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;

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.

Cystic adenomatoid malformation of the lung is a rare form of congenital pulmonary disease. It has been found in all lobes, it is rarely bilateral, and is associated to hydramnion, placental edema, and fetal hydrops. It is divided into three types, with different clinical and histologic characteristics. Intrauterine diagnosis is possible by means of ultrasound examination, and the treatment of choice is lobectomy. The authors describe four cases seen at their hospital and review the literature.

Keywords: Cystic adenomatoid malformation. Congenital malformation. Lung.

Necrotizing pneumonia is a serious complication of lobar pneumonia. Four children aged between ten months and three years were admitted with acute bacteremic pneumonia and developed sepsis, pleural effusion, and respiratory distress despite adequate antibiotic treatment and chest tube drainage. Decortication and pulmonary resection were performed in all of them. The observed complications were bronchopleural fistula and one death. The emergency pulmonary resection is indicated when pulmonary necrosis is associated to sepsis and massive bronchopleural fistula. In such circumstances, morbidity and mortality are higher than in other conditions.

Keywords: staphylococcal pneumonia; thoracotomy; lung, surgery

Idiopathic tracheal stenosis is uncommon. Herein, we report four cases, all presenting a similar clinical profile: diagnosed through bronchoscopy and having a history of being treated unsuccessfully for bronchospasm. Three of the patients were treated with dilatation and an oral corticosteroid. One of those three underwent tracheoplasty. In the remaining patient, the stenosis was more extensive (2 cm, with a 70% reduction in the size of the lumen), and dilatation was not an option. Therefore, that patient underwent laryngotracheal resection. In all four patients, the evolution was favorable. Idiopathic tracheal stenosis should be contemplated in cases of 'bronchitis' that are not resolved using conventional treatments. Bronchoscopy and dilatation have provided satisfactory results. Occasionally, laryngotracheal reconstruction is necessary.

Keywords: Trachea/surgery; Constriction; Pathologic; Case reports [publication type].

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.

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.

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.

Alveolar adenoma is a rare benign neoplasm of the lungs, and very few cases have been described in the literature. Patients with alveolar adenoma are frequently asymptomatic and are diagnosed through the accidental discovery of a singular, well-delineated nodule on a routine chest X-ray. The definitive diagnosis is made histologically, and the treatment consists of surgical resection of the nodule..

Keywords: Adenoma; Lung neoplasms; Pulmonary alveoli/pathology; Coin lesion, pulmonary;

Lymphangiomatosis, a rare diseases of controversial origin, occurs in individuals of any age, regardless of gender, but is predominantly seen in younger individuals. It often presents with thoracic involvement, although, the bones, spleen and liver can also be affected. Histologically, the pulmonar involvement includes proliferation, complex anastomoses and secondary dilatation of the lymphatic vessels. Clinically, the presentation is variable. Although radiographic findings can be suggestive of the disease, the final diagnosis is made histologically. We report two cases of lymphangiomatosis, both in females: one was oligosymptomatic and is being treated for the disease; the other had a more progressive form, was diagnosed quite late and ultimately died of the disease.

Keywords: Lymphangioma; Lymphatic System; Pleural Effusion; Lymphatic Diseases; Lymphatic System/abnormalities; Lung Diseases, Interstitial.

Paragonimiasis is a chronic infection caused by a trematode of the genus Paragonimus, the Paragonimus westermani being the one that most infects man. It is an endemic disease in East Asia, however, it is also seen in America. The authors report two cases of paragonimiasis, in which a patient showed a past history and clinical and radiological characteristics suggestive of this disease, diagnosed by the presence of eggs of P. westermani in the sputum, and another patient, a thoracic post-traumatism empyema whose paragonimiasis diagnosis was a histopathological finding in the pleura after a decortication. This diagnosis was confirmed by the presence of eggs of P. westermani in the sputum.

Keywords: paragonimiasis

Human pulmonary dirofilariasis is a rare disease caused by the parasite Dirofilaria immitis. It is usually seen as a solitary pulmonary nodule that mimics lung cancer. Although this disease is considered benign, its diagnosis often requires an excisional lung biopsy. Herein we report the epidemiological, clinical and radiological features observed in seven cases of human pulmonary dirofilariasis from Florianópolis. Six of our seven patients, showed a radiological finding of pulmonary nodule and underwent excisional lung biopsy for diagnosis. In one case, the radiological image was unavailable for review. Therefore, it was not described in this work, and the diagnosis was established through transbronchial biopsy.

Keywords: Dirofilariasis. Dirofilaria immitis. Coin lesion pulmonary.

Pulmonary hypertension occurs when the pressure of the pulmonary artery is disproportionally high for a certain level of pulmonary blood flow. Values of mean pulmonary artery pressure of more than 25 mm Hg at rest or 30 mm Hg during exercise allow the diagnosis of pulmonary hypertension. Sustained or chronic pulmonary hypertension may be secondary to known diseases, mainly to those of cardiac or pulmonary nature, or may be a primary abnormality of the pulmonary circulation, with or without identification of associated conditions. Advances in the knowledge of the mechanisms of vasoconstriction and vascular remodeling have brought better prospects for the treatment of the disease. The correct use of vasodilators and anticoagulants, the new vasodilators, as epoprostenol and its analogs, and surgical techniques have increased the survival of many patients. Pneumologists can view cases of pulmonary hypertension as complications of pulmonary diseases or as a result of dyspnea investigation. Despite its etiology, pulmonary hypertension represents a clear abnormality that affects the right ventricle and can be potentially fatal to patients. Image methods have made the diagnosis of pulmonary hypertension more accessible and non-invasive. Six cases of patients with pulmonary hypertension of different causes are presented and discussed. In conclusion, according to new concepts, idiopathic pulmonary hypertension is no longer an irreversible condition, and the identification of associated conditions with potential treatments can be favorable in the management of the patients with pulmonary hypertension.

Keywords: pulmonary hypertension, vascular resistance, lung diseases, vasodilator agents

Background: Pulmonary sequestration is a congenital anomaly involving the pulmonary parenchyma and its vascularization, and it presents as extralobar or intralobar. Aim: Describe the cases treated at the Institute of Heart (InCor) and Clinical Hospital of the Medical School of the University of São Paulo (FMUSP). Method: Retrospective analyses of the files of the patients treated over the 10-year period taking demographic, clinical and laboratorial data. Results: The patients were recurrent pulmonary infection and hemoptysis were frequent clinical findings in these patients. All cases were intralobar. The main localization was the left lower lobe (66%). Only one diagnosis was made intra-operatively. In the other eight cases, diagnosis was suggested on chest radiographs (100%) and confirmed by angiography (77%) and computed tomography (66%). Lobectomy (77%) was the most common surgical treatment, with low post-operative morbidity, and no mortality. Anatomic and histologic examination was performed in seven cases and confirmed the pathology. Conclusion: Pulmonary sequestration is an uncommon entity, in which the computed tomography and the angiography are the radiological investigations that provide the most information to a definitive and secure diagnosis. Resection of the involved tissue leads to excellent results.

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

Objectives: To evaluate the performance of a computer program designed to facilitate the detection of pulmonary nodules using multidetector computed tomography (MDCT) scans of the chest. Methods: We evaluated 24 consecutive MDCT scans of the chest at the Fleury Diagnostic Imaging Center during the period from October 7 to October 19 of 2006, using a 64-channel CT scanner. The study comprised 12 females and 12 males, ranging from 35 to 77 years of age (mean, 57.9 years). Double reading and a computer-aided diagnosis (CAD) system were used in order to perform two independent analyses of the data. The nodules found using both methods were recorded, and the data were compared. Results: The total sensitivity of CAD for the detection of nodules was 16.5%, increasing to 55% when nodules <4 mm in diameter were excluded. Sensitivity by diameter was 6.5% for nodules <4 mm, 45% for nodules of 4-6 mm, 100% for nodules of 6 mm-1 cm, and 0% for nodules >1 cm. More than 99% of true nodules detected by CAD were registered in the image double-reading process. Conclusions: In this preliminary 24-case study, the sensitivity of computer program tested was not significantly greater than that of the double-reading process that is routinely performed in this facility.

Keywords: Image interpretation, computer-assisted; Coin lesion, pulmonary; Tomography, X-ray computed.

Rhodococcus equi, the principal agent of rhodococcosis, is a pleomorphic, gram-positive, aerobic coccus bacillus that infects humans by inhalation or through a transcutaneous route. It is clinically manifested as a pulmonary abscess. The first two Brazilian cases of rhodococcosis are reported on. Both patients were immunocompromised and showed pulmonary infection. The first patient had AIDS and cavitating pneumonia in the left upper lobe, that was fatal. The second case presented Goodpasture syndrome and was under chronic corticotherapy. He displayed a cavitating nodular lesion in the right upper lobe, that was successfully treated with sulfametoxazol-trimethoprim.

Keywords: Actinomycetal infections. Pulmonary tuberculosis. Acquired immunodeficiency syndrome. Goodpasture syndrome. Brazil.

Coccidioidomycosis, a fungal illness acquired by the inhalation of arthroconidia of Coccidioides sp., was first described in 1894. Coccidioidomycosis is mainly restricted to areas with arid climate, alkaline soil and low rainfall. Consequently, most of the reported cases in Brazil have occurred in the northeastern region. We report three cases of pulmonary coccidioidomycosis occurring between 2005 and 2006 in an endemic area in the state of Ceará, Brazil. The three patients were immunocompetent adult males, hunters of armadillos (Dasypus novemcinctus), with complaints of cough, fever, dyspnea and pleuritic pain. All three patients presented pulmonary involvement, and one also presented cutaneous lesions. Chest X-rays and CT scans of the patients revealed characteristic coccidioidomycosis lesions. The diagnosis was confirmed by serological testing. All of the patients evolved to cure after antifungal treatment.

Keywords: Coccidioidomycosis; Lung diseases, fungal; Coccidioides.

This was descriptive study carried out in a medium-sized Brazilian city. In ≤ 15-year-old contacts of index cases of active pulmonary tuberculosis, we assessed compliance with the Brazilian national guidelines for tuberculosis control. We interviewed 43 contacts and their legal guardians. Approximately 80% of the contacts were not assessed by the municipal public health care system, and only 21% underwent tuberculin skin testing. The results obtained with the Chi-square Automatic Interaction Detector method suggest that health care teams have a biased attitude toward assessing such contacts and underscore the need for training health professionals regarding tuberculosis control programs.

Keywords: Tuberculosis, pulmonary\epidemiology, Tuberculosis, pulmonary\prevention and control; Contact tracing.