Update of the Brazilian consensus recommendations on Duchenne muscular dystrophy.

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.

Nas últimas décadas, houve progressos significativos no diagnóstico e no tratamento da distrofia muscular de Duchenne (DMD), considerada a distrofia muscular mais comum na infância. Diretrizes internacionais foram publicadas e revisadas recentemente. Um grupo de especialistas brasileiros desenvolveu um padrão de atendimento baseado em revisão de literatura, com recomendações graduadas pautadas em evidências compiladas em uma publicação dividida em duas partes. A implementação de melhores práticas de manejo ajudou a modificar a história natural desta doença crônica, progressiva, que, no passado, oferecia uma expectativa de vida muito limitada para crianças do sexo masculino. Desde a publicação desse consenso anterior, o diagnóstico, o tratamento com esteroides, a reabilitação e os cuidados sistêmicos ganharam novas possibilidades a partir da divulgação dos resultados de trabalhos originais em algumas dessas áreas. Além disso, as pesquisas e o desenvolvimento de novos fármacos estão em andamento, e algumas intervenções já foram aprovadas para uso em determinados países. Nesse contexto, identificamos a necessidade de rever as recomendações anteriores sobre o manejo dos pacientes brasileiros com DMD. Nosso objetivo principal foi elaborar uma atualização baseada em evidências sobre esses tópicos do consenso.

Update of the Brazilian consensus recommendations on Duchenne muscular dystrophy / Atualização das recomendações do consenso brasileiro para distrofia muscular de Duchenne

Abstract 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.

Resumo Nas últimas décadas, houve progressos significativos no diagnóstico e no tratamento da distrofia muscular de Duchenne (DMD), considerada a distrofia muscular mais comum na infância. Diretrizes internacionais foram publicadas e revisadas recentemente. Um grupo de especialistas brasileiros desenvolveu um padrão de atendimento baseado em revisão de literatura, com recomendações graduadas pautadas em evidências compiladas em uma publicação dividida em duas partes. A implementação de melhores práticas de manejo ajudou a modificar a história natural desta doença crônica, progressiva, que, no passado, oferecia uma expectativa de vida muito limitada para crianças do sexo masculino. Desde a publicação desse consenso anterior, o diagnóstico, o tratamento com esteroides, a reabilitação e os cuidados sistêmicos ganharam novas possibilidades a partir da divulgação dos resultados de trabalhos originais em algumas dessas áreas. Além disso, as pesquisas e o desenvolvimento de novos fármacos estão em andamento, e algumas intervenções já foram aprovadas para uso em determinados países. Nesse contexto, identificamos a necessidade de rever as recomendações anteriores sobre o manejo dos pacientes brasileiros com DMD. Nosso objetivo principal foi elaborar uma atualização baseada em evidências sobre esses tópicos do consenso.

Brazilian Thoracic Association Consensus on Sleep-disordered Breathing.

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.

Prevalence of chronic respiratory diseases and medication use among children and adolescents in Brazil - a population based cross-sectional study / Prevalência de doenças respiratórias crônicas e uso de medicamentos entre crianças e adolescentes no Brasil - um estudo transversal de base populacional

Abstract Objectives: to describe the prevalence of chronic respiratory diseases and their pharmacological management in children and adolescents in Brazil. Methods: data from the Pesquisa Nacional de Acesso, Uso e Promoção do Uso Racional de Medicamentos no Brasil (PNAUM)(National Access Survey, Use and Promotion of Rational Use of Medicines in Brazil),a population-based cross-sectional study, were analyzed. Household surveys were conducted between September 2013 and February 2014. We included the population under 20 years of age with chronic respiratory diseases. Prevalence of disease, indication of pharmacological treatment, and their use were assessed. Results: the prevalence of chronic respiratory diseases in children aged less than 6 years old was 6.1% (CI95%= 5.0-7.4), 4.7% (CI95%= 3.4-6.4) in those 6-12 years, and 3.9% (CI95%= 2.8-5.4) in children 13 years and older. Children under 6 showed a higher prevalence of pharmacological treatment indication (74.6%; CI95%= 66.0-81.7), as well as medication use (72.6%; CI95%= 62.8-80.7). Of those using inhalers, 56.6% reported using it with a spacer. The most frequent pharmacologic classes reported were short-acting β2 agonists (19.0%), followed by antihistamines (17.2%). Conclusion: children and adolescents who report chronic respiratory diseases living in urban areas in Brazil seem to be undertreated for their chronic conditions. Pharmacological treatment, even if indicated, was not used, an important finding for decision-making in this population.

Resumo Objetivos: descrever a prevalência de doenças respiratórias crônicas e seu manejo farmacológico em crianças e adolescentes no Brasil. Métodos: foram analisados os dados da Pesquisa Nacional de Acesso, Uso e Promoção do Uso Racional de Medicamentos no Brasil (PNAUM), um estudo transversal de base populacional. As pesquisas domiciliares foram realizadas entre setembro de 2013 e fevereiro de 2014. Incluímos a população com menos de 20 anos de idade com doenças respiratórias crônicas. Foi avaliada a prevalência de doença, indicação de tratamento farmacológico e seu uso. Resultados: a prevalência de doenças respiratórias crônicas em menores de 6 anos foi de 6,1% (IC95%= 5,0-7,4), 4,7% (IC95%= 3,4-6,4) naqueles 6-12 anos e 3,9% (IC95%= 2,8-5,4) em crianças com 13 anos ou mais. Crianças menores de 6 anos apresentaram uma maior prevalência de indicação de tratamento farmacológico (74,6%; IC95%= 66,0-81,7), assim como uso de medicamentos (72,6%; IC95%= 62,8-80,7). Dos usuários de inaladores, 56,6% relataram o uso com espaçador. As classes farmacológicas mais frequentemente relatadas foram β2 agonistas de curta ação (19,0%), seguidos por anti-histamínicos (17,2%). Conclusão: crianças e adolescentes que relatam doenças respiratórias crônicas residentes em áreas urbanas no Brasil parecem ser subtratados para suas condições crônicas. O tratamento farmacológico, mesmo quando indicado, não foi utilizado em sua totalidade, um achado importante para a tomada de decisão nessa população.

A multi-intervention protocol to improve sleep quality in a coronary care unit.

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.

Brazilian Thoracic Association Consensus on Sleep-disordered Breathing / Consenso em Distúrbios Respiratórios do Sono da Sociedade Brasileira de Pneumologia e Tisiologia

ABSTRACT 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.

RESUMO O sono é essencial para o adequado funcionamento de todos os indivíduos. Os distúrbios respiratórios do sono ocorrem em todas as faixas etárias, constituindo motivo frequente de consulta médica. O objetivo deste consenso foi atualizar os conhecimentos sobre os principais distúrbios respiratórios do sono tanto na população adulta quanto na pediátrica, com ênfase na apneia obstrutiva do sono. A apneia obstrutiva do sono é uma doença extremamente prevalente, porém frequentemente subdiagnosticada. Associa-se frequentemente a uma série de comorbidades, notadamente cardiovasculares, metabólicas e neurocognitivas, que impactam significativamente na qualidade de vida e na mortalidade. Por conta disso, o Departamento de Distúrbios Respiratórios do Sono da Sociedade Brasileira de Pneumologia e Tisiologia reuniu 14 especialistas com reconhecida e comprovada experiência em distúrbios respiratórios do sono para a elaboração deste documento.

CROSS-CULTURAL ADAPTATION AND VALIDATION OF THE MONTREAL CHILDREN'S HOSPITAL FEEDING SCALE INTO BRAZILIAN PORTUGUESE.

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.

Cross-cultural adaptation and validation of the montreal children's hospital feeding scale into brazilian portuguese / Adaptação transcultural e validação da montreal children's hospital feeding scale para o português falado no brasil

ABSTRACT 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.

RESUMO Objetivo: Realizar a adaptação transcultural e a validação da escala Montreal Children's Hospital Feeding Scale (MCH-FS) para a língua portuguesa falada no Brasil. Métodos: A MCH-FS, originalmente validada no Canadá, foi validada no Brasil como Escala Brasileira de Alimentação Infantil (EBAI) e desenvolvida a partir das seguintes etapas: tradução, montagem da versão em português brasileiro, teste da versão em inglês e da versão em português brasileiro, retrotradução, análise por experts e autora do questionário original e aplicação da versão final em estudo. A EBAI foi aplicada em 242 pais/cuidadores responsáveis pela alimentação de crianças de seis meses a seis anos e 11 meses de idade no período de fevereiro a maio de 2018, sendo 174 no grupo controle e 68 no grupo dos casos. As propriedades psicométricas avaliadas foram validade e confiabilidade. Resultados: No grupo dos casos, 79% dos pais/cuidadores relataram dificuldades alimentares, e no grupo controle, 13%. A EBAI apresentou boa consistência interna (alfa de Cronbach=0,79). Utilizando-se o ponto de corte sugerido de 45, o escore bruto (raw score) diferenciou casos de controles com sensibilidade de 79,4% e especificidade de 86,8% (área sob a curva ROC=0,87). Conclusões: Os resultados obtidos na validação da EBAI evidenciaram medidas psicométricas adequadas. Portanto, a escala pode ser utilizada na identificação de dificuldades alimentares em crianças brasileiras de seis meses a seis anos e 11 meses idade.

The effect of mandibular distraction osteogenesis on airway obstruction and polysomnographic parameters in children with Robin sequence.

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.

The Effects of Sleeping With or Without Prostheses on Sleep Quality, Sleep Bruxism, and Signs of Obstructive Sleep Apnea Syndrome: A Pilot Study.

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.

Association of polysomnographic parameters with clinical symptoms severity grading in Robin sequence patients: a cohort nested cross-sectional study.

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 R2 (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; R2 = 19.8%; p = 0.006); apnea/hypopnea Index (OR 1.13; 95% CI; 1.01-1.26; R2 = 12.5%; p = 0.02); sleep mean oxygen saturation (OR 0.16; 95% CI; 0.05-0.52; R2 = 22.6%; p = 0.002); oxygen saturation nadir (OR 0.73; 95% CI; 0.56-0.96; R2 = 10.0%; p = 0.02); percentage of time with oxygen saturation <90% (OR 9.49; 95% CI; 1.63-55.31, R2 = 37.6%; p = 0.012); and percentage of time presenting with obstruction (OR 2.5; 95% CI; 1.31-4.76; R2 = 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 R2 values. Therefore, those parameters, which have traditionally been undervalued in other clinical settings, should also be assessed in the polysomnographic evaluation of RS patients.

Diagnostic accuracy of current glossoptosis classification systems: A nested cohort cross-sectional study.

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.

Pulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings.

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 (SpO2 ), SpO2 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 SpO2 (OR = 10.8, CI 95% 1.7-67.3, P = .011). The cuttoff SpO2 < 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 SpO2 was associated with PH.

The role of flexible fiberoptic laryngoscopy in Robin Sequence: A systematic review.

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.

Severity of clinical manifestations and laryngeal exposure difficulty predicted by glossoptosis endoscopic grades in Robin sequence patients.

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.

Analysis of the effects of a mandibular advancement device on sleep bruxism using polysomnography, the BiteStrip, the sleep assessment questionnaire, and occlusal force.

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.

Validation of the Brazilian Portuguese version of the Pittsburgh Sleep Quality Index.

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.