ABSTRACT Fog is an atmospheric phenomenon that reduces horizontal visibility to 1000 m or less. These phenomena affect different human activities, mainly those associated with air, maritime, and road transport. Rio Grande city, located in southern Brazil, has one of the main Brazilian ports, and fog occurrences at this location cause several inconveniences, such as the interruption of port activities and increases in operating costs. Thus, a better understanding of fog formation and dissipation, and the consequent improvement in its forecasts, can bring significant help to the port activities in that region. Therefore, this work seeks to evaluate the WRF model’s ability to simulate fog events in Rio Grande city. Four study cases of dense fogs were performed for this goal, as well as a sensitivity test to select the best vertical resolution configuration of the model. Meteorological data from the Rio Grande pilotage station and the Brazilian Coastal Monitoring System buoys were analyzed. Satellite images and synoptic charts were also analyzed for the study cases. The numerical simulations were performed using the WRF model with three nested domains, where 1 km was the highest horizontal resolution. The Fog Stability Index (FSI) and two estimates of horizontal visibility were evaluated. Simulations show promising results, highlighting the ability of the model to identify the fog occurrence in the area of interest and represent aspects of its dissipation process. Furthermore, it is noteworthy that the FSI and the evaluated visibility estimates were sensitive to the occurrence of the events.

RESUMEN La niebla es un fenómeno atmosférico que reduce la visibilidad horizontal a 1000 m o menos. Este tipo de fenómenos afecta diferentes actividades, especialmente las asociadas con los transportes aéreo, marítimo y terrestre. La ciudad de Rio Grande, ubicada en el sur de Brasil, posee uno de los principales puertos de Brasil, y la ocurrencia de niebla en este lugar provoca inconvenientes como la interrupción de las actividades portuarias y el aumento de los costos operativos. Así, una mejor comprensión de la formación y disipación de la niebla y la mejora de sus pronósticos pueden ayudar a las actividades portuarias en esta región. Por lo tanto, este trabajo busca evaluar la capacidad del modelo WRF para simular eventos de niebla en Río Grande. Para ello se seleccionaron cuatro estudios de caso de niebla intensa, así como una prueba de sensibilidad para seleccionar la mejor configuración de resolución vertical del modelo. Los datos meteorológicos fueron analizados desde la estación de pilotaje de Río Grande y las boyas del Sistema de Monitoreo Costero Brasileño. También se analizaron imágenes satelitales y gráficos sinópticos. Las simulaciones numéricas se realizaron utilizando el modelo WRF con tres dominios anidados, donde la resolución horizontal más alta fue de 1 km. Se evaluaron el índice de estabilidad de la niebla (FSI, por su sigla en inglés) y otras dos estimaciones de visibilidad horizontal. Las simulaciones arrojaron resultados prometedores, destacando la capacidad del modelo para identificar la ocurrencia de niebla en el área de interés, además de representar aspectos de su proceso de disipación. Además, el FSI y las estimaciones de visibilidad fueran sensibles a los eventos.

SUMMARY: The lateral pterygoid muscle (LPM) is intimately related with the temporomandibular joint (TMJ), playing an important role in its physiology. This makes it of interest to researchers who investigate temporomandibular disorders. The literature indicates that anatomical variations exist in the insertion of the superior fascicle of the LPM. Imaging and cadaver studies have revealed that the LPM may present an accessory fascicle. The study object was to carry out macroscopic analysis of the LPM, examining the origin and insertion of its superior and inferior fascicles. The study used 38 half-heads of adult individuals fixed in formaldehyde 10 %. To carry out macroscopic analysis of the LPM, an initial incision was made along the lower margin of the zygomatic arch; the origin of the masseter muscle was then dissected, separating its insertion on the lateral face of the mandibular ramus and retracting the muscle to posteroinferior. Two incisions were made on the zygomatic arch and the insertion of the temporal muscle on the coronoid process was identified with dissection pincers; it was dissected to gain access to the infratemporal fossa and the two fascicles of the LPM. The superior fascicle (SF) originated on the infratemporal face of the greater wing of the sphenoid, and on the superior third of the lateral face of the lateral lamina of the pterygoid process of the sphenoid in 26 samples. In 12 samples, it originated on the greater wing of the sphenoid and the infratemporal crest of the sphenoid. Type I insertion was found in 20 samples, Type II in 6 samples and Type III in 12 samples. In all the samples analysed, the inferior fascicle (IF) originated on the inferior two thirds of the lateral face of the lateral lamina of the pterygoid process and on the lateral face of the pyramidal process of the palatine, with insertion on the pterygoid fovea. The accessory fascicle (AF) of the LPM was present in 6 samples. The AF originated on the greater wing of the sphenoid in 2 cases and inferior to the superior fascicle in 4 cases; its insertion was on the capsular disc complex in all cases. The results obtained in our study contribute anatomical data on the LPM in Brazilian adult individuals, with evaluation of its insertion points.

RESUMEN: El músculo pterigoideo lateral (MPL) presenta íntima relación con la articulación temporomandibular (ATM) y desempeña un rol importante en la fisiología de esta articulación, despertando el interés de investigadores que se dedican al estudio de los trastornos temporomandibulares. La literatura señala que existen variaciones anatómicas del MPL, con respecto a la inserción del fascículo superior. Los estudios cadavéricos e imagenológicos han revelado que el MPL puede presentar un fascículo accesorio. El objetivo del estudio fue realizar un análisis macroscópico del MPL, examinando el origen e inserción de sus fascículos superior e inferior. Se utilizaron 38 hemicabezas de individuos adultos fijadas en formaldehído al 10 %. Para realizar el análisis macroscópico del MPL se realizó inicialmente una incisión a lo largo del margen inferior del arco cigomático, luego se seccionó el origen del músculo masétero separando su inserción en la cara lateral de la rama de la mandíbula, retrayendo al músculo en sentido posteroinferior. Se hicieron dos cortes en el arco cigomático y con pinzas de disección se identificó la inserción del músculo temporal en el proceso coronoides, el cual se seccionó para ingresar a la fosa infratemporal y acceder a ambos fascículos del MPL. El fascículo superior (FS) del MPL se originó en la cara infratemporal del ala mayor del esfenoides y en el tercio superior de la cara lateral de la lámina lateral del proceso pterigoides del esfenoides en 26 muestras. En 12 muestras se originó en el ala mayor del esfenoides y cresta infratemporal del esfenoides. Con relación a su inserción, se encontró el Tipo I en 20 muestras; el Tipo II en 6 muestras y el Tipo III en 12 muestras. En todas las muestras analizadas el origen del fascículo inferior (FI) del MPL fue en los dos tercios inferiores de la cara lateral de la lámina lateral del proceso pterigoides y en la cara lateral del proceso piramidal del palatino insertándose en la fóvea pterigoidea. El fascículo accesorio (FA) del MPL estuvo presente en 6 muestras. El FA se originó en el ala mayor del esfenoides en 2 casos e inferior al fascículo superior en 4 casos y su inserción, en el complejo disco capsular en todos los casos. Mediante los resultados obtenidos en nuestro estudio estamos aportando datos anatómicos en relación al MPL en individuos brasileños adultos, evaluándolo con respecto a sus lugares de inserción.

Abstract Semi-analytical equations are developed for aiding the process of designing terahertz graphene-based rectangular dipole antennas lying on glass substrates. It directly provides the dipole length required for obtaining resonance at a desired frequency since antenna width and graphene chemical potential are known. By using the finite-difference time-domain (FDTD) method, a large number of computational simulations were performed considering several combinations of antenna dimensions and chemical potential values. The simulation results were used along with graphene electrostatic scaling law combined with the least squares method to optimize the formulation coefficients. With the optimized coefficients, we obtain very satisfying accuracy levels. In the frequency range from 0.5 THz to 3.0 THz, the average relative absolute error is 1.50%, with maximum relative absolute error of 6.77%.

RESUMEN Introducción: Los instrumentos normativos de la profesión declaran la necesidad de incluir en los servicios de emergencia (SE) Enfermeros Especializados en Enfermería de Rehabilitación (EEER). En este contexto, se intentó comprender la relevancia de la contribución del EEER en el contexto actual de SE. Metodología: Estudio cualitativo, exploratorio y descriptivo, utilizando un grupo focal como técnica de recolección de datos, en una muestra intencional de ocho enfermeras expertas, cuatro de ellas en el área de urgencia y cuatro EEER. Resultados: Surgen dos categorías: organización del cuidado, con mayor relevancia atribuida a las subcategorías equipos y organización de los SE, con un peso de 18,0% y 12,8% respectivamente en la totalidad del discurso; y prestación de cuidados, con mayor relevancia atribuida a las subcategorías prevención de complicaciones y formación de la persona / cuidador para el alta, con un peso de 11,9% y 11,6%, respectivamente. Discusión: Corroborando los hallazgos de este estudio, la evidencia destaca la relevancia en los dominios de liderazgo y atención interprofesional, percibiéndose también ganancias a nivel respiratorio y motor. Conclusión: Los participantes identifican los beneficios de la integración de una EEER en los SE en la organización del servicio y en la prestación asistencial, destacando la complementariedad de competencias comunes y específicas, cumpliendo así los referentes de la profesión sobre la necesidad de dotar de estos contextos a estos profesionales.

RESUMO Introdução: Os instrumentos reguladores da profissão declaram a necessidade de se incluirem nos serviço de urgência (SU) Enfermeiros Especialistas em Enfermagem de Reabilitação (EEER). Neste contexto procurou-se compreender a relevância do contributo do EEER no contexto atual nos SU. Metodologia: Estudo qualitativo, exploratório e descritivo, com recurso a focus group como técnica de recolha de dados, numa amostra intencional de oito enfermeiros peritos, quatro deles na área da urgência e quatro EEER. Resultados: Duas categorias emergem: organização dos cuidados, com maior relevância atribuída às subcategorias equipas e organização dos SU, com um peso 18,0% e 12,8% respetivamente na totalidade do discurso; e prestação dos cuidados, com maior relevância atribuída às subcategorias prevenção de complicações e capacitação da pessoa/cuidador para a alta, com um peso 11,9% e 11,6% respetivamente. Discussão: Corroborando os achados deste estudo, a evidência salienta a relevância nos domínios da liderança e cuidados interprofissionais, havendo igualmente ganhos percebidos a nível respiratório e motor. Conclusão: Os participantes identificam ganhos da integração do EEER no SU na organização do serviço e na prestação de cuidados, evidenciando a complementaridade entre competências comuns e específicas, indo assim ao encontro dos referenciais da profissão sobre a necessidade de dotar estes contextos destes profissionais.

ABSTRACT Introduction: The profession's regulatory instruments declare the need to include in the emergency services rehabilitation nurses. In this context, an attempt was made to understand the relevance of the contribution of rehabilitation nurses in the current context in emergency services. Methodology: Qualitative, exploratory and descriptive study, using a focus group as a data collection technique, in an intentional sample of eight expert nurses, four of them in the urgency area and four rehabilitation nurses. Results: Two categories emerge: organization of care, with greater relevance attributed to the subcategories teams and organization of the emergency services, with a weight of 18.0% and 12.8%, respectively, in the totality of the discourse; and provision of care, with greater relevance attributed to the subcategories prevention of complications and training of the person / caregiver for discharge, with a weight of 11.9% and 11.6%, respectively. Discussion: Corroborating the findings of this study, evidence highlights the relevance of rehabilitation nursing in the domains of leadership and interprofessional care, with gains also perceived at respiratory and motor levels. Conclusion: The participants identify gains from the integration of rehabilitation nurses in emergency services in the organization of the service and in the provision of care, highlighting the complementarity of common and specific competences, thus meeting the profession's references on the need to provide these contexts for these professionals.

SUMMARY: The lateral pterygoid muscle (LPM) is one of the muscles involved in jaw movements, and is therefore of great importance in the physiology of the temporomandibular joint. This muscle has classically been considered to have two heads, superior and inferior, however previous studies have indicated the presence of a third head (TH). The object of this research was therefore to evaluate, through a study in cadavers, the presence of the third head of the LPM and its relation with the joint disc of the TMJ in adult individuals. The study used 30 half-skulls of adult individuals, 11 right side and 19 left side. The number of heads on each LPM was analysed, with the length and thickness of each. The Chi-squared, Mann-Whitney U, Kruskall-Wallis and Spearman's correlation coefficient tests were applied, with a significance threshold of 5 %. The TH of the LPM was present in 20 % of the samples. Statistically significant differences were found in the thickness of the superior head (SH) vs. the inferior head (IH) (p<0.001) and between TH vs. SH and TH vs. IH (p=0.010). No correlation was found between the thickness of the heads or in their lengths. The LPM most frequently presents two heads, superior and inferior. The TH is an anatomical variation that may be present in 20 % of cases. The IH usually presents the greatest thickness. The use of cadavers is a good method for analysing the presence of the TH and the morphometry of the various heads of the LPM.

RESUMEN: El músculo pterigoideo lateral (MPL) es uno de los músculos involucrados en los movimientos mandibulares y por consiguiente tiene una gran importancia en la fisiología de la articulación temporomandibular (ATM). Clásicamente se ha considerado como un músculo que presenta dos cabezas, una superior y otra inferior, sin embargo estudios anteriores han señalado la presencia de una tercera cabeza (TC). El objetivo fue evaluar, mediante estudio en cadáveres, la presencia de la tercera cabeza del MPL y su relación con el disco articular de la ATM de individuos adultos. Se utilizaron 30 hemicabezas de individuos adultos, 11 del lado derecho y 19 en el lado izquierdo. Se analizó el número de cabezas, longitud y grosor de cada cabeza. Se aplicaron las pruebas de chi-cuadrado, U-Mann-Whitney, Kruskall-Walis y coeficiente de correlación de Spearman, con umbral de significación de 5 %. La TC del MPL estuvo presente en 20 % de las muestras. Se encontraron diferencias estadísticas significativas para el grosor del la cabeza superior (CS) vs. cabeza inferior (CI) (p<0,001) y entre TC vs. CS y TC vs. CI (p=0,010). No se encontró correlación entre el grosor de las cabezas o para la longitud de las cabezas. El MPL se presenta más frecuentemente con dos cabezas, una superior y otra inferior. La TC es una variación anatómica que puede estar presente en un 20 % de los casos. La CI suele ser la que presenta mayor grosor. En cuanto a los métodos para análisis de presencia de la TC y morfometría de las distintas cabezas del MPL el uso de cadáveres representa una buena alternativa.

Abstract The Amazon region has high lightning occurrence rates, with many lightning hot spots. Different rivers cross the Amazon rainforests and the people that live there use these rivers as roads to go from one place to another. Every year, lightning deaths are reported in the Brazilian Amazon region. In 2018, a lightning discharge struck a small riverboat, which killed 4 people and injured 5 in the North region of Brazil. In this study, we investigate lightning fatalities in Amazon rivers and compute electric field distribution in a small riverboat due to a lightning strike using the finite-difference time-domain (FDTD) method. The results show that the electric field produced inside the boat is large enough to cause air breakdown. Additionally, we propose a cost-effective method to improve lightning safety for people that use small boats in the Amazon region.

Resumen Este estudio tiene como objetivo verificar la participación del incentivo fiscal otorgado a las empresas en el crecimiento del ingreso per cápita de Maranhão. Los programas analizados fueron: "PróMaralhão" y "Mais Empresas” y el período verificado fue entre 2010 y 2017. La metodología utilizada fue el análisis de datos sobre los efectos de los incentivos fiscales en la generación de empleo e ingresos del Producto Interno Bruto (PIB) producción per cápita disponible en los sitios web: Instituto Maranhense de Estudios Socioeconómicos y Cartográficos (IMESC), Instituto Brasileño de Geografía y Estadística (IBGE) e Instituto de Investigación de Economía Aplicada (IPEA). Los principales resultados son una relación positiva entre el crecimiento del empleo y los ingresos en el estado de Maranhão, y que la política adoptada por el gobierno en la concesión de incentivos fiscales, a través de los programas PróMarhões y Mais Empresas, ha contribuido al crecimiento económico, por lo tanto, ha generado algún desarrollo social y económico para sus ciudadanos.

Abstract This study aims to verify the participation of the tax incentive granted to companies in the growth of per capita income in Maranhão. The programs analyzed were: “PróMaralhão” and “Mais Empresas” and the period verified was between 2010 and 2017. The methodology used was the analysis of data on the effects of tax incentives on the generation of employment and income from the Gross Domestic Product (GDP)) production, per capita available on the websites: Instituto Maranhense de Socioeconomic and Cartographic Studies (IMESC), Brazilian Institute of Geography and Statistics (IBGE) and Institute of Applied Economics Research (IPEA). The main results are a positive relationship between the growth of employment and income in the state of Maranhão, and that the policy adopted by the government in the granting of tax incentives, through the PróMarhões and Mais Empresas programs has contributed to economic growth, consequently, has generated some social and economic development for its citizens.

Abstract Background Fabry disease (FD) is caused by pathogenic variants in the GLA gene. A143T and R118C variants are considered not disease causing. Patient-reported outcomes provide information concerning the effects of their disease but should be carefully analyzed in rare diseases. Objectives To evaluate pain, depression, sleep disturbances, disability and quality of life in A143T or R118C Brazilian subjects and compare to data published for classic FD patients. Methods Nineteen subjects - 8:11 male:female - were evaluated and answered the questionnaires: Brief Pain Inventory (BPI), Hamilton Depression Rating Scale, Pittsburgh Sleep Quality Index, Health Assessment Questionnaire Disability Index (HAQ-DI), Short-Form Health Survey 36 (SF-36). Lyso-Gb3 and residual enzyme activity were obtained. Results Alpha-galactosidase A activity was low in males. Lyso-Gb3 levels were normal in all subjects. Comparing A143T/R118C subjects and FD patients, BPI severity, BPI interference, HAQ-DI values were not different (p>0.05) whereas raw scores for physical functioning (p=0.01) and general health perception (p<0.01) favored A143T/R118C. Depression and sleep disturbances were similar between groups. Conclusions A143T/R188C subjects had normal lyso-Gb3 levels. Depression, sleep disturbances and disability were frequent and under-recognized. However, findings depicted in this study are nonspecific and should not be considered as ground for diagnosing Fabry disease.

OBJECTIVES: Fabry disease (FD) is an X-linked lysosomal disease caused by variants of the GLA gene; the formation of defective alpha-galactosidase A contributes to the accumulation of substrates in several organs. Chronic inflammation is thought to contribute to organ damage in FD patients. METHODS: In total, 36 classic FD patients (15 men/21 women) and 25 healthy controls (20 men/8 women) were assessed. The Mainz Severity Score Index (MSSI) was established after conducting interviews with the patients and chart review. Serum IL-6, IL-1β, and TNF-α levels were evaluated in both groups. RESULTS: The mean age (years) for FD patients was 43.1±15.4 and that for the controls was 47.4±12.2 (p>0.05). Twenty-two patients (59.5%) were treated with enzyme replacement therapy (ERT). Serum IL-6 and TNF-α levels were significantly higher in FD patients than in the controls. Patients treated with ERT had higher serum IL-6 and TNF-α levels than those not treated with ERT. There was no difference in the serum IL-1β levels between patients treated with ERT and those who were not. The MSSI scores in the patients were correlated with serum levels of IL-6 (r=0.60, p<0.001) and TNF-α (r=0.45, p<0.001). CONCLUSION: FD was associated with elevated serum levels of IL-6 and TNF-α in this cohort. The FD patients treated with ERT, particularly, women, exhibited higher levels of serum IL-6 and TNF-α than those not treated with ERT; the serum IL-6 and TNF-α levels were correlated with the MSSI scores reflecting greater disease burden.

Resumo A quantidade e intensidade dos incêndios florestais têm sido destaque mediante as perdas e os impactos sociais, ambientais e econômicos. Dessa forma, o objetivo desta pesquisa foi identificar o perfil dos incêndios assim como suas causas e correlacionar a influência dos elementos meteorológicos sob as ocorrências de incêndios em florestas de produção para direcionar o uso de recursos em técnicas e medidas de prevenção, além de estruturas de combate aos incêndios florestais. O estudo teve como subsídio o banco de dados da série histórica de 10 anos de registro de ocorrência de incêndios e de variáveis meteorológicas oriundas de 45 estações meteorológicas de uma empresa produtora de eucalipto para celulose, no período de 2008 a 2017. De acordo com os resultados, 91,40% das 32.561 ocorrências ao longo dos 10 anos foram originadas por incendiários. Além disso, justificado pela causa do incêndio, a quantidade de ocorrências entre os anos analisados apresentou correlações negativas moderada e baixa respectivamente, para umidade relativa e precipitação e correlação desprezível para temperatura média. A distribuição mensal dos incêndios destacou-se por ser uniforme ao longo do ano, uma vez que, apesar de setembro e outubro apresentarem a maior incidência das ocorrências, eles diferiram apenas de abril ao nível de 5% de probabilidade pelo teste de Tukey. Dessa forma, regiões onde a origem dos incêndios florestais é majoritariamente por ação intencional e criminosa, as ações de prevenção, monitoramento, registro de informações confiáveis e combate aos incêndios florestais devem ser planejadas contemplando todos os meses do ano.

Abstract The amount and intensity of forest fires has been highlighted by the social, environmental and economic losses and impacts. Thus, the objective of this research was to identify the profile of forest fires as well as their causes and correlate the influence of meteorological elements under the occurrences of fires in production forests to direct the use of resources in prevention techniques and measures as well as structures to combat forest fires. The research had as subsidy the database of the historical series of 10 years of record of occurrence of Fires (ROIs) and meteorological elements from 45 meteorological stations of a company producing eucalyptus for cellulose, period from 2008 to 2017. According to the results, 91.40% of the 32,561 occurrences over the 10 years were originated by arsonists. Moreover, justified by the cause of the fire, the number of occurrences between the years analyzed presented moderate and low negative correlations, respectively, for relative humidity and amount of precipitation and negligible correlation for the average temperature. The monthly distribution of fires stood out because it was uniform throughout the year, since, although September and October presented the highest incidence of occurrences, they differed only from April to the level of 5% probability by the Tukey test. In this way, regions where the origin of forest fires is largely due to intentional and criminal action, the actions of prevention and combating of forest fires should be planned contemplating every month of the year.

ABSTRACT Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease characterized by multisystem involvement such as bone, muscle, endocrine, ophthalmologic, cardiovascular, central and peripheral nervous system, cognitive capacity, voice, and oral motor disorders. Nutritional studies in individuals with NF1 have been performed recently. While a previous study showed an inadequate nutrient intake in patients with NF1, the dietary patterns of this population have not yet been widely studied. This study aimed to characterize dietary patterns in Brazilian adults with NF1. Sixty NF1 individuals (51.7% women), ≥18 years of age underwent nutritional assessment including laboratory analysis, anthropometrics, and eating habits recorded on a food frequency questionnaire. Cluster analysis was used to distinguish between dietary patterns. Hypothesis tests were used to compare data. Two groups with distinct patterns were identified, “Healthy” (46.7%) and “Western” (53.3%). These groups were similar in most of the socioeconomic, anthropometric, demographic and laboratory parameters evaluated. However, the upper-arm total area and upper-arm muscle area (UAMA) were lower in the Western group than those in the Healthy group [59.8 (25.7) cm2 versus 65.6 (28.3) cm2, P=0.049; 35.6±12.4 cm2 versus 43.8±15.0 cm2, P=0.024, respectively]. In this study, most individuals with NF1 had a Western dietary pattern and this group showed a lower UAMA, which may indicate a potential contribution, even in part, of diet in the muscle phenotype in this population. This association between diet and muscle in NF1 individuals requires investigation in further studies.

RESUMEN La neurofibromatosis tipo 1 (NF1) es una enfermedad genética autosómica dominante caracterizada por la afectación multisistémica, alterando los sistemas óseo, muscular, endocrino, oftálmico, cardiovascular, nervioso central y periférico así como las capacidades cognitivas. Un estudio previo señaló una ingesta inadecuada de nutrientes en pacientes con NF1, pero los patrones dietéticos de esta población aún no han sido estudiados ampliamente. El objetivo de este est udio es caracterizar los patrones dietéticos en brasileños con NF1. Sesenta individuos con NF1 (51,7% mujeres) ≥18 años se sometieron a una evaluación nutricional que incluyeron análisis de laboratorio, antropometría y hábitos alimentarios registrados en un cuestionario de frecuencia alimentaria. El análisis de conglomerados se utilizó para distinguir los patrones dietéticos; las pruebas de hipótesis para comparar datos. Se identificaron dos grupos con patrones distintos, denominados Saludables (46,7%) y Occidentales (53,3%). Estos grupos fueron similares en la mayoría de los parámetros socioeconómicos, antropométricos, demográficos y de laboratorio evaluados. Sin embargo, las áreas total braquial (ATB) y muscular braquial (AMB) fueron menores en el grupo occidental que en el grupo sano [59,8 (25,7) cm2 y 65,6 (28,3) cm2, P= 0,049; 35,6 ± 12,4 cm2 y 43,8 ± 15,0 cm2, P= 0,024, respectivamente]. En este estudio, la mayoría de las personas con NF1 habían consumido un patrón dietético occidental y este grupo presentó un AMB menor, lo que puede indicar una contribución potencial, incluso en parte, de la dieta en el fenotipo muscular en esta población. Esta asociación entre dieta y músculo en personas con NF1 requiere investigaciones en estudios adicionales.

RESUMO INTRODUÇÃO A Neurofibromatose tipo 1 (NF1) é uma doença genética autossômica dominante caracterizada por envolvimento neurocutâneo e multissistêmico, incluindo baixa densidade mineral óssea (DMO). OBJETIVOS Avaliar características ósseas em indivíduos com NF1 e verificar associação com a ingestão de nutrientes. METODOLOGIA 26 adultos com NF1 submeteram-se a avaliação dos parâmetros ósseos usando absorciometria com raios-X de dupla energia (DXA), além da avaliação da ingestão alimentar. O grupo NF1 foi comparado e pareado com 26 indivíduos sem a doença. Peso, estatura e circunferência da cintura foram avaliados. DXA forneceu o conteúdo mineral ósseo (CMO) e a DMO do corpo total, coluna e fêmur. A ingestão de calorias, macronutrientes e micronutrientes foi avaliada. RESULTADOS O grupo NF1 apresentou redução da estatura (1,68 ± 0,1; 1,61 ± 0,1 cm; P=0,003) e do CMO (2,3 ± 0,4; 2,0 ± 0,5 kg; P=0,046). Indivíduos com NF1 também apresentaram redução da DMO de corpo total e coluna (g/cm2) (1,1 ± 0,1, 1,0 ± 0,1, P=0,036; 1,0 ± 0,1, 0,9 ± 0,1; P=0,015, respectivamente). A frequência de indivíduos com massa óssea abaixo do esperado para a idade foi maior no grupo NF1 (7,7%; 34,6%, P=0,016). Não houve diferenças no consumo energético. Não houve correlação entre CMO e DMO com a ingestão de nutrientes no grupo NF1. CONCLUSÕES O grupo NF1 apresentou redução do CMO e da DMO. Apesar de menor consumo de cálcio, ferro e vitamina A, e maior consumo de sódio e ômega-6, não foi observada relação entre o fenótipo ósseo e a ingestão de nutrientes.

SUMMARY BACKGROUND Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease characterized by multisystem involvement including low bone mineral density (BMD). OBJECTIVE To assess the bone phenotype of individuals with NF1 and verify its association with nutrient intake. METHODS Twenty-six adults with NF1 underwent bone phenotype assessments using dual-energy X-ray absorptiometry (DXA) and food intake evaluations. They were compared to 26 unaffected matched control patients. Weight, height, and waist circumference (WC) were measured. DXA provided total body, spine, and hip BMDs and bone mineral content (BMC) for all patients. Food intake was evaluated for energy, macro- and micro-nutrients. RESULTS Height (1.68 ± 0.1; 1.61 ± 0.1 cm; P = 0.003) and BMC (2.3 ± 0.4; 2.0 ± 0.5 kg; P = 0.046) were lower in the NF1 group. Individuals with NF1 also presented lower total body and spine BMDs (g/cm2) (1.1 ± 0.1, 1.0 ± 0.1, P = 0.036; 1.0 ± 0.1, 0.9 ± 0.1; P = 0.015, respectively). The frequency of total body bone mass below the expected level for patients’ ages was higher in the NF1 group (7.7%; 34.6%, P = 0.016). There were no differences in energy consumption. No correlations between BMC and BMD with nutrient intake were observed in the NF1 group. CONCLUSIONS The NF1 group presented lower BMCs and BMDs. Although a lower consumption of calcium, iron, and vitamin A, and a higher intake of sodium and omega-6 were observed, there was no relationship between bone phenotype and nutrient intake.

Resumo Introdução A doença periodontal é uma doença inflamatória crônica dos tecidos de proteção e suporte dos dentes. As doenças ou alterações de ordem sistêmica, como diabetes, alterações cardiovasculares e pulmonares, distúrbios hormonais e outras, não iniciam a doença periodontal, mas podem acelerar uma doença preexistente, aumentando sua progressão e destruição tecidual. Objetivo O presente estudo teve como objetivo avaliar, por meio de prontuários clínicos, uma possível associação entre as condições sistêmicas e a gravidade da doença periodontal em pacientes atendidos na Clínica-Escola de Odontologia da Universidade Federal de Campina Grande, campus CSTR (UFCG-CSTR). Material e método Para o estudo, foram avaliados 1.035 prontuários clínicos dos pacientes que procuraram atendimento na Clínica-Escola de Odontologia da UFCG-CSTR durante os anos de 2012 a 2017. Resultado A população estudada apresentou prevalência do sexo masculino (50,9%) e diagnóstico de doença gengival (63,6%) e periodontal (35,8%). As condições sistêmicas mais prevalentes foram hipertensão (15%), diabetes (7,5%) e cardiopatias (5,8%). Além disso, 20,2% relataram ser fumantes ou ex-fumantes, enquanto o uso de medicação foi observado em 28,3% dos casos. Foi verificada associação estatisticamente significativa entre doença periodontal, sexo masculino, faixa etária mais avançada, hipertensão arterial, tabagismo ou histórico de tabagismo e diabetes. Conclusão Foi observada uma quantidade de dentes igual ou menor do que 10 com maior frequência entre os pacientes com idade igual ou superior a 60 anos, hipertensos, diabéticos, cardiopatas e fumantes ou ex-fumantes, sugerindo, desse modo, uma maior gravidade da doença periodontal nesses indivíduos.

Abstract Introduction Periodontal disease is a chronic inflammatory disease of the protective and supporting tissues of the teeth. Systemic diseases or changes, such as diabetes, cardiovascular changes, lung changes, hormonal disorders and others, do not start periodontal disease, however they can accelerate a pre-existing disease increasing its progression and tissue destruction. Objective The present study aimed to evaluated, through clinical records, a possible association between systemic conditions and the severity of periodontal disease in patients seen at the Clinical School of Dentistry, Federal University of Campina Grande, Campus CSTR (UFCG-CSTR). Material and method The study evaluated 1035 medical records of patients who sought care at the Clinical School of Dentistry of the Federal University of Campina Grande, Campus CSTR during the years 2012 to 2017. Result The studied population had a prevalence of males (50.9%) and a diagnosis of gingival and periodontal disease of 63.6% and 35.8%, respectively. The most prevalent systemic conditions were hypertension (15.0%), diabetes (7.5%) and heart disease (5.8%). About 20.2% reported being smokers or ex-smokers. The use of medication was observed in 28.3% of the cases. There was a statistically significant association between periodontal disease, male gender, older age group, arterial hypertension, smoking or history of smoking and diabetes. Conclusion A number of teeth equal to or less than 10 was observed more frequently among patients aged 60 years or more; hypertensive, diabetic, cardiac patients and smokers or ex-smokers, thus suggesting a greater severity of periodontal disease in these individuals.

Resumo O maracujá-doce (Passiflora alata Curtis) produz frutos comercializados para consumo in natura com aroma agradável, baixa acidez e elevados teores de sólidos solúveis totais. Objetivou-se com este trabalho avaliar aspectos físico-químicos de frutos obtidos dos diferentes genótipos de P. alata. Foram avaliados os frutos de 14 genótipos previamente selecionados. Avaliaram-se o rendimento de polpa com e sem sementes, a composição química, teores de potássio e de sódio, pH, acidez total titulável, teor de sólidos solúveis totais, ratio, teores de compostos fenólicos e de flavonoides e cor. O experimento foi delineado em blocos casualizados (DBC) com quatro repetições. Foi realizada análise de variância e posteriormente teste de Scott-Knott, a 5% de probabilidade. Os genótipos 2, 5, 6, 7, 11, 13 e 14 se destacaram pelos maiores valores de ratio, sendo superiores a 11,0. Por outro lado, os genótipos 2, 3, 5, 7, 8, 9, 11, 12 e 13 apresentaram rendimento de polpa com semente acima de 18,0%. No que se refere aos compostos bioativos, destacaram-se os genótipos 1 e 7, em função dos teores de compostos fenólicos e de flavonoides, respectivamente. Os genótipos, obtidos a partir das seleções, demonstram a alta variabilidade na composição química da polpa e no conjunto de aspectos físicos e químicos do maracujá-doce.

Abstract Sweet passion fruit (Passiflora alata Curtis) produce fruits that are marketed for in natura consumption with pleasant aroma, low acidity and high soluble solids content. The objective of this work was to evaluate the physicochemical aspects of fruits obtained from different genotypes of P. alata. Fruits of 14 previously selected genotypes were evaluated. The pulp yield with and without seeds, chemical composition, potassium and sodium contents, pH, total titratable acidity, total soluble solids content, ratio, total phenolic and flavonoid contents and color were evaluated. The experiment was a randomized complete block design (RCBD) with four replicates. The variance analysis and the Scott-Knott test were performed at 5% probability. The genotypes 2, 5, 6, 7, 11, 13 and 14 were highlighted by the highest ratio values, being higher than 11.0. On the other hand, genotypes 2, 3, 5, 7, 8, 9, 11, 12 and 13 presented pulp yield with seed higher than 18.0%. As regards bioactive compounds, genotypes 1 and 7 were highlighted, as a function of the total phenolic and flavonoid contents, respectively. The genotypes, obtained from the selections, demonstrate the high variability in the chemical composition of the pulp and in the physical and chemical aspects of sweet passion fruit.

Abstract Background: Fabry disease (FD) is an X-linked lysosomal disorder due to mutations in the GLA gene resulting in defective enzyme alpha-galactosidase A. FD patients are frequently misdiagnosed, commonly for rheumatic diseases. Determining pathogenicity of a mutation depends of in silico predictions but mostly on available clinical information and interpretation may change in light of evolving knowledge. Similar signs and symptoms in carriers of GLA gene genetic variants of unknown significance or of benign variants may hamper diagnosis. This study reviews rheumatic and immune-mediated manifestations in a cohort of Brazilian FD patients with classic mutations and also in subjects with GLA gene A143T and R118C mutations. Misdiagnoses, time to correct diagnosis or determination of GLA gene status, time to treatment initiation and reasons for treatment prescription in A143T and R118C subjects are reviewed. Methods: Genotype confirmed classic FD patients (n = 37) and subjects with GLA gene mutations A143T and R118C (n = 19) were referred for assessment. Subjects with R118C and A143T mutations had been previously identified during screening procedures at hemodialysis units. All patients were interviewed and examined by a rheumatologist with previous knowledge of disease and/or mutation status. A structured tool developed by the authors was used to cover all aspects of FD and of common rheumatic conditions. All available laboratory and imaging data were reviewed. Results: Thirty-seven consecutive FD patients were interviewed – 16 male / 21 female (mean age: 43.1 years) and 19 consecutive subjects with GLA gene mutations R118C and A143T were evaluated – 8 male / 11 female (mean age: 39.6 years); 15 [R118C] / 4 [A143T]. Misdiagnosis in FD patients occurred in 11 males (68.8%) and 13 females (61.9%) of which 10 males and 9 females were previously diagnosed with one or more rheumatic conditions, most frequently rheumatic fever or “rheumatism” (unspecified rheumatic disorder). Median time for diagnosis after symptom onset was 16 years (range, 0–52 years). Twenty-two patients were treated with enzyme replacement therapy (ERT) – 13 male and 9 female. Median time to ERT initiation after FD diagnosis was 0.5 years (range, 0–15 years). Rheumatic manifestations occurred in 68.4% of R118C and A143T subjects. Two subjects had been prescribed ERT because of renal disease [R118C] and neuropsychiatric symptoms [A143T]. Conclusion: Misdiagnoses occurred in 64.8% of FD patients, most frequently for rheumatic conditions. Median time for correct diagnosis was 16 years. Rheumatic manifestations are also frequent in subjects with GLA gene R118C and A143T mutations. These results reinforce the need to raise awareness and increase knowledge about Fabry disease among physicians, notably rheumatologists, who definitely have a role in identifying patients and determining disease burden. Decision to start treatment should consider expert opinion and follow local guidelines.