Primary ciliary dyskinesia (PCD), previously known as immotile cilia syndrome, is an autosomal recessive hereditary disease that includes various patterns of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS), which accounts for 50% of all cases of PCD. The incidence of PCD ranges from 1:20,000 to 1:60,000. Since PCD causes deficiency or even stasis of the transport of secretions throughout the respiratory tract, it favors the growth of viruses and bacteria. As a result, patients have lifelong chronic and recurrent infections, typically suffering from bronchitis, pneumonia, hemoptysis, sinusitis, and infertility. Bronchiectasis and other chronic conditions infections can be the end result of the irreversible bronchial alterations, leading to chronic cor pulmonale and its consequences. Only half of the patients affected by PDC present all of the symptoms, a condition designated complete KS, compared with incomplete KS, typically defined as cases in which situs inversus does not occur. The diagnosis is made clinically and confirmed through transmission electron microscopy. Since there is no specific therapy for PCD, it is recommended that, upon diagnosis, secondary infections be treated with potent antibiotics and prophylactic interventions be implemented. In this paper, we report six cases of PCD (five cases of complete KS and one case of KS) and review the related literature, focusing on the diagnostic, therapeutic and clinical aspects of this disease.

Keywords: Kartagener syndrome; Ciliary motility disorders; Bronchiectasis; Dextrocardia.

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.

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.

Primary ciliary dyskinesia (PCD) is a genetic disorder that is typically inherited in an autosomal recessive manner. It is clinically characterized by recurrent respiratory infections. However, its repercussions for patient quality of life should not be overlooked. Studies have shown that PCD has a significant impact on the lives of patients, although there are as yet no PCD-specific markers of quality of life. To address that problem, researchers in the United Kingdom developed a quality-of-life questionnaire for patients with PCD. The present communication focuses on the process of translating that questionnaire into Brazilian Portuguese, through a partnership between researchers in Brazil and those in the United Kingdom, as well as its subsequent application in patients in Brazil.

Keywords: Quality of life; Kartagener syndrome; Surveys and questionnaires.

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

Objectives: To describe persistent symptoms and lung function in mild cases of COVID-19 six months after infection. Methods: Data collection was performed through a semi-structured questionnaire containing information on the participants' demographic and anthropometric data, the disease in the acute phase, and persistent symptoms six months after COVID-19 using spirometry and manovacuometry. Results: A total of 136 participants were evaluated, of whom 64% were male, with a mean age of 38.17 ± 14.08 years and a body mass index (BMI) of 29.71 ± 17.48 kg/m2. The main persistent symptoms reported were dyspnea on exertion (39.7%), memory loss (38.2%), and anxiety (48.5%). Considering lung function, the participants reached 88.87 ± 17.20% of the predicted forced vital capacity (FVC), 86.03 ± 22.01% of the forced expiratory volume in one second (FEV1), and 62.71 ± 25.04% of peak expiratory flow (PEF). Upon manovacuometry, 97.41 ± 34.67% of the predicted inspiratory force (Pimax) and 66.86 ± 22.97% of the predicted expiratory force (Pemax) were observed. Conclusions: Six months after COVID-19 infection, a reduction in PEF and MEP was observed. Among the most commonly reported persistent symptoms were fatigue, tiredness with the slightest exertion, anxiety and depression, memory loss, and deficits in concentration.

Keywords: Post-acute COVID-19 Syndrome, Respiratory Function Tests, Dyspnea.

Objective: To compare different levels of ablation in terms of the degree of patient satisfaction and extent of postoperative reflex sweating in sympathectomized patients. Methods: A retrospective study involving 521 patients with primary hyperhidrosis, submitted to thoracic sympathectomy at the Monte Sinai Hospital and University Hospital of the Federal University of Juiz de Fora, from January of 2001 to December 2005. All patients were submitted to thermal ablation of the sympathetic stem and were divided into three groups: up to T2 (group I, n = 162); up to T3 (group II, n = 65); and up to T4 (group III, n = 294). Results: Optimal postoperative control of palmar/axillary hyperhidrosis was achieved in, respectively, 94/82% of the patients of group I, 89/89% of those in group II and 80/80% of those in group III. Postoperative reflex sweating was observed in 67% of the patients in groups I and II, compared with 61.29% of those in group III. Severe reflex sweating occurred in 32% of the group I patients, 9% of the group II patients and 4% of the group III patients. Conclusion: Sympathectomy provided excellent patient satisfaction and a low incidence of complications. There was no significant difference between the levels of ablation in terms of reflex sweating, although the intensity of this complication decreased when lower levels of blockage, principally at the T4 level, were employed.

Keywords: Sympathectomy; Thoracoscopy; Hyperhidrosis.

The objective of this study was to provide access to and disseminate a questionnaire used as an instrument to assess the organizational elements and the performance of primary health care clinics regarding TB control in Brazil, comparing selected organizational dimensions by health care clinic, by municipality and by actor (patients, health care workers and managers). The results show that municipalities where the coverage of supervised treatment was more extensive presented more favorable indicators regarding access to TB treatment. The organizational format of the health care clinics involved in TB treatment-family health programs and referral centers with specialized teams in TB control programs (TCPs)-was not a factor that expanded access to diagnosis. The TCPs involving a smaller number of patients presented better performance regarding the health care professional-patient relationship. The majority of the patients faced economic and social difficulties, and most managers were unaware of the amount of resources allocated to TB control activities. The instrument proved to be viable and to have the potential to adequately assess the performance of health care clinics in the urban areas studied.

Keywords: Questionnaires; Patient satisfaction; Primary health care; Family health program; Tuberculosis.

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.

Objective: To evaluate health professionals working at a tertiary pediatric hospital in terms of their knowledge regarding the practical use of metered-dose inhalers. Methods: Practical and written tests on the use of metered-dose inhalers were applied to physicians, physical therapists, nurses and nursing assistants. A score from 0 to 10 was assigned to each evaluation, and median scores were calculated for each professional category. Questions with higher and lower correct values were identified, and a descriptive comparison was made regarding the performance of the various professional categories. Statistical analysis was performed using the Kruskal-Wallis method for comparison of medians. A sequential logistic multiple regression analysis was also performed. Results: A total of 30 resident physicians or interns in the pediatrics department, 23 attending physicians, 29 physical therapists, 33 nurses and 31 nursing assistants were evaluated. Resident physicians, physical therapists and attending physicians performed significantly better than did nurses and nursing assistants. Only resident physicians had a median score greater than 6. Conclusions: The health professionals evaluated, particularly the nurses and nursing assistants, who are directly involved in the practical use of metered-dose inhalers, possess insufficient knowledge regarding the use of such inhalers.

Keywords: Metered dose inhalers; Inhalation spacers; Asthma; Education, public health professional.

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.

Background: The six minutes walk test has been increasingly utilized to assess the effectiveness of different clinical and surgical treatment options in pulmonary diseases. However lack of standardization for their performance may influence measurements and jeopardize assessment of the functional capacity of patients with cardiopulmonary disease. Objective: To determine the possible effects of learning on the distance covered during the six minute walk test for bearers of chronic obstructive pulmonary disease. Method: A retrospective analysis of 35 medical records of COPD patients referred to the Pulmonary Rehabilitation Program of the University Hospital of Brasília was carried out. On alternate days these patients had performed two six minutes walk tests, spirometry and arterial blood gas analysis. Clinical and functional diagnosis was based upon the history of exposure to risk factors, mucus production, dyspnea and spirometric dysfunction after use of bronchodilators. The Student test was used for the comparison of results that were different. Results: The distances covered in the second six-minute walk test (515 ± 82 meters) were greater than those covered in the first six-minute walk test (480 ± 85 metros), with statistically significant differences (p<0.05). However measurements of the muscular effort and perception of dyspnea (Borg scale), peripheral blood oxygen saturation (SpO2), respiratory and heart rates did not disclose any statistically significant differences between the two tests (p<0.05). Conclusion: The present study suggests that in order to standardize the six minutes walk test, the procedure should be performed at least twice to better assess the functional capacity of patients, bearers of chronic obstructive pulmonary disease.

Keywords: Walking. Effectiveness. Lung diseases, obstrutive.

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.

Objective: To evaluate the epidemiological and clinical aspects of acute mediastinitis and to characterize its treatment. Methods: A retrospective study conducted through review of the medical charts of patients diagnosed with acute mediastinitis at the Hospital Escola da Faculdade de Medicina do Triângulo Mineiro (Triângulo Mineiro Medical School Hospital) between 1987 and 2004. Results: A total of 21 patients were studied. Most (76.2%) were male, and the mean age was 52.5 years. Six patients (28.6%) died. The most common cause (in 38.1%) was median sternotomy, followed by esophageal perforation (in 33.3%) and cervical infection (in 14.3%). Staphylococcus aureus and Staphylococcus epidermidis were the causative agents most frequently isolated. In most cases, the treatment of choice was antibiotic therapy accompanied by surgery. The most frequent complications of the acute mediastinitis were pleural effusions (in 23.8%) and osteomyelitis (in 19.0%). The average hospital stay was 26.6 days. Conclusion: Acute mediastinitis is a serious complication of some diseases and procedures. Despite its low incidence, the mortality rate is high. Staphylococcus aureus and Staphylococcus epidermidis are the most common causative agents. The treatment used was antibiotic therapy accompanied by surgery.

Objective: To compare histopathological findings and the degree of apoptosis among rat lungs preserved with low-potassium dextran (LPD) solution, histidine-tryptophan-ketoglutarate (HTK) solution, or normal saline (NS) at two ischemia periods (6 h and 12 h) using an experimental rat model of ex vivo lung perfusion. Methods: Sixty Wistar rats were anesthetized, randomized, and submitted to antegrade perfusion via pulmonary artery with one of the preservation solutions. Following en bloc extraction, the heart-lung blocks were preserved for 6 h or 12 h at 4°C and then reperfused with homologous blood for 60 min in an ex vivo lung perfusion system. At the end of the reperfusion, fragments of the middle lobe were extracted and processed for histopathological examination. The parameters evaluated were congestion, alveolar edema, alveolar hemorrhage, inflammatory infiltrate, and interstitial infiltrate. The degree of apoptosis was assessed using the TdT-mediated dUTP nick end labeling method. Results: The histopathological examination showed that all of the lungs preserved with NS presented alveolar edema after 12 h of ischemia. There were no statistically significant differences among the groups in terms of the degree of apoptosis. Conclusions: In this study, the histopathological and apoptosis findings were similar with the use of either LPD or HTK solutions, whereas the occurrence of edema was significantly more common with the use of NS.

Keywords: : Organ preservation; Organ preservation solutions; Lung transplantation; Reperfusion injury; Apoptosis.