Plastic bronchitis (PB), although a rare cause of airway obstruction, has mortality rates up to 50% in children after Fontan-type cardiac surgery. We present the case of an 18-month-old female patient with PB following pneumonia. At 6 months of age, the patient underwent the Glenn procedure due to functionally univentricular heart. Fiberoptic bronchoscopy revealed complete blockage of the left bronchus by mucoid casts. Pharmacotherapy consisted of glucocorticosteroids, azithromycin, and enalapril maleate. The child also received nebulized 3% NaCl solution, which proved to be beneficial. In children submitted to Fontan-type procedures, physicians must be alert for PB, which can be triggered by respiratory tract infection.

Keywords: Bronchitis; Heart defects, congenital; Saline solution, hypertonic.

Objective: To assess airway hyperresponsiveness to 4.5% hypertonic saline solution in comparison to that obtained through challenge with other bronchoconstriction agents and in relation to patient allergic sensitization. Methods: A cross-sectional, experimental study was conducted, initially involving 85 subjects. After exclusions, the final sample consisted of 62 patients, divided into two groups: a study group of those with asthma (n = 45) and a control group of those with no asthma or allergies (n = 17). Hypertonic saline was nebulized using an ultrasonic nebulizer and administered successively for 0.5, 1, 2, 4 and 8 minutes until a drop in forced expiratory volume in one second of = 15% was achieved in relation to the baseline value. The level of specific immunoglobulin E to Dermatophagoides pteronyssinus level was determined by ImmunoCAP assay and was considered positive when > 0.35 kU/L. Results: In the 36 asthma group subjects presenting a response, the mean drop in forced expiratory volume in one second after hypertonic saline nebulization was 27.4%. None of control group subjects (immunoglobulin E < 0.35 kU/L) presented a positive response to hypertonic saline. The mean forced expiratory volume in one second for control group subjects was 9%. The results of a bronchial provocation test were negative in 9 of the asthma group subjects. The frequency of bronchial provocation test positivity was higher in the subjects presenting elevated levels of specific immunoglobulin E, indicating that there is a relationship between bronchial hyperresponsiveness and the level of specific immunoglobulin E. The sensitivity and specificity of the test were 80% and 92%, respectively. Conclusion: Bronchial provocation with hypertonic saline presents satisfactory sensitivity and specificity. Therefore, in addition to being a low cost procedure that requires very little equipment, it is a useful means of assessing hyperresponsiveness in children and adolescents.

Keywords: Bronchial provocation tests; Bronchial hyperreactivity; Saline solution, hypertonic; Administration, inhalation;

Idiopathic pulmonary hemosiderosis (IPH), the main cause of pulmonary hemosiderosis in children, is characterized by intermittent alveolar bleeding and hemosiderin-laden macrophages in sputum and in gastric lavage. The treatment is based on corticosteroids and cytotoxic drugs, under special conditions. We describe the case of a 7-year-old girl with IPH who achieved partial clinical remission with high doses of corticosteroids. However, the treatment had to be discontinued because the patient developed Cushing's syndrome. Treatment was started with an azathioprine-corticosteroid combination and then changed to azathioprine alone, which was maintained for four years, with excellent results.

Keywords: Hemosiderosis; Azathioprine; Hemorrhage; Case reports [publication type].

To evaluate the characteristics of users of inhalers and the frequency of inhaler use among adolescents and adults with self-reported physician-diagnosed asthma, bronchitis, or emphysema. Methods: A population-based study conducted in the city of Pelotas, Brazil, involving 3,670 subjects ≥ 10 years of age, evaluated with a questionnaire. Results: Approximately 10% of the sample reported at least one of the respiratory diseases studied. Among those individuals, 59% reported respiratory symptoms in the last year, and, of those, only half reported using inhalers. The use of inhalers differed significantly by socioeconomic status (39% and 61% for the lowest and the highest, respectively, p = 0.01). The frequency of inhaler use did not differ by gender or age. Among the individuals reporting emphysema and inhaler use, the use of the bronchodilator-corticosteroid combination was more common than was that of a bronchodilator alone. Only among the individuals reporting physician-diagnosed asthma and current symptoms was the proportion of inhaler users higher than 50%. Conclusions: In our sample, inhalers were underutilized, and the type of medication used by the individuals who reported emphysema does not seem to be in accordance with the consensus recommendations.

Keywords: Metered dose inhalers; Asthma; Pulmonary disease, chronic obstructive; Bronchitis; Emphysema; Dry powder inhalers.

Objective: To determine the compliance with tuberculosis treatment among patients enrolled the tuberculosis control program in the city of Carapicuíba, Brazil, before and after the implementation of the directly observed treatment, short-course (DOTS) strategy. Methods: A retrospective historical cohort study of operational aspects based on records of attendance and treatment evolution of patients in self-administered treatment (SAT) and of those submitted to DOTS. Monthly treatment outcome tables were created, and the probability of compliance with the treatment was calculated for both groups of patients. Results: A total of 360 patients with tuberculosis met the inclusion criteria: 173 (48.1%) in the SAT group; and 187 (51.9%) in the DOTS group. Treatment compliance was 6.1% higher in the DOTS group than in the SAT group. The proportion of patients completing the six months of treatment was 91.6% and 85.5% in the DOTS group and in the SAT group, respectively. Conclusions: The results of this study show that DOTS can be successfully implemented at primary health care clinics. In this population of patients, residents of a city with low incomes and a high burden of tuberculosis infection, DOTS was more effective than was SAT.

Keywords: Tuberculosis; Mycobacterium tuberculosis; Treatment outcome; Medication adherence.

Background: Massive hemoptysis is a high morbidity and high mortality condition, independently of the treatment administered. A variety of methods are used to control the acute bleeding. The instillation of iced saline solution through a rigid bronchoscope was described in 1980. Objective: To establish the efficacy of repeated instillations of iced saline solution (4oC) using a rigid bronchoscope for the acute control of massive hemoptysis. Method: A group of 94 patients with massive hemoptysis was treated with rigid bronchoscopy and lavage with iced saline solution of the actively bleeding lung. The absence of bleeding recurrence within the following 15 days was considered a therapeutic success. The causes of hemoptysis included: pulmonary tuberculosis: 78 (83%), among which 48 had active disease, and 30 had tuberculosis sequelae, bronchiectasis (6), lung cancer (5), intracavitary aspergilloma (3), and unknown (2). The bleeding site was found in 93 patients (99%). The mean saline volume used in the bronchoscopy was 528 mL, ranging from 160 mL to 2,500 mL. Results: All patients stopped bleeding during the procedure. Fifteen patients were submitted to some kind of procedure (surgery, embolization, or radiation therapy) within 15 days, and the efficacy of lavage could not be assessed. Twenty of the 79 patients followed-up for more than 15 days had recurrence of hemoptysis. Cold saline lavage was repeated once in 13 patients, twice in 6 patients, and 3 times in one patient. Conclusion: The control of tracheobronchial hemorrhage through bronchoscopy and lavage with iced saline is an effective procedure and can be repeated in case of re-bleeding. It is a safe procedure, and allows the definitive treatment to occur in better clinical conditions.

Keywords: Hemoptysis. Tuberculosis. Bronchoscopy.

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.

In the atypical smooth muscle cells that are characteristic of lymphangioleiomyomatosis (LAM), there are estrogen and progesterone receptors. Therefore, anti-hormonal therapy, despite having produced controversial results, can be considered a treatment option. The objective of this retrospective study was to evaluate hormonal and spirometric data for nine women with LAM after one year of treatment with goserelin. The mean increase in FEV1 and FVC was 80 mL and 130 mL, respectively. There was effective blockage of the hormonal axis. It is still not possible to exclude a potential beneficial effect of the use of gonadotropin-releasing hormone analogues in LAM patients, which underscores the need for randomized trials.

Keywords: Lymphangioleiomyomatosis; Spirometry; Goserelin.

Scimitar syndrome is rare, but knowledge about it is important to pediatricians and pneumologists. X-ray can suggest diagnosis, but the interpretation can be wrong. If a diagnosis is made, further anomalies must be investigated with echocardiography and angiography, even if he patient is asymptomatic. This child showed evidence of pulmonary hypertension and required surgery. Postoperative evolution was excellent.

Keywords: Scimitar syndrome. Surgery. Pulmonary disease (specialty). Diagnosis.

Objective: Cystic fibrosis (CF) is a chronic multisystemic hereditary disease for which a multidisciplinary approach must be taken. The objective of this study was to show the evolution of a group of patients with CF after the implementation of multidisciplinary treatment. Methods: A retrospective study involving 19 patients (6-29 years of age) under clinical follow-up treatment at the University of São Paulo at Ribeirão Preto School of Medicine Hospital das Clínicas, located in the city of Ribeirão Preto, Brazil. The patients were divided into two groups: 6-12(6-12 years of age) and 13+ (> 12 years of age). We collected data regarding body mass index (BMI), Zscore, Shwachman score (SS), number of exacerbations/year, chronic colonization by Pseudomonas aeruginosa, and spirometric measurements (FVC, FEV1, FEV1%, and FEF25-75%). Data were collected at two different time points (before and after the implementation of the multidisciplinary treatment) and were analyzed with the Wilcoxon signed rank test. Results: The median age at the onset of symptoms was 10 months. In the 6-12 group, only BMI and FVC increased significantly. Although the other spirometric values increased, the differences were not significant. In the 13+ group, there were no significant differences between the two time points. There was a borderline significant decrease in SS and less than significant decreases in the spirometric measurements. However, the number of patients with alterations in volumes and flows decreased in both groups. Conclusions: Although our patient sample was small, the lack of changes in the spirometric parameters might reflect clinical and functional stability. In all of the patients evaluated, clinical, functional, and nutritional parameters remained stable throughout the study period. The implementation of a multidisciplinary approach might have contributed to this result.

Keywords: Cystic fibrosis; Spirometry; Body mass index; Physical therapy modalities.

Idiopathic pulmonary arterial hypertension (PAH) patients with a positive response to acute vasodilator challenge and a clinical response to calcium channel blockers (CCBs) for at least one year are traditionally designated true responders. Nevertheless, little is known about a sustained response to CCBs over longer periods of time. We evaluated the loss of response to CCBs after long-term treatment in a cohort of idiopathic PAH patients previously classified as being true responders. Our data suggest that idiopathic PAH patients can lose clinical response to CCBs even after one year of clinical stability, reinforcing the need for constant multidimensional reevaluation to assess the need for targeted PAH therapies and to classify these patients correctly.

Keywords: Pulmonary hypertension; Vasodilator agents; Calcium channel blockers.

Objective: To identify factors associated with the minimal clinically important difference (MCID) for health-related quality of life (HRQoL) after physical conditioning in patients with COPD. Methods: Thirty-five patients were submitted to a 12-week program of physical conditioning (strength training plus low-intensity aerobic exercise). Body composition, incremental treadmill test results, endurance treadmill test results, six-minute walk test results, peripheral muscle strength, MIP, baseline dyspnea index (BDI) and Saint George's Respiratory Questionnaire (SGRQ) scores were assessed at baseline and after the program, thus allowing the variations (Δ) to be calculated. The MCID for HRQoL was defined as a reduction of ≥ 4% in the SGRQ total score. Subjects who responded to the program, achieving the MCID for HRQoL, were allocated to the responders (R) group (n = 24), and the remainder were allocated to the non-responders (NR) group (n = 11). Results: The values obtained for the following variables were significantly higher in group R than in group NR (p < 0.05): FEV1 (1.48 ± 0.54 L vs. 1.04 ± 0.34 L); VEF1/FVC (47.9 ± 11.7% vs. 35.5 ± 10.7%); PaO2 (74.1 ± 9.7 mmHg vs. 65.0 ± 8.9 mmHg); and ΔBDI, expressed as median and interquartile range (2.0 [0.0-3.5] vs. 0.0 [0.0-1.0]). The ΔBDI correlated significantly with the ΔSGRQ symptoms domain score, activity domain score and total score (r = 0.44, 0.60 and 0.62, respectively, p < 0.01 for all). After logistic regression, only ΔBDI remained as a predictor of MCID for HRQoL. Conclusions: Achieving the MCID for HRQoL after physical conditioning is associated with dyspnea reduction in COPD patients. Therefore, there is a need to develop treatment strategies designed to interrupt the dyspnea-inactivity-dyspnea cycle in such patients.

Keywords: Pulmonary disease, chronic obstructive; Quality of life; Dyspnea; Exercise; Rehabilitation.

Primary pulmonary hypertension is a rare, progressive disease with high mortality rates. The treatment of primary pulmonary hypertension is still based on high-cost drugs, not yet available in developing countries. Recently, the use of sildenafil as an alternative drug for primary pulmonary hypertension treatment has been reported. It is reported a case of a 21 year-old female patient with primary pulmonary hypertension, who presented an acute worsening of symptoms and decrease of oxygen saturation. The investigation revealed the existence of a patent oval foramen not previously seen, with right-to-left shunt. Sildenafil treatment was instituted in escalating doses. After 40 days of treatment, the echocardiogram showed resolution of the shunt, concomitant to oxygenation improvement. The authors believe that sildenafil is a feasible alternative for primary pulmonary hypertension treatment, although larger clinical trials are necessary to determine its clinical safety and efficacy.

Keywords: Pulmonary hypertension/therapy. Vasodilator agents/administration & dosage.

Objective: To identify the risk factors for pulmonary complications after thoracotomy for the resection of pulmonary nodules in patients with sarcoma. Methods: A retrospective cohort study involving 68 consecutive patients diagnosed with sarcoma and submitted to a total of 174 thoracotomies for the resection of pulmonary nodules. The dependent variable was defined as the occurrence of any postoperative pulmonary complications. The independent variables were related to the patient, underlying diagnosis, and type of surgical procedure. We analyzed the data using a multivariate generalized estimating equations model with logistic link function and a symmetric correlation structure. Results: Complications were observed in 24 patients (13.8%, 95% CI: 9.0-19.8), and there was one death. The mean length of hospital stay was twice as long in the patients with postoperative complications as in those without (18.8 ± 10.0 days vs. 8.6 ± 6.0 days; p < 0.05). The variables that correlated with the outcome measure were the type of resection (wedge vs. anatomic; OR = 3.6; 95% CI: 1.5-8.8), the need for blood transfusion (OR = 9.8; 95% CI: 1.6-60.1), and the number of nodules resected (OR = 1.1; 95% CI: 1.0-1.1). The multivariate model showed an area under the ROC curve of 0.75 (95% CI: 0.65-0.85). Conclusions: Postoperative pulmonary complications were common after pulmonary nodule resection in patients with sarcoma, occurring in approximately 10% of the procedures. The occurrence of such complications can be expected when techniques other than wedge resection are employed, when blood transfusion is required, and when a great number of nodules are resected. Therefore, it is possible to identify patients at risk for pulmonary complications, who should be closely monitored in the immediate postoperative period. In such patients, all preventive measures should be taken.

Keywords: Sarcoma; Neoplasm metastasis; Risk; Thoracotomy; Thoracic surgery.

Objective: To analyze factors predictive of noncompliance with pulmonary tuberculosis treatment. Methods: A historical cohort study involving 481 of the 529 active tuberculosis patients registered with the Tuberculosis Control Program in the city of Cuiabá, located in the state of Mato Grosso, during the 1998-2000 period. Data were obtained by analyzing medical charts and registration records. In the bivariate analysis, the chi-square test was used to calculate noncompliance rate ratios (relative risk), 95% confidence intervals were determined, and Fisher's exact test was used. The choice to estimate the rate of incidence was the method of density and a multivariate logistic regression model was constructed in order to identify the variables that were most predictive of noncompliance, using a level of statistical significance of p < 0.05. Results: The global rate of incidence was 27.3%, equivalent to 5.1 noncompliant patients/100 patients/month, increasing in the second and third months of treatment. In the final logistic regression model, the following were considered predictors of noncompliance: unsupervised treatment (odds ratio: 2.58; 95% confidence interval: 1.64 - 4.06; p < 0.001); having been treated during the 1998-1999 period (odds ratio: 1.43; 95% confidence interval: 1.14 - 1.80; p = 0.002); being male (odds ratio: 1.39; 95% confidence interval: 1.10 - 1.76; p = 0.005) and having been out of compliance with previous treatment regimes (odds ratio: 1.37; 95% confidence interval: 1.06 - 1.78; p < 0.017). Conclusion: The results indicate an elevated incidence of noncompliance and show that unsupervised treatment, year in which treatment was received, male gender and prior noncompliance were predictors of future noncompliance.

Keywords: Tuberculosis, pulmonary/therapy; Treatment refusal; Cohort studies

Objective: To analyze the association between clinical/epidemiological characteristics and outcomes of tuberculosis treatment in patients with concomitant tuberculosis and chronic kidney disease (CKD) in Brazil. Methods: We used the Brazilian Ministry of Health National Case Registry Database to identify patients with tuberculosis and CKD, treated between 2007 and 2011. The tuberculosis treatment outcomes were compared with epidemiological and clinical characteristics of the subjects using a hierarchical multinomial logistic regression model, in which cure was the reference outcome. Results: The prevalence of CKD among patients with tuberculosis was 0.4% (95% CI: 0.37-0.42%). The sample comprised 1,077 subjects. The outcomes were cure, in 58%; treatment abandonment, in 7%; death from tuberculosis, in 13%; and death from other causes, in 22%. The characteristics that differentiated the ORs for treatment abandonment or death were age; alcoholism; AIDS; previous noncompliance with treatment; transfer to another facility; suspected tuberculosis on chest X-ray; positive results in the first smear microscopy; and indications for/use of directly observed treatment, short-course strategy. Conclusions: Our data indicate the importance of sociodemographic characteristics for the diagnosis of tuberculosis in patients with CKD and underscore the need for tuberculosis control strategies targeting patients with chronic noncommunicable diseases, such as CKD.

Keywords: Tuberculosis/epidemiology; Renal insufficiency, chronic/epidemiology; Risk factors; Logistic models.

Objective: Lymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day). Methods: We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus. Results: After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. Conclusions: Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols.

Keywords: Neoplasms; Lymphangioleiomyomatosis/therapy; TOR serine-threonine kinases; Sirolimus.