Amyloidosis is a disease characterized by extracellular deposition of pathologic fibrillar protein in organs and tissues. Diffuse primary tracheobronchial amyloidosis is rare. Herein, we report a case of a male patient with diffuse tracheobronchial amyloidosis, initially diagnosed as bronchial asthma.

Keywords: Asthma/pathology. Amyloidosis/diagnosis. Trachea/pathology.

Tracheobronchial amyloidosis is an uncommon localized form of amyloidosis, characterized by amyloid deposits restricted to the trachea, main bronchi and segmental bronchi. We present the case of a retired 67-year-old man with long-term progressive dyspnea, wheezing and chest pain. A diagnosis of tracheobronchial amyloidosis was made after the third fiberoptic bronchoscopy and histological confirmation through Congo red staining of tissue samples.

Keywords: Amiloidose/traquéia; Broncoscopia; Doenças da traquéia/diagnóstico.

Amyloidosis is a disease characterized by extracellular deposition of fibrillar protein in organs and tissues. Primary tracheal amyloidosis is rare. We report here a case of a 55-year-old man with tracheal amyloidosis hospitalized for acute respiratory insufficiency and with a history of recent episodes of pneumonia. Chest X-ray and chest computed tomography showed tracheal obstruction due to a tumor. A passage was created in order to relieve the symptoms. Histological examination (Congo red staining) revealed amyloid deposits but no evidence of neoplasia. Although this is a rare clinical condition, its importance is discussed regarding the differential diagnosis of tracheal tumors and the repercussions for therapeutic decision-making.

Keywords: Amyloidosis; Respiratory insufficiency; Congo red; Airway obstruction.

Air cysts abnormally emerging from the trachea and/or larger bronchi is an extremely rare disease. Only a few isolated cases of paratracheal air cysts associated with chronic lung diseases have been described in the literature. The aim of this communication is to describe two cases of this rare, clinical entity in members of the same family. The father of the patients died from this disease at 43 years of age and his two daughters are currently under treatment. The clinical findings are similar to those of chronic pulmonary suppuration syndrome. The diagnosis was established by bronchovideoscopy, axial CT with 3D reconstitution and virtual bronchoscopy. The patients are under observation and palliative treatment is being administered.

Keywords: Lung diseases. Cysts. Trachea. Bronchi. Bronchoscopy. Computed tomography.

A 27-year-old male patient presented with respiratory symptoms, bilateral enlargement of the cervical lymph nodes and enlarged liver. In the imaging studies, bilateral enlargement of the hilar nodes was observed, together with pulmonary infiltrate. The patient was submitted to lung and liver biopsies, which revealed noncaseating granulomas. The clinical, radiological and histopathological findings were consistent with sarcoidosis and lymph node amyloidosis. The combination of sarcoidosis and amyloidosis has rarely been reported.

Keywords: Amyloidosis; Sarcoidosis; Lymph nodes.

Primary ciliary dyskinesia is an autosomal recessive disorder characterized by a history of recurrent upper and lower respiratory tract infections with chronic otitis media, bronchitis and rhinosinusitis, associated with situs inversus in 50% of cases. The diagnosis is established by ciliary ultrastructural analysis of respiratory specimens, after ruling out some disorders as cystic fibrosis, a-1 anti-trypsin deficiency, immune deficiencies (IgG, neutrophils and complement) and Young's syndrome. The purpose of this paper is to review the clinical features, diagnosis and management of primary ciliary dyskinesia, including a diagnostic algorithm.

Keywords: Ciliary dyskinesia. Situs inversus. Diagnosis.

Two cases of complete bronchial rupture and one case of tracheobronchial rupture by blunt chest trauma were treated at the Thoracic Surgical and Respiratory Endoscopy Department of the Hospital Universitário Cajuru in a period of 2 years. The surgical technique used was end-to-end anastomosis with interrupt absorbable suture after either resection of the stenotic edges or debridement of the lacerated edges. All three patients have had good recovery. The mean period to remove the chest tubes was 3.6 days, and the mean period of discharge from the Thoracic Surgical Department was 5.6 days. The follow-up was made using chest X-ray (PA and lateral projections), lung function tests, and bronchoscopy.

Keywords: Tracheobronchial rupture. Blunt chest trauma. Evaluation. Treatment.

The surgical treatment of patients with tracheobronchial stenosis requires individualized attention due to the complexity of its origin though tracheoplasty is considered to be the ideal procedure. The most common causes of stenosis are tracheal intubation and the development of neoplasias. These are the very conditions that most benefit from endoscopic treatment when surgical correction is not indicated. Today, endoscopic procedures include the application of different kinds of laser and dilators usually delivered with a rigid bronchoscope, as well as radiotherapy and stents that may be used separately or associated. Basically, there are two types of stents, metal or silicone. The metallic ones are more frequently indicated in cases of tracheomalacia and extrinsic compressive stenosis. Silicone are more applicable in cases of tracheobronchial obstruction resultant from acute inflammation or endoluminal tumor. Although these methods can be used interchangeably in some cases, the best way to correct tracheobronchial stenosis is to correctly decide upon the treatment as early as the preliminary steps.

Keywords: Tracheal stenosis. Orthopedics fixation devices. Angioplasty.

Introduction: Inhalations, postural drainage and respiratory physiotherapy are not always effective in removing tracheobronchial secretions. Objectives: To evaluate an alternative surgical technique, modified crichothyroidotomy, to aspirate tracheobronchial secretions. Method: Modified crichothyroidotomy is the introduction of a catheter number 8, 10 or 12 into the crichothyroid membrane in order to stimulate coughing and the aspiration of secretions. This is a descriptive study of the benefits as well as immediate and late complications caused by the use of this technique in 45 patients with excessive tracheobronchial secretions inadequately removed by usual methods. Results: Immediate surgical complications were bleeding (10 patients), oropharynx deviation (3), subcutaneous emphysema (1) and difficult introduction (1). Mean catheter permanence was 14 ± 16 days and, in all cases, the catheter provoked coughing and allowed the easy aspiration of secretions. The most frequent occurrence (17 patients) was the expulsion of the catheter by coughing, inadequate catheter handling during aspiration and moving of the patients. Conclusion: This is a simple technique with a low morbidity rate and represents constitutes an effective alternative to be used in the aspiration of tracheobronchial secretions.

Keywords: Postural drainage. Aspiration.

Objective: To evaluate the effect that maintaining tracheobronchial sputum at room temperature has on the analysis of ciliary transport and cough, as well as on the contact angle. Methods: Hyaline sputum was collected from 30 individuals without pulmonary diseases, and purulent sputum was collected from patients with bronchiectasis. The samples were analyzed immediately after collection and again after 24 h. Results: After 24 h at room temperature, the purulent sputum presented an increase in cough-induced dislodgment (96 ± 50 vs. 118 ± 61 mm) and a decrease in the contact angle (32 ± 6 vs. 27 ± 6 degrees) (p < 0.05). For the hyaline sputum, there were no alterations in the parameters analyzed. Conclusion:  Hyaline tracheobronchial sputum can be stored in room temperature for 24 h without presenting alterations in ciliary transport or contact angle. However, purulent sputum should not be stored at room temperature for many hours, since ciliary transport and contact angle might be altered as a result.

Keywords: Temperature; Sputum; Cough; Mucociliary clearance

Pulmonary amyloidosis is a rare disease, characterized by extracellular deposition of fibrillary protein in the lungs. Amyloidosis is a generic term for a heterogeneous group of diseases, including Alzheimer's disease and type 2 diabetes mellitus. In the respiratory system, it appears in various forms: tracheobronchial; nodular pulmonary; and alveolar septal (diffuse parenchymal). We present the case of a woman who was a 20 pack-year smoker and had nodular pulmonary amyloidosis, as diagnosed through tests performed prior to laparoscopic cholecystectomy.

Keywords: Amyloidosis/diagnosis; Lymphoproliferative disorders; Neoplasms; Lung.

Primary ciliary dyskinesia (PCD) is a genetic disorder of ciliary structure or function. It results in mucus accumulation and bacterial colonization of the respiratory tract which leads to chronic upper and lower airway infections, organ laterality defects, and fertility problems. We review the respiratory signs and symptoms of PCD, as well as the screening tests for and diagnostic investigation of the disease, together with details related to ciliary function, ciliary ultrastructure, and genetic studies. In addition, we describe the difficulties in diagnosing PCD by means of transmission electron microscopy, as well as describing patient follow-up procedures.

Keywords: Kartagener Syndrome; Cilia; Mucociliary clearance; Ciliary motility disorders; Diagnosis.

With the advent of HRCT, primary spontaneous pneumothorax has come to be better understood and managed, because its etiology can now be identified in most cases. Primary spontaneous pneumothorax is mainly caused by the rupture of a small subpleural emphysematous vesicle (designated a bleb) or of a subpleural paraseptal emphysematous lesion (designated a bulla). The aim of this pictorial essay was to improve the understanding of primary spontaneous pneumothorax and to propose a description of the major anatomical lesions found during surgery.

Keywords: Pneumothorax; Pulmonary emphysema; Tomography, X-ray computed.

Fistulas in the tracheobronchial tree (bronchopleural and tracheoesophageal fistulas) have a multifactorial etiology and present a variable incidence in the literature. In general, the related morbidity and mortality are high. Once such a fistula has been diagnosed, surgical closure is formally indicated. However, the clinical status of affected patients is usually unfavorable, which precludes the use of additional, extensive surgical interventions. In addition, attempts at endoscopic closure of these fistulas have seldom been successful, especially when the fistula is large in diameter. We report the cases of three patients submitted to endoscopic closure of fistulas, two of which were larger than 10 mm in diameter, by means of the insertion of atrial septal defect occluders. The procedure was minimally invasive, and the initial results were positive. The results indicate that this is a promising technique for the resolution of tracheobronchial tree fistulas.

Keywords: Bronchial fistula; Tracheoesophageal fistula; Bronchoscopy; Respiratory therapy.

Primary infection with Paracoccidioides brasiliensis has rarely been observed. A 28-year-old male patient presented with a three-month history of fever, respiratory symptoms and malaise. Chest X-rays revealed bilateral apical infiltrates, right pleuritis and hilar lymphadenomegaly. The patient presented with leukocytosis, severe eosinophilia and increasing titers of anti-P. brasiliensis antibodies in serum. To our knowledge, this is the first report of the primary pulmonary lymph node complex of paracoccidioidomycosis accompanied by hypereosinophilia and affecting a previously healthy adult.

Keywords: Paracoccidioidomycosis; Eosinophilia; Lung diseases, fungal.

Primary pulmonary hypertension (PPH) is a disease of rare, unclear etiology, leading to elevations in pulmonary artery pressure and pulmonary vascular resistance. The vascular lesions range from mild - consisting of neo-intimal proliferation - to severe - characterized by concentric intimal fibrosis, plexiform lesions, and necrotizing arteritis. There have been since 1987 reports describing the association between human immunodeficiency virus (HIV) and PPH, with as many as 60 patients in 1997. The role of HIV in the pathogenesis remains unclear. It is reported a case of a woman in whom this association was observed.

Keywords: Primary pulmonary hypertension. Human immunodeficiency virus.