A case of obstructive sleep apnea syndrome (OSAS) with narcoleptic features is described; polysomnographic studies were carried out and revealed a severe nocturnal respiratory impairment due to apneic episodes. A multiple sleep latency test (MSLT) showed early REM sleep onset in two out of five series. Nasal CPAP utilization improved the quality of nocturnal sleep and reverted all narcoleptic features in the next day's MSLT.

Keywords: Obstructive sleep apnea syndrome (OSAS). Narcolepsy. Sleep deprivation. CPAP.

The authors report the case of a 7-year-old girl, with a history of recurrent respiratory infections since five months of age. Immunological evaluation at four years of age revealed a normal level of total serum immunoglobulins, but a deficient humoral response to Streptococcus pneumoniae. Although having all normal IgG subclasses, the patient had inadequate antibody levels to all serotypes tested (1, 3, 5, 6, 9, and 14). Concurrent radiological evaluation showed diffuse atelectasis and bronchiectasis. Patient was started on IVIG, and subsequent chest CT scan revealed resolution of the above-mentioned lesions. To prevent irreversible damage to lung parenchyma, due to recurrent lower respiratory infections, it is important to identify immunocompromised individuals and treat them accordingly.

Keywords: Immunologic deficiency syndromes. Streptococcus pneumoniae.

Background: Structural alterations to the pulmonary circulation characterize the vascular remodeling process and are likely correlated with local variations in flow and ischemia. Objective: To define the histological alterations to the pulmonary circulation seen after experimentally-induced ischemia of the pulmonary artery and to correlate those alterations with known patterns of blood redistribution and vascular remodeling. Method: Wistar rats (n = 48) were randomized into two groups with ligation of the pulmonary artery and without (controls) and were sacrificed on post-ischemia days 1, 7, 30 and 60. Lungs were removed and inspected for signs of parenchymal injury. External diameters, as well as wall thicknesses in the pulmonary, alveolar and bronchial end arterioles, were measured. Internal diameter and wall thickness percentage were calculated. Results: Infarction, necrosis and hemorrhage occurred only in ischemic lungs. In nonischemic lungs, there was a sustained increase in the internal and external arteriolar diameters, with an initial reduction in wall thickness on day 1, and day-60 values were similar to those seen in controls. In ischemic lungs, there was a transitory reduction in the internal and external diameters of the pulmonary and bronchial end arterioles, together with an initial, equally transitory, increase in their wall thickness. The alveolar arterioles presented sustained and progressive increases in external diameter and wall thickness, with concomitant reductions in internal diameter. Conclusion: This model mimics distal arterial disease in patients with chronic pulmonary thromboembolism. The vascular response in nonischemic lungs was consistent with a pattern of flow remodeling, whereas that seen in ischemic lungs was more consistent with flow and ischemia. In the pulmonary and bronchial end arterioles, the response was transitory, in contrast to the sustained and progressive response seen in the alveolar arterioles, which was probably caused by delayed local flow.

Keywords: Key-words: Pulmonary embolism. Pulmonary circulation. Vascular remodelling. Pulmonary artery.

A case of polycystic hydatid disease with radiological manifestations that mimicked pulmonary metastatic lesions is reported. The patient, from Brasília, DF, was admitted to the Brasilia University Hospital in February 1996, with a six-month history of unproductive cough, progressive dyspnea, thoracic pain and an episode of hemoptysis. Besides the pulmonary lesions, a CT scan showed hypodense lesions and calcifications in the right lobe of the liver. Repeated examinations of the sputum and bronchoalveolar lavage revealed no malignant cells. An open lung biopsy was then performed and allowed the authors to recognize the helminthic nature of the disease. Protoscolices, probably of Echinococcus vogeli, were found in tissue sections. The source of infection was supposed to be some hunting dog lodged in a kennel kept by the patient. Administration of albendazole brought no significant improvement to the pulmonary lesions, after one year of treatment not yet interrupted.

Keywords: Polycystic hydatid disease. Polycystic echinococcosis. Neotropical echinococcosis. Echinococcus vogeli.

A case of human pulmonary adiaspiromycosis - the 18th from Brazil - is reported for two purposes: to contribute to the knowledge of the clinical course of the disease and to discuss the basis for using the transbronchial biopsy in demonstrating the causative organism. The patient, an 18 year old male, lived in the Paranoa village, near Brasilia, DF, where he worked in a joinery, making straw-brooms. In February 1997, he was admitted to the Brasilia University Hospital, with severe respiratory manifestations. The main symptom was a ventilatory-dependent thoracic pain. A chest roentgenogram showed a diffuse interstitial infiltrate, in both lungs, with micronodular pattern suggestive of a miliary process. No bacterial or fungal organisms could be found in the sputum or bronchial lavages of the patient. A transbronchial biopsy was then performed and the microscopic examination of the lung tissue revealed several large adioconidia. Adiaspiromycosis is believed to be a self-healing condition, since in the great majority of the so far known cases spontaneous remission of the pulmonary lesions has been the rule. Transbronchial biopsy, due to its simplicity, is indicated as the procedure of choice to demonstrate the adiaconidia of the agent in tissue sections - the only way to definitely establish the diagnosis of adiaspiromycosis.

Keywords: Adiaspiromycosis. Pulmonary mycosis. Emmonsia crescens. Emmonsia parva var crescens. Chrysosporium parvum var crescens.

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.

Congenital lobar emphysema is a disease in which symptoms often begin during the first six months of age. In this report, the authors present the case of a twenty-year-old woman whose symptoms started when she had a spontaneous pneumothorax, an exceptional occurrence. The possible etiologies for that congenital lung malformation and the reasons for the performance of a conservative treatment are discussed.

Keywords: Pulmonary emphysema. Pneumothorax. Abnormalities.

The treatment of typical carcinoid tumors in bronchus is usually done by a small pulmonary resection with or without bronchoplasty. The authors report the case of a patient with typical carcinoid tumor in the left lower lobar bronchus, next to the lobar carina. They performed a longitudinal bronchotomy, tumor resection, and suture of the bronchus, preserving the lung tissue. The patient is currently well, three years after surgery, without any evidence of recurrence in the bronchoscopic control.

Keywords: Bronchial carcinoid tumor. Bronchoplasty. Bronchotomy. Lung tissue. Surgery.

In its 1991revision, the American Thorax Society (ATS) recommended that each region should define its own equations to determine the theoretical, or predicted values of the spirometric parameters that best represent the pulmonary functional reality of the local population. At that time, the United States already had various equations of its own as well as the European Community, specially Poland. In 1992, Pereira et al., analysing 4,200 spirograms on healthy Brazilians, found predicted values (PV) of forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1) and middle expiratory flow (MEF) different from those reported by American and European authors. The purpose of the present study was to compare the equations developed by Pereira et al., using a Brazilian sample population, with those determined by Knudson et al. for American populations, that are the most used by Brazilian specialists. This study included 1,070 spirograms performed in patients of both genders (389 males and 681 females) who came to our service for routine functional assessment. No pathology was excluded and the smoking status of the patients was not taken into account. For each patient the PV of FVC, FEV1 and MEF were calculated accordingly to the equations of Pereira et al. (PV-P) and Knudsen et al. (PV-K). Subsequently, the means, standard deviations and reports obtained by both methods were compared. Among females, the PV-K of FVC were 4,01% (p < 0.005) greater, leading to a higher rate of restrictive impairment whereas among males there was no significant difference. Regarding FEV1, PV-P were higher in both males (4.76%; p < 0.005) and females (5.04%; p < 0.05) resulting in a greater sensitivity in the identification of respiratory obstructions. No significant difference was observed between PV-P and PV-K as to FEF25-75 regardless of gender.

Keywords: Spirometry. Reference values. Forced expiratory flow rates.

Non-compliance of tuberculosis treatment has social and epidemiological implications. Purpose: To compare characteristics of patients that were compliant (control group) and non-compliant with the treatment at the Health Care Center at the Federal University of São Paulo, Brazil, between 1995 and 1997, and to verify if patients who had joined in educational classes on tuberculosis had enhanced chances to a positive outcome after expiration of the six months of treatment. Method: The authors conducted a retrospective and controlled study with 100 patients (38 non-compliant and 62 compliant) registered for tuberculosis treatment in order to verify the variables related to non-compliance. Sixty patients (16 non-compliant and 44 compliant) had educational classes. Results: The risk factors most related to non-compliance were: male sex, cigarette smoking, alcohol and drug abuse, risk factors for HIV, and previous hospitalization. Patients who had attended educational classes had the same characteristics as all patients, but they had lower frequency of non-compliance (p < 0.05). Conclusion: The authors conclude that, if they had adequate access to means of diagnosis and follow-up for the treatment of tuberculosis, all extra efforts should be concentrated on avoiding non-compliance, mainly in patients with risk factors, as mentioned above. The patients at risk of non-compliance should have educational classes about their disease.

Keywords: Prevalence. Diagnosis-related groups.

A case of a patient with Pancoast's syndrome associated with acquired immunodeficiency syndrome is reported. This association is rare and Kaposi's sarcoma and non-Hodgkin lymphoma are the most recurrent tumors in AIDS. The patient was an IV drug user and was admitted to the hospital for diagnosis of apex lung mass with signs of Pancoast's syndrome. HIV serology was positive and pathology of lung mass showed non-small cell lung cancer.

Keywords: Pancoast's syndrome. HIV. Bronchogenic carcinoma. Lung neoplasms. Acquired immunodeficiency syndrome.

INTRODUCTION: Cystic Fibrosis is usually diagnosed in childhood. In Brazil, few studies have approached CF diagnosed in adulthood. OBJECTIVE:The aim of this study was to describe demographic and clinical characteristics and spirometric data of patients with CF diagnosed in adulthood (over 16 years of age) in Bahia, Brazil. METHODS: Twenty-eight patients with cystic fibrosis diagnosed in adulthood were evaluated at the Reference Center of Cystic Fibrosis of the state of Bahia. Variables of interest were age, gender, race, body mass index (BMI), sputum culture, presence of sinusitis and bronchiectasis, spirometric tests with the best values performed for forced expiratory volume in the first second (FEV1), forced vital capacity (FVC) and response to bronchodilators. RESULTS: Mean age of diagnosis was 31.1 ± 12.4 years. Of the patients, 53.4% were black and mulatto and the mean BMI was 18.7 ± 3.0 kg/m2. P aeruginosa was present in 12 (43%) of the total patients. The mean percentages ± SD of FVC and FEV1 were 58.9 ± 21.6% and 44.1 ± 23%, respectively. In the group of patients with presence of P. aeruginosa in sputum cultures, mean of the spirometry parameters were lower than those of the P. aeruginosa-free group. However, this difference achieved a statistically significant difference (p = 0.0007) only in FVC . CONCLUSIONS: In agreement with many authors, this study strengthen the point of view that CF must be investigated in patients with recurrent infections, sinusitis and bronchiectasis even in adulthood. Values of the percentages of FVC and FEV1 in relation to those foreseen were lower in patients bearers of P. aeruginosa thereby showing a higher deterioration of the pulmonary function.

Keywords: Cystic fibrosis/diagnosis. Adult. Spirometry/methods.