J. Agostinho Marques

Estudo nacional de qualidade de vida na asma - Aplicação do Asthma Quality of LIfe Questionnaire de Marks (AQLQ-M) na população portuguesa

The Portuguese version of the Marks Asthma Quality of Life Questionnaire (AQLQ-M) has already proven to have good diagnostic properties, and is suitable for use with Portuguese asthma patients. The last forty years have seen a worldwide rise in the rate, morbidity, mortality and economic burden associated with asthma and the disease continues to be underdiagnosed and undertreated. In this study the AQLQ-M was administered to 826 asthmatic patients from continental Portugal (97.9% Caucasian, mean age 40.5 years, 30.2% male, 69.8% female), followedup by pulmonologists and allergologists. Patients were classified in line with the Global Initiative for Asthma (GINA) severity categories: 40.5% intermittent asthma, 26.9% mild persistent, 21.4% moderate persistent, 11.2% severe persistent. AQLQ-M scores maintained significant relationships with asthma severity, symptoms and lung function. Most of the patients were not managed and 74.6% were observed in an emergency room during last year. Women reported poorer QoL than men in all the questionnaire’s subscales. Surprisingly, smokers presented better QoL than non-smokers. The results of this study evidence that the Portuguese version of the AQLQ-M is a useful instrument for measuring health-related quality of life in adults with asthma. Rev Port Pneumol 2008; XIV (4): 459-486

Primary cysts and tumors of the mediastinum

OBJECTIVE To assess results in patients with primary cysts and tumours of the mediastinum who under- went surgery. METHODS A retrospective single-centre study was undertaken into patients with primary cysts and tumours of the mediastinum who underwent surgery between January 1992 and December 2004. We analysed demographic data, clinical presentation, type of surgery carried out and procedure, lesion location and histological diagnosis. Predictive malignancy factors were also evaluated. Postoperative morbidity and mortality were noted, as was medium-term results. RESULTS 171 patients underwent surgery over a 13-year period; 73 female (43%) and 98 male (57%). Mean age was 40.3+/-19.7 years (20 days-78 years). A primary cystic lesion was present in 15 patients (9%). The pri- mary tumours included thymic neoplasms (31%), lymphoma (22%), neurogenic tumours (16%), germ cell tumours (9%) and a miscellaneous group (13%). Malignant neoplasms were present in 78 patients (46%). The antero-superior mediastinum was the most commonly involved site to have a primary cyst or tumour (58%), followed by the posterior mediastinum (24%) and the middle mediastinum (18%). Symptoms were present in 68% of the patients and included chest pain (20%), fever and chills (13%), myasthenia gravis (11%), cough (10%), dyspnoea (10%), and superior vena caval syndrome (7%). Univariate analysis identified symptoms as a predictive factors of malignancy (p<0.001). Types of surgery carried out included postero-lateral thoracotomy (64 patients), median sternotomy (51 patients), anterior mediastinostomy (27 patients), antero-lateral thoracotomy (18 patients), video-assisted thoracic surgery (9 patients) and mediastinoscopy (2 patients). Total excision was performed in 116 patients, enlarged resection in 8 patients, subtotal re- section in 7 patients and biopsy in 40 patients. There was one postoperative death (0.6%). Follow-up was available in 165 patients (96.5%) and ranged from 34 days to 13.4 years (mean 5.7+/-4.0 years). Complementary treatment with chemo and/or radiotherapy was provided in 75 patients. Six patients had to be reoperated on for local recurrence (3) or metastasis (3) of the primary lesion. Fifteen patients died of their disease during the follow-up period. Actuarial survival at five years was 97.6% for benign lesions and 76.4% for malignant tumours. CONCLUSION Results support surgical resection for benign lesions and an aggressive multimodal approach for malignant tumours.

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.

Broncoscopia de intervenção

Bronchoscopy became over the last fifteen years a mainstream in the diagnostic investigation, treatment and follow up of the patients with respiratory diseases. The advances in technology make the procedures more easy and tolerable by patients. The safety of the local and general anesthesia, became more and more generalized the use of this diagnostic and (much more) therapeutical mean. Furthermore the traditional indications of bronchoscopy, mainly the fiberoptic bron-choscotpy has been performed more and more frequently in intensive care units on managing respiratory insuficient patients dealing with the treatment of atelectasis and with diagnostic of respiratory infections. The diagnosis of respiratory infections in imunocompromised a very frequent indication to performe fiberoptic bronchoscopy, manly in neutropenic and in acquired imunodeticient syndrome patients. In this work we intend to give an extended view about the indications, execution thechnics, results and complications of bronchoscopy in the treatment of the diseases involving the tracheo-bronchial tract We include the therapeutical methodology of accidentaly inhaled foreign bodies extraction, manly in children, the correction of benign tracheal stenosis and the treatment of tracheo-bronchial neoplasic obstructions. Lung cancer given their growing pathern frequently involve trachea, bronchis and the neighboring tissues causing ventilatory difficulties. This situation needs many times local treatment that must be integrated in the global lung cancer treatment strategy. We describe various therapeutical modalities including instrumental desobstrucsing with rigid bronchoscope, thermical means to destructing the tumour (cryotherapy, electrocautery, laser), brachitherapy and stents insertion. We conclude, as the lung cancer is the main cause of tracheo-bronchial obstruction, local therapy must be integrated with global lung cancer management in order to improve life quality and quantity.

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

Bilateral lung masses: The same aetiology?

The authors describe the case of a 50 year old woman, smoker, healthy until September 2003 when she presented persistent dry cough, fatigue and weight loss. Chest x-ray showed two lung masses, one in the superior right lobe and the other in the lingula lobe of the left lung. The patient underwent TFNA (transthoracic fine needle aspiration) and the cytological result was compatible with small cell lung cancer. Staging procedures identified hepatic lesions, probably secondary. Presence of hepatic metastasis and contralateral lung lesions defined the stage of the disease as disseminate. Chemotherapy with carboplatin and etoposide was started. Six months later the right lesion had decreased but the left lesion had increased. TFNA of this lesion revealed adenocarcinoma. A new treatment was started with vinorelbine and gemcitabine. After four cycles of chemotherapy without any response patient underwent radiotherapy of the left lesion. After 28 months of follow up the patient was asymptomatic and able to manage her normal daily routine. Multiple lung cancers can be considered as synchronous or metachronous, depending on the time of diagnosis. Metachronous lesions are the most frequent (50-70% of all cases) and adenocarcinoma the more frequent histological pattern. In this case the disease was at a disseminate stage, which did not suggest a synchronous lung tumour. While the disease was at an advanced stage with poor prognosis at diagnosis, the evolution of the two different lung tumours did not seem to compromise patients daily routine.

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

Suberose e doença dos criadores de aves: estudo comparativo do perfil radiológico, funcional e do lavado broncoalveolar

Extrinsic Allergic Alveolitis (EAA) is an immunologically mediated interstitial lung disease that may result from repeated inhalation of many different environmental agents. Heterogeneity of the clinical presentation and bronchoalveolar lavage profiles has been described, possibly related to different occupational exposures. The aim of our study was to compare bronchoalveolar lavage fluid (BALF), clinical, functional and radiological characteristics of the two most frequent forms of EAA seen in our practice: Suberosis and Bird Fancier’s Disease (BFD). We included 81 patients with Suberosis, with a mean age of 38.8 ± 11.3 years and a mean exposure of 20.0 ± 10.5 years and 32 patients with BFD, with a mean age of 46.3 ± 11.8 years and mean exposure of 10.5 ± 1.0 years. Patients with BFD had more acute forms, while subacute and chronic presentations predominated in Suberosis. Restrictive defect was the most frequent pattern of lung function impairment, and more severe in BFD. Ground glass opacities were the most frequent pattern in high-resolution computed tomography. A normal chest x-ray was more frequently seen in Suberosis. Both types of EAA had lymphocytic alveolitis in BALF: Suberosis 6.6 ± 5.7 x 105 ml-1 cells, 58.8 ± 18.9% lymphocytes; bird fancier’s disease - 9.0 ± 6.5 x 105 ml-1 cells, 61.7 ± 22.2% lymphocytes. Although BALF CD8 + lymphocytes predominated in both diseases, the proportion of CD4 + and CD4/CD8 ratios were significantly higher in Bird Fancier’s Disease (Suberosis: 0.47 ± 0.33 versus BFD: 1.1 ± 1.5; p < 0.005). Moreover, BALF cellularity and mast cell counts were also significantly higher in BFD. In conclusion, Suberosis and bird fancier’s disease are EAA with different clinical and laboratory profiles, suggesting that despite their pathophysiological similarities, different antigenic exposures may cause different immune and inflammatory response dynamics in the lung. REV PORT PNEUMOL 2004; X (1): 63-75

Prescrição e uso de oxigenoterapia de longa duração – situação actual no distrito do Porto

Introduction: Long-term oxygen therapy (LTOT) increases the survival of COPD patients suffering from severe chronic hypoxaemia. There are specific guidelines for its prescription. The Portuguese Pneumology Society published the Portuguese guideline in 1990. Some foreign studies describe both a lack of compliance to prescription guidelines by the doctors and a lack of adhesion to this therapy by the patients. Objective: Study of the present state of LTOT in Oporto district (North of Portugal), namely through the analysis of the prescription criteria, the patient adhesion levels, and the selected control procedures. Materials and methods: 325 patients were randomly selected from a universe of 2774 Oporto district patients undergoing LTOT therapy. 121 patients were questioned by trained interviewers, according to a structured questionnaire. The 121 patients averaged 69,7 ± IS years old; 67 (55,4%) were male and 54 (44,6%) were female. Results: COPD was the most frequent pathology (46,3%), followed by pneumoconiosis (9,1%). Dispnea was the most frequent symptom (87,6%) among patients, the majority of which were at Fletchers grade 5. 9,9% of the patients maintain their smoking habits. 93,4% of the patients were followed up at doctors appointments, 46,9% of which were pneumology appointments. 47,9% do respiratory function tests regularly and 55,4% do blood gas analysis periodicaly. 48,8% of the patients received their prescription from a pneumologist and 14% received it from the Family doctor. Only 10,7% did 15 hrs/day of oxygen and 43, 8% did not know the debit of oxygen used. 62% used the Venturi mask and 38% nasal cannula. 45,5% of the patients mentioned collateral effects, nasal discomfort being the strongest complain (17,4%). Conclusions: In a substantial part of LTOT patients both oxygen prescription (we observed a lack of compliance by doctors to prescription and follow up guidelines for LTOT) and usage (mainly in what the number of hours of treatment per day is concerned) were ineffective. This should be improved by education of both physicians and patients. REV PORT PNEUMOL 2001; VII (3):

National study of asthma quality of life--application of the Asthma Quality of Life Questionnaire (AQLQ-M) by Marks in the Portuguese population.

The Portuguese version of the Marks Asthma Quality of Life Questionnaire (AQLQ-M) has already proven to have good diagnostic properties, and is suitable for use with Portuguese asthma patients. The last forty years have seen a worldwide rise in the rate, morbidity, mortality and economic burden associated with asthma and the disease continues to be underdiagnosed and undertreated. In this study the AQLQ-M was administered to 826 asthmatic patients from continental Portugal (97.9% Caucasian, mean age 40.5 years, 30.2% male, 69.8% female), followed up by pulmonologists and allergologists. Patients were classified in line with the Global Initiative for Asthma (GINA) severity categories: 40.5% intermittent asthma, 26.9% mild persistent, 21.4% moderate persistent, 11.2% severe persistent. AQLQ-M scores maintained significant relationships with asthma severity, symptoms and lung function. Most of the patients were not managed and 74.6% were observed in an emergency room during last year. Women reported poorer QoL than men in all the questionnaires subscales. Surprisingly, smokers presented better QoL than non-smokers. The results of this study evidence that the Portuguese version of the AQLQ-M is a useful instrument for measuring health-related quality of life in adults with asthma.