Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

ABSTRACT COVID-19 is commonly associated with high serum levels of pro-inflammatory cytokines, and the post-infection status can disturb self-tolerance and trigger autoimmune responses. We are reporting a 45-year-old male who was admitted with fatigue, jaundice, elevated liver enzymes (with cholestatic pattern), and acute kidney injury two weeks after recovering from a mild SARS-CoV-2 infection. Serologies for viral hepatitis and anti-mitochondrial antibody were negative, while anti-nuclear and anti-smooth muscle antibodies were positive. There were no signs of chronic liver disease, and a magnetic resonance cholangiography showed no dilatation of biliary ducts. Histologic evaluation of the liver evidenced numerous foci of lobular necrosis without ductopenia or portal biliary reaction. Considering the autoantibody profile and histologic changes, the medical team started oral prednisone, but there was a suboptimal biochemical response in the outpatient follow-up. Two months later, a second liver biopsy was performed and revealed non-suppurative destructive chronic cholangitis, extensive areas of confluent necrosis with hepatocytes regenerating into pseudorosettes, and numerous plasma cells. According to the Paris Criteria, the patient was then diagnosed with an autoimmune hepatitis-primary biliary cholangitis overlap syndrome (AIH-PBC-OS). After adding azathioprine and ursodeoxycholic acid to the treatment, there was a satisfactory response. This is the second worldwide report of an AIH-PBC-OS triggered by COVID-19, but the first case with a negative anti-mitochondrial antibody. In this setting, histologic evaluation of the liver by an experienced pathologist is a hallmark of achieving the diagnosis and correctly treat the patient.

Diagnostic challenges in paediatric foreign body aspiration: a case report / Desafío diagnóstico en la aspiración de cuerpo extraño en pediatría: reporte de un caso

Abstract Introduction Foreign body aspiration (FBA) is a potentially fatal paediatric emergency. Our objective was to highlight the importance of a multidisciplinary approach to difficult/doubtful diagnosis. Case report 34-month-old girl referred for urgent rigid bronchoscopy after suspected metallic blade ingestion (found chewing on it). She had a previous recurrent history of wheezing. The physical examination revealed face/lip wounds, traces of powder on her teeth but no breathing difficulty. The plain X-Ray revealed radiopaque images of the upper pulmonary field and gastric chamber. In the absence of FBA clinical signs but considering a previous history of bronchial hyperresponsiveness, a direct digital radiographic study was performed. There were no images compatible with foreign bodies: the results were interpreted as artefacts and no bronchoscopy was performed. Conclusions A careful pre-anaesthetic evaluation, a high level of suspicion and excellent multidisciplinary communication led to the recognition of false radiologic findings. A conservative approach was followed and invasive procedures in a remote location, with high anaesthetic risk for the paediatric population were avoided.

Resumen Introducción La aspiración de cuerpo extraño (ACE) es una emergencia pediátrica potencialmente fatal. La intención del presente artículo es resaltar la importancia de un abordaje multidisciplinario en caso de un diagnóstico difícil/dudoso. Reporte de caso Se trata de una paciente de 4 meses de edad remitida para broncoscopia rígida de urgencia, luego de la sospecha de ingestión de una hojilla metálica (se encontró a la bebé mordiéndola). La paciente tenía antecedentes de sibilancia. Al examen físico se encontraron heridas en la cara y los labios, rastros de polvo en los dientes, pero no había dificultad respiratoria. El examen de rayos-x mostraba imágenes radio opacas en el cuadrante superior derecho del pulmón y en la cámara gástrica. En virtud de la ausencia de signos clínicos de ACE en una paciente con antecedentes de hiperresponsividad bronquial, se realizó un estudio radiográfico digital. No hubo imágenes compatibles con cuerpos extraños: los resultados se interpretaron como artefactos y no se realizó la broncoscopia. Conclusiones Un cuidadoso examen pre-anestesia, el alto grado de sospecha y una excelente comunicación multidisciplinaria, permitieron el reconocimiento de hallazgos radiológicos falsos. Se siguió un abordaje conservador, evitando así procedimientos invasivos en lugares remotos con alto grado de riesgo para la población pediátrica.

Clinical and cytogenetic characteristics of patients diagnosed with Turner syndrome in a clinical genetics service: cross-sectional retrospective study

ABSTRACT BACKGROUND: Turner syndrome (TS) is a rare genetic disease. Understanding its clinical findings contributes to better management of clinical conditions. OBJECTIVE: To investigate the clinical and karyotypic characteristics of patients diagnosed with TS at two reference services for clinical genetics in southern Brazil. DESIGN AND SETTING: Retrospective cross-sectional study conducted in two clinical genetics services in Porto Alegre (RS), Brazil. METHODS: The sample consisted of 59 patients with TS diagnosed from 1993 to 2019. A review of their medical records was performed and a standard protocol was filled out. RESULTS: The average age of the patients at diagnosis was 15.9 years, and 40.7% were over 13 years old. The largest proportion of them (42.4%) had been referred from an endocrinology department and their constitution was 45,X (40.7%). The most common clinical findings were short stature (85.7%), hypoplastic/ hyperconvex nails (61.2%), low posterior hairline (52.1%) and cubitus valgus (45.8%). There was no difference regarding the presence of short stature (P = 0.5943), number of dysmorphia (P = 0.143), anatomical regions affected and malformations identified through imaging examinations (P = 1.0000), regarding the presence or absence of 45,X constitution. Only 6% of the patients had used growth hormone and 43%, estrogen. CONCLUSION: We found that, in general, patients with TS were being diagnosed late. This has important implications for their treatment. In addition, only a small proportion of the patients were undergoing further examination or evaluation, which appeared to be leading to underdiagnosis of many abnormalities.

Early liver function improvement following successful treatment of chronic hepatitis C in patients with decompensated cirrhosis: a real-life study

OBJECTIVES: Despite higher rates of sustained virologic response (SVR), important concerns remain when patients with decompensated cirrhosis due to hepatitis C virus (HCV) are treated with direct-acting antiviral agents (DAA). Questions include efficacy, safety, and the magnitude of liver function improvement. Here, we aimed to evaluate HCV treatment data in this specific population in Brazil. METHODS: We included 85 patients with decompensated cirrhosis submitted to HCV therapy with DAA followed at two academic tertiary centers in the southeastern region of Brazil. RESULTS: Seventy-nine patients (92.9%) were Child-Pugh (CP) score B, and six (7.1%) were CP score C. The mean MELD score was 12.86. The most common treatment was sofosbuvir plus daclatasvir±ribavirin for 24 weeks. The overall intention-to-treat (ITT) SVR rate was 87.4% (74/85) and modified-ITT 96.1% (74/77). ITT SVR was associated with lower baseline INR values (p=0.029). Adverse events (AE) occurred in 57.9% (44/76) of patients. Serious AE were reported in 12.8% (10/78), and were related to the presence of hepatic encephalopathy (p=0.027). SVR was associated with improvement in CP (p<0.0001) and MELD scores (p=0.021). Among baseline CP score B patients with SVR, 46% (29/63) regressed to CP score A. Ascites was independently associated with no improvement in liver function in patients who achieved SVR (p=0.001; OR:39.285; 95% CI:4.301-258.832). CONCLUSIONS: Patients with decompensated HCV cirrhosis showed a high SVR rate with interferon-free therapy. Early liver function improvement occurred after successful HCV eradication. However, long-term follow-up of these patients after SVR remains strongly advised.

Is Football players' performance influenced by the quality of opposition? Application of the golden index formula in Club Atlético de Madrid 2016/2017

Aim: In this study, we sought to analyze the influence of the quality of opposition on players' performance of Club Atlético de Madrid (ATM) 2016/2017. For that, the Golden Index (GI) formula was applied to identify and rank the Golden Players of ATM when playing against opponents of different quality levels. Methods: GI formula variables were collected through notational analysis and three global adjacency matrices were constructed to record all successful ball-passing actions performed. Next, the matrices were imported to SocNetV to collect the network centrality metrics. To uniformize each variable, the statistical standardization technique was applied to all variables. ATM opponents were classified into three groups: high-level (n=4), medium-level (n=2) and low-level (n=2), according to their classification in La Liga and participation in the final stage of UEFA Champions League 2016/2017. Results: Koke was considered the Golden Player playing against high-level teams, while opposing low-level opponents he was ranked as second. Against medium- and low-level teams, Antoine Griezmann was considered the Golden Player, but was not classified in the three first positions against high-level opponents. Yannick Carrasco and Filipe Luís were ranked in the second and third positions, respectively, when playing against high- and medium-level opponents. Also, Saúl Ñíguez obtained the third higher index against low-level teams. Conclusions: This study evidenced that players' individual performance is influenced by the quality of the opposition. Additionally, the GI formula proved to be a potent tool in analyzing player's performance in attacking plays in Football.(AU)

Relação entre a etiologia da respiração oral e a pressão máxima da língua / Relationship between mouth breathing etiology and maximum tongue pressure

RESUMO Objetivo Verificar a existência de relação entre pressão máxima da língua e a etiologia da respiração oral em crianças respiradoras orais atendidas em um Ambulatório do Respirador Oral. Método Foi conduzido um estudo transversal observacional descritivo e analítico com 59 crianças respiradoras orais com idades entre três e 12 anos (média de 6,5 anos e DP=2,4). Para a coleta da pressão de língua, foi utilizado o Iowa Oral Performance Instrument - (IOPI) e dados sobre a etiologia da respiração oral e oclusão dentária foram coletados nos prontuários desses pacientes para análise. As associações entre a pressão máxima da língua e a etiologia da respiração oral, idade, gênero e oclusão dentária foram verificadas pelo teste T, ANOVA, coeficiente de Spearman e Teste de Tuckey, utilizando-se nível de significância de 5%. Resultados Houve correlação moderada e positiva entre idade e pressão máxima, verificou-se que houve diferença estatisticamente significativa entre a pressão máxima da língua e as variáveis hipertrofia da tonsila faríngea e hipertrofia das tonsilas palatinas. Não foram verificadas diferenças estatísticas entre as outras variáveis. Conclusão Conclui-se que as obstruções mecânicas, dentre elas a hipertrofia das tonsilas faríngea e palatinas alteram a pressão máxima de língua em crianças respiradoras orais.

ABSTRACT Purpose To verify the relationship between maximum tongue pressure and the etiology of oral breathing in oral breathing children attended at the Oral Respiratory Outpatient Clinic. Methods A descriptive and analytical cross-sectional study was accomplished with 59 mouth breathing children aged 3 to 12 years (mean age 6.5 years and SD: standard deviation= 2.4). To collect tongue pressure, the Iowa Oral Performance Instrument (IOPI) was used and data regarding the etiology of oral breathing and dental occlusion were collected in the records of these patients for analysis. The associations between the maximum tongue pressure and the etiology of oral breathing, age, gender and dental occlusion were verified by the T test, ANOVA, Spearman's coefficient and Tuckey's test, using a significance level of 5%. Results There was a moderate and positive correlation between age and maximum pressure, it was verified that there was a statistically significant difference between the maximum tongue pressure and the variables pharyngeal tonsil hypertrophy and palatine tonsil hypertrophy. There were no statistical differences between the other variables. Conclusion It was concluded that mechanical obstructions, among them the pharyngeal and palatine tonsil hypertrophy alter the maximum tongue pressure in oral breathing children.

Multiple hepatic metastases of cardiac angiosarcoma

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson's capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcome.

Pesquisa de contaminantes no doce de leite produzido no setor de laticínios de instituição pública de ensino superior no Estado do Ceará / Search of contaminants in milk candy produced in the dairy sector of a public higher education institution in the State of Ceará

O Doce de leite é um importante derivado do leite, amplamente consumido em nosso País e possui uma grande importância nutricional. Diante do crescimento do consumo do doce de leite por parte da população, surge a preocupação de garantir que este alimento esteja isento de qualquer sujidade, mantendo-se o padrão de qualidade e identidade do produto. O objetivo do trabalho foi investigar a presença de contaminantes físicos e biológicos em quinze amostras de doce de leite processadas no setor de laticínio de uma instituição pública de ensino superior do Ceará. A pesquisa, de caráter experimental com abordagem qualitativa, foi desenvolvida no Laboratório de Segurança Alimentar e Nutricional (LABSAN) da Universidade Estadual do Ceará, no período de 11 de novembro de 2015 a 12 de fevereiro de 2016. As metodologias empregadas na avaliação das amostras foram: pesquisa macroscópica e microscópica de contaminantes físicos e biológicos. Foi realizada a inspeção das amostras sob uma superfície plana e os materiais estranhos foram removidos fazendo-se o uso de pinça dente de rato e colher de aço inoxidável estéril, onde os contaminantes foram identificados e caracterizados. A contaminação biológica foi avaliada por meio do método de sedimentação espontânea, com modificação. Verificou- se que todas as amostras estavam contaminadas, dentre a amostragem, 29% estavam contaminadas com ácaros, 23% tinham fibras, 18% com pelos, 12% apresentavam fragmentos de insetos e 12% continham larva de inseto. Os achados significativos e sua comparação com os padrões estabelecidos pelas legislações sanitárias vigentes, permitiram sugerir que as amostras do doce de leite estão inapropriadas para o consumo humano, sendo importante adotar medidas que reduzam as chances de contaminação, a fim de favorecer a prevenção de doenças veiculadas por esses produtos.

The Milk caramel is an important derivative of milk, widely consumed in our country and has great nutritional importance. Faced with the growth of the consumption of sweets by the population, a safety concern arises that this food is free of any dirt, maintaining the standard of quality and identity of the product. The objective of this work is to investigate a presence of physical and biological contaminants in fifteen samples of processed milk candy in the education sector of a public institution of higher education in Ceará. An experimental research in a qualitative approach developed at the Laboratory of Food and Nutrition Security (LABSAN) of the State University of Ceará, from November 11, 2015 to February 12, 2016. As methodologies used in the evaluation of samples: research macroscopic and microscopic analysis of physical and biological contaminants. Samples were inspected under a flat surface and the foreign materials were removed using metal and stainless steel tongs where the contaminants were identified and characterized. A biological contamination was evaluated through the spontaneous sedimentation method, with modification. Twenty-nine percent of the samples were contaminated with mites, 23% with fibers, 18% with hairs, 12% with insect remains and 12% with insect larvae. Considering the significant findings and these have been compared with the standards established by law, the current sanitary solutions allowed to suggest that as samples of dulce de leche are inappropriate for human consumption, it is important to adopt measures that reduce the chances of contamination, in order to favor disease prevention through products.

Hand-foot syndrome due to hepatitis C therapy

SUMMARY INTRODUCTION Direct-acting antivirals are new drugs for chronic hepatitis C treatment. They are usually safe and well tolerated, but can sometimes cause serious adverse effects and there is no consensus on how to treat or prevent them. We described a case of hand-foot syndrome due to hepatitis C virus interferon-free therapy. METHODS We report the case of a 49-year-old man with compensated liver cirrhosis due to chronic hepatitis C genotype 1, treatment-naïve, who started viral treatment with sofosbuvir, simeprevir and ribavirin for 12 weeks. RESULTS At the sixth week of treatment he had anemia, requiring a lower dose of ribavirin. At the tenth week, he had erythematous, pruritic, scaly and flaky lesions on hands and feet, which showed a partial response to oral antihistamines and topical corticosteroids. It was not necessary to discontinue antiviral treatment, but in the first week after the end of treatment, there was worsening of injuries, including signs of secondary infection, that required hospitalization, antibiotics and oral corticosteroid, with progressive improvement. Biopsy of the lesions was consistent with pharmacodermia. The patient had sustained a virological response, despite the side effect. He had a history of pharmacodermia one year ago attributed to the use of topiramate, responsive to oral corticosteroid. CONCLUSION Interferon-free therapies can rarely lead to severe adverse reactions, such as skin lesions. Patients receiving ribavirin combinations and those who had a history of pharmacodermia or skin disease may be more susceptible. There is no consensus on how to prevent skin reactions in these patients.

RESUMO INTRODUÇÃO Antivirais de ação direta são as novas drogas utilizadas no tratamento da hepatite C crônica. São geralmente seguros, com boa tolerância, mas eventualmente podem causar efeitos adversos graves, e não há consenso sobre como tratá-los ou preveni-los. Descrevemos um caso de síndrome mão-pé secundária à terapia livre de interferon para hepatite C crônica. Materiais e métodos Relatamos o caso de um paciente de 49 anos com cirrose hepática compensada secundária à hepatite C crônica, genótipo 1, virgem de tratamento, que iniciou terapia com sofosbuvir, simeprevir e ribavirina por 12 semanas. Resultados Na sexta semana de tratamento, apresentou anemia, sendo necessária redução de dose da ribavirina. Na 20a semana, apresentou lesões eritematosas e descamativas, com prurido em mãos e pés, que teve resposta parcial ao uso de anti-histamínico oral e corticoide tópico. Não foi necessário descontinuar os antivirais, mas na primeira semana após o término do tratamento, houve piora das lesões, com sinais de infecção secundária, sendo necessárias hospitalização e terapia com antibiótico e corticoide oral, com melhora progressiva. Biópsias das lesões foram compatíveis com farmacodermia. O paciente teve resposta virológica sustentada, apesar dos efeitos adversos. Tinha história de farmacodermia há um ano, atribuída ao uso de topiramato, responsiva a corticoterapia oral. Conclusão Os tratamentos livres de interferon raramente causam eventos adversos graves, como lesões cutâneas. Pacientes em uso de ribavirina e com história de farmacodermia ou doença cutânea prévia podem ser mais susceptíveis. Não existe consenso sobre como prevenir reações cutâneas nesses pacientes.

Multiple genes contribute to anhydrobiosis (tolerance to extreme desiccation) in the nematode Panagrolaimus superbus

Abstract The molecular basis of anhydrobiosis, the state of suspended animation entered by some species during extreme desiccation, is still poorly understood despite a number of transcriptome and proteome studies. We therefore conducted functional screening by RNA interference (RNAi) for genes involved in anhydrobiosis in the holo-anhydrobiotic nematode Panagrolaimus superbus. A new method of survival analysis, based on staining, and proof-of-principle RNAi experiments confirmed a role for genes involved in oxidative stress tolerance, while a novel medium-scale RNAi workflow identified a further 40 anhydrobiosis-associated genes, including several involved in proteostasis, DNA repair and signal transduction pathways. This suggests that multiple genes contribute to anhydrobiosis in P. superbus.

Retrospective analysis of hepatitis B virus chronic infection in 247 patients: clinical stages, response to treatment and poor prognostic factors

Abstract Background: Chronic hepatitis B is a major cause of cirrhosis, and the natural history of the disease has several clinical stages that should be thoroughly understood for the implementation of proper treatment. Nonetheless, curing the disease with antiviral treatment remains a challenge. Aims: To describe the clinical course, response to treatment, and poor prognostic factors in 247 hepatitis B virus chronic infection patients treated in a tertiary hospital in Brazil. Methods: This was a retrospective and observational study, by analyzing the medical records of HBV infected patients between January 2000 and January 2015. Results: Most patients were male (67.2%) and 74.1% were HBeAg negative. Approximately 41% had cirrhosis and 8.5% were hepatitis C virus coinfected. The viral load was negative after two years on lamivudine, entecavir and tenofovir in 86%, 90.6%, and 92.9% of the patients, respectively. The five-year resistance rates for lamivudine, adefovir, entecavir, and tenofovir were 57.5%, 51.8%, 1.9%, and 0%, respectively. The overall seroconversion rates were 31.2% for HBeAg and 9.4% for HBsAg. Hepatocellular carcinoma was diagnosed in 9.7% of patients, liver transplantation was performed in 9.7%, and overall mortality was 10.5%. Elevations of serum alanine aminotransferase (p = 0.0059) and viral load (p < 0.0001) were associated with progression to liver cirrhosis. High viral load was associated with progression to hepatocellular carcinoma (p < 0.0001). Significant risk factors associated with death were elevated alanine aminotransferase (p = 0.0039), liver cirrhosis (p < 0.0001), high viral load (p = 0.007), and hepatocellular carcinoma (p = 0.0008). HBeAg positive status was not associated with worse outcomes, and treatment may have been largely responsible. Conclusions: Elevations of viral load and serum alanine aminotransferase may select patients with worse prognosis, especially progression to cirrhosis and hepatocellular carcinoma, which were strongly association with death.

Pesquisa e desenvolvimento responsável? Traduzindo ausências a partir da nanotecnologia em Portugal / Responsible research and development? Translating absences from nanotechnology in Portugal

Resumo Analisa como a inovação responsável é discutida e implementada no contexto de uma das principais ações de fomento à nanotecnologia do governo português. Por meio da teoria do ator-rede e da sociologia das ausências, investigamos o processo de coprodução do International Iberian Nanotechnology Laboratory para identificar como preocupações quanto ao desenvolvimento responsável têm sido implementadas ou ausentadas nas normas e práticas. O instituto foi concebido a partir de um imaginário sociotécnico que o vislumbra como unidade autônoma de produção de inovações tecnológicas voltadas exclusivamente para o aumento da competitividade num mercado global, o que tem servido como obstáculo para práticas robustas de desenvolvimento responsável.

Abstract This article analyzes how responsible innovation has been discussed and implemented in the context of one of the Portuguese government’s main activities to foster nanotechnology. Through the actor-network theory and the sociology of absences, we investigate the process of coproduction at the International Iberian Nanotechnology Laboratory to identify how concerns about responsible development have been implemented or ignored in the rules and practices. The institute emerged from a sociotechnical imagination that views it as an autonomous unit for producing technological innovations aimed exclusively at increasing competitiveness in a global market, which has been an obstacle to the materialization of robust responsible development practices.

Avaliação de tecnologias de saúde e políticas informadas por evidências / Health technology assessment and evidence-informed policies

O Instituto de Saúde é uma instituição de ensino e pesquisa da Secretaria de Estado da Saúde de São Paulo, criada em 1969, que tem como missão "produzir conhecimento científico e tecnológico no campo da Saúde Coletiva e promover sua apropriação para o desenvolvimento de políticas públicas, visando à melhoria da qualidade de vida da população, prestando assessoria e colaborando na formação de recursos humanos, em consonância com os princípios do SUS: universalidade, integralidade, equidade e participação social". Em 2009, passamos por uma reorganização na estrutura funcional, quando foi criado o Centro de Tecnologias de Saúde para o SUS-SP, em resposta a novas necessidades da gestão da saúde no estado de São Paulo. As atribuições desse Centro consistem na realização da avaliação de tecnologias de saúde em uso e a serem incorporadas pelo Sistema Único de Saúde, na proposição de parcerias e cooperação técnica para o desenvolvimento da avaliação de tecnologias de saúde, com as diferentes instâncias dos sistemas pertinentes, na difusão do conhecimento produzido, promovendo o fomento e a indução da avaliação de tecnologias de saúde para o SUS/SP, de acordo com a agenda de prioridades definidas pela SES-SP. Para realizar essas atribuições tivemos que buscar meios para rapidamente capacitar pesquisadores em Avaliação de Tecnologias de Saúde (ATS) e em Políticas Informadas por Evidências (PIE). Isso foi possível graças aos cursos presenciais e à distância, patrocinados pelo Ministério da Saúde e seus parceiros. A possibilidade de participarmos como membros da Rede Brasileira de Avaliação de Tecnologias em Saúde (REBRATS) desde 2012, e da Rede para Políticas Informadas por Evidências (EVIPNet Brasil) desde 2014, ampliou sobremaneira nosso aprendizado e capacidade de lidar com esses dois temas.

Mycosis fungoides and Kaposi's sarcoma association in an HIV-negative patient

Abstract The association of mycosis fungoides and kaposi’s sarcoma in HIV-negative patients is a rare phenomenon. The presence of human herpesvirus 8 (HHV-8) – associated with all forms of Kaposi’s sarcoma – has also been recently identified in mycosis fungoides lesions. However, a causal association between HHV-8 and the onset of mycosis fungoides has not been established yet. The present case reports a patient who developed Kaposi’s sarcoma lesions after a two-year UVB phototherapy to treat a mycosis fungoides. Negative immunohistochemistry staining for Kaposi’s sarcoma-associated herpesvirus in the initial mycosis fungoides lesions strengthens the absence of a link between Kaposi’s sarcoma-associated herpesvirus and mycosis fungoides. Immunosuppression caused by the lymphoma and prolonged phototherapy were probably the contribut ing factors for the onset of Kaposi’s sarcoma.

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

ABSTRACT In order to draw evidence-based recommendations concerning the management of autoimmune diseases of the liver, the Brazilian Society of Hepatology has sponsored a single-topic meeting in October 18th, 2014 at São Paulo. An organizing committee comprised of seven investigators was previously elected by the Governing Board to organize the scientific agenda as well as to select twenty panelists to make a systematic review of the literature and to present topics related to the diagnosis and treatment of autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis and their overlap syndromes. After the meeting, all panelists gathered together for the discussion of the topics and the elaboration of those recommendations. The text was subsequently submitted for suggestions and approval of all members of the Brazilian Society of Hepatology through its homepage. The present paper is the final version of the reviewed manuscript organized in topics, followed by the recommendations of the Brazilian Society of Hepatology.

RESUMO Para definir as recomendações baseadas em evidências científicas sobre o diagnóstico e tratamento das doenças autoimnus do fígado, a Sociedade Brasileira de Hepatologia organizou em Outubro de 2014, encontro monotemático em São Paulo. Um Comitê organizador de sete investigadores foi selecionado pela Diretoria da Sociedade para organizar a agenda científica, assim como para selecionar vinte debatedores para fazer uma revisão sistemática e apresentar tópicos relacionados à hepatite autoimune, colangite esclerosante primária, cirrose biliar primária e suas síndromes de superposição (overlap). O texto inicial do submetidoo a apreciação e aprovação da Sociedade Brasileira de Hepatologia através de consulta a todos associados através da home page da Sociedade, O trabalho apresentado representa a versão final do trabalho original, devidamente revisado e organizado em tópicos, segundo as recomendações da Sociedade Brasileira de Hepatologia.

Esthetic perception of the buccal corridor in different facial types by laypersons of different ages / Percepção estética do corredor bucal em diferentes tipos faciais por leigos de diferentes idades

The focus of this study was to test the hypothesis that the amount of buccal corridor associated with dolicofacial, mesofacial and brachyfacial facial patterns has no influence on smile evaluations by laypersons of different age groups. A photograph was constructed of a woman displaying a broad smile. Buccal corridors were modified digitally in increments of 5% and 10%, displaying from 0% to 30% buccal corridor compared with the inner commissural width. Using a visual analog scale(VAS), 150 laypersons divided into 3 groups(n=50) of age-ranges from 15-19, 35-44 and 65-74 years of age rated the attractiveness of five smiles with altered buccal corridors. Differences in the median esthetic scores were analyzed using the Kruskal-Wallis test (P<0.05). Laypersons over the age of 65 years made a less critical judgment as regards attractiveness of the smile. In the comparison between groups of individuals of different ageranges for the size of the buccal corridor at 0%, 10% and 15% there was no significant difference for any of the facial types(P>0.05). There was significant difference between the groups from 15-19 and 65-74 years of age in evaluating the attractiveness of the smile with the size of the buccal corridor at 20% and 30% for the short(P=0.045, and P=0.035) and long (P=0.029, and P=0.038) facial types, respectively. The hypothesis was rejected. Laypersons over the age of 65 years attributed higher scores. Irrespective of age, laypersons preferred broader smiles with a smaller buccal corridor, with 15% being the limit between the most and least attractive smile.

O foco deste estudo foi testar a hipótese que a quantidade de corredor bucal associada aos padrões faciais dolicofacial, mesofacial e braquifacial não tem qualquer influência sobre as avaliações do sorriso de leigos de diferentes faixas etárias. Uma fotografia de uma mulher exibindo um amplo sorriso foi construída. Corredores bucais foram modificados digitalmente em incrementos de 5% e 10%, de 0% a 30% de corredor bucal em comparação com a largura interna das comissuras. Utilizando uma escala analógica visual (VAS), 150 indivíduos leigos divididos em 3 grupos (n=50) de faixas etárias de 15-19, 35-44 e 65-74 de idade avaliaram a atratividade de cinco sorrisos com corredores bucais alterados. Diferenças nas pontuações estéticas medianas foram analisadas utilizando o teste de Kruskal-Wallis (P <0,05). Indivíduos leigos acima de 65 anos fizeram um julgamento menos critico quanto à atratividade do sorriso. Na comparação entre grupos de indivíduos de diferentes faixas etárias para o tamanho do corredor bucal em 0%, 10% e 15% não houve diferença significante para nenhum dos tipos faciais (P>0.05). Houve diferença significante entre os grupos de 15-19 e 65-74 anos na avaliação da atratividade do sorriso com tamanho do corredor bucal de 20% e 30% para os tipos faciais, curto (P=0.045, e P=0.035) e longo (P=0.029, e P=0.038), respectivamente. A hipótese foi rejeitada. Leigos acima de 65 deram as maiores pontuações. Independentemente da idade, leigos preferiram sorrisos mais largos com menor corredor bucal, sendo 15% o limite entre o sorriso mais e menos atraente.

Eficácia e segurança da terapia com idursulfase em pacientes com mucopolissacaridose tipo II, com e sem comparação com placebo: revisão sistemática e metanálise / Efficacy and safety of idursulfase therapy in patients with mucopolysaccharidosis type II with and without comparison to placebo: systematic review and meta-analysis / Eficacia y seguridad del tratamiento con idursulfasa en pacientes con mucopolisacaridosis tipo II con y sin comparación con el placebo: revisión sistemática y metaanálisis

A mucopolissacaridose tipo II (MPS II) é uma doença genética de amplo espectro clínico, caracterizada por deficiência da enzima iduronato-2sulfatase. Revisão sistemática avaliou a eficácia e segurança da terapia de reposição enzimática (TRE) com idursulfase (IDS) na MPS II. As bases de dados PubMed/MEDLINE, Embase, LILACS e Biblioteca Cochrane foram pesquisados até 30 de novembro de 2012. Apenas cinco estudos preencheram os critérios de inclusão (ensaios clínicos randomizados - ECRs, ECRs abertos ou séries de caso prospectivas, incluindo cinco ou mais pacientes e avaliando desfechos relevantes). Metanálise foi realizada para capacidade vital forçada (CVF; valores absolutos e em %) e para a distância percorrida no teste da caminhada dos seis minutos, com mudanças significativas em ambas as variáveis; também foi encontrado risco aumentado de reações leves relacionadas à infusão e de desenvolvimento de anticorpos IgG à IDS. Em face dos dados apresentados neste estudo, conclui-se que a TRE com IDS é segura e tem benefício potencial em MPS II, mas estudos adicionais são necessários.

Mucopolysaccharidosis type II (MPS II) is a genetic disease of broad clinical spectrum, characterized by a deficiency of the enzyme iduronate2-sulfatase. The aim of this study was to assess whether enzyme replacement therapy (ERT) with idursulfase (IDS) for MPS II is effective and safe. PubMed/MEDLINE, Embase, LILACS, and Cochrane Library were searched until November 30, 2012. Only five articles met the inclusion criteria (randomized controlled trials - RCTs, or open-label trials/prospective case series including > 5 patients and evaluating relevant outcomes). A meta-analysis was performed for forced vital capacity (FVC; absolute and %) and for distance walked on the 6-minute walking test (6MWT). There was a statistically significant increase, but not clinically relevant, in both variables; an increased risk for development of mild infusion-related reactions and IgG antibodies to IDS were also found. The data suggest that ERT with IDS is safe and has a potential benefit for MPS II patients, but further studies are required.

La mucopolisacaridosis tipo II (MPS II) es una enfermedad genética de amplio espectro clínico, caracterizada por una deficiencia de la enzima iduronato-2-sulfatasa. El objetivo fue evaluar la seguridad y eficacia de la Terapia de Reemplazo Enzimático (TRE) con idursulfasa (IDS) en la MPS II. En las bases PubMed/MEDLINE, EMBASE, LILACS y Cochrane Library se inició la búsqueda hasta el 30 de noviembre de 2012. Sólo cinco estudios cumplieron los criterios de inclusión (ensayos controlados aleatorios -ECA, o ECA abiertos o series de casos prospectivo incluyendo > 5 pacientes y evaluación de los resultados pertinentes). El metaanálisis se realizó para la capacidad vital forzada (FVC; absoluta y %) y la distancia caminada en 6 minutos, con cambios significativos en ambas variables; el riesgo también se encuentra aumentado por reacciones leves y anticuerpos IgG, relacionados con la infusión con IDS. El TRE con IDS es seguro y tiene un beneficio potencial en la MPS II, pero se necesitan estudios adicionales.

Pyrethroid toxicity in silver catfish, Rhamdia quelen / Toxicidade dos piretróides sobre Jundiás, Rhamdia quelen

This study aimed to determine both the lethal and sublethal concentrations of Cypermethrin in young Silver Catfish (Brazilian "Jundiá", Rhamdia quelen) on aquatic environment during 96 hours, as well as to determine the Cypermethrin and Deltamethrin sublethal concentrations during the initial embryonic development period of Rhamdia quelen, and to verify their respective rates of fertilization, hatching and survival. Pyrethroid nowadays is a widely used insecticide, which presents a high toxicity to fish. In order to determine lethal and sublethal concentrations, 120 silver catfish were used; each one had an average weight of 59.58±4.50g and an average size of 20.33±2.34cm. Concentrations used were 0, 1.0, 1.5, 2.0, 2.5, 3.0, 5.0, 10.0, 15.0 and 20.0mg of Cypermethrin per liter of water (mg/L). Fish were exposed to the product in 30-liter fish tanks. In each fish tank there were four fishes and the product was applied three times, i.e., a total of twelve fish were exposed to the product at each application, and a total of 120 fish during the entire experiment (n=120). In order to determine the Cypermethrin and Deltamethrin sublethal concentrations during the initial embryonic development, ovulation induction was performed on female fishes using hormones, and then and egg collection was performed. The eggs were then hydrated and fertilized in Cypermethrin and Deltamethrin in different concentrations: 0.001, 0.01, 0.1, 1.0 and 10.0mg/L of Cypermethrin and 0.001, 0.01, 0.1, 0.5 and 1.0mg/L of Deltamethrin, in addition to the control group (0mg/L). After fertilization, the eggs were kept in containers with the respective pesticides of Cypermethrin and Deltamethrin until hatching, when hatching rate was verified. Then the alevins, from the hatching, were kept on their respective concentrations of Cypermethrin and Deltamethrin so that the survival rate could be analyzed regarding the tested insecticides, during both 12-hour and 24-hour periods. Concerning the...

O presente estudo teve o objetivo de determinar a concentração letal e subletal da cipermetrina por via hídrica em 96 horas em jundiás (Rhamdia quelen) jovens, determinar a concentração subletal da cipermetrina e da deltametrina durante o período inicial do desenvolvimento embrionário do Rhamdia quelen e verificar suas respectivas taxas de fertilização, eclosão e sobrevivência. Os piretróides são inseticidas amplamente utilizados atualmente e apresentam alta toxicidade para peixes. Para determinação da concentração letal e subletal foram utilizados 120 jundiás com peso médio de 59,58±4,50g e comprimento médio de 20,33±2,34cm. As concentrações utilizadas foram 0; 1,0; 1,5; 2,0; 2,5; 3,0; 5,0; 10,0; 15,0 e 20,0mg de cipermetrina por litro de água (mg/L). Os peixes foram expostos ao produto dentro de aquários com capacidade para 30 litros. Em cada aquário foram colocados quatro peixes e cada tratamento teve três repetições, totalizando doze peixes por tratamento. Para determinar a concentração subletal da cipermetrina e deltametrina na fase de desenvolvimento embrionário foi realizada a indução da ovulação em fêmeas de jundiá através de hormônios, com posterior coleta dos ovos. Os ovos foram hidratados e fertilizados em soluções de cipermetrina e deltametrina em diferentes concentrações, sendo estas: 0,001; 0,01; 0,1; 1,0 e 10,0mg/L para cipermetrina e 0,001; 0,01; 0,1; 0,5 e 1,0mg/L para deltametrina, além do grupo controle (0mg/L). Após a fertilização, os ovos permaneceram em recipientes com os respectivos pesticidas até o momento da eclosão onde foi analisada a taxa de eclosão. Em seguida as larvas, a partir da eclosão dos ovos, foram mantidas nas respectivas concentrações de cipermetrina e deltametrina para analisar a taxa de sobrevivência frente aos inseticidas testados, durante 12 e 24 horas. Em relação as concentrações letais e subletais de jundiás jovens, estes apresentaram-se sensíveis à cipermetrina nas concentrações testadas, ...