Objective: Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy in children, and children with DMD die prematurely because of respiratory failure. We sought to determine the efficacy and safety of yoga breathing exercises, as well as the effects of those exercises on respiratory function, in such children. Methods: This was a prospective open-label study of patients with a confirmed diagnosis of DMD, recruited from among those followed at the neurology outpatient clinic of a university hospital in the city of São Paulo, Brazil. Participants were taught how to perform hatha yoga breathing exercises and were instructed to perform the exercises three times a day for 10 months. Results: Of the 76 patients who entered the study, 35 dropped out and 15 were unable to perform the breathing exercises, 26 having therefore completed the study (mean age, 9.5  2.3 years; body mass index, 18.2  3.8 kg/m2). The yoga breathing exercises resulted in a significant increase in FVC (% of predicted: 82.3  18.6% at baseline vs. 90.3  22.5% at 10 months later; p = 0.02) and FEV1 (% of predicted: 83.8  16.6% at baseline vs. 90.1  17.4% at 10 months later; p = 0.04). Conclusions: Yoga breathing exercises can improve pulmonary function in patients with DMD.

Keywords: Respiratory therapy; Forced expiratory volume; Vital capacity; Muscular dystrophy, Duchenne; Complementary therapies.

Objective: Respiratory complications are the main causes of morbidity and mortality in patients with neuromuscular disease (NMD). The objectives of this study were to determine the effects that routine daily home air-stacking maneuvers have on pulmonary function in patients with spinal muscular atrophy (SMA) and in patients with congenital muscular dystrophy (CMD), as well as to identify associations between spinal deformities and the effects of the maneuvers. Methods: Eighteen NMD patients (ten with CMD and eight with SMA) were submitted to routine daily air-stacking maneuvers at home with manual resuscitators for four to six months, undergoing pulmonary function tests before and after that period. The pulmonary function tests included measurements of FVC; PEF; maximum insufflation capacity (MIC); and assisted and unassisted peak cough flow (APCF and UPCF, respectively) with insufflations. Results: After the use of home air-stacking maneuvers, there were improvements in the APCF and UPCF. In the patients without scoliosis, there was also a significant increase in FVC. When comparing patients with and without scoliosis, the increases in APCF and UPCF were more pronounced in those without scoliosis. Conclusions: Routine daily air-stacking maneuvers with a manual resuscitator appear to increase UPCF and APCF in patients with NMD, especially in those without scoliosis.

Keywords: Neuromuscular diseases; Cough; Respiratory function tests; Respiratory therapy.

Objective: To evaluate cough efficiency using two manually-assisted cough techniques. Methods: We selected 28 patients with Duchenne muscular dystrophy. The patients were receiving noninvasive nocturnal ventilatory support and presented FVC values < 60% of predicted. Peak cough flow (PCF) was measured, with the patient seated, at four time points: at baseline, during a spontaneous maximal expiratory effort (MEE); during an MEE while receiving chest compression; during an MEE after air stacking with a manual resuscitation bag; and during an MEE with air stacking and compression (combined technique). The last three measurements were conducted in random order. The results were compared using Pearson's correlation test and ANOVA with repeated measures, followed by Tukey's post-hoc test (p < 0.05). Results: The mean age of the patients was 20 ± 4 years, and the mean FVC was 29 ± 12%. Mean PCF at baseline, with chest compression, after air stacking and with the use of the combined technique was 171 ± 67, 231 ± 81, 225 ± 80, and 292 ± 86 L/min, respectively. The results obtained with the use of the combined technique were significantly better than were those obtained with the use of either technique alone (F[3.69] = 67.07; p < 0.001). Conclusions: Both chest compression and air stacking techniques were efficient in increasing PCF. However, the combination of these two techniques had a significant additional effect (p < 0.0001).

Keywords: Muscular dystrophy, Duchenne; Intermittent positive-pressure ventilation; Cough; Insufflation;Peak expiratory flow rate.

Objective: To analyze the effects of Pilates mat exercises in patients with cystic fibrosis (CF). Methods: This was a clinical trial involving 19 CF patients recruited from either the CF Outpatient Clinic of the State University at Campinas Hospital de Clínicas or the Children's Institute of the University of São Paulo School of Medicine Hospital das Clínicas. All of the patients performed Pilates mat exercises for four months (one 60-min session per week). The variables studied (before and after the intervention) were respiratory muscle strength, MIP, MEP, FVC, and FEV1. Results: After the intervention, MIP was significantly higher in the male patients (p = 0.017), as were MIP and MEP in the female patients (p = 0.005 and p = 0.007, respectively). There were no significant differences between the pre- and post-intervention values of FVC or FEV1, neither in the sample as a whole nor among the patients of either gender. Conclusions: Our results show that Pilates mat exercises have beneficial effects on respiratory muscle strength in CF patients. (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR-86vp8x [http://www.ensaiosclinicos.gov.br])

Keywords: Cystic fibrosis; Muscle strength; Exercise movement techniques; Respiratory function tests.

Objective: To evaluate mouthpiece ventilation (MPV) in patients with Duchenne muscular dystrophy (DMD) who are noncompliant with noninvasive ventilation (NIV). Methods: We evaluated four young patients with DMD who had previously refused to undergo NIV. Each patient was reassessed and encouraged to try MPV. Results: The four patients tolerated MPV well and were compliant with NIV at home. MPV proved to be preferable and more comfortable than NIV with any other type of interface. Two of the patients required overnight NIV and eventually agreed to use a nasal mask during the night. Conclusions: The advantages of MPV over other types of NIV include fewer speech problems, better appearance, and less impact on the patient, eliminating the risk of skin breakdown, gastric distension, conjunctivitis, and claustrophobia. The use of a mouthpiece interface should be always considered in patients with DMD who need to start NIV, in order to promote a positive approach and a rapid acceptance of NIV. Using MPV during the daytime makes patients feel safe and more likely to use NIV at night. In addition, MPV increases treatment compliance for those who refuse to use other types of interfaces.

Keywords: Muscular dystrophy, Duchenne; Noninvasive ventilation; Patient compliance.

Objective: To evaluate breathing pattern and thoracoabdominal motion during breathing exercises. Methods: Twenty-four patients with class II or III obesity (18 women; 6 men) were studied on the second postoperative day after gastroplasty. The mean age was 37 ± 11 years, and the mean BMI was 44 ± 3 kg/m2. Diaphragmatic breathing, incentive spirometry with a flow-oriented device and incentive spirometry with a volume-oriented device were performed in random order. Respiratory inductive plethysmography was used in order to measure respiratory variables and thoracoabdominal motion. Results: Comparisons among the three exercises showed significant differences: tidal volume was higher during incentive spirometry (with the flow-oriented device or with the volume-oriented device) than during diaphragmatic breathing; the respiratory rate was lower during incentive spirometry with the volume-oriented device than during incentive spirometry with the flow-oriented device; and minute ventilation was higher during incentive spirometry (with the flow-oriented device or with the volume-oriented device) than during diaphragmatic breathing. Rib cage motion did not vary during breathing exercises, although there was an increase in thoracoabdominal asynchrony, especially during incentive spirometry with the flow-oriented device. Conclusions: Among the breathing exercises evaluated, incentive spirometry with the volume-oriented device provided the best results, because it allowed slower, deeper inhalation.

Keywords: Breathing exercises; Physical therapy (Specialty); Bariatric surgery; Obesity, morbid.

Background: Chronic obstructive pulmonary disease is detrimental to lung mechanics and peripheral muscles. The physical programs developed for this condition are usually targeted to an improvement on aerobics capacity. Programs that approach specifically the changes in thoracic mobility and thoracic muscles are rare. Objective: To assess the effects of a physical exercise program designed to increase chest wall mobility on functional and psychosocial capacity in moderate to severe chronic obstructive pulmonary disease patients. Methods: Thirty patients with moderate to severe chronic obstructive pulmonary disease were studied. They were randomized to 2 groups: control group (CG) and treated group (TG). The CG was submitted to an educational program and the TG was submitted to an educational program plus a physical exercise program aiming to increase chest wall mobility. Variables included spirometry, thoracic mobility, quality of life, anxiety and depression levels and a six minute walk test (6MWT). Results: After 2 months of training, only the TG presented improvements on chest wall mobility (from 4.20 ± 0.58 cm to 5.27 ± 0.58 cm; p = 0.05) and 6MWT (from 469.73 ± 31.99 m to 500.60 ± 27.38 m; p = 0.01). It was also observed that the TG presented improvement on the St. George's Respiratory Questionnaire (SGRQ) score, Chronic Respiratory Questionnaire (CRQ), and Beck's depression scale after 2 months of treatment. Pulmonary function did not improve either in the CG or in the TG group. Conclusion: Our results suggest that exercises aimed to the increasing of chest wall mobility improve thoracic mobility, quality of life, submaximal exercise capacity, and reduce dyspnea and depression symptoms in chronic obstructive pulmonary disease patients.

Keywords: COPD. Thoracic mobility. Quality of life. Capacity of exercise. Dyspnea.

Objective: To compare lung function between adolescents with and without substance use disorder (SUD). Methods: This was an observational, cross-sectional exploratory study. The sample consisted of 16 adolescents with SUD and 24 age-matched healthy controls. The adolescents in the clinical group were recruited from a psychiatric inpatient unit for detoxification and rehabilitation; their primary diagnosis was SUD related to marijuana, cocaine, or polysubstance use. Questionnaires and pulmonary function tests were applied for clinical evaluation. Results: We found that FVC, FEV1, and their percentages of the predicted values were significantly lower in the adolescents with SUD than in those without. Those differences remained significant after adjustment for BMI and the effects of high levels of physical activity. The largest effect size (Cohen's d = 1.82) was found for FVC as a percentage of the predicted value (FVC%), which was, on average, 17.95% lower in the SUD group. In addition, the years of regular use of smoked substances (tobacco, marijuana, and crack cocaine) correlated negatively with the FVC%. Conclusions: This exploratory study is innovative in that it demonstrates the early consequences of smoked substance use for the lung health of adolescents with SUD.

Keywords: Adolescent; Substance-related disorders; Lung/physiopathology; Respiratory tract diseases/etiology; Cocaine; Cannabis.

Objective: To evaluate the effects of an outpatient education program in patients with uncontrolled asthma. Methods: This was an uncontrolled study evaluating an educational intervention and involving patients with uncontrolled asthma ≥ 14 years of age. The participants completed a questionnaire designed to assess the level of asthma control, the inhalation technique, and quality of life. All of the patients underwent pulmonary function testing, after which they participated in an education program consisting of one 45-min face-to-face session, followed by phone interviews at two, four, and eight weeks. The participants were reevaluated after three months. Results: Sixty-three patients completed the study. There was a significant improvement in the level of asthma control (p < 0.001). Of the 63 patients, 28 (44.4%) and 6 (9.5%) were classified as having partially controlled asthma and controlled asthma, respectively. The mean FEV1 was 63.0 ± 20.0% and 68.5 ± 21.2% of the predicted value prior to and after the educational intervention, respectively (p = 0.002), and all of the quality of life scores improved (p < 0.05 for all). The same was true for the proportion of patients prior to and after the educational intervention using the proper inhalation technique when using metered dose inhalers (15.4% vs. 46.2%; p = 0.02) and dry powder inhalers (21.3% vs. 76.6%; p < 0.001). The logistic regression analysis revealed that an incorrect inhalation technique identified during the first evaluation was independently associated with a favorable response to the educational intervention. Conclusions: This study suggests that an outpatient education program for asthma patients improves the level of asthma control, lung function parameters, and quality of life. An incorrect inhalation technique identified during the first evaluation was predictive of a favorable response to the educational intervention.

Keywords: Asthma/prevention and control; Quality of life; Respiratory function tests; Ambulatory care; Health education.

Objective: To investigate the extent to which exercise is associated with symptoms in patients with severe obstructive sleep apnea (OSA). Methods: We included subjects with an apnea-hypopnea index (AHI) > 30 events/h who completed validated sleep and exercise questionnaires. We compared symptom frequency/scores between exercisers and nonexercisers, adjusting for the usual confounders. Results: The sample included 907 nonexercisers and 488 exercisers (mean age, 49 ± 14 years; mean AHI, 53 ± 20 events/h; 81% men). Nonexercisers and exercisers differed significantly in terms of obesity (72% vs. 54%), the mean proportion of sleep in non-rapid eye movement stage 3 sleep (9 ± 8% vs. 11 ± 6%), and tiredness (78% vs. 68%). Nonexercisers had a higher symptom frequency/scores and poorer sleep quality. Adjustment for exercise weakened the associations between individual symptoms and the AHI, indicating that exercise has a mitigating effect. In binary logistic models, exercise was associated with approximately 30% lower adjusted questionnaire1 score > 2, tiredness; poor-quality sleep, unrefreshing sleep, and negative mood on awakening. Although the odds of an Epworth Sleepiness Scale score > 10 were lower in exercisers, that association did not withstand adjustment for confounders. Conclusions: Exercise is associated with lower frequency/intensity of symptoms in patients with severe OSA. Because up to one third of patients with severe OSA might exercise regularly and therefore be mildly symptomatic, it is important not to rule out a diagnosis of OSA in such patients.

Keywords: Sleep apnea syndromes; Exercise; Sleepiness; Polysomnography.

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 correlate tomographic findings with pulmonary function parameters in patients with idiopathic pulmonary fibrosis (IPF). Methods: A cross-sectional study was carried out, in which 30 nonsmoking patients with IPF were evaluated. Using a semiquantitative scoring system, the following high-resolution computerized tomography (HRCT) findings were quantified: total interstitial disease (TID), reticular abnormality/honeycombing, and ground-glass opacity (GGO). The functional variables were measured by spirometry, forced oscillation technique (FOT), helium dilution method, as well as the single-breath method of measuring diffusion capacity of the lung for carbon monoxide (DLCO). Results: Of the 30 patients studied, 18 were female, and 12 were male, with a mean age of 70.9 years. We found that TID and reticular abnormality and honeycombing correlated significantly (negative correlations) with the measurements of forced vital capacity (FVC), total lung capacity (TLC), DLCO, and dynamic respiratory compliance were found, as well as that GGO correlated significantly (and positively) with residual volume/TLC. The ratio of forced expiratory flow between 25 and 75% of FVC to FVC (FEF25-75%/FVC) correlated positively with TID, reticular abnormality/honeycombing, and GGO. Conclusion: In IPF patients, the measurements of volume, diffusion, and dynamic compliance are the physiological variables which best reflect the extent of the interstitial disease on HRCT scans.

Keywords: Lung diseases, interstitial; Pulmonary fibrosis; Tomography, X-ray computed; Respiratory function tests.

Objective: To evaluate the impact that the distribution of emphysema has on clinical and functional severity in patients with COPD. Methods: The distribution of the emphysema was analyzed in COPD patients, who were classified according to a 5-point visual classification system of lung CT findings. We assessed the influence of emphysema distribution type on the clinical and functional presentation of COPD. We also evaluated hypoxemia after the six-minute walk test (6MWT) and determined the six-minute walk distance (6MWD). Results: Eighty-six patients were included. The mean age was 65.2 ± 12.2 years, 91.9% were male, and all but one were smokers (mean smoking history, 62.7 ± 38.4 pack-years). The emphysema distribution was categorized as obviously upper lung-predominant (type 1), in 36.0% of the patients; slightly upper lung-predominant (type 2), in 25.6%; homogeneous between the upper and lower lung (type 3), in 16.3%; and slightly lower lung-predominant (type 4), in 22.1%. Type 2 emphysema distribution was associated with lower FEV1, FVC, FEV1/FVC ratio, and DLCO. In comparison with the type 1 patients, the type 4 patients were more likely to have an FEV1 < 65% of the predicted value (OR = 6.91, 95% CI: 1.43-33.45; p = 0.016), a 6MWD < 350 m (OR = 6.36, 95% CI: 1.26-32.18; p = 0.025), and post-6MWT hypoxemia (OR = 32.66, 95% CI: 3.26-326.84; p = 0.003). The type 3 patients had a higher RV/TLC ratio, although the difference was not significant. Conclusions: The severity of COPD appears to be greater in type 4 patients, and type 3 patients tend to have greater hyperinflation. The distribution of emphysema could have a major impact on functional parameters and should be considered in the evaluation of COPD patients.

Keywords: Pulmonary disease, chronic obstructive; Pulmonary emphysema; Respiratory function tests; Tomography, X-ray computed.

Objective: To test the hypothesis that disease severity in patients with cystic fibrosis (CF) is correlated with an increased risk of sleep apnea. Methods: A total of 34 CF patients underwent clinical and functional evaluation, as well as portable polysomnography, spirometry, and determination of IL-1β levels. Results: Mean apnea-hypopnea index (AHI), SpO2 on room air, and Epworth Sleepiness Scale score were 4.8 ± 2.6, 95.9 ± 1.9%, and 7.6 ± 3.8 points, respectively. Of the 34 patients, 19 were well-nourished, 6 were at nutritional risk, and 9 were malnourished. In the multivariate model to predict the AHI, the following variables remained significant: nutritional status (β = −0.386; p = 0.014); SpO2 (β = −0.453; p = 0.005), and the Epworth Sleepiness Scale score (β = 0.429; p = 0.006). The model explained 51% of the variation in the AHI. Conclusions: The major determinants of sleep apnea were nutritional status, SpO2, and daytime sleepiness. This knowledge not only provides an opportunity to define the clinical risk of having sleep apnea but also creates an avenue for the treatment and prevention of the disease.

Keywords: Cystic fibrosis; Oxygenation; Sleep apnea, obstructive.

Objective: To compare the effects of voluntary breath stacking (VBS) and involuntary breath stacking (IBS) techniques on respiratory mechanics, lung function patterns, and inspiratory capacity in tracheostomized patients. Methods: This was a randomized crossover clinical trial involving 20 tracheostomized patients admitted to the ICU and submitted to the VBS and IBS techniques, in random order, with an interval of 5 h between each. Ten cycles of each technique were performed with an interval of 30 s between each cycle. In VBS, patients performed successive inspirations for up to 30 s through a one-way valve, whereas in IBS, successive slow insufflations were performed with a resuscitator bag until the pressure reached 40 cmH2O. Respiratory mechanics, inspiratory capacity, and the lung function pattern were evaluated before and after the interventions. Results: After IBS, there was an increase in static compliance (p = 0.007), which was also higher after IBS than after VBS (p = 0.03). There was no significant difference between the pre-VBS and post-VBS evaluations in terms of static compliance (p = 0.42). Inspiratory capacity was also greater after IBS than after VBS (2,420.7 ± 480.9 mL vs. 1,211.3 ± 562.8 mL; p < 0.001), as was airway pressure (38.3 ± 2.6 cmH2O vs. 25.8 ± 5.5 cmH2O; p < 0.001). There were no changes in resistance or lung function pattern after the application of either technique. Conclusions: In comparison with VBS, IBS promoted greater inspiratory capacity and higher airway pressure, resulting in an increase in static compliance.

Keywords: Mucociliary clearance; Respiratory care units; Respiratory mechanics; Physical therapy modalities.

Objective: A resolution passed by the government of the Brazilian state of São Paulo established a protocol for requesting free COPD medications, including tiotropium bromide, creating regional authorization centers to evaluate and approve such requests, given the high cost of those medications. Our objective was to analyze the requests received by an authorization center that serves cities in the greater metropolitan area of (the city of) São Paulo between 2011 and 2016. Methods: Data regarding the authorization, return, or rejection of the requests were compiled and analyzed in order to explain those outcomes. Subsequently, the clinical and functional data related to the patients were evaluated. Results: A total of 7,762 requests for dispensing COPD medication were analyzed. Requests related to male patients predominated. Among the corresponding patients, the mean age was 66 years, 12% were smokers, 88% had frequent exacerbations, and 84% had severe/very severe dyspnea. The mean FEV1 was 37.2% of the predicted value. The total number of requests decreased by 24.5% from 2012 to 2013 and was lowest in 2015. Most (65%) of the requests were accepted. The main reasons for the rejection/return of a request were a post-bronchodilator FEV1/FVC ratio > 0.7, a post-bronchodilator FEV1 > 50% of the predicted value, and failure to provide information regarding previous use of a long-acting β2 agonist. During the study period, the total number of requests returned/rejected decreased slightly, and there was improvement in the quality of the data included on the forms. Conclusions: Here, we have identified the characteristics of the requests for COPD medications and of the corresponding patients per region served by the authorization center analyzed, thus contributing to the improvement of local public health care measures.

Keywords: Pulmonary disease, chronic obstructive; Clinical protocols; Drug costs; Tiotropium bromide.

Objective: To determine whether pulmonary mechanics are altered in mice with sepsis. Methods: A total of 40 Balb/c mice were divided into two groups: survival (n = 21) and pulmonary mechanics (n = 19). The survival group was divided into three subgroups: control (n = 7), sublethal (n = 7) and lethal (n = 7). The pulmonary mechanics group was also divided into three subgroups: control (n = 5), sublethal (n = 7) and lethal (n = 7). Sepsis was induced through cecal ligation and puncture, the latter varying in degree (sublethal or lethal). At eight hours after the intervention, pulmonary mechanics were measured through end-inflation occlusion. In the pulmonary mechanics group, the following variables were studied: total pressure, resistance, viscoelasticity, dynamic compliance and static compliance. The data obtained were analyzed using one-way ANOVA. Results: The data for the survival group indicate the efficacy of the model employed. There were no statistically significant differences among the pulmonary mechanics subgroups in terms of dynamic compliance, static compliance, total pressure, resistance or viscoelasticity. Conclusion: At eight hours after cecal ligation and puncture, there were no changes in the lung parenchyma, nor were any alterations observed in the viscous and viscoelastic components of the lung.

Keywords: Sepsis; Respiratory mechanics; Lung/injuries; Punctures/instrumentation; Respiratory distress syndrome, adult; Mice

Objective: To evaluate the impact of thoracic radiotherapy on respiratory function and exercise capacity in patients with breast cancer. Methods: Breast cancer patients in whom thoracic radiotherapy was indicated after surgical treatment and chemotherapy were submitted to HRCT, respiratory evaluation, and exercise capacity evaluation before radiotherapy and at three months after treatment completion. Respiratory muscle strength testing, measurement of chest wall mobility, and complete pulmonary function testing were performed for respiratory evaluation; cardiopulmonary exercise testing was performed to evaluate exercise capacity. The total radiotherapy dose was 50.4 Gy (1.8 Gy/fraction) to the breast or chest wall, including supraclavicular lymph nodes (SCLN) or not. Dose-volume histograms were calculated for each patient with special attention to the ipsilateral lung volume receiving 25 Gy (V25), in absolute and relative values, and mean lung dose. Results: The study comprised 37 patients. After radiotherapy, significant decreases were observed in respiratory muscle strength, chest wall mobility, exercise capacity, and pulmonary function test results (p < 0.05). DLCO was unchanged. HRCT showed changes related to radiotherapy in 87% of the patients, which was more evident in the patients submitted to SCLN irradiation. V25% significantly correlated with radiation pneumonitis. Conclusions: In our sample of patients with breast cancer, thoracic radiotherapy seemed to have caused significant losses in respiratory and exercise capacity, probably due to chest wall restriction; SCLN irradiation represented an additional risk factor for the development of radiation pneumonitis.

Keywords: Breast neoplasms; Radiotherapy; Radiation pneumonitis; Respiratory function tests; Exercise test.

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.

Objective: To evaluate the effect of adenotonsillectomy on oxygen saturation measured through nocturnal pulse oximetry in children with sleep disordered breathing. Methods: A pre- and post-intervention study was carried out using nocturnal pulse oximetry. The study involved 31 children who were suspected of having sleep disordered breathing and had been referred for adenotonsillectomy. Results: A total of 27 children completed the study. The mean age was 5.2 ± 1.8 years, and 18 (66.7%) of them were male. Upon physical examination, 23 (85.2%) of the children presented class III or class IV hyperplasia of the palatine tonsils. There was significant improvement in the post-operative period over the pre-operative period in terms of the oxygen desaturation rate (OR = 0.65; 95% CI: 0.5-1.3 vs. OR = 1.63; 95% CI: 1.1-2.4; p < 0.001). Conclusion: Adenotonsillectomy significantly improved oxygen saturation, as measured through nocturnal pulse oximetry, in children with sleep disordered breathing.

Keywords: Sleep apnea obstructive; Tonsillectomy; Oximetry; Child