Clinical and radiographic features of pycnodysostosis : a case report

Pycnodysostosis is a rare disorder that was first described in 1962; however, it was only in 1996 that the defective gene was discovered, which led to a better understanding of this disease. This study reports and discuss a case of pycnodysostosis. In addition, a search of articles published in PubMed-Medline was performed. The case was a 13-year-old girl who was referred to a private clinic for dental treatment. Clinical examination showed midface hypoplasia, prominent cheeks, a high nasal bridge, beaked nose, spoon-shaped fingers, frontal bossing, open fontanelles and dental alterations, findings compatible with pycnodysostosis. Patients with this disease also suffer from fractures because of bone hardness with almost no elasticity, a fact that requires special care particularly in the case of children and adolescents. The diagnosis of pycnodysostosis is made based on clinical and radiographic findings. Clinicians should be aware of this disorder to provide adequate dental treatment

Utilização de endoprótese autoexpansível (stent) posicionada naaorta torácica do cão

We present 5 dogs who underwent treatment with an auto-expandable Dacron-covered stent in the thoracic aorta. The catheter with stents was deployed through the abdominal aorta. The procedure was performed under total anesthesia and full heparinization. A perfect integration of the stent and thoracic aorta was observed on histological examination. We believe that utilization is an improvement in relation to the elephant trunk technique and that it should contribute to improve surgical results in type B dissection.Este trabalho tem como objetivo a análise histológica de segmentos de aorta de cães submetidos ao implante de endopróteses (stents recobertos com Dacron). Para este fim, abordamos a aorta abdominal infra-renal por laparotomia paramediana esquerda. Após a heparinização, introduzimos neste segmento de aorta o cateter contendo o stent até a aorta torácica, onde procedemos à sua expansão. Todos os cães sobreviveram e foram sacrificados com 30, 60, 90 e 180 dias. A análise histológica demonstrou a perfeita integração da prótese com a parede da aorta e formação de uma neoíntima recobrindo a gaiola metálica. Acreditamos que a utilização dos stents deva contribuir para melhorar os resultados da correção cirúrgica das doenças da aorta.Escola Paulista de MedicinaUNIFESP, EPMSciEL

Eficiencia del tratamiento químico en la prevención del taponamiento de goteros aplicando agua residual sanitaria tratada

El objetivo de este trabajo fue el de verificar la eficiencia del tratamiento químico en la prevención del taponamiento de los sistemas de goteo, cuando se usa aguas residuales sanitarias tratadas. Para la realización del ensayo experimental fue construida una plataforma de ensayos con la finalidad de abastecer cuatro unidades de irrigación por goteo con aguas residuales sanitarias, tratada y filtradas mediante un filtro de disco de 120 mesh. Fueron ensayados tres modelos de goteros (M1, M2 y M3) durante un período de 560 horas. En el tratamiento químico fueron ensayadas las concentraciones de cloro residual libre de 0; 0,4; 1,0 y 1,9 mg L- 1. A partir de los resultados obtenidos se tienen las siguientes conclusiones: el agua residual sanitaria, tratada por aplicación vía sistema de irrigación por goteo propicio la formación de un biofilme en los goteros, resultante de la interacción entre colonias de bacterias y algas. La aplicación de cloro en el agua residual sanitaria tratada, minimizó el desarrollo del biofilme en el interior de los goteros y de las líneas laterales de irrigación; además, se encontró que los goteros con mayor diámetro en el laberinto y con mas de un filtro secundario (M2 y M3), son mas susceptibles al taponamiento cuando trabajan con aguas residuales tratadas. La concentración de cloro residual libre de 0,4 mg L-1 fue mas sustentable en la prevención de taponamiento por biofilme en sistemas de riego por goteo que operan con agua residual domestica tratada

Chemical characterization and sensory potential of Brazilian vanilla species

O objetivo deste trabalho foi avaliar o perfil químico e sensorial, bem como a aceitabilidade, de espécies brasileiras de baunilhas. As espécies de baunilhas avaliadas foram: Vanilla planifolia, Vanilla chamissonis, Vanilla bahiana, and Vanilla pompona. Para tanto, foram utilizados a metodologia do perfil descritivo otimizado, testes de aceitação de consumidores por meio da aplicação dos extratos a chás e cremes, e análise de espectrometria de massa por meio de cromatografia gasosa. Foram identificados compostos voláteis, tais como vanilina, acetato de anisila, 4-metil-guaiacol, p-cresol, álcool benzílico e 2,3-butanodiol. As espécies V. planifolia e V. pompona apresentaram maiores intensidades de aroma de baunilha, aroma floral, aroma frutado, sabor de baunilha, cor marrom e gosto doce. Além disso, V. bahiana mostrou maior intensidade do atributo amadeirado, enquanto V. chamissonis mostrou atributos com menor intensidade. O chá de baunilha da espécie V. pompona teve melhor aceitação pelos consumidores. Entretanto, quando as baunilhas foram aplicadas a cremes, houve aceitação por mais de 80% dos consumidores de todas as amostras. As baunilhas nativas brasileiras apresentam diferentes perfis sensoriais e de compostos voláteis, além de potencial para alta aceitação pelo consumidor.The objective of this work was to evaluate the chemical and sensory profile, as well as the acceptability, of Brazilian vanilla species. The evaluated vanilla species were Vanilla planifolia, Vanilla chamissonis, Vanilla bahiana, and Vanilla pompona. For this, the optimized descriptive profile methodology, consumer acceptance tests with extracts applied to teas and creams, and the analysis of mass spectrometry by gas chromatography were used. Volatile compounds, such as vanillin, anisyl acetate, 4-methyl guaiacol, p-cresol, benzyl alcohol, and 2,3-butanediol, were identified. The species V. planifolia and V. pompona exhibited a more intense vanilla aroma, floral aroma, fruity aroma, vanilla flavor, brown color, and sweet taste. In addition, V. bahiana showed a more intense woody attribute, while V. chamissonis showed less intense attributes. Vanilla tea from V. pompona had a better consumer acceptance. However, when the vanillas were applied to creams, there was an acceptance rate of over 80% of all samples. Brazilian native vanillas have different sensory and volatile profiles, besides potential for a high acceptance by consumers

Novel ultraviolet absorbers derived from cashew nut shell liquid: spectrophotometric, in silico and in vitro assays

The cashew nut shell liquid (CNSL) constituents were isolated by our group leading to four mixtures and seventeen pure compounds, which had chromophoric groups similar to organic ultraviolet (UV) absorbers. In addition, C15 and C8 CNSL-derivatives molecules were rationally planned as UV absorbers. Mixtures and isolated CNSL compounds were demonstrated to be non-phototoxic when evaluated in a phototoxicity assay using the yeast Saccharomyces cerevisiae. Considering the absorption values on the UV range, 6 compounds showed appropriate SPF values regarding the spectrophotometric test. Additionally, in silico and in vitro evaluations were performed, showing non-oral bioavailability, as well as non-mutagenic, non-genotoxic and non-phototoxic properties for the tested compounds. These results contribute favorably to the aimed use of the compounds under analysis as novel organic UV absorbers that have as precursor the phenolic lipid component of CNSL, a waste product obtained as the by-product of cashew nut food processing

Left ventricular free wall impeding rupture in post-myocardial infarction period diagnosed by myocardial contrast echocardiography: Case report

BACKGROUND: Left ventricular free wall rupture occurs in up to 10% of the in-hospital deaths following myocardial infarction. It is mainly associated with posterolateral myocardial infarction and its antemortem diagnosis is rarely made. Contrast echocardiography has been increasingly used for the evaluation of myocardial perfusion in patients with acute myocardial infarction, with important prognostic implications. In this case, we reported its use for the detection of a mechanical complication following myocardial infarction. CASE PRESENTATION: A 50-year-old man with acute myocardial infarction in the lateral wall underwent myocardial contrast echocardiography for the evaluation of myocardial perfusion in the third day post-infarction. A perfusion defect was detected in lateral and inferior walls as well as the presence of contrast extrusion from the left ventricular cavity into the myocardium, forming a serpiginous duct extending from the endocardium to the epicardial region of the lateral wall, without communication with the pericardial space. Magnetic resonance imaging confirmed the diagnosis of impending rupture of the left ventricular free wall. While waiting for cardiac surgery, patient presented with cardiogenic shock and died. Anatomopathological findings were consistent with acute myocardial infarction in the lateral wall and a left ventricular free wall rupture at the infarct site. CONCLUSION: This case illustrates the early diagnosis of left ventricular free wall rupture by contrast echocardiography. Due to its ability to be performed at bedside this modality of imaging has the potential to identify this catastrophic condition in patients with acute myocardial infarction and help to treat these patients with emergent surgery

Brazilian guidelines for the diagnosis of narcolepsy

Este artigo relata as conclusões da reunião de consenso com médicos especialistas sobre diagnóstico de narcolepsia baseada na revisão dos artigos sobre narcolepsia listados no Medline entre 1980 e 2010. A narcolepsia é uma doença crônica de início entre a primeira e segunda décadas de vida do indivíduo. Os sintomas essenciais são cataplexia e sonolência excessiva. A cataplexia é definida como episódios súbitos, recorrentes e reversíveis de fraqueza da musculatura esquelética desencadeados por situações de conteúdo emocional. Os sintomas acessórios são alucinações hipnagógicas, paralisia do sono e sono fragmentado. Critérios de diagnóstico clínico de acordo com a Classificação Internacional dos Transtornos do Sono são de sonolência excessiva e cataplexia. Recomenda-se a realização de polissonografia seguida do teste de latência múltipla do sono em um laboratório de sono para confirmação e diagnóstico de comorbidades. Quando não houver cataplexia, deve haver duas ou mais sonecas com sono REM no teste de latência múltipla do sono. Tipagem HLA-DQB1*0602 positiva com níveis de hipocretina-1 abaixo de 110pg/mL devem estar presentes para o diagnóstico de narcolepsia sem cataplexia e sem sonecas com sono REM.This manuscript contains the conclusion of the consensus meeting on the diagnosis of narcolepsy based on the review of Medline publications between 1980-2010. Narcolepsy is a chronic disorder with age at onset between the first and second decade of life. Essential narcolepsy symptoms are cataplexy and excessive sleepiness. Cataplexy is defined as sudden, recurrent and reversible attacks of muscle weakness triggered by emotions. Accessory narcolepsy symptoms are hypnagogic hallucinations, sleep paralysis and nocturnal fragmented sleep. The clinical diagnosis according to the International Classification of Sleep Disorders is the presence of excessive sleepiness and cataplexy. A full in-lab polysomnography followed by a multiple sleep latency test is recommended for the confirmation of the diagnosis and co-morbidities. The presence of two sleep-onset REM period naps in the multiple sleep latency test is diagnostic for cataplexy-free narcolepsy. A positive HLA-DQB1*0602 with lower than 110pg/mL level of hypocretin-1 in the cerebrospinal fluid is required for the final diagnosis of cataplexy- and sleep-onset REM period -free narcolepsy

Brazilian guidelines for the treatment of narcolepsy

Este artigo relata as conclusões da reunião de consenso com médicos especialistas sobre diagnóstico de narcolepsia baseada na revisão dos artigos sobre narcolepsia listados no Medline entre 1980 e 2010. A narcolepsia é uma doença crônica de início entre a primeira e segunda décadas de vida do indivíduo. Os sintomas essenciais são cataplexia e sonolência excessiva. A cataplexia é definida como episódios súbitos, recorrentes e reversíveis de fraqueza da musculatura esquelética desencadeados por situações de conteúdo emocional. Os sintomas acessórios são alucinações hipnagógicas, paralisia do sono e sono fragmentado. Critérios de diagnóstico clínico de acordo com a Classificação Internacional dos Transtornos do Sono são de sonolência excessiva e cataplexia. Recomenda-se a realização de polissonografia seguida do teste de latência múltipla do sono em um laboratório de sono para confirmação e diagnóstico de comorbidades. Quando não houver cataplexia, deve haver duas ou mais sonecas com sono REM no teste de latência múltipla do sono. Tipagem HLA-DQB1*0602 positiva com níveis de hipocretina-1 abaixo de 110pg/mL devem estar presentes para o diagnóstico de narcolepsia sem cataplexia e sem sonecas com sono REM.This manuscript contains the conclusion of the consensus meeting on the diagnosis of narcolepsy based on the review of Medline publications between 1980-2010. Narcolepsy is a chronic disorder with age at onset between the first and second decade of life. Essential narcolepsy symptoms are cataplexy and excessive sleepiness. Cataplexy is defined as sudden, recurrent and reversible attacks of muscle weakness triggered by emotions. Accessory narcolepsy symptoms are hypnagogic hallucinations, sleep paralysis and nocturnal fragmented sleep. The clinical diagnosis according to the International Classification of Sleep Disorders is the presence of excessive sleepiness and cataplexy. A full in-lab polysomnography followed by a multiple sleep latency test is recommended for the confirmation of the diagnosis and co-morbidities. The presence of two sleep-onset REM period naps in the multiple sleep latency test is diagnostic for cataplexy-free narcolepsy. A positive HLA-DQB1*0602 with lower than 110pg/mL level of hypocretin-1 in the cerebrospinal fluid is required for the final diagnosis of cataplexy- and sleep-onset REM period -free narcolepsy