Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with airflow obstruction is a rare form of lung injury. All of the reported cases have been diagnosed by surgical lung biopsy. Only three of the reported cases presented with diffuse interstitial lung opacities on HRCT scans. We report three additional cases of this entity. All of the patients were female and presented with mild-to-moderate airflow obstruction. In the first case, transbronchial biopsy and imaging data were sufficient to make the diagnosis. Although the HRCT scans of all three cases revealed a mosaic pattern, that of the third patient also revealed diffuse interstitial infiltrate. In extremely rare cases, HRCT findings can simulate those seen in other interstitial lung diseases.

Keywords: Neuroendocrine cells; Carcinoid tumor; Bronchiolitis obliterans.

Objective: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease characterized by tachypnea, retractions, crackles, and hypoxia. The aim of this study was to report and discuss the clinical, imaging, and histopathological findings in a series of NEHI cases at a tertiary pediatric hospital, with an emphasis on diagnostic criteria and clinical outcomes. Methods: Between 2003 and 2011, 12 full-term infants were diagnosed with NEHI, based on clinical and tomographic findings. Those infants were followed for 1-91 months. Four infants were biopsied, and the histopathological specimens were stained with bombesin antibody. Results: In this case series, symptoms appeared at birth in 6 infants and by 3 months of age in the remaining 6. In all of the cases, NEHI was associated with acute respiratory infection. The most common initial chest HRCT findings were ground-glass opacities that were in the middle lobe/lingula in 12 patients and in other medullary areas in 10. Air trapping was the second most common finding, being observed in 7 patients. Follow-up HRCT scans (performed in 10 patients) revealed normal results in 1 patient and improvement in 9. The biopsy findings were nonspecific, and the staining was positive for bombesin in all samples. Confirmation of NEHI was primarily based on clinical and tomographic findings. Symptoms improved during the follow-up period (mean, 41 months). A clinical cure was achieved in 4 patients. Conclusions: In this sample of patients, the diagnosis of NEHI was made on the basis of the clinical and tomographic findings, independent of the lung biopsy results. Most of the patients showed clinical improvement and persistent tomographic changes during the follow-up period, regardless of the initial severity of the disease or type of treatment.

Keywords: Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/treatment; Tomography, X-ray computed.

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.

A 35 year-old black woman, previously healthy, who complained about chronic cough and shortness of breath was investigated after non-response of symptoms. Her chest X-ray and high resolution chest CT-scan were normal. She presented with abnormal spirometry, a mild restrictive disturbance. After a negative initial clinical investigation, an open lung biopsy was indicated. Multiple microscopic peribronchiolar tumorlets carcinoids - neuroendocrine cell hyperplasia was revealed. The authors emphasize the importance of an early diagnosis of this unusual entity and discuss its possible pathophysiological mechanisms.

Keywords: Lung diseases. Neuroendocrine cell. Bronchiolitis. Carcinoid. Tumorlets.

In the present report, we describe an unusual presentation of post-infectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in a nine-year-old boy with persistent respiratory symptoms subsequent to an episode of acute bronchiolitis occurring at the age of seven months. After the episode, the persistent respiratory symptoms worsened significantly, and, by the age of seven, the patient began to have difficulty breathing after minimal exertion. Computed tomography of the chest presented findings consistent with bronchiolitis obliterans. Open lung biopsy revealed numerous hemosiderin-laden macrophages, as well as other findings consistent with bronchiolitis obliterans. Pulmonary hemosiderosis can occasionally be accompanied by bronchiolitis obliterans in children with severe sequelae after an episode of viral infection.

Keywords: Bronchoalveolar lavage; Bronchiolitis obliterans; Hemosiderosis; Case reports [Publication type]

Castleman's disease is a rare disorder of the lymphoid tissue. We report the case of a female patient with bilateral otosclerosis, no respiratory symptoms, and pleural effusion discovered as an incidental finding on a chest X-ray. Computed tomography of the chest revealed a mediastinal mass. The biopsy findings demonstrated that it was a plasmacytic variant of Castleman's disease. The patient underwent mediastinal mass resection. This resulted in near-total resolution of the effusion, which remained as a small loculation within the left pleural space.

Keywords: Giant lymph node hyperplasia; Pleural effusion; Case reports.

Objective: To determine the prevalence of asthma symptoms and of airflow obstruction in amateur swimmers between 8 and 17 years of age, as well as to assess the awareness of asthma and asthma management among these swimmers, their parents, and their coaches. Methods: Our sample comprised 1,116 amateur swimmers who completed a modified version of the International Study of Asthma and Allergies in Childhood written questionnaire, to which questions regarding the reasons to initiate swimming and regarding asthma management had been added. In addition, the participants underwent spirometry prior to a swimming competition. Results:   The prevalence of asthma symptoms in the last 12 months was 11.5%, and 327 (29.4%) of the participants reported "wheezing or whistling" in the past. Of the 223 swimmers who reported "asthma ever" or "bronchitis ever", only 102 (45.7%) reported having ever been treated: the most common "treatment" was swimming (in 37.3%), and only 12.7% used inhaled corticosteroids. Of the 254 participants (22.7%) with airflow obstruction, only 52  (20.5%) reported having asthma symptoms. Conclusions: Asthma symptoms are present in amateur swimmers, and a considerable number of such swimmers have airflow obstruction without symptoms. It is therefore likely that the prevalence of asthma is underestimated in this population. It is worrisome that, in our study sample, the swimmers previously diagnosed with asthma were not using the recommended treatments for asthma. The clinical implications of these findings underscore the importance of implementing educational measures for amateur swimmers, as well as for their parents and coaches, to help them recognize asthma symptoms and the consequent risks in the sports environment, in order to allow prompt diagnosis and early clinical intervention.

Keywords: Asthma/diagnosis; Asthma/prevention & control; Sports.

Castleman disease is a rare disorder generally characterized by a mediastinal nodule, with a great variety of alternative presentations regarding age, clinical manifestations and evolution. This case report describes a 40-year-old female patient presenting with uncharacteristic chest pain for a few years. A chest X-ray revealed a hypotransparency on the right side. Computed tomography and pulmonary arteriography did not elucidate the diagnosis, which was made through surgical resection and anatomopathological examination of the nodule, which presented characteristics of angiofollicular hyperplasia, or Castleman disease. This article emphasizes the importance of adding this disease to the list of morbidities in the differential diagnosis of pulmonary solitary nodules.

Keywords: Giant lymph node hyperplasia; Angiography; Coin lesion, Pulmonary; Case reports [publication type].

A 26-year-old patient with a voluminous primary pulmonary hemangiopericytoma in the right lung, diagnosed through previous surgical biopsy, presented irreversible cardiac arrest during the hilar dissection portion of a right pneumonectomy. The patient did not respond to resuscitation efforts. Autopsy showed total obstruction of the mitral valve by a tumor embolism. In cases of large lung masses with hilar involvement, as in the case presented, we recommend preoperative evaluation using transesophageal echocardiography, magnetic resonance imaging or angiotomography. If injury to the pulmonary vessels or atrial cavities is detected, surgery with extracorporeal circulation should be arranged in order to allow resection of the intravascular or cardiac mass, together with pulmonary resection. We recommend that care be taken in order to recognize and treat this problem in patients not receiving a preoperative diagnosis.

Keywords: Embolism; Heart arrest; Pneumonectomy.

Negative-pressure pulmonary edema accompanied by hemorrhage as a manifestation of upper airway obstruction is an uncommon problem that is potentially life-threatening. The principal pathophysiological mechanism involved is the generation of markedly negative intrathoracic pressure, which leads to an increase in pulmonary vascular volume and pulmonary capillary transmural pressure, creating a risk of disruption of the alveolar-capillary membrane. We report the case of an adult male with diffuse alveolar hemorrhage following acute upper airway obstruction caused by the formation of a cervical and mediastinal abscess resulting from the insertion of a metallic tracheal stent. The patient was treated through drainage of the abscess, antibiotic therapy, and positive pressure mechanical ventilation. This article emphasizes the importance of including this entity in the differential diagnosis of acute lung injury after procedures involving upper airway instrumentation.

Keywords: Hemorrhage; Pulmonary edema; Airway obstruction; Abscess; Prostheses and implants.

Systemic sclerosis (SS) is an uncommon disease characterized by small blood vessel vasculopathy and increased connective tissue in the skin and in other organs. The pulmonary involvement is common in SS; however, pulmonary hypertension without interstitial fibrosis is rare. The authors present a case of the diffuse form of SS with isolated pulmonary hypertension. The diagnosis of pulmonary hypertension was suggested by physical examination and confirmed by doppler-echocardiography which allowed for the estimation of the pulmonary artery systolic pressure in 80 mmHg. The authors started treatment with nifedipine; however, as the patient presented side effects, it was not possible to increase the dose beyond 30 mg/day. In this dosage, nifedipine did not decrease the pulmonary arterial pressure.

Keywords: Systemic scleroderma. Nifedipine. Pulmonary hypertension. Vasodilatador agents. Doppler echocardiography.

A 27 year-old female patient, cocaine user, presenting hemoptysis and progressive dyspnea with onset 48 hours prior to hospital admission, without any other signs or symptoms. Serum tests for infectious diseases, collagen disorders and vasculitis were negative. Urinalysis was type I. Computed tomography of the chest showed diffuse alveolar infiltrate, affecting mainly the lower left lobe. A thoracoscopic lung biopsy was performed to clarify the diagnosis. The histopathological findings showed capillaritis and diffuse intra-alveolar hemorrhage. Treated with steroid and cyclophosphamide pulse therapy, a good clinical and radiographical response was obtained. The recently described pauci-immune pulmonary capillaritis is characterized by the presence of isolated pulmonary capillaritis and negative serum testing for auto-immune diseases.

Keywords: Hemorrhage. Pulmonary alveoli/physiopathology.

In order to evaluate which prognostic factors were significant to the survival of patients with idiopathic pulmonary fibrosis (IPF), 121 histologically confirmed cases of the disease were studied at the Pereira Filho Hospital from 1970 to 1996. All patients were submitted to a standard thorax X-ray and spirometry and answered a standardized questionnaire when admitted to hospital. They also underwent diffusion tests (34 cases), total lung capacity (28), blood gas analysis (106), bronchoalveolar lavage (39), rheumatoid analyses (45), and thoracic CT (24). For further analysis, the patients were classified into two groups: group A (2-year survival) with 55 patients, and group B (more than 5-year survival) with 24 patients; these features were also analyzed according to their significance to survival. Age, increased dyspnea index, long symptomatic period, FVC, DCO, PaO2 and SaO2 reduction, honeycombing intensity, and greater profusion of the reticular pattern on HRCT were considered indicative of worse prognosis. A reduced FEV1 and TLC were also associated with shorter survival. The use of those criteria which had shown statistical significance when evaluated together may determine a more accurate prognostic evaluation of IPF patients resulting in social and therapeutic benefits to patient management.

Keywords: Pulmonary fibrosis. Interstitial lung diseases. Prognosis. Prospective studies. Survival analysis.