Enfoque de Tratamiento de una Adolescente con Amelogénesis Imperfecta / Treatment Approach for an Adolescent with Amelogenesis Imperfecta

La amelogénesis imperfecta (AI) es un grupo de tras-tornos hereditarios, clínica y etiológicamente hete-rogéneos, derivados de mutaciones genéticas, que se caracterizan por anomalías cualitativas y cuanti-tativas del desarrollo del esmalte, pudiendo afectar la dentición primaria y/o permanente. El tratamiento del paciente con AI es complejo y multidiscliplinario; supone un desafío para el odontólogo, ya que por lo general están involucradas todas las piezas dentarias y afecta no solo la salud buco dental sino el aspecto emocional y psicológico de los pacientes. Con el obje-tivo de describir el tratamiento integral y rehabilita-dor realizado en una paciente con diagnóstico de AI tipo III, se reporta el caso de un adolescente de sexo femenino de 13 años, que concurrió en demanda de atención a la Cátedra de Odontología Integral Niños de la Facultad de Odontología de la Universidad de Buenos Aires (FOUBA), cuyo motivo de consulta fue la apariencia estética y la hipersensibilidad de sus pie-zas dentarias. Durante el examen clínico intraoral, se observó que todas las piezas dentarias presentaban un esmalte rugoso, blando, con irregularidades y una coloración amarronada, compatible con diagnóstico de Amelogénesis Imperfecta tipo III hipomineralizada. Conclusión: El tratamiento rehabilitador de la AI en los pacientes en crecimiento y desarrollo estará diri-gido a intervenir de manera integral y temprana para resolver la apariencia estética y funcional, evitar las repercusiones sociales y emocionales, y acompañar a los pacientes y sus familias (AU)

Amelogenesis imperfecta (AI) is a group of clinically and etiologically heterogeneous hereditary disorders, derived from genetic mutations, characterized by qualitative and quantitative anomalies of enamel development, which can affect primary and/or permanent dentition. The treatment of patients with AI 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 comprehensive and rehabilitative treatment carried out in an adolescent patient with a diagnosis of type III AI. Case report: The case of a 13-year-old female patient, who required dental attention at the Department of Dentistry for Children of the School of Dentistry of the University of Buenos Aires, whose reason for consultation was esthetic appearance and hypersensitivity of her teeth. In the intraoral clinical examination, it was observed that all the teeth had rough, soft enamel, with irregularities and a brownish color, compatible with the diagnosis of type III hypomineralized Amelogenesis Imperfecta. Conclusion: Rehabilitative treatment of AI in growing and developing patients will be aimed at early and comprehensive intervention to resolve esthetic and functional appearance, avoid social and emotional repercussions and accompany patients and their families (AU)

Clinical and Molecular Disorders Caused by COVID-19 During Pregnancy as a Potential Risk for Enamel Defects

ABSTRACT This paper discusses the potential risk that COVID-19 generates for the development of enamel defects. This hypothesis was built based on the etiopathogenesis of enamel defects and the relationship with the symptom's characteristic of COVID-19. Pregnancy is a critical period for the child's development; exposure to pathological agents can cause systemic imbalances and risks of adverse perinatal and prenatal outcomes. The main clinical symptoms of this disease and its association with that dental outcome were considered. Fever, breathing, cardiovascular disorders, and diarrhea were related as potential etiological factors of ameloblast metabolism imbalance, which can interfere qualitatively and quantitatively in the development, maturation and mineralization of the tooth enamel. Molecular disorders derived from COVID-19, as well as their clinical symptoms, can be considered potential risk factors for the development of enamel defects. Individuals with enamel defects experienced high stress levels during pregnancy or early childhood. The approach adopted may help build new research to ensure understanding of the etiology of the development of dental enamel defects and its relationship with COVID-19. However, longitudinal studies need to be conducted to confirm the association between COVID-19 and adverse events during pregnancy.

What are the Systemic Factors Associated with the Molar-Incisor Hypomineralization Etiology?

ABSTRACT Objective: To evaluate the systemic factors associated with Molar-Incisor Hypomineralization (MIH) etiology. Material and Methods: A total of 731 8-year-old schoolchildren enrolled in the public school system in Curitiba, Brazil, was randomly selected. The MIH diagnosis was performed by calibrated examiners (Kappa >0.80) according to the European Academy of Pediatric Dentistry criteria (2003). The systemic factors were collected through a semi-structured questionnaire and applied to the children's mothers, addressing the medical history from pregnancy to the first three years of children's life. Associations were analyzed by Poisson regression analysis with robust variance (p<0.05). Results: The systemic factors in the prenatal and perinatal periods were not associated with MIH (p>0.05). The children who used medications during the first years of life had a significantly higher prevalence of MIH (PRc = 2.18 CI = 95% 1.06-4.48; p=0.033). Conclusion: The use of medications during the first three years of children's life is associated with a higher prevalence of MIH.

Dental trauma with intrusive luxation and six-year monitoring: clinical case report / Traumatismo dentário com luxação intrusiva com acompanhamento de seis anos: relato de caso clínico

Objective: this study is designed to report a clinical intrusion case, which exhibited enamel hypoplasia on the crown of tooth 11 and which underwent clinical and X-ray monitoring for six years. Case report: A threeyear- old female patient exhibited intrusion of deciduous teeth 51, 52, 61, and 62. After X-ray, it was detected that teeth 51 and 61 had perforated the nasal cavity floor, but all four teeth had re-erupted and began to be monitored. After six months, teeth 51 and 61 exhibited bone loss in the interproximal area, as well as mobility, which led to tooth extraction. Teeth 52 and 62 re-erupted in a satisfactory position, and exhibited no signs and symptoms of infection, periapical alteration, or mobility. These teeth were therefore maintained in the arch. After six years, it was detected that tooth 11 had erupted with enamel hypoplasia. Final considerations: Longterm clinical and X-ray control proved essential, as it allowed for the early diagnosis of potential alterations, and minimized potential sequelae to the permanent teeth.(AU)

Objetivo: relatar um caso clínico de intrusão com acompanhamento clínico e radiográfico de 6 anos, que apresentou hipoplasia de esmalte na coroa do dente 11. Relato de caso: paciente de 3 anos, sexo feminino, apresentou intrusão dos dentes decíduos 51, 52, 61 e 62. Após radiografia, foi detectado que os dentes 51 e 61 tinham perfurado o soalho da fossa nasal, porém, ocorreu re-erupção dos 4 dentes e foi realizado acompanhamento. Após 6 meses, nos dentes 51 e 61, foram observadas perda óssea na região interproximal e mobilidade, optando-se pelas exodontias. Os dentes 52 e 62 re-erupcionaram em posição satisfatória e com ausência de sinais e sintomas de infecções, alterações periapicais e mobilidade, sendo mantidos no arco. Após 6 anos, foi observado que o dente 11 erupcionou apresentando hipoplasia de esmalte. Considerações finais: mostrou-se essencial o controle, clínico e radiográfico, em longo prazo, diagnosticando precocemente possíveis alterações e minimizando sequelas que podem acometer os dentes permanentes.(AU)

Oral manifestations of renal tubular acidosis associated with secondary rickets: case report / Manifestações bucais da acidose tubular renal associada ao raquitismo secundário: relato de caso

ABSTRACT This report describes the oral manifestations of renal tubular acidosis (RTA) associated with secondary rickets and discusses the biological plausibility of these findings. The characteristic electrolyte changes during RTA or genetic mutations that trigger RTA may be responsible for impaired amelogenesis, dental malocclusion, impacted teeth, and absent lamina dura. This report reinforces the possibility of an association between RTA and the oral manifestations described.

RESUMO Este relato de caso descreve as manifestações bucais da acidose tubular renal (ATR) associada ao raquitismo secundário e discute a plausibilidade biológica desses achados. As alterações eletrolíticas características da ATR ou as mutações genéticas que a desencadeiam podem ser responsáveis pela amelogênese imperfeita, maloclusão dentária, dentes impactados e ausência de lâmina dura. Este relato reforça a possibilidade de uma associação entre ATR e as manifestações bucais descritas.

Factors associated with the development of dental defects acquired in the extrauterine environment

Abstract This study aimed to analyze the association of sociodemographic, child health, healthcare service, and access indicators with developmental defects of enamel (DDE) acquired outside the uterus, based on gestational factors. A cohort of births was carried out, and 982 children aged 12 to 30 months were examined. A total of 1,500 women were followed up as of the 5th month of gestation, and the child's gestational age was evaluated at follow-up. The clinical examination was performed as recommended by the World Health Organization, and defects were classified using the modified DDE index. Six models were considered: presence of DDE (Model 1) or opacities (Model 4), number of teeth with DDE (Model 2) or opacities (Model 5), and incidence rate of DDE (Model 3) or opacities (Model 6). Associations were estimated by relative risk (RR) in Poisson regression models. In the adjusted analysis, the mother's lowest education level was associated with the highest occurrence of DDE in Models 1 (RR = 26.43; p = 0.002), 2 (RR = 9.70; p = 0.009), and 3 (RR = 5.63; p = 0.047). Breastfeeding for over 12 months (RR = 0.45; p = 0.030) and recent use of anti-infection drugs (RR = 0.20; p = 0.039) had a protective effect on DDE (Model 1). The factors associated with the highest incidence of opacities were not having health insurance (RR = 2.00; p = 0.043) (Model 5), and belonging to a family of poor social class (RR = 4.67; p = 0.007) (Model 6). Children in a situation of socioeconomic vulnerability have a higher risk of presenting extrauterine DDE. Breastfeeding was a protection factor for DDE development.

Estado bucal en pacientes con fibrosis quística (FQ) / Oral status in patients with cystic fibrosis

En un estudio (Grasan, Sebelli, Anchava, Ferrari, y Biondi, 2016) se observó que niños con Fibrosis Quística (FQ) atendidos en un hospital revelaron menor patología dental comparados con pacientes sanos que concurrían a la Cátedra (FOUBA), siendo necesarios estudios comparativos con niños sin demanda de atención. Objetivo: determinar el estado bucal de pacientes pediátricos que concurren al Hospital Garrahan con diagnóstico de FQ y compararlos con niños sanos sin demanda de atención de la misma edad y sexo que concurren a una escuela pública del partido de Merlo, Buenos Aires. Método: Se empleó un diseño observacional, casos-control, en niños entre 3 y 16 años concurrentes Hospital (GFQ) y un grupo control de niños de la escuela (GS). A través de planillas se registraron datos personales, CPOD, CPOS, ceod, ceos, Indice de placa Silness y Loe, Hemorragia simplificado, Gingival Loe y Silness e Hipomineralización molar incisiva (HMI). Para la estimación de la prevalencia y otras variables cualitativas se calcularon las medias y desvíos estándar y Chi-cuadrado para las comparaciones. Resultados: cada grupo quedó conformado por 19 niños de 9+-6 años, 47% sexo femenino. Al comparar ambos grupos no se hallaron diferencias significativas en CPOD (p=0,236), CPOS (p=0,537), ceos (p=1,000), Indice de placa Silness y Loe (0,172) y Hemorragia simplificado (p=1,000) y HMI (p=0,001). Conclusiones: se concluye que los niños con FQ de la zona objeto del estudio, desde la muestra de 19 niños, no presentan diferencias en la salud bucal (AU)

Enamel defects and tooth eruption disturbances in children with sickle cell anemia

Abstract Sickle cell anemia, a genetic disease caused by a mutation in the beta-globin gene, can present oral manifestations such as delayed tooth eruption and hypomineralized enamel and dentin. The aim of the present study was to evaluate the prevalence and severity of developmental defects of enamel (DDE) and delayed tooth eruption in children with sickle cell anemia. The sample comprised 56 male and female children with sickle cell anemia aged 6 to 12 years and treated at the Hematology and Hemotherapy Center of Pernambuco, Brazil. The data were collected according to the WHO criteria for DDE and tooth eruption. The prevalence of DDE was 58.2% and increased with age, affecting 43.8% of children aged 6 to 8 years and 66.7% of those aged 10 to 12 years (p>0.05; Pearson's chi-square test). There was no significant association between DDE and sex; the most prevalent type of DDE was diffuse opacity (6.2%). Tooth eruption was delayed in 18 children (32.1%). The delay increased with age and was detected in 11.8% of children aged 6 to 8 years, in 20.0% of those aged 8 to 10 years and in 54.2% of those aged 10 to 12 years (p<0.05; Pearson's chi-square test). Delayed tooth eruption was higher in males (36.7%, p>0.05). The prevalence of DDE was high, increased with age and was similar between sexes, while delayed eruption was higher in males and showed a significant association with age.

Oral manifestations in children with mucopolysaccharidosis / Manifestações orais de mucopolissacaridose em crianças

BACKGROUND: Patients with mucopolysaccharidosis have several changes of the stomatognathic complex, representing a challenge for dentists. OBJECTIVE: The study aimed to evaluate and characterize oral health in patients with mucopolysaccharidosis in a reference center of Portugal. METHOD: The sample consisted of twelve participants with mucopolysaccharidosis followed in Metabolic Diseases Unit of the S. João Hospital Centre and twelve healthy participants followed at Faculty of Dental Medicine, University of Porto. The clinical oral evaluation was performed by a complete extra-oral and intra-oral examination to assess the presence of oral pathologies, gingival index and occlusion status. RESULTS: Mucoplysaccaridosis patients and controls presented similar age ranges and sex distribution. In comparison to controls, children with mucopolysaccharidosis presented a higher prevalence of tooth decay, gingival bleeding, macroglossia, dental hypoplasia, lingual interposition, delayed tooth eruption, anterior open bite, right and left posterior cross-bite, limitation of mouth opening, alteration on the size and shape of the teeth, diastemata and maxillary compression. CONCLUSIONS: Patients with mucopolysaccharidosis have a high prevalence eruption delay, teeth morphology alterations, occlusal problems, dental caries and bleeding gums, highlighting the need of oral health care providers to improve diagnostics and preventive protocols to overcome the factors that limit the oral health of these patients and promote together with parent/caregiver efficient oral care strategies.

INFORMAÇÕES GERAIS: Os pacientes com mucopolissacaridose apresentam diversas alterações do complexo estomatognático, representando um desafio para os médicos dentistas. OBJETIVO: O estudo pretendeu avaliar e caracterizar a saúde oral em pacientes com mucopolissacaridose num centro de referência em Portugal. MÉTODO: A amostra foi constituída por doze pacientes com mucopolissacaridose (MPS) seguidos na Unidade de Doenças Metabólicas do Centro Hospitalar de São João e doze participantes saudáveis seguidos na Faculdade de Medicina Dentária da Universidade do Porto. A avaliação clínica oral consistiu num completo exame extra-oral e intra-oral para avaliação de patologias orais, índice gengival e perfil oclusal. RESULTADOS: Pacientes com MPS e controlos apresentaram médias similares de idade e de distribuição de género. Em comparação com os controlos, crianças com mucopolissacaridose apresentam maior prevalência de dentes cariados, sangramento gengival, macroglossia, hipoplasia dentária, interposição lingual, erupção dentária atrasada, mordida aberta anterior, mordida cruzada posterior direita e esquerda, limitação da abertura da boca, alteração do tamanho e forma dentária, diastemas e compressão maxilar. CONCLUSÃO: Pacientes com mucopolissacaridose apresentam maior prevalência de erupção dentária atrasada, alterações morfológicas dentárias, problemas oclusais, cáries dentárias e gengivas inflamadas, reforçando a necessidade de prestadores de saúde oral para melhorar diagnósticos e protocolos preventivos para ultrapassar os fatores que limitam a saúde oral destes pacientes e promover em conjunto com pais/cuidadores estratégias de saúde oral eficientes.

Prevalence of enamel defects and associated risk factors in both dentitions in preterm and full term born children

OBJECTIVES: The aim of this study was to evaluate the prevalence of enamel defects and their risk factors on primary and permanent dentitions of prematurely born children and full-term born children born at Regional Hospital of Asa Sul, Brasília, DF, Brazil. MATERIAL AND METHODS: Eighty 5-10-year-old children of both genders were examined, being 40 born prematurely (G1) and 40 born full term (G2). The demographic variables, medical history and oral health behaviors were retrieved using a questionnaire and data obtained from clinical examination were recorded. The teeth were examined and the presence of enamel defects was diagnosed according to the DDE Index and registered in odontograms. Subsequently, the defects were categorized in four groups according to one of the criteria proposed in 1992 by the FDI Commission on Oral Health, Research and Epidemiology. Kruskal-Wallis, Chi-square, Kappa, Mann-Whitney tests and logistic regression were used for statistical analysis. RESULTS: 75% of total sample had enamel defects. There was a major prevalence of hypoplasia of the enamel in G1 (p<0.001). There was a significant relationship between low weight and presence of the imperfections on the enamel in G1 on the primary dentition. The logistic regression model showed that the other risk factors such as monthly per capita family income, educational level, dietary and hygiene habits, fluoride exposure, trauma, and diseases were not associated with enamel defects and caries. CONCLUSIONS: Pre-term labor can be a predisposing factor for the presence of the enamel hypoplasia in the primary dentition.

Traumatismos dentoalveolares que afectan a las estructuras de soporte de los dientes temporales y sus efectos en los sucesores definitivos / Dentoalveolar injuries affecting tooth support structures in temporary and the effects on permanent succesors

El propósito de este estudio fue determinar las secuelas producidas en la dentición definitiva luego de un traumatismo que afectó a las estructuras de soporte de dientes temporales. La muestra corresponde a 53 niños beneficiarios del Centro de Referencia Odontopediátrico Simón Bolívar, entre las edades de 1 a 7 años que sufrieron traumatismo dentoalveolar del sector anterior, durante el periodo 2005-2007 que se clasificó según Andreasen en subluxación, luxación lateral, luxación intrusiva, luxación extrusiva y avulsión. Los resultados obtenidos nos permiten determinar un 20 por ciento de secuelas clínicas en dientes sucesores permanentes, siendo las opacidades del esmalte con un 88 por ciento las lesiones mas frecuente, luego las hipoplasias del esmalte que alcanzó un 12 por ciento. La mayoría de las lesiones registradas según severidad son de tipo leves (65 por ciento) y el porcentaje de dientes con secuelas clínicas no cambia significativamente a través de los diferentes diagnósticos. La edad promedio de los niños al momento de sufrir el trauma y que presentaban secuelas fue de 4,7 años. No se registraron casos de luxación extrusiva puesto que no se observaron casos con secuelas. Se concluye que los dientes definitivos que remplazan dientes deciduos que han sufrido trauma como luxación intrusiva, subluxación, luxación lateral y avulsión, con frecuencia presentan secuelas que se expresan principalmente como opacidad e hipoplasias del esmalte. Las secuelas de estos traumas podrían disminuir si los niños expuestos a estas lesiones concurrieran tempranamente a centros de salud especializados en la atención del traumatismo dentoalveolar...

The purpose of this study was to determine the effects produced in the permanent teeth after trauma affecting the supporting structures of teeth. The sample is 53 children beneficiaries of the Reference Center Odontopediátrico Simon Bolivar, ages 1 to 7 years who suffered injury of the anterior dentoalveolar trauma during the period 2005-2007 that was classified according to Andreasen: subluxation, lateral luxation, intrusive luxation, extrusive luxation and avulsion. The results obtained allow us to determine 20 percent of clinical sequelae in permanent successor teeth, with enamel opacities with 88 percent more frequent injuries, then the enamel hypoplasias reached 12 percent. Most of the injuries recorded as mild severity are of type (65 percent) and the percentage of teeth with clinical sequelae not changes significantly across the different diagnoses. The average age when children suffer the trauma and had sequelae was 4.7 years. No cases of extrusive luxation since there were no cases with sequelae. It is concluded that the permanent teeth that replace primary teeth that have suffered trauma such as intrusive luxation, subluxation, lateral luxation and avulsion, often have consequences that are expressed mainly as opacity and enamel hypoplasia. The aftermath of this trauma could be reduced if children exposed to these lesions are entered into early health centers specialized in the care of dentoalveolar trauma...

Hipoplasia do esmalte: fundamentos para nomenclatura e identificação dos tipos e causas / Enamel hypoplasia: nomenclature foundations and identifications of types and causes

Quando as manchas e os defeitos no esmalte são nominados incorretamente, significa que as verdadeiras causas e mecanismos foram ignorados e uma abordagem terapêutica inadequada pode ser aplicada4. A formação do esmalte, ou amelogênese, é executada exclusivamente pelos ameloblastos, células altamente especializadas, que: produzem a matriz orgânica adamantina; mineralizam-o de uma forma muito específica, para resultar nos prismas; proporcionam uma estrutura altamente cristalina, ao reabsorverem as proteínas anteriormente incorporadas. A atividade do ameloblasto exige um grande refinamento metabólico, estrutural e organizacional, a ponto de ser considerado uma das células mais sensíveis frente aos agentes externos.

An Epidemiological study of developmental defects of enamel in a group of Nigerian School Children / Estudo epidemiológico dos defeitos de desenvolvimento do esmalte em um grupo de escolares Nigerianos

Objeti vo: Este estudo epidemiológico analisou a prevalência,distribuição e associação dos fatores eiológicos nodesenvolvimento de defeitos do esmalte em criançasnigerianas.Método: A amostra compreendeu a análise de 2.015 escolares(1.088 meninos e 927 meninas) com idades entre 4 a 16 anos,selecionadas aleatoriamente de 6 escolas em Lagos, Nigeria. Oexame intra-oral para o diagnósti co dos defeitos do esmalte foifeito com o uso método modifi cado proposto pela FederaçãoDentária Internacional (FDI). As mães das crianças foramentrevistadas a respeito dos fatores eti ológicos.Resultados: Um total de 226 (11,2%) das crianças examinadasapresentavam defeitos do esmalte. A hipoplasia do esmaltefoi o defeito mais prevalente (7,5%), e o ti po de distribuiçãomais comum foi a difusa (61,5%). Não se observou diferençaestati sti camente signifi cante na distribuição dos defeitos entreos sexos (p>0,05). A maioria dos defeitos localizavam-se nasfaces vesti bulares dos dentes afetados (40,26%). A diferençaobservada na localização dos defeitos dos dentes entre meninose meninas foi estati sti camente signifi cante (p<0,05).Conclusão: Estes defeitos de esmalte não estéti cos sãoprevalentes nas crianças nigerianas e estão, principalmente,associados com doenças da infância, uso de tetraciclina e baixacondição sócio-econômica...

Objecti ve: This epidemiological study of dental enamel defectsin a group of Nigerian school children assessed the prevalence,distributi on and associated eti ological factors of developmentaldefects of enamel.Method: The study subjects consisti ng of 2015 school children,(1088 males and 927 females) in the age range 4 to 16 yearswere randomly selected from 6 schools in Lagos, Nigeria. Intraoralexaminati on for dental enamel defects was carried out onall the children using a modifi cati on of the “Federati on DentaireInternati onale” system. The mothers of the children wereinterviewed on relevant associated eti ological factors.Results: A total of 226 (11.22%) of the children examined haddental enamel defects. The most prevalent dental enameldefect seen was chronologic enamel hypoplasia (7.5%), andmost common type of distributi on was the diff use patchy type(61.5%). No stati sti cally signifi cant diff erence in distributi onof defects between males and females. (p>0.05.) Most ofthe defects were on the buccal surfaces of the aff ected teeth(40.26%). The diff erence observed in the locati on of defectson the teeth between males and females was stati sti callysignifi cant (p<0.05).Conclusion: These unaestheti c enamel defects are prevalent inNigeria children and mainly associated with childhood illnesses,use of tetracycline and poor socio-economic conditi ons...

Oral manifestations leading to the diagnosis of familial tuberous sclerosis.

Clinical manifestations of tuberous sclerosis (TS) are variable, and oral involvement occurs in less than 10% of the affected patients. We reported herein a nine-year-old boy with dental enamel pits and gingival nodular lesions, histologically diagnosed as angiofibromas that leaded to the diagnosis of a family with TS. In this report, we demonstrated the multiprofessional importance in the diagnosis of TS. Early diagnosis of TS is essential for an appropriate treatment of the affected patients and genetic counseling.

Enfermedad celíaca y su relación con la odontopediatría / Celiac disease and its relationship to pediatric dentistry

La enfermedad celíaca (EC) es una intolerancia permanente al gluten presente en los alimentos que ocurre en personas genéticamente susceptibles. Las manifestaciones de la enfermedad son entre otras, baja tall, diarreas y en el medio bucal estomatitis aftosa recurrente e hipoplasia y/o hipomineralización del esmalte. Los anticuerpos salivales IgA para gliadina son potencialmente utilizables para el rastrillaje de pacientes que pueden tener la EC, particularmente en el estudio de niños por ser un método no invasivo. El objetivo del presente trabajo es alertar a los profesionales odontólogos, principalmente a los odontopediatras que acompañan el crecimiento y desarollo de los niños, a mantenerse atentos a los signos de la EC y realizar la derivación correspondiente al médico pediatra. El diagnóstico precoz puede mejorar la calidad de vida del paciente, su crecimiento, talla, estabilidad emocional, normalidad de la dentición y desarrollo general.

Displasia ectodérmica hipohidrótica: presentación de un caso / Hypohydrotic ectodermal dysplasia: presentation of a case

El síndrome de Christ-Siemens-Touraine o Displasia Ectodérmica Hipohidrótica, constituye la forma más frecuente de las llamadas displasias ectodérmicas: síndromes de carácter congénito, en los que se ven afectados uno o varios componentes derivados del ectodermo y que no presentan un curso progresivo. La displasia ectodérmica hipohidrótica se trata de un síndrome heredo familiar, transmitido como un rasgo recesivo ligado a X, que afecta en el 90 por ciento de los casos a los varones. La forma más comúnmente encontrada de displasia ectodérmica es el tipo hipohidrótico, caracterizada por la triada hipohidrosis, hipotricosis e hipodoncia. Una fascie caracterizada por abombamiento frontal, con depresión de tercio medio, hundimiento de puente nasal y proquelia por la ausencia de dientes son otros hallazgos. Se encuentran manifestaciones visuales, otorrinolaringológicas, desórdenes alérgicos, infecciones respiratorias y distrofia de las uñas de leve a moderada. El paciente llegó a nuestra consulta con APF de padre afectado de Displasia Ectodérmica Hipohidrótica, tercero de cuatro hermanos (dos varones y una mujer no afectados), único sobrino con Displasia Ectodérmica Hipohidrótica, hijo de su hermana. Refirió antecedentes de infecciones respiratorias frecuentes, xerosis y vesicoampollas en mucosa oral. Al examen físico encontramos: dermatosis solares, estomatitis, rasgos faciales característicos de frente y mentón prominente, nariz de punta fina, mejillas hundidas, labios gruesos y evertidos, así como pabellones auriculares grandes. Al examen físico dermatológico presentó la piel lisa, seca y arrugada alrededor de los ojos, dando un aspecto de envejecimiento prematuro. La hipotricosis es parcial en cuero cabelludo, cejas, barba, axilas y pubis. Al examen físico estomatológico presentaba inflamación de las encías, estomatitis y desarrollo dentario anormal.

The Christ-Siemens-Touraine syndrome or hypohydrotic ectodermal dysplasia, is the most frequent form of the so called ectodermal dysplasias; a congenital character syndrome in which one or several components of the ectoderm are affected, without presenting a progressive course. The hypohydrotic ectodermal dysplasia is an hereditary familiar syndrome, transmitted as a recessive characteristic linked to X, affecting 90 percent of the males. The most common form of the ectodermal dysplasia is the hypohydrotic one, characterized by the triad hypohydrosis, hypotrichosis and hypodoncia. Another finding was a fascia characterized by frontal convection, depression of the nasal bridge and proquelia for absence of teeth. We found otorhinolaryngologic manifestations, allergic disorders and from light to moderated nail dystrophy. The patient arrived to our consultation with previous familiar antecedents of father affected by Hypohydrotic Ectodermal Dysplasia, third of four siblings (two brothers and a sister), only a nephew, son of his sister, with Hypohydrotic Ectodermal Dysplasia. He told about antecedents of frequent respiratory infections, xerosis and blisters in oral mucosa. When we carried out the physical examination we found: sun dermatosis, stomatitis, characteristic facial features of prominent forehead and chin, everted and thick lips, and also big earlobes. The physical dermatologic examination showed smooth skin, dry and wrinkly around the eyes, giving an aspect of premature aging. The hypotrichosis is partial in the scalp, eyebrows, beard, armpits and pubis. The physical oral examination showed gums inflammation, stomatitis and abnormal dental development.

Alteraciones estructurales del esmalte: hipoplasias y opacidades del primer molar permanente / Enamel structural alterations: hypoplasia and hypomineralization of the first permanent molars

Las últimas décadas la odontología ha conseguido importantes avances para entender cabalmente la etiopatogenia y los mecanismos aptos para la prevención y tratamiento de una de sus enfermedades más prevalentes: la caries dental. Esto ha permitido especialmente a la odontopediatría, dirigir la atención a otras patologías que hoy se incrementan como las erosiones y las anomalías estructurales del diente. Dentro de estas últimas las hipoplasias dentales de origen genético van encontrando nuevas respuestas con el enfoque molecular merced al desarrollo de la biología molecular y la genética. El efecto nocivo que producen por vía sistémica las tetraciclinas o los fluoruros también han sido especialmente estudiados, así como el efecto deletéreo de factores locales como la infección y el trauma de las piezas primarias sobre la estructura dentaria de las piezas permanentes de reemplazo. Sin embargo la clínica muestra con una frecuencia cada vez mayor opacidades e hipoplasias de los primeros molares permanentes, con una severidad que varía desde aquellos que tienen los cuatro primeros molares afectados, donde se podría suponer que al desarrollarse simultáneamente obedecen a una causa sistémica, hasta otros que muestran una distribución azarosa afectando solo a uno, dos o tres molares. En esta materia queda aun mucho por resolver ya que plantea un esencial desconocimiento etiológico que impide el desarrollo de medidas preventivas y eficaces procedimientos terapéuticos.

Esthetic and endosurgical management of Turner's hypoplasia; a sequlae of trauma to developing tooth germ.

Turner's hypoplasia usually manifests as a portion of missing or diminished enamel, generally affecting one or more permanent teeth in the oral cavity. A case report of 8 year old girl who met with trauma at 2 years of age leading to primary incisors being knocked out, reported after 6 years with complaint of pain and discharge in her anterior malformed teeth is discussed in this article. The permanent incisors erupted with dilacerated crown, root malformations and missing enamel. Further, patient developed sinus, lateral root pathology, tooth mobility and malocclusion in relation to affected teeth which were managed by esthetic, functional, endodontic and surgical procedure. Root canal treatment along with palatal contouring and esthetic restoration by light cure composite was performed on the tooth with crown dilaceration and sinus, where as surgical management was considered for the tooth with root malformation.

Os defeitos do esmalte e a erupcão dentária em criancas prematuras / Evaluation of the dental eruption pattern and of enamel defects in the premature child

OBJETIVO: A proposta do trabalho foi verificar a relacão entre defeitos do esmalte e atraso da erupcão dentária com prematuridade. MÉTODOS: A amostra consistiu de 100 criancas prematuras, entre seis meses a seis anos de idade em acompanhamento no Instituto da Crianca da Faculdade de Medicina da USP. Foi feita uma anamnese da cavidade bucal por apenas um observador, avaliando o tempo da erupcão dentária e a ocorrência de defeitos no esmalte. Realizou-se também uma avaliacão médica, com dados referentes a possíveis problemas durante o período pré-natal, neonatal e pós-natal. A avaliacão estatística utilizou análise descritiva, freqüência média e intervalo de confianca de 95 por cento. RESULTADOS: Defeitos apareceram em 35 por cento das criancas prematuras; 51,43 por cento das que tinham defeitos haviam nascido com peso baixo (< 2500g) comparados aos 14,29 por cento que haviam nascido com peso normal (>2500g) . Não houve relacão entre ocorrência de defeitos com baixo Boletim de Apgar em 1 minuto, 2 minutos e 5 minutos (p=0,628; p=0,308; p=0,193). Os defeitos mais comuns foram as opacidades brancas, tanto na denticão decídua (19 por cento) quanto na permanente (100 por cento). Os tercos incisais e cervicais das superfícies vestibulares foram os mais afetados com valores de 88,04 por cento na denticão decídua e 100 por cento na permanente. Cerca de 42 por cento das criancas tiveram dentes irrompidos entre 6 e 10 meses. CONCLUSAO: Criancas prematuras podem apresentar defeitos do esmalte causados por diferentes fatores durante a gravidez com uma possível associacão entre baixo peso e defeito. Os dentes irromperam em tempo normal, no entanto, o número total de dentes até os 36 meses foi menor do que os encontrados em criancas nascidas a termo.