Respiratory muscle impairment evaluated with mep/mip ratio in children and adolescents with chronic respiratory disease / Fraqueza muscular respiratória avaliada por meio da relação pemax/pimax em crianças com doença respiratória crônica

ABSTRACT Objective: To evaluate the strength of respiratory muscles and to compare maximum inspiratory (MIP) and expiratory (MEP) pressure and MEP/MIP ratio between patients with chronic respiratory diseases and healthy individuals. Methods: Case-control study. Individuals with neuromuscular disease and post-infectious bronchiolitis obliterans were considered. In addition, they were also matched according to anthropometric and demographic characteristics with healthy children and adolescents. MIP, MEP in the three groups, and pulmonary function only in patients with chronic respiratory diseases were recorded. Results: A total of 52 subjects with CRD (25 with neuromuscular disease, and 27 with post-infectious bronchiolitis obliterans) and 85 healthy individuals were included, with an average age of 11.3±2.1 years. Patients with neuromuscular disease and post-infectious bronchiolitis obliterans presented lower MIP and MEP when compared with healthy individuals, although MEP/MIP ratio was lower in patients with neuromuscular disease (0.87±0.3) and higher in patients with post-infectious bronchiolitis obliterans (1.1±0.3) compared to the healthy group (0.97±0.2). Only in patients with neuromuscular disease a negative correlation was observed between MEP/MIP ratio and age (r=-0.50; p=0.01). Conclusions: Differences in the pattern of muscular weakness between patients with chronic respiratory diseases were observed. In patients with neuromuscular disease, a decrease in the MEP/MIP ratio depending on MIP was verified; and in those patients with post-infectious bronchiolitis obliterans, an increase in the MEP/MIP ratio depending on MIP was also observed.

RESUMO Objetivo: Avaliar a força dos músculos respiratórios e comparar a relação entre a pressão expiratória máxima (PEmáx) e a pressão inspiratória máxima (PImáx) em pacientes com doença respiratória crônica (DRC) e crianças saudáveis. Métodos: Estudo caso-controle. Foram selecionados indivíduos com doença neuromuscular e bronquiolite obliterante pós-infecciosa. Ademais, os grupos foram pareados com crianças e adolescentes saudáveis, considerando características antropométricas e demográficas. Foram registradas a PImáx e a PEmáx nos três grupos e a função pulmonar apenas em pacientes com doença respiratória crônica. Resultados: Foram incluídos 52 indivíduos com DRC (25 com doença neuromuscular e 27 com bronquiolite obliterante pós-infecciosa) e 85 indivíduos saudáveis, com idade média de 11,3±2,1 anos. Pacientes com doença neuromuscular e bronquiolite obliterante pós-infecciosa apresentaram menor PImáx e PEmáx em comparação aos indivíduos saudáveis, embora a relação PEmáx/PImáx tenha sido menor nos pacientes com doença neuromuscular (0,87±0,3) e maior nos pacientes com bronquiolite obliterante pós-infecciosa (1,1±0,3) em comparação ao grupo saudável (0,97±0,2). Somente em pacientes com doença neuromuscular foi observada uma correlação negativa entre a razão PEmáx/PImáx e a idade (r=-0,50; p=0,01). Conclusões: Foram observadas diferenças no padrão de fraqueza muscular em pacientes com doença respiratória crônica. Nos pacientes com doença neuromuscular, verificou-se diminuição na relação PEmáx/PImáx dependendo da PImáx; em pacientes com bronquiolite obliterante pós-infecciosa, foi observado aumento na relação dependendo da PImáx.

Pontos de corte da função pulmonar e capacidade funcional determinantes para sarcopenia e dinapenia em pacientes com DPOC / Pulmonary function and functional capacity cut-off point to establish sarcopenia and dynapenia in patients with COPD

RESUMO Objetivo Estabelecer ponto de corte entre as variáveis clínicas e funcionais para avaliar a prevalência de sarcopenia e dinapenia em pacientes com Doença Pulmonar Obstrutiva Crônica (DPOC), além de analisar o impacto da Disfunção Muscular Esquelética (DME) nestas variáveis. Métodos Realizado estudo transversal com pacientes diagnosticados com DPOC para determinar sarcopenia ou dinapenia por meio do índice de baixa massa muscular e Força de Preensão Palmar (FPP). Avaliadas a função pulmonar, força muscular respiratória e capacidade funcional (CF). A precisão das variáveis na determinação dos pontos de corte previstos para as doenças em questão foi obtida a partir da curva Receiver Operating Characteristic (ROC) e de uma análise bidirecional da variância. Resultados Ao final da análise, obtiveram-se 20 pacientes com DPOC estratificados por sarcopenia (n = 11) e dinapenia (n = 07). O grupo com sarcopenia apresentou menor massa magra e menor pressão inspiratória máxima (PImáx), diminuição da Força de Preensão Palmar (FPP) e redução da CF (p<0,050). O grupo com dinapenia apresentou redução da PImáx, menor FPP e menor distância percorrida no teste Incremental Shuttle Walk (ISWT) (p<0,050). Foram encontrados pontos de corte no Volume Expiratório Forçado (VEF1) durante o primeiro segundo na PImáx, na Pressão Expiratória Máxima (PEmáx) e no ISWT, o que possibilitou identificar sarcopenia ou dinapenia nestes pacientes. A partir destes resultados, foi possível encontrar coexistência das condições (efeito DME) na DPOC: redução da distância no ISWT (p = 0,002) e % ISWT (p = 0,017). Conclusões Em pacientes com DPOC moderada a muito grave, a sarcopenia pode ser prevista pelo VEF1 (% previsto) <52, PImáx <73 cm H2O, PEmáx <126 cm H2O e distância percorrida de <295 metros no ISWT. Já a dinapenia pode ser prevista pelo VEF1 <40%, PImáx <71 cm H2O, PEmáx <110 cm H2O e distância de <230 metros percorrida no ISWT.

ABSTRACT Objective To establish a cut-off point for clinical and functional variables to determinate sarcopenia and dynapenia in COPD patients, and to analyze the impact of skeletal muscle dysfunction (SMD) on these variables. Methods Cross-sectional study, screened COPD patients for sarcopenia or dynapenia through low muscle mass and hand grip strength (HGS). Clinical variables: pulmonary function, respiratory muscle strength and functional capacity (FC). The precision of the variables in determining points of predictive cut-off for sarcopenia or dynapenia were performed using the Receiver Operating Characteristic curve and two-way analysis of variance. Results 20 COPD patients stratified for sarcopenia (n = 11) and dynapenia (n = 07). Sarcopenia group presented lower lean mass and lower maximal inspiratory pressure (MIP), decreased HGS, reduced FC (p<0.050). Dynapenia group presented reduced MIP, lower HGS and walked a shorter distance at Incremental shuttle walk test (ISWT) (p<0.050). We found cut-off points of forced expiratory volume in one second (FEV1), MIP and maximal expiratory pressure (MEP) and ISWT. It is possible to identify sarcopenia or dynapenia in these patients. We found the coexistence of the conditions (SMD effect) in COPD - reduction in the distance in the ISWT (p = 0.002) and %ISWT (p = 0.017). Conclusion In moderate to very severe COPD patients the sarcopenia could be predicted by FEV1 (%predicted) < 52, MIP < 73 cmH2O, MEP < 126 cmH2O and distance traveled of < 295 m in ISWT. Whereas dynapenia could be predicted by FEV1 < 40%, MIP < 71 cmH2O, MEP < 110 cmH2O and distance of < 230 m traveled in ISWT.

Analysis of the muscle tissue of Wistar rats submitted to the sciatic nerve compression model and cryotherapy / Análise do tecido muscular de ratos Wistar submetidos ao modelo de compressão do nervo isquiático e à crioterapia

ABSTRACT Objective: To evaluate the effects of right sciatic nerve compression and cryotherapy on muscle tissue. Methods: We used 42 male Wistar rats, subdivided in the following Groups Control, Injury 3, Injury 8 and Injury 15 submitted to nerve compression and euthanized in the 3rd, 8th and 15th day after surgery. The Cryotherapy Injury 3 was entailed treatment with cryotherapy by immersion of the animal in recipient for 20 minutes during 1 day, then animals were euthanized at the 3rd day after surgery, and the Cryotherapy Injury 8 and the Cryotherapy Injury 15 was treated for 6 days, and euthanized at the 8th and 15th day after surgery. Functional evaluation was performed by the grasping strength of the right pelvic limb. The right tibialis anterior muscles were evaluated for mass, smaller diameter and cross-sectional area. In the Cryotherapy Injury 8 and the Cryotherapy Injury 15 groups, the hydroxyproline was dosed in the right soles. Results: In the compression there was a significant difference in the Injury Groups compared with the Control Group (p<0.05). In the smaller diameter, the compression in Control Group was higher than Injury 8 (p=0.0094), Injury 15 (p=0.002) and Cryotherapy Injury 15 (p<0.001) groups. The comparison between groups with euthanasia in the same post-operative period, a significant difference (p=0.0363) was seen in day 8th after surgery, and this result in Cryotherapy Injury Group was greater than Injury Group. In the fiber area, Control Group was also higher than the Injury 8 (p=0.0018), the Injury 15 (p<0.001) and the Cryotherapy Injury 15 (p<0.001). In hydroxyproline, no significant difference was seen between groups. Conclusion: Nerve damage resulted in decreased muscle strength and trophism, the cryotherapy delayed hypotrophy, but this effect did not persist after cessation of treatment.

RESUMO Objetivo: Avaliar os efeitos da compressão nervosa do isquiático direito e da crioterapia no tecido muscular. Métodos: Foram utilizados 42 ratos Wistar machos, subdivididos nos Grupos Controle, Lesão 3, Lesão 8 e Lesão 15, submetidos a compressão nervosa e eutanasiados, respectivamente, no 3°, 8° e 15° dias pós-operatório; Lesão Crioterapia 3, tratado com crioterapia, por imersão durante 20 minutos, por 1 dia, e eutanasiados no 3° dia pós-operatório; e Lesão Crioterapia 8 e Lesão Crioterapia 15, tratados durante 6 dias e eutanasiados no 8° e 15° dias pós-operatório. A avaliação funcional foi realizada pela força de preensão do membro pélvico direito. Os músculos tibiais anteriores direitos foram avaliados quanto a massa, menor diâmetro e área de secção transversa. Em Lesão Crioterapia 8 e Lesão Crioterapia 15, foi dosada a hidroxiprolina nos sóleos direitos. Resultados: Na preensão, houve diferença significativa nos Grupos Lesão quando comparados ao Grupo Controle (p<0,05). No menor diâmetro, o Grupo Controle foi maior que Lesão 8 (p=0,0094), Lesão 15 (p = 0,002) e Lesão Crioterapia 15 (p<0,001). Na comparação entre os grupos com eutanásia no mesmo pós-operatório, houve diferença significativa (p=0,0363) no 8° pós-operatório, sendo Lesão Crioterapia maior que Lesão. Na área das fibras, o Grupo Controle também foi maior que Lesão 8 (p=0,0018), Lesão 15 (p<0,001) e Lesão Crioterapia 15 (p<0,001). Na hidroxiprolina, não houve diferença significativa entre os grupos. Conclusão: A lesão nervosa resultou na diminuição da força e em trofismo muscular, e a crioterapia retardou a hipotrofia, porém este efeito não se manteve após o tratamento cessar.

Prevalência dos sintomas de LER/DORT, perda de força muscular manual e seu impacto na qualidade de vida de cirurgiões dentistas no município de Bauru/SP / Prevalence of symptoms of RSI/WMSD loss of hand muscle strength and its impact on quality of life of dentists in the municipality of Bauru/SP

O trabalho assume um papel cada vez mais central na vida das pessoas, assim, é previsível que modelos modernos de tecnologias de gestão se ocupem também da qualidade de vida no trabalho (QVT) e dos fatores que incorporam a satisfação do indivíduo em sua atividade laboral e na humanização das situações relacionadas ao trabalho humano. Alguns estudiosos aventam a possibilidade do estresse contínuo no ambiente de trabalho acarretar prejuízos à integridade física, psicológica e ao convívio social e familiar dos profissionais. Assim à medida que o trabalho evolui e se torna mais dependente da técnica, ocorre um maior desgaste físico, psíquico e mental nos trabalhadores, isto acontece em função das exigências impostas pelas ocupações de ocupacionais e aumenta de forma assustadora o número de acidentes e doenças ocupacionais. Desta forma, a prática da odontologia predispõe seus profissionais a desenvolverem, sobretudo doenças do sistema osteomuscular, levando a incapacitação para o trabalho se não forem adotados princípios de ergonomia aplicados ao serviço. De maneira geral, no ambiente profissional há uma série de estímulos que podem contribuir significativamente para o surgimento de um conjunto de sintomas que são classicamente associados ao estresse laboral ou ocupacional. O objetivo do presente estudo consistiu em investigar os possíveis sintomas que acometem os cirurgiões dentistas da rede pública e da rede privada do município de Bauru/SP e sua relação com a atividade laboral, por meio do questionário Nórdico de Sintomas Osteomusculares (QNSO), avaliando também a presença da perda muscular manual utilizando um dinamômetro e assim investigar o impacto que esses possíveis fatores de risco causam na qualidade de vida desses cirurgiões dentistas utilizando o questionário QWLQ-Bref (Quality of Working Life Questionnaire Questionário de Qualidade de Vida no Trabalho). Do total de 1066 cirurgiões dentistas regularmente inscritos no Conselho Regional de Odontologia de Bauru (CROSP), apenas 128 (100,00%) participaram desta pesquisa, onde 64 cirurgiões dentistas pertencem à rede pública e 64 cirurgiões dentistas pertencem à rede privada. Tendo em vista a relevância sobre o tema Sintomas de LER/DORT, assim como os resultados também mostram que ao comparar a rede pública com a rede privada pôde-se perceber que na rede privada é maior é a frequência dos relatos de queixa de dor, desconforto ou dormência.(AU)

Work plays an increasingly central role in people's lives, so it is expected that modern models of management technologies will also focus on quality of life at work (QWL) and factors that incorporate the satisfaction of the individual in his work activity And the humanization of situations related to human work. Some scholars point out the possibility of continuous stress in the work environment causing damages to the physical and psychological integrity and to the social and familiar life of the professionals. Thus, as the work evolves and becomes more dependent on the technique, there is a greater physical, mental and mental exhaustion in the workers, this happens in function of the exigencies imposed by occupations of occupational and increases in a frightening way the number of accidents and occupational diseases. In this way, the practice of dentistry predisposes its professionals to develop, especially diseases of the musculoskeletal system, leading to incapacitation for work if principles of ergonomics applied to the service are not adopted. In general, in the professional environment there are a number of stimuli that may contribute significantly to the appearance of a set of symptoms that are classically associated with occupational or occupational stress. The objective of the present study was to investigate the possible symptoms that affect the dental surgeons of the public network and the private network of the city of Bauru / SP and its relation with the work activity, through the Nordic Questionnaire of Osteomuscular Symptoms (QNSO), evaluating Also the presence of manual muscle loss using a dynamometer and thus investigate the impact that these possible risk factors cause in the quality of life of these dental surgeons using the QWLQ-Bref questionnaire (Quality of Working Life Questionnaire). Of the total of 1,066 dentists regularly enrolled in the Regional Council of Dentistry of Bauru (CROSP), only 128 (100.00%) participated in this research, where 64 dental surgeons belong to the public network and 64 dental surgeons belong to the private network. Considering the relevance of the topic "Symptoms of RSI / DORT", as well as the results also show that when comparing the public network with the private network it was possible to perceive that in the private network it is greater is the frequency of the complaints reports of Pain, discomfort or numbness.(AU)

The relevance of trunk evaluation in Duchenne muscular dystrophy: the segmental assessment of trunk control / A relevância da avaliação do controle de tronco na distrofia muscular de Duchenne: a Segmental Assessment of Trunk Control

ABSTRACT The aim was to describe trunk control in ambulant and non-ambulant patients with Duchenne muscular dystrophy (DMD). We conducted a cross-sectional analysis of a sample of 50 DMD patients, (M age = 16.7 years) who underwent the Segmental Assessment of Trunk Control (SATCo). A seven-level scale of trunk control was used (1: head control only; 7: control of entire trunk while unsupported). Static, active and reactive posture control were evaluated in ambulant and non-ambulant patients. Inter-rater reliability for all assessments was evaluated by calculating the kappa coefficient. More advanced disease (having higher Vignos scores), was associated with poorer trunk control. Ambulant patients showed better trunk control than non-ambulant patients (p = 0.003). There was strong inter-rater agreement for SATCo scale scores.

RESUMO O objetivo foi avaliar o controle de tronco em pacientes deambulantes e não-deambulantes com distrofia muscular de Duchenne (DMD). Estudo transversal composto por 50 pacientes com diagnóstico de DMD, média de idade 16,72 anos, avaliados pela Segmental Assessment of Trunk Control (SATCo). A escala apresenta sete níveis para o controle de tronco incluindo-se desde o controle de cabeça até controle de tronco total em que o paciente permanece sem apoio. Controle estático, ativa e reativa de postura do tronco foram avaliados em pacientes deambulantes e não-deambulantes. Para a confiabilidade entre os avaliadores, empregou-se a análise estatística Kappa. Quanto maior a pontuação na escala Vignos, maior a frequência de pacientes com DMD que apresentam pior controle de tronco. Pacientes deambulantes apresentaram controle de tronco melhor do que os pacientes não-deambulantes (p = 0,003). Houve forte confiabilidade entre avaliadores para a pontuação da escala SATCo.

Currents issues in cardiorespiratory care of patients with post-polio syndrome / Tópicos atuais no tratamento cardiorrespiratório de pacientes com síndrome pós-poliomielite

ABSTRACT Post-polio syndrome (PPS) is a condition that affects polio survivors years after recovery from an initial acute attack of the poliomyelitis virus. Most often, polio survivors experience a gradual new weakening in muscles that were previously affected by the polio infection. The actual incidence of cardiovascular diseases (CVDs) in individuals suffering from PPS is not known. However, there is a reason to suspect that individuals with PPS might be at increased risk. Method A search for papers was made in the databases Bireme, Scielo and Pubmed with the following keywords: post polio syndrome, cardiorespiratory and rehabilitation in English, French and Spanish languages. Although we targeted only seek current studies on the topic in question, only the relevant (double-blind, randomized-controlled and consensus articles) were considered. Results and Discussion Certain features of PPS such as generalized fatigue, generalized and specific muscle weakness, joint and/or muscle pain may result in physical inactivity deconditioning obesity and dyslipidemia. Respiratory difficulties are common and may result in hypoxemia. Conclusion Only when evaluated and treated promptly, somE patients can obtain the full benefits of the use of respiratory muscles aids as far as quality of life is concerned.

RESUMO Síndrome pós-polio (SPP) é uma condição que afeta sobreviventes da poliomielite aguda (PAA), anos após a recuperação de um ataque agudo inicial do vírus. Na maioria das vezes, os sobreviventes da polio começam a apresentar nova paresia gradual nos músculos que foram previamente afetados pela infecção. A incidência real de doenças cardiovasculares (DCV) em indivíduos que sofrem de SPP não é conhecida. Entretanto, há indícios para suspeitar de que sujeitos com SPP podem estar em maior risco. Método Realizou-se uma busca de artigos nas bases de dados: Bireme, Scielo e Pubmed, utilizando as seguintes palavras-chave: síndrome pós-poliomielite, função cardiorrespiratória e reabilitação, nos idiomas Inglês, Francês e Espanhol. Embora tenhamos selecionado um número expressivo de artigos, somente foram considerados os duplo-cegos, randomizados-controlados, além de consensos. Resultados e Discussão Certas características da SPP, tais como fadiga muscular, paresia, dor muscular e/ou articulares podem resultar em descondicionamento por inatividade física, além de obesidade e dislipidemia. Dificuldades respiratórias são comuns e podem resultar em hipoxemia. Conclusão Somente quando avaliados e tratados em tempo hábil, alguns pacientes são capazes de obter os benefícios do uso dos músculos respiratórios auxiliares em termos de qualidade de vida.

Muscular weakness represents the main limiting factor of walk, functional independence and quality of life of myelopathy patients associated to HTLV-1 / Fraqueza muscular representa o principal fator limitante da marcha, independência funcional e qualidade de vida de pacientes com mielopatia associada ao HTLV-1

ABSTRACT HTLV-1-associated myelopathy is a progressive disabling disease associated with gait abnormalities. Objective To identify and quantify the main muscles affected by weakness and spasticity, their impact on gait, functional capacity and on quality of life of HTLV-1-associated myelopathy patients. Method We evaluated lower limbs muscular strength according to the Medical Research Council scale, spasticity according to the modified Ashworth scale, daily activities according to the Barthel Index and quality of life according to the Short-Form Health Survey-36 of 26 HTLV-1-associated myelopathy patients. Results The muscles most affected by weakness included the dorsal flexors and knee flexors. Spasticity predominated in the hip adductor muscles and in plantar flexors. Assistance for locomotion, minimal dependence in daily activities, limitations in functional capacity and physical aspects were the most common findings. Conclusion The impairment of gait, functional dependence and quality of life were predominantly a consequence of intense muscle weakness in HTLV-1-associated myelopathy patients.

RESUMO Mielopatia associada ao HTLV-1 é uma doença inflamatória, incapacitante e progressiva que acomete o sistema nervoso central. Objetivo Identificar e quantificar os principais músculos comprometidos pela fraqueza e espasticidade, o impacto na capacidade funcional e na qualidade de vida dos pacientes com mielopatia associada ao HTLV-1. Método Força muscular ( Medical Research Council), espasticidade (escala Ashworth modificada), atividades de vida diária (Índice de Barthel) e qualidade de vida ( Short-Form Health Survey-36) foram avaliados em 26 pacientes . Resultados Os principais músculos comprometidos pela fraqueza incluíram os flexores dorsais e flexores do joelho. A espasticidade predominou nos músculos adutores do quadril e nos flexores plantares. Assistência para locomoção, dependência mínima nas atividades diárias, limitações na capacidade funcional e os aspectos físicos representaram os achados mais frequentes. Conclusão Dificuldade de deambulação, dependência funcional e prejuízo na qualidade de vida foram as principais consequências da intensa fraqueza muscular nos pacientes com mielopatia associada ao HTLV-1.

Current issues in the respiratory care of patients with amyotrophic lateral sclerosis / Tópicos atuais no tratamento respiratório de pacientes com esclerose lateral amiotrófica

Amyotrophic lateral sclerosis is a progressive neuromuscular disease, resulting in respiratory muscle weakness, reduced pulmonary volumes, ineffective cough, secretion retention, and respiratory failure. Measures as vital capacity, maximal inspiratory and expiratory pressures, sniff nasal inspiratory pressure, cough peak flow and pulse oximetry are recommended to monitor the respiratory function. The patients should be followed up by a multidisciplinary team, focused in improving the quality of life and deal with the respiratory symptoms. The respiratory care approach includes airway clearance techniques, mechanically assisted cough and noninvasive mechanical ventilation. Vaccination and respiratory pharmacological support are also recommended. To date, there is no enough evidence supporting the inspiratory muscle training and diaphragmatic pacing.

Esclerose lateral amiotrófica é uma doença neuromuscular progressiva que resulta em fraqueza muscular, redução dos volumes pulmonares, tosse ineficaz, retenção de secreção e insuficiência respiratória. Medidas como a capacidade vital, pressão inspiratória e pressão expiratória máximas, pressão inspiratória máxima nasal, pico de fluxo de tosse e oximetria de pulso são recomendados para monitorar a função respiratória. Os pacientes devem ser acompanhados por uma equipe multidisciplinar, buscando melhorias na qualidade de vida e melhores estratégias para lidar com os sintomas respiratórios. A abordagem de cuidados respiratórios inclui técnicas de desobstrução das vias respiratórias, tosse assistida mecanicamente e ventilação mecânica não invasiva. Vacinação e suporte farmacológico também são recomendados. Até o momento, não existem provas suficientes que suportam o treinamento muscular inspiratório e a estimulação diafragmática.

Factors associated with inspiratory muscle weakness in patients with HIV-1

Background: the impact of human immunodeficiency virus type 1 (HIV-1) on lung function is well known and associated with a reduction in pulmonary ventilation. Moreover, the use of highly active antiretroviral therapy has been associated with mitochondrial dysfunction and decreased muscle strength. However, there is scarce information about the factors associated with inspiratory muscle weakness in these patients. Objective: the purpose of the present study was to investigate the factors associated with inspiratory muscle weakness in patients with HIV-1. Methods: two-hundred fifty seven patients with HIV-1 were screened and categorized into two groups: (1) IMW+ (n = 142) and (2) IMW-(n = 115). Lung function (FEV1, FVC and FEV1 /FVC), maximum inspiratory pressure, distance on the six-minute walk test and CD4 cell count were assessed. Results: the mean duration of HIV infection was similar in the two groups. The following variables were significantly different between groups: mean duration of highly active antiretroviral therapy (81 ± 12 in IMW+ versus 38 ± 13 months in IMW-; p = 0.01), and CD4 cell count (327 ± 88 in IMW+ versus 637 ± 97 cells/mm3 in IMW-; p = 0.02). IMW+ presented reduced lung function (FEV1, FVC, FEV1/FVC). Conclusion: patients with IMW+ had lower distance on the six-minute walk test in comparison to the IMW- group. The duration of highly active antiretroviral therapy, distance traveled on the 6MWT and CD4 count were determinants of IMW in patients with HIV. .

Clinical Characteristics and Genotype-Phenotype Correlation of Korean Patients with Spinal and Bulbar Muscular Atrophy

PURPOSE: Spinal and bulbar muscular atrophy (SBMA) is an X-linked motor neuron disease characterized by proximal muscle weakness, muscle atrophy, and fasciculation. Although SBMA is not uncommon in Korea, there is only one study reporting clinical characteristics and genotype-phenotype correlation in Korean patients. MATERIALS AND METHODS: In this study, age at the onset of symptoms, the score of severity assessed by impairment of activities of daily living milestones, and rate of disease progression, and their correlations with the number of CAG repeats in the androgen receptor (AR) gene, as well as possible correlations among clinical characteristics, were analyzed in 40 SBMA patients. RESULTS: The median ages at onset and at diagnosis were 44.5 and 52.5 years, respectively, and median interval between onset and diagnosis and median rate of disease progression were 5.0 years and 0.23 score/year, respectively. The median number of CAG repeats in the AR gene was 44 and the number of CAG repeats showed a significant inverse correlation with the age at onset of symptoms (r=-0.407, p=0.009). In addition, patients with early symptom onset had slower rate of disease progression. CONCLUSION: As a report with the largest and recent Korean cohort, this study demonstrates clinical features of Korean patients with SBMA and reaffirms the inverse correlation between the age at disease onset and the number of CAG repeats. Interestingly, this study shows a possibility that the rate of disease progression may be influenced by the age at onset of symptoms.

Factors associated with loss of handgrip strength in long-lived elderly / Factores asociados con la reducción de la fuerza de agarre manual en ancianos longevos / Fatores associados à diminuição de força de preensão manual em idosos longevos

Objective To investigate the prevalence of reduced grip strength and associated factors in long-lived elderly, who are users of primary health care. Method Cross-sectional quantitative study, data were collected during the period of January to December of 2013, by applying tests and questionnaires. The convenience sampling was comprised of 157 seniors. Results The findings indicate that the reduction in grip strength presents a moderate prevalence (25.5%), predominantly among females (19.1%), in the age group of 80-89 years (18.5%) and in those with lower educational levels (15.9%). The association between reduced grip strength and the variables of age and body mass index showed a statistical significance. Conclusion Investigations about the handgrip strength are essential for identifying clinical conditions of Brazilian long-lived elderly, and contribute to the development of plans towards the management of frailty. .

Objetivo Investigar a prevalência da redução da força de preensão manual e fatores associados em idosos longevos, usuários da atenção básica de saúde. Método Estudo quantitativo transversal, cujos dados foram coletados no período de janeiro a dezembro de 2013, por meio de testes e aplicação de questionários. A amostra por conveniência compreendeu 157 idosos. Resultados Apontam que a redução da força de preensão manual possui prevalência moderada (25,5%), com predomínio do sexo feminino (19,1%), da faixa etária de 80 a 89 anos (18,5%) e baixa escolaridade (15,9%). A associação entre diminuição da força de preensão manual e as variáveis idade e índice de massa corpórea mostrou significância estatística. Conclusão As investigações sobre o componente da força de preensão manual são fundamentais para a identificação das condições clínicas dos idosos longevos brasileiros e contribuem para a construção de planos voltados para a gestão da fragilidade. .

Objetivo Investigar la prevalencia de la reducción de la fuerza de agarre manual y los factores asociados en ancianos longevos, usuarios de la atención básica a la salud. Método Estudio cuantitativo transversal, cuyos datos fueron recogidos en el período de enero a diciembre de 2013, por medio de pruebas y aplicación de cuestionarios. La muestra por conveniencia comprendió a 157 ancianos. Resultados Señalan que la reducción de la fuerza de agarre manual tiene prevalencia moderada (25,5%), con predominio del sexo femenino (19,1%), del rango de edad de 80 a 89 años (18,5%) y baja escolaridad (15,9%). La asociación entre la disminución de la fuerza de agarre manual y las variables de edad e índice de masa corpórea mostró significación estadística. Conclusión Las investigaciones acerca del componente de la fuerza de agarre manual son fundamentales para la identificación de las condiciones clínicas de los ancianos longevos brasileños y contribuyen para la construcción de planes dirigidos a la gestión de la fragilidad. .

Compensatory movements during functional activities in ambulatory children with Duchenne muscular dystrophy / Movimentos compensatorios durante atividades funcionais em criancas deambuladoras com distrofia muscular de Duchenne

Objective: During the transitional phase (ambulatory to non-ambulatory), synergies characterize the evolution of Duchenne muscular dystrophy (DMD). This study was performed to describe and quantify compensatory movements while sitting down on/rising from the floor and climbing up/down steps. Method: Eighty videos (5 children × 4 assessments × 4 tasks) were recorded quarterly in the year prior to gait loss. Compensatory movements from the videos were registered based on the Functional Evaluation Scale for DMD. Results: The most frequently observed compensatory movements were upper limb support on lower limbs/floor/handrail during all the tasks and lumbar hyperlordosis, trunk support on handrail, equinus foot, increased base of support, non-alternated descent, and pauses while climbing up/down steps. Conclusion: Climbing up/down steps showed a higher number of compensatory movements than sitting down on/rising from the floor, which seemed to be lost before climbing up/down steps in ambulatory children with DMD. .

Objetivo: Durante a fase de transição (deambulador – não-deambulador), sinergias musculares caracterizam a evolução da distrofia muscular de Duchenne (DMD). Este estudo visou descrever e quantificar os movimentos compensatórios durante o sentar/levantar do solo, subir/descer degraus. Oitenta vídeos (5 crianças × 4 avaliações × 4 tarefas) foram gravados trimestralmente durante o ano que antecedeu a perda da marcha. Método: Os movimentos compensatórios dos vídeos foram registrados utilizando a Escala de Avaliação Funcional para DMD. Resultados: Os movimentos compensatórios mais frequentemente observados foram apoio de membros superiores nos membros inferiores/solo/corrimão durante todas as tarefas funcionais e hiperlordose lombar, apoio de tronco no corrimão, pés equinos, aumento da base de suporte, descida não alternada e pausas ao subir/descer degraus. Subir/descer degraus apresentou um número maior de movimentos compensatórios do que sentar/levantar do solo. Conclusão: Sentar/levantar do solo foram habilidades perdidas antes de subir/descer degraus em crianças com DMD. .

Monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of pulmonary function evaluation in amyotrophic lateral sclerosis / Monitoramento da força muscular respiratória em detrimento do uso isolado da avaliação da função pulmonar para o diagnóstico precoce de disfunção respiratória em pacientes com esclerose lateral amiotrófica

It was study the relationship between respiratory muscle strength and forced vital capacity (FVC) in patients with amyotrophic lateral sclerosis (ALS) versus healthy subjects. Pulmonary function and respiratory muscle strength [maximal inspiratory (PImax), maximal expiratory (PEmax) and sniff nasal inspiratory pressure (SNIP)] were assessed in patients with ALS and healthy subjects, matched using cutoffs established in the literature for impaired pulmonary function and respiratory muscle weakness. Twenty-eight ALS patients and 28 healthy subjects were studied. We found sensitivity and specificity for PImax, PEmax and SNIP of 75/58%, 81/67% and 75/67%. The Receiver Operating Characteristic curve (ROC curve) indicated that the variables PImax, PEmax and SNIP can identify differences in respiratory muscle strength between ALS and healthy individuals at 0.89, 0.9 and 0.82, respectively. A positive correlation was recorded between FVC (%) versus SNIP, PImax and PEmax. In ALS, monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of FVC. .

Estudar a relação entre a força dos músculos respiratórios e a capacidade vital forçada (CVF) em pacientes com esclerose lateral amiotrófica (ELA) e sujeitos saudáveis. Avaliamos a função pulmonar e a força dos músculos respiratórios [pressão inspiratória (PImax), pressão expiratória (PEmax) e pressão inspiratória nasal de sniff (SNIP)] utilizando pontos de corte estabelecidos na literatura para diagnóstico de fraqueza muscular respiratória. Foram estudados 28 pacientes com ELA e 28 sujeitos saudáveis. Encontramos sensibilidade e especificidade para PImax, PEmax e SNIP de 75/58%, 81/67% e 75/67%. A curva ROC (Receiver Operating Characteristic) indicou que as variáveis PImax, PEmax e SNIP podem identificar diferenças na força dos músculos respiratórios em pacientes com ELA versus sujeitos saudáveis em 0,89, 0,9 e 0,82 respectivamente. Foi encontrada uma correlação positiva entre CVF (%) e SNIP, PImax e PEmax. Em pacientes com ELA, o monitoramento da força muscular respiratória auxilia no diagnóstico precoce da disfunção em detrimento do uso da CVF isolada. .

Ataxia crónica en pediatría / [Chronic ataxia in childhood].

Chronic ataxias are an heterogeneous group of disorders that affect the child at different ages. Thus, the congenital forms, generally non progressive are observed from first months of life and are expressed by hypotonia and motor delay long before the ataxia became evident. The cerebral magnetic resonance images (MRI) may be diagnostic in some pictures like Joubert syndrome. The group of progressive hereditary ataxias, usually begin after the infant period. The clinical signs are gait instability and ocular apraxia that can be associated with oculocutaneous telangiectasias (ataxia-telangiesctasia) or with sensory neuropathy (Friedreich ataxia). In this review are briefly described congenital ataxias and in more detailed form the progressive hereditary ataxias autosomal recessive, autosomal dominants and mitochondrials. The importance of genetic study is emphasized, because it is the key to obtain the diagnosis in the majority of these diseases. Although now there are no treatments for the majority of progressive hereditary ataxias, some they have like Refsum disease, vitamine E deficiency, Coenzyme Q10 deficiency and others, thus the diagnosis in these cases is even more important. At present the diagnosis of childhood hereditary ataxia not yet treatable is fundamental to obtain suitable handling, determine a precise outcome and to give to the family an opportune genetic counseling.

Predicted preoperative maximal static respiratory pressures in adult cardiac surgeries: evaluation of two formulas / Pressões respiratórias máximas no pré-operatório de cirurgias cardíacas em adultos: avaliação de duas fórmulas

OBJECTIVES: Cardiac surgery (CC) determines systemic and pulmonary changes that require special care. Awareness of the importance of respiratory muscle dysfunction in the development of respiratory failure motivated several studies conducted in healthy subjects to assess muscle strength. These studies were carried out by evaluating the maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) values. This study examined the concordance among the values predicted by the equations proposed by Black & Hyatt and Neder, and the measured values in cardiac surgery (CS) patients. METHODS: Data were collected from preoperative evaluation forms. The Lin coefficient and Bland-Altman plots were used for statistical concordance analysis. The multiple linear regression and analysis of variance (ANOVA) were used to produce new formulas. RESULTS: There were weak correlations of 0.22 and 0.19 in the MIP analysis and of 0.10 and 0.32 in the MEP analysis, for the formulas of Black & Hyatt and Neder, respectively. The ANOVA for both MIP and MEP were significant (P <0.0001), and the following formulas were developed: MIP = 88.82 - (0.51 x age) + (19.86 x gender), and MEP = 91.36 - (0.30 x age) + (29.92 x gender). CONCLUSIONS: The Black and Hyatt and Neder formulas predict highly discrepant values of MIP and MEP and should not be used to identify muscle weakness in CS patients.

OBJETIVOS: A cirurgia cardíaca (CC) determina alterações que demandam cuidados específicos no pós-operatório, incluindo as alterações pulmonares. A consciência da importância da disfunção da musculatura respiratória na insuficiência respiratória motivou o desenvolvimento de diversos estudos da força muscular em indivíduos saudáveis. Esses trabalhos utilizam valores de pressão inspiratória máxima (PIMÁX) e pressão expiratória máxima (PEMÁX). O presente estudo avaliou a concordância existente entre os valores preditos pelas equações propostas por Black & Hyatt e Neder et al., com valores observados em pacientes submetidos à CC. MÉTODOS: Os dados foram coletados das fichas de avaliação pré-operatória. Para a análise estatística verificou-se a concordância existente entre os valores preditos e observados pelas as equações de Black & Hyatt e Neder et al., sendo utilizado o coeficiente de concordância de Lin e o gráfico de Bland-Altman. Posteriormente, os dados foram submetidos à regressão linear múltipla e análise de variância, para proposição de novas fórmulas. RESULTADOS: Para PIMÁX, observou-se fraca concordância de 0,22 e 0,19 e para PEMÁX, 0,10 e 0,32, respectivamente, para as fórmulas de Black & Hyatt e Neder et al. Os valores da ANOVA para PIMÁX e PEMÁX, foram significativas (P<0,0001), permitindo propor as seguintes fórmulas: PIMÁX = 88,82 - (0,51 x Idade) + (19,86 x Sexo), e para PEMÁX = 91,36 - (0,30 x Idade) + (29,92 x Sexo). CONCLUSÃO: As fórmulas de Black e Hyatt e Neder et al. predizem valores de PIMÁX e PEMÁX discrepantes, não devendo ser utilizadas para identificar fraqueza muscular em pacientes submetidos a cirurgia cardíaca.

Parálisis flacida aguda, una presentación atípica del sindrome de Guillain Barre / Acute flaccid paralysis, an atypical presentation of Guillain Barre Syndrome

El s índrome de Guillain Barr é es una polineuropatia postinfecciosa que afecta principalmente a los nervios motores, pero en ocasiones afecta tambi én a los nervios sensitivos y aut ónomos. Este s índrome se puede presentar en personas de todas las edades y no es hereditario, su diagnó stico es esencialmente descriptivo siendo muy importante una buena anamnesis y un buen examen f ísico apoy ándose en estudios de conducci ón nerviosa. El objetivo de esta publicaci ón es la presentaci ón de un caso clínico de un paciente pe diátrico que ingresa a la unidad de cuidados intensivos del IHSS con una forma de presentaci ón at ípica de esta patologí a y una evoluci ón y deterioro clínico r ápido. ...(AU)

Tremor in X-linked recessive spinal and bulbar muscular atrophy (Kennedy's disease)

OBJECTIVE: To study tremor in patients with X-linked recessive spinobulbar muscular atrophy or Kennedy's disease. METHODS: Ten patients (from 7 families) with a genetic diagnosis of Kennedy's disease were screened for the presence of tremor using a standardized clinical protocol and followed up at a neurology outpatient clinic. All index patients were genotyped and showed an expanded allele in the androgen receptor gene. RESULTS: Mean patient age was 37.6 years and mean number of CAG repeats 47 (44-53). Tremor was present in 8 (80 percent) patients and was predominantly postural hand tremor. Alcohol responsiveness was detected in 7 (88 percent) patients with tremor, who all responded well to treatment with a β-blocker (propranolol). CONCLUSION: Tremor is a common feature in patients with Kennedy's disease and has characteristics similar to those of essential tremor.

Phenotypic and immunohistochemical characterization of sarcoglycanopathies

INTRODUCTION: Limb-girdle muscular dystrophy presents with heterogeneous clinical and molecular features. The primary characteristic of this disorder is proximal muscular weakness with variable age of onset, speed of progression, and intensity of symptoms. Sarcoglycanopathies, which are a subgroup of the limb-girdle muscular dystrophies, are caused by mutations in sarcoglycan genes. Mutations in these genes cause secondary deficiencies in other proteins, due to the instability of the dystrophin-glycoprotein complex. Therefore, determining the etiology of a given sarcoglycanopathy requires costly and occasionally inaccessible molecular methods. OBJECTIVE: The aim of this study was to identify phenotypic differences among limb-girdle muscular dystrophy patients who were grouped according to the immunohistochemical phenotypes for the four sarcoglycans. METHODS: To identify phenotypic differences among patients with different types of sarcoglycanopathies, a questionnaire was used and the muscle strength and range of motion of nine joints in 45 patients recruited from the Department of Neurology - HC-FMUSP (Clinics Hospital of the Faculty of Medicine of the University of São Paulo) were evaluated. The findings obtained from these analyses were compared with the results of the immunohistochemical findings. RESULTS: The patients were divided into the following groups based on the immunohistochemical findings: a-sarcoglycanopathies (16 patients), b-sarcoglycanopathies (1 patient), y-sarcoglycanopathies (5 patients), and nonsarcoglycanopathies (23 patients). The muscle strength analysis revealed significant differences for both upper and lower limb muscles, particularly the shoulder and hip muscles, as expected. No pattern of joint contractures was found among the four groups analyzed, even within the same family. However, a high frequency of tiptoe gait was observed in patients with a-sarcoglycanopathies, while calf pseudo-hypertrophy was most common in patients with non-sarcoglycanopathies. The a-sarcoglycanopathy patients presented with more severe muscle weakness than did y-sarcoglycanopathy patients. CONCLUSION: The clinical differences observed in this study, which were associated with the immunohistochemical findings, may help to prioritize the mutational investigation of sarcoglycan genes.

Deep breathing heart rate variability is associated with respiratory muscle weakness in patients with chronic obstructive pulmonary disease

BACKGROUND: A synchronism exists between the respiratory and cardiac cycles. However, the influence of the inspiratory muscle weakness in chronic obstructive pulmonary disease (COPD) on cardiac autonomic control is unknown. The purpose of the present investigation was to evaluate the influence of respiratory muscle strength on autonomic control in these patients. METHODS: Ten chronic obstructive pulmonary disease patients (69±9 years; FEV1/FVC 59±12 percent and FEV1 41±11 percent predicted) and nine age-matched healthy volunteers (64±5 years) participated in this study. Heart-rate variability (HRV) was obtained at rest and during respiratory sinusal arrhythmia maneuver (RSA-M) by electrocardiograph. RESULTS: Chronic obstructive pulmonary disease patients demonstrated impaired cardiac autonomic modulation at rest and during RSA-M when compared with healthy subjects (p<0.05). Moreover, significant and positive correlations between maximal inspiratory pressure (MIP) and the inspiratory-expiratory difference (ΔIE) (r = 0.60, p<0.01) were found. CONCLUSION: Patients with chronic obstructive pulmonary disease presented impaired sympathetic-vagal balance at rest. In addition, cardiac autonomic control of heart rate was associated with inspiratory muscle weakness in chronic obstructive pulmonary disease. Based on this evidence, future research applications of respiratory muscle training may bring to light a potentially valuable target for rehabilitation.

Função pulmonar e força muscular respiratória em pacientes com doença renal crônica submetidos à hemodiálise / Pulmonary function and respiratory muscle strength in chronic renal failure patients on hemodialysis

OBJETIVO: Avaliar a função pulmonar e a força muscular respiratória de pacientes com doença renal crônica e correlacioná-las com a variação de peso ligada à realização de hemodiálise; estudar a correlação entre o tempo de hemodiálise e possíveis alterações respiratórias. MÉTODOS: Foram avaliados 17 pacientes (mediana de idade, 47 anos; intervalo interquartílico, 41-52 anos) submetidos a três sessões semanais de hemodiálise (mediana de tempo, 27 meses; intervalo interquartílico, 14-55). Doze eram do sexo masculino. Realizaram espirometria e mensuração das pressões máximas inspiratória (PImáx) e expiratória (PEmáx) antes e após a primeira sessão semanal de hemodiálise. O peso corporal foi quantificado antes e após as três sessões semanais. RESULTADOS: Oito pacientes apresentaram distúrbio restritivo leve antes da primeira sessão de hemodiálise. Desses, 2 normalizaram após a sessão. Houve aumento da capacidade vital forçada (p = 0,02) e diminuição de peso (p = 0,0001) ao final da primeira sessão semanal. A variação de peso durante três dias sem hemodiálise tendeu a se correlacionar com a variação da capacidade vital forçada na primeira sessão (r = 0,47; p = 0,055). O tempo de hemodiálise correlacionou-se com os valores da porcentagem do predito da PImáx (r = -0,53; p = 0,03) e com a PEmáx (r = -0,63; p = 0,006) pré-diálise. CONCLUSÕES: O maior ganho de peso no período interdialítico está associado com a piora da função pulmonar, que pode ser quase totalmente revertida com hemodiálise. Além disso, o maior tempo de hemodiálise está associado à diminuição da força muscular respiratória.

OBJECTIVE: To evaluate pulmonary function and respiratory muscle strength in chronic renal failure patients, correlating these variables with hemodialysis-related weight fluctuation; to study the correlation between the duration hemodialysis and potential respiratory alterations. METHODS: Seventeen patients (median age, 47 years; interquartile range, 41-52 years), submitted to three weekly hemodialysis sessions for a median of 27 months (interquartile range, 14-55) were evaluated. Twelve of the patients were male. The patients underwent spirometry. Maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) were measured prior to and after the first hemodialysis session of the week. Body weight was quantified prior to and after each of the three weekly sessions. RESULTS: Before the first hemodialysis session of the week, 8 patients presented mild restrictive defect, which normalized after the session in 2 of those patients. After dialysis, there was a significant increase in forced vital capacity (p = 0.02) and a significant decrease in body weight (p = 0.0001). Weight fluctuation over 3 days without hemodialysis tended to correlate with the variation in forced vital capacity in the first weekly session (r = 0.47; p = 0.055). Duration of hemodialysis correlated with predialysis MIP (r = -0.3; p = 0.03) and MEP (r = -0.63; p = 0.006). CONCLUSIONS: More pronounced weight gain in the interdialytic period is associated with worsening of lung function, which is almost fully reversible by hemodialysis. In addition, longer duration of hemodialysis is associated with decreased respiratory muscle strength.