Características clínico-radiológicas bucofaciales del síndrome de McCune-Albright en un adulto: reporte de un caso / Orofacial clinical-radiological features of McCune-Albright syndrome in an adult: a case report / Características clínico-radiológicas orofaciais da síndrome de McCune-Albright num adulto: relato de um caso

RESUMEN La displasia fibrosa es una enfermedad ósea benigna y progresiva, de base genética que puede afectar a uno o varios huesos. Su alta incidencia en la edad pediátrica sirvió de base para que anteriormente fuera considerada exclusiva de esta etapa; sin embargo, a la fecha, existen reportes que evidencian su desarrollo y crecimiento posterior en la segunda década de vida. Cuando está asociada a desequilibrios endocrinos y manchas café con leche, forma parte del síndrome de McCune-Albright. El presente reporte de caso muestra las características clínicas y radiológicas de la región bucofacial de un paciente adulto portador del síndrome de McCune-Albright. Las lesiones en los maxilares afectan la morfología y causan disfunción. A nivel dentario, los cambios producidos se traducen en maloclusión y defectos estructurales. En los estudios radiológicos se observaron cambios con densidad mixta y el patrón de vidrio esmerilado.

ABSTRACT Fibrous dysplasia is a benign and progressive bone disease of genetic basis that can affect one or several bones. Its high incidence in the pediatric age served as a basis for it to be previously considered exclusive to this stage; however, there are reports to date that show its development and later growth in the second decade of life. When associated with endocrine imbalances and café-au-lait spots, it is part of the McCune-Albright syndrome. The present case report shows the clinical and radiologic features of the oral-facial region of an adult patient with McCune-Albright syndrome. Lesions in the jaws affect the morphology and cause dysfunction. At the dental level, the changes produced result in malocclusion and structural defects. Radiological studies showed changes with mixed density and ground glass pattern.

RESUMO A displasia fibrosa é uma doença óssea benigna, progressiva e de base genética que pode afetar um ou mais ossos. A sua elevada incidência no grupo etário pediátrico foi a base para que anteriormente fosse considerada exclusiva desta fase; no entanto, existem relatos até à data do seu desenvolvimento e crescimento mais tardio na segunda década de vida. Quando associada a desequilíbrios endócrinos e manchas café-com-leite, faz parte da síndrome de McCune-Albright. Este relato de caso mostra as características clínicas e radiológicas da região bucofacial de um paciente adulto com síndrome de McCune-Albright. As lesões nos maxilares afetam a morfologia e provocam disfunções. A nível dentário, as alterações produzidas resultam em má oclusão e defeitos estruturais. Nos estudos radiológicos, foram observadas alterações com densidade mista e padrão de vidro despolido.

Asociación entre la neurofibromatosis tipo 1 y el cáncer de mama / Association between neurofibromatosis type 1 and breast cancer

La neurofibromatosis tipo 1 (NF1) o enfermedad de Von Recklinghausen es un trastorno hereditario caracterizado por la aparición de lesiones en la piel y por un mayor riesgo de neoplasias tanto benignas como malignas, siendo las más frecuentes los gliomas intracraneales. Está demostrado que las pacientes menores de 50 años con NF1 pueden tener entre 5 y 11 veces aumentado el riesgo de padecer cáncer de mama. Presentamos el caso de una paciente portadora de la mutación diagnosticada de cáncer de mama unilateral tratada en nuestro centro. Para ello realizamos una actualización y revisión de la asociación entre la NF1 y el cáncer de mama. (AU)

Type I neurofibromatosis (NF1) or von Recklinghausen’s disease is a hereditary disease characterized by skin lesions and increased risk of benign and malignant tumors, especially intracranial gliomas. Patients younger than 50 with NF1 have increased risk of breast cancer. Here we present a case of a NF1 patient diagnosed with unilateral breast cancer at our institution. We also review the evidence of the association between NF1 and breast cancer. (AU)

Cronología del diagnóstico de la neurofibromatosis tipo 1 en la infancia / Neurofibromatosis Type 1: Diagnostic Timelines in Children

Antecedentes y objetivos El diagnóstico de la neurofibromatosis 1 (NF1) plantea dificultades en niños sin antecedentes familiares durante la primera infancia. En este estudio pretendemos estimar la demora diagnóstica de los pacientes sin antecedentes familiares de NF1 y definir la repercusión de considerar las manchas café con leche y las efélides como un único criterio diagnóstico. Pacientes y métodos Estudio observacional descriptivo retrospectivo en el que se revisaron los hitos diagnósticos de la NF1 en las historias clínicas de los pacientes menores de 18 años atendidos en nuestro centro. Distribuimos a los pacientes en dos grupos en función de la existencia de antecedentes de NF1 entre sus progenitores, considerando las manchas café con leche y las efélides como un único criterio y aceptando el estudio genético como criterio de confirmación en casos de elevada sospecha. Resultados Se incluyeron en el estudio 108 menores con diagnóstico de NF1. La edad media de diagnóstico en nuestra serie fue de 3,94 años (desviación estándar:±3,8 años). En el grupo 1, sin antecedentes, la edad media de diagnóstico fue de 4 años y 8 meses, mientras que en el grupo 2, con antecedentes, fue de 12 meses, siendo la demora en el diagnóstico de 3 años y 8 meses entre ambos grupos. Conclusión Las lesiones cutáneas representan, en la mayoría de los casos, las primeras manifestaciones clínicas de la enfermedad. Consideramos necesaria la actualización de los criterios diagnósticos del NIH con el fin de facilitar el diagnóstico en los primeros años de vida (AU)

background and objectives The neurofibromatosis 1 (NF1) diagnosis is challenging in young children without a family history of NF1. The aims of this study were to estimate diagnostic delays in children without a family history of NF1 and to examine the effects of using café au lait macules and skin fold freckling as a single diagnostic criterion. Patients and methods Retrospective, descriptive, observational study of all patients diagnosed with NF1 before the age of 18 years who were seen at our hospital. The medical records of those included were reviewed to identify the date on which the diagnostic criteria of NF1 were objectified. The patients were categorized into 2 groups: those with a known parental history of NF1 and those without. Café au lait macules and skin fold freckling were assessed as a single diagnostic criterion, and genetic evidence was considered to confirm highly suspicious cases. Results We studied 108 patients younger than the age of 18 years with a diagnosis of NF1. Mean (SD) age at diagnosis was 3.94 (±3.8) years for the overall group, 1 year for patients with a parental history of NF1, and 4 years and 8 months for those without. Diagnosis was therefore delayed by 3 years and 8 months in patients without a family history. Conclusion Skin lesions were the first clinical manifestation of NF1 in most patients. We believe that the National Institutes of Health's diagnostic criteria for NF1 should be updated to aid diagnosis in young children (AU)

[Translated article] Neurofibromatosis Type 1: Diagnostic Timelines in Children / Cronología del diagnóstico de la neurofibromatosis tipo 1 en la infancia

background and objectives The neurofibromatosis 1 (NF1) diagnosis is challenging in young children without a family history of NF1. The aims of this study were to estimate diagnostic delays in children without a family history of NF1 and to examine the effects of using café au lait macules and skin fold freckling as a single diagnostic criterion. Patients and methods Retrospective, descriptive, observational study of all patients diagnosed with NF1 before the age of 18 years who were seen at our hospital. The medical records of those included were reviewed to identify the date on which the diagnostic criteria of NF1 were objectified. The patients were categorized into 2 groups: those with a known parental history of NF1 and those without. Café au lait macules and skin fold freckling were assessed as a single diagnostic criterion, and genetic evidence was considered to confirm highly suspicious cases. Results We studied 108 patients younger than the age of 18 years with a diagnosis of NF1. Mean (SD) age at diagnosis was 3.94 (±3.8) years for the overall group, 1 year for patients with a parental history of NF1, and 4 years and 8 months for those without. Diagnosis was therefore delayed by 3 years and 8 months in patients without a family history. Conclusion Skin lesions were the first clinical manifestation of NF1 in most patients. We believe that the National Institutes of Health's diagnostic criteria for NF1 should be updated to aid diagnosis in young children (AU)

Antecedentes y objetivos El diagnóstico de la neurofibromatosis 1 (NF1) plantea dificultades en niños sin antecedentes familiares durante la primera infancia. En este estudio pretendemos estimar la demora diagnóstica de los pacientes sin antecedentes familiares de NF1 y definir la repercusión de considerar las manchas café con leche y las efélides como un único criterio diagnóstico. Pacientes y métodos Estudio observacional descriptivo retrospectivo en el que se revisaron los hitos diagnósticos de la NF1 en las historias clínicas de los pacientes menores de 18 años atendidos en nuestro centro. Distribuimos a los pacientes en dos grupos en función de la existencia de antecedentes de NF1 entre sus progenitores, considerando las manchas café con leche y las efélides como un único criterio y aceptando el estudio genético como criterio de confirmación en casos de elevada sospecha. Resultados Se incluyeron en el estudio 108 menores con diagnóstico de NF1. La edad media de diagnóstico en nuestra serie fue de 3,94 años (desviación estándar:±3,8 años). En el grupo 1, sin antecedentes, la edad media de diagnóstico fue de 4 años y 8 meses, mientras que en el grupo 2, con antecedentes, fue de 12 meses, siendo la demora en el diagnóstico de 3 años y 8 meses entre ambos grupos. Conclusión Las lesiones cutáneas representan, en la mayoría de los casos, las primeras manifestaciones clínicas de la enfermedad. Consideramos necesaria la actualización de los criterios diagnósticos del NIH con el fin de facilitar el diagnóstico en los primeros años de vida (AU)

Neurofibromatosis Type 1: Diagnostic Timelines in Children. / Cronología del diagnóstico de la neurofibromatosis tipo 1 en la infancia.

BACKGROUND AND OBJECTIVES: The neurofibromatosis 1 (NF1) diagnosis is challenging in young children without a family history of NF1. The aims of this study were to estimate diagnostic delays in children without a family history of NF1 and to examine the effects of using café au lait macules and skin fold freckling as a single diagnostic criterion. PATIENTS AND METHODS: Retrospective, descriptive, observational study of all patients diagnosed with NF1 before the age of 18 years who were seen at our hospital. The medical records of those included were reviewed to identify the date on which the diagnostic criteria of NF1 were objectified. The patients were categorized into 2 groups: those with a known parental history of NF1 and those without. Café au lait macules and skin fold freckling were assessed as a single diagnostic criterion, and genetic evidence was considered to confirm highly suspicious cases. RESULTS: We studied 108 patients younger than the age of 18 years with a diagnosis of NF1. Mean (SD) age at diagnosis was 3.94 (±3.8) years for the overall group, 1 year for patients with a parental history of NF1, and 4 years and 8 months for those without. Diagnosis was therefore delayed by 3 years and 8 months in patients without a family history. CONCLUSION: Skin lesions were the first clinical manifestation of NF1 in most patients. We believe that the National Institutes of Health's diagnostic criteria for NF1 should be updated to aid diagnosis in young children.

Odontología restauradora mínimamente invasiva: caso clínico / Minimally invasive restorative dentistry: a clinical case

La decoloración de las piezas dentarias puede te-ner un impacto estético y social que lleva a los pa-cientes a buscar una intervención para mejorar su sonrisa. Las manchas superficiales y las irregula-ridades del esmalte pueden deberse a hipoplasias, hipomineralización molar, fluorosis, uso de medica-mentos, manchas blancas causadas por traumatis-mos o infección en la dentición primaria, o manchas post ortodóncicas. El diagnóstico de los defectos del esmalte se realiza a través de un examen visual por transiluminación. Se han propuesto técnicas micro abrasivas con diferentes agentes para eliminar las manchas superficiales del esmalte, así también como el uso de agentes blanqueadores a baja concentra-ción para equilibrar el color de las piezas dentarias. Si las manchas son profundas se requiere de una mega abrasión y posterior restitución anatómica con resinas compuestas. Los avances tecnológicos en los materiales de restauración adhesivos permi-ten imitar las piezas dentarias naturales permitien-do la mínima destrucción de la estructura dental sin comprometer futuras opciones de restauración. El objetivo de este trabajo es mostrar una secuencia de procedimientos mínimamente invasivos para devol-ver la estética perdida en una paciente que concurre a la Cátedra de Odontología Restauradora (AU)

The discoloration of dental pieces can have an aesthetic and social impact that leads patients to seek an intervention to improve their smile. Superficial stains and enamel irregularities may be due to hypoplasia, molar hypomineralization, fluorosis, drug use, white spots caused by trauma or infection in the primary dentition, or post-orthodontic stains. The diagnosis of enamel defects is made through a visual examination by transillumination. Microabrasive techniques with different agents have been proposed to remove surface stains from the enamel, as well as the use of low-concentration whitening agents to balance the color of the teeth. If the stains are deep, a mega abrasion and subsequent anatomical restoration with composite resins are required. Technological advances in adhesive restorative materials make it possible to mimic natural teeth, allowing minimal destruction of tooth structure without compromising future restorative options. The objective of this work is to show the sequence of minimally invasive procedures to return the lost aesthetics in a patient who attends the Chair of Restorative Dentistry (AU)

Protocolo de diagnóstico y seguimiento de pacientes adultos con neurofibromatosis tipo 1 en una unidad de referencia española / Diagnostic and follow-up protocol for adult patients with neurofibromatosis type 1 in a Spanish reference unit

La neurofibromatosis tipo 1 es uno de los trastornos genéticos neurocutáneos más frecuentes. La característica de esta enfermedad es la afectación cutánea en forma de manchas «café con leche», efélides y los característicos neurofibromas cutáneos. Otras manifestaciones frecuentes incluyen las alteraciones óseas, la «vasculopatía por neurofibromatosis tipo 1» y los problemas neurocognitivos. Además, los pacientes tienen más riesgo de padecer una gran variedad de neoplasias malignas, incluida la transformación maligna de neurofibromas plexiformes. Para ser capaces de brindar una atención óptima a estos pacientes, que presentan una afectación multisistémica y potencialmente grave, es necesario conocer las diversas características clínicas de este trastorno, así como propiciar un seguimiento y abordaje terapéutico precoz y multidisciplinar. En esta revisión, resumimos el diagnóstico, las principales características clínicas y proponemos un protocolo de cribado y seguimiento de pacientes adultos con neurofibromatosis tipo 1 (AU)

Neurofibromatosis type 1 is one of the most common genetic neurocutaneous disorders. The hallmark of this disease is skin lesions in the form of café-au-lait spots, ephelides, and the characteristic cutaneous neurofibromas. Other common manifestations include bone abnormalities, «neurofibromatosis type 1 vasculopathy», and neurocognitive disorders. In addition, patients are at an increased risk for a wide variety of malignant neoplasms, including the malignant transformation of plexiform neurofibromas. It is necessary to know the various clinical characteristics of this disorder and to provide an early, multidisciplinary follow-up and treatment approach in order to provide optimal care to these patients, who present with a multisystemic disease that is potentially severe. This review summarizes the diagnosis and main clinical characteristics and suggests a protocol for screening and follow-up of adult patients with neurofibromatosis type 1 (AU)

Chromatic disorder in Trachops cirrhosus (Spix, 1823) (Chiroptera: Phyllotomidae) on Ecuadorian Amazon

We present a case of chromatic disorder observed in a specimen of Trachops cirrhosus from lowland forests of the Ecuadorian Amazon. During the field work, six individuals of this species were captured with mist nets in an intervened habitat, of which, a juvenile male specimen was distinguishable from the rest of the individuals due to the presence of whitish spots on the ventral region, on the chest and abdomen. We discuss the previous reports of this type of pigmentary disorders in bats from Ecuador, and in the Neotropic, documenting the first record of Trachops cirrhosus with this condition for the country.

Presentamos un caso de desorden cromático observado en un ejemplar de Trachops cirrhosus proveniente de bosques de tierras bajas de la Amazonía ecuatoriana. Durante el trabajo de campo, seis individuos de esta especie fueron capturados con redes de niebla en un hábitat intervenido, de los cuales, un ejemplar macho juvenil fue distinguible del resto de individuos debido a la presencia manchas blanquecinas en la región ventral, en el pecho y abdomen. Discutimos sobre los reportes previos de este tipo de trastornos pigmentarios en murciélagos de Ecuador, y en el Neotrópico, documentando el primer registro de esta especie con esta condición para el país.

The Cleaning and Whitening Ability of Charcoal Containing Toothpastes and Homeopathic Remedies on Human Tooth Enamel

ABSTRACT: The purpose of this research was to determine the cleaning and whitening ability of two charcoal containing toothpastes compared with baking soda, activated charcoal and a hydrogen peroxide containing whitening toothpaste. Tea-stained enamel/dentin slices were mounted dentin side down in cylinders of auto-polymerizing acrylic and subjected to simulated brushin g. Total color, ΔE, and stained area were measured using a reflectance spectrophotometer and ImageJ software using a Hue, Chroma, and Brightness color threshold method; respectively, at prescribed brushing intervals of baseline, months 1, 6 and 12. A two- w ay repeated measure analysis of variance was performed, with included factors being toothpaste group and time. Statistical analys is revealed the included factor of brushing time interval was significant (p<0.05) for both ΔE and stained area. There was significant interaction for both main effects (p<0.05) for the dependent variable of stained area alone. Significant reduction in ΔE values occurred in all groups except Optic White at the 1-month interval. Statistically significant reduction in stained area occurred at 1-mon th for the baking soda group only. Post-hoc Tukey HSD tests revealed there was significant group*time interaction for baking soda at month 1 for the variable stained area. The charcoal containing (Crest 3D with Charcoal and Colgate Essentials) and non-charcoal contai ning toothpastes (Optic White) only produced a significant stain removal at the 6-month interval.

RESUMEN: El propósito de esta investigación fue determinar la capacidad de limpieza y blanqueamiento de dos pastas dentales que contienen carbón en comparación con bicarbonato de sodio, carbón activado y una pasta dental blanqueadora que contiene peróxido de hidrógeno. Los cortes de esmalte/dentina teñidos con té se montaron con el lado de la dentina hacia abajo, en cilindros de acrílico autopolimerizable y se sometieron a un cepillado simulado. El color total, ΔE y el área teñida se midieron usando un espectrofotómetro de reflectancia y el software ImageJ usando un método de umbral de color de tono, croma y brillo; respectivamente, en los intervalos de cepillado prescritos al inicio, los meses 1, 6 y 12. Se realizó un análisis de varianza de medidas repetidas de dos vías, con factores incluidos como el grupo de pasta de dientes y el tiempo. El análisis estadístico reveló que el factor incluido del intervalo de tiempo de cepillado fue significativo (p<0,05) tanto para ΔE como para el área manchada. Hubo interacción significativa para ambos efectos principales (p<0,05) para la variable dependiente del área teñida sola. Se produjo una reducción significativa en los valores de ΔE en todos los grupos excepto Optic White en el intervalo de 1 mes. Se produjo una reducción estadísticamente significativa en el área manchada al mes solo para el grupo de bicarbonato de sodio. Las pruebas post-hoc Tukey HSD revelaron que hubo una interacción significativa entre grupo y tiempo para el bicarbonato de sodio en el mes 1 para el área manchada variable. Las pastas dentales que contenían carbón (Crest 3D con Charcoal y Colgate Essentials) y las pastas de dientes que no contenían carbón (Optic White) solo produjeron una eliminación significativa de las manchas en el intervalo de 6 meses.

Diagnostic and follow-up protocol for adult patients with neurofibromatosis type 1 in a Spanish reference unit.

Neurofibromatosis type 1 (NF1) is one of the most common genetic neurocutaneous disorders. The hallmark of this disease is skin lesions in the form of café-au-lait spots, ephelides, and the characteristic cutaneous neurofibromas. Other common manifestations include bone abnormalities, "NF1 vasculopathy," and neurocognitive disorders. In addition, patients are at an increased risk for a wide variety of malignant neoplasms, including the malignant transformation of plexiform neurofibromas. It is necessary to know the various clinical characteristics of this disorder and to provide an early, multidisciplinary follow-up and treatment approach in order to provide optimal care to these patients, who present with a multisystemic disease that is potentially severe. This review summarizes the diagnosis and main clinical characteristics and suggests a protocol for screening and follow-up of adult patients with NF1.

New sources of botanical acaricides from species of Croton with potential use in the integrated management of Tetranychus urticae / Nuevas fuentes de acaricidas botánicos de especies de Croton con uso potencial en el manejo integrado de Tetranychus urticae

Essential oils from the leaves and stems of Croton adenocalyx, C. grewioides, C. heliotropiifoliusand C. blanchetianus obtained through hydrodistillation were analyzed by GC-MS. We then investigated the lethal and sublethal effects of the Croton oils and 15 major constituents against Tetranychus urticae. ß-Caryophyllene was the major component in the leaf and stem oils from C. heliotropiifolius and C. adenocalyx. Spathulenol and (E)-anethole were the major constituents identified in the leaf and stem oils of C. blanchetianus and C. grewioides, respectively. The oil with the greatest lethal and sublethal effects was those from C. adenocalyx. Among the constituents ß-caryophyllene and spatulenol were the most toxic to the mite, where as eugenol and methyl eugenol were the most repellent. The toxicity and repellency of the Croton oils, particularly the oils from C. adenocalyx, demonstrate that these oils constitute a promising alternative to synthetic acaricides for use in the control of T. urticae.

Los aceites esenciales de las hojas y tallos de Croton adenocalyx, C. growioides, C. heliotropiifolius y C. blanchetianus obtenidos mediante hidrodestilación fueron analizados a través de GC-MS. Se investigaron los efectos letales y subletales de los aceites de Croton y 15 componentes principales contra Tetranychus urticae. El ß-cariofileno fue el componente principal en los aceites de hojas y tallos de C. heliotropiifolius y C. adenocalyx. El espatulenol y el (E)-anetol fueron los principales componentes identificados en los aceites de hojas y tallos de C. blanchetianus y C. growioides, respectivamente. El aceite con los mayores efectos letales y subletales fue el de C. adenocalyx. Entre los componentes, el ß-cariofileno y el espatulenol fueron los más tóxicos para el ácaro, mientras que el eugenol y el metil eugenol fueron los más repelentes. La toxicidad y la repelencia de los aceites de Croton, particularmente los aceites de C. adenocalyx, demuestran que estos aceites constituyen una alternativa prometedora respecto a los acaricidas sintéticos para uso en el control de T. urticae.

Results of a Diagnostic Procedure Based on Multiplex Technology on Dried Blood Spots and Buccal Swabs for Subjects With Suspected Alpha1 Antitrypsin Deficiency.

INTRODUCTION: The objective of this analysis was the evaluation of a new national circuit used for diagnosing alpha1 antitrypsin deficiency (AATD) based on multiplex technology using online registration and mail posted samples from dried blood spots (DBS) and buccal swabs. METHODS: This is an observational, ongoing study conducted in Spain since March 2018. Samples are coded on a web platform and sent by postal mail to the central laboratory. Allele-specific genotyping for the 14 most common mutations was done with the Luminex 200 Instrument System. Gene sequencing was done if none of the mutations were found and the AAT serum level was <60mg/dl, or by request from the clinician in charge. RESULTS: At the time of the present report, 5803 (92.9%) samples were processed, 4984 (85.9%) from buccal swab and 819 (14.1%) from DBS. The prevalence of the frequent allele combinations were: MS 19.0%, MZ 14.4%, SS 2.9%, SZ 3.7%, and ZZ: 1.4%. Globally, Z carriers represented 20.0% and S carriers 26.6% of this population, with differences seen between regions. 209 (3.6%) were identified carrying rare alleles, 12 (0.2%) carrying null alleles and 14 (0.3%) new mutations were described. Respiratory diseases other than COPD, including poorly controlled asthma or bronchiectasis, also presented AATD mutations. CONCLUSIONS: The availability of a diagnostic system based on the simultaneous testing of 14 genetic variants from buccal swabs or DBS sent by postal mail and with web registration has proven to be useful, and the system can improve the timely diagnosis of AATD.

Cladosporium species causing "Cladosporium rot" on "Bosc" pear fruit in Argentina.

"Cladosporium rot" on "Bosc" pear fruit during cold storage causes significant economic losses and has been reported in recent years in the productive valleys of Río Negro and Neuquén. The species involved were not determined. During 2016-2017, "Bosc" pears (Pyrus communis) in cold storage chambers exhibited external brownish black circular spots caused by Cladosporium spp. The objective of this work was to determine the Cladosporium species that caused the above mentioned symptoms. The morphological and molecular analyses of the partial sequence of the actin gene (ACT) supported the identification. Cladosporium macrocarpum, Cladosporium subtilissimum and Cladosporium floccosum were determined as the species involved in the disease. Although Cladosporium has been reported to cause pear rot, this is the first report to identify these species as causal agents of this fruit disease.

Resolução estética de lesões de mancha branca pós-tratamento ortodôntico / Esthetic resolution of white spot lesions after orthodontic treatment

O objetivo deste estudo foi comparar as técnicas de microabrasão de esmalte (ME) e infiltração de resina (IR) em relação à resolução estética de lesões cariosas de mancha branca (LCMB) e avaliar a correlação entre a luminosidade das lesões quando transiluminadas e a resolução do problema (estudo 1); avaliar em um estudo "in vitro", a eficácia do clareamento dental, em amostras de dentes submetidos previamente a IR (estudo 2); avaliar a efetividade do clareamento dental 12 meses após a realização dos protocolos de mínima intervenção, em um estudo de série de casos (estudo 3). Para o estudo 1 foram selecionados 17 pacientes que foram divididos em 2 grupos: ME (N=8) e IR (N=9). Um dente de cada paciente foi fotografado sob condição normal e do mesmo dente com auxilio de dispositivo translume. Em seguida os pacientes foram submetidos às técnicas de mínima intervenção. Após 7 dias de acompanhamento, o mesmo protocolo fotográfico foi realizado. Para resolução estética, foi delimitado a área do dente em relação a área da mancha por software. Para avaliação da cor da mancha quando evidenciada, os valores de luminosidade (L*) das coordenadas L*a*b* foram avaliados antes e após a intervenção, sendo mensurados valores das áreas sadias e área da mancha mais enegrecida. Os dados de correlação foram submetidos ao teste de Person. A resolutividade dos tratamentos foi dada por um Teste t-Student e a diferença de luminosidade antes e após os tratamentos foi dado pelo teste Anova 2 fatores de medidas repetidas (p<0,05). Para o estudo 2, quarenta superfícies de esmalte bovino foram submetidas à desmineralização. Após, foram estabelecidos dois protocolos de manchamento. Vinte espécimes foram imersos em caldo de manchamento por 24 horas (Lab 1) ou 7 dias (Lab 2). Dez corpos-de-prova de cada grupo receberam a aplicação de IR. Todas as amostras foram clareadas com gel de peróxido de carbamida 15% por 14 dias (8 horas diárias). A mensuração da cor foi realizada em espectrofotômetro de refletância em três momentos: inicial, após o manchamento e após o clareamento. Os dados (CIEDE00) foram analisados pelo teste t-Student (p <0,05). Para o estudo 3, foram selecionados 4 voluntários, que tinham LCMB nos dentes anteriores superiores. Os pacientes foram divididos de forma aleatória em dois grupos, sendo estes: ME e IR na área de mancha. Para resolução estética das LCMB, foi determinada a área da mancha em relação a área do dente e a porcentagem da redução pós protocolos. Após 12 meses de acompanhamento, os pacientes receberam clareamento dental, com peróxido de carbamida 10%, durante 21 dias. Os dados do estudo 1 mostram que não houve correlação entre as áreas da mancha (normal e transiluminada) e a resolução estética das mesmas (p>0.05). Em relação a resolutividade, não houve diferença entre os tratamentos (p=0.403). O valor de Luminosidade aumentou após a realização dos procedimentos (p=0.001). Para o estudo 2, nenhuma diferença significativa foi observada no Lab 1 (p=0,560). Para o Lab 2, foram detectadas diferenças significativas (p=0,031). Uma vez que o clareamento foi mais efetivo no grupo não submetido a IR (Lab2). Os resultados do estudo 3 mostram que ambos os protocolos reduziram e/ou suavizaram as áreas das manchas. Após 12 meses de acompanhamento, a técnica de ME apresentou redução no valor da área de mancha. Por fim, o clareamento dental foi efetivo em ambos os protocolos, sem diferenças entre as técnicas. Frente as metodologias propostas, pode-se concluir que: não houve diferenças entre os tratamentos em relação a remoção ou mascaramento das LCMB; a cor das manchas quando transiluminadas não estão relacionadas a resolução estética das mesmas; "in vitro", dentes fortemente manchados, submetidos ao tratamento com RI, o produto parece se comportar como uma barreira semipermeável à penetração do peróxido. Por fim, ambas as técnicas podem ser indicadas para reduzir ou suavizar as áreas de mancha. A ME apresentou redução das áreas de mancha no período avaliado (12 meses). Clinicamente, o clareamento dental pode ser indicado para melhorar a cor dos dentes, independentemente do protocolo pré-estabelecido

The aim of this study was to compare enamel microabrasion (EM) and resin infiltration (RI) techniques in relation to the esthetic resolution of white spot carious lesions (WSL) and to evaluate the correlation between the luminosity of the lesions when transilluminated and the resolution of the problem (study 1); To evaluate in an "in vitro" study, the effectiveness of dental bleaching in samples of teeth previously submitted to RI (study 2); to evaluate the effectiveness of dental bleaching 12 months after the minimal intervention protocols were performed, in a case series study (study 3). For study 1, 17 patients were selected and divided into 2 groups: ME (N=8) and IR (N=9). One tooth from each patient was photographed under normal condition and the same tooth with the aid of a translume device. Then the patients were subjected to the minimal intervention techniques. After 7 days of follow-up, the same photographic protocol was performed. For esthetic resolution, the area of the tooth was delimited in relation to the area of the stain using software. To evaluate the color of the stain when evidenced, the luminosity values (L*) of the coordinates L*a*b* were evaluated before and after the intervention, being measured values of the healthy areas and area of the darker stain. The correlation data were submitted to Person's test. The resolution by the treatments was analyzed by Student's t-test, and the difference in luminosity before and after treatments was analyzed by 2-way repeated measures Anova (p<0.05). For study 2, forty bovine enamel surfaces were subjected to demineralization. Two staining protocols were established. Twenty specimens were immersed in staining broth for 24 hours (Lab 1) or 7 days (Lab 2). Ten specimens from each group received IR application. All specimens were bleached with 15% carbamide peroxide gel for 14 days (8 hours daily). Color measurement was performed by spectrophotometry at three periods: bselien, after staining, and after bleaching. Data (CIEDE00) were analyzed by Student's T test (p <0.05). For study 3, 4 volunteers with WSL in the maxillary teeth were selected. The patients were randomly divided into EM or RI for treating lesions. For esthetic resolution of WSL, the spot area relative to tooth area and the percentage of reduction after protocols were determined. After 12 months of follow-up, the patients received dental bleaching with 10% carbamide peroxide for 21 days. The data from study 1 show that there was no correlation between the stain areas (transilluminated or not) and the esthetic resolution (p>0.05). Regarding resolution, there was no difference between treatments (p=0.403). The Luminosity value increased after the procedures were performed (p=0.001). For study 2, no significant difference was observed in Lab 1 (p=0.560). For Lab 2, significant differences were detected (p=0.031), and whitening was more effective in the group not subjected to IR (Lab2). The results of study 3 show that both protocols reduced and/or softened the areas of the stains. After 12 months of follow-up, the EM technique showed a reduction in the stain area value. Finally, dental bleaching was effective with both protocols, with no differences between the techniques. Based on the proposed methodologies, it can be concluded that: there were no differences between the treatments regarding the removal or masking of WSL; the color of the stains when transilluminated is not related to their esthetic resolution; "in vitro", heavily stained teeth submitted to treatment with RI, the product seems to behave as a semi-permeable barrier to peroxide penetration. Finally, both techniques can be indicated to reduce or soften stained areas. The EM showed a reduction of stained areas in the evaluated period (12 months). Clinically, dental bleaching can be indicated to improve the color of teeth, regardless of the pre-established protocol

Bactéria cromogênica associada a manchas negras / Black-spotted chromogenic bactéria

Objetivos: Este trabalho tem como objetivo avaliar a associação das bactérias Prevotella Melaninogênica e Actinomyces ao biofilme escuro em dentes permanentes. Relato de Caso: Um paciente apresentou manchas enegrecidas ao longo do contorno cervical dos dentes foi convidado a participar do presente estudo. Foi coletado com o auxílio de uma cureta estéril, uma parte deste biofilme que transferido para um eppendorff contendo soro fisiológico. O material coletado foi agitado em um Vórtex por 15 segundos e em seguida, inoculadas em 100 microlitros (µl) da solução em um meio de ágar sangue suplementado com Canamicina 5%, seguido de cultivo por 72 horas. A partir do crescimento bacteriano, as mesmas foram isoladas em ágar macconkey, chocolate e sangue em anaerobiose. Em seguida foram realizados testes de bioquimismo, utilizando-se os meios MIO (motilidade, indol e ornitina), citrato, TSI (triplo açúcar com ferro) e LIA (ágar lisina com ferro). Conclusão: Através das características clínicas associadas ao bioquimismo empregado nos testes, podemos concluir que a bactéria que está associada as manchas negras presentes no paciente é do gênero prevotella spp(AU)

Objectives: This study aims to evaluate the association of Prevotella Melaninogenic and Actinomyces bacteria with dark biofilm in permanent teeth. Case Report: A patient with black spots along the cervical contour of the teeth was invited to participate in the present study. After its clarification and acceptance, completing the IC, was collected with the aid of a sterile curette, a part of this biofilm that was inserted into an eppendorff containing saline. The collected material was vortexed for 15 seconds and then inoculated into 100 microliters (µl) of the solution in a 5% kanamycin supplemented blood agar medium, followed by cultivation for 72 hours. From bacterial growth, they were isolated on macconkey agar, chocolate and anaerobic blood. Biochemical tests were then performed using MIO (motility, indole and ornithine), citrate, TSI (triple sugar with iron) and LIA (iron lysine agar) media. Conclusion: Through the clinical characteristics associated with the biochemism employed in the tests, we can conclude that the bacteria that is associated with black spots present in the patient is of the genus prevotella spp(AU)

Caso Clínico: Neurofibromatosis Tipo 1, resolución quirúrgica / Case Report: Neurofibromatos Type 1, Surgical Management

INTRODUCCIÓN: La neurofibromatosis tipo 1(NF1) descrita por primera vez por von Recklinghausen en 1884, es una enfermedad de transmisión genética autosómica dominante, causada por un espectro de mutaciones en el gen NF-1, de evolución impredecible, que afecta múltiples órganos y sistemas, como la piel y el sistema nervioso, afectando también el desarrollo infantil y al estado neurocognitivo. Representa para el médico la toma de decisiones de manejo a menudo complejas, que requieren un enfoque multidisciplinario. CASO CLÍNICO: Paciente femenino de 9 años de edad, con antecedente de neurofibromatosis tipo 1. Presenta masa en región cervical anterior, que ocupa además el hemitórax izquierdo, comprimiendo el mediastino anterior, la tráquea del lado izquierdo, el corazón, el pulmón izquierdo; la masa produce sintomatología: disnea, disfagia y dolor, por lo que se programa intervención quirúrgica para exéresis del tumor. Se realizó cervico-esternotomía- toracotomía izquierda, se extirpó masa de 12 x 7 cm, ubicada a nivel de cayado aórtico, íntimamente adherida a la columna vertebral cervico-torácica. EVOLUCIÓN: Durante el acto quirúrgico la paciente tuvo un sangrado cuantioso, proveniente de arteria colateral de carótida común izquierda, por lo que se decidió empaquetar para resolución posterior, 24 horas después se desempaquetó, logrando control del sangrado tras el procedimiento. Los resultados de anatomía patología establecieron que la masa fue un neurofibroma. El seguimiento posterior determinó mejoría de la sintomatología, sin complicaciones quirúrgicas tardías. CONCLUSIÓN: La Neurofibromatosis es una patología congénita, poco frecuente y de complejo manejo. Aunque no existe tratamiento definitivo para la neurofibromatosis tipo 1, se puede realizar un manejo integral para mejorar la calidad de vida del paciente. Los tumores pueden extirparse mediante cirugía, aunque a menudo vuelven a crecer.(au)

BACKGROUND: Neurofibromatosis type 1, first described by von Recklinghausen in 1884, is an autosomal dominant genetically transmitted disease, caused by a spectrum of mutations in the NF-1 gen, with unpredictable evolution, that affects multiple organs and systems, such as the skin and the nervous system, also affecting child development and neurocognitive functions. It often requires complex management decisions from the physician that involve a multidisciplinary approach. CASE REPORTS: 9 year old female patient with a history of type 1 neurofibromatosis. She presented with a mass located in the anterior cervical region, occupying also the left hemithorax, compressing the anterior mediastinum, the trachea from its left side, the heart, and the left lung; the mass produced symptoms: dyspnea, dysphagia and pain, so surgical excision of the mass was scheduled. A left cervical-sternotomy-thoracotomy was performed, a 12 x 7cm mass, located at aortic arch level, closely adhered to the cervical-thoracic spine, was excised. EVOLUTION: During the surgical procedure, the patient had profuse bleeding, coming form the left common carotid colateral artery, so abdominal packing was performed, for later resolution. 24 hours later, bleeding control was achieved after the surgical re-intervention. The pathology lab results established that the mass was in fact a neurofibroma. Subsequent follow-up determined symptoms improvement, without late surgical complications. CONCLUSIONS: Neurofibromatosis is a congenital infrequent, disease, with a complex treatment. Although there is no definitive treatment for type 1 neurofibromatosis, comprehensive management can be achieved to improve the patient's quality of life. Tumors can be surgically removed, although they often grow back.(au)

Neuroibromatosis tipo l: relación genotipo-fenotipo / Type 1 neurofibromatosis: genotype-phenotype relationship

RESUMEN INTRODUCCIÓN: La neurofibromatosis (enfermedad de von Recklinghausen) es una enfermedad autosómica dominante que presenta principalmente manifestaciones cutáneas y neurológicas. El objetivo es describir actualmente si existe o no relación entre las mutaciones encontradas en los pacientes con neurofibromatosis tipo 1 y las características clínicas que presentan. MÉTODOS: Se realizó un artículo de revisión narrativa para evaluar la relación con el genotipo y fenotipo de los pacientes con neurofibromatosis tipo 1. Se revisaron las bases de datos PubMed, Embase y Lilacs. Se utilizaron los siguientes términos Mesh: Neurofibromatosis, neurofibromatosis tipo 1, genes, genotipo, fenotipo, mutaciones, secuenciación de exoma. Los estudios identificados fueron revisados y analizados. Se presentan los datos de manera cualitativa. RESULTADOS: De 425 artículos, 62 contenían la información necesaria para hacer el análisis. A pesar de que algunos estudios han presentado evidencia de asociación en relación a las mutaciones encontradas y la clínica, realmente no existe una correlación genotipo-fenotipo comprobada en neurofibromatosis tipo 1. Esto sugiere que para los fenotipos discordantes con genotipo similar existen otros factores que deben considerarse tales como la epigenética, alteraciones genéticas o incluso factores ambientales. CONCLUSIONES: Es necesario realizar estudios con cohortes más grande de pacientes para seguir estudiando si existe una relación directa o no.

SUMMARY INTRODUCTION: Neurofibromatosis (von Recklinghausen disease) is an autosomal dominant disease that mainly presents cutaneous and neurological manifestations. The objective is to describe if there is a relationship between the mutations found in patients with neurofibromatosis type 1 and the clinical characteristics they present. METHODS: A narrative review of the literature was carried out in relation to the genotype and phenotype of patients with Neurofibromatosis type 1 using PubMed, Embase, and Lilacs. The following Mesh terms were used: Neurofibromatosis, neurofibromatosis type 1, genes, genotype, phenotype, mutations, exome sequencing. The identified studies were reviewed and analyzed. Data are presented qualitatively RESULTS: Of 425 articles, 62 contained the information necessary to make the analysis. Although some studies have presented evidence of association in relation to the mutations found and the clinical one, there is still no proven genotype-phenotype correlation in neurofibromatosis type 1. This suggests that, for discordant phenotypes with a similar genotype, there are other factors that must be considered such as epigenetics, genetic alterations or even environmental factors. CONCLUSIONS: It is necessary to perform studies with larger cohort of patients to continue studying whether there is a direct relationship or not.

Comparativo entre o verniz fluoretado e a nanohidroxiapatita na remineralização de lesões de manchas brancas ativas: estudo clínico prospectivo / Comparison between fluoride varnish and nano-hydroxyapatite in the remineralization of active white spot lesions: a prospective clinical study

A expressão inicial da cárie dental, mancha branca, ainda é frequente e requer cuidados profissionais que podem ser preventivos ou não. Torna-se importante a realização de pesquisas que avaliem diferentes métodos de remineralização de lesões iniciais do esmalte, tanto na dentição decídua como na permanente. Deste modo, o objetivo deste estudo foi comparar o potencial de remineralização de tais lesões, utilizando o verniz fluoretado (Duraphat®) e a nanohidroxiapatita (creme dental ReminPro®). Para pesquisa foram selecionadas 21 crianças de 6 a 12 anos de idade, com manchas brancas ativas não cavitadas (opacas e rugosas) em dentes decíduos e permanentes. Como metodologia foram realizados procedimentos de remineralização em hemiarcos distintos sendo que, cada dente recebeu um produto diferente: Duraphat® com protocolo padrão descrito na literatura científica; e creme dental nanoparticulado ReminPro® com técnica de profilaxia profissional, ambos em 4 sessões, uma vez por semana. Os resultados obtidos foram analisados por um examinador calibrado sem conhecimento prévio do produto aplicado (cego); através de critérios visuais e mensuração das manchas brancas em seu maior diâmetro com paquímetro digital. Neste exame clínico, foram observadas mudanças no brilho, cor, textura e integridade superficial. Os dados coletados receberam tratamento estatístico através do teste t-Student, o qual identificou a significância do verniz fluoretado (p<0,05) na redução das manchas brancas ativas. Os resultados clínicos dentro dos critérios estabelecidos, foram considerados positivos na remineralização de tais manchas com os dois produtos utilizados. Diante disso, o estudo teve a intenção de demonstrar a possibilidade da utilização do creme dental nanoparticulado como alternativa clínica dos métodos preventivos, visto que pode ser um aliado nos cuidados diários do paciente para evitar a progressão das lesões de manchas brancas(AU)

A white-spot lesion is frequently the initial expression of dental caries and requires professional care, which can be either preventive or not. Therefore, it is important to perform studies assessing different methods of remineralization of these lesions in both deciduous and permanent dentitions. The objective of this study was compare the remineralization potential of such lesions using fluoride varnish (Duraphat®) and nano-hydroxyapatite (ReminPro® toothpaste). For the research, 21 children aged 6 12 years were selected with active white spots (opaque and wrinkled) on young permanent and deciduous teeth. Remineralization procedures were performed in different hemi-arches in which each tooth received a different material: fluoride varnish with standard protocol described in the scientific literature; and nanoparticulate toothpaste with professional prophylaxis technique, both in four sessions, once a week. The results were analyzed by a calibrated examiner without previous knowledge of applied product (blind); through visual criteria and measurement of white spots in their largest diameter with a digital calliper. In this clinical examination changes in the gloss, color, texture, superficial integrity were observed. The collected data received statistical treatment through the T-student test, wich identified the significance of fluoride varnish (p<0.05) in the reduction of active white spots. The clinical results within the established criteria were considered positive in the remineralization of such spots with the two products used. Therefore, the study was aimed to demonstrate the possibility of using nanoparticulate toothpaste as a clinical alternative for preventive methods, since it can be ally in the day care of the patient to prevent the progression of white spot lesions(AU)

Effects of the fungus Pestalotiopsis maculans (Ascomycota: Amphisphaeriales) on the gametophytic development of the fern Lygodium venustum (Lygodiaceae)

Lygodium Sw. is the only genus of the family Lygodiaceae, with around 25 to 40 species inhabiting mostly tropical regions worldwide. In Argentina two species develop; one of them, L. venustum, grows in the Northeast of the country. Some species of Lygodium are invasive whereas medicinal uses have been reported for some taxa. As part of a project to ascertain on the reproductive biology of native ferns in Argentina, a fungus was found during in vitro spore culture of L. venustum. Dark brown spots and eruptive pustules were also observed in fertile leaves. Thus, emerged as objectives of this work: 1) to retrieve and identify the fungus from the sporophyte and from the spores cultures, 2) to inquire on the impact of the fungus on gametophyte generation, and 3) to perform experiments with untreated spores to demonstrate that the fungus is associated to the spores collected from infested sporophytes. Phytopathological techniques were applied in order to isolate the fungus and then to achieve monosporic cultures. The morpho-biometric features of the conidia corresponded with those of Pestalotiopsis maculans (Corda) Nag Raj [= Pestalotiopsis guepinii (Desm.) Steyaert] (Amphisphaeriales, Ascomycota). Re-inoculation of conidia on healthy gametophytes conducted to their necrosis and death. The percentages of spore germination were initially higher in unsterilized cultures, suggesting that disinfection protocol may have affected spore germination. This is the first report of Pestalotiopsis maculans on the sporophytes of Lygodium venustum. The information that emerges from our work could be useful for the biocontrol of other species of Lygodium, considered weeds in other regions of the world.

Efectos del hongo Pestalotiopsis maculans (Ascomycota: Amphisphaeriales) en el desarrollo gametofítico del helecho Lygodium venustum (Lygodiaceae). Lygodium Sw. es el único género de la familia Lygodiaceae, con alrededor de 25 a 40 especies que habitan en su mayoría en regiones tropicales del mundo. En Argentina se desarrollan dos especies; uno de ellas, L. venustum, crece en el noreste del país. Algunas especies de Lygodium son invasivas, mientras que para algunos taxones se han reportado usos medicinales. Como parte de un proyecto para indagar sobre la biología reproductiva de los helechos nativos en Argentina, se encontró un hongo durante el cultivo in vitro de esporas de L. venustum. Asimismo, se observaron manchas marrones oscuras y pústulas eruptivas en hojas fértiles. Así, surgieron como objetivos de este trabajo: 1) recuperar e identificar el hongo presente en el esporofito y en los cultivos de esporas, 2) investigar el impacto del hongo en la generación gametofítica y 3) realizar experimentos con esporas no tratadas para demostrar que el hongo está asociado a las esporas recolectadas de esporofitos infestados. Se aplicaron técnicas fitopatológicas para aislar el hongo y luego lograr cultivos monospóricos. Las características morfo-biométricas de los conidios correspondieron a las de Pestalotiopsis maculans (Corda) Nag Raj [= Pestalotiopsis guepinii (Desm.) Steyaert] (Amphisphaeriales, Ascomycota). La re-inoculación de conidios en gametofitos sanos condujo a su necrosis y muerte. Los porcentajes de germinación de esporas fueron inicialmente más altos en los cultivos no esterilizados, lo que sugiere que el protocolo de desinfección pudo haber afectado la germinación de las esporas. Este es el primer reporte de Pestalotiopsis maculans sobre los esporofitos de Lygodium venustum. La información que surge de nuestro trabajo podría ser útil para el control biológico de otras especies de Lygodium, consideradas malezas en otras regiones del mundo.

Hypertensive choroidopathy and adrenal carcinoma: A new association. / Coroidopatía hipertensiva y carcinoma adrenal: una nueva asociación.

A 17 year-old Caucasian female presented with decreased vision, with no other symptoms reported at that time. Ophthalmological examination revealed typical signs of hypertensive choroidopathy, Elschnig spots, and Siegrist streaks. Further medical examination found high blood pressure (220/155mmHg), with a metastatic hormone-secreting adrenal carcinoma being the underlying cause of these alterations. To our knowledge, this is the first reported case of hypertensive choroidopathy associated with adrenal carcinoma.