Although hemophagocytic syndrome is a rare clinical condition, it is associated with high mortality and the number of cases described in the literature has progressively increased. The diagnosis of hemophagocytic syndrome is made on the basis of a finding of hemophagocytosis. Sarcoidosis is a highly prevalent disease whose course and prognosis might correlate with the initial clinical presentation and the extent of the disease. We report the case of a patient with long-standing sarcoidosis who presented with intermittent fever and fatigue. The diagnosis of hemophagocytic syndrome was made by bone marrow aspiration, and specific treatment was ineffective. This is the third case of sarcoidosis-related hemophagocytic syndrome reported in the literature and the first reported in Latin America. All three cases had unfavorable outcomes.

Keywords: Lymphohistiocytosis, hemophagocytic; Ferritins; Sarcoidosis, pulmonary; Macrophage activation syndrome.

Fat embolism is defined as mechanical blockage of the vascular lumen by circulating fat globules. Although it primarily affects the lungs, it can also affect the central nervous system, retina, and skin. Fat embolism syndrome is a dysfunction of these organs caused by fat emboli. The most common causes of fat embolism and fat embolism syndrome are long bone fractures, although there are reports of its occurrence after cosmetic procedures. The diagnosis is made clinically, and treatment is still restricted to support measures. We report the case of a female patient who developed adult respiratory distress syndrome due to fat embolism in the postoperative period following liposuction and fat grafting. In this case, the patient responded well to alveolar recruitment maneuvers and protective mechanical ventilation. In addition, we present an epidemiological and pathophysiological analysis of fat embolism syndrome after cosmetic procedures.

Keywords: Respiratory distress syndrome, adult; Embolism, fat; Lipectomy.

Objective: To evaluate the health-related quality of life of patients submitted to resection of the pulmonary parenchyma due to neoplasia. Methods: The Medical Outcomes Study 36-item Short-Form Health Survey was used to evaluate patients in the preoperative period and on postoperative days 30, 90 and 180. We used the GEE statistical model, in which the dependent variable (quality of life) changes for each patient over the course of the evaluation. Independent variables were gender, age, educational level, type of surgery, radiotherapy, chemotherapy, forced vital capacity and 6-minute walk test. The level of significance adopted was 5%. Results: The final study sample comprised 36 patients, 20 of whom were men. Of those 36 patients, 17 were submitted to lobectomy, 10 to pneumonectomy, 6 to segmentectomy, and 3 to bilobectomy. Chemotherapy was used in 15 patients, radiotherapy in 2, and a combination of radiotherapy and chemotherapy in 2. Improved quality of life was seen in the following domains: social (on postoperative day 90); physical/functional (some patients presenting better forced vital capacity and 6-minute walk test performance); and physical (in patients undergoing smaller resections). Lowered quality of life was seen in the following domains: social (for female patients); physical/social (resulting from radiotherapy, chemotherapy or both); and physical/functional (by postoperative day 30). Conclusions: It is important that studies evaluating the various determinants of quality of life, as well as the impact that cancer treatment modalities have on such variables, be conducted. The knowledge provided by such studies can contribute to improving the quality of life of patients undergoing pulmonary resection due to neoplasia.

Keywords: Lung neoplasms/surgery; Pneumonectomy; Quality of life; Questionnaires

Epidemiologic data on idiopathic pulmonary fibrosis are relatively scarce, and its real incidence and prevalence are unknown. Recent studies suggest that idiopathic pulmonary fibrosis mortality is rising in developed countries. Objectives: To describe idiopathic pulmonary fibrosis mortality in the State of Rio Grande do Sul (RS), Brazil, from 1970 to 2000, analyzing its trend and comparing it with that from other countries. Methods: Prevalence study, using data from the Brazilian Institute of Geography and Statistics (IBGE), analyzing death certificates in which idiopathic pulmonary fibrosis was stated as ultimate cause of death. Results: Annual mortality adjusted to population was 0.22/100,000 persons in the 1970's, 0.3/100,000 persons in the 1980's, and 0.48/100,000 persons in the 1990's. Total mortality rised 36% from 1970 to 1980, and 70% from 1980 to 1990. Mortality adjusted to population rised 36% and 60% during the same periods. The rise in both total and adjusted mortality from IPF was statistically significant (p < 0.05). The mean mortality rate per 100,000 persons during 1996 to 1998, however, was 0.683, corresponding to a rise of 70% when compared to the previous three-year period, which was 0.4 (p = 0.0002), probably reflecting encoding practices. Conclusion: There was a significant increase in IPF mortality in RS from 1970 to 2000, in part due to changes in coding practices. This increase is in conformity with observations in other countries, althought mortality rates in RS are considerably lower.

Keywords: Pulmonary fibrosis. Mortality.

Objective: To identify and characterize alterations seen on HRCT scans in nonsmoking females with COPD due to wood smoke exposure. Methods: We evaluated 42 nonsmoking females diagnosed with wood smoke-related COPD and 31 nonsmoking controls with no history of wood smoke exposure or pulmonary disease. The participants completed a questionnaire regarding demographic data, symptoms, and environmental exposure. All of the participants underwent spirometry and HRCT of the chest. The COPD and control groups were adjusted for age (23 patients each). Results: Most of the patients in the study group were diagnosed with mild to moderate COPD (83.3%). The most common findings on HRCT scans in the COPD group were bronchial wall thickening, bronchiectasis, mosaic perfusion pattern, parenchymal bands, tree-in-bud pattern, and laminar atelectasis (p < 0.001 vs. the control group for all). The alterations were generally mild and not extensive. There was a positive association between bronchial wall thickening and hour-years of wood smoke exposure. Centrilobular emphysema was uncommon, and its occurrence did not differ between the groups (p = 0.232). Conclusions: Wood smoke exposure causes predominantly bronchial changes, which can be detected by HRCT, even in patients with mild COPD.

Objective: The aim of this study was to use a Serbian-language version of the disease-specific, self-report Sarcoidosis Health Questionnaire (SHQ), which was designed and originally validated in the United States, to assess health status in sarcoidosis patients in Serbia, as well as validating the instrument for use in the country. Methods: This was a cross-sectional study of 346 patients with biopsy-confirmed sarcoidosis. To evaluate the health status of the patients, we used the SHQ, which was translated into Serbian for the purposes of this study. We compared SHQ scores by patient gender and age, as well as by disease duration and treatment. Lower SHQ scores indicate poorer health status. Results: The SHQ scores demonstrated differences in health status among subgroups of the sarcoidosis patients evaluated. Health status was found to be significantly poorer among female patients and older patients, as well as among those with chronic sarcoidosis or extrapulmonary manifestations of the disease. Monotherapy with methotrexate was found to be associated with better health status than was monotherapy with prednisone or combination therapy with prednisone and methotrexate. Conclusions: The SHQ is a reliable, disease-specific, self-report instrument. Although originally designed for use in the United States, the SHQ could be a useful tool for the assessment of health status in various non-English-speaking populations of sarcoidosis patients.

Keywords: Sarcoidosis; Health status; Validation studies; Questionnaires; Self report; Serbia.

The authors report the case of a 58 year-old woman with nephrolithiasis, who after being submitted to percutaneous lithotripsy developed xantogranulomatous pyelonephritis, lung abscess and sepsis. Initially, treatment included open drainage of the retroperitoneum, nephrectomy and tubular drainage of the lung abscess and pleural space, through right lateral thoracotomy. Pastereula aerogenes was isolated from both lung and retroperitoneal secretions. To our knowledge, this is the first case of nephrobronchial fistula related with this bacteria in medical literature.

Keywords: Bronchial fistula. Xantogranulomatous pyelonephritis. Lung abscess. Gram-negative bacterial infections. Lithiasis. Kidney.

Psittacosis, also known as ornithosis, is a disease caused by Chlamydia psittaci. The most common clinical presentation is insidious onset, mild symptoms resembling a nonspecific viral illness and preference for the lungs. It is rarely a systemic and fatal disease. It is described a rare case of pneumonia due to Chlamydia psittaci that progressively developed into respiratory failure, requiring mechanical ventilation. It is very important to consider psittacosis in cases of atypical pneumonia whose evolution is unsatisfactory, with no response to antimicrobial therapy and epidemiology is positive for exposure to birds. Prompt recognition is vital as the response to appropriate treatment is excellent. Delayed diagnosis may lead to a severe course and fatal outcome.

Keywords: Mycoplasma pneumonia. Ornithosis.

Sarcoidosis is a systemic disease of unknown etiology, characterized by noncaseating granulomas. Although it may affect any organ, morbidity and mortality are most commonly related to pulmonary involvement, which is found in 80-90% of patients. This study illustrates the principal manifestations of sarcoidosis seen in high-resolution computed tomography scans, including typical as well as atypical forms.

Keywords: Sarcoidosis. Lung. High-resolution computed tomography.

Abstract Lemierre's syndrome is characterized by acute oropharyngeal infection, complicated by internal jugular venous thrombosis secondary to septic thrombophlebitis, and by metastatic infections in various distant organs-most commonly in the lungs. We report a case of Lemierre's syndrome in a 56-year-old female who presented with right-sided neck mass and fever. Right internal jugular venous thrombosis was demonstrated on an ultrasound. A computed tomography scan of the chest revealed multiple opacities throughout both lungs. An open surgical biopsy was performed due to suspicion of pulmonary metastases. Anatomopathological examination revealed septic emboli in lung parenchyma. Retrospectively, the patient reported a history of pharyngitis two weeks prior to hospitalization. After the diagnosis had been made, the patient was treated with broad-spectrum antibiotics (cefuroxime for 7 days and azithromycin for 5 days; subsequently, because fever persisted, cefepime for 7 days). One month later, a computed tomography scan of the chest revealed resolution of the opacities.

Keywords: Pulmonary embolism; Lung abscess; Thrombophlebitis; Jugular veins; Pharyngitis.

Objectives: To verify the prevalence of tuberculosis (TB)/HIV co-infection and the ability of the clinical history to detect the HIV infection in TB inpatients. Setting: Eduardo de Menezes Hospital, reference for both TB and AIDS. Patients and methods: All patients admitted with TB in a pneumology ward were evaluated prospectively from 1/1/1997 to 1/31/1998. The clinical history was directed to the presence of risk factors for AIDS or TB, previous treatments or abandoned treatments for TB, and TB clinical forms. Patients with AIDS defining illnesses, except for TB, and with previous anti-HIV tests were excluded. All patients had an ELISA anti-HIV serology, and when positive, a Western-Blot test was performed to confirm the previous result. X-square test and Fisher test were used for statistical analysis. Results: Sixty-five patients were divided into group I (positive serology for HIV, n = 6) and group II (negative serology for HIV, n = 59). There were no statistical differences between the groups comparing the risk factors for AIDS and TB, previous treatments or abandoned treatments for TB, or TB clinical forms. Conclusions: The high prevalence of TB/HIV co-infection (9.2%) reinforces that: 1) the clinical history was not able to detect a significant number of patients with TB/HIV co-infection and that: 2) anti-HIV serology should be performed in all patients with active TB forms.

Keywords: tuberculosis, acquired immundeficiency syndrome, prevalence

Patients with active tuberculosis (Tb) can be admitted to hospital due to other health problems, Tb not being the admission diagnosis. In this study, the authors analyzed 141 inpatients with Tb, notified to the São Paulo Hospital Central Control of Infectious Diseases, during the period of august, 1999 to April, 2001, trying to identify causes of admission, risk factors, and duration of hospitalization. Of the total number of patients, 63% were male and 37% female, mean age being 38 years-old. 28% were smokers, 23% were alcoholics, and 17% were drug addicts. 23% of them reported previous Tb. 42% were HIV positive. 54.6% of the patients presented pulmonary tuberculosis and 67.5% of them had positive sputum. 22% of the patients had side effects to Tb treatment during hospitalization, drug-induced hepatitis (65.7%) and gastric intolerance (25.7%) being the most frequent. 8.5% of the patients required intensive care, staying there for 11 days, and 54% of the patients stayed at an infirmary with respiratory isolation. 17.7% of the patients died in hospital and 52% due to Tb. The patients were hospitalized for 29 days and isolated for 18 days. Drug addicts and smokers patients had longer hospital stays. HIV positive patients who had positive sputum had the same hospitalization period as others.

Objective: To investigate the significance of cellular immune markers, as well as that of collagen and elastic components of the extracellular matrix, within granulomatous structures in biopsies of patients with pulmonary or extrapulmonary sarcoidosis. Methods: We carried out qualitative and quantitative evaluations of inflammatory cells, collagen fibers, and elastic fibers in granulomatous structures in surgical biopsies of 40 patients with pulmonary and extrapulmonary sarcoidosis using histomorphometry, immunohistochemistry, picrosirius red staining, and Weigert's resorcin-fuchsin staining. Results: The extrapulmonary tissue biopsies presented significantly higher densities of lymphocytes, macrophages, and neutrophils than did the lung tissue biopsies. Pulmonary granulomas showed a significantly higher number of collagen fibers and a lower density of elastic fibers than did extrapulmonary granulomas. The amount of macrophages in the lung samples correlated with FVC (p < 0.05), whereas the amount of CD3+, CD4+, and CD8+ lymphocytes correlated with the FEV1/FVC ratio and VC. There were inverse correlations between TLC and the CD1a+ cell count (p < 0.05), as well as between DLCO and collagen/elastic fiber density (r = −0.90; p = 0.04). Conclusions: Immunophenotyping and remodeling both showed differences between pulmonary and extrapulmonary sarcoidosis in terms of the characteristics of the biopsy samples. These differences correlated with the clinical and spirometric data obtained for the patients, suggesting that two different pathways are involved in the mechanism of antigen clearance, which was more effective in the lungs and lymph nodes.

Keywords: Sarcoidosis; Granulomatous disease, chronic; Extracellular matrix; Immunophenotyping; Respiratory function tests.

Hantavirus pulmonary and cardiovascular syndrome is a recently identified and often fatal disease, which presents as acute respiratory distress syndrome (ARDS). Since the first outbreak, in Nov/Dec 1993, in Juquitiba, Brazil, 226 cases have been registered by FUNASA (National Health Foundation).(4) The disease occurs in previously healthy subjects, presenting with fever and symptoms similar to the common cold, and may rapidly evolve to pulmonary edema, respiratory failure and shock. Hemoconcentration and thrombocytopenia are common features, and the typical radiological finding is a bilateral diffuse interstitial infiltrate that evolves to alveolar consolidations in parallel to the worsening of the clinical condition. Initially, mortality was around 75%, but it declined to approximately 35% in the last few years. Patients who survive usually recover completely, about a week after the onset of the respiratory symptoms. The causal agent is a previously unrecognized hantavirus whose natural reservoirs are rodents of the family Muridae, sub-family Sigmodontinae. Specific antiviral treatment for hantavirus pulmonary and cardiovascular syndrome has not yet been well established, and the efficacy of ribavirin is currently being studied. Intensive care, including mechanical ventilation and invasive hemodynamic monitoring, is required for the more severe presentations of the disease. These measures may improve the prognosis and survival of patients with hantavirus pulmonary and cardiovascular syndrome if started early in the course of the disease.

Keywords: Hantavirus pulmonary syndrome/diagnoses. Hantavirus infections/diagnoses. Brazil.