Peripheral odontogenic fibroma in a child with Ellis-van Creveld syndrome: Case report.

INTRODUCTION: Ellis-van Creveld (EVC) syndrome is an autosomal recessive disorder predominantly characterized by a disproportionate dwarfism, ectodermal dysplasia, postaxial polydactyly, and congenital heart malformations and pulmonary hypoplasia. OBJECTIVE: In this article, we hereby present a case of a 6-year-old Brazilian boy with EVC syndrome who presented a rare oral lesion as well as a remarkable number of classical and uncommon oral and dental features. CASE REPORT: Clinical and radiographic examination revealed multiple enamel hypoplasia, teeth agenesis, conical teeth, lower canine rotation, bilateral posterior crossbite, taurodontism of deciduous and permanent molars and delayed tooth eruption, dental caries, and absent vestibular sulcus. Additionally, a whitish lobulated nodule located in the alveolar ridge in the anterior region of the mandible was noted. Anatomopathological examination was compatible with the diagnosis of peripheral odontogenic fibroma (POF). In a 10-month clinical follow-up, no signs of recurrence were observed. CONCLUSION: In view of the characteristic oral findings of EVC syndrome and the potential for recurrence of POF, the pediatric dentist plays an essential role in clinical follow-up, planning and preventive, and rehabilitative treatment.

Luxación intrusiva en dentición primaria: reporte de caso / Luxação intrusiva na dentição decídua: relato de caso clínico / Intrusive luxation in primary dentition: clinical report

La luxación intrusiva es el tipo más común de lesión traumática en la dentición primaria, caracterizada por el desplazamiento del diente hacia el interior del alvéolo. Requieren minucioso examen clínico, buena radiografía y seguimiento del caso, siendo el prognóstico desfavorable, principalmente por la probabilidad de daño al germen del diente permanente. Este trabajo tiene como objetivo reportar un caso clínico de luxación intrusiva en dentición primaria, desde el diagnóstico hasta el seguimiento. Se trata de un paciente masculino, de 3 años, que acudió a la Emergencia de la Clínica de Especialidades Infantiles ­ Universidad Estatal de Londrina (UEL) por traumatismo dentoalveolar en la región antero-superior ocurrido hace tres días. En el examen físico, se observó que el diente 61 presentaba intrusión de grado II con un tercio de la corona visible y el diente 62 intrusión de grado I, con más de un tercio de la corona visible. Radiográficamente se observa inclinación vestibular, sin fractura de la placa ósea, por lo que se optó por un tratamiento conservador, a la espera de la reerupción espontánea de los dientes afectados. Se informó a los padres sobre posibles consecuencias de este tipo de traumatismo y la necesidad de consultas de seguimiento clínico y radiológico. Después de ocho meses de seguimiento, los dientes erupcionaron satisfactoriamente. Es de suma importancia establecer un protocolo de observación y control del diente en el cual se ha identificado el trauma, observando signos y síntomas de los tejidos involucrados en el trauma para reducir secuelas en ambas denticiones

A luxação intrusiva é o tipo de lesão traumática mais frequente na dentição decídua, caracterizada pelo deslocamento do dente para o alvéolo. As intrusões requerem exame físico minucioso, radiografias de valor diagnóstico e acompanhamento do caso, pois o prognóstico é desfavorável, principalmente pela probabilidade de danos ao germe dentário permanente. Este trabalho tem como objetivo relatar um caso clínico de luxação intrusiva na dentição decídua, desde o diagnóstico até o acompanhamento. Trata-se de paciente do sexo masculino, 3 anos de idade, atendido no Pronto Socorro do Ambulatório de Especialidades Infantis da Universidade Estadual de Londrina (UEL) devido a trauma dento-alveolar em região ântero-superior ocorrido há três dias. Ao exame físico intraoral, observou-se que o dente 61 apresentava intrusão grau II (um terço da coroa visível) e o dente 62 apresentava intrusão grau I, com mais de um terço da coroa visível. Radiograficamente foi observada inclinação vestibular, sem fratura da tábua óssea. Assim, optou-se pelo tratamento conservador, aguardando a reerupção espontânea dos dentes envolvidos. Além disso, os pais foram informados sobre as possíveis consequências deste tipo de trauma e a necessidade de consultas de proservação clínica e radiográfica. Após oito meses de acompanhamento, os dentes erupcionaram satisfatoriamente. É de extrema importância estabelecer um protocolo de observação e controle do dente traumatizado, observando sinais e sintomas dos tecidos envolvidos no trauma para reduzir sequelas em ambas as dentições.

Intrusive dislocation is the most frequent type of traumatic injury in the primary dentition, characterized by displacement of the tooth into the alveolus. Intrusions require a thorough physical examination, radiographs of diagnostic value, and follow-up of the case, since the prognosis is unfavorable, mainly due to the probability of damage to the permanent tooth germ. This work aims to report a clinical case of intrusive dislocation in the primary dentition, from diagnosis to follow-up. This is a male patient, 3 years old, who attended in the Emergency of the Children's Specialty Clinic ­ State University of Londrina (UEL) due to dento-alveolar trauma in the antero-superior region that occurred three days ago. On clinical examination, tooth 61 had grade II intrusion (one-third of the crown visible), and tooth 62 with grade I intrusion, with more than one-third of the crown visible. Radiographically, a buccal inclination was observed, with no fracture of the bone plate. Thus, we opted for conservative treatment, waiting for the spontaneous re-eruption of the teeth involved. Furthermore, parents were informed about the possible consequences of this type of trauma and the need for clinical and radiographic follow-up consultations. After eight months of follow-up, the teeth erupted satisfactorily. It is extremely important to establish a protocol for observation and control of the traumatized tooth, observing signs and symptoms of the tissues involved in the trauma to reduce sequelae in both dentitions

Manifestaciones orales en paciente infantil con síndrome de Ellis-van Creveld: informe de caso / Manifestações orais em paciente infantil com síndrome de Ellis-van Creveld: relato de caso / Oral manifestations in child patient with Ellis-van Creveld syndrome: case report

El síndrome de Ellis-van Creveld es un trastorno autosómico recesivo caracterizado por una tétrada de enanismo desproporcionado, displasia ectodérmica, polidactilia postaxial y malformaciones cardíacas congénitas. En este artículo, presentamos el caso de un niño brasileño de 6 años con síndrome de Ellis-van Creveld que presentó un número notable de características orales y dentales clásicas y hallazgos poco comunes como taurodontismo. El examen clínico reveló hipoplasia múltiple del esmalte, surco vestibular ausente, aserraduras alveolares en la región anterior del maxilar, dientes ausentes, dientes cónicos, canino inferior girado, mordida cruzada posterior bilateral, caries dental y un nódulo. Radiográficamente se observa agenesia dentaria, taurodontismo de molares primarios y permanentes y retraso en la erupción dentaria. Los hallazgos clínicos y radiográficos pueden estar presentes desde el nacimiento y el odontopediatra tiene un papel fundamental en el diagnóstico precoz del síndrome de Ellis-van Creveld, así como en la prevención de problemas orales, rehabilitación e intervenciones estéticas.

A síndrome de Ellis-van Creveld é uma doença autossômica recessiva caracterizada por uma tétrade de baixa estatura desproporcional, displasia ectodérmica, polidactilia pós-axial e malformações cardíacas congênitas. Neste artigo, será relatado um caso de um menino brasileiro de 6 anos de idade com síndrome de Ellis-van Creveld que apresenta um número notável de características orais e dentárias clássicas e achados incomuns como taurodontismo. Ao exame clínico foi revelado hipoplasia múltipla do esmalte, sulco vestibular ausente, serrilhas alveolares na maxila anterior, dentes ausentes, dentes cônicos, canino inferior rotacionado, mordida cruzada posterior bilateral, cárie dentária e um nódulo. Radiograficamente, foi observado agenesia dentária, taurodontismo de molares decíduos e permanentes e atraso na erupção dentária. Os achados clínicos e radiográficos podem estar presentes desde o nascimento e o odontopediatra tem papel fundamental no diagnóstico precoce da síndrome de Ellis-van Creveld, bem como na prevenção de problemas bucais, reabilitação e intervenções estéticas.

Ellis­van Creveld syndrome is an autosomal recessive disorder characterized by a tetrad of disproportionate dwarfism, ectodermal dysplasia, postaxial polydactyly, and congenital heart malformations. In this article, we hereby present a case of a 6-year-old Brazilian boy with Ellis­van Creveld syndrome who presented with a remarkable number of classical oral and dental features and uncommon findings such as taurodontism. Clinical examination revealed multiple enamel hypoplasia, absent vestibular sulcus, alveolar serrations in the maxilla anterior region, missing teeth, conical teeth, lower canine rotation, bilateral posterior crossbite, dental caries, and a nodule. Radiographically were observed teeth agenesis, taurodontism of deciduous and permanent molars, and delayed tooth eruption. Clinical and radiographic findings may be present from birth and the pediatric dentist has a fundamental role in the early diagnosis of Ellis­van Creveld syndrome, as well as oral problems prevention, rehabilitation, and aesthetic interventions.

Relationship of levels of trace elements in saliva and dental caries in preschool children using total reflection X-ray fluorescence technique (TXRF)⋆.

BACKGROUND: Considering that studies on the relationship between dental caries and trace elements present contradictory and inconclusive results, the purpose of this study was to determine the levels of salivary trace elements in saliva samples of preschool children and investigate their relationship with dental caries. METHODS: In total, 120 samples of unstimulated saliva were collected from children aged 36-72 months, of both sexes, who participate in the preventative educational program in oral health at the State University of Londrina, Brazil. The children were divided into two groups, caries (n = 60) and non-caries (n = 60). Levels of Al, Cu, Fe, Mn, and Zn were analyzed by total reflection X-ray fluorescence (TXRF). Descriptive statistics, the Student's t-test, Mann-Whitney U test, and Pearson's Chi-squared test were performed (P < 0.05). RESULTS: The concentrations of Mn and Fe were significantly higher in the caries group (Mn =0.015 mg/L [0.007-0.020]; Fe =0.080 mg/L [0.031-0.239] than the non-caries group (Mn =0.010 mg/L [0.001 - 0.017]; Fe =0.044 mg/L [0.023 - 0.107]). CONCLUSION: The results suggest a relationship between trace elements and dental caries, indicating possible involvement of these elements in the metabolism of microorganisms involved in the carious process. In addition, the use of TXRF presented satisfactory results, with a simple and fast methodology for the detection of the studied elements.

Different DNA methylation profile is demonstrated in paracoccidioidomycosis patients without oral lesions.

BACKGROUND: Paracoccidioidomycosis (PCM) is a neglected fungal infection with a high impact on the quality of life of the affected patients. The disease presents primary pulmonary involvement and systemic dissemination may occur. About 50% of the cases show oral involvement, and the factors that lead to this manifestation are not clear. OBJECTIVES: The aim of this study was to evaluate the DNA methylation profile in PCM patients with oral lesions. MATERIAL AND METHODS: Genomic DNA was extracted from whole blood of eighteen PCM patients, being ten with oral lesions and eight with no oral lesion. Analysis of methylation profile was performed using the technique of methylation-sensitive arbitrarily primed PCR (MS-AP-PCR). The sequences of recombinant plasmids obtained were evaluated according to parameters that define a CpG island, as well as their relative position in the known human genome genes and/or CpG islands. RESULTS: After DNA amplification, three different expressed bands were observed between the two groups, being found in the samples of patients with no oral manifestations. The cloned fragment in the plasmid showed similarity with a DNA sequence present in chromosome 20, next to the YTHDF1 gene. Other bands showed homology with intronic region in the genes RBPMS2 and DPH6 and no CpG island was identified. CONCLUSIONS: DNA methylation was found in PCM patients with no oral lesion affecting the YTHDF1 gene. Further studies are necessary to elucidate to role of YTHDF1 gene in the oral PCM manifestations.