Search

In the last few decades, there have been considerable improvements in the diagnosis and care of Duchenne muscular dystrophy (DMD), the most common childhood muscular dystrophy. International guidelines have been published and recently reviewed. A group of Brazilian experts has developed a standard of care based on a literature review with evidence-based graded recommendations in a two-part publication. Implementing best practice management has helped change the natural history of this chronic progressive disorder, in which the life expectancy for children of the male sex in the past used to be very limited. Since the previous publication, diagnosis, steroid treatment, rehabilitation, and systemic care have gained more significant insights with new original work in certain fields. Furthermore, the development of new drugs is ongoing, and some interventions have been approved for use in certain countries. Therefore, we have identified the need to review the previous care recommendations for Brazilian patients with DMD. Our objective was to create an evidence-based document that is an update on our previous consensus on those topics., Competing Interests: The authors have no conflict of interests to declare., (Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Sleep is essential for the proper functioning of all individuals. Sleep-disordered breathing can occur at any age and is a common reason for medical visits. The objective of this consensus is to update knowledge about the main causes of sleep-disordered breathing in adult and pediatric populations, with an emphasis on obstructive sleep apnea. Obstructive sleep apnea is an extremely prevalent but often underdiagnosed disease. It is often accompanied by comorbidities, notably cardiovascular, metabolic, and neurocognitive disorders, which have a significant impact on quality of life and mortality rates. Therefore, to create this consensus, the Sleep-Disordered Breathing Department of the Brazilian Thoracic Association brought together 14 experts with recognized, proven experience in sleep-disordered breathing.

Aims: Poor sleep is a frequent occurrence in the critical illness. Evaluate sleep quality and test the effect of a multi-intervention sleep care protocol in improving sleep quality in a coronary care unit (CCU)., Methods and Results: Quasi-experimental study, carried out in two phases. During the first phase, the control group (n = 58 patients) received usual care. Baseline sleep data were collected through the Richards-Campbell Sleep Questionnaire (RCSQ) and the Sleep in the Intensive Care Unit Questionnaire (SICUQ). During the second phase (n = 55 patients), a sleep care protocol was implemented. Interventions included actions to promote analgesia, reduce noise, brightness, and other general measures. Sleep data were collected again to assess the impact of these interventions. The intervention group had better scores in overall sleep depth [median (interquartile range)] [81 (65-96.7) vs. 69.7 (50-90); P = 0.046]; sleep fragmentation [90 (65-100) vs. 69 (42.2-92.7); P = 0.011]; return to sleep [90 (69.7-100) vs. 71.2 (40.7-96.5); P = 0.007]; sleep quality [85 (65-100) vs. 71.1 (49-98.1); P = 0.026]; and mean RCSQ score [83 (66-94) vs. 66.5 (45.7-87.2); P = 0.002] than the baseline group. The main barriers to sleep were pain [1 (1.0-5.5)], light [1 (1.0-5.0)], and noise [1 (1.0-5.0)]. The most rated sources of sleep-disturbing noise were heart monitor alarm [3 (1.0-5.25)], intravenous pump alarm [1.5 (1.0-5.00)]. and mechanical ventilator alarm [1 (1.0-5.0)]. All were significantly lower in the intervention group than in the baseline group., Conclusion: A multi-intervention protocol was feasible and effective in improving different sleep quality parameters and reducing some barriers to sleep in CCU patients., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.)

Objective: To cross-culturally adapt and validate the Montreal Children's Hospital Feeding Scale (MCH-FS) into Brazilian Portuguese., Methods: The MCH-FS, originally validated in Canada, was validated in Brazil as Escala Brasileira de Alimentação Infantil (EBAI) and developed according to the following steps: translation, production of the Brazilian Portuguese version, testing of the original and the Brazilian Portuguese versions, back-translation, analysis by experts and by the developer of the original questionnaire, and application of the final version. The EBAI was applied to 242 parents/caregivers responsible for feeding children from 6 months to 6 years and 11 months of age between February and May 2018, with 174 subjects in the control group and 68 ones in the case group. The psychometric properties evaluated were validity and reliability., Results: In the case group, 79% of children were reported to have feeding difficulties, against 13% in the control group. The EBAI had good internal consistency (Cronbach's alpha=0.79). Using the suggested cutoff point of 45, the raw score discriminated between cases and controls with a sensitivity of 79.4% and specificity of 86.8% (area under the ROC curve=0.87)., Conclusions: The results obtained in the validation process of the EBAI demonstrate that the questionnaire has adequate psychometric properties and, thus, can be used to identify feeding difficulties in Brazilian children from 6 months to 6 years and 11 months of age.

Purpose: To assess the current diagnostic methods for sleep bruxism (SB)., Materials and Methods: This review of the literature evaluates all available instrumental and noninstrumental methods of bruxism/SB diagnosis., Results: SB diagnosis can be performed using self-reports and clinical examination, but these methods have little agreement with polysomnography. Two portable electromyography/electrocardiography appliances have been validated against polysomnography (BiteStrip and Bruxoff), but they are indicated only for primary SB. Polysomnography is considered the gold standard and is indicated for secondary SB; however, it is expensive and time-consuming., Conclusion: No perfect method of SB diagnosis exists, and future research should concentrate on improving SB self-reports.

Objective: To validate the Pediatric Obstructive Sleep Apnea Screening tool for use in Brazil., Materials and Methods: The Brazilian version of this questionnaire, originally validated and tested in the United States, was developed as follows: (a) translation; (b) back-translation; (c) completion of the final version; (d) pre-testing. The questionnaire was applied prior to polysomnography to children aged 3-9 years from October 2015 to October 2016, and its psychometric properties (i.e., validity and reliability) were evaluated. The accuracy was assessed from comparisons between polysomnographic results and corresponding questionnaire scores., Results: Sixty patients were enrolled, and based on polysomnographic findings, 48% patients had normal apnea-hypopnea index, while the remaining 52% met the criteria for obstructive sleep apnea. Minimum O 2 saturation level was significantly lower among obstructive sleep apnea children (p=0.021). Satisfactory concordance was found between individual apnea-hypopnea index and questionnaire scores. Bland-Altman plot-derived bias was 0.1 for the difference between measures, with 5.34 (95% CI: 4.14-6.55) and -5.19 (95%CI: -6.39 to -3.98) for the upper and lower agreement range. Internal consistency derived from Cronbach's alpha was 0.84 (95%CI: 0.78-0.90)., Conclusion: The questionnaire was translated to and validated into Brazilian-Portuguese version, and showed good reliability and concordance with apnea-hypopnea index. This questionnaire offers a reliable screening option for sleep-disordered breathing in children., (Copyright © 2018 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Objetive: The aim of this study was to characterize the acoustic signal of silent tracheal aspiration in children with oropharyngeal dysphagia (OPD)., Method: Thirty-two children with OPD were examined with combined digital cervical auscultation (DCA) and videofluoroscopic swallow study (VFSS). Power spectral density (PSD, in 1/√Hz) of the acoustic signal from a sequential series of five liquid swallows was used for comparisons between children who silently aspirated and children who did not aspirate on VFSS. Fourteen children were excluded due to either DCA/VFSS artifact or non-silent aspiration (cough, choking)., Results: The remaining 18 participants (median age 6 years, range 2-12.8) were classified based on VFSS as aspirators (n = 8) and non-aspirators (n = 10). The PSD curve of aspirators presented an ascending pattern (1st vs. 5th deglutition: 695.2 vs. 4421.9 1/√Hz), while the curve of non-aspirators was flat (1st vs. 5th deglutition: 509 vs. 463.4 1/√Hz), with marked differences being observed from the 3rd measure onwards (p < .001). In this study, DCA was able to identify silent tracheal aspiration in children with OPD., Conclusion: This non-invasive technique identified aspiration by an increase in the PSD curve in aspiration sounds.

Introduction: The optimal surgical technique for the management of patients with Robin Sequence (RS) has not been established. One of the most commonly used surgical techniques, mandibular distraction osteogenesis (MDO), is still controversial because of its potential risks and the lack of clear evidence of its efficacy., Objectives: To assess variations in airway patency, clinical symptoms, and polysomnographic parameters in children with RS who underwent MDO., Methods: In this prospective cohort study, 38 patients with RS were evaluated before and after MDO. Symptom severity was classified using a grading scale for RS clinical manifestations. Patients underwent flexible fiberoptic laryngoscopy, and the images were classified by a blinded examiner using two validated grading scales for airway obstruction. Patients not requiring ventilatory support underwent a polysomnography., Results: Patients' symptoms significantly improved after MDO, as shown by a decreased score in the grading scale for RS clinical manifestations (preoperative score of 2.20 vs. postoperative score of 0.81; P < 0.001). The two endoscopic grading scales also showed a statistically significant postoperative improvement in airway obstruction (first scale: preoperative score of 1.56 vs. postoperative score of 0.92; second scale: preoperative score of 2.19 vs. postoperative score of 1.16; P < 0.001 for both). Moreover, there was a statistically significant variation in the following polysomnographic parameters evaluated pre- and postoperatively: apnea-hypopnea index, total sleep time, oxygen desaturation nadir, and oxygen desaturation index (P < 0.05)., Conclusions: MDO seems to be an effective surgical option for children, as shown by postoperative improvements in clinical symptoms, endoscopic grading scales, and polysomnographic parameters., (Copyright © 2018. Published by Elsevier Ltd.)

Purpose: To evaluate the effects of sleeping with or without a maxillary mucosa-supported complete denture (CD) in edentulous patients wearing a mandibular fixed implant-supported CD on sleep quality, sleep bruxism (SB) activity, and signs of obstructive sleep apnea syndrome (OSAS)., Materials and Methods: A total of 18 female patients with maxillary mucosa-supported CDs opposing mandibular fixed implant-supported CDs were selected and tested for sleep quality, SB activity, and signs of OSAS during one night while wearing the maxillary CD and another night without wearing it, with a washout period of 7 days in between. The validated Brazilian Portuguese versions of the Pittsburgh Sleep Quality Index (PSQI-BR), Sleep Assessment Questionnaire (SAQ), and Epworth Sleepiness Scale (ESSBR) were used to assess subjective sleep quality. Diagnosis of OSAS followed the American Academy of Sleep Medicine (AASM) guidelines and was confirmed with an objective and validated type 3 portable cardiorespiratory monitor (ApneaLink Plus v.9.00) by measuring the respiratory disturbance index (RDI). SB activity was assessed with a subjective self-report questionnaire and an objective and validated portable electromyographic device (BiteStrip)., Results: The objective measurements of SB activity and the RDI showed significant reduction (ie, improvement) when patients did not wear the maxillary CD. The subjective measurements of sleep quality and self-reports of SB activity showed no significant differences between wearing and not wearing a maxillary CD., Conclusion: The present results have shown that not wearing a maxillary CD at night is preferable as far as SB and OSAS are concerned, but larger-scale studies are still needed.

Objectives: To evaluate the association of polysomnographic parameters with clinical symptom severity in Robin sequence (RS) patients., Methods: All patients diagnosed as presenting with RS at Hospital de Clínicas de Porto Alegre from October 2012 to June 2016 were enrolled. They were classified as isolated RS, RS-plus, and syndromic RS. Polysomnography (PSG) was performed, except for those patients in need of respiratory support. Symptom severity was evaluated as defined by the Cole et al., Classification: Ordinal OR (for the chance of increase in one grade on the clinical severity scale) and R 2 (determination coefficient from ordinal logistic regression) were computed from data analysis., Results: A total of 80 participants were enrolled in the study. Fifty-five of these were able to undergo polysomnography. Worsening of the studied PSG parameters was associated with increase in clinical severity grading, as follows: desaturation index (OR 1.27; 95% CI; 1.07-1.51; R 2  = 19.8%; p = 0.006); apnea/hypopnea Index (OR 1.13; 95% CI; 1.01-1.26; R 2  = 12.5%; p = 0.02); sleep mean oxygen saturation (OR 0.16; 95% CI; 0.05-0.52; R 2  = 22.6%; p = 0.002); oxygen saturation nadir (OR 0.73; 95% CI; 0.56-0.96; R 2  = 10.0%; p = 0.02); percentage of time with oxygen saturation <90% (OR 9.49; 95% CI; 1.63-55.31, R 2  = 37.6%; p = 0.012); and percentage of time presenting with obstruction (OR 2.5; 95% CI; 1.31-4.76; R 2  = 25.1%; p = 0.006)., Conclusions: Polysomnography parameters were associated with severity of clinical manifestations in patients with RS. Oxyhemoglobin saturation-based parameters had surprisingly significant R 2 values. Therefore, those parameters, which have traditionally been undervalued in other clinical settings, should also be assessed in the polysomnographic evaluation of RS patients., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objectives/hypothesis: To assess the performance of endoscopic grading systems of glossoptosis in identifying severe clinical manifestations in children with Robin sequence (RS)., Study Design: Nested cohort cross-sectional study., Methods: All RS patients diagnosed at Hospital Clinics of Porto Alegre from October 2012 to June 2016 were enrolled in this cohort. Patients underwent sleep endoscopy and were classified according to Yellon (Y) and de Sousa et al. (S) scales. Symptom severity evaluation was performed as defined by Cole et al. The outcome of interest was Cole's clinical classification grade 3., Results: Eighty patients were eligible for analysis. Sensitivity (Y: 56.2%, S: 28.1%, P < .001) and specificity (Y: 85.4%, S: 93.8%, P = .038) in identifying severe clinical symptoms patients (i.e., Cole grade 3) were statistically different between Y and S classifications. A low but significant overall correlation was observed for both Y (rho = 0.372, P < .001) and S (rho = 0.439, P < .001) classifications when compared with Cole classification. Diagnostic odds ratio (DOR) for Y (DOR: 7.53, 95% confidence interval [CI]: 4.15-10.90) and S (DOR: 5.87, 95% CI: 1.86-9.87) were equivalent (P = .92). Also, receiver operating characteristic curves area under the curve were not significantly different between them. The positive likelihood ratio was 3.86 (95% CI: 1.82-8.16) and 4.50 (95% CI: 1.32-15.36) for Y and S, respectively., Conclusions: Y and S grading systems showed a low sensitivity and moderate to high specificity in detecting patients with severe clinical manifestations. Correlation between Y/S and Cole et al. grading were also considered low. Development of a more discriminative anatomic grading system is still needed for this specific disorder., Level of Evidence: 2b. Laryngoscope, 128:502-508, 2018., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

Background: In cystic fibrosis (CF) patients, end stage of pulmonary disease is characterized by pulmonary hypertension (PH), hypoxemia, decrease in exercise tolerance, and sleep quality., Objective: To evaluate the association between clinical, lung function, sleep quality, and polysomnographic variables with PH in CF patients aged 16 years or older., Methods: In a cross-sectional study, 51 clinically stable CF patients underwent a clinical evaluation, an overnight polysomnography and answered sleep questionnaires (Pittsburgh Sleep Quality Index and Epworth sleepiness scale). Also, CF patients had their pulmonary function, 6-minute walk test (6MWT) and echocardiography assessed., Results: Fifty-one CF patients participated in the study; 47% were female. The mean age was 25.1 ± 8.8 years. Pulmonary artery systolic pressure (PASP) was greater than 35 mm Hg in 11 (27.5%) patients. Variables associated with PASP>35 mm Hg in univariate analysis were Shwachman-Kulczycki clinical score, forced expiratory volume in 1 second % of predicted, Pseudomonas aeruginosa in sputum culture, at-rest peripheral capillary oxygen saturation (SpO 2 ), SpO 2 at end of 6MWT and time of oxygen desaturation <90% during sleep. These variables were included in the binary logistic regression. The independent variable associated with the PASP > 35 mm Hg was at-rest SpO 2 (OR = 10.8, CI 95% 1.7-67.3, P = .011). The cuttoff SpO 2 < 94% had the sensitivity = 7/11 = 64%, specificity = 40/40 = 100%, positive predicted values = 7/7 = 100% and negative predicted values = 40/44 = 91% to the diagnosis of PH., Conclusion: the present study showed a high rate of PH in adolescent and adult CF patients. At-rest SpO 2 was associated with PH., (© 2016 John Wiley & Sons Ltd.)

Objective: Systematically search literature for flexible fiberoptic laryngoscopy (FFL) use in Robin Sequence (RS) patients, in diverse clinical scenarios., Data Sources: Pubmed, LILACS and SCIELO., Review Methods: Systematic review using a sensitive search strategy focused on RS patients and FFL., Results: There were 48 full text articles included in this systematic review. No summary meta-analytic measurement could be calculated due to heterogeneity of interventions and outcomes. FFL approaches were grouped in five topics, as follows: Endoscopic classification: no evidence on superiority of awake over light sedation and correlation of grading scales with symptom severity. Airway abnormalities: high incidence of concomitant lesions besides glossoptosis. Swallowing evaluation: no validation against fluoroscopy (gold standard) yet. Intubation aid for mechanical ventilation: ultra-thin bronchoscopes improve success rates of intubation. Treatment outcome monitoring: no consensus on ideal parameters to be checked., Conclusion: Some applications have their roles already well established in the management of RS patients, like the evaluation of glossoptosis and associated lesions and as an intubation assistance tool, while others need to be the subject of further research, like the exact method of evaluation, its association with clinical manifestations, its role in swallowing investigation and as a postoperative success predictor., (Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Objective: To evaluate the performance of two glossoptosis airway obstruction classifications in predicting symptom severity and laryngeal exposure difficulty in Robin Sequence (RS) patients., Setting: Public tertiary hospital otolaryngology section (Hospital de Clínicas de Porto Alegre - HCPA)., Patients: All RS patients diagnosed at HCPA from October 2012 to February 2015 were enrolled, a total of 58 individuals. They were classified in isolated RS, RS-Plus and syndromic RS., Intervention: Patients were submitted to sleep endoscopy and a score was attributed according to Yellon and de Sousa by a blinded researcher. Symptom severity evaluation was performed as defined by Cole classification., Main Outcome Measure: Association between endoscopic findings and clinical symptoms severity and laryngeal exposure difficulty., Results: Twenty four patients were identified as isolated RS (41.4%), 19 patients presented as RS-Plus (32.7%) and 15 patients had well defined diagnosed syndromes (25.9%). Concomitant airway anomalies were found in 18 patients (31%). Specifically 17.4% in isolated RS, 55.6% in RS- Plus and 28.6% in the syndromic group had such anomalies (P = 0,03). Probability of presenting severe clinical symptoms as graded by Cole was higher in grade 3 Yellon classification (68.4%, P = 0.012) and in moderate and severe de Sousa classification (61.5% and 62.5%, respectively, P = 0.015) than in milder grades of obstruction. This findings were considered significant even after controlling for patient age. Laryngeal exposure difficulty was correlated with de Sousa and Yellon (Rho = 0,41 and Rho = 0,43, respectively; P < 0,05)., Conclusion: Patients with higher degrees of obstruction in sleep endoscopy had a higher probability of presenting a more severe clinical manifestation and a more difficult laryngeal exposure. Since the number of patients included in this study was small for subgroup analyses, it is not clear if this association is restricted to a specific group of RS., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: Health-related quality of life (HRQOL) has received much attention in patients with cystic fibrosis (CF). The goal of this study was to evaluate the association between clinical, lung function, sleep quality, and polysomnographic variables with 2 HRQOL questionnaires, the shorter-version World Health Organization Quality of Life (WHOQOL-BREF) and Cystic Fibrosis Quality of Life (CFQOL) questionnaires, in adult subjects with CF., Methods: In a cross-sectional study, 51 subjects underwent clinical evaluation and overnight polysomnography and answered WHOQOL-BREF, CFQOL, Pittsburgh Sleep Quality Index, and Epworth Sleepiness Scale questionnaires. In addition, pulmonary function tests, 6-min walk tests, and echocardiography were performed., Results: For WHOQOL-BREF scores, the sleep quality index was associated with the physical domain; the percent-of-predicted 6-min walk distance (6MWD) and sleepiness scale were associated with the psychological domain; the percent-of-predicted FEV1 and sleep quality index were associated with the social relationship domain; and the sleep quality index was associated with the environment domain. For CFQOL scores, age at diagnosis, clinical score, and sleep quality index were associated with the physical functioning domain; the percent-of-predicted 6MWD and pulmonary arterial systolic pressure were associated with the role domain; sex and sleep quality index were associated with the vitality domain; the apnea-hypopnea index was associated with the emotional functioning domain; sex and body mass index (BMI) were associated with the body image domain; the percent-of-predicted 6MWD and sleep quality index were associated with the health perception domain; age, sex, BMI, and arousal index were associated with the weight domain; age, sex, percent-of-predicted FEV1, percent-of-predicted 6MWD, and pulmonary arterial systolic pressure were associated with the respiratory symptom domain; and the clinical score was associated with the digestive symptom domain., Conclusions: The sleep quality index score, 6MWD, sleepiness scale score, and FEV1 were predictors of WHOQOL-BREF scores. Age at diagnosis, clinical score, sleep quality score, 6MWD, sex, apnea-hypopnea index, BMI, current age, arousal index, FEV1, and pulmonary arterial systolic pressure were predictors of CFQOL scores., (Copyright © 2015 by Daedalus Enterprises.)

Purpose: This before and after study evaluated the effects of a mandibular advancement device (MAD) on sleep bruxism (SB) activity and its associated signs and symptoms., Materials and Methods: Nineteen young adults (39.9 ± 12.9 years, 58% women) with a clinical history of SB without sleep or neurologic disorders and no spontaneous temporomandibular disorder pain were selected. SB activity was assessed after a habituation period of 2 weeks. The results of a 3-month treatment with a thermoplastic monoblock MAD were compared to baseline using electromyogram polysomnography and the BiteStrip, a portable EMG device. Sleep disorders were assessed and validated against the polysomnography sleep assessment questionnaire (SAQ). Additionally, common signs and symptoms of SB were evaluated with the research diagnostic criteria for temporomandibular disorders. Occlusal force was compared to baseline using a cross-arch force transducer., Results: There was a significant improvement in both SB activity and sleep scores (including SB episodes per hour) according to the BiteStrip and the SAQ, respectively. There was also a significant reduction in the signs and symptoms of SB, including grinding and/or clenching, temporomandibular joint (TMJ) sounds, muscle pain, and occlusal force. None of the SB subjects experienced MAD breakage, but in 24% of patients, the MAD treatment had to be interrupted due to TMJ/muscle pain and/or discomfort., Conclusion: The MAD treatment resulted in the reduction of SB activity, SB signs and symptoms, sleep disorders, and occlusal force.

MPS encompasses a group of rare lysosomal storage disorders that are associated with the accumulation of glycosaminoglycans (GAG) in organs and tissues. This accumulation can lead to the progressive development of a variety of clinical manifestations. Ear, nose, throat (ENT) and respiratory problems are very common in patients with MPS and are often among the first symptoms to appear. Typical features of MPS include upper and lower airway obstruction and restrictive pulmonary disease, which can lead to chronic rhinosinusitis or chronic ear infections, recurrent upper and lower respiratory tract infections, obstructive sleep apnoea, impaired exercise tolerance, and respiratory failure. This review provides a detailed overview of the ENT and respiratory manifestations that can occur in patients with MPS and discusses the issues related to their evaluation and management.

Purpose: Questions remain about the polysomnographic findings and the predictors for sleep desaturation in cystic fibrosis (CF) patients. Our study aimed to evaluate sleep parameters in a sample of adult CF patients comparing them with healthy controls and to determine the best predictors of sleep desaturation in CF patients with awake resting peripheral oxygen saturation (SpO(2)) ≥90%., Methods: In a cross-sectional study, with data collected prospectively, 51 clinically stable adult CF patients (mean age 25.1 ± 6.7 years) and 25 age-matched controls underwent an overnight polysomnography and answered sleep questionnaires. CF patients had their pulmonary function, 6-min walk test, and echocardiography assessed., Results: CF patients and control subjects had similar sleep architecture. However, CF patients had impaired subjective sleep quality and a higher arousal index than controls. The apnea-hypopnea index was similar in both groups, and only two CF patients (3.9%) fulfilled the criteria for obstructive sleep apnea syndrome. Sleep desaturation was more common in CF patients (29.4% vs 0%; p < 0.001). In a logistic regression model, we observed that awake resting SpO(2) was the single best variable associated with sleep desaturation in CF population (p < 0.001). The awake SpO(2) <94% had a sensitivity, specificity, positive and negative predictive value for sleep desaturation of, respectively, 93.3%, 100%, 100%, and 97.3%., Conclusions: CF patients had a worse subjective sleep quality and small changes in sleep architecture. In nonhypoxic, adult CF patients, sleep desaturation is common, is not associated with obstructive sleep events, and can be accurately predicted by awake resting SpO(2) <94%.

Objective: This validation study compared an electromyography (EMG) device, Bitestrip, versus polysomnography in the diagnosis of sleep bruxism (SB)., Study Design: Forty-nine consecutive patients (32 women and 17 men, 41.2 ± 12.9 years old) with a clinical history of SB were included in the study from the Orofacial Pain Clinic, Faculty of Dentistry, Pontifical Catholic University of Rio Grande do Sul in Brazil., Results: Initially, we compared an SB positive/negative test result for both systems (agreement 87.8%, sensitivity 84.2%, positive predictive value 100%, and crude kappa 0.71). We then compared a 4-scale test (no, light, moderate, or severe bruxism) between the 2 methods (agreement 80.27%, weighted kappa 0.51, and Kendall W coefficient 0.575)., Conclusions: The Bitestrip can be considered as a moderate screening method for the diagnosis of SB, because it is more precise in detecting the presence or absence of SB but less precise in detecting its intensity., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Introduction: The Pittsburgh Sleep Quality Index (PSQI) is a questionnaire that assesses sleep quality and disturbances over a 1-month period. It is a valuable tool for research purposes. The aim of this study was to validate a Brazilian Portuguese version of the PSQI., Methods: The Brazilian Portuguese version (PSQI-BR) was developed according to the following steps: (a) translation, (b) back-translation, (c) comparison between translation and back-translation performed by a group of experts, and (d) pretest in bilingual individuals. Between January 2006 and September 2007, the PSQI-BR was applied to a group of consecutive patients who were submitted to overnight polysomnography with clinical suspicion of obstructive sleep apnea syndrome (OSAS) or insomnia. As in the original article, a group of patients with depression and a control group were included. The depression group was composed of patients from the Mood Disorders Unit of the Psychiatry Department of Hospital de Clínicas de Porto Alegre (HCPA), Brazil. The control group was composed of subjects with a history of normal sleep habits, without noticed snoring., Results: A total of 83 patients and 21 controls completed the questionnaire and were submitted to overnight polysomnography. The seven-component scores of the PSQI-BR had an overall reliability coefficient (Cronbach's α) of 0.82, indicating a high degree of internal consistency. The groups included 43 patients with OSAS, 21 with insomnia, 19 with depression and 21 controls. The mean (±SD) PSQI-BR score was 8.1±4.0 for patients with OSAS, 12.8±3.7 for insomnia patients, 14.5±3.7 for those with depression and 2.5±2.0 for control subjects. The one-way ANOVA demonstrated significant differences in PSQI-BR scores across the four diagnostic groups (p<0.001). Post hoc tests between paired groups showed that scores for OSAS, depression and insomnia were significantly higher than for controls (p<0.05). PSQI-BR scores for insomnia did not differ from those obtained for depression (p>0.05), but both were higher than for OSAS (p<0.05)., Conclusions: The results of the present study demonstrate that the PSQI-BR is a valid and reliable instrument for the assessment of sleep quality and equivalent to its original version when applied to individuals who speak the Brazilian Portuguese language. Despite relevant influences of language and cultural background, no major cultural adaptations were necessary during the validation process. The PSQI-BR can be a tool either for clinical management or research., (Copyright © 2010 Elsevier B.V. All rights reserved.)

Childhood obstructive sleep apnea syndrome (OSAS) is characterized by recurrent episodes of partial or complete upper airway obstruction during sleep. The disease encompasses a continuum from primary snoring (a benign condition without physiological alterations or associated complications) to increased upper airway resistance, obstructive hypoventilation and OSAS. The prevalence of snoring is high, ranging from 1.5% to 15%, depending on how it is defined. Based on parent-reported questionnaires and complementary tests, the prevalence of OSAS is 1-4%. This syndrome is more common in boys, overweight children, of African ancestry, with a history of atopy and prematurity. The most common symptoms are snoring that is frequent and loud; family-reported apnea; and restless sleep. The physical examination should assess growth status, signs of chronic upper airway obstruction, and craniofacial malformations. Overnight polysomnography is the gold standard test for the diagnosis and for the determination of the appropriate positive pressure level, as well as for postsurgical treatment evaluation. Intermittent hypoxia and multiple arousals resulting from obstructive events contribute to the well-described cardiovascular, neurocognitive, and behavioral consequences in pediatric patients with OSAS. Although the main treatment for OSAS in children is adenotonsillectomy, treatment with CPAP or Bilevel is becoming more widely used in the pediatric population.

Purpose: A before-and-after experimental clinical study was carried out with the objective of evaluating the effect of a mandibular advancement device (MAD; 75% advancement), made of a thermoplastic material, on sleep bruxism (SB) and sleep scores., Materials and Methods: After a habituation period of 1 week, SB scores were taken at baseline and after use of the MAD for 30 days. Scores were compared using the newly developed BiteStrip, which registers the number of contractions of the unilateral masseter muscle after a 5-hour period, giving a severity score from 0 to 3 after the registrations. To assess sleep, the Sleep Assessment Questionnaire (SAQ), a screening tool with scores ranging from 0 to 68, was used before and after use of the MAD. Twenty-eight subjects (13 women, 15 men; mean age: 42.9 +/- 12.0 years) with a clinical history of SB and no spontaneous temporomandibular disorder (TMD) pain were selected. The clinical diagnosis of either moderate or severe SB was further confirmed through use of the BiteStrip (scores 2 or 3) at baseline. A 30-day follow-up period was used for evaluation. Both methods were validated against polysomnography. In addition, common signs and symptoms of TMD based on the Research Diagnostic Criteria for Temporomandibular Disorders were also evaluated before and after use to assess the side effects of the MAD., Results: There was a statistically significant improvement in both SB and sleep scores based on the BiteStrip and the SAQ (Wilcoxon signed rank and Student paired t test, P < .05). In the signs and symptoms of TMD, there was a significant reduction in temporomandibular joint sounds as well as in masseter and temporalis tenderness to palpation. None of the SB subjects experienced any breakage of the MAD., Conclusion: The MAD had a positive effect on SB and sleep scores, measured by the BiteStrip and the SAQ, respectively, and did not increase any traditional signs and symptoms of TMD in a 30-day evaluation period.

Objective: The aim of this study was to develop a Portuguese-language version of the Epworth sleepiness scale (ESS) for use in Brazil., Methods: The steps involved in creating the ESS in Brazilian Portuguese (ESS-BR) were as follows: translation; back-translation; comparison (by a committee) between the translation and the back-translation; and testing in bilingual individuals. The ESS-BR was applied to a group of patients who were submitted to overnight polysomnography in order to identify obstructive sleep apnea-hypopnea syndrome (OSAHS), insomnia and primary snoring. A control group was composed of subjects with a history of normal sleep habits, without reported snoring., Results: A total of 114 patients and 21 controls were included. The 8-item scores of the ESS-BR had an overall reliability coefficient of 0.83. The study group was composed of 59 patients with OSAHS, 34 patients with primary snoring and 21 patients with insomnia. One-way ANOVA demonstrated significant differences in ESS-BR scores among the four diagnostic groups (p < 0.001). Post-hoc tests between groups showed that the ESS-BR scores of the patients with insomnia did not differ from those of the controls (p > 0.05). The ESS-BR scores were significantly higher for OSAHS patients and for primary snorers than for controls (p < 0.05). In addition, the scores for OSAHS patients were significantly higher than were those for primary snorers (p < 0.05)., Conclusions: The results of the present study demonstrate that the ESS-BR is a valid and reliable instrument for the assessment of daytime sleepiness, equivalent to its original version when applied to individuals who speak Brazilian Portuguese.

Parkinson's Disease Sleep Scale (PDSS) is a specific scale for the assessment of sleep disturbances in subjects with Parkinson's Disease (PD). This cross-sectional study set out to validate the PDSS in a Brazilian Portuguese Version (PDSS-BR). Ninety-five patients with PD participated in the study; their PD symptoms were evaluated by Unified Parkinson's Disease Rating Scale (UPDRS sections I-IV) and Hoehn and Yahr scale. Patients completed Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), Beck Depression Inventory (BDI) and PDSS-BR. PDSS-BR internal consistency was satisfactory (Cronbach's alpha: 0.82; all PDSS-BR items were significantly and positively associated with total score). Test-retest reliability for total PDSS-BR score was 0.94. PDSS-BR score was highly correlated with sleep PSQI scale (r(s) = -0.63; p < 0.0001) and moderately with ESS (r(s) = -0.32; p < 0.001) and UPDRS sections I (r(s) = -0.38; p < 0.0001) and II (r(s) = -0.36; p < 0.0001) and BDI (r(s) = -0.55; p < 0.0001). Depressive symptoms, as determined by the BDI, were associated with significantly worse quality of nocturnal sleep, as measured by the PDSS-BR. The psychometric attributes of the PDSS-BR were satisfactory and consistent with those of previous studies. In summary, PDSS-BR can be useful for clinical and research purposes in Brazil.

Background: A higher frequency of nocturnal gastroesophageal reflux (GER) in adult patients with respiratory symptoms has been demonstrated. The aim of this study was to determine the prevalence of nocturnal GER by using prolonged intraesophageal pH monitoring and compare it with spirometry results in children with persistent asthma., Methods: Thirty-eight patients with persistent asthma for at least 2 years were studied. Gastrointestinal symptoms suggestive of GER were considered as regurgitation, heartburn, and abdominal pain. All patients underwent prolonged intraesophageal pH study and spirometry. GER was considered positive when a reflux index (RI) was higher than 5%. Forced vital capacity (FVC), forced expiratory volume in 1 second (FEV(1)), forced mid-expiratory flow rate (FEF(25-75%)), and FEV(1)/FVC ratio were measured., Results: Median age was 10 years of age (range 5 to 15) and 58% were male; GER prevalence was 47.3%. Median (range) of reflux index during supine and upright periods from GER patients were, respectively, 8.7% (3.2 to 23.6) and 10.5% (5.2 to 15.0) (p = 0.913), and only FEF(25-75%) was below the predicted value: 54.5% (39.4 to 96.9). Reflux index was not significantly correlated with FVC, FEV(1) and FEF(25-75%)., Conclusions: A high prevalence of GER was found in children and adolescents with persistent asthma, equally distributed in the supine (nocturnal) and upright positions. There was no correlation with pulmonary function test.