Pulmonary alveolar microlithiasis (PAM) is a rare disease that affects both lungs. It is characterized by the presence of small calculi (calcium phosphate) within the alveolar spaces. We report the case of a 26-year-old female whose diagnosis was based on characteristic findings on chest X-rays and high-resolution computed tomography scans. The patient, 28 weeks pregnant, was rehospitalized 10 months after the diagnosis, presenting hypoxemic acute respiratory failure and severe restrictive ventilatory defect on spirometry. After 32 completed weeks of gestation (228 days), she was submitted to cesarean section, and the outcome was successful for mother and newborn. PAM has a variable clinical course. It is suggestive of an autosomal recessive inheritance pattern and has been associated with positive family history. The etiology of PAM is unclear, and many authors speculate that there is a local enzymatic defect responsible for the intra-alveolar accumulation of calcium. Reports of patients with PAM who become pregnant are exceptional, and this is the first case described in Brazil. The course of this disease is usually slow and progressive, and patients typically die of cardiorespiratory failure. The present case illustrates the need to offer female patients, especially those with advanced disease, genetic counseling and orientation regarding the risks of pregnancy. Currently, the only effective therapy is lung transplantation.

Keywords: Pulmonary alveoli; Pregnancy; Respiratory insufficiency.

Objective: To present the high-resolution computed tomography (HRCT) findings of pulmonary alveolar microlithiasis. Methods: The HRCT scans of 10 adult patients (seven females and three males; mean age, 38.7 years) were retrospectively analyzed. The films were studied independently by two radiologists. Results: The most common tomographic findings were ground-glass attenuation and linear subpleural calcifications, which were seen in 90% of the patients. Other relevant findings were small parenchymal nodules, calcification along the interlobular septa, nodular cissures, subpleural nodules, subpleural cysts, dense consolidations, and a mosaic pattern of attenuation. Conclusions: The HRCT findings presented by individuals with pulmonary alveolar microlithiasis are distinct. In most cases, such findings can form the basis of the diagnosis, eliminating the need to perform a lung biopsy.

Keywords: Tomography, X-Ray Computed; Lithiasis/lung; Lung diseases.

Objective: Pregnancy brings about significant changes in respiratory function, as evidenced by alterations in lung volumes and capacities, which are attributable to the mechanical impediment caused by the growing foetus. This study was undertaken in order to identify changes in respiratory function during normal pregnancy and to determine whether such changes are more pronounced in twin pregnancy than in singleton pregnancy. Methods: Respiratory function was assessed in 50 women with twin pregnancies and in 50 women with singleton pregnancies (during the third trimester in both groups), as well as in 50 non-pregnant women. We measured the following pulmonary function test parameters: FVC; FEV1; PEF rate; FEV1/FVC ratio; FEF25-75%; and maximal voluntary ventilation. Results: All respiratory parameters except the FEV1/FVC ratio were found to be lower in the pregnant women than in the non-pregnant women. We found no significant differences between women with twin pregnancies and those with singleton pregnancies, in terms of respiratory function. Conclusions: Despite its higher physiological demands, twin pregnancy does not appear to impair respiratory function to any greater degree than does singleton pregnancy.

Keywords: Respiratory function tests; Respiratory mechanics; Pregnancy, twin; Pregnancy.

Objective: To investigate how social and psychological characteristics differ between pregnant women who smoke and do not smoke. To explore associations between social and psychological features with changes of smoking habits by the end of pregnancy. Methods: A case-control study was set up. Smokers cases were never-smokers and ex-smokers controls. Pregnant women (n=328) from public prenatal services were interviewed. Socio-demographic data and psychological variables - personality traits, anxiety, depression, perceived stress, maternal fetal-attachment - were measured. Saliva samples were collected to measure cotinine and to check self-informed smoking status. In addition, 66 smokers were also assessed regarding smoking habits by late pregnancy. Smoking status was defined as a dependent variable. Exposure factors were analyzed through odds ratios. Logistic models and contingency tables were employed according to the nature of variables. "Qualitative change in smoking" was defined as a dependent variable for the last evaluation, and a logistic regression model was built. Results: Lower schooling, higher age, use of alcohol and drugs, living without a partner, and passive smoking showed associations with smoking. Anxiety, depression and perceived stress also exhibited positive association with smoking. Among personality traits, only Neuroticism was associated with smoking. None of the variables were associated with qualitative change in smoking by the end of pregnancy. Conclusion: Smoking during pregnancy is associated with more unfavorable social conditions. Pregnant women who smoke exhibit more negative psychological states than nonsmokers, including a profile of accentuated Neuroticism. None of the investigated variables could predict changes in smoking during pregnancy.

Keywords: Smoking; Pregnancy; Psychology; Psychosocial factors; Personality traits; Big five factor model of personality; Perceived stress; Anxiety; Depression.

Nocardiosis is a localized or disseminated infection caused by gram-positive bacteria of the genus Nocardia. The infection most commonly affects the lungs, skin and central nervous system. Nocardiosis principally occurs in individuals with cellular immunodeficiency and should be considered in the differential diagnosis when such individuals present respiratory, cutaneous or neurological alterations. Herein, we report a case of pulmonary and cutaneous nocardiosis in a patient receiving oral corticosteroids to treat bronchiolitis obliterans accompanied by organizing pneumonia of unknown origin. After long-term treatment with sulfamethoxazole-trimethoprim, the clinical and radiological profile improved.

Keywords: Nocardia infections; Lung diseases; Bronchiolitis obliterans; Adrenal cortex hormones; Case reports [Publication type]

Objective: The aim of this study was to present the evolution of four patients presenting pulmonary alveolar proteinosis and treated at the State University of Londrina School of Medicine. We focus on the importance of whole-lung lavage as the treatment of choice. Methods: A retrospective study of four patients, three females and one male, 22 to 34 years old, presenting similar histories of progressive dyspnea and dry cough. The final diagnosis was established through open-lung biopsy. Three of the patients underwent whole-lung lavage in the Department of Surgery. The procedures were performed under general anesthesia and using a double-lumen endotracheal tube. Results: One patient presented spontaneous regression of the pulmonary alveolar proteinosis without the need for whole-lung lavage. In the other three cases, the number of lavages varied: in one patient, a single unilateral lavage resulted in complete remission of the bilateral process; in another patient, three lavages yielded no significant improvement; in the remaining patient, four lavages provided intervening periods of transient improvement. Conclusion: In the cases evaluated, whole-lung lavage proved an efficient treatment for pulmonary alveolar proteinosis. Although some patients presented a certain resistance to the procedure, it might lead to complete remission of the disease in others.

Keywords: Pulmonary alveolar proteinosis /diagnosis; Bronchoalveolar lavage; Macrophages, alveolar;

Microscopic Polyangiitis is a form of Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated small-vessel vasculitis that preferentially involves venules, capillaries and arterioles and may also involve arteries and veins. It is one of the most common primary systemic small-vessel vasculitis. Its clinical presentation is not distinguishable from the Wegener's granulomatosis (WG) and the Churg-Strauss syndrome (CSS). These types of small-vessel vasculitis are histologically similar and can be differentiated by the presence of granulomatous inflammation in WG or asthma in CSS. The case of a 66-year-old man with microscopic polyangiitis presenting with alveolar hemorrhage is reported with a discussion of the differential diagnosis of other types of pulmonary small-vessel vasculitis.

Keywords: Microscopic polyangiitis. Alveolar hemorrhage. Pulmonary vasculitis

We report the case of a patient with chronic obstructive pulmonary disease and bronchiectasis, chronically using corticosteroids, who acquired pulmonary nocardiosis, which presented as multiple cavitated nodules. The principal symptoms were fever, dyspnea and productive cough with purulent sputum. Chest X-ray and computed tomography of the chest revealed nodules, some of which were cavitated, in both lungs. Sputum smear microscopy and culture revealed the presence of Nocardia spp. The patient was treated with imipenem and cilastatin, which produced an excellent clinical response.

Keywords: Pulmonary disease, chronic obstructive; Nocardia infections; Bronchiectasis.

Objective: Pulmonary rehabilitation (PR) improves exercise capacity in most but not all COPD patients. The factors associated with treatment success and the role of chest wall mechanics remain unclear. We investigated the impact of PR on exercise performance in COPD with severe hyperinflation. Methods: We evaluated 22 COPD patients (age, 66 ± 7 years; FEV1 = 37.1 ± 11.8% of predicted) who underwent eight weeks of aerobic exercise and strength training. Before and after PR, each patient also performed a six-minute walk test and an incremental cycle ergometer test. During the latter, we measured chest wall volumes (total and compartmental, by optoelectronic plethysmography) and determined maximal workloads. Results: We observed significant differences between the pre- and post-PR means for six-minute walk distance (305 ± 78 vs. 330 ± 96 m, p < 0.001) and maximal workload (33 ± 21 vs. 39 ± 20 W; p = 0.02). At equivalent workload settings, PR led to lower oxygen consumption, carbon dioxide production (VCO2), and minute ventilation. The inspiratory (operating) rib cage volume decreased significantly after PR. There were 6 patients in whom PR did not increase the maximal workload. After PR, those patients showed no significant decrease in VCO2 during exercise, had higher end-expiratory chest wall volumes with a more rapid shallow breathing pattern, and continued to experience symptomatic leg fatigue. Conclusions: In severe COPD, PR appears to improve oxygen consumption and reduce VCO2, with a commensurate decrease in respiratory drive, changes reflected in the operating chest wall volumes. Patients with severe post-exercise hyperinflation and leg fatigue might be unable to improve their maximal performance despite completing a PR program.

Keywords: Pulmonary disease, chronic obstructive/rehabilitation; Exercise therapy; Respiratory therapy.

Objective: Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. Methods: This was a retrospective study involving patients with PAP followed between 2002 and 2016. We analyzed information regarding clinical history, diagnostic methods, treatments, and outcomes, as well as data on lung function, survival, and complications. Results: We evaluated 12 patients (8 of whom were women). The mean age was 41 ± 15 years. Most of the patients were diagnosed by means of BAL and transbronchial biopsy. The mean number of WLLs performed per patient was 2.8 ± 2.5. One third of the patients never underwent WLL. Four patients (33.3%) had associated infections (cryptococcosis, in 2; nocardiosis, in 1; and tuberculosis, in 1), and 2 (16.6%) died: 1 due to lepidic adenocarcinoma and 1 due to complications during anesthesia prior to WLL. When we compared baseline data with those obtained at the end of the follow-up period, there were no significant differences in the functional data, although there was a trend toward an increase in SpO2. The median follow-up period was 45 months (range, 1-184 months). The 5-year survival rate was 82%. Conclusions: To our knowledge, this is the largest case series of patients with PAP ever conducted in Brazil. The survival rate was similar to that found at other centers. For symptomatic, hypoxemic patients, the treatment of choice is still WLL. Precautions should be taken in order to avoid complications, especially opportunistic infections.