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Advances in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) over the past decade changed the disease landscape, yet global insight on clinical practices remains limited. The CTEPH global cross-sectional scientific survey (CLARITY) aimed to gather information on the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the treatment and management of CTEPH patients. The survey was circulated to hospital-based medical specialists through Scientific Societies and other medical organizations from September 2021 to May 2022. The majority of the 212 respondents involved in the treatment of CTEPH were from centers performing up to 50 pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA) procedures per year. Variation was observed in the reported proportion of patients deemed eligible for PEA/BPA, as well as those that underwent the procedures, including multimodal treatment and subsequent follow-up practices. Prescription of pulmonary arterial hypertension-specific therapy was reported for a variable proportion of patients in the preoperative setting and in most nonoperable patients. Reported use of vitamin K antagonists and direct oral anticoagulants was similar (86% vs. 82%) but driven by different factors. This study presents heterogeneity in treatment approaches for CTEPH, which may be attributed to center-specific experience and region-specific barriers to care, highlighting the need for new clinical and cohort studies, comprehensive clinical guidelines, and continued education.
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Early recognition and diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is crucial for improving prognosis and reducing the disease burden. Established clinical practice guidelines describe interventions for the diagnosis and evaluation of CTEPH, yet limited insight remains into clinical practice variation and barriers to care. The CTEPH global cross-sectional scientific survey (CLARITY) was developed to gather insights into the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the recognition and diagnosis of CTEPH and the referral and evaluation of these patients. The survey was offered to hospital-based medical specialists through Scientific Societies and other medical organizations, from September 2021 to May 2022. Response data from 353 physicians showed that self-reported awareness of CTEPH increased over the past 10 years among 96% of respondents. Clinical practices in acute pulmonary embolism (PE) follow-up and CTEPH diagnosis differed among respondents. While 50% of respondents working in a nonexpert center reported to refer patients to an expert pulmonary hypertension/CTEPH center when CTEPH is suspected, 51% of these physicians did not report referral of patients with a confirmed diagnosis for further evaluation. Up to 50% of respondents involved in the evaluation of referred patients have concluded a different operability status than that indicated by the referring center. This study indicates that early diagnosis and timely treatment of CTEPH is challenged by suboptimal acute PE follow-up and patient referral practices. Nonadherence to guideline recommendations may be impacted by various barriers to care, which were shown to vary by geographical region.
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ABSTRACT This review aimed to provide an overview of pulmonary arteriovenous malformations, including the major clinical and radiological presentations, investigation, and treatment algorithm of the condition. The primary etiology of pulmonary arteriovenous malformations is hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, with mutations in the ENG gene on chromosome 9 (HHT type 1) or in the ACVRL1/ALK1 complex (HHT type 2). Epistaxis should always be evaluated when repeated, when associated with anemia, and in some cases of hypoxemia. In the investigation, contrast echocardiography and chest CT are essential for evaluating this condition. Embolization is the best treatment choice, especially for correction in cases of hypoxemia or to avoid systemic infections. Finally, disease management was addressed in special conditions such as pregnancy. CT follow-up should be performed every 3-5 years, depending on the size of the afferent and efferent vessels, and antibiotic prophylactic care should always be oriented. Ultimately, knowledge of the disease by health professionals is a crucial point for the early diagnosis of these patients in clinical practice, which can potentially modify the natural course of the disease.
RESUMO Esta revisão teve como objetivo fornecer uma visão geral das malformações arteriovenosas pulmonares, incluindo as principais apresentações clínicas e radiológicas, investigação e algoritmo de tratamento da condição. A principal etiologia das malformações arteriovenosas pulmonares é a telangiectasia hemorrágica hereditária (THH), também conhecida como síndrome de Rendu-Osler-Weber, com mutações no gene ENG no cromossomo 9 (THH tipo 1) ou no complexo ACVRL1/ALK1 (THH tipo 2). A epistaxe sempre deve ser avaliada quando repetida, quando associada à anemia e em alguns casos de hipoxemia. Na investigação, a ecocardiografia e TC de tórax com contraste são essenciais para avaliar essa condição. A embolização é a melhor escolha terapêutica, especialmente para correção em casos de hipoxemia ou para evitar infecções sistêmicas. Por fim, o manejo da doença foi abordado em condições especiais, como a gravidez. O acompanhamento por TC deve ser feito a cada 3-5 anos, dependendo do tamanho dos vasos aferentes e eferentes, e a antibioticoprofilaxia deve sempre ser orientada. Em última análise, o conhecimento da doença pelos profissionais de saúde é um ponto crucial para o diagnóstico precoce desses pacientes na prática clínica, o que pode potencialmente modificar o curso natural da doença.
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ABSTRACT Objectives Pulmonary endarterectomy (PEA) is the gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed at reporting outcomes of CTEPH patients undergoing PEA within 10 years, focusing on advances in anesthetic and surgical techniques. Methods We evaluated 102 patients who underwent PEA between January 2007 and May 2016 at the Instituto do Coração do Hospital das Clínicas da Universidade de São Paulo. Changes in techniques included longer cardiopulmonary bypass, heating, and cooling times and mean time of deep hypothermic circulatory arrest and shortened reperfusion time. Patients were stratified according to temporal changes in anesthetic and surgical techniques: group 1 (January 2007-December 2012), group 2 (January 2013-March 2015), and group 3 (April 2015-May 2016). Clinical outcomes were any occurrence of complications during hospitalization. Results Groups 1, 2, and 3 included 38, 35, and 29 patients, respectively. Overall, 62.8% were women (mean age, 49.1 years), and 65.7% were in New York Heart Association functional class III-IV. Postoperative complications were less frequent in group 3 than in groups 1 and 2: surgical complications (10.3% vs. 34.2% vs. 31.4%, p=0.035), bleeding (10.3% vs. 31.5% vs. 25.7%, p=0.047), and stroke (0 vs. 13.2% vs. 0, p=0.01). Between 3 and 6 months post-discharge, 85% were in NYHA class I-II. Conclusion Improvements in anesthetic and surgical procedures were associated with better outcomes in CTEPH patients undergoing PEA during the 10-year period.
RESUMO Objetivo A endarterectomia pulmonar (EAP) é o tratamento padrão ouro para hipertensão pulmonar tromboembólica crônica (HPTEC). O objetivo deste estudo foi relatar a evolução de pacientes com HPTEC submetidos a EAP em 10 anos, com foco nos avanços nas técnicas anestésicas e cirúrgicas. Métodos Foram avaliados 102 pacientes submetidos à EAP entre janeiro de 2007 e maio de 2016 no Instituto do Coração do Hospital das Clínicas da Universidade de São Paulo. Mudanças nas técnicas incluíram circulação extracorpórea, tempos de aquecimento e resfriamento mais longos e tempo médio de hipotermia profunda com parada circulatória e tempo de reperfusão reduzido. Os pacientes foram estratificados de acordo com as mudanças temporais nas técnicas anestésicas e cirúrgicas: grupo 1 (janeiro de 2007 a dezembro de 2012), grupo 2 (janeiro de 2013 a março de 2015) e grupo 3 (abril de 2015 a maio de 2016). Os desfechos clínicos foram qualquer ocorrência de complicações durante a hospitalização. Resultados Os grupos 1, 2 e 3 incluíram 38, 35 e 29 pacientes, respectivamente. No geral, 62,8% eram mulheres (idade média, 49,1 anos) e 65,7% estavam em classe funcional III-IV da New York Heart Association. As complicações pós-operatórias foram menos frequentes no grupo 3 do que nos grupos 1 e 2: complicações cirúrgicas (10,3% vs. 34,2% vs. 31,4%, p=0,035), sangramento (10,3% vs. 31,5% vs. 25,7%, p=0,047) e acidente vascular cerebral (0 vs. 13,2% vs. 0, p=0,01). Entre 3 e 6 meses após a alta, 85% estavam na classe I-II da NYHA. Conclusão Melhorias nos procedimentos anestésicos e cirúrgicos foram associadas a melhores resultados em pacientes com HPTEC submetidos a EAP durante o período de 10 anos.
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Humanos , Femenino , Persona de Mediana Edad , Embolia Pulmonar/cirugía , Hipertensión Pulmonar/cirugía , Alta del Paciente , Arteria Pulmonar , Brasil , Enfermedad Crónica , Resultado del Tratamiento , Cuidados Posteriores , EndarterectomíaRESUMEN
Resumo Muitos avanços ocorreram nas últimas décadas na terapêutica da hipertensão arterial pulmonar (HAP), uma doença grave, progressiva, incurável e potencialmente fatal. Para seu tratamento adequado, são fundamentais o diagnóstico hemodinâmico e a classificação de sua etiologia, em que várias delas (colagenoses, hipertensão portal, cardiopatia congênitas, esquistossomose) requerem medidas específicas, além do tratamento farmacológico característico para HAP. O tratamento com fármacos-alvo para HAP baseia-se em produtos farmacêuticos que interferem em três vias fisiopatológicas moleculares: da prostaciclina, da endotelina e do óxido nítrico. Tais fármacos apresentam múltiplas apresentações (oral, endovenosa, subcutânea e inalatória) e mudaram a história da HAP. Essas medicações e suas estratégias de uso, assim como particularidades das diferentes formas de HAP, são o foco desta revisão.
Abstract In the last decades, important advances have been made in the treatment of pulmonary arterial hypertension (PAH), a severe, progressive, incurable, and potentially fatal disease. For an adequate therapy, correct hemodynamic diagnosis and etiology classification are fundamental. Many etiologies - rheumatic disease, portal hypertension, congenital heart diseases, schistosomiasis - require specific measures, in addition to drug therapy for PAH. The specific therapy for PAH is based on medications that act on three pathophysiological pathways - prostacyclin, endothelin, and nitric oxide pathways. These drugs have multiple presentations (oral, intravenous, subcutaneous, and inhaled) and have changed the history of PAH. This review presents an overview of drug therapy strategies and different forms and peculiarities of PAH.
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Humanos , Hipertensión Arterial Pulmonar , Hipertensión Pulmonar/tratamiento farmacológico , HemodinámicaRESUMEN
ABSTRACT Objective: To evaluate third- and sixth-year medical students in Brazil in terms of their knowledge of and attitudes toward the WHO MPOWER policies to reduce tobacco use. Methods: The WHO Global Health Professions Student Survey was applied in five cohorts of medical students evaluated in their third and sixth years of medical school, between 2008 and 2015. Comparisons were drawn between the two years of medical school in terms of the proportions of students who experimented with or used tobacco products in the last 30 days prior to the survey; knowledge of and compliance with smoke-free policies on the university campus; formal training on smoking cessation strategies; and self-recognition as role models for patients/society. Results: Of the 943 students who completed the survey, approximately 6% had smoked cigarettes in the last 30 days prior to the survey. Comparing the third and sixth years of medical school, we observed a significant increase in the proportion of students who were knowledgeable about smoking cessation strategies (22.74% vs. 95.84%; p < 0.001) and in that of those who recognized their role as models for patients/society (84.5% vs. 89.7%; p = 0.023). Student knowledge of the smoking policies on the university campus was associated with an increase in self-recognition as role models (adjusted absolute difference = 6.7%; adjusted p = 0.050). Conclusions: Knowledge of smoking cessation strategies and self-recognition as role models for patients/society increase over the course of medical school and are associated with the implementation of smoke-free policies.
RESUMO Objetivo: Avaliar estudantes de medicina do terceiro e sexto anos no Brasil em relação a seu conhecimento e atitudes sobre as políticas MPOWER da OMS para redução do uso do tabaco. Métodos: A Pesquisa Global de Estudantes de Profissões da Saúde da OMS foi aplicada em cinco coortes de estudantes de medicina avaliados no terceiro e sexto anos da graduação, entre 2008 e 2015. Foram feitas comparações entre os dois anos da graduação em relação às proporções de estudantes que experimentaram ou fizeram uso de produtos de tabaco nos últimos 30 dias antes da pesquisa; conhecimento e cumprimento das políticas antifumo no campus universitário; treinamento formal sobre estratégias de cessação do tabagismo; e autorreconhecimento como modelo de comportamento para pacientes/sociedade. Resultados: Dos 943 estudantes que preencheram a pesquisa, aproximadamente 6% haviam fumado cigarro nos últimos 30 dias antes da pesquisa. Ao compararmos o terceiro e sexto anos da graduação, observamos um aumento significativo na proporção de estudantes que tinham conhecimento sobre estratégias de cessação do tabagismo (22,74% vs. 95,84%; p < 0,001) e na daqueles que reconheciam seu papel como modelo para pacientes/sociedade (84,5% vs. 89,7%; p = 0,023). O conhecimento dos estudantes sobre as políticas de tabagismo existentes no campus universitário associou-se ao aumento no autorreconhecimento como modelo de comportamento (diferença absoluta ajustada = 6,7%; p ajustado = 0,050). Conclusões: O conhecimento sobre estratégias de cessação do tabagismo e a autorreconhecimento como modelo de comportamento para pacientes/sociedade aumentaram ao longo da graduação e estão associados à implementação de políticas antifumo.
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Humanos , Estudiantes de Medicina , Organización Mundial de la Salud , Brasil , Actitud , Uso de TabacoRESUMEN
OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by recanalized thromboembolic material. CTEPH has a wide range of radiologic presentations. Commonly, it presents as main pulmonary artery enlargement, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns. Nevertheless, other uncommon presentations have been described, such as lung cavities. These lesions may be solely related to chronic lung parenchyma ischemia but may also be a consequence of concomitant chronic infectious conditions. The objective of this study was to evaluate the different etiologies that cause lung cavities in CTEPH patients. METHODS: A retrospective data analysis of the medical records of CTEPH patients in a single reference PH center that contained or mentioned lung cavities was conducted between 2013 and 2016. RESULTS: Seven CTEPH patients with lung cavities were identified. The cavities had different sizes, locations, and wall thicknesses. In two patients, the cavities were attributed to pulmonary infarction; in 5 patients, an infectious etiology was identified. CONCLUSION: Despite the possibility of being solely associated with chronic lung parenchyma ischemia, most cases of lung cavities in CTEPH patients were associated with chronic granulomatous diseases, reinforcing the need for active investigation of infectious agents in this setting.
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Humanos , Masculino , Femenino , Embolia Pulmonar/diagnóstico , Tromboembolia/etiología , Enfermedad Granulomatosa Crónica/patología , Hipertensión Pulmonar/diagnóstico , Embolia Pulmonar/complicaciones , Embolia Pulmonar/terapia , Angiografía/métodos , Tomografía Computarizada por Rayos X/métodos , Enfermedad Crónica , Estudios Retrospectivos , Resultado del Tratamiento , Imagen de Perfusión , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/terapia , Pulmón/irrigación sanguínea , Anticoagulantes/uso terapéuticoRESUMEN
ABSTRACT Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.
RESUMO A hipertensão pulmonar tromboembólica crônica (HPTEC) é uma doença grave e debilitante, causada pela oclusão do leito arterial pulmonar por êmbolos hemáticos e por material fibroso induzido pela presença desses êmbolos. Essa oclusão eleva a resistência vascular e, por consequência, a pressão do território arterial pulmonar, caracterizando a presença de hipertensão pulmonar. Esse aumento da carga imposta ao ventrículo direito leva a progressiva insuficiência do mesmo e, finalmente, ao óbito. No entanto, ao contrário das outras formas de hipertensão pulmonar, a HPTEC possui uma particularidade muito significativa: a existência de tratamento potencialmente curativo através da tromboendarterectomia pulmonar. Dessa forma, o objetivo primordial do manejo deve ser a avaliação do potencial cirúrgico do paciente em um centro de referência em HPTEC. Entretanto, nem todos os pacientes podem ser submetidos à cirurgia. Para esses pacientes outras alternativas terapêuticas viáveis são a angioplastia de artérias pulmonares e o tratamento farmacológico. Nestas recomendações, discutir-se-ão as bases fisiopatológicas para o surgimento de HPTEC, a partir da embolia pulmonar aguda, bem como o quadro clínico apresentado pelo paciente, o algoritmo diagnóstico a ser seguido e as alternativas terapêuticas disponíveis.
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ABSTRACT Objective: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. Methods: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). Results: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. Conclusions: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.
RESUMO Objetivo: Apresentar uma série de casos de malformações arteriovenosas pulmonares (MAVP) e descrever os principais achados clínicos, a quantidade e localização das MAVP, as complicações clínicas e os tratamentos realizados. Métodos: Estudo retrospectivo observacional que avaliou pacientes com MAVP divididos em dois grupos: telangiectasia hemorrágica hereditária (THH) e MAVP idiopática (MAVPi). Resultados: Foram avaliados 41 pacientes, sendo 33 pacientes portadores de MAVP. Após a avaliação clínica, 27 e 6 foram diagnosticados com THH e MAVPi, respectivamente. No grupo THH a média de idade foi de 49,6 anos e 88,9% eram do sexo feminino. Desses pacientes, 4 tinham SpO2 < 90% e o achado clínico mais frequente era epistaxe. No grupo MAVPi a média de idade foi de 48,1 anos, sendo que 83,3% eram do sexo feminino. Desses, 3 tinham SpO2 < 90%. Após a realização de angiotomografia de tórax observou-se que a maior parte das MAVP se situava nos lobos inferiores, totalizando 56,4% e 85,7% nos grupos THH e MAVPi, respectivamente. O tratamento por embolização foi realizado em 23 pacientes nos dois grupos, enquanto 10 aguardavam o procedimento até o momento da escrita deste estudo. Um paciente submetido à embolização foi encaminhado para ressecção pulmonar. Conclusões: Em ambos os grupos de pacientes com MAVP observou-se uma predominância de mulheres e de fístulas localizadas nos lobos inferiores. A maioria era assintomático respiratório com SpO2 > 90%. O tratamento de escolha para todos foi a embolização percutânea por cateter.
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Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Adulto Joven , Malformaciones Arteriovenosas/etiología , Malformaciones Arteriovenosas/terapia , Malformaciones Arteriovenosas/diagnóstico por imagen , Arteria Pulmonar/anomalías , Arteria Pulmonar/diagnóstico por imagen , Telangiectasia Hemorrágica Hereditaria/complicaciones , Cateterismo/métodos , Angiografía/métodos , Ecocardiografía , Radiografía Torácica/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Embolización Terapéutica/métodos , Dispositivo Oclusor Septal , Angiografía por Tomografía ComputarizadaRESUMEN
OBJECTIVES: Chronic thromboembolic pulmonary hypertension is one of the most prevalent forms of pulmonary hypertension and is a major complication of acute pulmonary embolism. One mainstay of chronic thromboembolic pulmonary hypertension treatment is lifelong anticoagulation. The recent advent of direct oral anticoagulants for acute pulmonary embolism treatment has provided a viable and effective alternative for treating this condition. However, little is known about the efficacy of this new class of drugs for treating chronic thromboembolic pulmonary hypertension. We aimed to evaluate the safety and efficacy of direct oral anticoagulants in the treatment of chronic thromboembolic pulmonary hypertension. METHODS: A cohort of chronic thromboembolic pulmonary hypertension patients who initiated treatment with direct oral anticoagulants between June 2015 and November 2016 were enrolled in this study. RESULTS: Sixteen patients used rivaroxaban, three used dabigatran and one used apixaban for a mean follow-up of 20.9 months. The mean age was 51 years, and eighteen patients were classified as functional class II/III. Eight patients underwent a pulmonary endarterectomy and exhibited clinical, hemodynamic and functional improvement and currently continue to use direct oral anticoagulants. No episode of venous thromboembolism recurrence was identified during the follow-up period, but there was one episode of major bleeding after a traumatic fall. CONCLUSIONS: Although direct oral anticoagulants appear to be a safe and effective alternative for treating chronic thromboembolic pulmonary hypertension, larger studies are needed to support their routine use.
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Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Embolia Pulmonar/tratamiento farmacológico , Pirazoles/administración & dosificación , Piridonas/administración & dosificación , Antitrombinas/administración & dosificación , Dabigatrán/administración & dosificación , Hipertensión Pulmonar/tratamiento farmacológico , Vitamina K/antagonistas & inhibidores , Enfermedad Crónica , Administración Oral , Reproducibilidad de los Resultados , Resultado del TratamientoRESUMEN
ABSTRACT Objective: To determine the level of agreement with effective tobacco control measures recommended by the World Health Organization and to assess the attitudes toward, knowledge of, and beliefs regarding smoking among third-year medical students at University of São Paulo School of Medicine, located in the city of São Paulo, Brazil. Methods: Between 2008 and 2012, all third-year medical students were invited to complete a self-administered questionnaire based on the Global Health Professionals Student Survey and its additional modules. Results: The study sample comprised 556 students. The level of agreement with the World Health Organization recommendations was high, except for the components "received smoking cessation training" and "raising taxes is effective to reduce the prevalence of smoking". Most of the students reported that they agree with banning tobacco product sales to minors (95%), believe that physicians are role models to their patients (84%), and believe that they should advise their patients to quit cigarette smoking (96%) and using other tobacco products (94%). Regarding smoking cessation methods, most of the students were found to know more about nicotine replacement therapy than about non-nicotine therapies (93% vs. 53%). Only 37% of the respondents were aware of the importance of educational antismoking materials, and only 31% reported that they believe in the effectiveness of encouraging their patients, during medical visits. In our sample, the prevalence of current cigarette smoking was 5.23%; however, 43.82% of the respondents reported having experimented with water-pipe tobacco smoking. Conclusions: Our results revealed the need to emphasize to third-year medical students the importance of raising the prices of and taxes on tobacco products. We also need to make students aware of the dangers of experimenting with tobacco products other than cigarettes, particularly water-pipe tobacco smoking.
RESUMO Objetivo: Determinar o grau de concordância com medidas eficazes de controle do tabaco recomendadas pela Organização Mundial da Saúde e avaliar as atitudes, o conhecimento e as crenças a respeito do tabagismo em alunos do terceiro ano de medicina da Faculdade de Medicina da Universidade de São Paulo, em São Paulo (SP). Métodos: Entre 2008 e 2012, todos os alunos do terceiro ano de medicina foram convidados a preencher um questionário autoaplicável baseado na Global Health Professions Student Survey e em seus módulos adicionais. Resultados: A amostra consistiu em 556 estudantes. O grau de concordância com as recomendações da Organização Mundial da Saúde foi alto, à exceção de "receberam treinamento a respeito de cessação do tabagismo" e "aumentar os impostos é uma medida eficaz para reduzir a prevalência do tabagismo". A maioria dos estudantes relatou que concorda com a proibição da venda de produtos do tabaco a menores de idade (95%), acredita que os médicos são modelos de comportamento para seus pacientes (84%) e acredita que deveriam aconselhar seus pacientes a parar de fumar cigarros (96%) e de usar outros produtos do tabaco (94%). No tocante aos métodos de cessação do tabagismo, observamos que a maioria dos estudantes sabe mais sobre terapia de reposição da nicotina do que sobre terapias não nicotínicas (93% vs. 53%). Apenas 37% dos participantes estavam cientes da importância de material educacional antitabagismo, e apenas 31% relataram que acreditam na eficácia de incentivar seus pacientes, durante as consultas médicas, a parar de fumar. Em nossa amostra, a prevalência de tabagismo atual foi de 5,23%; entretanto, 43,82% dos participantes relataram ter experimentado fumar tabaco com um narguilé. Conclusões: Nossos resultados revelaram a necessidade de deixar claro para os alunos do terceiro ano de medicina o quão importante é aumentar os preços e impostos dos produtos do tabaco. É também preciso conscientizar os alunos dos perigos de experimentar outros produtos do tabaco que não os cigarros, particularmente o narguilé.
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Humanos , Masculino , Femenino , Adulto , Adulto Joven , Conocimientos, Actitudes y Práctica en Salud , Cese del Hábito de Fumar/métodos , Prevención del Hábito de Fumar , Fumar/epidemiología , Estudiantes de Medicina/estadística & datos numéricos , Tabaquismo/prevención & control , Brasil/epidemiología , Prevalencia , Autoinforme , Distribución por Sexo , Factores Sexuales , Factores de TiempoRESUMEN
ABSTRACT Objective: To describe diagnostic and treatment aspects of hard metal lung disease (HMLD) and to review the current literature on the topic. Methods: This was a retrospective study based on the medical records of patients treated at the Occupational Respiratory Diseases Clinic of the Instituto do Coração, in the city of São Paulo, Brazil, between 2010 and 2013. Results: Of 320 patients treated during the study period, 5 (1.56%) were diagnosed with HMLD. All of those 5 patients were male (mean age, 42.0 ± 13.6 years; mean duration of exposure to hard metals, 11.4 ± 8.0 years). Occupational histories were taken, after which the patients underwent clinical evaluation, chest HRCT, pulmonary function tests, bronchoscopy, BAL, and lung biopsy. Restrictive lung disease was found in all subjects. The most common chest HRCT finding was ground glass opacities (in 80%). In 4 patients, BALF revealed multinucleated giant cells. In 3 patients, lung biopsy revealed giant cell interstitial pneumonia. One patient was diagnosed with desquamative interstitial pneumonia associated with cellular bronchiolitis, and another was diagnosed with a hypersensitivity pneumonitis pattern. All patients were withdrawn from exposure and treated with corticosteroid. Clinical improvement occurred in 2 patients, whereas the disease progressed in 3. Conclusions: Although HMLD is a rare entity, it should always be included in the differential diagnosis of respiratory dysfunction in workers with a high occupational risk of exposure to hard metal particles. A relevant history (clinical and occupational) accompanied by chest HRCT and BAL findings suggestive of the disease might be sufficient for the diagnosis.
RESUMO Objetivo: Descrever aspectos relacionados ao diagnóstico e tratamento de pacientes com doença pulmonar por metal duro (DPMD) e realizar uma revisão da literatura. Métodos: Estudo retrospectivo dos prontuários médicos de pacientes atendidos no Serviço de Doenças Respiratórias Ocupacionais do Instituto do Coração, localizado na cidade de São Paulo, entre 2010 e 2013. Resultados: Entre 320 pacientes atendidos no período do estudo, 5 (1,56%) foram diagnosticados com DPMD. Todos os pacientes eram do sexo masculino, com média de idade de 42,0 ± 13,6 anos e média de tempo de exposição a metal duro de 11,4 ± 8,0 anos. Os pacientes foram submetidos a avaliação clinica, história ocupacional, TCAR de tórax, prova de função pulmonar, broncoscopia com LBA e biópsia pulmonar. Todos apresentaram distúrbio ventilatório restritivo. O achado de imagem à TCAR de tórax mais frequente foi de opacidades em vidro fosco (em 80%). Em 4 pacientes, o LBA revelou presença de células gigantes multinucleadas. Em 3, foi diagnosticada pneumonia intersticial por células gigantes na biópsia pulmonar. Houve o diagnóstico de pneumonia intersticial descamativa associada à bronquiolite celular em 1 paciente e de pneumonite de hipersensibilidade em 1. Todos foram afastados da exposição e tratados com corticoide. Houve melhora em 2 pacientes e progressão da doença em 3. Conclusões: Apesar de ser uma entidade rara, a DPMD deve ser sempre considerada em trabalhadores com risco ocupacional elevado de exposição a metais duros. A história clínica e ocupacional associada a achados em TCAR de tórax e LBA sugestivos da doença podem ser suficientes para o diagnóstico.
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Humanos , Masculino , Adulto , Persona de Mediana Edad , Aleaciones/efectos adversos , Aleaciones/toxicidad , Cobalto/efectos adversos , Cobalto/toxicidad , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Profesionales/diagnóstico , Exposición Profesional/efectos adversos , Tungsteno/efectos adversos , Tungsteno/toxicidad , Corticoesteroides/uso terapéutico , Biopsia , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/patología , Pulmón/diagnóstico por imagen , Pulmón/patología , Enfermedades Profesionales/tratamiento farmacológico , Enfermedades Profesionales/patología , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
Após a tromboembolia pulmonar, cerca de 1% a 3% dos pacientes desenvolvem embolismo crônico, seguido de elevação da pressão da artéria pulmonar e aumento da resistência vascular pulmonar. O aumento contínuo da pressão vascular leva ao remodelamento das pequenas artérias. Em pacientes selecionados, a tromboendarterectomia pulmonar é o procedimento de escolha para o tratamento da hipertensão pulmonar tromboembólica, com redução significativa da pressão pulmonar e melhora hemodinâmica. A morbimortalidade dessa cirurgia vem diminuindo progressivamente, especialmente nos centros com maior número de procedimentos. A melhora da qualidade de vida é evidente e grande parte dos pacientes retorna às suas atividades habituais...
After pulmonary embolism, 1% to 3% of these patients develop a chronic embolism, with elevated pulmonary artery pressure and increased pulmonary vascular resistance. The continuous elevated pressure leads to the remodeling of the small arteries. In selected patients, pulmonary thromboendarterectomy is the procedure of choice for the treatment of thromboembolic pulmonary hypertension, with significant reduction in pulmonary pressure and hemodynamic improvement.The morbidity and mortality of this surgery has been decreasing steadily, especially in centers with larger numbers of procedures. The improvement in quality of life is evident and most patients return to their usual activities...
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Humanos , Masculino , Femenino , Endarterectomía , Hipertensión Pulmonar , Embolia PulmonarRESUMEN
OBJECTIVE: Water-pipe tobacco smoking is becoming increasingly more common among young people. The objective of this study was to estimate the prevalence of the use of water pipes and other forms of tobacco use, including cigarette smoking, among medical students, as well as to examine the attitudes, beliefs, and knowledge of those students regarding this issue. METHODS: We administered a questionnaire to students enrolled in the University of São Paulo School of Medicine, in São Paulo, Brazil. The respondents were evaluated in their third and sixth years of medical school, between 2008 and 2013. Comparisons were drawn between the two years. RESULTS: We evaluated 586 completed questionnaires. Overall, the prevalence of current cigarette smokers was low, with a decline among males (9.78% vs. 5.26%) and an increase among females (1.43% vs. 2.65%) in the 3rd and 6th year, respectively. All respondents believed that health professionals should advise patients to quit smoking. However, few of the medical students who smoked received physician advice to quit. Experimentation with other forms of tobacco use was more common among males (p<0.0001). Despite their knowledge of its harmful effects, students experimented with water-pipe tobacco smoking in high proportions (47.32% and 46.75% of the third- and sixth-year students, respectively). CONCLUSIONS: The prevalence of experimentation with water-pipe tobacco smoking and other forms of tobacco use is high among aspiring physicians. Our findings highlight the need for better preventive education programs at medical schools, not only to protect the health of aspiring physicians but also to help them meet the challenge posed by this new epidemic. .
OBJETIVO: O fumo de narguilé com tabaco está aumentando entre os jovens. O objetivo deste trabalho foi estimar a prevalência do uso de narguilé e outras formas de consumo de tabaco, incluindo o fumo de cigarros, entre estudantes de medicina, assim como as atitudes, crenças e conhecimento desses alunos sobre esse assunto. MÉTODOS: Um questionário foi aplicado aos estudantes da Faculdade de Medicina da Universidade de São Paulo. Os entrevistados eram alunos de terceiro e sexto anos entre 2008 e 2013. As respostas foram comparadas entre os dois anos de graduação. RESULTADOS: 586 estudantes responderam ao questionário. A prevalência de fumantes foi baixa, com um declínio entre os homens (9,78% contra 5,26%) e um aumento no sexo feminino (1,43% contra 2,65%) no 3º e 6º ano, respectivamente. Todos os entrevistados acreditavam que profissionais de saúde devem aconselhar os pacientes a parar de fumar. No entanto, a maioria dos estudantes de medicina fumantes não recebeu aconselhamento médico para deixar de fumar. A experimentação de outros produtos derivados do tabaco foi maior entre os homens (p < 0.0001). Apesar do conhecimento de seus efeitos nocivos à saúde, a experimentação de narguilé foi alta (47,32% e 46,75% entre alunos do terceiro e sexto anos, respectivamente. CONCLUSÕES: A prevalência da experimentação de narguilé com tabaco e de outras formas de uso de tabaco é alta entre os futuros médicos. Nossos achados enfatizam a necessidade de melhores programas de educação preventiva em universidades médicas para proteger a saúde dos futuros médicos e para ajudá-los a enfrentar esse novo desafio epidêmico. .
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Adulto , Femenino , Humanos , Masculino , Adulto Joven , Actitud Frente a la Salud , Fumar/epidemiología , Fumar/prevención & control , Estudiantes de Medicina/estadística & datos numéricos , Trastornos Relacionados con Sustancias/epidemiología , Trastornos Relacionados con Sustancias/prevención & control , Brasil/epidemiología , Conocimientos, Actitudes y Práctica en Salud , Prevalencia , Factores Sexuales , Encuestas y Cuestionarios , Productos de Tabaco/clasificación , UniversidadesRESUMEN
Os sarcomas de artéria pulmonar são tumores raros e de difícil diagnóstico, simulando frequentemente o tromboembolismo pulmonar crônico. Relatamos dois casos de pacientes do sexo feminino com quadro clínico de dispneia e massas pulmonares associadas a falhas de enchimento na artéria pulmonar em angiotomografia de tórax. A tomografia por emissão de pósitrons com 18F fluordesoxiglicose mostrou hipercaptação das respectivas lesões. O sarcoma de artéria pulmonar foi confirmado posteriormente por exame anatomopatológico. Ressaltamos a importância do uso desse tipo de tomografia como exame não invasivo no auxílio diagnóstico desses tumores.
Pulmonary artery sarcomas are rare, difficult-to-diagnose tumors that frequently mimic chronic pulmonary thromboembolism. We report the cases of two female patients with clinical signs of dyspnea and lung masses associated with pulmonary artery filling defects on chest CT angiography. We performed 18F-fluorodeoxyglucose positron emission tomography, which revealed increased radiotracer uptake in those lesions. Pulmonary artery sarcoma was subsequently confirmed by anatomopathological examination. We emphasize the importance of this type of tomography as a noninvasive method for the diagnosis of these tumors.
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Anciano , Femenino , Humanos , Persona de Mediana Edad , Tomografía de Emisión de Positrones/métodos , Arteria Pulmonar , Radiofármacos , Sarcoma , Neoplasias Vasculares , Enfermedad Crónica , Diagnóstico Diferencial , Embolia Pulmonar/diagnóstico , Neoplasias Vasculares/patologíaRESUMEN
OBJECTIVE: To evaluate the effectiveness of nicotine replacement therapy (NRT), bupropion, nortriptyline and combination therapy and describe factors associated with treatment success. INTRODUCTION: Clinical trials clearly demonstrate the efficacy of pharmacotherapy in smoking cessation. However, it is only after its use in real-life settings that clinical effectiveness and limitations of a treatment are fully known. METHODS: Patients attended a four-session cognitive-behavioral program and received medicines free of charge. Abstinence from smoking was assessed at each visit. RESULTS: A total of 868 smokers (68.8 percent women) were included. Their mean age was 49.6 years; the amount smoked was 25 cigarettes/day and the Fagerströ m Score was 6.6. Abstinence rates after 6 months and 1 year were 36.5 percent and 33.6 percent. In univariate analysis, male gender, age (>50), higher number of cigarettes smoked, cardiovascular comorbidities, longer interval from the last cigarette and combined treatment of nortriptyline plus NRT were predictive of abstinence, while neuropsychiatric comorbidities and the answer ''yes'' to the question ''Do you smoke more often during the first hours after waking'' were correlated with failure. In a multivariate model, predictors of abstinence were neuropsychiatric comorbidities, the answer ''yes'' to the question ''Do you smoke more often during the first hours after waking'' and combined treatment of nortriptyline plus NRT. Male gender and a longer period from the last cigarette were correlated with lower abstinence rate. CONCLUSION: Satisfactory success rates were obtained in a teaching hospital. Factors such as age, daily cigarette consumption, number of pack-years and dependency score were not reliable markers of abstinence. The combination nortriptyline+NRT was independently associated with higher abstinence rates.
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Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Cese del Hábito de Fumar/métodos , Fumar/tratamiento farmacológico , Factores de Edad , Bupropión/uso terapéutico , Terapia Combinada , Estudios de Seguimiento , Análisis Multivariante , Nicotina/uso terapéutico , Nortriptilina/uso terapéutico , Estudios Retrospectivos , Factores Sexuales , Factores de Tiempo , Resultado del TratamientoRESUMEN
INTRODUCTION: Chronic thromboembolic pulmonary hypertension is a disease affecting approximately 4,000 people per year in the United States. The incidence rate in Brazil, however, is unknown. The estimated survival for patients with chronic thromboembolic pulmonary hypertension without treatment is approximately three years. Pulmonary thromboendarterectomy for select patients is a potentially curative procedure when correctly applied. In Brazil, the clinical and hemodynamic profiles of chronic thromboembolic pulmonary hypertension patients have yet to be described. OBJECTIVES: To evaluate the clinical and hemodynamic characteristics of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy in a referral center for chronic thromboembolic pulmonary hypertension treatment in Brazil. METHODS: From December 2006 to November 2009, patients were evaluated and scheduled for pulmonary thromboendarterectomy. The subjects were classified according to gender, age and functional class and were tested for thrombofilia and brain natriuretic peptide levels. RESULTS: Thirty-five consecutive chronic thromboembolic pulmonary hypertension patients were evaluated. Two patients tested positive for schistosomiasis, and 31 were enrolled in the study (19 female, 12 male). The majority of patients were categorized in functional classes III and IV. Hemodynamic data showed a mean pulmonary vascular resistance (PVR) of 970.8 ± 494.36 dynas·s·cm-5 and a low cardiac output of 3.378 ± 1.13 L/min. Linear regression revealed a direct relation between cardiac output and pulmonary vascular resistance. Paradoxical septal movement was strongly correlated with pulmonary vascular resistance and cardiac output (p=0.001). Brain natriuretic peptide serum levels were elevated in 19 of 27 patients. CONCLUSIONS: In a referral center for pulmonary hypertension in Brazil, chronic thromboembolic pulmonary hypertension patients evaluated for pulmonary thromboendarterectomy had a hemodynamically severe status and had elevated brain natriuretic peptide serum levels. There was a predominance of females in our cohort, and the prevalence of hematological disorders and schistosomiasis was low (less than 10 percent).
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Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Gasto Cardíaco Bajo/fisiopatología , Hemodinámica/fisiología , Hipertensión Pulmonar/fisiopatología , Embolia Pulmonar/fisiopatología , Esquistosomiasis/complicaciones , Resistencia Vascular/fisiología , Brasil , Enfermedad Crónica , Endarterectomía , Hipertensión Pulmonar/cirugía , Modelos Lineales , Péptido Natriurético Encefálico/sangre , Embolia Pulmonar/cirugía , Distribución por Sexo , Factores SexualesRESUMEN
A tromboembolia pulmonar constitui, juntamente com a trombose venosa profunda, a condição denominada tromboembolismo venoso. Apesar dos avanços, a morbidade e a mortalidade atribuídas a essa doença ainda são elevadas, pois os pacientes apresentam doenças mais complexas, são submetidos a um maior número de procedimentos invasivos e sobrevivem por mais tempo. Embora existam inúmeras diretrizes internacionais disponíveis, optou-se por redigir estas recomendações para sua aplicação na prática médica nacional, embasadas nas melhores evidências na literatura e na opinião do grupo de consultores. Este documento é apenas uma ferramenta para o atendimento dos pacientes, e, embora possa ser aplicado na maioria das situações, o médico deve adaptar as informações a sua realidade local e ao caso específico. O diagnóstico de tromboembolia pulmonar é realizado através da combinação da probabilidade clínica pré-teste (escores) com o resultado dos exames de imagem, sendo atualmente o método de eleição a angiotomografia computadorizada. É fundamental a estratificação do risco de desfecho desfavorável, sendo a instabilidade hemodinâmica o preditor mais importante. Pacientes de baixo risco devem ser tratados com heparina, comumente as de baixo peso molecular. Pacientes de alto risco requerem vigilância intensiva e uso de trombolíticos em alguns casos. A longo prazo, os pacientes devem receber anticoagulantes por no mínimo três meses, sendo sua manutenção decidida pela presença de fatores de risco para a recorrência e a probabilidade de sangramento. A profilaxia é altamente eficaz e deve ser amplamente utilizada, tanto em pacientes clínicos como cirúrgicos, conforme os grupos de risco. Finalmente, são feitas recomendações relacionadas ao diagnóstico, tratamento e prevenção da tromboembolia pulmonar.
Pulmonary thromboembolism and deep vein thrombosis together constitute a condition designated venous thromboembolism. Despite the advances, the morbidity and the mortality attributed to this condition are still high, because the patients present with more complex diseases, are submitted to a greater number of invasive procedures and survive longer. Although there are various international guidelines available, we decided to write these recommendations for their application in medical practice in Brazil. These recommendations are based on the best evidence in the literature and the opinion of the advisory committee. This document is only a tool for use in the management of patients. Although the recommendations it contains can be applied to most situations, physicians should adapt its content depending on their local context and on a case-by-case basis. Pulmonary thromboembolism is diagnosed by evaluating pre-test clinical probability (scores) together with the results of imaging studies, the current method of choice being CT angiography. Stratification of the risk for an unfavorable outcome is fundamental. Hemodynamic instability is the most important predictor. Low-risk patients should be treated with heparin, commonly low-molecular-weight heparins. High-risk patients require intensive monitoring and, in some cases, thrombolytic therapy. In the long term, patients should receive anticoagulants for at least three months. The decision to prolong this treatment is made based on the presence of risk factors for the recurrence of the condition and the probability of bleeding. Prophylaxis is highly effective and should be widely used in clinical and surgical patients alike, according to their risk group. Finally, we include recommendations regarding the prevention, diagnosis and treatment of pulmonary thromboembolism.
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Humanos , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/terapia , Anticoagulantes/uso terapéutico , Diagnóstico Diferencial , Medicina Basada en la Evidencia/normas , Neoplasias/complicaciones , Pronóstico , Embolia Pulmonar/complicaciones , Factores de RiesgoRESUMEN
A silicose, a mais prevalente das pneumoconioses, é provocada pela inalação de partículas de sílica cristalina. Indivíduos expostos à sílica, com ou sem silicose, apresentam risco aumentado de tuberculose e de micobacterioses não-tuberculosas. O risco de silicóticos desenvolverem tuberculose em relação a controles sadios varia de 2,8 a 39 vezes, em conformidade com a gravidade da doença de base. Têm sido estudados diferentes esquemas de quimioprofilaxia para tuberculose em silicóticos, todos com eficácia semelhante e com redução final de risco para cerca da metade em relação ao uso de placebo. São, no entanto, esquemas de longa duração, o que, acrescido dos possíveis efeitos colaterais (particularmente hepatotoxicidade), podem prejudicar a aderência. As diretrizes atuais recomendam a realização de prova tuberculínica e, se positiva, a instituição de quimioprofilaxia. São vários os esquemas possíveis, tanto em termos de drogas quanto de duração. Nossa recomendação é de que se use isoniazida na dose de 300 mg/dia (ou 10 mg/kg/dia) por seis meses para os indivíduos com silicose ou sadios com exposição superior a 10 anos, se forem reatores fortes à prova tuberculínica (induração > 10 mm). São necessários, no entanto, novos estudos para que indicações, drogas, doses e duração da profilaxia sejam definidas mais apropriadamente.
Silicosis, the most prevalent of the pneumoconioses, is caused by inhalation of crystalline silica particles. Silica-exposed workers, with or without silicosis, are at increased risk for tuberculosis and nontuberculous mycobacteria-related diseases. The risk of a patient with silicosis developing tuberculosis is higher (2.8 to 39 times higher, depending on the severity of the silicosis) than that found for healthy controls. Various regimens for tuberculosis chemoprophylaxis in patients with silicosis have been studied, all of which present similar efficacy and overall risk reduction to about one half of that obtained with placebo. Long-term regimens have potential side effects (particularly hepatotoxicity). In addition, the use of such regimens can jeopardize adherence to treatment. The current guidelines recommend that tuberculin skin tests be performed, and, if positive, that chemoprophylaxis be instituted. There are several possible regimens, varying in terms of the drugs prescribed, as well as in terms of treatment duration. We recommend the use of isoniazid at 300 mg/day (or 10 mg/kg/day) for six months for patients with silicosis, as well as for healthy patients with periods of exposure to silica longer than 10 years and strongly positive tuberculin skin test results (induration > 10 mm). Nevertheless, further studies are necessary so that indications, drugs, doses and duration of chemoprophylaxis regimens can be more properly defined.