Isabel Gomes

Pulmonary rehabilitation in patients with bronchiectasis: pulmonary function, arterial blood gases, and the 6-minute walk test.

BACKGROUND: Information regarding the effects of pulmonary rehabilitation (PR) on pulmonary function (PF), arterial blood gases (ABG), and 6-minute walk distance (6MWD) in patients with bronchiectasis is scant in the literature. METHODS: To evaluate the effects of PR on these indices in this population, a retrospective evaluation of those who attended PR from 2007 to 2010, was made. Pulmonary rehabilitation lasted a mean of 12 weeks and included cycle ergometer exercise for 30 minutes, 3 times per week, with additional upper limbs and quadriceps training. PF, ABG, and 6MWD were evaluated before and after PR to determine the potential influence of gender, exacerbations, underlying cause of bronchiectasis, severity of obstruction, and colonization with bacteria. RESULTS: Forty-one patients (48.8% males; median age, 54 years) were included; 25 had severe obstruction and 19 were colonized with bacteria. Following PR, no significant changes were detected in PF or ABG. Median 6MWD before PR was 425 m and post-PR was 450 m (P = .431). Outcomes did not show any interaction with gender, colonization, or exacerbations. However, patients with idiopathic bronchiectasis did show a significant improvement in forced vital capacity in percent of predicted and residual volume after PR (P = .016 and .048, respectively). Patients with severe obstruction showed a statistically significant decrease in percent of predicted residual volume (P = .025). CONCLUSION: There appears to be a beneficial impact of PR on PF in certain groups of patients with bronchiectasis. In addition, PR indications and protocols for patients with bronchiectasis may need to be adapted to accommodate specific patients, so that expressive exercise capacity improvement can be achieved.

Nontuberculous mycobacteria in non-AIDS patients

UNLABELLED Nontuberculous mycobacteria (NTM) play an increasingly significant pathogenic role in HIV -positive patients, in patients with chronic lung disease, in other chronic conditions and in the elderly. AIMS Evaluate the importance of NTM isolation in respiratory samples in patients without HIV-infection. METHODS Retrospective evaluation of our hospital patients with no known AIDS, with at least one NTM positive respiratory sample, from 1997 -2004. RESULTS We found 102 patients, with a median age of 63 years; 67% male. Sixty -three (62%) had underlying lung disease, mainly tuberculosis sequelae (n=19). The majority (47%) of the isolations were Mycobacterium avium complex (MAC). A diagnosis of Mycobacterium pulmonary disease was made in 16 patients (15.7%), 14 of which met the American Thoracic Society diagnostic criteria. Ten male and 6 female; median age 65 years. Twelve had underlying lung disease. All of them had respiratory infection complaints. Chest X -rays showed mainly pulmonary infiltrates, linear opacities and cavitation. MAC was the cause of mycobacterium respiratory disease in 12 patients (75%). CONCLUSION NTM isolation did not equal pulmonary NTM disease in the majority of cases, even in patients with underlying lung disease. MAC was the most commonly isolated agent and its relative importance was higher in the presence of NTM disease.

Lung cancer: atypical brain metastases mimicking neurocysticercosis.

The authors describe a case of a 47-year-old male smoker with a 3-month history of hearing loss, tinnitus and dizziness. Physical examination revealed neurosensory hearing loss. Small rounded hypodensities without mass effect were evident in a computed tomography scan of the head, confirmed by brain magnetic resonance imaging as multiple cystic lesions in both cerebral and cerebellar hemispheres, without perilesional edema or gadolinium enhancement, suggestive of neurocysticercosis. Extraparenchymal involvement was also noted. Albendazole and dexamethasone were started. As a chest radiograph showed a bilateral reticulonodular pattern, a bronchoscopy was performed showing normal results. However, transbronchial biopsy revealed lung adenocarcinoma. Thoracoabdominopelvic computed tomography scan showed secondary lung and bone lesions. Since brain lesions were not suggestive of secondary tumor lesions, a brain biopsy was performed confirming metastatic disease. This case illustrates some peculiar imagiological features of brain metastases in lung cancer, indicating that sometimes invasive procedures are required to establish a definitive diagnosis.

Fibrose quística: Revisão

Resumo A fibrose quistica (FQ) e a doenca autossomica recessiva mais frequente na raca caucasiana. Caracteriza-se por mutacoes na CFTR, uma proteina transmembranar responsavel pelo transporte de cloretos. Esta proteina tem uma ampla distribuicao epitelial, o que da um caracter sistemico a esta doenca e consequentemente multiplas manifestacoes clinicas de gravidade variavel. A melhoria dos cuidados de saude, associada ao desenvolvimento do arsenal terapeutico, permitiu um aumento da sobrevida destes doentes, de tal forma que a FQ ja nao pode ser abordada como doenca da idade pediatrica. Tambem a evolucao tecnica na transplantacao abriu novas perspectivas quanto ao tratamento desta afeccao. Assim, cada vez mais esta patologia implica um envolvimento multidisciplinar no qual a pneumologia tem uma parte preponderante.

Bronquiolite constritiva ocupacional (?) em doente com exame físico, radiológico e funcional normal

Constrictive bronchiolitis is characterized by alterations in the walls of membranous and respiratory bronchioles. These changes lead to concentric narrowing or complete obliteration of the airway lumen. Suspicion of possible bronchiolar disorders may arise from clinical, funcional, and radiologic findings. However, constrictive bronchiolitis may be present even with normal physical, functional and image findings, which turns the diagnosis difficult. A high index of suspicion is necessary to justify invasive tests that lead to pulmonary biopsy. In this report, we describe a patient with cough and dyspnoea, with normal physical, functional and image findings, whose work-up leaded to the diagnosis of constrictive bronchiolitis. Rev Port Pneumol 2009; XV (4): 729-732

Cystic fibrosis in adults

The authors reviewed adult cystic fibrosis patients followed in the Pulmonology Unit from 1994-2004 (n=8), five female and three male, aged 20-34 years old (median= 27 years). Patients were diagnosed at 18 months - 31 years old by sweat testing (positive in six patients) and genotyping (four patients homozygous for Delta F508 mutation). Respiratory involvement was characterised by sinusitis and bronchiectasis. Pulmonary involvement was accompanied by functional abnormalities and gas exchange impairment in the majority of the patients. Bronchial tree was colonised permanently in five patients: Pseudomonas aeruginosa in four and Staphilococcus aureus in four (three patients affected by both agents simultaneously). The main causes of exacerbation were respiratory infections and haemoptysis. Non-respiratory involvement was variable. Four patients had digestive involvement (one with hepatic cirrhosis), one had renal failure and only one had a sperm count to document infertility. Four patients had osteopaenia. Treatment included chest physiotherapy, bronchodilators, dornase alfa, mucolytics, digestive enzymes, vitamins, antibiotics and oxygen therapy. At review, one had left follow-up, one had died, one was awaiting lung transplant and the others evidenced no difference in clinical characteristics. In this group of patients the severity of the pulmonary disease was not related to a late diagnosis. It can be explained by the diversity of cystic fibrosis presentation in adults.

Fibrose quística em adultos

The authors reviewed adult cystic fibrosis patients followed in the Pulmonology Unit from 1994-2004 (n = 8), five female and three male, aged 20-34 years old (median = 27 years). Patients were diagnosed at 18 months-31 years old by sweat testing (positive in six patients) and genotyping (four patients homozygous for ΔF508 mutation). Respiratory involvement was characterised by sinusitis and bronchiectasis. Pulmonary involvement was accompanied by functional abnormalities and gas exchange impairment in the majority of the patients. Bronchial tree was colonised permanently in five patients: Pseudomonas aeruginosa in four and Staphilococcus aureus in four (three patients affected by both agents simultaneously).The main causes of exacerbation were respiratory infections and haemoptysis. Non-respiratory involvement was variable. Four patients had digestive involvement (one with hepatic cirrhosis), one had renal failure and only one had a sperm count to document infertility. Four patients had osteo-paenia. Treatment included chest physiotherapy, bronchodilators, dornase alfa, mucolytics, digestive enzymes, vitamins, antibiotics and oxygen therapy. At review, one had left follow-up, one had died, one was awaiting lung transplant and the others evidenced no difference in clinical characteristics. In this group of patients the severity of the pulmonary disease was not related to a late diagnosis. It can be explained by the diversity of cystic fibrosis presentation in adults Rev Port Pneumol 2007; XIII (3): 335-347

Pneumotórax espontâneo num pulmão vicariante

Resumo O pneumotorax contralateral apos pneumonectomia e uma situacao rara. E necessario um alto indice de suspeicao para um correcto diagnostico, na ausencia de exames complementares de diagnostico. O trata-mento e um desafio, uma vez que a intervencao cirur-gica, se necessaria, e um procedimento de risco. A pleu-rodese quimica pode ser uma medida simples e eficaz, em casos sem fistula broncopleural importante. Descrevemos o caso clinico de uma doente de 21 anos com pneumotorax espontâneo em pulmao unico, que colocou varias dificuldades, diagnosticas e terapeuticas. A presenca de pulmao vicariante dificultou a interpreta-cao inicial do RX toracico. A doente apresentava fistula broncopleural de alto debito, sem expansao pulmonar completa, mesmo apos colocacao de um segundo dreno toracico, e a cirurgia toracica foi considerada de risco. Foi efectuada instilacao de talco atraves de um dos drenos toracicos, com posterior resolucao. Estamos convencidos de que a resolucao da fistula broncopleural foi facilitada pela talcagem, mesmo sem expansao pulmonar total, que nunca se tinha conseguido. Rev Port Pneumol 2007; XIII (4): 613-617

Hemotórax e hemopneumotórax espontâneos: causas raras de internamento

RESUMO Os autores fazem a revisao e descricao sumaria dos 5 casos de hemotorax e hemopneumotorax espontâneos internados no servico de Pneumologia do H.S.Joao entre 1983 e 1995, comentando as situacoes em que foi possivel identificar patologia subjacente com eles relacionada. Foram diagnosticados 1 caso de neuroepitelioma periferico,1 caso de aderencias pleurais e 1 caso de bolhas subpleurais. Os restantes 2 casos foram considerados idiopaticos. Todos foram submetidos a drenagem toracica e apenas 1 doente necessitou de transfusao. O “follow-up” variou entre 6 meses e 3 anos, nao se tendo observado recidiva de hemotorax em nenbum caso. Nao se verificaram sequelas e todos os doentes se mantem assintomatieos apos a resolucao do hemopneumotorax.

Bronquiectasias: etiopatogenia, clínica, métodos de diagnóstico eterapêutica

The authors review several aspects related to bronchiectasis, namely their pathogenesis, classification, clinical presentation, diagnosis and therapy.