Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital malformations. When the patient presented symptoms, including cough, wheezing, and dyspnea, with no clinical improvement after a period of 30 days, imaging studies were conducted and the diagnosis was made.

Keywords: Congenital abnormalities; Respiratory tract diseases; Bronchoscopy.

Objective: The aim of this study was to examine the parenchymal and extracellular matrix remodeling process in two histologic patterns-nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP)-in cases of idiopathic and sclerosis/systemic sclerosis (SSc)-associated interstitial pneumonia. Methods: We examined 15 cases of idiopathic NSIP, 10 cases of idiopathic UIP, 5 cases of SSc-UIP and 9 cases of SSc-NSIP. In the lung parenchyma, epithelial cells, endothelial cells and myofibroblasts were evaluated by immunohistochemical staining, whereas histochemical staining was used in order to evaluate collagen/elastic fibers in the extracellular matrix. Results: The percentage of surfactant protein A-positive epithelial cells was significantly greater in idiopathic NSIP than in SSc-NSIP, as well as being greater in idiopathic UIP than in SSc-UIP. Idiopathic NSIP and idiopathic UIP presented significantly higher immunoexpression of alpha smooth muscle actin in myofibroblasts than did SSc-NSIP and SSc-UIP. The percentage of CD34 endothelial cells in the pulmonary microvasculature was significant lower in idiopathic UIP than in SSc-UIP. The density of collagen fibers was significantly greater in idiopathic NSIP and idiopathic UIP than in SSc-NSIP and UIP. In contrast, the elastic fiber density was significantly lower in idiopathic UIP than in SSc-UIP. Conclusions: Increased collagen synthesis, destruction of elastic fibers, high myofibroblast proliferation and poor microvascularization might represent a remodeling process found in idiopathic interstitial pneumonia, whereas the reverse might represent a repair process in SSc-associated interstitial pneumonia.

Keywords: Epithelial cells; Neovascularization, pathologic; Collagen; Elastin; Idiopathic interstitial pneumonias; Scleroderma, systemic.

The increasing demand in transplantation research requires efficient and less expensive animal models in order to obtain reliable results that are reproducible in larger animal models and, ultimately, applied clinically. The model of unilateral left lung transplantation in rats has proven to be a useful alternative for those purposes. We demonstrate a technical modification of this model, which consists of the isolation and ligation of the contralateral (right) pulmonary artery, allowing blood circulation exclusively in the transplanted lung. This model is feasible and reproducible. However, the short survival time restricts the assessment of the transplanted lung to a maximum period of three hours.

The authors describe a case of a woman submitted to radiation therapy for breast carcinoma who presented, 40 days after the end of the treatment, fever, dry cough, dyspnea on exertion, and weight loss. Chest imaging revealed migratory opacities. The histopathological examination of transbronchial biopsy specimens showed characteristic findings of BOOP. Corticosteroid therapy resulted in dramatic clinical improvement, together with complete clearing of the pulmonary opacities on chest imaging. The authors present a brief review of the literature concerning radiation-induced lung injury, emphasizing the importance of including BOOP in the differential diagnosis in these complications. The present case, as well as the two other reports from the literature, provide further evidence for the role of irradiation injury as a cause of clinicopathological syndrome identical to idiopathic BOOP, i.e., radiation-induced BOOP.

Keywords: Bronchiolitis obliterans organizing pneumonia. BOOP. Radiation pneumonitis. Radiotherapy. Breast carcinoma.

Bronchial stenosis can impair regional lung ventilation by causing abnormal, asymmetric airflow limitation. Electrical impedance tomography (EIT) is an imaging technique that allows the assessment of regional lung ventilation and therefore complements the functional assessment of the lungs. We report the case of a patient with left unilateral bronchial stenosis and a history of tuberculosis, in whom regional lung ventilation was assessed by EIT. The EIT results were compared with those obtained by ventilation/perfusion radionuclide imaging. The patient was using nasal continuous positive airway pressure (CPAP) for the treatment of obstructive sleep apnea syndrome. Therefore, we studied the effects of postural changes and of the use of nasal CPAP. The EIT revealed heterogeneous distribution of regional lung ventilation, the ventilation being higher in the right lung, and this distribution was influenced by postural changes and CPAP use. The EIT assessment of regional lung ventilation produced results similar to those obtained with the radionuclide imaging technique and had the advantage of providing a dynamic evaluation without radiation exposure.

Keywords: Tomography; Electric impedance; Positive-pressure respiration; Pulmonary ventilation; Airway obstruction; Tuberculosis, pulmonary.