Objective: To retrospectively analyze the medical charts of patients with pulmonary malformations submitted to surgical treatment and to investigate the clinical evolution prior to the definitive diagnosis. Methods: We analyzed the medical charts of patients with pulmonary malformations operated on at the São Paulo Hospital-Federal University of São Paulo/Paulista School of Medicine-from 1969 to 2004. Each medical chart was analyzed as to the following aspects: clinical profile; diagnosis; previous treatment; surgical treatment; and nosocomial complications. The inclusion criteria were having received a diagnosis of pulmonary malformation, having undergone pulmonary resection, and chart data being complete. Results: The analysis of the medical charts revealed that 60 patients diagnosed with pulmonary malformations-27 cases of bronchogenic cyst, 14 cases of congenital lobar emphysema, 10 cases of pulmonary sequestration, and 9 cases of cystic adenomatoid malformation-underwent surgery. Ages ranged from 4 days to 62 years (mean, 17.9 years). There was a predominance of males (55%). Ninety-two percent of the patients presented symptoms (mean duration, 15.37 months). Of the 60 patients undergoing surgery, 27 (45%) received preoperative home or hospital treatment with antibiotics. Regarding complications, we observed that morbidity was 23%, and mortality was 3.3%. Surgical times ranged from 1 to 8 h (mean, 3.2 h). Conclusions: Misdiagnosis or delayed diagnosis of pulmonary malformations resulted in unnecessary treatments and hospitalizations, as well as in frequent, recurrent infectious complications. We believe that the definitive treatment is surgery, which is curative and has low morbidity and mortality rates.

Keywords: Bronchogenic cyst; Bronchopulmonary sequestration; Cystic adenomatoid malformation of lung, congenital; Respiratory tract infections; Thoracic surgery.

Objective: To determine the main congenital lung malformations treated and the principal diagnostic methods employed, as well as the indications for surgical treatment and the results obtained, at a referral facility for pediatric thoracic surgery. Methods: We reviewed the medical charts of 52 patients anatomopathologically diagnosed with congenital lung malformations and who had been submitted to pulmonary resection between January of 1997 and December of 2006. Exclusion criteria were age ≥ 12 years and incomplete clinical data. The final sample comprised 35 patients. Results: In this sample, the mean age was 31 months, and there was a predominance of males (n = 21). The anatomopathological findings were cystic adenomatoid malformation (n = 14), congenital lobar emphysema (n = 13), pulmonary sequestration (n = 8) and arteriovenous malformation (n = 1). The most common type of lung resection was left lower lobectomy (in 25.71%) followed by different types of segmentectomy (in 22.85%), left upper lobectomy (in 22.85%), right upper lobectomy (in 14.28%), right lower lobectomy (in 8.57%) and middle lobectomy (in 5.71%). Of the 35 patients, 34 (97.14%) were submitted to closed pleural drainage, with a mean duration of thoracic drainage of 3.9 days. Ten patients (28.5%) presented with postoperative complications. There were no deaths in our sample. Conclusions: Pulmonary resection for the treatment of congenital lung malformations is a safe procedure, presenting low morbidity and no mortality at a referral facility for pediatric thoracic surgery.

Keywords: Cystic adenomatoid malformation of lung, congenital; Bronchopulmonary sequestration; Pneumonectomy; Pulmonary surgical procedures; Pulmonary emphysema.

The objective of this study was to analyze the outcome of surgical treatment of pulmonary aspergilloma. To that end, we evaluated 14 adult patients so treated between 1981 and 2009 at the Botucatu School of Medicine University Hospital, in the city of Botucatu, Brazil. Data were collected from the medical records of the patients. Ten patients (71%) presented with simple pulmonary aspergilloma, and 4 (29%) presented with complex pulmonary aspergilloma. Hemoptysis was the most common symptom, and tuberculosis was the most prevalent preexisting lung disease. Two patients (14%) underwent surgery on more than one occasion. There were no intraoperative deaths. Half of the patients developed postoperative complications, prolonged air leak and empyema being the most common.

Keywords: Pulmonary aspergillosis; Tuberculosis, pulmonary; Thoracic surgery.

Objective: To describe the results of the surgical treatment of children with necrotizing pneumonia. Methods: A retrospective analysis of the medical charts of 20 children diagnosed with necrotizing pneumonia and submitted to surgical treatment between March of 1997 and September of 2008 in the thoracic surgery departments of two hospitals in the city of Manaus, Brazil. We compiled data regarding age, gender, etiologic agent, indications for surgery, type of surgical resection performed, and postoperative complications. Results: The mean age of the patients was 30 months. Of the 20 patients studied, 12 (60%) were female. The most common etiologic agents were Staphylococcus aureus, in 5 patients (25%), and Klebsiella sp., in 2 (10%). The indications for surgery were sepsis, in 16 patients (80%), and bronchopleural fistula, in 4 (20%). The types of surgical procedures performed were lobectomy, in 12 patients (60%), segmentectomy, in 7 (35%), and bilobectomy, in 1 (5%). There were 8 patients (40%) who also underwent decortication. The postoperative complications were as follows: bronchopleural fistula, in 4 patients (20%); empyema, in 1 (5%); pneumatocele, in 1 (5%); and phlebitis of the left arm, in 1 (5%). Four (20%) of the patients died. Conclusions: Surgical resection should be considered in patients with evidence of pulmonary necrosis. Resection is indicated in cases of severe sepsis, high output bronchopleural fistula, or acute respiratory failure that are refractory to clinical treatment.

Keywords: Pneumonia; Necrosis; Lung abscess; Empyema, pleural.

Objective: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. Methods: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). Results: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. Conclusions: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.

Keywords: Telangiectasia, hereditary hemorrhagic; Arteriovenous malformations/diagnosis; Arteriovenous malformations/therapy.

Objective: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods: This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results: Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions: In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

Keywords: Anemia, sickle cell; Respiratory function tests; Exercise test.

Objective: Barriers to physical activity can affect the functional capacity and quality of life of patients with pulmonary hypertension (PH). This study aimed to identify the main barriers to physical activity in patients with PH and to examine whether those barriers are related to functional capacity, echocardiographic variables, or quality of life. Methods: This was a cross-sectional observational study involving 70 patients. Participants scored seven potential barriers to their activities, with a score = 5 indicating a significant barrier. Participants completed the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36) and Manchester Respiratory Activity of Daily Living questionnaire, as well as the six-minute walk test. Correlation analysis, univariate analysis and multiple logistic regression were performed. Results: As a perceived barrier to physical activity, 'lack of will' or 'lack of energy' was cited by 67% of the patients. The 'lack of will' barrier was found to correlate with all SF-36 domains except bodily pain. We also identified a correlation between the SF-36 vitality domain and the barriers 'lack of energy', 'lack of will' and 'lack of structure'. The logistic regression analysis indicated that the vitality domain correlated significantly with the barriers 'social influence', 'lack of energy', 'lack of will', and 'lack of structure'. For each unit decrease in the vitality score, there was a 10% increase in the probability of citing the barrier 'lack of will'. No significant correlations were identified between any of the perceived barriers and echocardiographic parameters. Conclusions: The perceived barrier most commonly reported was 'lack of will/energy', which correlated with almost all SF-36 domains, especially vitality. The 'lack of will' barrier also correlated with functional capacity.

Keywords: Pulmonary arterial hypertension; Exercise; Motivation; Quality of life; Surveys and questionnaires; Walk test.

Objective: To evaluate clinical features of malformations of lung and mediastinum. Methods: Retrospective analysis of 51 patients with malformations identified in the author's division between 1982 and 1997. They verified the clinical manifestations, diagnostic procedures, and treatment. Results: The most frequent anomaly was esophageal atresia/tracheoesophageal fistula (12), followed by vascular ring (11), congenital lobar emphysema (7), diaphragmatic hernia/eventration (7), bronchogenic cyst (5), pulmonary hypoplasia (4), pulmonary sequestration (3), and arterial-venous fistula (2). Age at diagnosis varied from birth to 10 years. Most of them exhibited symptoms at first month, some even at birth. The main respiratory symptoms were tachypnea, coughing, retractions, dyspnea, wheezing, pneumonia and rales. Chest X-rays with barium swallow were most important for diagnosis. When chest radiology was inconclusive, CT scans were next in achieving diagnosis. Surgical intervention was the effective treatment in 86% of cases. Conclusions: Lung and mediastinal congenital malformations with respiratory symptoms require adequate diagnostic evaluation and immediate intervention. The most frequent respiratory symptoms were coughing and tachypnea and chest X-rays with barium swallow was the main diagnostic method. Survival is good and mortality is secondary to associated malformations.

Keywords: Bronchopulmonary malformations. Respiratory distress. Retrospective studies.

Congenital lung malformations are rare and vary widely in their clinical presentation and severity, depending mostly on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods. Recently, there has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. The clinical manifestation of these malformations varies from respiratory distress in the immediate postnatal period to an incidental finding on chest X-rays. Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. Lobectomy is the procedure of choice and yields excellent long-term results. This article describes the principal congenital lung malformations, their diagnosis, and the controversies regarding treatment.

Keywords: Cystic adenomatoid malformation of lung, congenital; Bronchopulmonary sequestration; Pulmonary surgical procedures; Diagnosis.

Objective: To evaluate the differences between surgical biopsies of distinct lung lobes in terms of the histopathological features of usual interstitial pneumonia, using a semiquantitative score. Methods: We selected all of the patients diagnosed with idiopathic pulmonary fibrosis and submitted to surgical biopsy in two distinct lobes between 1995 and 2005 at the Hospital São Paulo and other hospitals operated by the Federal University of São Paulo. In the histological evaluation of the specimens, we used a semiquantitative method based on previous studies, assigning a score to each of the biopsied sites. Results: In this sample of patients, we found no statistically significant differences that would alter the stage of the disease, based on the score used. This finding was independent of the biopsy site (middle lobe or lingular segment). Conclusions: No significant histological differences were found between the lung lobes studied. The definitive histological diagnosis of usual interstitial pneumonia did not alter the stage of the disease.

Keywords: Lung diseases, interstitial; Pulmonary fibrosis; Thoracic surgery; Pathology; Thoracic surgery, ­video-assisted.

Objective: To validate and develop an immunonephelometric assay for the determination of alpha-1 antitrypsin (AAT) levels in dried blood spots from COPD patients in Brazil. Methods: We determined AAT levels in serum samples and dried blood spots from 192 COPD patients. For the preparation of dried blood spots, a disk (diameter, 6 mm) was placed into a tube, eluted with 200 µL of PBS, and stored overnight at 4°C. All of the samples were analyzed by immunonephelometry in duplicate. We used the bootstrap resampling method in order to determine a cut-off point for AAT levels in dried blood spots. Results: The correlation coefficient between the AAT levels in serum samples and those in dried blood spots was r = 0.45. For dried blood spots, the cut-off value was 2.02 mg/dL (97% CI: 1.45-2.64 mg/dL), with a sensitivity, specificity, positive predictive value, and negative predictive value of 100%, 95.7%, 27.2%, and 100%, respectively. Conclusions: This method for the determination of AAT levels in dried blood spots appears to be a reliable screening tool for patients with AAT deficiency.