Down syndrome: orofacial pain, masticatory muscle hypotonia, and sleep disorders.

The aim of the present study was to investigate orofacial pain in individuals with Down syndrome (DS) and determine possible associations with masticatory muscle hypotonia (MMH), maximum mouth opening (MMO), and sleep disorders. Twenty-three individuals with DS underwent a standardized clinical examination using Axis I of the Diagnostic Criteria for Temporomandibular Disorders, for the diagnosis of pain in the masseter and temporal muscles and temporomandibular joint (TMJ). MMH was investigated using electromyography of the temporal and masseter muscles and the measurement of maximum bite force (MBF). MMO was measured using an analog caliper. Sleep disorders (obstructive sleep apnea [OSA], snoring index [SI], and sleep bruxism index [SBI]) were investigated using type II polysomnography. Statistical analysis was performed. Nonsignificant differences were found in muscle and TMJ pain between the sexes. However, myalgia and referred myofascial pain in the left masseter muscle were more frequent in males (69%) than females (40%). Electrical activity of the temporal (left: p = .002; right: p = .004) and masseter (left: p = .008) muscles was significantly lower in males than in females. MBF range was lower in males than females, indicating the highest MMH among males. OSA, SI, and SBI were identified in both sexes, but with no statistically significant differences. We concluded that myalgia and referred myofascial pain were found in some individuals with DS, especially in males. Arthralgia was found mainly in females. Temporal and masseter myalgia may have exerted an influence on the severity of MMH in males, particularly on the left side.

Evaluation of the masticatory biomechanical function in Down syndrome and its Influence on sleep disorders, body adiposity and salivary parameters.

OBJECTIVE: To evaluate the phenotypic features of the masticatory biomechanics in atypical subjects with Down syndrome (DS). Its influence was analysed on sleep disorders, body adiposity and its risks, and some physicochemical properties of saliva. METHODS: Seventy subjects were enrolled to assess masticatory biomechanical function and divided into two groups: DS and control groups. Electrical activities of the masseter and temporal muscles (at rest and in maximum voluntary clench-MVC), maximum bite force-MBF and maximum mouth opening-MMO were investigated. Among the atypical subjects, just 24 participants underwent the anthropometry, the polysomnography II and the saliva testing (salivary flow rate-SFR, buffer capacity-BC and salivary cortisol levels, morning/SC-AM and night/SC-PM). RESULTS: MVC and MBF values showed high statistical significance in the control group (P < .001) than in the DS group of 35. MMO values were slightly increased in the DS group in relation to the control group. Overweight and obesity were found in both genders. Atypical women showed higher risk to develop cardiovascular-metabolic diseases than in atypical men. OSA severe was 20% for atypical women and 42.8% for atypical men, whereas snoring index was present in all genders. SFR was reduced in 100% of atypical subjects (hyposalivation in 10% women and 28.5% men). Furthermore, 100% BC, 66.6% SC-AM and 91.6% SC-PM showed normal patterns. CONCLUSION: Masseter and temporal muscle hypotonia was found in all atypical subjects with DS. This muscle dysfunction strongly was related to overweight/obesity, risks for development of cardiovascular/metabolic diseases, OSA severity, successive snoring episodes and salivary flow reduction in DS.

Effect of a home-based exercise program on functional mobility and quality of life in elderly people: protocol of a single-blind, randomized controlled trial.

BACKGROUND: Elderly people have high rates of functional decline, which compromises independence, self-confidence, and quality of life (QoL). Physical exercise leads to significant improvements in strength, balance, functional mobility, and QoL, but there is still reduced access to this therapeutic strategy due to difficulties in locomotion to training centers or lack of adaptation to the exercise environment. METHODS/DESIGN: The purpose of this clinical trial will be to verify the effect of a progressive and semi-supervised, home-based exercise program on the functional mobility, and in the QoL of sedentary elderly people. This is a protocol of a consecutive, single-center, single-blind, and randomized controlled trial. The design, conduct, and report follows the SPIRIT (Standard Protocol Items: Recommendations for Interventional Trials) guidelines. Sedentary elderly people will be enrolled, and randomly allocated into two groups. The intervention group will perform exercises in their own home and the control group will not perform exercises. The evaluations will occur at study enrollment and after 3 months of intervention, and will be performed using the functional mobility Timed Up & Go (TUG) test and sociodemographic and QoL questionnaires. In the statistical analysis, comparisons of mean and correlation analyses will be performed. The primary expected outcome is the improvement in functional mobility verified through the TUG test and the secondary outcome is the improvement in QoL verified by the WHOQOL-OLD. DISCUSSION: The lack of scientific evidence demonstrating the benefits of semi-supervised home exercise on functional mobility and QoL in elderly people represents an obstacle to the development of guidelines for clinical practice and for policy-makers. The World Health Organization highlighted the importance of musculoskeletal health programs for elderly people, and the exercise program described in this protocol was designed to be viable, easy to implement, and inexpensive, and could be performed at the home of elderly subjects after receiving only guidelines and follow-up via periodic visits. Based on these facts, we hope that this study will demonstrate that a well-structured, home-based exercise program can be effective in improving functional mobility and QoL of sedentary elderly people, even without constant supervision during exercise. TRIAL REGISTRATION: Registro Brasileiro de Ensaios Clínicos (ReBEC), Identifier: RBR-3cqzfy . Registered on 2 December 2016.

Quantitative Myasthenia Gravis Score: a Brazilian multicenter study for translation, cultural adaptation and validation.

OBJECTIVE: To perform the translation, cross-cultural adaptation and validation of the Quantitative Myasthenia Gravis Score (QMGS) to Brazilian Portuguese in accordance with international ethical standards. METHODS: The following steps were taken: (1) implementation of the translation protocol and transcultural adaptation, (2) validation of the adapted content, and (3) assessment of reliability. To check intra- and inter-observer reproducibility, each patient underwent two interviews with interviewer-A and one with B. The QMGS was compared to the Myasthenia Gravis Composite Scale and Myasthenia-specific Quality of Life Questionnaire. RESULTS: Our study group consisted of 30 patients, with a mean age of 47.6±11.4 years and a mean duration of illness of 11.33±8.49 years. Correlation between the QMGS and MGC was very strong (r = 0.928; p < 0.001) and substantial between the QMGS and MG-QOL 15 (r = 0.737; p < 0.001). CONCLUSION: The Brazilian Portuguese translation, and validation of the QMGS was successfully performed.

Quantitative Myasthenia Gravis Score: a Brazilian multicenter study for translation, cultural adaptation and validation / Teste Quantitativo para Miastenia Gravis: estudo multicêntrico brasileiro para tradução, adaptação cultural e validação

ABSTRACT Objective To perform the translation, cross-cultural adaptation and validation of the Quantitative Myasthenia Gravis Score (QMGS) to Brazilian Portuguese in accordance with international ethical standards. Methods The following steps were taken: (1) implementation of the translation protocol and transcultural adaptation, (2) validation of the adapted content, and (3) assessment of reliability. To check intra- and inter-observer reproducibility, each patient underwent two interviews with interviewer-A and one with B. The QMGS was compared to the Myasthenia Gravis Composite Scale and Myasthenia-specific Quality of Life Questionnaire. Results Our study group consisted of 30 patients, with a mean age of 47.6±11.4 years and a mean duration of illness of 11.33±8.49 years. Correlation between the QMGS and MGC was very strong (r = 0.928; p < 0.001) and substantial between the QMGS and MG-QOL 15 (r = 0.737; p < 0.001). Conclusion The Brazilian Portuguese translation, and validation of the QMGS was successfully performed.

RESUMO Objetivo O objetivo foi realizar a tradução e validação do teste quantitativo para Miastenia Gravis (QMGS) para Português do Brasil, de acordo com as diretrizes internacionais. Métodos Foram realizadas as etapas de implementação do protocolo de tradução e adaptação transcultural, validação do conteúdo adaptado e avaliação da confiabilidade. Para verificar a reprodutibilidade intra e inter-observador cada paciente foi submetido a duas entrevistas por um entrevistador-A e um B. O QMGS foi comparado ao MG Composite Scale e Myasthenia-specific Quality of Life Questionnaire. Resultados O estudo inclui 30 pacientes, com a média de idade de 47,6±11,4 anos e tempo médio de doença de 11,33±8,49 anos. A correlação entre QMGS e MGC apresentou-se muito forte (r = 0,928; p < 0,001) e substancial entre QMGS e MG-QOL 15 (r = 0,737; p < 0,001). Conclusão A tradução, e validação do QMGS para o português do Brasil foi realizada com sucesso.

Sleep, lung function, and quality of life in patients with myasthenia gravis: A cross-sectional study.

The purpose of this study was to investigate the physiological variables of lung function, respiratory muscle strength, and sleep in clinically stable patients with myasthenia gravis. This was a prospective cross-sectional study conducted in accordance with the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement. Patients adhering to the eligibility criteria were consecutively recruited from the Research Department of Neuromuscular Diseases at the Federal University of Sao Paulo and the Department of Neurology at Santa Casa de Misericordia of Sao Paulo and were referred to the Nove de Julho University Sleep Laboratory (Sao Paulo, Brazil). The study included 25 patients (21 female) with a mean age of 45.28 ± 12.33 years. Only one patient exhibited a restrictive ventilatory pattern. The maximum ventilatory pressures observed were considerably reduced in most patients as compared to reference values. In sleep studies, the patients exhibited significantly reduced oxygen saturation, reduced rapid eye movement sleep time, increased non-rapid eye movement stage 3 sleep, and considerable apnoea/hypopnoea indexes. Clinically stable patients with myasthenia gravis exhibit a high prevalence of sleep-disordered breathing, significant reductions in maximum ventilatory pressures, and impairment of health-related quality of life.

Brazilian-Portuguese translation, cross-cultural adaptation and validation of the Myasthenia Gravis Composite scale. A multicentric study.

OBJECTIVE: To perform the translation, cultural adaptation and validation of the Myasthenia Gravis Composite (MGC) scale in Brazil. METHODS: The study was conducted at three neuromuscular disease research centers in accordance with the international ethical standards, following a multi-modal approach and was conducted in three steps consisting of translation, cultural adaptation, and validation according to international guidelines. The final version of the MGC was applied in a sample of 27 MG patients and the total score was compared to a Portuguese version of the MG-QOL-15. RESULTS: The internal consistency verified by Cohen's Kappa test was excellent (0.766). The correlation between the MGC and MG-QOL-15 was strong (R = 0.777; p = 0.000). No significant differences were found between the responses of patients in the first and second applications of the MGC. CONCLUSION: The MGC scale, validated into Brazilian Portuguese, has proven to be a reliable instrument that is easy to use, and is highly reproducible.

Brazilian-Portuguese translation, cross-cultural adaptation and validation of the Myasthenia Gravis Composite scale. A multicentric study / Tradução, adaptação cultural e validação da escala composta de Miastenia Grave para a lingua portuguesa do Brasil. Estudo multicêntrico

ABSTRACT Objective To perform the translation, cultural adaptation and validation of the Myasthenia Gravis Composite (MGC) scale in Brazil. Methods The study was conducted at three neuromuscular disease research centers in accordance with the international ethical standards, following a multi-modal approach and was conducted in three steps consisting of translation, cultural adaptation, and validation according to international guidelines. The final version of the MGC was applied in a sample of 27 MG patients and the total score was compared to a Portuguese version of the MG-QOL-15. Results The internal consistency verified by Cohen’s Kappa test was excellent (0.766). The correlation between the MGC and MG-QOL-15 was strong (R = 0.777; p = 0.000). No significant differences were found between the responses of patients in the first and second applications of the MGC. Conclusion The MGC scale, validated into Brazilian Portuguese, has proven to be a reliable instrument that is easy to use, and is highly reproducible.

RESUMO Objetivo Realizar a tradução e a adaptação transcultural da escala composta de Miastenia Grave (ECMG) Myasthenia Gravis Composite (MGC) no Brasil. Métodos O estudo foi realizado em três centros de investigação em doenças neuromusculares, de acordo com as normas éticas internacionais, consistindo em tradução, adaptação cultural e validação de acordo com as diretrizes internacionais. A versão final do MGC ECMG foi aplicada em vinte e sete pacientes com MG e a pontuação total foi comparada ao questionário MG-QOL 15. Resultados A consistência interna verificada pelo teste Kappa de Cohen foi excelente (0,766) e a correlação entre o a ECMG MGC e MG-QOL 15 foi positiva (R = 0,777; p = 0,000). Não foram encontradas diferenças entre as respostas dos pacientes na primeira e segunda aplicação da MGC. Conclusão A ECMG escala MGC validada para o Português do Brasil provou ser um instrumento confiável, de fácil aplicação e altamente reprodutível.

A systematic review of sleep disorders in patients with chronic kidney disease undergoing hemodialysis.

The purpose of this study was to conduct a systematic review of the available evidence on sleep disorders in patients with end stage renal disease (ESRD) undergoing hemodialysis (HD). [Subjects and Methods] Two independent reviewers performed a computer-assisted search of the MEDLINE, SciELO, LILACS, and BIREME Virtual Health Library medical databases from their inception to November 2015. [Results] One thousand one hundred twenty-six articles were found that met the inclusion criteria. Articles were excluded if they were not in English, the patients did not undergo HD, or the studies were not cross-sectional or clinical trials. After reading the full text, a further 300 studies were excluded because they did not use polysomnography. The remaining 18 studies with ESRD patients undergoing HD comprised 8 clinical trials and 10 cross-sectional studies. This systematic review followed the criteria outlined by the PRISMA declaration. [Conclusion] In this systematic review, a high prevalence of sleep disorders was observed in ESRD, including sleep-disordered breathing. This knowledge may enable health professionals to devise new strategies for the diagnosis and treatment of these patients, in order to reduce morbidity and mortality and improve their quality of life.

The impact of abdominal adiposity measured by sonography on the pulmonary function of pre-menopausal females.

BACKGROUND: The Body Mass Index (BMI) is a widely used parameter to study obesity; however it does not assess the distribution of body adiposity. Ultrasonography is a reliable method of measuring subcutaneous (SAT), visceral (VAT) and Total adipose tissue of the abdomen (TAT) to determine the influence of abdominal fat on pulmonary function by directly measuring abdominal adipose tissue. METHODS: Eighty pre-menopausal, non-smoker, sedentary females with no history of pulmonary disease were subdivided into three groups: 25 normal-weight, 28 overweight, 27 obese. Absolute and predictive spirometric values were obtained: FVC, FEV1, FEV1/FVC, IC, ERV. RESULTS: A positive correlation between increased %IC and decreased %ERV was observed with increased BMI (p < 0.02; 0.001 respectively); %FVC, %FEV1 and %ERV decreased significantly as SAT (p = 0.01, p = 0.02; p < 0.001) and TAT (p = 0.01, p = 0.03, p < 0.001) increased, whereas VAT was negatively correlated only with %ERV (p < 0.001). Increments of 5 mm in TAT, VAT and SAT were followed by a reduction of 0.83 %, 0.81 %, 1.90 % in %FVC, respectively, as well as a reduction of 4.25 %, 4.31 % and 9.44 % in %ERV, respectively. CONCLUSIONS: Subcutaneous abdominal adipose tissue deposition in obese females has a greater negative influence on pulmonary function than visceral adipose tissue deposition.

Observational study of sleep, respiratory mechanics and quality of life in patients with non-cystic fibrosis bronchiectasis: a protocol study.

INTRODUCTION: Bronchiectasis is a chronic disorder characterised by permanent and irreversible abnormal dilation of the bronchi and bronchioles, primarily caused by repeated cycles of pulmonary infections and inflammation, which lead to reduced mucociliary clearance and to the excessive production of sputum. Patients with non-cystic fibrosis bronchiectasis may be predisposed to hypoxemia during sleep, or to symptoms that may lead to arousals and thereby reduce the quality of life, because of the irreversible dilation of the bronchi and the presence of secretions and airflow obstruction. METHODS AND ANALYSIS: For this cross-sectional observational study, patients with a clinical diagnosis of non-cystic fibrosis bronchiectasis will be recruited from the Bronchiectasis Clinic of the Pneumology Department of the Santa Casa de Misericordia Hospital and the Federal University of São Paulo (São Paulo, Brazil). Patients of either sex will be included if high-resolution CT of the thorax and classic sweat test confirms they have non-cystic fibrosis bronchiectasis, are between 18 and 80 years old, use long-acting bronchodilators, are clinically stable for a least 1 month, agree to participate in the study and they sign a statement of informed consent. The first part of the study will involve a clinical evaluation, maximal respiratory pressures, spirometry and the Saint George's Respiratory Questionnaire. The Sleep Laboratory of the Master's and Doctoral Postgraduate Program in Rehabilitation Sciences of the Nove de Julho University (São Paulo, Brazil) will perform the polysomnographic studies, Berlin Questionnaire, Epworth Sleepiness Scale, waist and neck circumferences, modified Mallampati classification and tonsil index. ETHICS AND DISSEMINATION: This protocol has been approved by the Human Research Ethics Committees of Santa Casa de Misericordia Hospital (process number 178/2012) and Human Research Ethics Committee of Nove de Julho University (process number 370474/2010). All participants will sign a statement of informed consent. The study findings will be published in peer-reviewed journals and presented at conferences.

Cardiovascular risk and mortality in end-stage renal disease patients undergoing dialysis: sleep study, pulmonary function, respiratory mechanics, upper airway collapsibility, autonomic nervous activity, depression, anxiety, stress and quality of life: a prospective, double blind, randomized controlled clinical trial.

BACKGROUND: Chronic kidney disease (CKD) is one of the most serious public health problems. The increasing prevalence of CKD in developed and developing countries has led to a global epidemic. The hypothesis proposed is that patients undergoing dialysis would experience a marked negative influence on physiological variables of sleep and autonomic nervous system activity, compromising quality of life. METHODS/DESIGN: A prospective, consecutive, double blind, randomized controlled clinical trial is proposed to address the effect of dialysis on sleep, pulmonary function, respiratory mechanics, upper airway collapsibility, autonomic nervous activity, depression, anxiety, stress and quality of life in patients with CKD. The measurement protocol will include body weight (kg); height (cm); body mass index calculated as weight/height(2); circumferences (cm) of the neck, waist, and hip; heart and respiratory rates; blood pressures; Mallampati index; tonsil index; heart rate variability; maximum ventilatory pressures; negative expiratory pressure test, and polysomnography (sleep study), as well as the administration of specific questionnaires addressing sleep apnea, excessive daytime sleepiness, depression, anxiety, stress, and quality of life. DISCUSSION: CKD is a major public health problem worldwide, and its incidence has increased in part by the increased life expectancy and increasing number of cases of diabetes mellitus and hypertension. Sleep disorders are common in patients with renal insufficiency. Our hypothesis is that the weather weight gain due to volume overload observed during interdialytic period will influence the degree of collapsibility of the upper airway due to narrowing and predispose to upper airway occlusion during sleep, and to investigate the negative influences of haemodialysis in the physiological variables of sleep, and autonomic nervous system, and respiratory mechanics and thereby compromise the quality of life of patients. TRIAL REGISTRATION: The protocol for this study is registered with the Brazilian Registry of Clinical Trials (ReBEC RBR-7yhr4w and World Health Organization under Universal Trial Number UTN: U1111-1127-9390 [http://www.ensaiosclinicos.gov.br/rg/RBR-7yhr4w/]).