Miopatias com padrão distal: descrição clínica de pacientes do Estado da Bahia / Distal myopathies: clinical description of patients from the State of Bahia

INTRODUÇÃO: As miopatias são doenças cuja etiologia decorre de alterações estruturais e/ou funcionais no músculo esquelético. As miopatias distais são doenças musculares primárias em que fraqueza e, frequentemente atrofia, tem início nas mãos, antebraços, pés e segmento distal das pernas. Apesar de terem sido divididas como um grupo restrito de doenças, outras miopatias podem se manifestar com um padrão distal, como a miopatia nemalínica e as distrofias musculares cintura-membros. Devido à escassez de trabalhos que descrevem clinicamente as miopatias distais, este trabalho visou contribuir com essa caracterização. METODOLOGIA: Os pacientes foram selecionados no ambulatório de doenças neuromusculares do Hospital Universitário Professor Edgar Santos, em seguida avaliados clinicamente, através de exame físico e também com exames complementares: eletroneuromiografia, exames laboratoriais, estudo molecular e histopatológico. RESULTADOS: Quinze pacientes com padrão distal foram analisados, sendo 40% do sexo feminino, média de idade de 29,8 anos, seis (40|%) pacientes naturais da capital, Salvador-Bahia. Quanto ao padrão de distribuição de fraqueza, sete apresentavam padrão distal, enquanto oito, padrão distal-proximal. Os pacientes foram agrupados de acordo com a idade de início dos sintomas, sendo 11 iniciados na infância e adolescência (T em homozigose), um com sarcoglicanopatia (mutação c.229C>T em homozigose) e um com miopatia nemalínica (histopatológico com presença de corpos nemalínicos). DISCUSSÃO: Os achados identificados nos pacientes com diagnósticos firmados foram compatíveis com o que é visto na literatura, como apresentação clínica e mutações identificadas previamente. Destaca-se o componente distal pronunciado da paciente com sarcoglicanopatia, considerado incomum. Além disso, a descrição da ressonância magnética realizada nos indivíduos demonstrou um padrão típico. Na maior parte dos pacientes não se chegou a um diagnóstico etiológico, a despeito da investigação realizada com os exames complementares e clínicos. CONCLUSÃO: O presente estudo caracterizou uma amostra de pacientes com miopatias distais, corroborando que essas doenças se manifestam clinicamente de forma heterogênea. A caracterização e divisão entre grupos visa tornar mais fácil a investigação, devendo ser feita com exames complementares, considerados imprescindíveis para se estabelecer o diagnóstico etiológico dessas doenças

INTRODUCTION: Myopathies are diseases which etiology results from structural and/or functional changes in skeletal muscle. Distal myopathies are a group of muscular pathologies in which weakness and atrophy begins and predominates in distal limbs, like hands and feet. Although it has been divided as a restrict group of diseases, other myopathies can manifest with that pattern of weakness, such nemaline myopathy and limb-girdle muscular dystrophies. Due to the scarcity of studies that described clinically the distal myopathies, this study focuses on clinical characterization of myopathies with distal pattern of weakness. METHODOLOGY: The patients were selected in the outpatient clinic for neuromuscular diseases at Professor Edgar Santos University Hospital. Those subjects were clinically evaluated through physical examination, laboratory tests, electroneuromyography, magnetic resonance (MRI) and histopathological study. RESULTS: Fifteen patients with distal pattern were analyzed, being 40% female, mean age 29.8 years, six (40 %) patients were born in the capital, Salvador-Bahia. As for the pattern of weakness distribution, seven had an exclusive distal pattern, while eight had a distal-proximal pattern. Patients were grouped according to the age of onset of symptoms, of which 11 were initiated in childhood and adolescence ( T in homozygous in exon 53 in another and one patient were diagnosed by biopsy), one with sarcoglicanopathy (mutation c.229C> T in homozygous) and one with nemaline myopathy (histopathological with the presence of nemalinic bodies). DISCUSSION: The findings identified in patients with established diagnoses were compatible with what is seen in the literature, such as clinical presentation and previously identified mutations. We highlight the pronounced distal component of the patient with sarcoglicanopathy, considered to be uncommon. In addition, the description of MRI performed in the individuals demonstrated a typical pattern. Most of the patients were not diagnosed, despite the research done with the complementary and clinical exams. CONCLUSION: The present study characterized a sample of patients with distal myopathies, corroborating that these diseases manifest themselves clinically heterogeneously. The characterization and division between groups aims to make the investigation easier, and should be done with complementary tests, considered essential to establish the etiological diagnosis of these diseases

Factores de riesgo biomecánicos y psicosociales presentes en la industria venezolana de la carne / Biomechanical and psychosocial risk factors in the Venezuelan meat processing industry

INTRODUCCIÓN: la identificación y evaluación de factores de riesgo relacionados a trastornos musculoesqueléticos (TME) representa la base para una efectiva adaptación de las condiciones de trabajo. OBJETIVO: evaluar los principales factores de riesgo biomecánico y psicosocial a los que se exponen trabajadores de la industria cárnica. Método: el estudio es descriptivo, transversal, basado en observación directa, encuestas y análisis de videos, para la aplicación de las metodologías RULA, OCRA, ecuación de NIOSH, ERGO y CoPsoQ-ISTAS21, sobre 71 tareas. RESULTADOS: el mayor riesgo postural recae en tareas de embutido y empaque, alcanzándose puntajes RULA de 7. En cuanto a repetitividad destaca el empaque de productos cocidos con 60% de sus tareas en nivel medio. Los mayores riesgos por levantamiento de cargas se ubicaron en las áreas de embutido, con índices inaceptables entre 25% y 50% de sus tareas. En empujes y arrastres de cargas, cinco de las seis áreas presentaron niveles inaceptables de riesgo. Los factores psicosociales de inseguridad, doble presencia, estima y exigencias psicológicas fueron percibidos negativamente por más del 50% de los trabajadores. DISCUSIÓN: entre las principales causantes de los altos niveles de riesgo destacan: levantamiento de productos por encima de hombros, realización de actividades manuales de pre-empaque y el empuje manual de contenedores pesados. CONCLUSIONES: el sector analizado reúne niveles de riesgo biomecánico y psicosocial que lo hace vulnerable a la aparición de TME.

INTRODUCTION: the identification and assessment of risk factors related to Musculoskeletal Disorders (MSD) is the basis for an effective adaptation of working conditions. OBJECTIVE: the aim is to evaluate the main biomechanical and psychosocial risk factors in workers of a meat processing industry. METHOD: the study is descriptive, transversal, based on direct observation, surveys and analysis of videos, for the application of the methodologies RULA, OCRA, NIOSH's equation, ERGO and CoPsoQ-ISTAS21, about 71 tasks. RESULTS: the greater postural risk is in task of inlay and packaging, which reach RULA's scores of 7. In terms of repeatability, the packaging of products cooked stand out with 60% of its tasks at moderate level. The greatest risks by lifting were located in the inlay areas, with unacceptable rates between 25% and 50% of its tasks. Five of the six areas showed unacceptable levels of risk in push and drag. The psychosocial factors: insecurity, double presence, esteem and psychological demands were perceived negatively by more than 50% of the workers. DISCUSSION: the main causes of the high levels of risk include: lifting of products above shoulders, manual activities of pre-packaging and the manual pushing of heavy containers. CONCLUSIONS: the meat industry has biomechanical and psychosocial risk levels that make it vulnerable to MSD.

Exercise training prevents skeletal muscle damage in an experimental sepsis model

OBJECTIVE: Oxidative stress plays an important role in skeletal muscle damage in sepsis. Aerobic exercise can decrease oxidative stress and enhance antioxidant defenses. Therefore, it was hypothesized that aerobic exercise training before a sepsis stimulus could attenuate skeletal muscle damage by modulating oxidative stress. Thus, the aim of this study was to evaluate the effects of aerobic physical preconditioning on the different mechanisms that are involved in sepsis-induced myopathy. METHODS: Male Wistar rats were randomly assigned to either the untrained or trained group. The exercise training protocol consisted of an eight-week treadmill program. After the training protocol, the animals from both groups were randomly assigned to either a sham group or a cecal ligation and perforation surgery group. Thus, the groups were as follows: sham, cecal ligation and perforation, sham trained, and cecal ligation and perforation trained. Five days after surgery, the animals were euthanized and their soleus and plantaris muscles were harvested. Fiber cross-sectional area, creatine kinase, thiobarbituric acid reactive species, carbonyl, catalase and superoxide dismutase activities were measured. RESULTS: The fiber cross-sectional area was smaller, and the creatine kinase, thiobarbituric acid reactive species and carbonyl levels were higher in both muscles in the cecal ligation and perforation group than in the sham and cecal ligation and perforation trained groups. The muscle superoxide dismutase activity was higher in the cecal ligation and perforation trained group than in the sham and cecal ligation and perforation groups. The muscle catalase activity was lower in the cecal ligation and perforation group than in the sham group. CONCLUSION: In summary, aerobic physical preconditioning prevents atrophy, lipid peroxidation and protein oxidation and improves superoxide dismutase activity in the skeletal muscles of septic rats.

Adaptação temporária de mão de obra (ATMO) / Temporary adaptation of workers

Este trabalho foi desenvolvido no intuito de facilitar e acelerar o processo de reabilitação dos funcionários de uma indústria automotiva, controlando aincidência e prevalência de casos de distúrbios osteomusculares junto ao quadro de funcionários da empresa, utilizando uma ferramenta denominada adaptação temporária de mão de obra. O aumento do conhecimento científico nas modernas condições das atividades laborativas e suas consequências na máquina humana estão permitindo as companhias trabalharem nas melhorias das condições de trabalho, objetivando propiciar uma melhor condição de vida ao colaborador. O estudo mostra resultados de redução do absenteísmo da fábrica através do monitoramento epidemiológico; redução dos dias perdidos de produção para a empresa além de ace4lerar o porcesso de reabilitação dos funcionários acometidos de algum problema osteomuscular

Tratamento da dermatomiosite juvenil / Treatment of juvenile dermatomyositis

O tratamento da Dermatomiosite Juvenil envolve uma abordagem racional das muitas manifestaçöes clínicas que podem estar presentes como a doença muscular, as alteraçöes cutâneas, o envolvimento de diversos orgäos e a calcinose. O tratamento com drogas é o mais importante mas näo se deve descuidar dos cuidados gerais, da fisioterapia na prevençäo e correçäo das deformidades e, às vezes, da cirurgia corretora de sequelas e alívio de calcinose. O início do controle da doença muscular deve ser feio com corticosteróides administrados por via oral ou endovenosa e, na falha destes, por imunossupressores ou gamaglobulina endovenosa. O texto também aborda as controvérsias a respeito do tratamento da pele e da calcinose, citando algumas opçöes que têm sido relatadas em literatura médica