Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease in which most patients die of respiratory failure. Although volume-targeted non-invasive bilevel positive airway pressure (BPAP) ventilation has been studied in patients with chronic respiratory failure of various etiologies, its use in ALS has not been reported. We present the case of a 66-year-old woman with ALS and respiratory failure treated with volume-targeted BPAP ventilation for 15 weeks. Weekly data downloads showed that disease progression was associated with increased respiratory muscle weakness, decreased spontaneous breathing, and increased use of non-invasive positive pressure ventilation, whereas tidal volume and minute ventilation remained relatively constant.

Keywords: Amyotrophic lateral sclerosis; Respiratory insufficiency; Hypoventilation; Intermittent positive-pressure ventilation; Sleep.

Objective: To study the impact that heated humidification instituted in the beginning of automatic positive airway pressure (APAP) therapy has on compliance with and the side effects of the treatment. Methods: Thirty-nine treatment-naïve patients with obstructive sleep apnea were randomized into two groups to receive APAP using one of two modalities: with heated humidification (APAPwith group); and without heated humidification (APAPw/o group).Patients were evaluated at 7 and 30 days after APAP initiation. The following parameters were analyzed: compliance with treatment (mean number of hours/night); side effects (dry nose or mouth, nasal obstruction and rhinorrhea); daytime sleepiness (Epworth sleepiness scale score) and subjective comfort (visual analog scale score). Patients were also evaluated in terms of residual apnea-hypopnea index (AHI), as well as mean pressures and leaks registered in the ventilators. Results: There were no differences between the two groups in terms of mean age (APAPwith: 57.4 ± 9.2; APAPw/o: 56.5 ± 10.7 years), AHI (APAPwith: 28.1 ± 14.0; APAPw/o: 28.8 ± 20.5 events/hour of sleep), baseline Epworth score (APAPwith: 11.2 ± 5.8; APAPw/o: 11.9 ± 6.3) and initial nasal symptoms. Compliance was similar in both groups (APAPwith: 5.3 ± 2.4; APAPw/o: 5.2 ± 2.3 h/night). There were no differences in any of the other parameters analyzed. Conclusions: The introduction of heated humidification at the beginning of APAP therapy provided no advantage in terms of treatment compliance or side effects of treatment.

Keywords: Humidity; Positive-pressure respiration/adverse effects; Patient compliance.

Recent studies suggest that non-invasive positive pressure ventilation (NPPV) improves clinical signs and gas exchange, apart from avoiding the need for endotracheal intubation in selected patients with acute respiratory failure. In this prospective, open trial in the Intensive Care Unit (Hospital Universitário da Universidade Federal de Juiz de Fora), the authors evaluated the efficacy and security of using NPPV in patients with acute respiratory failure. Twenty-five patients with clinical or gasometric diagnosis of acute respiratory failure (respiratory rate > 25 breaths/min, accessory muscle use, PaO2 < 60 mmHg, PaCO2 > 50 mmHg after supplemental oxygen) received NPPV. Fifteen patients (60%) were successfully ventilated with NPPV, avoiding the tracheal intubation. After two hours with NPPV, the respiratory rate was reduced (from 36 ± 2 breaths/min to 26 ± 1 breaths/min, p < 0.01) and the PaO2 increased (from 76 ± 6 mmHg to 100 ± 12 mmHg, p < 0.05). Among the patients with hypercapnia, after two hours, the PaCO2 decreased (from 60 ± 2 mmHg to 49 ± 3 mmHg, p < 0.05). Four patients (16%) presented complications with facial skin necrosis, but only one had the NPPV stopped. Among the patients who did not respond to the method, three did not adapt to the NPPV and seven had it interrupted because of clinical deterioration. The authors conclude that NPPV is a safe option that can be used to treat selected patients with acute respiratory failure.

Background: Nasal and pharyngeal symptoms are common in patients with obstructive sleep apnoea (OSA) treated with nasal continuous positive airway pressure (CPAP). However, these symptoms are common in OSA patients even before the treatment. Objectives: Determine the impact of nasal CPAP on nasal and pharyngeal symptoms in OSA patients. Method: Thirty-five adult patients (28 males), age 54±10 years old, with OSA diagnosed by polissonography. All patients answered to a questionnaire about the presence and quantification of sneezing, rhinorrhea, nasal pruritus, obstruction and bleeding, nasal and pharyngeal dryness. The questionnaire was answered before and after at least three months of CPAP therapy. Results: The apnea-hypopnea index was 50±25 events per hour. Twenty six patients (74%) presented at least one naso-pharingeal symptom before treatment. Nasal obstruction was the most common symptom, being referred by 18 patients (51%). Among the patients that were initially assymptomatic (n=9), 78% developed adverse nasal reactions to CPAP. In contrast, among the patients that presented nasal symptoms before treatment, there was a significant reduction in nasal obstruction, nasal and throat dryness scores as well as nasal bleeding after CPAP therapy. Conclusions: Nasal na pharyngeal symptoms are frequent in OSAS patients. CPAP therapy may originate nasal symptoms in patients previously assymptomatic, as well as reduce the intensity of these symptoms in patients that are previously symptomatic.

Keywords: Obstructive sleep apnea syndrome. Nasal obstruction. Continuous positive airway pressure, compliance.

Objective: To evaluate the effects of non-invasive positive pressure ventilation (NIPPV) on acute respiratory failure and to identify the factors associated with its failure or success. Patients and methods: This open and prospective study analyzed 60 episodes of NIPPV use in 53 patients with respiratory failure. Results: In 37 occasions (62%), the patients were successfully ventilated with NIPPV (Success Group, SG) whereas in 23 episodes (38%) intubation was necessary (Failure Group, FG). FG patients showed a higher Apache II score (30.4 ± 9 versus 22.2 ± 8, p = 0.001). The use of NIPPV reduced respiratory rate after 2 hs. This reduction was less evident in FG (from 33 ± 9 to 30 ± 8 bpm, p = 0.094) than in SG (from 39 ± 11 to 28 ± 9 bpm, p < 0.001). PaO2 increased from 62 ± 22 to 101 ± 65 mmHg, (p < 0.001) with no differences between groups. Among patients with hypercapnia, PaCO2 level decreased in SG (from 76 ± 20 to 68 ± 21mmHg, p = 0.032) but not in FG (from 89 ± 23 to 93 ± 40mmHg, p = 0.54). Arterial pH increased in SG (from 7.25 ± 0.10 to 7.34 ± 0.11, p = 0.007) and not in FG (from 7.24 ± 0.07 to 7.21 ± 0.12, p = 0.48). NIPPV was used for longer in SG (3.4 ± 2.5 versus 2.3 ± 2 days, p = 0.003). Higher IPAP levels were used in SG (13.2 ± 3 versus 11 ± 4cmH2O, p = 0.02). The 10 patients (17%) who died, all belonged to FG. Skin lesions at the site of mask contact was the most common complication of NIPPV (5, 8%). NIPPV was efficient in treating acute respiratory failure in 2/3 of patients. The factors associated with failure were: greater severity of the illness, smaller reduction in the respiratory rate, no improvement of respiratory acidosis in 2 h and use of lower IPAP levels. The high mortality rate observed in FG (10, 43%) justifies efforts to optimize NIPPV implementation and to recognize its failure, as early as possible, in order to avoid delays in endotracheal intubation.

Keywords: Non-invasive positive pressure ventilation. Respiratory failure. Apache II. Hypercapnia.

Objective: To analyze the effects of the continuous positive airway pressure (CPAP), with 30% of FiO2 and in compressed air, used during the performance of submaximal physical activity on an ergometric treadmill. Materials and methods: Nine male individuals with COPD were submitted to physical activity on the ergometric treadmill using CPAP with 30% FiO2 .The authors evaluated the behavior of the spirometric parameters, the forced vital capacity (FVC), the forced expiratory volume in the first second (FEV1) before and after physical activity, the respiratory frequency (f), the arterial oxygen saturation (SaO2), the cardiac frequency (cf), the systolic arterial pressure (SAP), and the diastolic arterial pressure (DAP) at rest and during physical activity. Results and conclusion: Results have shown that, when using CPAP in both situations analyzed, there has been an increase in the FVC and a decrease in f and in cf with statistically significant difference (α = 0.05). SaO2 has showned a significantly higher mean when CPAP was used with 30% of FiO2 (α = 0.05). The FEV1, the SAP and DAP have maintained the same value in the statistical analyses.

Keywords: Exercise test. Forced expiratory volume. Vital capacity.

Objective: To determine whether different levels of CPAP improve the lung volumes and capacities of healthy subjects immersed in water. Methods: This was a randomized clinical trial, conducted between April and June of 2016, involving healthy female volunteers who were using oral contraceptives. Three 20-min immersion protocols were applied: control (no CPAP); CPAP5 (CPAP at 5 cmH2O); and CPAP10 (CPAP at 10 cmH2O). We evaluated HR, SpO2, FVC, FEV1, the FEV1/FVC ratio, peak expiratory flow rate (PEFR), and FEF25-75%) at three time points: pre-immersion; 10 min after immersion; and 10 min after the end of each protocol. Results: We evaluated 13 healthy volunteers. The CPAP10 protocol reversed the restrictive pattern of lung function induced by immersion in water, maintaining pulmonary volumes and capacities for a longer period than did the CPAP5 protocol. Conclusions: When the hemodynamic change causing a persistent lung disorder, only the application of higher positive pressures is effective in maintaining long-term improvements in the pulmonary profile.

Keywords: Physical therapy modalities; Noninvasive ventilation; Continuous positive airway pressure.

Objective: To evaluate the construct validity and reproducibility of the six-minute step test (6MST) in individuals with obstructive sleep apnea (OSA) treated with continuous positive airway pressure (CPAP). Methods: We evaluated 48 volunteers diagnosed with OSA and treated with CPAP for at least two months. The volunteers underwent the six-minute walk test (6MWT) and the 6MST, in random order and on different days, with an interval of, at most, seven days between the two tests. Results: A moderate positive correlation was found between the distance walked on the 6MWT and the number of steps climbed on the 6MST (r = 0.520; p < 0.001). There was no significant difference between the two 6MSTs in terms of the number of steps climbed (121.7 ± 27.1 vs. 123.6 ± 26.7). Reproducibility for performance on the 6MST and for cardiovascular variables was considered excellent (intraclass correlation coefficient > 0.8). Regarding cardiovascular responses, the 6MST produced higher values than did the 6MWT for HR at six minutes, percent predicted maximum HR, and leg fatigue at six minutes, as well as for systolic blood pressure at six minutes and at one minute of recovery. Conclusions: The 6MST is valid and reproducible, producing greater cardiovascular stress than does the 6MWT. However, the 6MST is also characterized as a submaximal test for the assessment of exercise tolerance in individuals with OSA treated with CPAP.

Keywords: Sleep apnea, obstructive; Reproducibility of results; Exercise test; Exercise tolerance.

Objective: To evaluate systematically the effects of continuous positive airway pressure (CPAP) on blood pressure in patients with resistant hypertension and obstructive sleep apnea (OSA). Methods: The Cochrane Library, PubMed, ScienceDirect, and the Web of Science were searched for studies investigating the effects of CPAP on blood pressure in patients with resistant hypertension and OSA. The selected studies underwent quality assessment and meta-analysis, as well as being tested for heterogeneity. Results: Six randomized controlled trials were included in the meta-analysis. The pooled estimates of the changes in mean systolic blood pressure and mean diastolic blood pressure (as assessed by 24-h ambulatory blood pressure monitoring) were −5.40 mmHg (95% CI: −9.17 to −1.64; p = 0.001; I2 = 74%) and −3.86 mmHg (95% CI: −6.41 to −1.30; p = 0.00001; I2 = 79%), respectively. Conclusions: CPAP therapy can significantly reduce blood pressure in patients with resistant hypertension and OSA.

Keywords: Continuous positive airway pressure; Sleep apnea, obstructive; Hypertension; Meta-analysis.

Objective: To evaluate the electromyographic activity (EA) of sternocleidomastoid (SCM) and scalene muscles during and after the use of expiratory positive airway pressure (EPAP) in patients with COPD. Methods: This was a clinical single-blind trial involving 13 healthy subjects as controls and 12 patients with stable COPD. At baseline, we determined EA during spontaneous respiration, lung function parameters, and respiratory muscle strength. Subsequently, EPAP at 15 cmH2O was applied by means of a face mask for 25 min, during which the EA of the SCM and scalene muscles was recorded every 5 min. A final record was obtained 10 min after the mask removal. Results: We found that the behavior of the EA of SCM and scalene muscles was comparable between the controls and the COPD patients (p = 0.716 and p = 0.789, respectively). However, during the use of EPAP, both muscles showed a trend toward an increase in the EA. In addition, there was a significant decrease in the EA of the SCM between the baseline and final measurements (p = 0.034). Conclusions: The use of EPAP promoted a significant reduction in the EA of the SCM in the controls and in the patients with stable COPD. However, this did not occur regarding the EA of the scalene muscle.

Keywords: Electromyography; Respiratory muscles; Pulmonary disease, chronic obstructive; Positive-pressure respiration.

Objective: To investigate the effects of manual chest compression (MCC) on the expiratory flow bias during the positive end-expiratory pressure-zero end-expiratory pressure (PEEP-ZEEP) airway clearance maneuver applied in patients on mechanical ventilation. The flow bias, which influences pulmonary secretion removal, is evaluated by the ratio and difference between the peak expiratory flow (PEF) and the peak inspiratory flow (PIF). Methods: This was a crossover randomized study involving 10 patients. The PEEP-ZEEP maneuver was applied at four time points, one without MCC and the other three with MCC, which were performed by three different respiratory therapists. Respiratory mechanics data were obtained with a specific monitor. Results: The PEEP-ZEEP maneuver without MCC was enough to exceed the threshold that is considered necessary to move secretion toward the glottis (PEF − PIF difference > 33 L/min): a mean PEF − PIF difference of 49.1 ± 9.4 L/min was achieved. The mean PEF/PIF ratio achieved was 3.3 ± 0.7. Using MCC with PEEP-ZEEP increased the mean PEF − PIF difference by 6.7 ± 3.4 L/min. We found a moderate correlation between respiratory therapist hand grip strength and the flow bias generated with MCC. No adverse hemodynamic or respiratory effects were found. Conclusions: The PEEP-ZEEP maneuver, without MCC, resulted in an expiratory flow bias superior to that necessary to facilitate pulmonary secretion removal. Combining MCC with the PEEP-ZEEP maneuver increased the expiratory flow bias, which increases the potential of the maneuver to remove secretions.

Keywords: Physical therapy modalities; Critical care; Respiration, artificial; Bodily secretions.

Objective: To determine whether the use of a gel pillow with side cutouts designed to accommodate a continuous positive airway pressure (CPAP) mask and reduce head temperature improves the efficacy of and adherence to auto-CPAP therapy. Methods: Twenty-three consecutive CPAP-naïve patients with obstructive sleep apnea were enrolled in the study. Patients were given an auto-CPAP machine with an appropriate CPAP mask and were instructed to use CPAP for 15 nights. They were instructed to sleep with their own pillow (the control pillow) from nights 1 to 5 and with either a foam pillow or a gel pillow, both of which had side cutouts, for 5 consecutive nights each, in random order. After night 15, auto-CPAP machine data were downloaded and patients rated their satisfaction with each pillow on a visual analog scale. Results: Twenty-two patients completed the protocol. The pressures administered, residual apnea-hypopnea index, air leaks, and mean duration of CPAP use did not differ among the periods during which each pillow was used. Patients were significantly more satisfied with the gel pillow than with the control pillow and the foam pillow (p = 0.022 and p = 0.004, respectively), their level of satisfaction with the gel pillow correlating significantly with excessive daytime sleepiness (r2 = 0.19; p = 0.0443). Conclusions: Among obstructive sleep apnea patients treated with nasal CPAP, the use of a gel pillow with side cutouts appears to have no impact on treatment effectiveness. Nevertheless, such patients seem to prefer a gel pillow over other types of pillows.

Keywords: Sleep; Continuous positive airway pressure; Sleep apnea, obstructive; Masks.

Objective: Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement. Methods: A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts. Results: We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 ± 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8%, as was the five-year survival rate. Conclusions: Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.

Keywords: Behcet Syndrome; Lung diseases, interstitial; Pulmonary circulation; Hypertension, pulmonary; Pulmonary embolism; Alveolar.

Continuous positive airway pressure (CPAP) is the gold standard for the treatment of obstructive sleep apnea (OSA). Although CPAP was originally applied with a nasal mask, various interfaces are currently available. This study reviews theoretical concepts and questions the premise that all types of interfaces produce similar results. We revised the evidence in the literature about the impact that the type of CPAP interface has on the effectiveness of and adherence to OSA treatment. We searched the PubMed database using the search terms "CPAP", "mask", and "obstructive sleep apnea". Although we identified 91 studies, only 12 described the impact of the type of CPAP interface on treatment effectiveness (n = 6) or adherence (n = 6). Despite conflicting results, we found no consistent evidence that nasal pillows and oral masks alter OSA treatment effectiveness or adherence. In contrast, most studies showed that oronasal masks are less effective and are more often associated with lower adherence and higher CPAP abandonment than are nasal masks. We concluded that oronasal masks can compromise CPAP OSA treatment adherence and effectiveness. Further studies are needed in order to understand the exact mechanisms involved in this effect.

Keywords: Sleep apnea, obstructive; Continuous positive airway pressure; Masks.

Objective: To determine the efficacy of chest compression accompanied by a 10-cmH2O increase in baseline inspiratory pressure on pressure support ventilation, in comparison with that of aspiration alone, in removing secretions, normalizing hemodynamics, and improving respiratory mechanics in patients on mechanical ventilation. Methods: This was a randomized crossover clinical trial involving patients on mechanical ventilation for more than 48 h in the ICU of the Porto Alegre Hospital de Clínicas, in the city of Porto Alegre, Brazil. Patients were randomized to receive aspiration alone (control group) or compression accompanied by a 10-cmH2O increase in baseline inspiratory pressure on pressure support ventilation (intervention group). We measured hemodynamic parameters, respiratory mechanics parameters, and the amount of secretions collected. Results: We included 34 patients. The mean age was 64.2  14.6 years. In comparison with the control group, the intervention group showed a higher median amount of secretions collected (1.9 g vs. 2.3 g; p = 0.004), a greater increase in mean expiratory tidal volume (16  69 mL vs. 56  69 mL; p = 0.018), and a greater increase in mean dynamic compliance (0.1  4.9 cmH2O vs. 2.8  4.5 cmH2O; p = 0.005). Conclusions: In this sample, chest compression accompanied by an increase in pressure support significantly increased the amount of secretions removed, the expiratory tidal volume, and dynamic compliance. (ClinicalTrials.gov Identifier:NCT01155648 [http://www.clinicaltrials.gov/])

Keywords: Physical therapy modalities; Respiration, Artificial; Intensive care units; Respiratory therapy.

Objective: To determine the effectiveness of oscillating positive expiratory pressure (OPEP) using predetermined expiratory pressures on the viscosity and transportability of sputum in patients with bronchiectasis. Methods: The study involved 15 stable patients with bronchiectasis (7 males; mean age = 53 ± 16 years), submitted to two consecutive OPEP interventions, with a 24-h interval between the two, using positive expiratory pressures set at 15 cmH2O (P15) and 25 cmH2O (P25). The protocol consisted of a voluntary cough; another voluntary cough 20 min later, designated time zero (T0); a 10-min rest period; and two 10-min series (S1 and S2, using OPEP at P15 and P25 in both), with a 10-min interval between the two. The viscosity and transportability of sputum were evaluated by viscometry, relative transport velocity on frog palate, transport in a simulated cough machine and contact angle. Sputum samples were collected at T0, after S1 and after S2. Specific statistical tests were performed depending on the type of data distribution. Results: In comparison with the values obtained at T0, sputum viscosity decreased significantly after S1 at P15 and after S2 at P25. There were no significant differences among all of the samples in terms of transportability. Conclusions: The fact that sputum viscosity decreased whether OPEP was performed at P15 or at P25 suggests that there is no need to generate high expiratory pressure to achieve the desired result.

Keywords: Viscosity; Bronchiectasis; Physical therapy (specialty); Sputum.

In 1993 the first Brazilian cluster of Hantavirus Cardiopulmonary Syndrome (HCPS) was described in Juquitiba, SP. Since then, there have been descriptions of new cases specially on the southeast and south states of Brazil. Only in 2002 there were observed the first three cases of HCPS in our city: São Carlos. One of our patients was successfully supported with CPAP through facial mask. This is the first severe case of acute respiratory failure induced by Hantavirus that was successfully managed with this kind of respiratory strategy.

Keywords: Noninvasive Ventilation, CPAP, Hantavirus Pulmonary and Cardiovascular Syndrome, Acute Hipoxemic Respiratory Failure.

Objective: To evaluate tuberculosis diagnosis with the use of the lateral flow urine lipoarabinomannan assay (LF-LAM) in addition to the Xpert® MTB/RIF Ultra assay on sputum in HIV-positive inpatients and outpatients. Methods: This was a prospective cross-sectional study including HIV-positive patients = 18 years of age with an indication for LF-LAM in accordance with Brazilian National Ministry of Health criteria. Results: A total of 140 patients were included in the study. LF-LAM was positive in 23 (16.4%). An additional 12 (8.6%) were diagnosed with the aid of LF-LAM, this increase in tuberculosis detection being statistically significant. LF-LAM-positive patients were younger and had lower CD4 counts in comparison with LF-LAM-negative patients. Smoking was more common among LF-LAM-negative patients than among LF-LAM-positive patients. Conclusions: The use of LF-LAM significantly increases the detection of tuberculosis in HIV-positive patients, mostly in those who are hospitalized. These findings highlight the utility of LF-LAM, especially in regions with high tuberculosis and HIV infection incidence.

Keywords: Tuberculosis; Mycobacterium tuberculosis; HIV infections; Diagnosis.