Organising pneumonia - the experience of an outpatient clinic of a central hospital.

AIM: to characterise outpatients of a Portuguese central hospital diagnosed with organising pneumonia (OP) and compare results with current literature. METHODS: medical processes with diagnosis of OP were retrospectively studied as to demographics, aetiology, clinical and radiological features, average time until and date of diagnosis, laboratory and histological changes, treatment and relapse. Results - thirteen patients with a mean follow -up of 171.6 weeks (max 334 and min 28 weeks) were evaluated. Nine of these patients (70%) had cryptogenic OP (COP) while 30% had secondary OP (SOP), two with rheumatoid arthritis, one with dermatomyositis and another undergoing radiotherapy for breast cancer. Mean age was 55.6 (+ -15.3 years), 92% female, 77% were non -smokers. Average time until diagnosis was 77.2 weeks (min 3 and max 432 weeks). Symptoms at presentation were tiredness (92%), cough (85%), fever (65%), shortness of breath (54%), thoracic pain (23%) and weight loss (23%). At the time of diagnosis, the mean erythrocyte sedimentation rate was 70mm (max 170mm and min 16mm). C -reactive protein level was increased in eight patients. Significant leucocytosis was absent. Chest X -ray and chest CT scan showed bilateral distribution in 12 patients (92%). Consolidation with an air bronchogram was present in 12 patients and in four (31%), consolidation was migratory. Four patients (30%) underwent transbronchial pulmonary biopsy, all uncharacteristic and eight patients surgical pulmonary biopsy, four showed histological confirmation of SOP. Corticosteroids were started in 11 patients and average treatment was 61.6 weeks (16-288 weeks). 15% (2/13) had spontaneous resolution. Four patients (31%) relapsed, one of them five times. Two patients are dependent on a low dose of corticosteroids, one due to underlying disease and another due to multiple relapses. Therapy of relapse was corticosteroids alone in minimum effective dosage or associated to azathioprine or ciclosporin. DISCUSSION AND CONCLUSION: such a high incidence in females (92%) may be explained by the limited sample of patients. In 70% of the patients diagnosis were established by clinical and radiology criteria. Mean time to diagnosis was very variable which suggests that in some cases the disease was not diagnosed and treated as another interstitial lung disease or as recurrent pneumonia. Most patients (53.8%) had a favourable clinical course after treatment with corticosteroids with a very low number of relapses (30.8%), much lower than described by other authors (60%). Only in experienced centres should the diagnosis of OP established by clinical and radiological criteria.

[Epipericardial fat necrosis - Case report]. / Enfarte da gordura epipericárdica - A propósito de um caso clínico.

Epipericardial fat necrosis is an uncommon benign entity of unknown cause, with only 20 cases reported in the English-language literature. It should be remembered as a possible diagnosis in a person who presents with acute pleuritic chest pain and paracardiac density or pleural effusion on chest radiography (X-ray). The computed tomography (CT) or surgical approach allows the final diagnosis and characterization. The authors describe a case of a 48-year-old woman, admitted to our department because of complaints of fatigue, cough and sudden pleuritic pain on thoracic left side. The chest X-ray showed mild/moderate left pleural effusion and CT scan revealed local slight thickening of pericardium and epipericardial fat, surrounded by thick rim of higher density in the left cardiophrenic space. This made the diagnosis of epipericardial fat necrosis. During hospitalization the patient remained stable and was discharged home with symptomatic relieve therapy. Because of benign, self-limited nature of this entity, only conservative treatment is indicated.

[Relapsing pleural effusion and gastric polyposis: a case report]. / Derrame pleural recidivante e polipose gástrica - Caso clínico.

The authors present the case of a lymphoproliferative disorder (LPD) with pleuro-pulmonary involvement. It was a very aggressive primary gastric lymphoma of the MALT subtype, diagnosed in an 86-year-old woman admitted in Pulmonology ward at our Hospital with pleural effusion. Helicobacter pylori search was negative, what is infrequent. The disease was already disseminated at the time of the diagnosis and did not respond to various treatment modalities, what is also an uncommon finding. Clinical presentation, diagnosis and management of this disease are reviewed, pointing out its unique features and comparing the particular aspects of this case with the published literature. The authors stress that LPD is an important diagnosis to be held in mind in the case of a pleural effusion of unknown aetiology.

Four years' follow up at a smoking cessation clinic.

UNLABELLED: Smoking is an important cause of pulmonary pathology and this addiction can be regarded as a chronic, recurrent disease. The benefits of smoking cessation are unquestionable and all physicians should become more active and assertive in recommending it. AIM: To characterise the population seeking medical support for smoking cessation and understand why some successfully stop smoking and others do not. MATERIAL AND METHODS: Retrospective analysis of medical records of outpatients in follow-up between January 2003 and June 2006. Age, gender, age at smoking initiation, smoking burden (number of pack-years), associated diseases, degree of dependence (Fagerström test for nicotine addiction), prior attempts at and motivation for smoking cessation, need for cognitive/behavioural support and success and abandonment rates were evaluated. RESULTS: Five hundred and twenty six patients were studied, 50% male with an average age of 45.5+/-11.4 years. Almost half (43.1 %; n=227) of the patients started smoking before the age of 15. Average smoking burden was 35.8+/-20 pack-years although 21.4% (n=113) smoked more than 50 pack-years. Respiratory disease was present in 52.1% (COPD, 39.9% and others, 12.2%) and cardiovascular disease in 14.6% of the patients. In 46% of patients (n=242) a relevant psychiatric disorder was identified; depression (21.4%), anxiety disorder (19.4%), other dependencies (2.1%) bipolar disorder (1.5%) and schizophrenia (0.6%). The evaluation of degree of addiction revealed maximum level in 69.7% of the patients (n=380). Many patients (72.2%; n=380) reported prior attempts to quit smoking. The strongest reasons for giving up smoking were concern over health (83.5%), financial issues (8.2%) and search for better quality of life (5.7%). Most patients (81.7%; n=430) had undergone nicotine replacement therapy; skin patches (53.3%), chewing gum (1.1%) or both (45.6%). Psychopharmacological treatment included administration of sedative-hypnotics (86.5%), bupropion hydrochloride (2.3%) and antidepressants (0.6%). Seventy six patients (14%) benefited from cognitive/ behavioural support. Two hundred and twenty three patients (42.4%) were successful in giving up smoking while 219 (41.6%) abandoned follow up, the majority after the first appointment. Most patients that abandoned follow up reported lack of motivation and the price of therapy. CONCLUSIONS: The population under study had a high rate of psychiatric disorders and a high level of dependence and lack of motivation that might justify the drop-out rate. Successful treatment was associated with close follow up, behavioural support and pharmacological therapy.

[Whole lung lavage - Report of four cases of alveolar proteinosis]. / Lavagem pulmonar total - A propósito de quatro casos de proteinose alveolar.

Whole lung lavage is a technique that was developed in the 1960s with the purpose of removing lipoproteinaceous material that accumulates in the bronchi of patients with alveolar proteinosis, leading to clinical and functional improvement. There has been an evolution in the technique; initially it was performed under local anesthesia to each segment of the lung and currently it is performed under general anesthesia sequentially to both lungs. This review describes the whole lung lavage, its major indication, alveolar proteinosis and some data on the experience of our hospital.

An economic analysis of erlotinib, docetaxel, pemetrexed and best supportive care as second or third line treatment of non-small cell lung cancer.

AIM: Evaluate costs and benefits of erlotinib as 2nd or 3rd line treatment of advanced or metastatic nonsmall cell lung cancer (NSCLC) versus docetaxel, pemetrexed and best supportive care. MATERIALS AND METHODS: Cost-minimisation and cost-utility analysis were performed. Time horizon of two years. Portuguese National Health System (NHS) perspective was applied. Survival and time to progression were obtained from three clinical trials. Base-case analysis: 2nd or 3rd line patients with advanced or metastatic NSCLC. Quality Adjusted Life Years (QALYs) were obtained from a UK study. Resource consumption was estimated by a Portuguese panel of experts. Costs were calculated according to official Portuguese databases (updated to 2008). Only direct health costs were applied. Annual discount rate: 5%. Sensitivity analysis included different subpopulations, a three year time horizon and a probabilistic analysis. RESULTS: The cost per patient was lower with erlotinib (26,478 euro) than docetaxel (29,262 euro) or pemetrexed (32,762 euro) and higher than best supportive care (16,112 euro). QALYs per patient were higher with erlotinib (0.250) than docetaxel (0.225), pemetrexed (0.241) or best supportive care (0.186). Erlotinib was dominant in the cost-utility analysis, with a lower cost and a higher efficacy than docetaxel and pemetrexed. The sensitivity analysis confirmed the robustness of the base-case analysis results. CONCLUSIONS: The use of erlotinib instead of docetaxel or pemetrexed could contribute to annual savings for the NHS (substitution rates: 5%-65%) ranging from 135,046 euro-1,755,602 euro (docetaxel replacement) and 291,801 euro-3,793,409 euro (pemetrexed replacement), with a gain in terms of QALYs.

Intravascular pulmonary lymphoma with good response to treatment. A case report.

Intravascular lymphoma is a very rare form of large B cell non-Hodgkin's lymphoma, characterised by the presence of lymphoma cells in the lumina of small vessels only, particularly in the capillaries. We report a 54 year-old female non-smoker, admitted to hospital for further examination of a four month long clinical condition involving high fever, night sweats, unqualified weight loss and progressive dyspnea. Patient's temperature was 38.5 degrees C, pulse 100/min and respiratory 22 cycles/min. Patient's haemoglobin was 9.4 g/dL, she had leukocytosis, elevated LDH and arterial blood gas analysis with moderate hypoxaemia (FiO2 1l/m: PaO2-63.6 mm Hg). Chest X-ray revealed diffuse interstitial changes. All the possible causes of unknown origin fever were excluded. Diagnosis was made through lung biopsy and treatment with combined chemotherapy and rituximab was prescribed leading to a 48 hours clinical remission. We present this case to show how difficult this diagnosis can be and how a good response to therapy is possible.

[Epidemiological study of lung cancer in Portugal (2000/2002)]. / Estudo epidemiológico do cancro do pulmão em Portugal nos anos de 2000/2002.

Lung cancer is the most common form of cancer death in the world. Five-year survival is about 15%, without any change to this picture envisaged. It is the 3rd most prevalent type of cancer in Portugal and the primary cause of cancer death. 85% of lung cancer cases are attributable to smoking. One study performed in Portugal for 3 years (2000/2002) by the Lung Oncology Work Committee of the Portuguese Society of Pulmonology in 22 Hospitals showed that of a total of 4396 patients with lung cancer, 81.8% were male and 18.2% were female, with a mean age of 64.49 +/- 11.28 years. About 70% of patients were smokers or former smokers, with 50.3% of patients presenting with performance status (Zubrod) 1. Histologically, 37.5% were adenocarcinoma, followed by squamous carcinoma in 30.5% of cases, and small cell lung cancer in 12.5%; neuroendocrine carcinoma presented in 1.4% of cases; non small cell lung cancer in 10.5%; mixed carcinoma in 0.7%; large cell carcinoma in 2.3%; and others/not specified in 4.6% of cases. Staging (known in 4097 patients), showed 113 patients in stage IA (2.8%)and 250 patients in stage IB (6.1%); only 0.8% in stage IIA and 4.5% in stage IIB; 9.1% in stage IIIA and 29.9% in stage IIIB; 46.9% were already in stage IV by the time of diagnosis. The first therapeutic option was known in 3855 patients. Surgery was performed in 8.2% and 21.8% of cases were treated with combined therapies (surgery and chemotherapy or radiotherapy, or combination of chemotherapy and radiotherapy); chemotherapy alone was first choice in 43.7% of patients and in 20.3% only best support therapy was chosen.

[Sequential chemo-radiation in non-small cell lung cancer: a retrospective study of 100 patients]. / Quimio-radioterapia sequencial no carcinoma pulmonar não de pequenas células: estudo retrospectivo de 100 doentes.

Combined chemotherapy and radiotherapy has shown to be the correct treatment of unresectable non-small cell lung cancer, after many years of poor survival figures with standard radiotherapy alone. It has also been demonstrated that the benefit of chemotherapy is mainly achieved if cisplatin-based schedules are used. The authors present a retrospective study of 100 cases of stage III non-small cell lung cancer treated with a sequential approach of chemotherapy and radiotherapy and evaluate median and overall survival, local progression-free survival and distant progression-free survival. The results of our series are quite similar to those published in literature.

[Chronic obstructive pulmonary disease]. / Doença pulmonar obstrutiva crónica.

Chronic obstructive pulmonary disease (COPD) includes two entities, Chronic Bronchitis (C.B.) and Emphysema (E.), assembled by their common physiopathological feature, a progressive expiratory airflow obstruction. The presence of hyperreactivity is common and partial reversibility may be observed. The coexistence of CB and E. is largely the most prevalent. Cigarette smoking is the main risk factor per se or associated with other urban or occupational air pollutants. Therefore epidemiological COPD rats follow the prevalence of smoking habits in the population. The main goal of the strategy against the disease are preventive decisions and measures, as far as smoking is concerned. FEV1 values lower than 75% of FVC and a decline of FEV1 higher than 30 ml/year indicate a high risk of COPD among smokers. The assessment of FEV1 is important for monitoring and guiding treatment and is valid for grading the severity of COPD. Once the disease is confirmed the therapeutic skills are the following: to reduce the symptoms; to prevent and treat the exacerbations; to attenuate the functional impairment, both short and long term; to achieve better performance in daily activities and quality of life; to avoid and minimise the adverse effects of medication. The increase in life expectancy and the reduction of mortality are tangible goals related to a global and multidisciplinary approach. They mainly depend on smoking cessation, reducing the bronchial obstruction, and correcting chronic hypoxemia.

[Pleural effusion of non-neoplastic etiology in a patient with a rare form of myeloma]. / Derrame pleural de etiologia não neoplásica num doente com uma forma rara de mieloma.

Pleural effusions can be a manifestation of several nosological entities. Etiologic diagnosis involves a good clinical history, followed by thoracocentesis with pleural biopsy and eventually bronchoscopy or thoracoscopy. The differentiation between transudates and exudates, by the biochemical characteristics of the pleural effusions, can orientate the underlying disorder. It is known that there are more than 35 different etiological entities of exudative pleural effusions. However, pneumonia, malignancies, pulmonary embolism, abdominal disease and tuberculosis are the major causes (around 90%). Transudative effusions are more frequently due to congestive heart failure, renal or hepatic failure. The AA present a clinical situation of pleural effusion, the etiology of which was initially attributed to congestive heart failure, with a good response, clinical and radiological response to the treatment established. However the laboratory alteration persisted (anaemia, renal failure, acute inflammation). The subsequent study showed the existence of a rare syndrome, a Myeloma Ig M lambda that can lead to differential diagnosis with Waldenström's Macroglobulinemia, about which the authors make some theoretical considerations showing the difficulty in etiologic diagnosis of some pleural effusions.

[Pulmonary sequestration. Report of a clinical case]. / Sequestração pulmonar. A propósito de um caso clínico.

Concerning a clinical situation of intralobar pulmonary sequestration, in a seventeen year old young man, the authors review this nosological entity. They emphasize the importance of the complementary examens of diagnosis, namely of thoracic scan and aortography, for the establishment of the said diagnosis.

Mastoplastia redutora: manobra tática de Silveira Neto mofidicada para ascençäo do complexo aréolo-mamilar / Reduced mastoplasty: modified Silveira Neto's tactical maneuver for ascension of the nipple-areola complex

Fazendo uma revisäo sobre técnicas de transposiçäo de mamilo e aréola, selecionams a técnica de Sileira Neto, porque permite maior suprimento sangüíneo, maior segurança e facilidade de manipulaçäo. Este método apresenta o melhor resultado em relaçäo ao contorno natural do corpo

Intoxicaçäo pela fumaça / Intoxication due to smoke

Os autores destacam no presente trabalho a importância da intoxicaçäo pela fumaça ba gênese da mortalidade e morbidade do paciente queimado. Analisam sua composiçäo, descrevem a fisiopatologia da agressäo pulmonar, enumeram os principais sinais, sintomas e exames para a conclusäo diagnóstica e expöem as principais diretrizes para o tratamento