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]

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.

We report the case of an 84-year-old male who was admitted to the hospital with persistent cough and dyspnea. An initial chest X-ray revealed pulmonary infiltrates. Nocardia asteroides was detected in sputum, and the patient was treated with antibiotics. However, his symptoms did not completely resolve. He was admitted multiple times, and his symptoms relapsed after every discharge. He was finally suspected of having hypersensitivity pneumonitis and was diagnosed with bird fancier's lung. Pulmonary nocardiosis is likely to develop in patients with chronic pulmonary disorders, such as COPD, as well as in immunosuppressed hosts. To our knowledge, this is the first report of a case of bird fancier's lung complicated by pulmonary nocardiosis.

Keywords: Respiratory hypersensitivity; Bird fancier's lung; Nocardia asteroides; Alveolitis, extrinsic allergic.

Post-infectious bronchiolitis obliterans (PIBO) is a small airways disease characterized by fixed airflow limitation. Therefore, inhaled bronchodilators and corticosteroids are not recommended as maintenance therapy options. The management of PIBO currently consists only of close monitoring of affected patients, aimed at the prevention and early treatment of pulmonary infections. In recent years, there has been an increase in the incidence of PIBO in the pediatric population. Patients with PIBO are characterized by a progressive decline in lung function, accompanied by a decrease in overall functional capacity. Here, we report the case of a relatively young man diagnosed with PIBO and followed for three years. After short- and long-term therapy with an inhaled corticosteroid/long-acting β2 agonist combination, together with an inhaled long-acting antimuscarinic, the patient showed relevant improvement of airway obstruction that had been irreversible at the time of the bronchodilator test. The lung function of the patient worsened when he interrupted the triple inhaled therapy. In addition, a 3-week pulmonary rehabilitation program markedly improved his physical performance.

Keywords: Bronchiolitis obliterans/therapy; Infection/complications; Adrenergic beta-2 receptor antagonists/therapeutic use; Administration, inhalation; Anti-inflammatory agents/therapeutic use; Muscarinic antagonists/therapeutic use; Lung diseases/rehabilitation.

The authors present a 62-year-old male patient who had pulmonary nocardiosis with poor evolution. He also presented mental confusion, septic shock and a significant leukocytosis. Poor evolution happened even when sulfamethoxazole + trimethoprim replaced by ceftriaxone + clindamycin as an antibiotic therapy were used. Diagnostic methods, epidemiology and therapy of this infection are discussed and revised. The authors emphasize the importance of an early diagnosis, isolation in culture, and antibiogram examination of Nocardia ssp samples for therapeutic adapting.

Keywords: Pneumonia. Nocardia.

A síndrome POEMS é uma rara doença de plasmócitos. A ocorrência de hipertensão pulmonar como complicação respiratória da síndrome é pouco frequente e pode estar ligada ao aumento de várias citocinas, quimiocinas e fatores de crescimento como parte dos fenômenos inflamatórios que cercam a fisiopatologia da síndrome POEMS. Descrevemos o caso de uma mulher de 54 anos com síndrome POEMS e hipertensão pulmonar, que foi tratada com corticoide como terapia de primeira linha. Tratava-se de uma paciente com clássicos sintomas dessa síndrome: polineuropatia (confirmada por eletroneuromiografia), organomegalia, hipotireoidismo subclínico, gamopatia monoclonal em dosagem urinária e alterações cutâneas. A cateterização cardíaca direita revelou pressão arterial pulmonar média de 48 mmHg, débito cardíaco de 4,1 L/min e resistência vascular pulmonar de 8,05 Woods. O nível sérico de brain natriuretic peptide (BNP) foi de 150 pg/mL. Nenhuma outra doença foi encontrada durante investigação. Prednisona (1 mg/kg por três meses) foi iniciada, com dramática melhora clínica e funcional, além de normalização dos níveis dos hormônios tireoidianos e de proteína em urina por eletroforese. A pressão arterial pulmonar média caiu para 26 mmHg, o débito cardíaco para 3,8 L/min e a resistência vascular pulmonar para 2,89 Woods. O nível sérico de BNP caiu para 8pg/mL. Nossos achados indicam o potencial papel da corticoterapia como primeira linha de tratamento na hipertensão pulmonar associada à síndrome POEMS. Diante da raridade dessa apresentação, um registro multicêntrico deveria ser desenvolvido para permitir a aquisição de mais dados que suportem essa conduta.

Keywords: Síndrome POEMS; Hipertensão pulmonar; Glucocorticoides.

Idiopathic pulmonary hemosiderosis (IPH), the main cause of pulmonary hemosiderosis in children, is characterized by intermittent alveolar bleeding and hemosiderin-laden macrophages in sputum and in gastric lavage. The treatment is based on corticosteroids and cytotoxic drugs, under special conditions. We describe the case of a 7-year-old girl with IPH who achieved partial clinical remission with high doses of corticosteroids. However, the treatment had to be discontinued because the patient developed Cushing's syndrome. Treatment was started with an azathioprine-corticosteroid combination and then changed to azathioprine alone, which was maintained for four years, with excellent results.

Keywords: Hemosiderosis; Azathioprine; Hemorrhage; Case reports [publication type].

Objective: To determine primary and acquired resistance to pyrazinamide in Mycobacterium tuberculosis strains isolated in sputum samples from patients with pulmonary tuberculosis. Methods: This was a prospective, descriptive study conducted between April and November of 2011 at a referral hospital for tuberculosis in the city of Recife, Brazil. Cultures, drug sensitivity tests, and tests of pyrazinamidase activity were conducted in a private laboratory in Recife. Results: Of the 71 patients included in the study, 37 were treatment-naïve and 34 represented cases of retreatment. Pyrazinamide-resistant strains were isolated in 14 (41.2%) of the 34 patients who had previously been treated for tuberculosis and in none of the 37 treatment-naïve patients. Of the 14 isolates, 10 (90.9%) tested negative for pyrazinamidase activity. A total of 60 isolates tested positive for pyrazinamidase activity. Of those, 56 (93.3%) were found to be sensitive to pyrazinamide. Conclusions: The high frequency of pyrazinamide-resistant strains (41.2%) in patients previously treated for tuberculosis highlights the need for drug susceptibility testing prior to the adoption of a new treatment regimen.

Keywords: Mycobacterium tuberculosis; Pyrazinamide; Tuberculosis, multidrug-resistant

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.

The synchronous presentation of pulmonary and hepatic nodules in a patient with previously resected bronchogenic carcinoma raises suspicion of recurrence and mandates restaging. We present the case of a 71-year-old male with a history of lobectomy with pericardial resection and mediastinal lymphadenectomy (T3N0M0). At five years after the operation, he presented with a new pulmonary lesion. Restaging detected a synchronous nodule in the liver. Despite the strong suspicion of tumor recurrence, further investigation with a percutaneous liver biopsy revealed hepatocellular carcinoma. In order to investigate the etiology of the pulmonary lesion (hypotheses of recurrent bronchial cancer and of metastatic hepatocellular carcinoma), an open lung biopsy was performed, which revealed chronic inflammatory tissue with foci of anthracosis and dystrophic calcification. The patient was submitted to a non-anatomic resection of the liver lesion. The postoperative course was uneventful, and the patient was discharged on postoperative day 10. This report highlights the relevance of the histopathological diagnosis in patients with a history of bronchogenic carcinoma and suspicion of tumor recurrence. Differential diagnoses and the treatment administered are discussed.

Keywords: Carcinoma, bronchogenic; Neoplasm metastasis; Carcinoma, hepatocellular.

Spontaneous pneumomediastinum is an uncommon event, the clinical picture of which includes retrosternal chest pain, subcutaneous emphysema, dyspnea, and dysphonia. The pathophysiological mechanism involved is the emergence of a pressure gradient between the alveoli and surrounding structures, causing alveolar rupture with subsequent dissection of the peribronchovascular sheath and infiltration of the mediastinum and subcutaneous tissue with air. Known triggers include acute exacerbations of asthma and situations that require the Valsalva maneuver. We described and documented with HRCT scans the occurrence of pneumomediastinum after a patient with bleomycin-induced interstitial lung disease underwent pulmonary function testing. Although uncommon, the association between pulmonary function testing and air leak syndromes has been increasingly reported in the literature, and lung diseases, such as interstitial lung diseases, include structural changes that facilitate the occurrence of this complication.

Keywords: Mediastinal emphysema; Subcutaneous emphysema; Spirometry; Bleomycin.

The authors report the case of a 7-year-old girl, with a history of recurrent respiratory infections since five months of age. Immunological evaluation at four years of age revealed a normal level of total serum immunoglobulins, but a deficient humoral response to Streptococcus pneumoniae. Although having all normal IgG subclasses, the patient had inadequate antibody levels to all serotypes tested (1, 3, 5, 6, 9, and 14). Concurrent radiological evaluation showed diffuse atelectasis and bronchiectasis. Patient was started on IVIG, and subsequent chest CT scan revealed resolution of the above-mentioned lesions. To prevent irreversible damage to lung parenchyma, due to recurrent lower respiratory infections, it is important to identify immunocompromised individuals and treat them accordingly.

Keywords: Immunologic deficiency syndromes. Streptococcus pneumoniae.

Objective: To estimate the rates of recurrence, cure, and treatment abandonment in patients with pulmonary tuberculosis treated with a four-drug fixed-dose combination (FDC) regimen, as well as to evaluate possible associated factors. Methods: This was a retrospective observational study involving 208 patients with a confirmed diagnosis of pulmonary tuberculosis enrolled in the Hospital Tuberculosis Control Program at the Institute for Thoracic Diseases, located in the city of Rio de Janeiro, Brazil. Between January of 2007 and October of 2010, the patients were treated with the rifampin-isoniazid-pyrazinamide (RHZ) regimen, whereas, between November of 2010 and June of 2013, the patients were treated with the rifampin-isoniazid-pyrazinamide-ethambutol FDC (RHZE/FDC) regimen. Data regarding tuberculosis recurrence and mortality in the patients studied were retrieved from the Brazilian Case Registry Database and the Brazilian Mortality Database, respectively. The follow-up period comprised two years after treatment completion. Results: The rates of cure, treatment abandonment, and death were 90.4%, 4.8%, and 4.8%, respectively. There were 7 cases of recurrence during the follow-up period. No significant differences in the recurrence rate were found between the RHZ and RHZE/FDC regimen groups (p = 0.13). We identified no factors associated with the occurrence of recurrence; nor were there any statistically significant differences between the treatment groups regarding adverse effects or rates of cure, treatment abandonment, or death. Conclusions: The adoption of the RHZE/FDC regimen produced no statistically significant differences in the rates of recurrence, cure, or treatment abandonment; nor did it have any effect on the occurrence of adverse effects, in comparison with the use of the RHZ regimen.

Keywords: Tuberculosis, pulmonary, Drug combinations; Recurrence.

We report the case of a 50-year-old male with a rare combination: pulmonary tuberculosis and cutaneous leukocytoclastic vasculitis. The patient sought emergency treatment presenting with headache, arthralgia, cutaneous rash, and weight loss (4 kg) in the last 20 days. A chest X-ray, performed in a previous outpatient visit, revealed cavitation in the middle and upper lobes of the right lung, as confirmed by computed tomography. Sputum smear microscopy (Ziehl-Neelsen staining) was positive in three consecutive samples, and the result of the skin lesion biopsy was consistent with cutaneous leukocytoclastic vasculitis. The patient was therefore diagnosed with cutaneous leukocytoclastic vasculitis accompanied by pulmonary tuberculosis. Our objective was to describe a combination rarely reported in the medical literature and to discuss the possible pathogenic mechanisms of this combination.

Keywords: Vasculitis, hypersensitivity; Tuberculosis; Hypersensitivity.

Objective: To describe the profile of patients with cystic fibrosis (CF). Methods: A prospective, cross-sectional study involving CF patients ≤ 18 years of age, evaluated between March and July of 2006 at a referral center in northeastern Brazil. Nutritional assessment was performed using Z scores for height/age (H/A), weight/age (W/A) and weight/height (W/H), as well as %weight/height (%W/H) and body composition measurements. Socioeconomic and clinical data were obtained. Results: Twenty-one patients were evaluated, 12 (57.1%) of whom were female. Mean age at diagnosis was 3.8 ± 3.9 years. The principal features at diagnosis were respiratory infection (85.7%), steatorrhea (66.7%) and nutritional deficit (47.6%). The mean Z scores for W/A, H/A and W/H were 0.73 ± 0.28, 0.34 ± 0.21 and 0.73 ± 0.35, respectively. Mean %W/H was 94.52 ± 1.58. The percentage of malnourished children assessed by Z score differed from that assessed by %W/H (nutritional deficit in 66.7% and 33.3%, respectively; p > 0.05). Socioeconomic status, clinical status and Shwachman score were better among well-nourished patients than among those classified as malnourished (p < 0.05 for Shwachman score). Conclusions: Normal nutritional status was identified based on nutritional indicators (W/A, H/A and W/H), whereas nutritional deficit was identified by assessing body composition. Socioeconomic factors proved favorable, especially maternal education and per capita income. Age at diagnosis was higher than that reported in the literature, although the Shwachman score and the incidence of respiratory infections demonstrated that the patients presented good clinical status.

Keywords: Cystic fibrosis; Nutritional assessment; Symptoms, clinical; Socioeconomic factors.