A rare LMNA missense mutation causing a severe phenotype of mandibuloacral dysplasia type A: a case report.

OBJECTIVE: To report the case of a girl presenting a severe phenotype of mandibuloacral dysplasia type A (MADA) characterized by prominent osteolytic changes and ectodermal defects, associated with a rare homozygous LMNA missense mutation (c.1579C>T). CASE DESCRIPTION: A 6-year-old girl was evaluated during hospitalization exhibiting the following dysmorphic signs: subtotal alopecia, dysmorphic facies with prominent eyes, marked micrognathia and retrognathia, small beaked nose, teeth crowding and thin lips, generalized lipodystrophy, narrow and sloping shoulders, generalized joint stiffness and bone reabsorption in the terminal phalanges. In dermatological examination, atrophic skin, loss of cutaneous elasticity, hyperkeratosis, dermal calcinosis, and hyperpigmented and hypochromic patches were observed. Radiology exams performed showed bilateral absence of the mandibular condyles, clavicle resorption with local amorphous bone mass confluence with the scapulae, shoulder joints with subluxation and severe bone dysplasia, hip dysplasia, osteopenia and subcutaneous calcifications. COMMENTS: MADA is a rare autosomal recessive disease caused by mutations in LMNA gene. It is characterized by craniofacial deformities, skeletal anomalies, skin alterations, lipodystrophy in certain regions of the body and premature ageing. Typical MADA is caused by the p.R527H mutation in the LMNA gene. However, molecular analysis performed from oral epithelial cells obtained from the patient showed the rare mutation c.1579C>T, p. R527C in the exon 9 of LMNA. This is the sixth family identified with this mutation described in the literature.

A rare LMNA missense mutation causing a severe phenotype of mandibuloacral dysplasia type A: a case report / Uma rara mutação missense no gene LMNA causando um fenótipo grave de displasia mandibuloacral tipo A: relato de caso

ABSTRACT Objective: To report the case of a girl presenting a severe phenotype of mandibuloacral dysplasia type A (MADA) characterized by prominent osteolytic changes and ectodermal defects, associated with a rare homozygous LMNA missense mutation (c.1579C>T). Case description: A 6-year-old girl was evaluated during hospitalization exhibiting the following dysmorphic signs: subtotal alopecia, dysmorphic facies with prominent eyes, marked micrognathia and retrognathia, small beaked nose, teeth crowding and thin lips, generalized lipodystrophy, narrow and sloping shoulders, generalized joint stiffness and bone reabsorption in the terminal phalanges. In dermatological examination, atrophic skin, loss of cutaneous elasticity, hyperkeratosis, dermal calcinosis, and hyperpigmented and hypochromic patches were observed. Radiology exams performed showed bilateral absence of the mandibular condyles, clavicle resorption with local amorphous bone mass confluence with the scapulae, shoulder joints with subluxation and severe bone dysplasia, hip dysplasia, osteopenia and subcutaneous calcifications. Comments: MADA is a rare autosomal recessive disease caused by mutations in LMNA gene. It is characterized by craniofacial deformities, skeletal anomalies, skin alterations, lipodystrophy in certain regions of the body and premature ageing. Typical MADA is caused by the p.R527H mutation in the LMNA gene. However, molecular analysis performed from oral epithelial cells obtained from the patient showed the rare mutation c.1579C>T, p. R527C in the exon 9 of LMNA. This is the sixth family identified with this mutation described in the literature.

RESUMO Objetivo: Relatar o caso de uma jovem que apresentava um fenótipo grave de displasia mandibuloacral tipo A (MADA) caracterizado por alterações osteolíticas proeminentes e defeitos ectodérmicos, associados a uma rara mutação homozigótica missense no gene LMNA (c.1579C>T). Descrição do caso: Uma menina de seis anos foi avaliada durante hospitalização apresentando os seguintes sinais dismórficos: alopecia subtotal, fácies dismórfica com olhos proeminentes, micrognatia e retrognatia acentuada, nariz pequeno e adunco, dentes apinhados e lábios finos, lipodistrofia generalizada, ombros estreitos e inclinados, rigidez articular e reabsorção óssea nas falanges terminais. Ao exame dermatológico, observou-se pele atrófica, perda da elasticidade cutânea, hiperceratose, calcinose dérmica e manchas hiperpigmentadas e hipocrômicas. Exames radiológicos realizados mostraram ausência de côndilos mandibulares bilaterais, reabsorção da clavícula com massa óssea amorfa local confluindo com as escápulas, articulações do ombro com subluxação e displasia óssea severa, com displasia coxofemoral, osteopenia e calcificações subcutâneas. Comentários: MADA é uma doença autossômica recessiva rara causada por mutações no gene LMNA. Caracteriza-se por deformidades craniofaciais, anomalias esqueléticas, alterações cutâneas, lipodistrofia em determinadas regiões do corpo e envelhecimento precoce. MADA típica é causada pela mutação p.R527H no gene LMNA. No entanto, a análise molecular realizada com células epiteliais orais obtidas da paciente mostrou a mutação rara c.1579C>T, p. R527C no exon 9 do gene LMNA. Esta é a sexta família identificada com essa mutação descrita na literatura.

Soil chemical attributes in areas under conversion from forest to pasture in southern Brazilian Amazon.

The south of the Brazilian Amazon is one of the largest cattle-producing regions in Brazil, however, most of the pastures are in low fertility soils. Thus, cattle breeders compensate for the low production of pastures, increasing the size of the areas, generating more deforestation and burning. These practices increase the chemical degradation process of Amazonian soils, making them increasingly infertile when improperly managed. With this, the objective of the work was to evaluate the impacts caused in the chemical attributes of soils, in areas under forest-to-pasture conversion, in the south of the Brazilian Amazon. The study was carried out in the district of União Bandeirantes, in an area of forest and two areas with pastures (brachiaria and mombaça grass). In the field, soil samples were collected at two depths (0.00-0.10 and 0.10-0.20 m), to carry out chemical analyzes. Further, uni, bi and multivariate statistical analyzes were carried out, besides geostatistical analyzes were carried out to study spatial variability and management zones. The conversion of forest to pasture increased the pH and exchangeable bases levels, reducing the availability of exchangeable aluminum and potential acidity, however, it induces losses of phosphorus and organic carbon from the soil. Among the pasture environments, the mombaça grass area presented higher fertility. Greater spatial variability of chemical attributes was observed in the environment with mombaça grass, indicating greater heterogeneity in the distribution of attributes in the area. We attribute this behavior to the higher grazing intensity and the micro-reliefs in the area that direct the flow of water and nutrients.

Seasonality, soil attributes and root biomass in cerrado, cerradão and forest environments, western Amazon

The Amazon rainforest region presents a phytophysiognomy that ranges from savannas to cerrados, all of them intimately associated to climate and soil characteristics. Evidence has been given that plant growth and development are affected by soil quality and seasonality, thus making it crucial to understand them and how they are related to each other in order to grasp the dynamics of the whole ecosystem. In this context, the goal of this research was to assess how seasonality, soil attributes, and root system biomass are related in natural cerrado, cerradão, and forest areas in southern Amazonas State, in Brazil. Soil samples were collected during dry (June/2018) and rainy (December/2018) seasons from three different layers 0.00­0.05m; 0.05­0.15m, and 0.15­0.30m deep. In each area ten sampling points were randomly chosen. Two kinds of soil samples were collected: the first using 4.0 cm height by 5.1cm internal-diameter soil sample rings; and the second were intact soil lumps. Physical and Chemical soil attributes assessed were macro-porosity (MaP), micro-porosity (MiP), total porosity (TP), soil density (SD), aggregates texture and stability (GMD and WAR), gravimetric humidity (HG), organic carbon (OC), exchangeable aluminum (Al3+), potential acidity (H+Al), sum of bases (SB), cation exchange capacity (CEC), and root biomass (RB). All data were analysed via Tukey t test and student T test to compare results between seasons and areas. Increasing vegetation density (cerrado < cerradão < forest) was followed by an increment in CEC and OC, showing the importance of these attributes to maintaining biodiversity in environments. In amazon cerrado, rainy season as well a sandier soil textures provided favourable conditions to the growth and development of plants' root system. Soil attributes were little affected by seasonality, that had greater effect on MiP, TP, SD, and OC, leading to lesser values for these variables during rainy season.

Physical soil attributes in areas under forest/pasture conversion in northern Rondônia, Brazil.

The main cause of physical degradation in pasture areas is overgrazing, and when combined with poorly productive soils, it causes the loss of millions of hectares of agricultural soils a year. Thus, work is needed to indicate which physical attributes are most sensitive to degradation, generating information so that soil management can be proposed, with a view to economic, social, and environmental aspects. Therefore, the objective of the work was to evaluate the impacts caused on the physical attributes of the soil, in forests converted to pastures in northern Rondônia, Brazil. The study was carried out in three areas within the municipality of Porto Velho, Rondônia, one area with forest and two with pastures (brachiaria and mombaça grass). In the field, deformed soil samples were collected at a depth of 0.00-0.10 and 0.10-0.20 m in the three study areas. In the laboratory, physical analyses of texture, aggregates and porosity, compaction, and an additional analysis of soil organic carbon were carried out. Then, univariate, bivariate, and multivariate analyses were performed, as well as geostatistical analysis. The conversion of forest to pasture had a negative impact on aggregates, compaction, porosity, and accumulation of organic carbon in the soil. The studied environments are influenced by the high levels of sand and clay, which interfere in the aggregation, compaction, porosity, and accumulation of organic carbon in the soil. We observed greater spatial variability of physical attributes in the environment with mombaça grass and attributed this to the greater grazing and trampling intensity of the animals.

Correction: Health conditions associated with overweight in climacteric women.

[This corrects the article DOI: 10.1371/journal.pone.0218497.].

Health conditions associated with overweight in climacteric women.

This study aims to investigate the association between health conditions and overweight in climacteric women assisted by primary care professionals. It is a cross-sectional study conducted with 874 women from 40 to 65 years of age, selected by probabilistic sampling between August 2014 and August 2015. In addition to the outcome variable, overweight and obesity, other variables such as sociodemographic, reproductive, clinical, eating and behavioural factors were evaluated. Descriptive analyses of the variables investigated were performed to determine their frequency distributions. Then, bivariate analyses were performed through Poisson regression. For the multivariate analyses, hierarchical Poisson regression was used to identify factors associated with overweight and obesity in the climacteric period. The prevalence of overweight and obesity was 74%. Attending public school (PR: 1.30-95% CI 1.14-1.50), less schooling (PR: 1.11-95% CI 1.01-1.23), gout (PR: 1.18-95% CI 1.16-1.44), kidney disease (PR: 1.18-95% CI 1.05-1.32), metabolic syndrome (MS) (PR: 1.19-95% CI 1.05-1.34) and fat intake (PR: 1.12-95% CI 1.02-1.23) were considered risk factors for overweight. Having the first birth after 18 years of age (PR: 0.89-95% CI 0.82 to 0.97) was shown to be a protective factor for overweight and obesity. The presence of overweight and obesity is associated with sociodemographic, reproductive, clinical and eating habits.

Soils characterization and classification in clean field, dirty field and forest areas in amazonian environments / Caracterização e classificação de solos em área de campo limpo, campo sujo e floresta em ambientes amazônicos

The soil and vegetation characteristics of the southern Amazonas region include highly weathered soils, high aluminum content and some hydromorphic conditions, its vegetation is composed from grasslands to small isolated trees and forest galleries along the rivers streams. In this way, this work aims to characterize and classify the soil in areas of clean field, dirty field, and forest in Humaitá region AM. Soil trenches were opened in the clean field, dirty field, and forest environments, soil profiles were morphologically characterized, and samples were collected from their horizons. Physical analysis of texture, dispersed clay in water, flocculation, bulk density, particle density and porosity were performed. The chemical analyzes included pH and KCl in water; Ca, Mg, K, Al, and; available P; H+Al and organic C; SiO2, Al2O3 and Fe2O3 sulfuric attack. The soils were classified according to criteria established by the Brazilian System of Soil Classification and Soil Taxonomy. The forest, dirty field (high) and clean field (low) showed different soil types, Typic Dystrudept for the first two environments and Typic Fluvaquents for last. Multivariate techniques expressed the similarity relations presenting between the different environments studied, characterizing, which are of great importance in the relation landscape-soil studies.

As características do solo e da vegetação da região sul do Amazonas incluem solos altamente intemperizados, alto teor de alumínio e algumas condições hidromórficas, sendo sua vegetaçãocomposta por pastos, pequenas árvores isoladas e galerias florestais ao longo dos cursos d'água. Desta forma, este trabalho tem como objetivo caracterizar e classificar o solo em áreas de campo limpo, campo sujo e floresta na região de Humaitá AM. As valas foram abertas no campo limpo, no campo sujo e nos ambientesflorestais, os perfis dos solos foram caracterizados morfologicamente e as amostras foram coletadas de seus horizontes. Análises físicas de textura, argila dispersa em água, floculação, densidade do solo, densidade de partículas e porosidade foram realizadas. As análises químicas incluíram pH e KCl em água; Ca, Mg, K, Al e; P disponível; H + Al e C orgânico; Ataque sulfúrico de SiO2, Al2O3 e Fe2O3. Os solos foram classificados de acordo com critérios estabelecidos pelo Sistema Brasileiro de Classificação de Solos e Taxonomia de Solos. A mata, o campo sujo (alto) e o campo limpo (baixo) apresentaram diferentes tipos de solo, distritos típicos para os dois primeiros ambientes e fluídicos típicos para o último. Técnicas multivariadas expressaram as relações de similaridade que se apresentam entre os diferentes ambientes estudados, caracterizando, que são de grande importância na relação paisagem-solo.

Physical and chemical properties of soils in different physiographic environments in the southern Amazonas region / Atributos físicos e químicos de solos em diferentes ambientes fisiográficos na região sul do Amazonas, Brasil

The Amazon region has a great diversity of landscapes such as forests galleries, natural fields ("Cerrados Amazônicos"), dense forest, these environments made possible the formation of a broad class of soils over time. The aim of this study was to evaluate the physical and chemical properties of soils in different physiographic environments in southern Amazonas, Brazil. Three areas of representative physiographies were selected, all of them in natural conditions: natural field / forest and floodplain / dry land transitions, and corrugated relief areas. Soil samples were collected in layers of 0.0 to 0.20 and 0.80-1.0 m. From the samples collected the following physical analyzes were performed: particle size, bulk density, particle density, total porosity and saturated hydraulic conductivity; and chemical: exchangeable calcium, magnesium, aluminum and potassium available, phosphorus, potential acidity, pH and organic carbon. Based on the results of chemical analysis were calculated the sum of bases and base saturation. The results were submitted to multivariate statistics analysis, at the discretion of the principal component analysis (PCA). From the results it is clear that different physiographic environments studied influence the formation of different soil classes, featuring the diversity of Amazonian soils. The PCA allowed the distinction and formation of differentsimilarity groups, thus enabling to relate the physical and chemical properties with the physiographic formation in which they are inserted.

A região amazônica possui uma grande diversidade de paisagens, como galerias de florestas, campos naturais (Cerrados Amazônicos), floresta densa, possibilitando a formação de uma ampla classe de solos ao longo do tempo. O objetivo deste estudo foi investigar as propriedades físicas e químicas de solos em diferentes ambientes fisiográficos no sul do Amazonas, Brasil. Três áreas de fisiografias representativas foram selecionadas, todas em condições naturais: campo natural / floresta e transições de várzea / terra firme e áreas de relevo corrugado. Amostras de solo foram coletadas em camadas de 0,0 a 0,20 e 0,80-1,0 m. Das amostras coletadas foram realizadas as seguintes análises físicas: tamanho de partícula, densidade do solo, densidade de partículas, porosidade total e condutividade hidráulica saturada; e químico: cálcio trocável, magnésio, alumínio e potássio disponíveis, fósforo, acidez potencial, pH e carbono orgânico. Com base nos resultados da análise química foram calculados a soma das bases e saturação por bases. Os resultados foram submetidos à análise estatística multivariada, a critério da análise de componentes principais (PCA). A partir dos resultados, percebe-se que diferentes ambientes fisiográficos estudados influenciam a formação de diferentes classes de solos, caracterizando a diversidade de solos amazônicos. O PCA permitiu a distinção e formação de diferentes grupos de similaridade, permitindo relacionar as propriedades físico-químicas com a formação fisiográfica em que estão inseridos.

Quality of life in climacteric women assisted by primary health care.

This cross-sectional study evaluated the quality of life and associated factors of climacteric women in Brazil using a random and representative sample of women assisted by primary care professionals. We investigated the variables using the Menopause-Specific Quality of Life Questionnaire, MENQOL, whose mean scores were compared using Mann-Whitney and Kruskal-Wallis tests according to the sample characteristics. The variables associated with the outcomes in univariate analyses with a p≤0.2 were jointly evaluated using multiple linear regression. In this study, 849 women ranging in age from 40 to 65 years were evaluated. The predictors of poor quality of life in the vasomotor domain were women with severe climacteric symptoms (p<0.001), increased Body Mass Index (BMI) (p = 0.006), sleep (p = 0.022), and postmenopausal (p<0.001) alterations. For the psychosocial domain, the associated variables were severe climacteric symptoms (p<0.001) and sleep alterations (p<0.001); for the physical domain, the associated variables were severe climacteric symptoms (p<0.001), increased BMI (p<0.001), sleep (p<0.001), and postmenopausal (p<0.001) alterations. Severe climacteric symptoms, low sleep quality, increased BMI, and postmenopausal status were factors that were more associated with impairments in quality of life. With the increase in life expectancy, we suggest that greater attention should be paid to women's quality of life associated with climacteric symptoms.

Terapia de fotobiomodulação no tratamento das lesões orais da síndrome de Stevens-Johnson: relato de caso / Photobiomodulation therapy in the treatment of Stevens-Johnson syndro-me oral lesions: case report

Introdução: A Síndrome de Stevens-Johnson é uma reação rara que se manifesta por exantema que evolui para um extenso deslocamento epidérmico. Na maioria dos casos, há o surgimento de lesões dolorosas em mucosa oral, que podem reduzir a ingestão de nutrientes e dificultar a higiene bucal. A terapia de fotobiomodulação pode ter um importante papel antálgico, na modulação da inflamação e na cicatrização dessas lesões. Objetivo: Relatar um caso clínico no qual as lesões orais da Síndrome de Stevens-Johnson foram tratadas com o auxílio da terapia de fotobiomodulação. Relato de caso: Paciente de 17 anos, do sexo feminino, com diagnóstico de síndrome de Stevens-Johnson, apresentava-se com edema labial, úlceras orais, febre e odinofagia intensa, com tolerância apenas para dieta líquida. Encontrava-se com redução da ingestão alimentar, sem comunicação oral eficiente e com interrupção da higiene bucal devido a dor intensa. A terapia de fotobiomodulação foi utilizada no tratamento das lesões orais. Conclusão: A terapia de fotobiomodulação auxiliou na redução da dor, favoreceu a ingestão de alimentos por via oral e reduziu o tempo de cicatrização das lesões.

Introduction: Stevens-Johnson Syndrome is a rare reaction manifested by a rash that evolves into extensive epidermal dislocation. In most cases there are painful oral mucosal lesions that can reduce nutrient intake and make oral hygiene difficult. Photobiomodulation therapy can play an important antalgic role in modulating inflammation and healing these lesions. Objective: To report a clinical case in which Stevens-Johnson Syndrome oral lesions were treated with the aid of photobiomodulation therapy. Case Report: A 17-year-old female patient diagnosed with Stevens-Johnson syndrome presented with lip edema, oral ulcers, fever, and severe odynophagia, tolerating only a liquid diet. She experienced reduced food intake, no efficient oral communication, and an interruption of oral hygiene due to intense pain. Photobiomodulation therapy was requested to treat the oral lesions. Conclusion: Photobiomodulation therapy helped reduce pain, favored oral food intake, and reduced lesion healing time.

Dental age assessment: Which is the most applicable method?

To evaluate which method is the most applicable for estimating estimate dental age in Brazilian children, Nolla or Demirjian. For this purpose, both methods were reviewed and the differences between them brought to light. A total of 403 healthy children between the ages of 7 and 13 years with satisfactory panoramic radiographs, similar socio-economic background and ethnic origin were included. Panoramic radiographs were examined by the Nolla and Demirjian methods. Dental age was calculated for both methods, and the differences between the dental age and the chronological age were compared with analysis of variance with post hoc Dunnett. The Nolla method showed no significant difference in relation to chronological age in the majority of age groups for boys and girls, except for 12-year-old boys (over-estimation of 1.00) and between 11- and 12-year-old girls (over-estimation of 0.51 and 0.59, respectively). An over-estimation of the dental age was observed by using the Demirjian method (for boys 0.89-1.84 and for girls 0.69-1.97) for all age groups. The Nolla method is suitable for Brazilian children when it comes to age estimate with care to growth spurt beginning (around 11 and 12 years). However, the Dermijian method should not be used, because it over-estimated the age in both sexes.

Metachronous ameloblastic fibro-odontoma and dentigerous cyst in the posterior mandible.

An ameloblastic fibro-odontoma (AFO) is a rare mixed odontogenic tumor with histologic features of an ameloblastic fibroma in conjunction with the presence of dentin and enamel. It usually appears as a well-circumscribed radiolucency with radiopaque foci and slow growth and is commonly seen in children and young adults. A 13-year-old boy presented with an asymptomatic swelling in the posterior right region of the mandible and the right ascending ramus. The clinical, imaging, and histopathologic findings confirmed the diagnosis of an AFO. After 8 months, a radiolucent lesion involving the unerupted mandibular left third molar was observed; a final diagnosis of a dentigerous cyst (DC) was established for this lesion. Although coincidental events, metachronous odontogenic lesions suggest a possible common genetic origin, since both can be caused by related cellular signaling pathways. Complete enucleation is recommended for both AFOs and DCs; rates of recurrence are low.

Enamel-renal syndrome in 2 patients with a mutation in FAM20 A and atypical hypertrichosis and hearing loss phenotypes.

Enamel-renal syndrome (OMIM #204690) is an uncommon disorder characterized by amelogenesis imperfecta and nephrocalcinosis and is caused by mutations in FAM20 A. We report 2 patients with enamel-renal syndrome who exhibited the typical features of this syndrome and a homozygous nonsense mutation in the FAM20 A gene (c.406 C>T), genetically confirming the diagnosis. They also exhibited 2 undescribed clinical features, hypertrichosis and hearing loss. Alterations in genes frequently associated with nonsyndromic hearing loss in the Brazilian population, including connexin 26 (GJB2), connexin 30 (GJB6) and mitochondrial 12 S rRNA (m.A1555 G mutation), were not found. These results suggest a putative function of FAM20 A in the development of the inner ear and in the formation of hair. The presence of nephrocalcinosis is a risk factor for renal impairment, and it is important to perform regular renal monitoring in order to avoid renal failure.

Ultrastructural evaluation of gingival connective tissue in hereditary gingival fibromatosis.

OBJECTIVE: To describe the ultrastructural features of hereditary gingival fibromatosis (HGF) in affected family members and compare microscopic findings with normal gingival (NG) tissue. STUDY DESIGN: Gingival tissue samples from nine patients with HGF from five unrelated families were evaluated by transmission electron microscopy. Nine NG tissue samples were used for comparison. RESULTS: Areas containing collagen fibrils forming loops and folds were observed in both groups, whereas oxytalan fibers were frequently identified in the HGF group. The diameter of collagen fibrils and the interfibrillar space among them were more uniform in the NG group than in the HGF group. Fibroblasts were the most common cells found in both the HGF and NG groups and exhibited enlarged, rough endoplasmic reticulum, mitochondria with well-preserved crests, conspicuous nucleoli, and euchromatic chromatin. Other cells, such as mast cells, plasma cells, and macrophages, were also observed. CONCLUSIONS: HGF tissues had ultrastructural characteristics that were very similar to those of NG tissues. Oxytalan fibers were observed more frequently in the HGF samples than in the NG samples. Other studies of HGF in patients from different families should be performed to better understand the pathogenesis of this hereditary condition.

Influence of the interocclusal splint in condylar position of patientswith tmd: a cone beam computed tomography study / Influência da placa interoclusal na posição condilar de indivíduos com dtm: um estudo por tomografia de feixe cônico

Objective: The aim of this study was to evaluate the condylar position in patients with intra-articular temporomandibular disorders (TMD) before, during and after treatment with interocclusal stabilization splint (ISS) through cone beam computed tomography (CBCT). Material and Methods: Twenty-two patients diagnosed with intra-articular TMD (Research Diagnostic Criteria for Temporomandibular Disorders - Group II) were submitted to the therapy with ISS during 90 days. Three CTCB exams were performed in three moments: T1 (initial moment, before the ISS therapy, with the patient in dental occlusion), T2 (after 90 days of treatment, in occlusion on the ISS) and T3 (after 90 days of treatment, with the patient in dental occlusion). Afterwards the anterior (AS), superior (SS) and posterior articular spaces (PS) in sagittal sections were then measured and the data were statistically analyzed using the t-test. Results: There was a statistically significant increase in the comparison between T1 and T2 for the AS and PS (p < 0.0001), between T3 and T2 for AS (p = 0.0008) and PS (p < 0.0001). In comparison T1 /T3 there was a significant increase in AS (p = 0.01) and SS (p = 0.04), and non-significant in PS (p = 0.89). Conclusion: The interocclusal splint provides temporary changes in condylar position, with a tendency to increase the joint space, varying in accordance with individual characteristics. Therefore, it should not be used as a single therapy, but combined with other strategies that include the TMD multidimensionality. The interocclusal splint has proved to be reversible and conservative therapeutic modality, as it does not generate permanent changes in joint tissues.

Objetivo: O objetivo deste estudo foi avaliar a posição condilar em pacientes com disfunção temporomandibular (DTM) articular antes e após tratamento com placa oclusal estabilizadora (POE) por meio de tomografia computadorizada de feixes cônicos (TCFC). Material e Métodos: Vinte e dois pacientes diagnosticados com DTM articular (Research Diagnostic Criteria for Temporomandibular Disorders – Grupo II) foram submetidos à terapia com POE durante 90 dias. Foram realizados três exames de TCFC: T1 (momento inicial, antes da terapia com POE, na posição de oclusão dental), T2 (após 90 dias de tratamento em oclusão na POE) e T3 (após 90 dias de tratamento, em oclusão dental). Foram então mensurados o espaço articular anterior (EAA), superior (EAS) e posterior (EAP) em cortes sagitais. Os dados foram analisados estatisticamente por meio do teste t. Resultados: Houve aumento estatisticamente significante na comparação entre T1 e T2 para o EAS e EAP (p < 0,0001), entre T3 e T2 para EAS (p = 0,0008) e EAP (p < 0,0001). Na comparação entre T1 e T3 houve aumento significante no EAA (p = 0,01) e EAS (p = 0,04), e não significante no EAP (p = 0,89). Conclusão: A placa interoclusal promove alterações temporárias na posição condilar, com tendência de aumentar o espaço articular, variando de acordo com características individuais. Portanto, ela não deve ser utilizada como terapia única, e sim combinada com outras estratégias que abordem a multidimensionalidade da DTM. A placa interoclusal provou ser uma terapia reversível e conservadora, já que não gera alterações permanentes nos tecidos articulares.

Taurodontism in patients with nonsyndromic cleft lip and palate in a Brazilian population: a case control evaluation with panoramic radiographs.

OBJECTIVES: The purpose of this study was to evaluate the prevalence of taurodontism in patients with nonsyndromic cleft lip and palate (NSCLP) within a Brazilian population. STUDY DESIGN: The study was designed as an epidemiologic case-control single-center study. Three hundred eighty-eight patients were included: 88 had NSCLP, and 300 comprised the control group. The first and second permanent mandibular molars were included in this study. By using panoramic radiographs, taurodontism was categorized as mesotaurodontism, hypotaurodontism, and hypertaurodontism. RESULTS: Seventy patients (23.3%) from the control group and 36 patients (40.9%) from the case group presented taurodontism (P < .001). In the control group, 108 (9%) teeth showed taurodontism, whereas in the case group with cleft lip and palate (CLP), 64 (18.2%) teeth showed dental anomalies (P < .001). In both groups, most taurodontic teeth presented hypotaurodontism, followed by mesotaurodontism, while hypertaurodontism was found in only two teeth. The probability of taurodontism in patients with cleft lip (CL) was 2.36 (P = .010) times higher compared with those with CLP, whereas the occurrence of taurodontism in patients with cleft palate (CP) was 3.15 (P = .002) times greater than in patients with CLP. CONCLUSIONS: The results from this study indicate a close relationship between taurodontism and NSCLP and the possibility of different cleft subphenotypes.

Aspectos tomográficos de ameloblastoma multicístico de células granulares: relato de caso / Computed tomography imaging features of multicystic granular cell ameloblastoma: a case report

O ameloblastoma multicístico é uma variante clínica do ameloblastoma, muitas vezes localmente agressivo, com significante aumento de morbidade e mortalidade. O ameloblastoma é frequentemente assintomático, sendo identificado incidentalmente durante exames odontológicos de rotina. Trata-se de uma lesão de crescimento lento e persistente, podendo ser notada, em alguns casos, uma expansão gradual da mandíbula, produzindo assimetria facial. Ameloblastomas raramente apresentam um comportamento maligno. A avaliação clínica inclui uma revisão cuidadosa da história clínica do paciente, bem como exame físico da cabeça e pescoço. Modalidades de imagens radiográficas são úteis para localizar a lesão, avaliar suas dimensões e os efeitos sobre as estruturas adjacentes. O manejo da lesão representa um desafio para todos os profissionais envolvidos na área de cirurgia de cabeça e pescoço. Assim, o objetivo deste trabalho foi descrever as características tomográficas de um ameloblastoma multicístico de células granulares em um paciente do sexo masculino, leucoderma, de 40 anos de idade...

Multicystic ameloblastoma is a clinical variant of ameloblastoma. It is often locally aggressive with significant impact on patient morbidity and mortality. Ameloblastoma is often asymptomatic and is usually detected as an incidental finding during routine dental examinations. It presents slow and persistent growth; in some cases a gradual expansion of the jaw producing facial asymmetry may be noticed . Although the occurrence is rare, ameloblastomas can exhibit malignant behavior and metastasize. Clinical evaluation includes careful review of the patient's medical history and physical examination of the head and neck region. Radiographic imaging techniques are useful for locating the lesion, estimating its dimensions and assessing its effects on adjacent structures. Management of ameloblastoma is a challenge for all involved in the field of head and neck surgery. The aim of this paper was therefore to describe the tomographic features of a multicystic granular cell ameloblastoma in a 40-year-old white male patient...

Síndrome de Ellis-van Creveld: achados sistêmicos e orais / Ellis-van Creveld syndrome: systemic and oral findings

Aims: To present a case of Ellis-van Creveld syndrome highlighting the systemic and oral manifestations and expandthe phenotypic spectrum of the disorder.Case description: A 4-year-old female, with an initial diagnosis of Ellis-van Creveld syndrome, was referred for dentaltreatment. The patient had hexadactyly of the hands, thorax disproportionate to the shortened limbs, hypopigmentedand dry skin, short stature, a wide gap between the 1st and 2nd toes of the right foot and markedly dystrophic toenails.The oral manifestations were upper labial frenulum causing large vestibule and absence of diastema, labiogingivalfrenulum, enamel hypoplasia, conical teeth, claw-like cusp, oligodontia, microdontia and malocclusion.Conclusions: Ellis-van Creveld syndrome is one among several syndromes with oral manifestations that demandsthe participation of a multidisciplinary team to better patient assessing, treatment and monitoring. Dentists have theleading role in the control and treatment of the oral manifestations.

Objetivos: Apresentar um relato de caso da síndrome de Ellis-van Creveld, destacando as manifestações sistêmicas e orais e expandindo o espectro fenotípico da doença. Descrição do caso: Uma menina de 4 anos com diagnóstico inicial de síndrome de Ellis-van Creveld foi encaminhada para tratamento odontológico. A paciente apresentava hexadactilia das mãos, tórax encurtado e desproporcional aos membros, cabelo fino, pele hipopigmentada e seca, baixa estatura, espaçamento entre o primeiro e o segundo dedos do pé direito e unhas dos pés acentuadamente distróficas. As manifestações orais foram representadas por freio labial superior amplo causando ausência de vestíbulo e diastema, frênulos labiogengivais, hipoplasia do esmalte, dentes cônicos, cúspide em garra, oligodontia, microdontia e maloclusão. Conclusões: A síndrome de Ellis-van Creveld representa uma entre as diversas síndromes com manifestações orais que demandam a participação de uma equipe multidisciplinar para melhor avaliar, tratar e acompanhar os pacientes. O cirurgião dentista tem o principal papel no controle e tratamento das manifestações orais.