Sao Paulo Medical Journal, Volume: 131, Issue: 4, Published: 2013
  • From classroom to bedside: integration of the basic science curriculum in medical teaching Editorial

    Mariani, Alessandro Wasum; Pêgo-Fernandes, Paulo Manuel
  • Clustering and combining pattern of metabolic syndrome components in a rural Brazilian adult population Original Article

    Pimenta, Adriano Marçal; Felisbino-Mendes, Mariana Santos; Velasquez-Melendez, Gustavo

    Abstract in Portuguese:

    CONTEXTO E OBJETIVO A síndrome metabólica é caracterizada pela agregação de fatores de risco cardiovasculares como obesidade, dislipidemia, resistência à insulina, hiperinsulinemia, intolerância à glicose e hipertensão arterial. Este estudo objetivou estimar a probabilidade de agregação e o padrão de combinação de três ou mais componentes da síndrome metabólica em população rural adulta brasileira. TIPO DE ESTUDO E LOCAL Estudo transversal, conduzido em duas comunidades rurais da região do Vale do Jequitinhonha, Minas Gerais. MÉTODOS A amostra foi constituída de 534 adultos, de ambos os sexos, dos quais foram aferidas a circunferência da cintura, a pressão arterial e características demográficas, do estilo de vida e bioquímicas. Prevalências da síndrome metabólica e seus componentes foram estimados usando a definição da National Cholesterol Education Program – Adult Treatment Panel III. A equação da distribuição binomial foi utilizada para avaliar a probabilidade de agregação dos componentes da síndrome metabólica. O nível de significância estatística estabelecido foi 5% (P < 0,05). RESULTADOS Síndrome metabólica foi mais frequente em mulheres (23,3%) que homens (6,5%). A agregação de três ou mais componentes da síndrome metabólica foi maior do que esperada ao acaso. Combinações mais comuns para três componentes da síndrome metabólica foram hipertrigliceridemia + baixos níveis de HDL-c + hipertensão arterial, obesidade abdominal + baixos níveis de HDL-c + hipertensão arterial. Para quatro componentes, obesidade abdominal + hipertrigliceridemia + baixos níveis de HDL-c + hipertensão arterial. CONCLUSÃO Na população estudada, a prevalência da síndrome metabólica foi alta entre mulheres e houve agregação dos seus componentes acima do esperado que ocorra ao acaso, sugerindo padrão não aleatório de combinação.

    Abstract in English:

    CONTEXT AND OBJECTIVE Metabolic syndrome is characterized by clustering of cardiovascular risk factors such as obesity, dyslipidemia, insulin resistance, hyperinsulinemia, glucose intolerance and arterial hypertension. The aim of this study was to estimate the probability of clustering and the combination pattern of three or more metabolic syndrome components in a rural Brazilian adult population. DESIGN AND SETTING This was a cross-sectional study conducted in two rural communities located in the Jequitinhonha Valley, Minas Gerais, Brazil. METHODS The sample was composed of 534 adults (both sexes). Waist circumference, blood pressure and demographic, lifestyle and biochemical characteristics were assessed. The prevalences of metabolic syndrome and its components were estimated using the definitions of the National Cholesterol Education Program – Adult Treatment Panel III. A binomial distribution equation was used to evaluate the probability of clustering of metabolic syndrome components. The statistical significance level was set at 5% (P < 0.05). RESULTS Metabolic syndrome was more frequent among women (23.3%) than among men (6.5%). Clustering of three or more metabolic syndrome components was greater than expected by chance. The commonest combinations of three metabolic syndrome components were: hypertriglyceridemia + low levels of HDL-c + arterial hypertension and abdominal obesity + low levels of HDL-c + arterial hypertension; and of four metabolic syndrome components: abdominal obesity + hypertriglyceridemia + low levels of HDL-c + arterial hypertension. CONCLUSION The population studied presented high prevalence of metabolic syndrome among women and clustering of its components greater than expected by chance, suggesting that the combination pattern was non-random.
  • Obstructive sleep apnea syndrome: complaints and housing characteristics in a population in the United States Original Article

    Ansarin, Khalil; Sahebi, Leyla; Sabur, Siamak

    Abstract in Portuguese:

    CONTEXTO E OBJETIVO Síndrome de apneia obstrutiva do sono (SAOS) é uma das principais causas de morbidade e mortalidade em adultos. Detecção precoce da doença e descoberta de fatores de risco com questionários padronizados levarão a redução dos danos por SAOS. O principal objetivo deste estudo foi estimar a prevalência de sintomas de SAOS e examinar sua associação com características da habitação. TIPO DE ESTUDO E LOCAL Estudo transversal em faculdade de medicina. MÉTODOS Dados demográficos, habitacionais e de medidas corporais sobre 5.545 indivíduos de 16 anos ou mais, de diversas raças, foram selecionados do National Health and Nutrition Examination Survey. Analisamos a probabilidade de SAOS com base no ronco habitual combinada com sonolência diurna e/ou apneia testemunhada. Análise univariada e regressão linear múltipla foram usadas. RESULTADOS 9,8% dos homens e 6,9% das mulheres relataram sintomas sugestivos de SAOS (ronco habitual, sonolência diurna e/ou apneia). A prevalência de sintomas em homens e mulheres, respectivamente, foi: ronco frequente 35,1%, 22,3%, sonolência excessiva diurna 6,4%, 3,4% e apneia frequente 14,9%, 20,6%. Através de regressão linear múltipla, sintomas de SAOS foram relacionados com gênero, idade, índice de massa corpórea (IMC), estado civil e educação. Das características da habitação, mofo ou cheiro de mofo e animais de estimação no ambiente foram associados com alta probabilidade de SAOS. CONCLUSÃO Sintomas de SAOS foram mais prevalentes do que nos países em desenvolvimento. O meio ambiente foi um fator de risco importante, porém é mais fácil controlar e manejar fatores ambientais do que outras variáveis, como IMC ou status socioeconômico.

    Abstract in English:

    CONTEXT AND OBJECTIVE Obstructive sleep apnea syndrome (OSAS) is one of the leading causes of morbidity and mortality in adults. Early detection of the disorder and discovery of risk factors through standardized questionnaires will lead to reduction of the OSAS burden. The main aim of this study was to estimate the prevalence of OSAS symptoms and examine their association with housing characteristics. DESIGN AND SETTING Cross-sectional study at a medical school. METHODS Demographic, housing and body measurement data on 5,545 individuals aged 16 years and over of various races were selected from the National Health and Nutrition Examination Survey. We analyzed the probability of OSAS based on habitual snoring combined with daytime sleepiness and/or witnessed apnea. Univariate and multiple linear regression were used. RESULTS 9.8% of the men and 6.9% of the women reported symptoms suggestive of OSAS (habitual snoring, daytime sleepiness and/or apnea). The following prevalences of symptoms were found among males and females respectively: frequent snoring 35.1%, 22.3%, excessive daytime sleepiness 6.4%, 3.4% and frequent apnea 14.9%, 20.6%. Using multiple linear regression, OSAS symptoms were correlated with gender, age, body mass index (BMI), marital status and education. Regarding housing characteristics, mildew or musty smell and pets in the environment were associated with a high probability of OSAS. CONCLUSION OSAS symptoms were more prevalent than in developing countries. The environment was an important risk factor, but environmental factors are easier to control and manage than other variables like BMI or socioeconomic status.
  • Circumstances and factors associated with accidental deaths among children, adolescents and young adults in Cuiabá, Brazil Original Article

    Martins, Christine Baccarat de Godoy; Mello-Jorge, Maria Helena Prado de

    Abstract in Portuguese:

    CONTEXTO E OBJETIVO Ao analisar os acidentes sob o prisma dos segmentos populacionais, observa-se grande incidência em crianças, adolescentes e jovens. A frequência e as características e circunstâncias do evento estão intimamente relacionadas com fatores educacionais, econômicos, sociais e culturais, e a identificação desses fatores pode contribuir para minimizar essas causas, muitas vezes fatais. O objetivo foi identificar os fatores ambientais, químicos, biológicos e culturais associados com óbitos por acidentes, ocorridos entre crianças, adolescentes e jovens em Cuiabá, em 2009. TIPO DE ESTUDO E LOCAL Este é um estudo descritivo, transversal, realizado em Cuiabá, Mato Grosso, Brasil. RESULTADOS Foram analisados 39 óbitos acidentais ocorridos de 0 a 24 anos (56,4% por acidente de transporte, 25,6% por afogamento, 10,3% por aspiração de leite, 5,1% por queda e 2,6% por disparo acidental de arma de fogo). Houve predomínio no sexo masculino (82,1%). Observou-se a presença de fatores químicos, ambientais e biológicos na quase totalidade das residências. Quanto aos fatores culturais e hábitos, grande parte das famílias não soube referir se o acidente constitui evento previsível e outra parte não acredita que os hábitos da família favorecem sua ocorrência. Delegar aos filhos menores de 10 anos os afazeres domésticos ou o cuidado de irmãos menores é comum entre as famílias estudadas. CONCLUSÃO Os resultados apontam para a necessidade de adotar comportamentos e ambientes seguros e saudáveis, bem como monitorizar a ocorrência dos acidentes, estruturando e consolidando o atendimento às vítimas.

    Abstract in English:

    CONTEXT AND OBJECTIVE Analysis on accidents from the perspective of population segments shows there is higher incidence among children, adolescents and young adults. Since the characteristics and circunstances of the event are closely related to educational, economic, social and cultural issues, identifying them may contribute towards minimizing the causes, which are often fatal. The aim here was to identify the environmental, chemical, biological and cultural factors associated with deaths due to accidents among children, adolescents and young adults in Cuiabá, in 2009. DESIGN AND SETTING This was a descriptive cross-sectional study conducted in Cuiabá, Mato Grosso, Brazil. RESULTS Thirty-nine accidental deaths of individuals aged 0 to 24 years were examined: 56.4% due to traffic accidents; 25.6%, drowning; 10.3%, aspiration of milk; 5.1%, falls; and 2.6%, accidentally triggering a firearm. Male victims predominated (82.1%). The presence of chemical, environmental and biological risk factors was observed in almost all of the homes. Regarding cultural factors and habits, a large proportion of the families had no idea whether accidents were foreseeable events and others did not believe that the family's habits might favor their occurrence. Delegation of household chores or care of younger siblings to children under the age of 10 was common among the families studied. CONCLUSION The results point towards the need to have safe and healthy behavioral patterns and environments, and to monitor occurrences of accidents, thereby structuring and consolidating the attendance provided for victims.
  • Clinical and hematological effects of hydroxyurea therapy in sickle cell patients: a single-center experience in Brazil Original Article

    Silva-Pinto, Ana Cristina; Angulo, Ivan Lucena; Brunetta, Denise Menezes; Neves, Fabia Idalina Rodrigues; Bassi, Sarah Cristina; Santis, Gil Cunha De; Covas, Dimas Tadeu

    Abstract in Portuguese:

    CONTEXTO E OBJETIVO A doença falciforme (SCD) é o distúrbio genético mais comum entre afrodes-cendentes, afetando aproximadamente 3.500 recém-nascidos a cada ano no Brasil. A hidroxiureia (HU) é a única droga efetiva para o tratamento dos pacientes com SCD, reduzindo a morbidade e a mortalidade da doença. O objetivo do estudo foi analisar os efeitos da HU em pacientes com SCD em nossa instituição. TIPO DE ESTUDO E LOCAL Estudo retrospectivo realizado em um centro de anemia falciforme em Ribeirão Preto, São Paulo, Brasil. MÉTODOS Nós analisamos os dados clínicos e laboratoriais de 37 pacientes. Os parâmetros hematológicos e eventos clínicos que ocorreram no ano anterior e durante o primeiro ano de tratamento com HU foram analisados. A dose média de HU foi 24.5 ± 5.5 mg/kg/dia. RESULTADOS Houve aumento em três parâmetros estudados: hemoglobina (8,3 g/dl para 9,0 g/dl, P = 0,0003), hemoglobina fetal (HbF) (2,6% para 19,8%, P < 0,0001) e volume corpuscular médio (VCM) (89 para 105 fl, P = 0,001); e redução do número de leucócitos (10.050/µl para 5.700/µl, P < 0,0001), neutrófilos (6.200/µl para 3.400/µl, P = 0,001), plaquetas (459.000/µl para 373.000/µl, P = 0,0002), crises dolorosas (1,86 para 0,81, P = 0,0014), síndrome torácica aguda (0,35 para 0,08, P = 0,0045), infecções (1,03 para 0,5, P = 0,047), hospitalizações (1,63 para 0,53, P = 0,0013) e número de transfusões (1,23 para 0,1, P = 0,0051). CONCLUSÃO Os pacientes apresentaram melhora clínica e hematológica, com aumento da HbF e redução da taxa de infecção, dado este não explorado na maioria dos estudos clínicos já publicados.

    Abstract in English:

    CONTEXT AND OBJECTIVES Sickle cell disease (SCD) is the most common genetic disorder among people of African descent, affecting approximately 3,500 newborns each year in Brazil. Hydroxyurea (HU) is the only effective drug to treating patients with SCD, thereby reducing morbidity and mortality. The objective was to analyze the effects of HU on SCD patients at our institution. DESIGN AND SETTING Retrospective study conducted at a sickle cell centre in Ribeirão Preto, São Paulo, Brazil. METHODS We analyzed clinical and laboratory data on 37 patients. The hematological parameters and clinical events that occurred during the year before and the first year of treatment with HU were analyzed. The mean dose of HU was 24.5 ± 5.5 mg/kg/day. RESULTS There were rises in three parameters: hemoglobin (8.3 g/dl to 9.0 g/dl, P = 0.0003), fetal hemoglobin (HbF) (2.6% to 19.8%, P < 0.0001) and mean cell volume MCV (89 to 105 fl, P = 0.001); and reductions in the numbers of leukocytes (10,050/µl to 5,700/µl, P < 0.0001), neutrophils (6,200/µl to 3,400/µl, P = 0.001), platelets (459,000/µl to 373,000/µl, P = 0.0002), painful crises (1.86 to 0.81, P = 0.0014), acute chest syndromes (0.35 to 0.08, P = 0.0045), infections (1.03 to 0.5, P = 0.047), hospitalizations (1.63 to 0.53, P = 0.0013) and transfusions (1.23 to 0.1, P = 0.0051). CONCLUSION The patients presented clinical and hematological improvements, with an increase in HbF and a reduction in the infection rate, which had not been addressed in most previous studies.
  • Translation, cross-cultural adaptation and validation of the Brazilian version of the Nonarthritic Hip Score Original Article

    Castillo, Letícia Nunes Carreras Del; Leporace, Gustavo; Cardinot, Themis Moura; Levy, Roger Abramino; Oliveira, Liszt Palmeira de

    Abstract in Portuguese:

    CONTEXTO E OBJETIVO O Nonarthritic Hip Score (NAHS) é um questionário de avaliação clínica que foi desenvolvido na língua inglesa para avaliar a função do quadril em pacientes jovens e fisicamente ativos. O objetivo desse estudo foi traduzir o questionário NAHS para a língua portuguesa do Brasil, adaptá-lo à cultura brasileira e validá-lo. TIPO DE ESTUDO E LOCAL Estudo de coorte, realizado entre 2008 e 2010, na Universidade do Estado do Rio de Janeiro (UERJ). MÉTODOS Questões sobre atividades físicas e tarefas domésticas foram modificadas para melhor adaptação à cultura brasileira. Reprodutibilidade, consistência interna e validade (correlação entre o Algofunctional Lequesne Index e o Western Ontario McMaster Universities Arthritis Index [WOMAC]) foram testadas. Os questionários NAHS-Brasil, Lequesne e WOMAC foram aplicados em 64 pacientes jovens e fisicamente ativos (média de idade, 40,9 anos; 31 mulheres). RESULTADOS O coeficiente de correlação intraclasse que avalia reprodutibilidade mostrou valores de 0,837 (P < 0,001). O gráfico de Bland-Altman revelou um erro médio da diferença das duas medidas de 0,42. A consistência interna foi avaliada pelo alfa de Cronbach com valores de 0,944. A validade entre os questionários NAHS-Brasil/Lequesne e NAHS-Brasil/WOMAC mostraram alta correlação, r = 0,7340 e r = 0,9073; respectivamente. O NAHS-Brasil mostrou boa validade sem efeitos de chão ou de teto. CONCLUSÃO O NAHS foi traduzido para a língua portuguesa do Brasil e adaptado para a cultura brasileira, mostrando ser uma ferramenta útil na prática clínica para avaliar a qualidade de vida de pacientes jovens e fisicamente ativos com dor no quadril.

    Abstract in English:

    CONTEXT AND OBJECTIVE The Nonarthritic Hip Score (NAHS) is a clinical evaluation questionnaire that was developed in the English language to evaluate hip function in young and physically active patients. The aims of this study were to translate this questionnaire into the Brazilian Portuguese language, to adapt it to Brazilian culture and to validate it. DESIGN AND SETTING Cohort study conducted between 2008 and 2010, at Universidade do Estado do Rio de Janeiro (UERJ). METHODS Questions about physical activities and household chores were modified to better fit Brazilian culture. Reproducibility, internal consistency and validity (correlations with the Algofunctional Lequesne Index and the Western Ontario and McMaster Universities Arthritis Index [WOMAC]) were tested. The NAHS-Brazil, Lequesne and WOMAC questionnaires were applied to 64 young and physically active patients (mean age, 40.9 years; 31 women). RESULTS The intraclass correlation coefficient (which measures reproducibility) was 0.837 (P < 0.001). Bland-Altman plots revealed a mean error in the difference between the two measurements of 0.42. The internal consistency was confirmed through a Cronbach alpha of 0.944. The validity between NAHS-Brazil and Lequesne and between NAHS-Brazil and WOMAC showed high correlations, r = 0.7340 and r = 0.9073, respectively. NAHS-Brazil showed good validity with no floor or ceiling effects. CONCLUSION The NAHS was translated into the Brazilian Portuguese language and was cross-culturally adapted to Brazilian culture. It was shown to be a useful tool in clinical practice for assessing the quality of life of young and physically active patients with hip pain.
  • The IDEAL classification system: a new method for classifying fractures of the distal extremity of the radius – description and reproducibility Original Article

    Belloti, João Carlos; Santos, João Baptista Gomes dos; Moraes, Vinícius Ynoe de; Wink, Felipe Vitiello; Tamaoki, Marcel Jun Sugawara; Faloppa, Flávio

    Abstract in Portuguese:

    CONTEXTO E OBJETIVO Não existe consenso sobre qual é a melhor classificação para as fraturas do rádio distal e os métodos existentes apresentam baixa reprodutibilidade. Este estudo tem como objetivo descrever e avaliar a reprodutibilidade de uma nova classificação (a IDEAL) comparando-a com as classificações mais amplamente utilizadas. TIPO DE ESTUDO E LOCAL Estudo de reprodutibilidade, Disciplina de Cirurgia da Mão, Universidade Federal de São Paulo. MÉTODOS Apresentamos a classificação IDEAL e sua fundamentação teórica baseada em evidências. Sessenta radiografias (anteroposterior e de perfil) de pacientes com fraturas do rádio distal foram classificadas por seis examinadores: um especialista e um residente de cirurgia da mão, um ortopedista, um residente de ortopedia e dois estudantes de medicina. Cada um, independentemente, avaliou as radiografias em três momentos diferentes. Analisamos a concordância intra e interobservador da classificação IDEAL, AO, Frykman e Fernandez, utilizando o kappa (κ) de Cohen (para dois observadores) e κ de Fleiss (para mais de dois observadores). RESULTADOS A concordância demonstrou-se elevada para a classificação IDEAL (κ = 0,771) e moderada para Frykman (κ = 0,556), Fernandez (κ = 0,671) e AO (κ = 0,650). A concordância interobservador foi moderada para a classificação IDEAL (κ = 0,595), mas insatisfatória para Frykman (κ = 0,344), Fernandez (κ = 0,496) e AO (κ = 0,343). CONCLUSÃO A reprodutibilidade da classificação IDEAL se demonstrou superior quando comparada às analisadas neste estudo, tornando a classificação IDEAL adequada para aplicação. Estudos complementares confirmarão se esta classificação é adequada para previsão de terapia e prognóstico.

    Abstract in English:

    CONTEXT AND OBJECTIVE There is no consensus concerning which classification for distal radius fractures is best and the existing methods present poor reproducibility. This study aimed to describe and assess the reproducibility of the new IDEAL classification, and to compare it with widely used systems. DESIGN AND SETTING Reproducibility study, Hand Surgery Section, Universidade Federal de São Paulo. METHODS The IDEAL classification and its evidence-based rationale are presented. Sixty radiographs (posteroanterior and lateral) from patients with distal radius fractures were classified by six examiners: a hand surgery specialist, a hand surgery resident, an orthopedic generalist, an orthopedic resident and two medical students. Each of them independently assessed the radiographs at three different times. We compared the intra and interobserver concordance of the IDEAL, AO, Frykman and Fernandez classifications using Cohen's kappa (κ) (for two observers) and Fleiss's κ (for more than two observers). RESULTS The concordance was high for the IDEAL classification (κ = 0.771) and moderate for Frykman (κ = 0.556), Fernandez (κ = 0.671) and AO (κ = 0.650). The interobserver agreement was moderate for the IDEAL classification (κ = 0.595), but unsatisfactory for Frykman (κ = 0.344), Fernandez (κ = 0.496) and AO (κ = 0.343). CONCLUSION The reproducibility of the IDEAL classification was better than that of the other systems analyzed, thus making the IDEAL system suitable for application. Complementary studies will confirm whether this classification system makes adequate predictions for therapy and prognosis.
  • Trends in treatment of anterior cruciate ligament injuries of the knee in the public and private healthcare systems of Brazil Original Article

    Astur, Diego Costa; Batista, Rodrigo Ferreira; Arliani, Gustavo Gonçalves; Cohen, Moises

    Abstract in Portuguese:

    CONTEXTO E OBJETIVO Cirurgias ortopédicas implicam alto custo para a saúde pública e privada. O objetivo deste estudo foi de entender melhor as diferenças entre os sistemas público e privado em relação ao tratamento da lesão do ligamento cruzado anterior, que é uma lesão comum do joelho. TIPO DE ESTUDO E LOCAL Estudo transversal descritivo, realizado durante Congresso Brasileiro de Ortopedia em Brasília. MÉTODOS Aplicamos questionários durante o Congresso Brasileiro de Ortopedia de 2010, com a participação de 241 cirurgiões de joelho de 24 estados brasileiros. Posteriormente foi feita análise estatística nos dados obtidos. RESULTADOS Diferentes condutas e opções de tratamento em diferentes estados brasileiros são realizadas pelos ortopedistas avaliados, quando comparadas em relação à atuação no sistema público e privado. CONCLUSÃO No Brasil, tanto no sistema público como no privado nem sempre a melhor decisão é tomada por conta de efeitos outros que não a decisão médica, fato que tem sido maléfico para o paciente e para o médico.

    Abstract in English:

    CONTEXT AND OBJECTIVE Orthopedic surgery implies high costs for both public and private healthcare. The aim of this study was to better understand the differences between the public and private sectors regarding treatment of a damaged anterior cruciate ligament, which is a common knee injury. DESIGN AND SETTING Descriptive cross-sectional study conducted during the Brazilian Orthopedics Congress in Brasília. METHODS We applied questionnaires during the 2010 Brazilian Orthopedics Congress, with participation by 241 knee surgeons from 24 Brazilian states. This was followed by statistical analysis on the data that were obtained. RESULTS The orthopedic surgeons who were evaluated used different approaches and treatment options in different Brazilian states, comparing between the public and private systems. CONCLUSION Both in the public and in the private systems in Brazil, because of non-medical issues surrounding the treatment, the best medical decision is not always made. This may be harmful both to patients and to physicians.
  • Diagnosis and treatment of mast cell disorders: practical recommendations Review Article

    Sandes, Alex Freire; Medeiros, Raphael Salles Scortegagna; Rizzatti, Edgar Gil

    Abstract in Portuguese:

    CONTEXTO E OBJETIVO O termo mastocitose abrange um grupo de raras doenças caracterizado por proliferação neoplásica e acúmulo de mastócitos clonais em um ou mais órgãos. O objetivo do presente estudo foi avaliar os principais elementos para o diagnóstico e tratamento dessas desordens. TIPO DE ESTUDO E LOCAL Revisão narrativa da literatura realizada no Grupo Fleury, São Paulo, Brasil. MÉTODOS O presente estudo revisou artigos científicos publicados nas bases de dados PubMed, Embase (Excerpta Medica Database), Lilacs (Literatura Latino-Americana e do Caribe em Ciências da Saúde) e Cochrane Library, que foram identificados com o termo de busca “mastocitose”. RESULTADOS A apresentação clínica da mastocitose é marcadamente heterogênea, variando de lesões cutâneas que podem regredir espontaneamente, até formas agressivas associadas a falência de órgãos e curta sobrevida. Atualmente, sete subtipos de mastocitose são reconhecidos pela classificação de tumores hematopoéticos da Organização Mundial de Saúde; o diagnóstico é realizado com base nas manifestações clínicas e na identificação de mastócitos neoplásicos por métodos morfológicos, imunofenotípicos, genéticos e moleculares. Mastócitos anômalos apresentam morfologia atípica, frequentemente fusiforme, e expressão aberrante dos antígenos CD25 e CD2. Aumento de triptase sérica é um achado comum em alguns subtipos; e mais que 90% dos pacientes apresentam mastócitos com a mutação KIT D816V. CONCLUSÃO No presente artigo, descrevemos os sintomas e sinais mais comuns em pacientes com mastocitose e sugerimos uma prática abordagem para o diagnóstico, classificação e tratamento clínico inicial.

    Abstract in English:

    CONTEXT AND OBJECTIVE The term mastocytosis covers a group of rare disorders characterized by neoplastic proliferation and accumulation of clonal mast cells in one or more organs. The aim of this study was to assess the principal elements for diagnosing and treating these disorders. DESIGN AND SETTING Narrative review of the literature conducted at Grupo Fleury, São Paulo, Brazil. METHODS This study reviewed the scientific papers published in the PubMed, Embase (Excerpta Medica Database), Lilacs (Literatura Latino-Americana e do Caribe em Ciências da Saúde) and Cochrane Library databases that were identified using the search term “mastocytosis.” RESULTS The clinical presentation of mastocytosis is remarkably heterogeneous and ranges from skin lesions that may regress spontaneously to aggressive forms associated with organ failure and short survival. Currently, seven subtypes of mastocytosis are recognized through the World Health Organization classification system for hematopoietic tumors. These disorders are diagnosed based on clinical manifestations and on identification of neoplastic mast cells using morphological, immunophenotypic, genetic and molecular methods. Abnormal mast cells display atypical and frequently spindle-shaped morphology, and aberrant expression of the CD25 and CD2 antigens. Elevation of serum tryptase is a common finding in some subtypes, and more than 90% of the patients present the D816V KIT mutation in mast cells. CONCLUSION Here, we described the most common signs and symptoms among patients with mastocytosis and suggested a practical approach for the diagnosis, classification and initial clinical treatment of mastocytosis.
  • Bilateral tibial hemimelia type 1 (1a and 1b) with T9 and T10 hemivertebrae: a novel association Case Report

    Salinas-Torres, Victor Michael; Barajas-Barajas, Leticia Oralia; Perez-Garcia, Nicolas; Perez-Garcia, Guillermo

    Abstract in Portuguese:

    CONTEXTO Ausência congênita da tíbia é uma anomalia rara, com incidência em 1 por 1.000.000 de nascidos vivos, é principalmente esporádica e pode ser identificada como um distúrbio isolado ou como parte de síndromes de malformações. RELATO DO CASO Criança do sexo masculino, nascida de pais não afetados e não consanguíneos, apresentou-se com encurtamento das pernas e adução de ambos os pés. O exame físico realizado com seis meses de idade mostrou perímetro cefálico 44,5 cm (percentil 75), comprimento de 60 cm (percentil < 3), peso 7.700 g (percentil 50), encurtamento da coxa esquerda e as duas pernas com o pé varo bilateralhavia. Não houve dismorfismos craniofaciais, nem tórax, abdômen, genitais e anomalias das extremidades superiores. O desenvolvimento psicomotor foi normal. Os exames, incluindo ultrassonografia renal e da cabeça, potenciais auditivos evocados de tronco cerebral e exames oftalmológicos e cardiológicos, estavam normais. Raios-X revelou ausência bilateral da tíbia com fíbula intacta, hipoplasia distal do fêmur esquerdo e fêmur direito normal. Além disso, as radiografias de coluna mostraram hemivértebras em T9 e T10. CONCLUSÃO Esta associação nova expande o espectro de hemimelia tibial. Além disso, esta observação destaca a utilidade de tal método diagnóstico barato (raios-X), caracterizando a grande variabilidade clínica e radiológica de hemimelia tibial.

    Abstract in English:

    CONTEXT Congenital absence of the tibia is a rare anomaly with an incidence of one per 1,000,000 live births. It is mostly sporadic and can be identified as an isolated disorder or as part of malformation syndromes. CASE REPORT A male child, born to unaffected and non-consanguineous parents, presented with shortening of the legs and adduction of both feet. Physical examination at six months of age showed head circumference of 44.5 cm (75th percentile), length 60 cm (< 3rd percentile), weight 7,700 g (50th percentile), shortening of the left thigh and both legs with varus foot. There were no craniofacial dysmorphisms or chest, abdominal, genital or upper-extremity anomalies. Psychomotor development was normal. His workup, including renal and cranial ultrasonography, brainstem auditory evoked potential, and ophthalmological and cardiological examinations, was normal. X-rays showed bilateral absence of the tibia with intact fibulae, distally hypoplastic left femur, and normal right femur. In addition, spinal radiographs showed hemivertebrae at T9 and T10. CONCLUSION This novel association expands the spectrum of tibial hemimelia. Moreover, this observation highlights the usefulness of this inexpensive diagnostic method (X-rays) for characterizing the great clinical and radiological variability of tibial hemimelia.
  • Ginseng: potential for the antileishmanial arsenal? Letter To The Editor

    Pazyar, Nader; Yaghoobi, Reza
  • Medicine and creativity in medical psychology Letter To The Editor

    Natrielli Filho, Décio Gilberto; Silva, Mailu Enokibara; Natrielli, Décio Gilberto
  • Should every adult patient in the hospital have an internist? Letter To The Editor

    Tanriover, Mine Durusu; Guven, Goksel; Buldukoglu, Cagin; Diker, Omer; Halacli, Burcin; Yildirim, Gonul; Topeli, Arzu
  • Untitled document Cochrane Highlights

    Offringa, Martin; Newton, Richard

    Abstract in English:

    BACKGROUND Febrile seizures occurring in a child older than one month during an episode of fever affect 2% to 4% of children in Great Britain and the United States and recur in 30%. Rapid-acting antiepileptics and antipyretics given during subsequent fever episodes have been used to avoid the adverse effects of continuous antiepileptic drugs. OBJECTIVE To evaluate the effectiveness and safety of antiepileptic and antipyretic drugs used prophylactically to treat children with febrile seizures. METHODS Search methods: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2011. Issue 3); MEDLINE (1966 to May 2011); EMBASE (1966 to May 2011); Database of Abstracts of Reviews of Effectiveness (DARE) (May 2011). No language restrictions were imposed. We also contacted researchers in the field to identify continuing or unpublished studies. Selection criteria: Trials using randomized or quasi-randomized patient allocation that compared the use of antiepileptic or antipyretic agents with each other, placebo or no treatment. Data collection and analysis: Two review authors (RN and MO) independently applied pre-defined criteria to select trials for inclusion and extracted the pre-defined relevant data, recording methods for randomization, blinding and exclusions. Outcomes assessed were seizure recurrence at 6, 12, 18, 24, 36 months and at age 5 to 6 years in the intervention and non-intervention groups, and adverse medication effects. The presence of publication bias was assessed using funnel plots. MAIN RESULTS Thirty-six articles describing 26 randomized trials with 2740 randomized participants were included. Thirteen interventions of continuous or intermittent prophylaxis and their control treatments were analyzed. Methodological quality was moderate to poor in most studies. We could not do a meta-analysis for 8 of the 13 comparisons due to insufficient numbers of trials. No significant benefit for valproate, pyridoxine, intermittent phenobarbitone or ibuprofen versus placebo or no treatment was found; nor for diclofenac versus placebo followed by ibuprofen, acetominophen or placebo; nor for intermittent rectal diazepam versus intermittent valproate, nor phenobarbitone versus intermittent rectal diazepam. AUTHORS' CONCLUSIONS No clinically important benefits for children with febrile seizures were found for intermittent oral diazepam, phenytoin, phenobarbitone, intermittent rectal diazepam, valproate, pyridoxine, intermittent phenobarbitone or intermittent ibuprofen, nor for diclofenac versus placebo followed by ibuprofen, acetominophen or placebo. Adverse effects were reported in up to 30% of children. Apparent benefit for clobazam treatment in one recent trial needs to be replicated to be judged reliable. Given the benign nature of recurrent febrile seizures, and the high prevalence of adverse effects of these drugs, parents and families should be supported with adequate contact details of medical services and information on recurrence, first aid management and, most importantly, the benign nature of the phenomenon.
  • Untitled document Cochrane Highlights

    Pereira, Nirmal; Liolitsa, Danae; Iype, Satheesh; Croxford, Anna; Yassin, Muhhamed; Peter, Lang; Ukaegbu, Obioha; Issum, Christopher van

    Abstract in English:

    BACKGROUND Haemorrhoids are variceal dilatations of the anal and perianal venous plexus and often develop secondary to the persistently elevated venous pressure within the haemorrhoidal plexus. Phlebotonics are a heterogenous class of drugs consisting of plant extracts (i.e. flavonoids) and synthetic compounds (i.e. calcium dobesilate). Although their precise mechanism of action has not been fully established, they are known to improve venous tone, stabilize capillary permeability and increase lymphatic drainage. They have been used to treat a variety of conditions including chronic venous insufficiency, lymphoedema and haemorrhoids. OBJECTIVE The aim of this review was to investigate the efficacy of phlebotonics in alleviating the signs, symptoms and severity of haemorrhoidal disease and verify their effect post-haemorrhoidectomy. METHODS Search methods: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) in the Cochrane Library 2011 issue 9, MEDLINE (1950 to September 2011) and EMBASE (1974 to September 2011). Selection criteria: Only randomized controlled trials evaluating the use of phlebotonics in treating haemorrhoidal disease were used. No cross-over or cluster-randomized trials were included for analysis and any trial which had a quasi-random method of allocation was excluded. Data collection and analysis: Two authors independently extracted the data and analyzed the eligibility of the data for inclusion. Disagreements were resolved by meaningful discussion. MAIN RESULTS We considered twenty-four studies for inclusion in the final analysis. Twenty of these studies (enrolling a total of 2344 participants) evaluated the use of phlebotonics versus a control intervention. One of these twenty studies evaluated the use of phlebotonics with a medical intervention and another study with rubber band ligation. AUTHORS' CONCLUSIONS The evidence suggests that there is a potential benefit in using phlebotonics in treating haemorrhoidal disease as well as a benefit in alleviating post-haemorrhoidectomy symptoms. Outcomes such as bleeding and overall symptom improvement show a statistically significant beneficial effect and there were few concerns regarding their overall safety from the evidence presented in the clinical trials.Only randomized controlled trials evaluating the use of phlebotonics in treating haemorrhoidal disease were used. No cross-over or cluster-randomized trials were included for analysis and any trial which had a quasi-random method of allocation was excluded.
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