Mindfulness-based treatment for smoking cessation: a randomized controlled trial.

OBJECTIVE: To evaluate the efficacy of a mindfulness-based treatment (MBT) for smoking cessation or reduction and compare it with that of cognitive behavioral therapy (CBT). METHODS: This was a single-center randomized controlled clinical trial including 113 patients divided into two groups: MBT (n = 54) and CBT (n = 59). The interventions comprised eight 90-min sessions. The primary outcome was smoking cessation at 16 weeks after program initiation. Secondary outcomes included reduction in the mean number of cigarettes smoked/day at 16 weeks after treatment initiation, as well as smoking cessation and reduction in the number of cigarettes smoked/day at the last program session. Participants had to attend ≥ 50% of the sessions to be included in the primary outcome analysis. An intention-to-treat analysis was also performed. RESULTS: There was no difference between the groups regarding the primary outcome (30.4% in the MBT group vs. 31.6% in the CBT group, p = 0.68) or immediate abstinence rates (47.8% in the MBT group vs. 36.8% in the CBT group, p = 0.47). Both treatments were equally effective in reducing the number of cigarettes smoked/day at the last program session (a reduction of 93.33% [0-100%] in the MBT group and of 70% [33.3-100%] in the CBT group, p = 0.92) and at 16 weeks after program initiation (a reduction of 57.1% [0-100%] in the MBT group and of 70% [25-100%] in the CBT group, p = 0.49). CONCLUSIONS: MBT appears to be as effective as CBT for smoking cessation or reduction and can be an option for the treatment of tobacco use disorders in Brazil (Brazilian Registry of Clinical Trials identifier: RBR-3w2scz [http://www.ensaiosclinicos.gov.br]).

Mindfulness-based treatment for smoking cessation: a randomized controlled trial / Tratamento baseado em atenção plena (mindfulness) para cessação do tabagismo: ensaio clínico controlado randomizado

ABSTRACT Objective: To evaluate the efficacy of a mindfulness-based treatment (MBT) for smoking cessation or reduction and compare it with that of cognitive behavioral therapy (CBT). Methods: This was a single-center randomized controlled clinical trial including 113 patients divided into two groups: MBT (n = 54) and CBT (n = 59). The interventions comprised eight 90-min sessions. The primary outcome was smoking cessation at 16 weeks after program initiation. Secondary outcomes included reduction in the mean number of cigarettes smoked/day at 16 weeks after treatment initiation, as well as smoking cessation and reduction in the number of cigarettes smoked/day at the last program session. Participants had to attend ≥ 50% of the sessions to be included in the primary outcome analysis. An intention-to-treat analysis was also performed. Results: There was no difference between the groups regarding the primary outcome (30.4% in the MBT group vs. 31.6% in the CBT group, p = 0.68) or immediate abstinence rates (47.8% in the MBT group vs. 36.8% in the CBT group, p = 0.47). Both treatments were equally effective in reducing the number of cigarettes smoked/day at the last program session (a reduction of 93.33% [0-100%] in the MBT group and of 70% [33.3-100%] in the CBT group, p = 0.92) and at 16 weeks after program initiation (a reduction of 57.1% [0-100%] in the MBT group and of 70% [25-100%] in the CBT group, p = 0.49). Conclusions: MBT appears to be as effective as CBT for smoking cessation or reduction and can be an option for the treatment of tobacco use disorders in Brazil (Brazilian Registry of Clinical Trials identifier: RBR-3w2scz [http://www.ensaiosclinicos.gov.br])

RESUMO Objetivo: Avaliar a eficácia de um mindfulness treatment (MT, tratamento baseado em atenção plena) para a cessação ou redução do tabagismo e compará-la à da terapia cognitivo-comportamental (TCC). Métodos: Ensaio clínico controlado randomizado realizado em um único centro, com 113 pacientes divididos em dois grupos: MT (n = 54) e TCC (n = 59). As intervenções consistiram em oito sessões de 90 min cada. O desfecho primário foi a cessação do tabagismo 16 semanas após o início do programa. Os desfechos secundários foram a redução da média de cigarros fumados/dia em 16 semanas após o início do programa, bem como a cessação do tabagismo e redução do número de cigarros fumados/dia na última sessão do programa. Os participantes deveriam comparecer a ≥ 50% das sessões para que fossem incluídos na análise do desfecho primário. Foi também realizada uma análise por intenção de tratamento. Resultados: Não houve diferença entre os grupos quanto ao desfecho primário (30,4% no grupo MT vs. 31,6% no grupo TCC, p = 0,68) ou às taxas de abstinência imediata (47,8% no grupo MT vs. 36,8% no grupo TCC, p = 0,47). Ambos os tratamentos foram igualmente eficazes na redução do número de cigarros fumados/dia na última sessão do programa [redução de 93,33% (0-100%) no grupo MT e de 70% (33,3-100%) no grupo TCC, p = 0,92] e em 16 semanas após o início do programa [redução de 57,1% (0-100%) no grupo MT e de 70% (25-100%) no grupo TCC, p = 0,49]. Conclusões: A MT parece ser tão eficaz quanto a TCC para a cessação ou redução do tabagismo e pode ser uma opção para o tratamento do tabagismo no Brasil. [Registro Brasileiro de Ensaios Clínicos - ReBEC; número de identificação: RBR-3w2scz (http://www.ensaiosclinicos.gov.br)]

Conhecimento da população sobre pneumologia, doenças respiratórias e tabagismo / Population knowledge about pneumology, respiratory diseases, and smoking

Objetivo: Avaliar o conhecimento da população sobre pneumologia, doença pulmonar obstrutiva crônica , asma e tabagismo. Métodos: Estudo transversal conduzido entre julho e outubro de 2017, avaliando o conhecimento da população de Curitiba, a partir de 18 anos, a respeito de pneumologia, asma, doença pulmonar obstrutiva crônica e tabagismo, por meio de questionários aplicados em espaços públicos. Médicos foram excluídos. Resultados: Foram entrevistadas 384 pessoas, com idade média de 35±14 anos, sendo 52% mulheres. Tinham Ensino Superior (completo ou incompleto) 55%, 44% não ultrapassaram o Ensino Médio e 5%, o Fundamental. Fumantes atuais ou pregressos representaram 32% da amostra. Sabiam o significado da palavra pneumologista 77%, já o termo "doença pulmonar obstrutiva crônica" foi reconhecido por apenas 7%. A maioria procuraria um clínico geral se apresentasse tosse persistente (69%) ou dispneia (63%). As doenças associadas ao tabagismo mais lembradas foram neoplasia de pulmão (86%) e doenças pulmonares (37%). O tabagismo foi reconhecido como causador de dependência por 98% e como doença por 64%. Em relação aos sintomas da asma, foram citados dispneia (90%), tosse (18%), aperto no peito (16%) e chiado (10%). Da amostra, 53% acreditavam que os dispositivos inalatórios engordavam e 59% que geravam dependência. Maior escolaridade associou-se com menor frequência de tabagismo, maior conhecimento sobre o pneumologista e sobre a associação do tabagismo com doença pulmonar obstrutiva crônica. Conclusão: Os curitibanos têm conhecimento limitado a respeito da doença pulmonar obstrutiva crônica, asma e tabagismo, além de acreditarem em mitos relacionados aos dispositivos inalatórios. A procura pelo médico pneumologista é baixa. (AU)

Objective: To evaluate the population knowledge about pneumology, chronic obstructive pulmonary disease, asthma and smoking. Methods: This is a cross-sectional study conducted from July to October 2017 to evaluate the knowledge of the population of the city of Curitiba, above 18 years old, about pneumology, chronic obstructive pulmonary disease, asthma and smoking, using questionnaires applied in public places. Physicians were excluded. Results: A total of 384 people was interviewed, with an average age of 35±14 years, 52% of them being women. Fifty-five percent reported (complete or incomplete) higher education, 44% did not go further than high school, and 5%, intermediate school. Current or past smokers accounted for 32% of the sample. The meaning of the word pulmonologist was known by 77% of people, while the term "chronic obstructive pulmonary disease" was recognized by only 7%. Most would go to a general practitioner if they had persistent cough (69%) or dyspnea (63%). The most remembered smoking-related diseases were: lung cancer (86%) and lung diseases (37%). Smoking was recognized to cause dependence by 98%, and as a disease by 64%. Dyspnea (90%), cough (18%), chest tightness and wheezing (10%) were mentioned as symptoms of asthma. Fifty-three percent of the sample believed that inhaling devices led to weight gain, and 59% believed they generated dependence. Higher level of education was associated with a lower smoking frequency, greater knowledge about the pulmonologist and about the association of smoking with respiratory diseases. Conclusions: The population of Curitiba has a limited knowledge about chronic obstructive pulmonary disease, asthma and smoking, and frequently believes in myths related to inhaling devices. The search for a pulmonologist is low. (AU)

Association of Pulmonary Cysts and Nodules in a Young Female Patient.

A 27-year-old female patient was referred to our outpatient clinic with a 1-year history of shortness of breath when walking fast on level ground or when climbing stairs. Symptoms worsened after a second episode of spontaneous left pneumothorax, when a chest tube was placed in another hospital for complete lung expansion. During this hospitalization, an open lung biopsy was performed. There was no history of rhinorrhea, nasal congestion, cough, hemoptysis, wheezing, or expectoration.

Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations.

OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day). METHODS: We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus. RESULTS: After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. CONCLUSIONS: Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols.

Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations / Uso de sirolimo no tratamento de linfangioleiomiomatose: resposta favorável em pacientes com diferentes manifestações extrapulmonares

OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day). METHODS: We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus. RESULTS: After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. CONCLUSIONS: Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols. .

OBJETIVO: A linfangioleiomiomatose (LAM) é uma doença rara que é atualmente considerada uma neoplasia de baixo grau com potencial metastático e evolução variável. Os inibidores de mammalian target of rapamycin (mTOR), como o sirolimo e o everolimo, recentemente se tornaram uma opção para o tratamento de pacientes com LAM, especialmente daqueles com manifestações extrapulmonares. O objetivo deste estudo foi descrever quatro casos de pacientes com LAM no Brasil que apresentaram melhora, especialmente das manifestações extrapulmonares, após tratamento com sirolimo (em doses de 1-4 mg/dia). MÉTODOS: Descrevemos quatro casos de pacientes com LAM e diferentes manifestações extrapulmonares tratados com sirolimo. RESULTADOS: Após o tratamento com sirolimo por 12 meses, um paciente apresentou resolução do quilotórax de difícil manejo, um paciente teve redução significativa do volume do angiomiolipoma renal, e uma paciente apresentou regressão importante de linfangioleiomiomas retroperitoneais e linfonodomegalias abdominais. Após tratamento com sirolimo por 6 meses, um paciente apresentou redução significativa de volumoso linfangioleiomioma retroperitoneal. CONCLUSÕES: Nossos achados confirmam que os inibidores de mTOR são benéficos para pacientes com LAM, especialmente para aqueles com manifestações extrapulmonares, tais como angiomiolipomas renais, linfangioleiomiomas e derrames de origem quilosa. Entretanto, alguns pontos, tais como a dose ideal, a duração do tratamento e os efeitos adversos em longo prazo, ainda precisam ser esclarecidos para que os inibidores de mTOR possam ser incorporados na abordagem da LAM. .

Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features.

OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). METHODS: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). RESULTS: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. CONCLUSIONS: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.

Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features / Colagenose pulmão dominante em pacientes com doença pulmonar intersticial: prevalência, estabilidade funcional e manifestações extratorácicas comuns

OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). METHODS: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). RESULTS: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. CONCLUSIONS: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria. .

OBJETIVO: Descrever as características de uma coorte de pacientes com colagenose pulmão dominante (CPD). MÉTODOS: Estudo retrospectivo de pacientes com doença pulmonar intersticial (DPI), anticorpo antinuclear (ANA) positivo (≥ 1/320), com ou sem autoanticorpos específicos, e com a presença de ao menos uma manifestação clínica sugestiva de doença do tecido conjuntivo (DTC). RESULTADOS: Dos 1.998 avaliados, 52 preencheram inicialmente os critérios para o diagnóstico de CPD: 37% eram homens; a média de idade ao diagnóstico era de 56 anos e a mediana do tempo de seguimento era de 48 meses. Durante o seguimento, 8 pacientes preencheram os critérios para um diagnóstico definitivo de DTC. Os 44 pacientes restantes formaram o grupo CPD, no qual as manifestações extratorácicas mais prevalentes foram artralgia, doença do refluxo gastroesofágico e fenômeno de Raynaud. Os autoanticorpos mais prevalentes nesse grupo foram ANA (89%) e anti-SSA (anti-Ro, 27%). A média de CVF no início e na última avaliação foi de 69,5% e 74,0% do predito, respectivamente (p > 0,05). Pneumonia intersticial não específica e pneumonia intersticial usual foram identificadas em 45% e 9% das TCARs, respectivamente; 36% das TCARs eram não classificáveis. Uma prevalência semelhante foi identificada na histologia. Dilatação esofágica difusa foi identificada em 52% das TCARs. Capilaroscopia subungueal foi realizada em 22 pacientes; 17 apresentavam um padrão de esclerodermia. CONCLUSÕES: No grupo CPD, houve predominância feminina, e os pacientes apresentaram alterações espirométricas leves ao diagnóstico, com diferentes padrões de DPI, em sua maioria não classificáveis, tanto em TCAR como na histologia. Estabilidade funcional foi identificada no seguimento. A dilatação esofágica em TCAR e o padrão de esclerodermia na capilaroscopia subungueal foram achados frequentes que poderiam servir como critérios diagnósticos. .

Avaliação do impacto de um programa de reabilitação pulmonar na capacidade de exercício em portadoras de linfangioleiomiomatose

Introdução: A linfangioleiomiomatose (LAM) é uma neoplasia de baixo grau, frequentemente associada à redução na capacidade de exercício, secundária a múltiplos fatores incluindo alteração de troca gasosa, limitação ventilatória e hiperinsuflação dinâmica (HD). A reabilitação pulmonar (RP) tem benefícios bem estabelecidos em diversas doenças pulmonares crônicas, porém não foi estudada na LAM. Objetivos: Avaliar o impacto de um programa de RP, comparativamente a um grupo controle, em portadoras de LAM, nos seguintes parâmetros: capacidade de exercício (objetivo primário), HD, dispneia, nível de atividade física diária, qualidade de vida, ansiedade e depressão, função pulmonar e força muscular. Metodologia: Ensaio clínico, controlado, não-randomizado, incluindo 21 pacientes com LAM no grupo RP e 19 no grupo controle. A RP teve duração de 3 meses, compreendendo 24 sessões de uma hora de duração (30 minutos de exercício aeróbico e 30 minutos de treinamento de força muscular). A avaliação inicial incluiu um teste de exercício cardiopulmonar (TECP) máximo incremental. As seguintes variáveis foram avaliadas antes e após a RP ou observação (grupo controle): capacidade de exercício, através do tempo até o limite da tolerância (Tlim) no teste de exercício cardiopulmonar (TECP) com carga constante; distância percorrida e dessaturação de oxigênio no teste de caminhada de 6 minutos (TC6M); dispneia (escala de dispneia do Medical Research Council modificada - mMRC, Índice de Dispneia Basal - BDI, e Índice Transicional de Dispneia - TDI); nível de atividade física diária (pedômetro); qualidade de vida relacionada à saúde (Questionário Respiratório de St George's, SGRQ); ansiedade e depressão (Escala Hospitalar de Ansiedade e Depressão, HADS); provas de função pulmonar (PFP) e força muscular (uma repetição máxima, 1 RM)...

Introduction: Lymphangioleiomyomatosis (LAM) is a low-grade neoplasm, which is frequently associated with reduced exercise capacity, secondary to multiple factors including gas exchange impairment, ventilatory limitation and dynamic hyperinflation (DH). Pulmonary rehabilitation (PR) has proven benefits in many chronic pulmonary diseases but it was not evaluated in LAM. Objectives: To evaluate the impact of a PR program in women with LAM, when compared to a control group, in the following parameters: exercise capacity (primary outcome), DH, dyspnea, daily physical activity, quality of life, anxiety and depression, lung function and muscle strength. Methods: A non-randomized controlled clinical trial that included 21 LAM patients in the PR group and 19 in the control group. The PR program lasted 3 months, comprising 24 sessions of 1 hour (30 minutes of aerobic exercise and 30 minutes of muscle strength training). The initial evaluation included a maximum incremental cardiopulmonary exercise test (CPET). The following variables were assessed before and after PR or observation (control group): exercise capacity using the tolerable limit duration (Tlim) in constant work rate (CWR) exercise testing; walking distance and oxygen desaturation (six-minute walk test, 6MWT), dyspnea (Modified Medical Research Council Dyspnea Scale -mMRC; Basal Dyspnea Index - BDI, and Transitional Dyspnea Index -TDI); daily physical activity (pedometer); health-related quality of life (St George's Respiratory Questionnaire, SGRQ); anxiety and depression (Hospital Anxiety and Depression Scale, HADS); pulmonary function tests (PFT) and muscle strength (one-repetition maximum, 1RM)...

Evaluation of the extent of pulmonary cysts and their association with functional variables and serum markers in lymphangioleiomyomatosis (LAM).

PURPOSE: Although computed tomography (CT) has been used previously to assess disease severity in lymphangioleiomyomatosis (LAM), the associations between the extent of pulmonary cysts on CT and six-minute walk test (6MWT), matrix metalloproteinases (MMPs) and vascular endothelial growth factor (VEGF-D) are not well established. We performed a cross-sectional study to quantify the extent of pulmonary cysts in CT and to establish their correlations with pulmonary function tests (PFTs), 6MWT results, including a composite index (desaturation-distance ratio, DDR), and levels of VEGF-D and MMPs in LAM. METHODS: Twenty-three LAM patients underwent CT scanning to automatically quantify the extent of pulmonary cysts and performed PFTs and 6MWT. Serum levels of MMP-2, MMP-9, and VEGF-D were also measured. RESULTS: The severity of pulmonary cystic involvement was mild (the extent of cysts was 6.8 %) and correlated best with FEV1/FVC (r = -0.84), residual volume (r = 0.66), DLCO (r = -0.82), the DDR index (r = 0.77), and desaturation during the 6MWT (r = -0.81). There was a weak correlation with VEGF-D (r = 0.45), but no association was found with MMP-2 and MMP-9. CONCLUSIONS: The severity of pulmonary cystic involvement was mild in this sample of LAM patients and correlated best with airway obstruction, air trapping, reduced DLCO, the DDR index, and desaturation during the 6MWT. Serum VEGF-D cannot be completely defined as a valuable marker of disease severity and there may be a mechanism independent of MMPs to explain the formation of pulmonary cysts.

Clinical course and characterisation of lymphangioleiomyomatosis in a Brazilian reference centre.

BACKGROUND AND OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare disease that promotes pulmonary cystic destruction and impairs pulmonary function. We aim to describe features and clinical course of LAM patients from Brazil. METHODS: We described the clinical and functional features, performance in six minute walk test (6MWT), management details, survival and clinical course of 84 LAM patients followed in a Brazilian reference centre. RESULTS: All subjects were women, the average age at onset of symptoms was 38 years, and the average at diagnosis was 42 years. The major symptoms during the course of the disease were dyspnoea and pneumothorax. The patients experienced impaired quality of life, with worse scores in the physical and emotional domains. The most common abnormalities in pulmonary function tests were an obstructive pattern and reduced diffusion capacity, whereas a quarter of the patients had normal spirometric results. In the 6MWT, although patients had preserved exercise capacity, more than half of the patients had significant desaturation. Hormonal blockage and doxycycline were the most common treatment modalities employed in our patients. The survival probability from diagnosis was 90% at 5 years, whereas the mean annual rate of decline in FEV1 was 60 ± 78 mL. CONCLUSIONS: Clinical and functional features of the LAM patients from our centre are similar to those from other countries. Our sample showed preserved exercise capacity, with desaturation in the 6MWT, and impaired quality of life. Survival was similar, whereas the annual rate of decline of FEV1 was slightly lower than in recent studies.

Pneumomediastinum, subcutaneous emphysema, and pneumothorax after a pulmonary function testing in a patient with bleomycin-induced interstitial pneumonitis.

Spontaneous pneumomediastinum is an uncommon event, the clinical picture of which includes retrosternal chest pain, subcutaneous emphysema, dyspnea, and dysphonia. The pathophysiological mechanism involved is the emergence of a pressure gradient between the alveoli and surrounding structures, causing alveolar rupture with subsequent dissection of the peribronchovascular sheath and infiltration of the mediastinum and subcutaneous tissue with air. Known triggers include acute exacerbations of asthma and situations that require the Valsalva maneuver. We described and documented with HRCT scans the occurrence of pneumomediastinum after a patient with bleomycin-induced interstitial lung disease underwent pulmonary function testing. Although uncommon, the association between pulmonary function testing and air leak syndromes has been increasingly reported in the literature, and lung diseases, such as interstitial lung diseases, include structural changes that facilitate the occurrence of this complication.

Pneumomediastinum, subcutaneous emphysema, and pneumothorax after a pulmonary function testing in a patient with bleomycin-induced interstitial pneumonitis / Pneumomediastino, enfisema subcutâneo e pneumotórax após prova de função pulmonar em paciente com pneumopatia intersticial por bleomicina

Spontaneous pneumomediastinum is an uncommon event, the clinical picture of which includes retrosternal chest pain, subcutaneous emphysema, dyspnea, and dysphonia. The pathophysiological mechanism involved is the emergence of a pressure gradient between the alveoli and surrounding structures, causing alveolar rupture with subsequent dissection of the peribronchovascular sheath and infiltration of the mediastinum and subcutaneous tissue with air. Known triggers include acute exacerbations of asthma and situations that require the Valsalva maneuver. We described and documented with HRCT scans the occurrence of pneumomediastinum after a patient with bleomycin-induced interstitial lung disease underwent pulmonary function testing. Although uncommon, the association between pulmonary function testing and air leak syndromes has been increasingly reported in the literature, and lung diseases, such as interstitial lung diseases, include structural changes that facilitate the occurrence of this complication.

O pneumomediastino espontâneo é um evento incomum, cujo quadro clínico inclui dor pleurítica retroesternal, enfisema subcutâneo, dispneia e disfonia. O mecanismo fisiopatológico implicado é o surgimento de uma diferença de pressão entre os alvéolos e estruturas adjacentes, ocasionando ruptura alveolar com posterior dissecção da bainha peribroncovascular e infiltração do mediastino e do tecido subcutâneo pelo ar. Desencadeantes conhecidos incluem exacerbação aguda de asma e situações que exigem a realização de manobra de Valsava. Descrevemos e documentamos por imagens tomográficas a ocorrência de pneumomediastino após a realização de prova de função pulmonar em um paciente com pneumopatia intersticial induzida por bleomicina. Apesar de incomum, a associação entre provas de função pulmonar e síndromes de vazamento de ar tem sido relatada cada vez mais na literatura, e doenças pulmonares, como as pneumopatias intersticiais, contemplam alterações estruturais que facilitam a ocorrência da complicação. .

Lung cysts in chronic paracoccidioidomycosis.

On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases.

Lung cysts in chronic paracoccidioidomycosis / Cistos pulmonares na paracoccidioidomicose crônica

On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases. .

Os cistos pulmonares na TCAR são caracterizados por áreas arredondadas de baixo coeficiente de atenuação no parênquima pulmonar com uma interface bem definida com o pulmão adjacente normal. As doenças pulmonares císticas mais comuns são linfangioleiomiomatose, histiocitose de células de Langerhans e pneumonia intersticial linfocítica. Em uma análise retrospectiva de achados de TCAR em 50 pacientes com diagnóstico de paracoccidioidomicose crônica residual, observou-se a presença de cistos pulmonares em 5 casos (10%), mostrando que pacientes com paracoccidioidomicose podem apresentar cistos pulmonares na TCAR. Portanto, essa infecção deve entrar no diagnóstico diferencial das doenças císticas pulmonares. .

Abscesso esplênico: relato de caso / Splenic abscess: case report

JUSTIFICATIVA E OBJETIVOS: O abscesso esplênico é uma doença rara, porém com um número crescente de casos publicados nos últimos anos. Geralmente, resulta de disseminação hematogênica, especialmente relacionada à endocardite, mas também pode ocorrer por contiguidade e relacionada a outros focos infecciosos.A grande maioria dos casos ocorre em pacientes imunossuprimidos. A doença é responsável por graves complicações e apresenta alto índice de mortalidade na ausência de terapêutica adequada. Oobjetivo deste estudo foi lembrar aos clínicos dessa possibilidade diagnóstica, que exige alto grau de suspeição, devido ao quadro clínico inespecífico. RELATO DO CASO: Paciente do sexo feminino, 44 anos, com quadro clínico de dor abdominal difusa, mais acentuada em flanco esquerdo com evolução de um mês e febre de 40º C com início há 5 dias. Previamente hígida, exceto por história de hipertensão arterial sistêmica, insuficiência cardíaca e prótese metálica em valva mitral. Ao exame encontrava-se em regular estado geral, desorientada,hipotensa, com depleção extracelular de 30%, afebril e eupneica. O abdômen era plano, flácido, doloroso à palpação em flanco esquerdo e hipogástrio e sem sinais de irritação peritoneal. Realizada tomografia computadorizada, que evidenciou imagem hipodensa no interior do baço. Diagnosticado abscesso esplênico,sendo tratada com esplenectomia e antibioticoterapia. CONCLUSÃO: Esse relato demonstrou que o abscesso esplênico pode ocorrer em pacientes imunocompetentes e sem outra fonte de infecção.

BACKGROUND AND OBJECTIVES: Splenic abscess is a rare disease, however the number of cases published in literature has increase in recent years. Generally, it appears after hematogenous dissemination, especially related with endocarditis, but can also occurby contiguity and related with other infections sites. The majorityof the cases happen in immunocompromised hosts. The disease is responsible for serious complications and has high mortality rate without appropriate treatment. The objective of this report was to remind doctors of this diagnostic, which needs a high degree of suspicion due its nonspecific clinical manifestation. CASE REPORT: Female patient, 44-years-old, with clinical symptoms of diffuse abdominal pain, more important in left flank,with one month of evolution, and 40º C fever that started withinfive days. Previously healthy, except for history of hypertension,cardiac failure and metallic prosthesis in the mitral valve. On examinationshe was in regular condition, disoriented, hypotensive, with extracellular depletion of 30%, afebrile and eupneic. The abdomen was flat, flaccid, painful to palpation in the left flank and hypogastric and without signs of peritoneal irritation. A CT scan was performed which showed a hypodense image on spleen. Diagnosed splenic abscess, the patient was treated with splenectomy and antimicrobial therapy. CONCLUSION: This report showed that splenic abscess may occurin immunocompetents patients without other source of infection.

Mudanças no padrão de conduta do transplante de córnea após campanha informativa / Education and pattern behavior on corneal donation

OBJETIVO: Confrontar informações obtidas sobre doação de córneas através de questionários aplicados antes e após a realização de um ciclo de palestras informativas aos profissionais da área de saúde. Verificar o grau de conhecimento destes profissionais de hospital universitário em relação a informações básicas sobre o transplante de córnea. Documentar a reação pessoal destas pessoas frente ao procedimento. MÉTODOS: Foi realizada uma campanha através de aulas com material audio visual específico trazendo informações detalhadas sobre o processo de doação e transplante de córneas. Estas palestras foram direcionadas a 115 profissionais da área de saúde: 46 médicos e 69 acadêmicos de medicina. Para a avaliação foi elaborado um questionário, aplicado antes e depois das aulas com intervalo de 1 mês, composto de 7 perguntas referentes ao procedimento do transplante em si, e 3 perguntas direcionadas à posição pessoal do médico em relação à doação de córneas. RESULTADOS: Inicialmente obteve-se 61,72 por cento de acertos nas perguntas objetivas, aumentando para 77,70 por cento após o acesso às palestras. Nas perguntas subjetivas a porcentagem de profissionais que doariam suas córneas foi 91,5 por cento, subindo para 93 por cento após as aulas. De forma semelhante o número que autorizaria a doação de córnea de um familiar aumentou de 91,5 por cento para 94,4 por cento. Quando questionados se já haviam abordado uma família espontâneamente e solicitado o procedimento de doação de córneas a resposta foi positiva em 4,8 por cento dos profissionais avaliados, e esta porcentagem subiu apenas para 5,5 por cento após a realização das palestras. CONCLUSÃO: O despreparo dos profissionais de saúde em relação aos vários aspectos que concernem o transplante de córneas é nítido. Iniciativas isoladas como palestras informativas, apesar de necessárias, não são suficientes para mudar esta realidade.

OBJECTIVE: To compare obtained data about cornea donation through a questionnaire applied before and after the realization of an expositive lecture given to health professionals. To identify these professional's knowledge about basic cornea transplant information. To verify their personal reaction towards the procedure. METHODS: A campain was performed using specific audio-visual classes about the cornea transplant's and donation process. These lectures were directed to 115 health professionals: 46 doctors and 69 medical students. For the evaluation a test was created and applied before and after the classes, with a one month break. This test included 10 questions, 7 about cornea transplant's specific information, and the other 3 were about their donation's opinion. RESULTS: The questions about the procedure were corrected 61, 72 percent at the first application and 77, 70 percent at the second, answered a month later than the first. At the subjective questions the percentage of professionals that would donate their corneas was 91,5 percent, increasing to 93 percent after the classes. In a similar way, the number that would authorize a family's cornea donation changed from 91,5 percent to 94,4 percent. When they were asked about previous cornea transplant solicitation, the answer was positive at 4,8 percent of the tests and increased only to 5,5 percent after the campain. CONCLUSION: Doctors and medical students are not prepared to deal with the multiple aspects of the cornea transplant procedure. And although needed, isolated actions as informative campains are not enough to change this reality.