Adelina Amorim

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.

Bronchiectasis: A retrospective study of clinical and aetiological investigation in a general respiratory department

BACKGROUND Bronchiectasis can result from many diseases, which makes the aetiological investigation a complex process demanding special resources and experience. The aetiological diagnosis has been proven to be useful for the therapeutic approach. OBJECTIVE Evaluate how accurately and extensive the clinical and aetiological research was for adult bronchiectasis patients in pulmonology outpatient service which were not following a pre-existing protocol. METHODS We retrospectively reviewed the records of 202 adult patients with bronchiectasis, including the examinations performed to explain the aetiology. RESULTS The mean age of the patients was 54 ± 15 years, there was a predominance of female (63.9%) and non-smoker (70%) patients. Functional evaluation showed a mild airway obstruction. The sputum microbiological examination was available for 168 patients (43.1% had 3 or more sputum examinations during one year). Immunoglobulins and α1-antitrypsin were measured in around 50% of the patients. The sweat test and the CF genotyping test were performed in 18% and 17% of the patients, respectively. The most commonly identified cause was post-infectious (30.3%), mostly tuberculosis (27.2%). No definitive aetiological diagnosis was established in 57.4% of the patients. We achieved a lower aetiological diagnosis if we compare our series with studies in which a diagnostic algorithm was applied prospectively. CONCLUSIONS The general characteristics of our patients were similar with other series. Detailed investigation of bronchiectasis is not a standard practice in our outpatient service. These results suggest that the use of a predefined protocol, based on current guidelines, could improve the assessment of these patients and facilitate the achievement of a definitive aetiology.

New advances in the therapy of non-cystic fibrosis bronchiectasis

Non-cystic fibrosis bronchiectasis remains a common and important respiratory disease to date. It is a chronic pathology and consequently the patients usually require continuous treatment. In recent decades therapies that do not have scientific evidence of their benefits have been commonly used in non-cystic fibrosis bronchiectasis. Cystic fibrosis has provided the experience to extrapolate therapeutic approaches to other bronchiectasis patients. Finally, in the last few years some trials have been carried out specifically in non-cystic fibrosis bronchiectasis which aim to assess the efficacy of some of the treatments which are commonly used but sometimes without clear indication. This review will discuss the recent results from these trials, namely mucoactive, anti-inflammatory and antibiotic therapy. Several trials are ongoing and we hope they will be able to add clarification to the management of these patients.

Tuberculose endobrônquica – alterações clínicas e broncoscópicas

Endobronchial tuberculosis (ET) is a serious complication of pulmonary tuberculosis and is a major cause of morbidity. The aim of our retrospective study was to characterize the clinical, radiological, microbiological and bronchoscopic features of ET. Between January 1999 and June 2002 a total of 14 patients were diagnosed as having ET in our hospital. There were 8 (57%) men and 6 women with a median age of 39.6 +/- 18.1 years (range from 20 to 78 years). Cough was the most common complain and it was present in 71.4% of patients. Only 5 patients were sputum smear positive. Five patients (35.7%) had parenchymal infiltration and this was the most common roentgenographic appearance. Forms of ET were classified into subtypes: actively caseating (n=4), granular (n=3), tumorous (n=3), edematous-hyperemic (n=2) and ulcerative (n=2). The upper lobes were affected in 9 (64.3%) patients. Nine patients had involvement of the left bronchial tree, 3 of the right and in 2 there were bilateral lesions. The diagnosis could be established in 11 (78.6%) cases by bronchial biopsy. All patients had positive bronchial lavage cultures for acid-fast bacilli. Clinical manifestations and roentgenographic appearance of ET are not specific and so bronchoscopy is mandatory for the prompt diagnosis and follow-up of its evolution.

Cystic fibrosis - characterization of the adult population in Portugal.

INTRODUCTION The incidence of cystic fibrosis (CF) in Portugal is estimated at 1:8000 live births, although there is a lack of accurate statistics. The average life expectancy has been steadily increasing and CF is no longer an exclusively pediatric disease. OBJECTIVES Characterize the Portuguese adult population with the diagnosis of CF. METHODS Retrospective study based on clinical data of adult CF follow-up patients in the three specialized centers in Portugal where all of CF patients are seen, during 2012. RESULTS In 2012, there were 89 follow-up patients, 48 (54%) female and 15 (17%) lung transplanted. The average age was 31.3±9 years. The median age at diagnosis was 13 years and 34 (38%) were diagnosed in adulthood. The most frequent mutation was F508del (54.9%). Of the 89 patients, 49 patients (56%) had pancreatic insufficiency, 7 (9%) were diabetic and 42 patients (47.7%) had a body mass index (BMI) <20kg/m(2). As to ventilatory function, the average value of the forced expiratory volume in 1s (FEV1) was 58.45±28.59%. Only one of 77 patients did not have chronic airway infection. The most commonly isolated germ was methicillin-sensitive Staphylococcus aureus in 49 patients (55%). During 2012, two patients (2.2%) died at the ages of 21 and 36 years. DISCUSSION This study is the first description of the Portuguese adult CF population, which is particularly important since it can give us a better understanding of the real situation. A significant percentage of these patients were diagnosed in adulthood, which highlights the need for diagnostic suspicion in a patient with chronic lung disease and atypical manifestations.

Azitromicina como terapêutica adjuvante na pneumonia organizativa criptogénica

There are literature data about the immunomodulatory properties of some macrolides in cryptogenic organizing pneumonia (COP) as an alternative to corticosteroids in mild disease or as adjuvant to standard therapy. A sixty-year-old female, with a controlled intrinsic asthma, presented with COP and recurrent respiratory exacerbations despite corticosteroid and immunossupressant therapy. Azithromycin (500mg, on alternate days) as an adjuvant to steroids was then started, with clinical and functional improvement and regression of lung infiltrates. Withdrawal of steroids was possible in one year, without evidence of relapse in the next six months. Azithromycin was maintained (three times per week) with no documentation of adverse side effects. This clinical case reinforces the potential role of macrolides anti-inflammatory properties in COP as corticosteroids adjuvant therapy.

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.

Cystic fibrosis – Comparison between patients in paediatric and adult age

Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians. Although most cases are diagnosed in childhood, diagnosis in adults is apparently increasing. OBJECTIVE Evaluate the adult population with CF, comparing patients who were diagnosed before and after 18 years of age. METHODS Retrospective analysis of patients followed in three main medical centres in Portugal in 2012. Comparison of two groups: G1 - patients diagnosed at <18 years and G2 - patients diagnosed at ≥18 years. RESULTS 89 adults were identified: 61.8% in G1, 38.2% in G2. Gender distribution was similar in both groups. Average age in G2 was higher (38.3±8.4 vs. 26.8±6.1 years, p<0.001). Respiratory symptoms most frequently led to CF diagnosis in all patients, mainly in adulthood. There was a greater percentage of patients homozygous for the mutation delF508 in G1 (43.6 vs. 8.8%, p=0.02). Respiratory and pancreatic function, and body mass index (BMI) showed a higher severity in G1 (G1 vs. G2: FEV1: 54.6±27.3 vs. 29.9±64.6%, p=0.177; pancreatic insufficiency 72.7 vs. 26.5%, p<0.001; BMI 20.2±3.4 vs. 22.2±4.8, p=0.018). Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus were the most frequently isolated microorganisms. Lung transplantation rate was higher in G2 (20.6 vs. 10.9%, p=0.231) while mortality rate was higher in G1 (0 vs. 3.6%, p=0.261). Hospital admission rate was higher in G1 as well as mortality rate. CONCLUSION The results suggest that patients with CF diagnosed in childhood have characteristics that distinguish them from those diagnosed in adulthood, and these differences may have implications for diagnosis, prognosis and life expectancy.

Streptococcus pneumoniae – caused CAP in hospitalised patients: mortality predictors

Probably the most important decision in the management of Community-Acquired Pneumonia (CAP) is patient site of care. Patients with Streptococcus pneumoniae-caused CAP admitted to our hospital between 1st January and 31st December 2006 were retrospectively analysed. Samples of blood, sputum, bronchial and bronchoalveolar lavage and urine were collected for microbiological testing using standard culture techniques and urine antigen detection. Pneumonia Severity Index (PSI) and British Thoracic Society (BTS) CURB-65 scoring tools were evaluated. The statistical treatment was performed using the SPSS 14.0 program. We included 104 patients, 67.3% male, median age 63 years old, mortality 13.4%. There was a significant association between the PSI and CURB-65 score and mortality. Despite advances, CAP is still an important health problem with a high attendant morbi-mortality. This study confirms the value of PSI and CURB-65 in the prediction of severe pneumonia.

Internamento devido a PAC por Streptococcus pneumoniae – Avaliação de factores de mortalidade

Resumo A avaliacao da gravidade perante qualquer caso de pneumonia adquirida na comunidade (PAC) e de suma importância, pois dela decorrem decisoes como a necessidade de internamento e o tratamento empirico inicial. Os autores apresentam um estudo retrospectivo, que incluiu doentes internados devido a pneumonia por Streptococcus pneumoniae durante o ano de 2006, no Hospital de Sao Joao. A confirmacao etiologica de infeccao foi feita por isolamentos no sangue, liquido pleural, secrecoes traqueobronquicas, lavado bronquico, lavado broncoalveolar e pesquisa de antigenuria. Foram analisados os factores de risco e avaliados, com base nas normas PSI (Pneumonia Severity Index) e da British Thoracic Society (BTS) -CURB-65. A analise estatistica foi efectuada utilizando teste T para amostras independentes e ANOVA, usando o programa de analise estatistica SPSS 14.0. Foram incluidos 104 doentes com idade mediana de 63 anos, sendo 67,3% do sexo masculino. O estudo revelou existir uma associacao com significado estatistico entre os resultados de PSI e CURB-65 e a evolucao para a mortalidade. Apesar da melhoria dos meios diagnosticos e profilacticos, e da terapeutica antibiotica, a pneumonia pneumococica permanece uma entidade de grande morbilidade e mortalidade. O valor preditivo das normas PSI e CURB-65 foi confirmado nesta populacao de doentes, documentando uma correlacao entre o numero de factores de risco e a evolucao da doenca. Rev Port Pneumol 2008; XIV (5): 601-615