We report the case of a patient with severe dyspnea upon reclining. Lung disease, neuromuscular disorders and heart disease were ruled out. However, during the course of the investigation, bilateral diaphragmatic paresis was discovered. A key sign leading to the diagnosis was evidence of paradoxical respiration in the dorsal decubitus position. When the patient was moved from the orthostatic position to the dorsal decubitus position, oxygenation and forced vital capacity worsened. The orthostatic fluoroscopy was normal. Maximal inspiratory pressure was severely reduced. The responses to transcutaneous electric stimulation of the diaphragm were normal. However, electric stimulation of the phrenic nerve produced no response, leading to the diagnosis of bilateral diaphragmatic paresis.

Keywords: Paresis; Respiratory insufficiency; Diaphragm; Respiration

We report on a 37-year-old female patient who was first seen on account of a progressive dyspnea of 18 month's duration. Admission diagnosis was bronchial asthma. Over the past seven years, the patient has had seven pneumonia episodes involving mainly the lingular segment. Seventeen years prior to admission (1982) she was orally intubated for 15 days due to meningococcus meningitis and coma. Fiberoptic bronchoscopy revealed marked widening of the carina and stenosis of the main bronchi, confirmed by virtual bronchoscopy. The stenosis was shorter and more severe to the right side (1 cm) and longer and less severe on the left side (2 cm). The patient was submitted to carina resection and anastomosis of the right mainstem bronchus to the trachea and the left main bronchus to the bronchus intermedius. Fifteen months after surgery the patient shows good clinical, radiological and functional evolution.

Keywords: Carinal stenosis. Mainstem bronchus stenosis. Carinal reconstruction.

Hard metal pneumoconiosis, first described in 1964, is a diffuse disease caused by the inhalation of cobalt particles. The disease can manifest as occupational asthma, interstitial disease or allergic alveolitis. We report the case of a young male, working as a tool sharpener, who presented with dry cough and progressive dyspnea for one year, as well as with spontaneous bilateral pneumothorax at admission. The diagnosis was confirmed by open lung biopsy.

Keywords: Lung diseases, interstitial; Pneumoconiosis; Pneumothorax.

The diagnosis and correction of a rare case of traumatic transpericardial diaphragmatic hernia is presented with spontaneous intraoperative reduction by the verification of the lesions of the left lateral wall of the pericardium, diaphragmatic pericardium, and the central tendon, with intact left hemidiaphragm. Only one such case has been published in the last thirty years.

Keywords: Transpericardial diaphragmatic hernia. Thoracic injuries

Pneumothorax is a common complication in cystic fibrosis and is associated with worsening of lung function. However, bilateral simultaneous pneumothorax in cystic fibrosis is a rare condition. We describe the case of a 17-year-old female with cystic fibrosis who presented with spontaneous pneumothorax. Clinically, she presented right-sided chest pain and progressive breathlessness. The pneumothorax failed to resolve after the initial treatment (chest drainage). However, the patient was later successfully treated with additional chest drainage and talc pleurodesis. We also discuss the etiology and management of pneumothorax in patients with cystic fibrosis, since pneumothorax is associated with increased morbidity and mortality among such patients.

Keywords: Cystic fibrosis/physiopathology; Pneumothorax; Pleurodesis.

Objective: Video-assisted thoracoscopic surgery (VATS) has been a surgical intervention of choice for the treatment of spontaneous pneumothorax (SP) with lung bulla. Our objective was to introduce a uniportal VATS approach for simultaneous bilateral bullectomy and to evaluate its therapeutic efficacy. Methods: Between May of 2011 and January of 2012, five patients underwent bilateral bullectomy conducted using this approach. All of the patients presented with bilateral SP. Preoperative HRCT revealed that all of the patients had bilateral apical bullae. We reviewed the surgical indications, surgical procedures, and outcomes. Results: All of the patients were successfully submitted to this approach for bilateral bullectomy, and there were no intraoperative complications. The median time to chest tube removal was 4.2 days, and the median length of the postoperative hospital stay was 5.2 days. The median postoperative follow-up period was 11.2 months. One patient experienced recurrence of left SP three weeks after the surgery and underwent pleural abrasion. Conclusions: Bilateral bullectomy through uniportal VATS combined with contralateral access to the anterior mediastinum is technically reliable and provides favorable surgical outcomes for patients with bilateral SP who develop bilateral apical bullae. However, among other requirements, this surgical procedure demands that surgeons be experienced in VATS and that the appropriate thoracoscopic instruments are available.

Keywords: Pneumothorax; Thoracic surgery, video-assisted; Pleural cavity; Mediastinum.

Objective: To determine whether COPD patients with diaphragmatic dysfunction present higher risk of mortality than do those without such dysfunction. Methods: We evaluated pulmonary function, diaphragm mobility and quality of life, as well as determining the Body mass index, airway Obstruction, Dyspnea, and Exercise capacity (BODE) index, in 42 COPD patients. The patients were allocated to two groups according to the degree to which diaphragm mobility was impaired: low mobility (LM; mobility ≤ 33.99 mm); and high mobility (HM; mobility ≥ 34.00 mm). The BODE index and the quality of life were quantified in both groups. All patients were followed up prospectively for up to 48 months in order to determine the number of deaths resulting from respiratory complications due to COPD. Results: Of the 42 patients evaluated, 20 were allocated to the LM group, and 22 were allocated to the HM group. There were no significant differences between the groups regarding age, lung hyperinflation or quality of life. However, BODE index values were higher in the LM group than in the HM group (p = 0.01). During the 48-month follow-up period, there were four deaths within the population studied, and all of those deaths occurred in the LM group (15.79%; p = 0.02). Conclusions: These findings suggest that COPD patients with diaphragmatic dysfunction, characterized by low diaphragm mobility, have a higher risk of death than do those without such dysfunction.

Keywords: Pulmonary disease, chronic obstructive/mortality; Diaphragm; Exercise tolerance; Quality of life.

Every year, a large number of individuals become dependent on mechanical ventilation because of a loss of diaphragm function. The most common causes are cervical spinal trauma and neuromuscular diseases. We have developed an experimental model to evaluate the performance of electrical stimulation of the diaphragm in rabbits using single-channel electrodes implanted directly into the muscle. Various current intensities (10, 16, 20, and 26 mA) produced tidal volumes above the baseline value, showing that this model is effective for the study of diaphragm performance at different levels of electrical stimulation.

Keywords: Rabbits; Diaphragm; Electric stimulation; Models, animal.

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.

Objective: To describe the clinical and functional findings recently reported in the medical literature for patients diagnosed with emphysema involving the upper lobes and idiopathic pulmonary fibrosis (IPF) involving the lower lobes. Methods: Eleven patients with emphysema and IPF were identified retrospectively. All of the patients underwent high-resolution computed tomography of the lung and pulmonary function tests. Results: Of the 11 patients, 8 were male and 3 were female. The mean age was 70.7 ± 7.2 years (range, 61-86 years). All of the patients were smokers (mean smoking history, 61.5 ± 43.5 pack-years). The mean values of forced vital capacity (FVC), forced expiratory volume in one second (FEV1) and FEV1/FVC were 72.1 ± 12.7%, 68.2 ± 11.9% and 74.4 ± 10.8, respectively. Lung volumes were normal in 7 patients. A restrictive pattern was observed in 3 patients, and hyperinflation was present in one. The diffusing capacity was moderatelyto- severely reduced in all of the patients (mean, 27.7% ± 12.9% of predicted). Ten of the 11 patients performed the six-minute walk test. The mean distance covered was 358.4 ± 143.1 m, and 9 of the 10 patients presented desaturation ≥ 4%. Echocardiographic findings suggestive of pulmonary hypertension were present in 4 patients (mean systolic pulmonary artery pressure, 61.8 mmHg; range, 36-84 mmHg). Conclusions: The concomitant presence of emphysema and IPF causes characteristic changes on pulmonary function tests. The most significant finding is a discrepancy between diffusing capacity and spirometry results.

Keywords: Pulmonary emphysema; Pulmonary fibrosis; Lung Diseases, interstitial; Anoxia; Hypertension, pulmonary.

Although idiopathic pulmonary fibrosis remains a devastating diagnosis, recent advances have improved our understanding of many facets of this disease. These breakthroughs, combined with the increased general availability of therapeutic trials, hold the promise of a brighter future for idiopathic pulmonary fibrosis patients. For example, we now have a more comprehensive understanding of the diagnostic criteria and natural history of the disease. Several studies have shown that simple measurement of pulmonary physiology or gas exchange can be used to predict patient survival. By identifying several molecular pathways that play significant roles in the pathogenesis of idiopathic pulmonary fibrosis, investigators have produced a growing list of novel potential therapeutic targets for the disease. Several prospective, controlled therapeutic trials have been conducted. Others are ongoing or are still in the planning stages. These efforts have advanced our current knowledge of idiopathic pulmonary fibrosis and have raised new important questions, as well as having generated the interest and momentum needed to gain additional ground in the fight against this challenging disease. This article offers the reader a view of the recent advances in idiopathic pulmonary fibrosis research, with a focus on natural history, pathogenesis and treatment.

Keywords: Pulmonary fibrosis/diagnosis; Pulmonary fibrosis/drug therapy; Fibroblasts; Transforming growth factor beta; Lung/pathology, Anti-inflammatory agents/therapeutic use

Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. IPF has been at the forefront of new diagnostic algorithms and treatment developments that led to a shift in patients' care in the past decade, indeed influencing the management of fibrotic interstitial lung diseases other than IPF itself. Clinical presentation, pathophysiology, and diagnostic criteria are briefly addressed in this review article. Additionally, evidence regarding the use of antifibrotics beyond the settings of clinical trials, impact of comorbidities, and therapeutic approaches other than pharmacological treatments are discussed in further detail.

Keywords: Idiopathic pulmonary fibrosis/diagnosis; Idiopathic pulmonary fibrosis/physiopathology; Idiopathic pulmonary fibrosis/therapy; Idiopathic pulmonary fibrosis/rehabilitation.

Objective: To study the effects that pneumoperitoneum achieved through carbon dioxide insufflation has on diaphragmatic excursion in pigs. Methods: A total of 14 male Landrace pigs, 30 to 45 days of age and weighing five to seven kilograms each, were used. The sample was randomly and equally divided into two groups: one (n = 7) in which pneumoperitoneum was maintained at 10 mmHg for 60 minutes; and another (n = 7) in which pneumoperitoneum was maintained at 15 mmHg (also for 60 minutes). After anesthetic induction, the animals were intubated. Flow volume was monitored, and the amplitude of diaphragmatic excursion was analyzed using noninvasive ultrasound imaging of the right hemidiaphragm. Results: In both groups, restricted diaphragmatic excursion was observed only during the procedure. There was no statistical difference between the two pressure levels studied. Conclusion: The amplitude of diaphragmatic excursion was restricted during abdominal insufflation, independent of the pressure level (within the 10-15 mmHg range), during the study period.

Keywords: Pneumoperitoneum/chemically induced; Carbon dioxide; Diaphragm; Video-assisted surgery; Swine

We report the case of a child with tetraplegia after cervical trauma, who subsequently underwent diaphragmatic pacemaker implantation. We reviewed the major indications for diaphragmatic pacing and the types of devices employed. We highlight the unequivocal benefit of diaphragmatic pacing in the social and educational reintegration of individuals with tetraplegia.

Keywords: Spinal cord injuries; Respiration, artificial; Pacemaker, artificial; Quadriplegia.

O diafragma é o principal músculo da respiração e age de forma contínua e ininterrupta para mantê-la. Muitas patologias podem causar disfunção diafragmática, que é geralmente subdiagnosticada na prática clínica em virtude de sua apresentação inespecífica. Embora várias técnicas tenham sido usadas na avaliação da função diafragmática, o diagnóstico de disfunção diafragmática ainda é problemático. A ultrassonografia diafragmática ganhou importância em virtude de suas muitas vantagens: não é invasiva, não expõe os pacientes à radiação, está amplamente disponível, fornece resultados imediatos, é altamente precisa e é repetível à beira do leito. Vários autores descreveram técnicas ultrassonográficas para avaliar a excursão e o espessamento diafragmático na zona de aposição. Estudos recentes propuseram a padronização dos métodos. Este artigo de revisão analisa a utilidade da ultrassonografia na avaliação da função diafragmática, abordando os detalhes da técnica, os principais achados e as aplicações clínicas.

Keywords: Ultrassonografia; Diafragma/diagnóstico por imagem; Músculos respiratórios; Estado terminal; Doenças respiratórias; Doenças neuromusculares.