor efeito antimicrobiano, por meio de ensaios em placas de poliestireno e em dentina radicular bovina por meio da análise por microscopia confocal. Os resultados mostraram que o flavonoide taxifolina não foi tóxico para os fibroblastos em nenhuma das concentrações analisadas, enquanto que os flavonoides crisina, pinocembrina e galangina apresentaram efeitos citotóxicos. Crisina, pinocembrina e galangina não apresentaram efeito antimicrobiano frente E. faecalis and S. mutans nas concentrações testadas. A taxifolina foi capaz de inibir todas as bactérias testadas, eliminar biofilmes de E. faecalis e S. mutans em placas de poliestireno e reduzir significantemente o biofilme de E. faecalis em túbulos dentinários. O capítulo 2 avaliou a citotoxicidade do flavonoide taxifolina e o seu potencial sobre a indução de marcadores de mineralização dentinária (produção de fosfatase alcalina - ALP, nódulos de mineralização ­ NM e expressão dos genes DSPP ­ sialofosfoproteína dentinária e DMP-1 ­ proteína da matriz dentinária - 1) em células semelhantes a odontoblastos MDPC-23, após tratamentos de 24, 72h e contínuo. A taxifolina não apresentou citotoxicidade em nenhum dos três tipos de tratamento analisados. Todas as concentrações do tratamento de 24h e as concentrações de 10 e 5µM do tratamento de 72h aumentaram a atividade de ALP. A formação de NM aumentou com os tratamentos de taxifolina à 10µM em ambos os tratamentos de 24 e 72h, e à 5µM no tratamento de 24h. A expressão de DMP-1 aumentou com o tratamento de taxifolina em ambos os tratamentos de 24 e 72h, enquanto que a de DSPP aumentou apenas com o tratamento de 72h na concentração de 5µM. O capítulo 3 avaliou a citotoxicidade da taxifolina, e o seu potencial sobre a indução de marcadores de mineralização óssea (ALP, NM e expressão dos genes ALP e colágeno 1 - Col-1) em células semelhantes a osteoblastos Saos-2, após tratamentos de 24, 72h e contínuo. Os resultados mostraram que os tratamentos com taxifolina nas concentrações de 10, 5 e 1µM não foram citotóxicos em nenhum dos períodos analisados. O tratamento de 72h da taxifolina à 10µM foi capaz de aumentar a atividade de ALP e a formação de NM, além de aumentar a expressão de Col-1 após 13 dias. O tratamento de 24h da taxifolina na concentração de 10µM aumentou a expressão de ALP após 6 dias. Conclui-se que a taxifolina é um flavonoide com potencial uso para o tratamento endodôntico de dentes permanentes jovens, devido à sua ação antimicrobiana/antibiofilme, baixa citotoxicidade e capacidade de estimular a mineralização em odontoblastos e osteoblastos(AU)

The endodontic treatment of young permanent teeth represents a great clinical challenge due to the presence of thin, divergent or parallel dentin walls and the open apex that makes it difficult to disinfect and perform conventional endodontic procedures. Endodontic regeneration therapies involve the use of materials capable of promoting effective disinfection without causing cytotoxicity, in addition to inducing differentiation of stem cells or biostimulating remaining pulp tissue cells even after injury. In this context, the flavonoids, polyphenols present in fruits and vegetables, could be interesting agents for the endodontic treatment of immature teeth due to the wide therapeutic use. Thus, the objective of the present study was to evaluate the antimicrobial, cytotoxic effects and capacity of mineralization induction of flavonoids for endodontic application. This thesis was divided into three chapters. The chapter 1 evaluated the toxicity of taxifolin, chrysin, pinocembrin and galangin flavonoids on fibroblasts by the MTT method, antimicrobial activity by determining the minimum inhibitory and bactericidal concentrations and analyzed the antibiofilm action of the flavonoid with the best antimicrobial effect, by means of the biofilm assays in polystyrene plates and in bovine root dentin and confocal microscopy analysis. The results showed that the flavonoid taxifolin was not toxic on fibroblasts in any tested concentration, while chrysin, pinocembrin and galangin flavonoids showed cytotoxic effects. Chrysin, pinocembrin and galangin showed no antimicrobial effect against E. faecalis and S. mutans in any tested concentrations. Taxifolin was able to inhibit all tested bacteria, to eliminate E. faecalis and S. mutans biofilms on polystyrene plates and significantly reduce E. faecalis biofilms from the dentin tubules. The chapter 2 evaluated the cytotoxicity of taxifolin and its potential on the induction of dentin mineralization markers (alkaline phosphatase production - ALP, mineralization nodules - MN and expression of genes DSPP - dentin sialophosphoprotein and DMP-1 - dentin matrix protein - 1) on odontoblast-like cells MDPC-23, after treatments of 24, 72h and continuous. Taxifolin did not present cytotoxicity at any of the three types of treatments analyzed. All concentrations of 24h-treatment and 10 and 5µM of 72htreatment increased ALP activity. NM formation increased with taxifolin treatments at 10µM in both 24 e 72h treatments, and at 5µM in the 24h-treatment. Expression of DMP-1 increased with taxifolin in both 24 e 72h-treatments, whereas DSPP expression increased only with 72h-treatment at 5µM. The chapter 3 evaluated the cytotoxicity of taxifolin and its potential on the induction of bone mineralization markers (ALP, NM and expression of ALP and collagen 1 -Col-1 genes) on Saos-2 osteoblast-like cells, after treatments of 24, 72h and continuous. The results showed that taxifolin treatments at 10, 5 and 1µM were not cytotoxic in any of the periods analyzed. The 72h-treatment of taxifolin at 10µM was able to increase ALP activity and NM formation, in addition to increasing Col-1 expression after 13 days. The 24h-treatment of taxifolin at 10µM increased ALP expression after 6 days. It is concluded that taxifolin is a flavonoid with potential use for endodontic treatment of young permanent teeth due to its antimicrobial/antibiofilm action, low cytotoxicity and ability to stimulate mineralization in odontoblasts and osteoblasts(AU)

Influência do peróxido de hidrogênio sobre a diferenciação celular e a mineralização do tecido pulpar após procedimento clareador: estudo histológico e imunoistoquímico / Influence of hydrogen peroxide on cell differentiation and mineralization of pulp tissue after bleaching: histological and immunohistochemical study

Introdução Através de modelo experimental caracterizado por nosso grupo de pesquisa, e protocolo clareador adaptado, verificamos que o peróxido de hidrogênio (H2O2) contido no gel clareador pode gerar efeitos ao tecido pulpar, que ainda não estão completamente compreendidos. Outros estudos mostram uma indução à mineralização, levando à posterior calcificação de grande parte do tecido pulpar e à formação de nódulos. Objetivos Os objetivos deste trabalho foram divididos em duas etapas: 1 ­ Verificar os efeitos do H2O2 na expressão de marcadores da mineralização no tecido pulpar, por meio da imunomarcação de osteocalcina (OCN) e osteopontina (OPN); e a presença de resposta celular específica ao estresse oxidativo, por meio de imunomarcação com anticorpo para espécies reativas de oxigênio (EROs); 2 ­ Determinar a capacidade de resposta ao estresse oxidativo gerado pelo H2O2 no tecido pulpar, por meio da imunomarcação de Heme-oxigenase-1 (HO-1); investigar os efeitos do gel clareador sobre a diferenciação odontoblástica, por meio da imunomarcação do fator de transcrição Jun-D; e a influência do estresse oxidativo gerado pelo H2O2 na identificação de células-tronco mesenquimais (CTMs) do tecido pulpar, por meio da técnica de imunofluorescência, com identificação concomitante de positividade celular para CD90, CD73, CD105 e negatividade para CD45. Materiais e métodos Foram utilizados 60 ratos Wistar que tiveram os molares superiores direitos ou esquerdos clareados com 0,01 mL de H2O2 35%, em uma aplicação de 30 minutos, de forma randomizada. Os molares do lado não clareado serviram de controle. Após 0 horas, 2, 3, 7, 15 e 30 dias (n=10), os animais foram mortos e as maxilas processadas para avaliação histológica, imunoistoquímica (OCN, OPN, EROs, HO-1 e Jun-D) e de imunofluorescência (CD90, CD73, CD105, CD45). Os resultados foram submetidos a testes estatísticos específicos (p<0,05). Resultados No tempo de 0 horas, houve necrose em toda a polpa coronária (p<0,05), e aos 2 e 3 dias, no terço oclusal (p<0,05); aos 7, 15 e 30 dias, não houve inflamação, assim como no controle (p>0,05). Dentina terciária estava presente aos 7 dias, aumentando em 15 e 30 dias (p<0,05). Em relação aos marcadores de mineralização, OCN foi ausente imediatamente após procedimento clareador, aumentando ao longo dos períodos, se tornando significativa aos 15 e 30 dias (p<0,05); OPN apresentou maior imunomarcação aos 7 e 15 dias no grupo clareado (p<0,05). A imunomarcação de EROs foi significativa em todos os terços da polpa coronária no grupo clareado aos 7 e 15 dias, e no terço cervical aos 2 e 30 dias, comparada ao controle (p<0,05). HO-1 revelou maior imunomarcação no grupo clareado nos terços médio e cervical da polpa coronária aos 2 e 3 dias, em todos os terços aos 7 dias, e no terço oclusal aos 15 dias, quando comparado ao grupo controle (p<0,05). Imunomarcação nuclear para Jun-D foi significativa no grupo clareado no terço cervical da polpa coronária aos 2 e 3 dias, e nos terços oclusal e médio aos 7 dias, quando comparado ao grupo controle (p<0,05), reduzindo nos demais períodos (p>0,05). Poucas células CD90+/CD73+/CD105+/CD45- foram observadas no tecido pulpar do grupo controle e do grupo clareado em todos os períodos de análise (p>0,05). Conclusões Pode-se concluir que: 1 ­ A redução da inflamação e o processo de reparo pulpar após procedimento clareador está associado com o aumento de OCN, e OPN participa durante o processo de reparo; EROs está presente no processo de defesa celular contra o estresse oxidativo decorrente do H2O2. 2 ­ As células pulpares apresentam capacidade de resposta ao estresse oxidativo expressando HO-1 nos períodos onde há inflamação, até o início do reparo; Jun-D é presente no tecido pulpar durante a redução do processo inflamatório e início da produção de dentina terciária; a presença de estresse oxidativo não influencia o número de células CD90+/CD73+/CD105+/CD45- identificadas in vivo no tecido pulpar(AU)

Introduction Through an experimental model characterized by our research group, and adapted bleaching protocol, we verified that the hydrogen peroxide (H2O2) of bleaching gel can generate effects on the pulp tissue, which are not yet completely understood. Studies show an induction to mineralization, leading to subsequent calcification of a large part of the pulp tissue and to the formation of nodules. Objectives The objectives of this study were divided into two stages: 1 ­ To verify the effects of H2O2 on the expression of mineralization markers in pulp tissue, through of immunolabelling of the osteocalcin (OCN) and osteopontin (OPN); and the presence of specific cellular response to oxidative stress, by immunolabeling with antibody to reactive oxygen species (ROS); 2 ­ To determine the capacity of response to oxidative stress generated by H2O2 in pulp tissue, through of immunolabelling of Heme-oxigenase-1 (HO-1); to investigate the effects of the bleaching gel on the odontoblastic differentiation, through the immunolabelling of the transcription factor Jun-D; and the influence of the oxidative stress generated by H2O2 on the identification of mesenchymal stem cells (MSCs) of the pulp tissue by the immunofluorescence technique, with the concomitant identification of cellular positivity for CD90, CD73, CD105 and negativity for CD45. Materials and methods Sixty Wistar rats were used, and the right or left upper molars were bleached with 0.01 mL of 35% H2O2, in a direct application of 30 minutes, randomly. The molars on the unbleached side served as controls. After 0 hours, 2, 3, 7, 15 and 30 days (n=10), the animals were killed and the jaws processed for histological, immunohistochemical (OCN, OPN, ROS, HO-1 and JunD) and immunofluorescence (CD90, CD73, CD105, CD45) analysis. The results were submitted to specific statistical tests (P<0.05). Results At 0 hours, there was necrosis throughout the coronary pulp (P<0.05), and at 2 and 3 days, in the occlusal third (P<0.05); at 7, 15 and 30 days, there was no inflammation, as well as in the control (P>0.05). Tertiary dentin was present at 7 days, increasing in 15 and 30 days (P<0.05). In relation to mineralization markers, OCN was absent immediately after bleaching procedure, increasing over the periods, becoming significant at 15 and 30 days (P<0.05); OPN has higher immunolabelling at 7 and 15 days in the bleached group (P<0.05). Immunolabelling of ROS was significant in all thirds of the coronary pulp in the bleached group at 7 and 15 days, and in the cervical third at 2 and 30 days, compared to the control group (P<0.05). HO-1 showed higher immunolabelling in the bleached group in the middle and cervical thirds of the coronary pulp at 2 and 3 days, in all thirds at 7 days, and in the occlusal third at 15 days, when compared to the control group (P<0.05). Nuclear immunolabelling for Jun-D was significant in the bleached group in the cervical third of the coronary pulp at 2 and 3 days, and in the occlusal and middle thirds at 7 days, when compared to the control group (P<0.05), reducing in the other periods (P>0.05). Low number of CD90+/CD73+/CD105+/CD45- cells were observed in the pulp tissue of the control group and the bleached group in all periods of analysis (P>0.05). Conclusions It is concluded that: 1 ­ The reduction of inflammation and the pulp repair process after bleaching is associated with increased of OCN, and OPN participates during the repair process; ROS are present in the cellular defence process against oxidative stress by H2O2. 2 ­ The pulp cells had capacity to respond to oxidative stress expressing HO-1 in the periods where there is inflammation, until the beginning of the repair; Jun-D is present in the pulp tissue during the reduction of the inflammatory process and the beginning of the production of tertiary dentin; the presence of oxidative stress does not influence the number of CD90+/CD73+/CD105+/CD45- cells identified in vivo in the pulp tissue(AU)

Osteogênese imperfeita: relato de caso de uma família / Osteogenesis imperfecta: case report of a family

A osteogênese imperfeita (OI) é uma doença rara do tecido conjuntivo cuja principal causa é uma mutação dominante nos genes do colágeno tipo I, a proteína mais abundante do osso. As principais manifestações clínicas da doença incluem a ocorrência de fraturas de repetição, encurvamento dos ossos, esclera azulada, dentinogênese imperfeita, perda auditiva, escoliose, aumento da frouxidão ligamentar, deformidade basilar do crânio e baixa estatura. Neste estudo, descrevemos cinco casos de pacientes de uma mesma família com OI tipo I, destacando os aspectos clínicos da doença. Todos os pacientes apresentam esclera azulada e tiveram múltiplas fraturas ao longo da vida. Eles permanecem em seguimento a nível ambulatorial e a maioria faz tratamento com alendronato de sódio, suplementação de cálcio e vitamina D.

Osteogenesis imperfecta is a rare disease of connective tissue in which the most common cause is the dominant mutation in collagen type I, the most abundant protein in the bone. The main clinical manifestations of the disease include the occurrence of repeat fractures, bowing of the bones, blue sclera, dentinogenesis imperfecta, hearing loss, scoliosis, increased ligament laxity, basilar skull deformity and short stature. In this study, we describe five cases of patients with osteogenesis imperfecta type I of the same family, highlighting the clinical aspects of the disease. All patients have blue sclera and had multiple fractures. They remain in follow-up and most of them do treatment with alendronate, supplementation of calcium and vitamin D.

Manejo estomatológico del paciente con osteogénesis imperfecta: presentación de un caso / Dentistry handling patient with osteogenesis imperfecta. Report of a case

La osteogénesis imperfecta es un desorden hereditario que comprende unamplio espectro de presentaciones fenotípicas cuya principal característicaes la fragilidad ósea. La dentinogénesis imperfecta es un trastorno de origen hereditario en el desarrollo de la dentina, cuya incidencia se estimaen alrededor de 1:8,000. Objetivo: Implementar un abordaje estomatoló-gico con enfoque en nuevas tendencias rehabilitadoras y preventivas entratamientos para pacientes con dentinogénesis imperfecta. Presentación del caso: Paciente masculino de tres años de edad que acude al Servicio de Estomatología del Instituto Nacional de Pediatría, diagnosticado con osteogénesis imperfecta tipo IV. Se observan las coronas con coloración ámbar generalizada, atrición y pérdida de la estructura dentaria por caries en diversos órganos dentarios. Se realiza la rehabilitación bucal bajo anestesia general, restaurando los dientes afectados con coronas de acero cromoy colocando selladores de fosetas y fi suras en molares con esmalte íntegro así como fluoruro en barniz al 5 por ciento. Conclusiones: El tratamientode la dentinogénesis imperfecta depende de la severidad que presente elpaciente. Es esencial dar un seguimiento estrecho, resolviendo de manera oportuna las necesidades que vayan surgiendo con un tratamiento no tan radical como se recomendaba anteriormente.

Osteogenesis imperfecta is a hereditary disorder that encompasses abroad spectrum of phenotypic presentations whose main characteristicis bone fragility. Dentinogenesis imperfecta is a disorder in developinghereditary dentin whose incidence is estimated to about 1:8,000.Objective: Implement a focused approach dentistry new trends inrehabilitative and preventive treatments for patients with dentinogenesisimperfecta. Case report: Male patient age three who comes toDentistry Service of the National Institute of Pediatrics, diagnosed withosteogenesis imperfecta type IV. Crowns with generalized amber colorobserved oral rehabilitation is performed under general anesthesia,restoring the aff ected teeth with stainless steel crown and placingsealant in the molar pit and fi ssure enamel integral and placementof fl uoride varnish to 5%. Conclusions: Dentinogenesis imperfectatreatment depends on the severity with which the patient presents. Itis very important to closely monitor, timely meeting the needs as theyarise, conducting a treatment not as radical as it was in the beginning.

Dentinogenesis imperfecta type II: A case report with 17 years of follow-up

Dentinogenesis imperfecta is a dominant autosomal hereditary disorder of dentin formation that affects the deciduous and permanent teeth. Its etiology is characterized by inadequate cell differentiation during odontogenesis. The clinical characteristics of dentinogenesis imperfecta are discolored teeth with a translucency that varies from gray to brown or amber. Radiographically, the teeth exhibit pulp obliteration, thin and short roots, bell-shaped crowns, and periapical bone rarefaction. The aim of this report was to present a case of dentinogenesis imperfecta type II that was followed up over a 17-year period. This report also presents scanning electron microscopy images of the enamel and dentin, showing that both were altered in the affected teeth. The disease characteristics and the treatments that were administered are reported in this study to guide dentists with respect to the need for early diagnosis and adequate follow-up to avoid major sequelae.

The role of nuclear factor I-C in tooth and bone development

Nuclear factor I-C (NFI-C) plays a pivotal role in various cellular processes such as odontoblast and osteoblast differentiation. Nfic-deficient mice showed abnormal tooth and bone formation. The transplantation of Nfic-expressing mouse bone marrow stromal cells rescued the impaired bone formation in Nfic(-/-) mice. Studies suggest that NFI-C regulate osteogenesis and dentinogenesis in concert with several factors including transforming growth factor-β1, Krüppel-like factor 4, and β-catenin. This review will focus on the function of NFI-C during tooth and bone formation and on the relevant pathways that involve NFI-C.

Dentinogenesis imperfecta type Ⅲ with supernumerary teeth: A case report / 实用口腔医学杂志

This report presents a case of dentinogenesis imperfecta type Ⅲ with supernumerary teeth,the etiopathogenesis,clinical manifestations and therapy are discussed.

Dentinogenesis Imperfecta: A Case Report of Five Patients in the Same Family Group / Dentinogenesis Imperfecta: Un Reporte de Caso de Cinco Pacientes en el Mismo Grupo Familiar

Dentinogenesis imperfecta (DI) is a type of dentin dysplasia that affects the dentin structure of one or both dentitions, which may be classified in three types. The aim of this report was to show the clinical and radiographic features of the four cases of DI in the same family group. Five brothers were checked clinically and radiographically. Two individuals were diagnosed, by their phenotypic features and medical history, with DI type I; two of them with DI type II and one case without signs of DI. It is important to know the features of dentinogenesis imperfecta to perform a comprehensive dental care, including the right diagnosis and an effective treatment plan.

La dentinogénesis imperfecta (DI) es un tipo de displasia de la dentina que afecta su estructura en una o ambas denticiones. La DI puede clasificarse en tres tipos. El objetivo de este informe fue demostrar las características clínicas y radiológicas de los cuatro casos de DI en un mismo grupo familiar. Cinco hermanos fueron controlados clínica y radiográficamente. Dos individuos fueron diagnosticados, por sus características fenotípicas y antecedentes clínicos, con el tipo de DI I; dos de ellos con DI de tipo II y un caso sin signos de DI. Es importante conocer las características de la dentinogénesis imperfecta para poder realizar una atención odontológica integral, lo que permitirá desarrollar un diagnóstico correcto y un plan de tratamiento efectivo.