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Sample records for adiposo-muscular estimados mediante

  1. GRASA CORPORAL E ÍNDICE ADIPOSO-MUSCULAR ESTIMADOS MEDIANTE IMPEDANCIOMETRÍA EN LA EVALUACIÓN NUTRICIONAL DE MUJERES DE 35 A 55 AÑOS

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    Vicente Martín Moreno

    2002-01-01

    Full Text Available Fundamentos: La evaluación nutricional durante la premenopausia y la menopausia tiene un papel relevante para valorar los cambios que acontecen en la mujer. El objetivo de este estudio es comparar los parámetros grasa corporal e índice adiposo- muscular corporal (IAMC estimados mediante impedanciometría respecto al índice de masa corporal (IMC en la definición del estado nutricional y la composición corporal. Métodos: Estudio descriptivo transversal. Mediante muestreo aleatorio (base de tarjeta sanitaria fueron seleccionadas 30 mujeres de 35 a 55 años, valorándose en ellas la asociación entre grasa corporal e IAMC con el IMC; diferentes estimadores de la distribución de la grasa corporal: circunferencias de la cintura y cresta ilíaca, cocientes cintura-cadera e ilíaca-cadera y diámetros ilíaco y sagital; presiones arteriales sistólica (PAS y diastólica (PAD y niveles plasmáticos de glucosa, colesterol, HDL-colesterol y triglicéridos. Resultados: El porcentaje de grasa corporal correlacionó intensamente (p 33% presentaba un IMC menor de 30. Conclusiones: La estimación de los parámetros grasa corporal e IAMC en mujeres de 35 a 55 años complementa la evaluación nutricional realizada mediante el IMC, aunque para definir su verdadero valor en esta evaluación es necesario establecer los parámetros de normalidad en la población.

  2. Grasa corporal e índice adiposo-muscular estimados mediante impedanciometría en la evaluación nutricional de mujeres de 35 a 55 años

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    Martín Moreno Vicente

    2002-01-01

    Full Text Available Fundamentos: La evaluación nutricional durante la premenopausia y la menopausia tiene un papel relevante para valorar los cambios que acontecen en la mujer. El objetivo de este estudio es comparar los parámetros grasa corporal e índice adiposo-muscular corporal (IAMC estimados mediante impedanciometría respecto al índice de masa corporal (IMC en la definición del estado nutricional y la composición corporal. Métodos: Estudio descriptivo transversal. Mediante muestreo aleatorio (base de tarjeta sanitaria fueron seleccionadas 30 mujeres de 35 a 55 años, valorándose en ellas la asociación entre grasa corporal e IAMC con el IMC; diferentes estimadores de la distribución de la grasa corporal: circunferencias de la cintura y cresta ilíaca, cocientes cintura-cadera e ilíaca-cadera y diámetros ilíaco y sagital; presiones arteriales sistólica (PAS y diastólica (PAD y niveles plasmáticos de glucosa, colesterol, HDL-colesterol y triglicéridos. Resultados: El porcentaje de grasa corporal correlacionó intensamente (p<0,001 con IMC (r=0,919, circunferencias de la cintura (r=0,866 e ilíaca (r=0,841 y diámetros sagital (r=0,783 e ilíaco (r=0,772, y, menos intensamente, con la glucemia (r=0,385; p=0,036 y la PAS (r=0,497; p=0,005 y PAD (r=0,582; p=0,001. El IAMC presentó similares resultados, sin asociarse significativamente con ningún parámetro bioquímico. El 46,7% de las mujeres obesas según su porcentaje de grasa corporal (33% presentaba un IMC menor de 30. Conclusiones: La estimación de los parámetros grasa corporal e IAMC en mujeres de 35 a 55 años complementa la evaluación nutricional realizada mediante el IMC, aunque para definir su verdadero valor en esta evaluación es necesario establecer los parámetros de normalidad en la población.

  3. Grado de acuerdo entre los índices adiposo-musculares obtenidos a partir de medidas antropométricas del brazo, pliegues cutáneos e impedancia bioeléctrica Interchangeability of the fat-to-fat-free mass ratios obtained by arm anthropometric measures, skinfold thickness and bioelectrical impedance

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    V. Martín Moreno; J. Benito Gómez Gandoy; M. de Oya Otero; Agustín Gómez de la Cámara; M.ª J. Antoranz González

    2003-01-01

    Objetivo: La masa grasa, la masa libre de grasa y la relación entre ambas, el índice adiposo-muscular, son parámetros útiles para valorar clínicamente al paciente. El objetivo de este estudio es medir el grado de acuerdo entre los índices adiposo-musculares corporales (IAMC) obtenidos mediante los pliegues cutáneos con la ecuación de Siri para cuatro pliegues (IAMC Siri) y para el pliegue del tríceps (IAMCtríceps); por bioimpedanciometría (IAMC Omron); y mediante las áreas adiposa y muscular ...

  4. Papel del colágeno VI en la homeostasis del músculo esquelético y tejido adiposo: implicaciones en la patofisiología de las distrofias musculares

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    Rodríguez García, Mª Ángeles

    2016-01-01

    [spa] El colágeno VI es una proteína de la matriz extracelular cuyos defectos conducen a una variedad de trastornos musculares en humanos caracterizados por una debilidad muscular entre otros múltiples síntomas. Tanto el colágeno VI, como su fragmento soluble carboxilo terminal llamado endotrofina se encuentran altamente expresados en los dos principales tejidos periféricos que regulan la glucemia, el músculo esquelético y tejido adiposo. Los niveles de ARNm de colágeno VI en el tejido adipos...

  5. De la biología al injerto de tejido adiposo: cómo mejorar el lipoinjerto

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    A.C. Girard

    2013-12-01

    Full Text Available A pesar de que el uso del injerto de grasa ha ganado popularidad, no hay consenso sobre la mejor manera de manejar el tejido adiposo. Los protocolos difieren y los resultados son a menudo variables. Diversos factores influyen en la calidad de la grasa inyectada, entre los que encontramos las moléculas tóxicas provenientes de la infiltración, procedimiento previo a la liposucción. En este trabajo, hemos confirmado el efecto nocivo de los anestésicos sobre las células madre derivadas del tejido adiposo, determinando el efecto del lavado y la centrifugación en el tejido graso con el fin de proponer un protocolo simple y optimizado para mejorar la supervivencia del injerto. Evaluamos la citotoxicidad de la lidocaína sobre las células madre derivadas de tejido adiposo (ADSC mediante ensayo de LDH. Sometimos el tejido adiposo conjunto a varios tipos de centrifugación (de 1 segundo a 10 minutos y desde 0 g a 1800 g, y el volumen de líquido y el aceite liberado se midió inmediatamente después de la centrifugación. Tras la determinación de las condiciones óptimas para la manipulación de tejidos (400 g/1 minuto, inyectamos el tejido adiposo de liposucción sin o con lidocaína en ratones inmunodeficientes. Un mes después de la inyección, evaluamos la calidad de los injertos mediante histología, y en comparación con los injertos obtenidos a partir de un protocolo convencional: una simple sedimentación. La lidocaína ejerce un efecto citotóxico sobre las ADSC, y este efecto depende del tiempo de incubación y de las concentraciones. En cuanto al tejido adiposo, una centrifugación intensa (900 g, 1800 g es perjudicial en comparación con una centrifugación suave (100 g, 400 g. Además, las secciones histológicas de los injertos de tejido adiposo no centrifugados mostraron la presencia de grandes vacuolas de aceite mientras que los injertos resultantes de lavado con protocolo de centrifugación suave (400g/1minuto no lo hacen. En

  6. Crescimento alométrico dos tecidos ósseo, muscular e adiposo na carcaça de cordeiros Santa Inês e Bergamácia Allometric growth of bone, muscular and fat tissues in carcass of Santa Ines and Bergamacia lambs

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    Cristiane Leal dos Santos

    2001-04-01

    Full Text Available O trabalho foi realizado com objetivo de realizar um estudo alométrico dos tecidos ósseo, muscular e adiposo na meia carcaça esquerda de cordeiros em crescimento. Foram utilizados 36 cordeiros machos inteiros, sendo 24 Santa Inês e 12 Bergamácia. Os animais foram confinados em gaiolas individuais e alimentados ad libitum. O abate ocorreu quando os animais atingiram os pesos vivos de 15, 25, 35 e 45 kg. Após a carcaça ter sido limpa e resfriada, foram obtidos os cortes comerciais a partir da meia carcaça esquerda. A quantidade dos diferentes tecidos foi obtida a partir da dissecação da perna, do lombo, da costeleta, costela/fralda e paleta. A quantidade total dos tecidos ósseo, muscular e adiposo foi obtida pela adição das quantidades de cada corte comercial. O estudo do desenvolvimento relativo da composição tecidual foi feito por meio do modelo de HUXLEY (1932. Constatou-se crescimento heterogônico negativo (b 1 para a gordura, em relação ao peso da ½ carcaça.The work was conducted with the objective to study the allometric growth of the bone, muscular and fat tissues in the left side of growing lamb carcasses. Thirty-six, twenty-four Santa Ines and twelve Bergamacia lambs were used. The animals were kept in individual pens and fed ad libitum diets. The slaughtering was done when the animals reached the live weight of 15, 25, 35 and 45 kg. The amount of the different tissues was obtained through the dissection of the left half of the carcass. The amount of the different tissues was obtained through the dissection of the leg, longissimus muscle, rib, breast and shoulder. The total amount of bone, muscle and fat tissues were obtained by the sum of each amount of commercial cut. The study of the relative tissue composition development was carried out using the model developed by HUXLEY (1932. A negative heterogonic (b 1 for the fat tissue in relation to the half side of the carcass was observed.

  7. The importance of mdx mouse in the pathophysiology of Duchenne's muscular distrophy

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    Seixas, Sandra Lopes; Lagrota-Cândido, Jussara; Savino, Wilson; Quirico-Santos, Thereza

    1997-01-01

    O camundongo mdx desenvolve distrofia muscular recessiva ligada ao cromossoma X (locus Xp21.1) e não expressa distrofina. Embora não apresente intensa fibrose do tecido muscular e acúmulo de tecido adiposo, é considerado o modelo animal mais adequado da distrofia muscular de Duchenne. As alterações estruturais no tecido muscular associadas à mionecrose e presença do infiltrado inflamatório com predomínio de linfócitos e monócitos/macrófagos sugerem uma participação do sistema imunológico nest...

  8. Modelamiento y simulación de la contracción muscular mediante la estimulación magnética externa

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    Bermeo Moyano, Juan Pablo; Sánchez Sánchez, Carlos Felipe

    2017-01-01

    El proyecto inicia revisando las ecuaciones de Maxwell, para simular y estimar las corrientes inducidas en un tejido muscular por un campo magnético mediante el MEF: M. Elementos Finitos. Se simula la fuerza muscular con el modelo de seis parámetros y optimizado por algoritmos genéticos para ajustar el modelo teórico con las medidas experimentales. The present project begins with the revision of the Maxwell equations, to simulate and estimate the currents induced in a muscle tissue by a ma...

  9. Desenvolvimento do tecido adiposo e muscular em suínos de raça Alentejana Development of adipose and muscular tissues in Alentejano pig breed

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    A. Freitas

    2007-01-01

    Full Text Available O presente trabalho, pretendeu estudar o efeito do peso de abate sobre a evolução do tecido adiposo e muscular de porcos Alentejanos destinados à produção de carne para consumo em fresco. Utilizaram-se 30 suínos de raça alentejana (sexos neutralizados alojados e alimentados individualmente com um alimento composto comercial equilibrado (3100 Kcal ED e 150g de Proteína Bruta por Kg, fornecido a 85% do Ad Libitum. Efectuaram-se abates sucessivos de 5 animais aos 40, 70, 80, 90, 100 e 110 kg de peso vivo. As carcaças foram refrigeradas a 4 ºC durante 24 horas e seguidamente procedeu-se à pesagem da gordura perirenal (banha e à medição da gordura subcutânea dorsal e da área do músculo Longissimus dorsi entre a décima e décima primeira costela, ao nível da última costela e entre a terceira e quarta vértebra lombar. O aumento do peso de abate influenciou significativamente (PTo study the effects of slaughter weight on adipose and muscular tissues development of Alentejano hogs thirty animals were used. With an average live weight of 35 kg, pigs were allocated in individual pens at outdoor and fed a commercial diet (15% CP and 3100 Kcal ED/kg offered at 85% Ad Libitum. Pigs were sequentially slaughtered in groups (n=5 with an average live weight of 40, 70,80, 90, 100 and 110 kg. After carcass chilling at 5º C for 24 h flare fat was weighed and subcutaneous backfat thickness and loin eye area were recorded at 1011 ribs, last rib and 3-4 lombar vertebras. Flare fat increased significantly (P<0,01 with the increase of slaughter weight: 1,26, 2,08, 3,22, 3,36, 3,68 e 4,02 Kg, respectively at 40, 70, 80, 90, 100 e 110 Kg. Subcutaneous back fat thickness also increased strongly with the increase of slaughter weight: 1,56, 3,21, 4,12, 5,10, 5,41 e 5,38 cm, respectively at 40, 70, 80, 90, 100 e 110 Kg. Between 90 and 110 kg averages were similar. The area of Longissimus dorsi increased slightly (15,49 to 20,36 cm² with the increase of

  10. Fracción vascular estromal de tejido adiposo: cómo obtener células madre y su rendimiento de acuerdo a la topografía de las áreas donantes: estudio preliminar

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    K. A. Almeida

    Full Text Available La obtención de tejido adiposo supone un nuevo y prometedor mercado de trabajo para los cirujanos plásticos, ya que los bancos de tejidos escogerán de forma acertada la grasa como el medio más fácil para obtener fuentes de células madre de alto rendimiento, en la medida en que este tejido es capaz de producir al menos cinco veces más unidades formadores de colonias (UFCs que la médula ósea. El objetivo del presente trabajo es mostrar lo que se puede esperar del tejido adiposo como origen de células adultas de fracción vacular estromal (FVE, y señalar las mejores áreas del cuerpo humano para ser elegidas como donantes de tejido adiposo, extraído mediante liposucción. Describimos la rutina seguida para la obtención de células de FVE mediante la digestión de las muestras de tejido adiposo humano con colagenasa. En el momento de su recolección, esas células presentaban una viabilidad de 92+/- 1% basada en exclusión por Azul de Trypan. Las células de FVE recontadas después de permanecer 48 horas en medio de cultivo de Eagle modificado por Dulbecco (DMEM, dentro de una cámara de Neubauer, tras lo cual el rendimiento medio de las células de FVE fue de 7,2 +/- 1,3 x 103 células por mililitro de tejido lipoaspirado. En conclusión, pensamos que supone un desafío en la actualidad el mejorar las estrategias para la obtención de células de FVE. Este trabajo, por ahora preliminar, muestra que las células de FVE pueden ser fácilmente obtenidas por medio de lipoaspiración. La comparación entre las diferentes áreas donantes, mostró un rendimiento 22% más alto para las células de FVE cuando el tejido adiposo había sido obtenido del tronco, en comparación a cuando lo había sido de los miembros.

  11. Propuesta de un nuevo puntaje para optimizar estimados ecográficos de peso fetal: estudio piloto

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    José Rojas Camayo

    2009-06-01

    Full Text Available Introducción: La estimación del peso fetal es importante para las decisiones obstétricas; la precisión de los estimados ecográficos no ha sido estudiada. Objetivos: Determinar la precisión del estimado ecográfico del peso fetal mediante un puntaje basado en parámetros clínicos, ecográficos y maternos. Diseño: Estudio de tipo transversal. Lugar: Hospital Nacional Guillermo Almenara Irigoyen, Lima, Perú. Participantes: 130 gestantes y sus fetos. Intervenciones: Se consignó datos clínicos, ecográficos y maternos. Se procedió a dicotomizar los aciertos entre menor o igual a ±10% (acertadas y mayor a ±10% (erradas del peso real. Se analizó las variables clínicas, ecográficas y maternas mediante el uso de las curvas ROC, para identificar aquellas que tuvieron más influencia en la precisión ecográfica. Con las variables identificadas, se procedió al desarrollo de un modelo de regresión logística, para establecer un puntaje que estimara la probabilidad de variación, teniendo como punto de corte ±10% del peso real. Principales medidas de resultados: Precisión de un puntaje que relaciona la edad gestacional con la circunferencia abdominal y el diámetro biparietal del feto, el ponderado clínico y el peso materno. Resultados: El nuevo puntaje tuvo un buena capacidad de discriminación de las ecografías, según su variación a ±10% del peso real (área bajo la curva ROC: 0,76 p<0,001. Según los puntajes obtenidos, se obtuvo tres grupos: rendimiento alto 20%, rendimiento convencional 44,4% y rendimiento bajo 71,2%. Conclusiones: El nuevo puntaje propuesto tiene la capacidad de estimar la probabilidad de variación de los estimados ecográficos mayores al ±10% del peso real, conformando tres grupos de rendimiento: alto, convencional y bajo.

  12. Características biológicas del tejido adiposo: el adipocito como célula endocrina

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    Reyes J. Marcela, Dra.

    2012-03-01

    Full Text Available La obesidad se caracteriza por un aumento de la masa adiposa secundaria a un balance energético positivo mantenido en el tiempo. El incremento en el volumen del tejido adiposo se acompaña de otros cambios en las características biológicas habituales de éste, que se vuelve disfuncional. El depósito visceral de la grasa, la hipertrofia y cambio del perfil secretor de los adipocitos, junto con la infiltración del tejido adiposo por células inflamatorias son algunas de las características que determinan una comunicación alterada del tejido adiposo con otros órganos. Se ha planteado que la disfunción del tejido adiposo, explicaría parte de la etiopatogenia de las enfermedades metabólicas y cardiovasculares asociadas a obesidad; sujetos obesos que mantienen un tejido adiposo funcional no presentan las alteraciones metabólicas propias de la malnutrición por exceso. La modulación de las características biológicas del tejido adiposo permitiría tener un menor riesgo cardiovascular asociado a obesidad.

  13. Diagnóstico diferencial das distrofias musculares com referência especial às alterações enzimáticas

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    H. Heick

    1967-06-01

    Full Text Available Depois de apresentar os dados clínicos e genéticos relativos a 221 casos de distrofia muscular progressiva (125 casos do tipo Duchenne, 63 do tipo cintura pélvica e 25 do tipo fácio-escápulo-umeral, os autores analisam os resultados das dosagens sôro-enzimáticas que fizeram; em alguns casos foram pesquisas também as atividades de 25 enzimas em homogeneizados de tecido muscular. Os mesmos exames foram feitos em casos de miopatias de outra etiologia e de distrofias musculares neuropáticas e mielopáticas. Foi verificado que o teor das sôro-enzimas aumenta em todas as formas de distrofia muscular progressiva hereditária. No tipo Duchenne as-atividades enzimáticas são elevadas desde o nascimento das crianças acometidas, precedendo o aparecimento da sintomatologia clínica. Com o progredir da moléstia e com o decorrer dos anos os teores sôro-enzimáticos tendem a diminuir, podendo reduzir-se aos valores normais nas fases finais da moléstia. No tecido muscular não existe diferença significante, no que respeita à composição enzimática, entre os diversos tipos de distrofia muscular progressiva e hereditária. Com exceção de poucas enzimas cuja atividade é maior nos tecidos conjuntivo e adiposo que progressivamente substituem o tecido muscular que entra em atrofia, o teor das mio-enzimas diminui com o progredir da moléstia, como expressão do crescente esgotamento do citoplasma da célula muscular. Nas distrofias musculares neuropáticas e mielopáticas as alterações são idênticas, embora de menor intensidade. Algumas diferenças quantitativas podem ser explicadas pela idade dos pacientes e pela intensidade do processo. A mesma diferença pode ser demonstrada mediante exames histopatológicos: nas formas que acometem crianças (Werdnig-Hoffmann as fibras musculares ainda não denervadas se apresentam com aspecto normal, ao passo que, nas formas do adulto (Kugelberg-Welander, possivelmente pelo desgaste provocado pelos esfor

  14. Free radial forearm adiposo-fascial flap for inferior maxillectomy defect reconstruction

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    Thankappan, Krishnakumar; Trivedi, Nirav P.; Sharma, Mohit; Kuriakose, Moni A.; Iyer, Subramania

    2009-01-01

    A free radial forearm fascial flap has been described for intraoral reconstruction. Adiposo-fascial flap harvesting involves few technical modifications from the conventional radial forearm fascio-cutaneous free flap harvesting. We report a case of inferior maxillectomy defect reconstruction in a 42-year-old male with a free radial forearm adiposo-fascial flap with good aesthetic and functional outcome with minimal primary and donor site morbidity. The technique of raising the flap and closing the donor site needs to be meticulous in order to achieve good cosmetic and functional outcome. PMID:19881028

  15. Free radial forearm adiposo-fascial flap for inferior maxillectomy defect reconstruction

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    Thankappan Krishnakumar

    2009-01-01

    Full Text Available A free radial forearm fascial flap has been described for intraoral reconstruction. Adiposo-fascial flap harvesting involves few technical modifications from the conventional radial forearm fascio-cutaneous free flap harvesting. We report a case of inferior maxillectomy defect reconstruction in a 42-year-old male with a free radial forearm adiposo-fascial flap with good aesthetic and functional outcome with minimal primary and donor site morbidity. The technique of raising the flap and closing the donor site needs to be meticulous in order to achieve good cosmetic and functional outcome.

  16. Epitelización inducida por células troncales derivadas del tejido adiposo

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    M. Meruane

    2014-06-01

    Full Text Available El tratamiento de lesiones con pérdida de tejido cutáneo ha mejorado notablemente con el advenimiento de la bioingeniería tisular. Una alternativa en desarrollo es la utilización de sustitutos dérmicos combinados con células troncales derivadas del tejido adiposo autólogo. Estudios previos nos muestran que con esta técnica es posible optimizar la angiogénesis y la síntesis de colágeno, sin embargo potenciar la epitelización es un tema pendiente por resolver. En el presente estudio evaluamos la progresión y diferenciación epitelial en un período de tiempo prologando. Obtuvimos las células troncales a partir del tejido adiposo (ASC de la región inguinal de 4 ratas Sprague Dawley. Cultivamos las células frescas en una matriz de Integra® durante un período total de 48 horas, y las marcamos con un vector lentiviral-GFP (proteína fluorescente verde. Posteriormente, injertamos en las mismas ratas la matriz dérmica con células troncales y un implante contralateral sin células, como control. A las 4 semanas, evaluamos el avance epitelial mediante planimetría de superficie e histología. Los resultados macroscópicos muestran que el cierre de la herida por contracción de los bordes no tiene diferencias significativas (82,63% ± 3,4% vs. 80,66% ± 3,89%; p=0,08, pero el cierre por epitelización fue significativamente mayor en el lado intervenido con ASCs (93,47% ± 5,98% vs. 79,88% ± 6,28%; p=0,0028. Todas las muestras obtuvieron tinción positiva para el anticuerpo anti-citoqueratina 34βE12 y el avance epitelial lineal cuantificado por microscopía resultó significativamente mayor en el lado con ASCs (6408 ± 275μm vs. 5375 ± 250μm; p < 0,001. Identificamos las células GFP positivas formando parte de la dermis regenerada, no así en la epidermis. En conclusión, las células troncales derivadas del tejido adiposo autólogo sembradas en una matriz de Integra® aumentan la formación epitelial significativamente

  17. Curva dos valores normais de peso fetal estimado por ultra-sonografia segundo a idade gestacional

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    José Guilherme Cecatti

    Full Text Available Este trabalho teve por objetivo avaliar a evolução do peso fetal estimado em gestações normais de 20 a 42 semanas. Quanto ao desenho e métodos empregados na pesquisa, realizou-se estudo descritivo de um universo constituído por 2.874 gestantes normais da cidade de Campinas, efetuando-se exame ultra-sonográfico obstétrico de rotina com medida da biometria fetal e utilizando-se, para o cálculo do peso fetal, a fórmula de Hadlock et al. (1991. Calcularam-se os valores dos percentis 10, 50 e 90 do peso fetal, por idade gestacional, com construção de curva posteriormente alisada mediante ajuste polinomial de 2º grau. Como resultado, obteve-se que o valor do percentil 50 foi de 368 gramas na 20ª semana, 1.512 gramas na 30ª semana, atingindo 3.417 gramas na 42ª semana. Constatou-se ter havido ganho médio de 200 gramas de peso por semana, de 27 a 38 semanas, a partir de quando o incremento diminuiu. Concluiu-se que a disponibilidade de curva brasileira do peso fetal estimado normal permitirá o diagnóstico pré-natal de seus desvios pela ultra-sonografia.

  18. Mecanismos de regulación del anabolismo lipídico en el tejido adiposo del paciente obeso

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    Ortega Delgado, Francisco José

    2012-01-01

    La obesidad es uno de los problemas de salud pública más importante. Los estudios de expresión aplicados a los depósitos de grasa en sujetos obesos han aportado importantes indicios sobre la fisiopatología del tejido adiposo. Los datos recogidos en esta tesis doctoral demuestran que la síntesis de grasa (lipogénesis) está disminuida en el tejido adiposo del paciente obeso, y describen el comportamiento de un nuevo factor lipogénicos. Se demuestra además que el tejido adiposo subcutáneo (situa...

  19. Cambios en el tono muscular en dos niños con parálisis cerebral espástica mediante la hipoterapia: reporte de casos

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    Yasmín Andrea Rodríguez Laiseca

    2015-06-01

    Full Text Available Introducción: Estudios previos han demostrado que la hipoterapia como terapia complementaria mejora la función motora gruesa en niños con parálisis cerebral. Sin embargo la evidencia no es concluyente. Objetivo: Determinar los cambios en el tono muscular de dos pacientes con parálisis cerebral tipo cuadriparesia espástica mediante la hipoterapia. Descripción del caso: El estudio se llevó a cabo con dos sujetos de género masculino de dos años de edad, con diagnóstico de parálisis cerebral tipo cuadriparesia espástica, nivel V según la escala Gross Motor Function Clasification System (GMFCS. Los niños realizaron 27 sesiones de hipoterapia distribuidas de la siguiente manera: 3 sesiones de acoplamiento en hipoterapia en la primera semana y 24  sesiones de monta terapéutica con una frecuencia de tres veces por semana y una intensidad de 20 minutos por sesión. Se llevó a cabo la valoración del tono muscular mediante la escala de Ashworth modificada (MAS de la siguiente forma: una inicial, dos de control, cuatro post-monta, una final tras 9 semanas de tratamiento continuo y tres mediciones más en la semana uno, dos y tres de inactividad terapéutica. Resultados y discusión: La hipoterapia demostró efecto modulador sobre el tono muscular de los dos sujetos finalizadas las 27 sesiones de hipoterapia, pero este se pierde progresivamente durante el periodo de inactividad terapéutica. Por lo tanto se confirma que esta técnica puede ser una estrategia de tratamiento para el manejo de la espasticidad en pacientes con parálisis cerebral, siempre y cuando se aplique continuamente.

  20. El tejido adiposo: algo más que un reservorio de energía

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    Sanhueza C, Julio

    2009-12-01

    Full Text Available Adipose tissue is a reservoir of energy and also an organ that contributes to the aesthetics and health of human body working as an endocrine tissue. White adipose tissue, which is formed by unilocular adipose cells, can modify organic homeostasis by controlling energy expenditure and consumption and by producing adipokines that regulate food consumption, and carbohydrate and lipid metabolic utilization. Brown adipose tissue is structured by multilocular cells containing many small fat drops that can be easily hydrolyzed. This tissue is involved in adaptative or facultative thermogenesis because it contains the uncoupling-1 protein (UCP-1 which by inhibiting ATP synthesis releases heat. The size of brow adipose tissue is reduced with aging and the most recent future strategies to fight obesity may be by transforming white cells into brown adipose tissue. The present work reviews the main structural and functional characteristics of white and brown adipose tissue with emphasis in its endocrine and regulatory function.El tejido adiposo es un reservorio de grasa y también un órgano que contribuye a la estética y a la salud de las personas, tarea que cumple funcionando como un tejido endocrino. El tejido adiposo blanco, formado por células adiposas uniloculares puede regular la homeostasis orgánica entre el gasto y el consumo de energía. Produce una serie de adipokinas que regulan el consumo de alimentos, la utilización de glucosa y de lípidos por parte del organismo. El tejido adiposo pardo o marrón, estructurado por células multiloculares, contiene numerosas gotas de grasa de pequeño tamaño, las que pueden ser hidrolizadas con mayor facilidad. El tejido participa en la termogénesis adaptativa o facultativa debido a la proteína desacoplante-1 (UCP-1 que inhibe la síntesis de ATP produciendo calor. Con la edad, este tejido se reduce y por ello actualmente se considera que una forma de combatir la obesidad es lograr que el tejido

  1. Importância do camundongo mdx na fisiopatologia da distrofia muscular de Duchenne The importance of mdx mouse in the pathophysiology of Duchenne's muscular distrophy

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    Sandra Lopes Seixas

    1997-09-01

    Full Text Available O camundongo mdx desenvolve distrofia muscular recessiva ligada ao cromossoma X (locus Xp21.1 e não expressa distrofina. Embora não apresente intensa fibrose do tecido muscular e acúmulo de tecido adiposo, é considerado o modelo animal mais adequado da distrofia muscular de Duchenne. As alterações estruturais no tecido muscular associadas à mionecrose e presença do infiltrado inflamatório com predomínio de linfócitos e monócitos/macrófagos sugerem uma participação do sistema imunológico nesta miopatia. Além disso a modulação na expressão dos componentes da matriz extracelular no microambiente muscular nas várias fases da doença (início, mionecrose, regeneração indicam um papel importante do conjuntivo no direcionamento das células inflamatórias para o foco da lesão muscular. O camundongo mdx coloca-se como um excelente modelo para o estudo dos mecanismos patogenéticos da mionecrose e regeneração na distrofia muscular de Duchenne, possibilitando inclusive o desenvolvimento de estratégias terapêuticas mais adequadas.The mdx mouse develop an X-linked recessive muscular dystrophy (locus Xp21.1 and lack dystrophin expression. Despite showing less intense myofibrosis and scarce deposition of fatty tissue, mdx mice are considered an adequate animal model for studies on the pathogenesis of Duchenne-type muscular dystrophy. Marked histological alterations in the muscular tissues associated to myonecrosis and inflammatory mononuclear cell infiltrate (lymphocytes, monocytes/macrophages suggest a participation of the immune system in this myopathy. Modulation of the extracellular matrix (ECM components in the muscular tissue during all phases (onset, myonecrosis and regeneration of disease, indicate an important role for the ECM driving inflammatory cells to the foci of lesion. Therefore mdx mice should be regarded as an important tool for studies on pathogenetic mechanisms of Duchenne-type muscular dystrophy. Such

  2. AISLAMIENTO, CARACTERIZACIÓN Y POTENCIAL DE DIFERENCIACIÓN DE CÉLULAS MADRE MESENQUIMALES CANINAS DERIVADAS DE TEJIDO ADIPOSO

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    N. B. Riaño

    2014-01-01

    Full Text Available Se caracterizaron células obtenidas del tejido adiposo subcutáneo de caninos para demostrar su naturaleza de células madre mesenquimales (ad-MSCs. Las células se cultivaron y expan - dieron bajo condiciones in vitro . Se determinó la presencia de marcadores de superficie CD73, CD90 y CD105 por citometría de flujo y PCR en tiempo real, siendo idéntico el perfil de expresión obtenido al reportado en células madre mesenquimales humanas. Se demostró su potencial de diferenciación mediante análisis de expresión génica por PCR en tiempo real luego de inducirlas a procesos de diferenciación osteogénica y adipogénica. La capacidad de diferenciación osteogénica se evidenció a través de la expresión de genes de tejido óseo (colágeno tipo I y osteonectina en una cinética de expresión concordante con los eventos moleculares y celulares del desarrollo óseo in vivo . El potencial adipogénico se evaluó por la formación de vacuolas lipídicas intracelulares y la expresión del gen de lipoprotein-lipasa, acorde con el proceso in vivo . Se concluye que las células mesenquimales obtenidas del tejido adiposo de caninos cumplen los criterios morfológicos, de expresión de marcadores de superficie espe - cíficos y de capacidad de diferenciación mesodérmica, confirmando su naturaleza de células madre mesenquimales. Los resultados del presente trabajo indicaron que las ad-MSCs de los caninos son un excelente modelo preclínico para el desarrollo de aplicaciones en medicina regenerativa, tanto en medicina veterinaria, como en medicina humana.

  3. Cambios en el tono muscular en dos niños con parálisis cerebral espástica mediante la hipoterapia: reporte de casos

    OpenAIRE

    Rodríguez Laiseca, Yasmín Andrea; Lerma Castaño, Piedad Rocío

    2015-01-01

    Introducción: Estudios previos han demostrado que la hipoterapia como terapia complementaria mejora la función motora gruesa en niños con parálisis cerebral. Sin embargo la evidencia no es concluyente. Objetivo: Determinar los cambios en el tono muscular de dos pacientes con parálisis cerebral tipo cuadriparesia espástica mediante la hipoterapia. Descripción del caso: El estudio se llevó a cabo con dos sujetos de género masculino de dos años de edad, con diagnóstico de parálisis cerebral tipo...

  4. Encapsulación de moléculas pequeñas mediante la precipitación salina de poliuretanos catioméricos

    OpenAIRE

    Fernández d’Arlas, Borja; Corcuera, María Ángeles; Eceiza, Arantxa

    2015-01-01

    En este trabajo se estudia un copolímero de poliuretano catiomérico (PU) con alta proporción de uretano como agente encapsulante de fármacos modelos (FM) mediante la encapsulación inducida por precipitación salina del PU y FM a pH < pI del PU. Mediante espectroscopia UV-Vis se ha estimado el porcentaje de encapsulación de varios FMs proponiéndose un modelo semi-empírico para determinar la distribución observada en las eficiencias de encapsulación, E, en función de su volumen molar...

  5. OBTENCIÓN DE CÉLULAS MADRE DEL TEJIDO ADIPOSO Y SU POTENCIAL DE DIFERENCIACIÓN OSTEOGÉNICO

    OpenAIRE

    Pineda Molina, Catalina; Londoño Peláez, Carolina

    2009-01-01

    El tejido adiposo es una de las principales fuentes de células madre de fácil obtención y con alto potencial de diferenciación hacia linajes celulares especializados. Con el objetivo de estandarizar la obtención de estas células y dirigir su diferenciación hacia el linaje osteogénico, se utilizó tejido adiposo procedente de liposucción y de abdominoplastia. Se aplicaron métodos de disgregación mecánica y enzimática del tejido para obtener las células. La morfología celular obtenida fue simila...

  6. Tejido adiposo como glándula endocrina. Implicaciones fisiopatológicas.

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    Illovis Ramírez Arteaga

    2011-08-01

    Full Text Available

    El tejido adiposo se considera en la actualidad como un órgano con importante función endocrina; distinguiéndose dos tipos: el pardo y el blanco. El tejido adiposo es capaz de secretar diversas sustancias conocidas como adipocinas que se encuentran implicadas en la regulación del peso corporal, en el sistema inmune, en la función vascular y en la sensibilidad insulínica. En la obesidad, principalmente visceral, se encuentran aumentadas las adipocinas proinflamatorias. Estas se asocian a la inflamación clínica y subclínica, a la insulinorresistencia, al estrés oxidativo y a la lesión endotelial; desarrollan un papel clave en el síndrome metabólico e incrementan el riesgo cardiometabólico. El objetivo de la presente revisión bibliográfica ha sido exponer las características morfofuncionales del tejido adiposo y los mecanismos que lo vinculan con la génesis de diversas enfermedades.

    ADIPOSE TISSUE AS AN ENDOCRINE GLAND. PATHOPHYSIOLOGICAL IMPLICATIONS.

    Adipose tissue is currently recognized as an organ with an important endocrine function. Two main types of adipose tissue are distinguished: the brown and the white. Adipose tissue can secrete a variety of substances known as adipokines that are implicated in the regulation of body weight, the immune system and the vascular functioning and the insulin sensitivity. In cases of obesity (mainly visceral obesity, proinflammatory adipokines are increased. They are commonly associated with clinical and subclinical inflammation, insulin resistance, oxidative stress and endothelial injury. Proinflammatory adipokines also play a key role in the metabolic syndrome and increase cardiometabolic risk. The present review is aimed at exposing the morphofunctional characteristics of adipose tissue and the mechanisms that link this tissue to the origin of a variety

  7. Espressione genica e proteica dell’albumina negli adipociti umani maturi. Relazione tra albuminemia e tessuto adiposo nei pazienti in trattamento dialitico.

    OpenAIRE

    Sirico, Maria Luisa

    2013-01-01

    Scopo: Lo scopo di questo lavoro era quello di indagare l’espressione genica dell’albumina negli adipociti umani maturi, la regolazione di tale espressione da parte dell’infiammazione e il contributo del tessuto adiposo ai livelli circolanti di albumina. Metodi: Campioni di tessuto adiposo sono stati prelevati da soggetti sani e infiammati. Sono state effettuate analisi di espressione genica (RT-PCR e Real-Time PCR) e proteica (Western Blot ) sugli adipociti e preadipociti estratti. Inoltr...

  8. Food consumption and adipose tissue DDT levels in Mexican women Consumo de alimentos y niveles de DDT en tejido adiposo de mujeres mexicanas

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    Marcia Galván-Portillo

    2002-04-01

    Full Text Available This article analyzes food consumption in relation to levels of DDE (the principal metabolite of DDT in the adipose tissue of 207 Mexican women residing in States with high and low exposure to DDT. Data on the women's dietary habits and childbearing history were obtained from a personal interview. Adipose tissue DDE levels were measured by gas-liquid chromatography and compared by analysis of variance (ANOVA and multiple linear regression. Adipose tissue DDE levels increased significantly with age (p = 0.005 and residence in coastal areas (p = 0.002 and non-significantly with the consumption of onion, cauliflower, prickly pear, squash blossoms, sweet corn, broad beans, chili pepper sauce, ham, and fish. Even so, during breastfeeding there was a non-significant reduction in these levels. The findings suggest that certain foods serve as vehicles for DDE residues and confirm that breastfeeding is a mechanism for the elimination of this insecticide, which accumulates over the years in the human body.En este trabajo se presenta el consumo de alimentos en relación a los niveles de DDE (principal metabolito del DDT en tejido adiposo de 207 mujeres Mexicanas residentes en estados de alta y baja exposición a DDT. La información acerca de los hábitos dietéticos y la historia reproductiva de cada una de las participantes se indagaron mediante una entrevista personal. Los niveles de DDE en el tejido adiposo se determinaron por medio de cromatografía de gas-líquido y se compararon por medio de análisis de varianza (ANOVA y de regresión lineal múltiple. Los niveles de DDE en tejido adiposo se incrementaron significativamente de acuerdo a la edad (p = 0.005 y al hecho de residir en zonas costeras (p = 0,002 y, de manera no significativa, con el consumo de: cebolla, coliflor, nopal, flor de calabaza, elote, habas, salsas preparadas con chile, jamón y pescado. Asimismo, el aumento en los meses de lactancia representó una reducción no significativa

  9. Fracción vascular estromal de tejido adiposo: cómo obtener células madre y su rendimiento de acuerdo a la topografía de las áreas donantes: estudio preliminar Fração vascular estromal de tecido adiposo: como obter células-tronco e seu rendimiento de acordo com a topografia as áreas doadoras: nota prévia Stromal vascular fraction from fat tissue: obtaining stem cells and their yield according to the topography of the donor areas: previous note

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    K. A. Almeida

    2008-03-01

    Full Text Available La obtención de tejido adiposo supone un nuevo y prometedor mercado de trabajo para los cirujanos plásticos, ya que los bancos de tejidos escogerán de forma acertada la grasa como el medio más fácil para obtener fuentes de células madre de alto rendimiento, en la medida en que este tejido es capaz de producir al menos cinco veces más unidades formadores de colonias (UFCs que la médula ósea. El objetivo del presente trabajo es mostrar lo que se puede esperar del tejido adiposo como origen de células adultas de fracción vacular estromal (FVE, y señalar las mejores áreas del cuerpo humano para ser elegidas como donantes de tejido adiposo, extraído mediante liposucción. Describimos la rutina seguida para la obtención de células de FVE mediante la digestión de las muestras de tejido adiposo humano con colagenasa. En el momento de su recolección, esas células presentaban una viabilidad de 92+/- 1% basada en exclusión por Azul de Trypan. Las células de FVE recontadas después de permanecer 48 horas en medio de cultivo de Eagle modificado por Dulbecco (DMEM, dentro de una cámara de Neubauer, tras lo cual el rendimiento medio de las células de FVE fue de 7,2 +/- 1,3 x 103 células por mililitro de tejido lipoaspirado. En conclusión, pensamos que supone un desafío en la actualidad el mejorar las estrategias para la obtención de células de FVE. Este trabajo, por ahora preliminar, muestra que las células de FVE pueden ser fácilmente obtenidas por medio de lipoaspiración. La comparación entre las diferentes áreas donantes, mostró un rendimiento 22% más alto para las células de FVE cuando el tejido adiposo había sido obtenido del tronco, en comparación a cuando lo había sido de los miembros.The harvest of adipose tissue will be a promising labor marketing for plastic surgeons, since tissue banks will certainly choose fat as the easiest way to obtain a high-yield source of stem cells, as this type of tissue can produce al

  10. O tecido adiposo como órgão endócrino: da teoria à prática Adipose tissue as an endocrine organ: from theory to practice

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    Miriam Helena Fonseca-Alaniz

    2007-11-01

    Full Text Available OBJETIVOS Mostrar os avanços na pesquisa sobre o papel fisiológico do tecido adiposo branco, ressaltando o seu papel endócrino em processos inflamatórios, no comportamento alimentar, na sensibilização à insulina e na modulação do processo de aterogênese. Abordar o potencial papel do tecido adiposo como fonte de células-tronco para regeneração de tecidos, com especial ênfase para a adipogênese e suas conseqüências para a geração de obesidade. FONTES DE DADOS: Informações importantes constantes da literatura científica foram compiladas de modo a que esta leitura contenha uma síntese esclarecedora dos aspectos mencionados acima. SÍNTESE DOS DADOS:O tecido adiposo possui, além das suas funções clássicas como principal estoque de energia metabólica, suprindo as necessidades energéticas em períodos de carência mediante a lipólise, a capacidade de sintetizar e secretar vários hormônios, as adipocinas. Estas agem em diversos processos, como o controle da ingestão alimentar (leptina e o controle da sensibilidade à insulina e de processos inflamatórios (TNF-alfa, IL-6, resistina, visfatina, adiponectina. Além disso, como o tecido adiposo contém também células indiferenciadas, tem a habilidade de gerar novos adipócitos, regenerando o próprio tecido (adipogênese, bem como originar outras células (mioblastos, condroblastos, osteoblastos, fato este que tem grande potencial terapêutico em futuro não muito distante. CONCLUSÃO: Amplia-se o leque de possibilidades funcionais do tecido adiposo. A compreensão dessas potencialidades pode fazer deste tecido o grande aliado no combate de moléstias que atualmente vêm assumindo proporções epidêmicas (obesidade, diabetes melito, hipertensão arterial e arteriosclerose, nas quais o tecido adiposo ainda é tido como um grande vilão.OBJECTIVES: To describe the advances in research into the physiological role of white adipose tissue, with emphasis on its endocrinal role

  11. Obtención de células madre del tejido adiposo y su potencial de diferenciación ontogénico

    OpenAIRE

    Pineda-Molina, C. (Catalina); Londoño-Peláez, C. (Carolina)

    2009-01-01

    El tejido adiposo es una de las principales fuentes de células madre de fácil obtención y con alto potencial de diferenciación hacia linajes celulares especializados. Con el objetivo de estandarizar la obtención de estas células y dirigir su diferenciación hacia el linaje osteogénico, se utilizó tejido adiposo procedente de liposucción y de abdominoplastia. Se aplicaron métodos de disgregación mecánica y enzimática del tejido para obtener las células. La morfología celular obtenida f...

  12. Contenidos de isómeros trans de los ácidos grasos en productos cárnicos. (III Tejido adiposo y grasa intramuscular de vacuno

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    Lluch, M. C.

    1993-06-01

    Full Text Available We give the results obtained for the determination of trans fatty acids in subcutaneous and intramuscular fats from different veal carcasses, using gas chromatography. FA composition differs clearly between both tissues, showing mean values of 58.7% SFA, 39.1% MUFA and 2.7% PUFA in the adipose tissue, and of 44.7% SFA, 46.1% MUFA and 9.4% PUFA in the muscle. Trans fatty acid values obtained in muscle and adipose tissue show statistically significant differences (mean values of 7% total trans fatty acids in intramuscular fat and 10.5% in adipose tissue fat. Besides, we observed a clearly different distribution for C 16: 1t, that didn't show these differences, while C 18: 1t showed a similar distribution than total trans fatty acids. Related to the factors that could determine the trans fatty acid contents in veal carcasses, we found that the race showed more differences between groups than the origin of the animal and their commercial category. On the other hand, correlations we found between trans fatty acids and the total of saturated, mono and polyunsaturated fatty acids showed an opposite sign according to the type of tissue (muscular or adipose. Thus, an increase in the trans fatty acid content was associated with a parallel increase of saturated and decrease in polyunsaturated fatty acid contents in the intramuscular fat. On the contrary, an increase of trans fatty acid content in adipose fat was correlated to a decrease of saturated and an increase of mono and polyunsaturated fatty acid contents.

    Se presentan los resultados obtenidos para la determinación de ácidos grasos en una serie de muestras de tejidos subcutáneo y muscular, procedentes de canales de vacuno, por aplicación de la cromatografía en fase gaseosa, para los que se obtuvieron unos valores medios de 58.7% de ácidos saturados, 39.1 % de monoinsaturados y 2.7% de polinsaturados, en el tejido adiposo, y de 44.7% de saturados, 46.1% de monoinsaturados y 9.4% de

  13. Comparación del gasto energético en reposo determinado mediante calorimetría indirecta y estimado mediante fórmulas predictivas en mujeres con grados de obesidad I a III

    OpenAIRE

    Alicia Parra-Carriedo; Loren Cherem-Cherem; Daniela Galindo-De Noriega; Mary Carmen Díaz-Gutiérrez; Ana Bertha Pérez-Lizaur; César Hernández-Guerrero

    2013-01-01

    Introducción: La determinación del gasto energético en reposo (GER) se calcula cotidianamente a partir de fórmulas predictivas aunque el resultado varía dependiendo de la población. Objetivo: Comparar la determinación del GER mediante calorimetría indirecta y mediante las ecuaciones Harris-Benedict (HB), Mifflin (MF), Organización Mundial de la Salud (OMS), "Institute of Medicine" (IOM), Fórmula Rápida (FR) y Valencia (VA) en mujeres con grados de obesidad I a III. Métodos: Mujeres adultas me...

  14. Aplicación de técnicas estereológicas para la cuantificación del tejido adiposo

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    L. Santamaría-Solís

    Full Text Available El conocimiento de la estructura y función del tejido adiposo es fundamental no sólo por el papel que desempeña en el metabolismo lipídico y en enfermedades endocrinas como la diabetes, sino también por su importante misión como elemento estructural de soporte mecánico y de modelado del contorno orgánico (facial, por ejemplo; de ahí su relevancia en el campo de la Cirugía Plástica, Reparadora y Estética. La cuantificación es un método que se practica desde los albores de la Histología. En el presente estudio nos referiremos exclusivamente a las metodologías estereológicas, herramientas libres de sesgo que aprovechan propiedades geométricas intrínsecas de las estructuras a cuantificar. En esencia, la estereología consiste en un conjunto de métodos morfométricos que obtienen datos cuantitativos tridimensionales a partir de elementos bidimensionales, utilizando propiedades geométricas intrínsecas a la estructura que se está estudiando. Describimos las metodologías estereológicas que conducen a la estimación no sesgada de la fracción de volumen de tejido adiposo en relación con el espacio de referencia, número relativo de adipocitos, y longitud relativa del lecho microvascular que irriga el tejido adiposo.

  15. La Adiponectina, una adipocina del tejido adiposo clave en la obesidad durante la adolescencia

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    Gerardo Fernando Fernández Soto

    2016-12-01

    Full Text Available La obesidad Infantil es una epidemia mundial que simultáneamente con la diabetes tipo 2 se han incrementado en la población pediátrica, que representa alto impacto económico en la asistencia y el tratamiento de sus comorbilidades asociadas, para los distintos sistemas de salud, que han impulsados nuevas investigaciones relacionadas con la obesidad y, particularmente con un actualizado análisis de la biología del tejido adiposo. Uno de los resultados de esta actividad investigadora ha sido el mejor conocimiento de la importante actividad endocrina del tejido adiposo, ejercida por medio de la síntesis y secreción de una gran cantidad de hormonas, factores tisulares y citocinas, denominadas adipocinas que están implicadas en la regulación de varios procesos metabólicos fisiológicos. La adiponectina es una adipocina considerada un biomarcador bioquímico y antiinflamatorio en los trastornos metabólicos, ya que tiene un efecto sensibilizador de insulina, aumentando la oxidación de ácidos grasos y juega papel importante en minimizar el estrés oxidativo, que disminuye el desarrollo de trastornos metabólicos como la diabetes y la obesidad.

  16. Crosstalk entre osso e tecido adiposo na doença renal crônica

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    Sandra Mara Silva de Azevedo Marinho

    2012-06-01

    Full Text Available Dentro do conceito de que os hormônios são regulados por um ciclo de reciprocidade, o fato de osteoblastos e adipócitos serem desenvolvidos a partir de células-tronco mesenquimais e da remodelação óssea ser regulada pela leptina, traz a ideia de possíveis participações do osso no metabolismo energético e vice-versa. Estudos recentes têm demonstrado que a diferenciação e as funções das células ósseas são reguladas pela leptina, que parece desencadear uma resposta bimodal central, via sistema nervoso simpático, e uma local, na qual a leptina agiria sobre o osso. De fato, estudos têm revelado complexa interação entre osso, tecido adiposo e cérebro; no entanto, existem poucos estudos sobre esse crosstalk em pacientes com doença renal crônica (DRC. Como tais pacientes têm tendência à diminuição da densidade mineral óssea e elevados níveis de leptina, o presente artigo apresentou uma revisão sobre o possível envolvimento entre tecido adiposo e massa óssea, em pacientes com DRC.

  17. Revisión sistemática: células madre derivadas del tejido adiposo, utilidad en lipoatrofia facial

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    M.D. Cruz-Sánchez

    2014-06-01

    Full Text Available El objetivo de la presente revisión sistemática es determinar si los lipoinjertos enriquecidos con células madre derivadas del tejido adiposo son superiores a los injertos de tejido graso convencionales en el tratamiento de la lipoatrofia facial. Realizamos una búsqueda sistemática de publicaciones en las bases de datos: MEDLINE, SCOPUS, OVID SP, SCIELO y EMBASE y posteriormente, una valoración basada en criterios actuales de medicina basada en la evidencia de las publicaciones encontradas por dos revisores independientes; las discrepancias en el análisis se resolvieron por acuerdo. Los criterios de inclusión fueron: ensayos clínicos aleatorizados, series clínicas y reportes de casos en humanos, excluyendo los ensayos en animales. Encontramos 6 publicaciones que cumplían con los criterios previamente mencionados. Entre éstas se incluyeron 2 ensayos clínicos aleatorizados controlados que recogían la superioridad de los lipoinjertos enriquecidos con células madre derivadas del tejido adiposo en el tratamiento de la lipoatrofia facial en términos de resistencia a la reabsorción (preservación del volumen y menor necesidad de nuevos lipoinjertos, en comparación con los lipoinjertos convencionales. Concluimos diciendo que existen publicaciones con buenos niveles de evidencia que sugieren que los lipoinjertos enriquecidos con células madre derivadas del tejido adiposo son superiores a los lipoinjertos convencionales en el tratamiento de la lipoatrofia facial. No obstante, son estudios de reciente publicación y aún es necesario tener seguimientos a más largo plazo y con mayor número de pacientes para mejorar los ya buenos niveles de evidencia disponibles.

  18. Efeitos da fadiga muscular induzida por exercícios no tempo de reação muscular dos fibulares em indivíduos sadios Efectos de la fatiga muscular inducida por ejercicios sobre el tiempo de reacción muscular peronea en individuos sanos Effects of the exercise-induced muscular fatigue on the time of muscular reaction of the fibularis in healthy individuals

    Directory of Open Access Journals (Sweden)

    Bruno Araújo Rego Santos Silva

    2006-04-01

    ón del tobillo. Se estudiaron 14 individuos saludables masculinos (con edad: entre 20-35 años, que tenían su TRM estimado a través de la eletromiografia (EMG de superficie. El principio de la actividad muscular se definió como el promedio del resto + 3x la desviación normal (DP. TRM de músculos peroneos estava moderado después de una inversión súbita de 20º cumplida en una plataforma. La inversión súbita se realizaba antes y después de la fatiga muscular, que era inducido por los ejercicios localizados en los músculos peróneos hasta agotamiento. Los resultados mostraron que había un aumento significante del tiempo de reacción muscular después de la fatiga (p The muscular fatigue (MF is a common phenomenon in the daily sports activities that results in a worsening of the motor performance. It is considered one of the major factors for muscle-skeletal damages, such as the ankle sprain, when the MF would affect both the afferent and the efferent systems. Several studies have been analyzing the influence of the MF on the neuromuscular control (NMC. Nevertheless, there are few researches comprising that influence on the velocity of the muscular reaction. The purpose of this study was to check the effects of the MF on the time of the muscular reaction (TMR in the fibularis muscles, which are the first to respond to an inversion stress of the ankle. Fourteen healthy male individuals (age: 20-35 years were studied, who had their TMR assessed by means of the surface electromyography (EMG. The beginning of the muscular activity was defined as the mean resting value +3x the standard deviation (SD. The TMR of the fibularis was measured after a sudden 20º inversion performed on a platform. The sudden inversion was performed before and after the muscular fatigue, which was induced through localized exercises of the fibularis up to the exhaustion. The results have shown a significant increase in the time of the muscular reaction after the fatigue (p < 0.01. While

  19. Respuesta al entrenamiento en EPOC: Diferencia entre limitación por fatiga muscular y por disnea

    OpenAIRE

    Martín Sívori; Laura Bustamante; Alejandro Martínez Fraga; Marta Almeida; César Saenz

    2011-01-01

    Este estudio comparó la respuesta post entrenamiento al ejercicio en pacientes limitados por fatiga muscular (LF) vs. disnea (LD). Se incluyeron pacientes con EPOC moderada y grave (definición GOLD), clasificándolos en LF si la respuesta a cicloergometría máxima tenía ≥ 2 puntos en la escala de Borg para fatiga muscular vs. disnea; LD a la inversa. Se realizaron ergometría submáxima, 6 minutos y pruebas de calidad de vida mediante cuestionario. Fueron entrenados 3 veces/semana, 90 min/sesión ...

  20. Epitelización inducida por células troncales derivadas del tejido adiposo

    OpenAIRE

    M. Meruane; S. Benítez; M. Rojas; A. Sagredo; K. Marcelain; B. Villalobos

    2014-01-01

    El tratamiento de lesiones con pérdida de tejido cutáneo ha mejorado notablemente con el advenimiento de la bioingeniería tisular. Una alternativa en desarrollo es la utilización de sustitutos dérmicos combinados con células troncales derivadas del tejido adiposo autólogo. Estudios previos nos muestran que con esta técnica es posible optimizar la angiogénesis y la síntesis de colágeno, sin embargo potenciar la epitelización es un tema pendiente por resolver. En el presente estudio evaluamos l...

  1. Aplicaciones clínicas de las células madre del tejido adiposo

    OpenAIRE

    B. Castro

    2013-01-01

    En la actualidad, las células madre del tejido adiposo (ASC) juegan un papel crucial en las estrategias de reparación o regeneración tisular. Son células mesenquimales multipotenciales que se pueden obtener fácilmente de lipoaspirados. Tras un proceso de digestión enzimática y centrifugación, se recoge la fracción estromal vacular (SVF) en el sedimento obtenido y se purifican las ASC a través del cultivo en plástico y realización de varios subcultivos. Hay diversos aspectos que han convertido...

  2. EL PROTEOMA DEL TEJIDO ADIPOSO SUBCUTÁNEO MUESTRA HETEROGENEIDAD ANATÓMICA

    Science.gov (United States)

    Martos-Moreno, G.A.; Sackmann-Sala, L.; Berryman, D.E.; Blome, D.W.; Argente, J.; Kopchick, y J.J.

    2015-01-01

    Introducción El tejido adiposo blanco (TAB) subcutáneo (Sc) humano podría variar dependiendo de su localización anatómica, con diferencias en su perfil proteómico. Pacientes y métodos Se obtuvieron aspirados de TAB-Sc de seis mujeres con IMC >25 kg/m2, sometidas a liposucción. Dicho TAB-Sc se obtuvo de seis localizaciones anatómicas: abdominal superior e inferior, muslo, dorsal, flanco y cadera, analizándose su perfil proteómico mediante electroforesis bidimensional. En muslo y abdomen superior se compararon, además, las muestras obtenidas de las dos capas del TAB-Sc (profunda y superficial). Resultados Se detectaron 21 proteínas que mostraban una intensidad de expresión diferente entre las seis localizaciones anatómicas y 14 entre las capas superficial y profunda de una misma región. Entre las proteínas identificadas se incluyen: vimentina (proteína estructural); proteínas “heat-shock” (HSPs), superóxido-dismutasa, (estrés/chaperoninas); proteína fijadora de ácidos grasos 4 (FABP-4) y alfa-enolasa (metabolismo lipídico y de los hidratos de carbono, respectivamente) y ATP-sintetasa (producción de energía). Entre las regiones estudiadas, el TAB-Sc dorsal mostraba un perfil proteómico particular, con menor expresión de proteínas implicadas en la producción de energía y metabolismo (ATP-sintetasa, alfa-enolasa, HSPs y FABP-4) que el resto de regiones. Conclusiones Los niveles de expresión de diversas proteínas en el TAB-Sc humano no son homogéneos, difiriendo entre localizaciones anatómicas. Esto sugiere la existencia de diferencias funcionales en el TAB-Sc de acuerdo con su localización anatómica, lo que debe considerarse antes de asumir la extrapolación de los datos derivados del TAB-Sc de una determinada localización al de otras partes de la anatomía. PMID:23228439

  3. Estudio preliminar del desarrollo muscular y su posible funcionalidad en una población cazadora fueguina

    Directory of Open Access Journals (Sweden)

    Kozameh, Livia Febes

    2001-01-01

    Full Text Available En una primera etapa, este estudio propone analizar las fijaciones de ciertas inserciones musculares correspondientes a cinturas escapulares, brazos y antebrazos. El desarrollo de estas fijaciones, observado en restos óseos de una población cazadora, es tomado como indicador de algunos movimientos predominantes, los que sumados al tipo de esfuerzo muscular demandado podrían indicar distintas actividades cotidianas de estos pobladores. Los restos utilizados provienen de diversos sitios excavados durante la década del ´80 en Península Mitre, Isla Grande de Tierra del Fuego. El análisis osteológico se llevó a cabo mediante la observación macroscópica estructural y con lupa binocular. Se observó un marcado desarrollo en las diversas inserciones musculares analizadas, lo que sugeriría una gran demanda funcional de la estructura muscular de estos individuos.

  4. Diagnóstico de distrofia muscular de Duchenne mediante análisis del ácido desoxinucleotico y su aplicación en la prevención

    Directory of Open Access Journals (Sweden)

    Mayra Rodríguez Hernández

    1996-04-01

    Full Text Available Se define una estrategia para la prevención en Cuba de la distrofia muscular de Duchenne (DMD, una de las enfermedades hereditarias letales más frecuentes, y se evalúan la factibilidad de su aplicación y los problemas que pudieran dificultar su implantación al nivel nacional. La estrategia se basa fundamentalmente en la necesidad de detectar las familias afectadas, la definición de las mujeres portadoras o en riesgo de serlo y el estudio molecular de los miembros de interés con anterioridad al ofrecimiento de los servicios de diagnóstico prenatal mediante análisis directo -detección de deleciones en el gen DMD mediante reacción en cadena de la polimerasa (PCR o análisis indirecto- empleo de los marcadores denominados polimorfismos en la longitud de los fragmentos de restricción (RFLPs en análisis de ligamento. Se concluye en que la aplicación de esta estrategia es factible y conveniente, pues permite ofrecer el diagnóstico prenatal al 75 % de las mujeres portadoras. Su eficiencia en la prevención del nacimiento de nuevos enfermos DMD se demuestra en 2 diagnósticos prenatales realizados, uno de los cuales detectó un embarazo afectado que fue interrumpido por solicitud de los padres.A strategy is defined for the prevention in Cuba of the Duchenne's muscular dystrophy (DMD, one of the most frequent lethal hereditary diseases, and the feasibility of its application, and the troubles that might difficult its implantation at a national level, are evaluated. This strategy is mainly based on the need of detecting the affected families, the definition of the carrier women, or the women at risk of being carriers, and the molecular study of the members of interest with anteriority to the offering of prenatal diagnosis services by direct analysis -detection of DMD gen deletions by (PCR polymerase chain reaction, or indirect analysis-, use of the markers called polymorphisms in the length of the restriction fragments (RFLPs in ligament

  5. Aplicaciones clínicas de las células madre del tejido adiposo

    Directory of Open Access Journals (Sweden)

    B. Castro

    2013-12-01

    Full Text Available En la actualidad, las células madre del tejido adiposo (ASC juegan un papel crucial en las estrategias de reparación o regeneración tisular. Son células mesenquimales multipotenciales que se pueden obtener fácilmente de lipoaspirados. Tras un proceso de digestión enzimática y centrifugación, se recoge la fracción estromal vacular (SVF en el sedimento obtenido y se purifican las ASC a través del cultivo en plástico y realización de varios subcultivos. Hay diversos aspectos que han convertido a las ASC en una importante opción en terapia celular en los últimos años: su seguridad demostrada en ensayos clínicos; la mayor cantidad de células mesenquimales que se obtienen del tejido adiposo con respecto a la médula ósea, con un proceso menos invasivo y con menor morbilidad; su carácter inmunorregulador, bien a través de la secreción de mediadores moleculares o interacción directa; y finalmente, su gran acción trófica con el objetivo de crear un microambiente adecuado para activar los procesos de reparación del propio tejido. En este trabajo también se indican las dosis celulares y las vías de administración que se están utilizando para la aplicación de las ASC y la SVF. Los próximos años serán cruciales para determinar, gracias a distintos ensayos clínicos en marcha, el potencial terapéutico de las ASC y sus mecanismos de acción.

  6. Fatores maternos associados ao peso fetal estimado pela ultra-sonografia

    OpenAIRE

    Melo,Adriana Suely de Oliveira; Amorim,Melania Maria Ramos de; Assunção,Paula Lisiane; Melo,Fabiana de Oliveira; Gondim,Sheila Sherezaide Rocha; Carvalho,Danielle Franklin de; Cardoso,Maria Aparecida Alves

    2008-01-01

    OBJETIVO: avaliar o efeito de variáveis maternas, socioeconômicas e obstétricas, assim como a presença de incisuras na 20ª e na 24ª semana, sobre o peso fetal estimado no final da gravidez (36ª semana) em gestantes atendidas pelo Programa Saúde da Família em uma cidade do interior do Nordeste do Brasil. MÉTODOS: estudo longitudinal incluindo 137 gestantes. As gestantes foram acompanhadas a cada quatro semanas para aferição das condições clínicas, socioeconômicas e obstétricas, incluindo o pes...

  7. Rendimiento deportivo: glucógeno muscular y consumo proteico

    OpenAIRE

    Pérez-Guisado, Joaquín

    2008-01-01

    Para conseguir un óptimo rendimiento deportivo mediante el control de parámetros relacionados directa o indirectamente con la ingesta, puede resultar de gran importancia conocer y saber controlar el entramado metabólico relacionado con el glucógeno muscular y la optimización del consumo proteico. La recuperación de las reservas de glucógeno tras la realización del ejercicio físico es un proceso lento que puede llevar de 24 a 48 h según las pérdidas producidas. La velocidad de resíntesis del g...

  8. Influence of soft tissues on mandibular gray scale levels Influência dos tecidos moles sobre os níveis de cinza mandibulares

    Directory of Open Access Journals (Sweden)

    Paulo Henrique Couto Souza

    2004-03-01

    Full Text Available The purpose of this study was to analyze the gray levels, expressed in pixels, of the mandibular retromolar region, with regard to the influence of muscular and fat soft tissues near this region. Fifteen dry mandibles were X-rayed with the presence of soft tissue simulators. The radiographs were digitized and evaluated by Digora® software. A one cm thick layer of wax was used as a simulator of the muscular soft tissue. Animal fat samples of different thicknesses - 0.5, 1.0, 1.5 and 2.0 cm - were used as a simulator of the fat soft tissue. Results showed that the fat soft tissue simulator influenced the gray level values in pixels of the mandibular retromolar region when analyzed in different thicknesses using the Digora® digitized image software.O presente trabalho foi desenvolvido com o objetivo de analisar os níveis de cinza, expressos em "pixels", da região retromolar mandibular, considerando a influência dos tecidos moles muscular e, principalmente, adiposo adjacentes a essa região. Para o estudo, 15 mandíbulas secas foram tecnicamente radiografadas, sendo que as radiografias obtidas foram escaneadas e avaliadas pelo programa de imagens digitalizadas Digora®. Como simuladores dos tecidos muscular e adiposo, foram utilizadas cera utilidade na espessura de 1,0 cm e amostras de gordura animal em espessuras diferentes de 0,5; 1,0; 1,5 e 2,0 cm. Os resultados mostraram que o tecido adiposo foi capaz de influenciar a análise dos valores de níveis de cinza em "pixels" da região retromolar mandibular quando estudado em espessuras diferentes pelo programa de imagens digitalizadas Digora®.

  9. Contribución para el criterio diagnóstico de la Dismorfia Muscular (Vigorexia

    Directory of Open Access Journals (Sweden)

    Irene Gonz\\u00E1lez-Mart\\u00ED

    2012-01-01

    Full Text Available La Dismorfia Muscular o Vigorexia, es un trastorno mental cuyo foco de preocupación es la subestimación del tamaño y forma muscular, como consecuencia de la distorsión en la imagen corporal que padecen las personas afectadas. Se trata de un trastorno novedoso y, por lo tanto, no se encuentra clasificado en los manuales de desórdenes y trastornos mentales existentes. Mientras unos autores proponen su clasificación bajo un Trastorno de la Conducta Alimentaria (TCA, otros defienden que se trata de un Trastorno Dismórfico Corporal (TDC, o incluso se ha llegado a justificar su posible clasificación como Trastorno Obsesivo-Compulsivo (TOC. Ante la disparidad de criterio, en el presente estudio pretendemos contribuir en la clasificación de la Dismorfia Muscular, como desorden mental, mediante la técnica de clasificación árbol de decisión. Los elementos que cobran mayor relevancia dentro de este método clasificatorio son aquellos relacionados con el tamaño y forma corporal que los participantes tienen, se perciben y desean. A la luz de estos resultados contribuimos a clasificar la Dismorfia Muscular bajo el prisma de un TDC.

  10. Regeneracion osea por implante de celulas madre mesenquimales derivadas de tejido adiposo humano en un modelo animal

    OpenAIRE

    Linero Segrera, Itali Marcelly

    2012-01-01

    La reconstrucción y restauración de la función esquelética continúa siendo un reto en el campo de la cirugía maxilofacial y la ortopedia. Una alternativa o terapia coadyudante a los tratamientos convencionales es la ingeniería de tejidos que surge como una posibilidad prometedora para la regeneración del tejido óseo. Con el fin de crear un modelo para la aplicación clínica de las células madre mesenquimales derivadas de tejido adiposo humano (Ad-MSCs) desarrollamos una matriz tridimensi...

  11. Descripción de las cadenas cinéticas de la reptación refleja de Vojta en adultos mediante análisis electromiográfico

    OpenAIRE

    Sánchez Gonzalez, Juan Luis

    2016-01-01

    La locomoción refleja de Vojta consiste en dos patrones motores globales llamados reptación refleja y volteo reflejo. Vojta descubrió que la actividad muscular dependía de la posición inicial del paciente (supina, lateral o prono) y de los diferentes puntos de presión. La contracción de las cadenas musculares se dirige hacia los puntos de apoyo, de tal manera que, cuando cambia el punto de apoyo cambia la dirección de la contracción. Mediante la electromiografía de superficie somos capaces de...

  12. Correlación ecográfico-anatómica de la medición del volumen prostático total y de la zona transicional mediante ecografía transrectal: correlation with surgical specimen

    OpenAIRE

    Cabello Benavente, R.; Jara Rascón, J.; Monzó, J.I.; López Díez, I.; Subirá Ríos, D.; Lledó García, E.; Herranz Amo, F.; Hernández Fernández, C.

    2006-01-01

    Objetivo: Determinar la fiabilidad de la ecografía transrectal (ECOTR) en la medición del volumen prostático total y de la zona transicional (ZT) y buscar coeficientes de correlación capaces de mejorar la equiparación entre ambas medidas y el peso real prostático. Material y Métodos: Comparamos los volúmenes estimados mediante ECOTR con el peso de la pieza quirúrgica en pacientes con cáncer prostático localizado (grupo A, n=33) o HBP (grupo B, n= 37) sometidos a cirugía. El volumen se calculó...

  13. Crescimento e produção de plantios comerciais de eucalipto estimados por duas categorias de modelos

    Directory of Open Access Journals (Sweden)

    Renato Vinícius Oliveira Castro

    2013-03-01

    Full Text Available O objetivo deste trabalho foi avaliar e comparar duas categorias de modelos de crescimento e produção em plantios comerciais de eucalipto. Para isso, foram ajustados um modelo de crescimento e produção para povoamento e outro para árvore individual, por meio de equações simultâneas e redes neurais artificiais, respectivamente. O volume de madeira por área foi estimado em diferentes idades e classes de produtividade. Foram avaliados dados de 63 parcelas permanentes de plantios clonais, não desbastados, do híbrido Eucalyptus grandis x E. urophylla, com os dados de 33 parcelas utilizados para o ajuste do modelo e o treinamento das redes neurais, e os das 30 parcelas restantes, para a validação dos modelos. As duas categorias de modelos ajustaram-se bem aos dados observados. No entanto, na validação dos modelos com dados independentes, o volume de madeira por área foi mais bem estimado com o modelo para árvore individual.

  14. Myotonic Muscular Dystrophy

    Science.gov (United States)

    ... Marie-Tooth Disease (CMT) Congenital Muscular Dystrophy (CMD) Duchenne Muscular Dystrophy (DMD) Emery-Dreifuss Muscular Dystrophy Endocrine Myopathies Metabolic Diseases of Muscle Mitochondrial Myopathies (MM) Myotonic Dystrophy (DM) Spinal-Bulbar ...

  15. Composicion corporal y prevalencia estandarizada de desnutricion en niños de 6 a 12 años de edad, La Costa, Argentina

    Directory of Open Access Journals (Sweden)

    Bolzán Andrés

    2003-01-01

    Full Text Available OBJETIVOS: evaluar la composición corporal y la prevalencia estandarizada de desnutrición en niños de seis a 12 años de edad de La Costa, Argentina. MÉTODOS: se midieron la estatura, perímetro braquial y el pliegue cutáneo tricipital, calculando los siguientes indicadores: circunferencia muscular braquial, área muscular braquial, área grasa braquial (AGB, índice área muscular braquial/estatura (AMBE, índice energía/proteína (EP e índice grasa/músculo braquiales. Se calculó la prevalencia estandarizada de desnutrición (PE. Se consideraron además tres indicadores sociales. El análisis estadístico incluyó ANOVA multifactorial, correlación de Pearson y de Spearman. RESULTADOS: existió dimorfismo sexual sólo para el AGB. A mayor tamaño de la familia los indicadores antropométricos fueron menores. La PE mostró diferencias con el estándar. El tejido muscular resultó más desviado que el adiposo, aunque el índice AMBT se mantuvo dentro de la normalidad. CONCLUSIONS: El tamaño de la familia modificó el crecimiento global de la población viéndose más afectados los indicadores que incluyen tejido adiposo. Las PE reflejaron mayor sesgo de los indicadores de reserva proteica: AM, PMB que los de reserva energética: PCT, AG.

  16. Mecanismos moleculares involucrados en la diferenciación neuronal de células madre mesenquimales humanas derivadas de tejido adiposo

    OpenAIRE

    Cardozo, Alejandra Johana

    2014-01-01

    Cardozo, A. J. (2015). Mecanismos moleculares involucrados en la diferenciación neuronal de células madre mesenquimales humanas derivadas de tejido adiposo (Tesis de Doctorado). Universidad Nacional de Quilmes, Bernal, Argentina. Como el sistema nervioso tiene una capacidad limitada de autorreparación, y la neurogénesis en el adulto está restringida a ciertas regiones del cerebro, existe un gran interés en la posibilidad de reparar el sistema nervioso a través de la utilización de técnica...

  17. Medida de similaridad entre imágenes de marcas de ganado mediante distribuciones de forma

    Directory of Open Access Journals (Sweden)

    Germán Sánchez Torres

    2014-12-01

    Full Text Available Este documento reporta los resultados de una investigación orientada hacia el diseño de un método de tratamiento de imágenes digitales para la automatización de los procesos de registro y control de marcas de ganado requeridas por las regulaciones del sector ganadero en Colombia. El método permite automatizar los procesos de búsqueda y de comparación necesarios para garantizar la unicidad de las marcas dentro de un sistema asistido por computadora. Se inicia con la generación de un histograma estimado de la geometría de la marca, lo que permite comparar y detectar similitudes entre las figuras previamente almacenadas, mediante una métrica de similitud basada en la distancia de Minkowski. Los resultados obtenidos indican que el método es adecuado para realizar un proceso de discriminación de dichas imágenes, reducir las ambigüedades y garantizar la unicidad de los registros. Los resultados obtenidos y un análisis de su aplicación son reportados.

  18. Electroestimulación neuromuscular intradiálisis, fuerza muscular, capacidad funcional y composición corporal

    Directory of Open Access Journals (Sweden)

    Sandra Rubio Páez

    2015-12-01

    Full Text Available Introducción: La capacidad funcional disminuida y la importante atrofia muscular caracterizan a los pacientes en hemodiálisis (HD. El ejercicio físico intradiálisis y recientemente la electroestimulación neuromuscular (EMS, representan dos serias opciones terapéuticas para mejorar esta deteriorada condición física. Actualmente, no existen estudios publicados sobre el papel de la EMS y la composición corporal en los pacientes en HD. Objetivo: Analizar que efecto produce un programa de EMS sobre la fuerza muscular, capacidad funcional, parámetros nutricionales y composición corporal en nuestros pacientes en HD. Material y Métodos: Estudio unicéntrico, prospectivo de 12 semanas de duración. Los pacientes incluidos realizaron un programa adaptativo de EMS en ambos cuádriceps intradiálisis mediante el dispositivo Compex R Theta 500i. Analizamos: 1.- Parámetros nutricionales (Albumina, pre albúmina, triglicéridos, colesterol total y fracciones, ferritina y Proteína C reactiva. 2.- Datos musculares: Composición muscular cuadriceps, Fuerza extensión máxima cuádriceps (FEMQ y handgrip (HG brazo dominante. 3.- Test funcionales: "Sit to stand to sit" (STS10 y "six-minutes walking test" (6MWT. 4.- Composición corporal mediante biompedancia electrica (BIA. Resultados: 13 pacientes incluidos: (69.2% hombres. Edad media: 65.7 años y 33.9 meses en HD. I.Charlson medio 9.1. La principal etiología de la ERC fue la DM (38.5%. Al final del estudio se observó una mejoría en (*p<0.05: FEMQ* (11.7±7.1 vs 13.4±7.4 Kg, STS10 (39.3±15.5 vs 35.8±13.7 seg, 6MWT* (9.9%, 293.2 vs 325.2 m. En relación a la composición corporal, se observó únicamente un aumento significativo del área muscular (AMQ*: 128.6 ± 30.2 vs 144.6 ± 22.4 cm² y una disminución del área grasa (AGQ*: 76.5 ± 26.9 vs 62.1 ± 20.1 cm² a nivel quadricipital, sin cambios en el resto de datos analizados (% grasa abdominal, peso graso, peso magro, agua corporal total

  19. Influencia del tipo de ejercicios de fuerza excéntrica en la prevención de lesiones musculares de bíceps femoral en futbolistas profesionales. Proyecto de investigación

    OpenAIRE

    Llurda Almuzara, Luis

    2017-01-01

    PREGUNTA DE INVESTIGACIÓN ¿Es más efectiva la prevención de lesiones musculares de bíceps femoral mediante ejercicios que se asemejen al mecanismo lesional en comparación con ejercicios que no tengan en cuenta este aspecto en futbolistas profesionales? OBJETIVOS El objetivo general de este proyecto de investigación es valorar si la elección de unos ejercicios u otros influye o no en el número de lesiones musculares de bíceps femoral registradas durante una temporada en futbolistas pr...

  20. Muscular Dystrophy

    Science.gov (United States)

    ... sets of muscles and cause different degrees of muscle weakness. Duchenne muscular dystrophy is the most common and the most severe ... can walk independently. Prednisone If a child has Duchenne muscular ... to help slow the rate of muscle deterioration. By doing so, the child may be ...

  1. Injertos de tejido adiposo: variables que influyen en la viabilidad del adipocito y de las células madre mesenquimales Fat grafting: variables that influence the viability of the adipocyte and mesenchimal stem cells

    Directory of Open Access Journals (Sweden)

    J. Benito Ruiz

    2011-12-01

    Full Text Available Si bien los injertos de tejido adiposo se han venido utilizando durante décadas a lo largo del siglo pasado, la técnica se venía considerando como poco útil dado que el injerto tendía a la reabsorción. Fue Coleman quien introdujo un sistema de obtención-procesamiento-infiltración atraumático que aumentó el porcentaje de supervivencia del injerto y la obtención de resultados positivos similares en su aplicación en todo el mundo. Aún así, la publicación de resultados dispares en la literatura respecto a la supervivencia del injerto, con diferentes porcentajes de reabsorción y el descubrimiento de las células madre en el tejido adiposo, han llevado a la realización de numerosos trabajos de investigación encaminados a comprender la biología del injerto de tejido adiposo, a estudiar el papel de las células madre en este proceso y la influencia que sobre la viabilidad celular tienen los distintos pasos a los que los cirujanos plásticos sometemos al tejido adiposo para su trasplante. Hemos hecho una revisión de la literatura científica al respecto para reunir la información disponible sobre estas controversias.Though adipose tissue grafts have been used for decades in last century, the technique has been considered as slightly usefull provided that the graft was tending to reabsorption. It was Coleman who introduced a system of atraumatic harvesting-processing-infiltration that increased the percentage of survival of the graft obtaining positive similar results in its application all over the world. Nonetheless, publication of unlike results in the literature regarding survival of the graft with different percentages of reabsorption and the discovery of stem cells in the adipose tissue, have led to the accomplishment of numerous research works directed to understanding the biology of the fat graft, the role of the stem cells in this process and the influence that the different steps for transplantation have on the cellular

  2. Curva dos valores normais de peso fetal estimado por ultra-sonografia segundo a idade gestacional

    OpenAIRE

    Cecatti,José Guilherme; Machado,Maria Regina Marrocos; Santos,Fernanda Fioravanti Azank dos; Marussi,Emílio Francisco

    2000-01-01

    Este trabalho teve por objetivo avaliar a evolução do peso fetal estimado em gestações normais de 20 a 42 semanas. Quanto ao desenho e métodos empregados na pesquisa, realizou-se estudo descritivo de um universo constituído por 2.874 gestantes normais da cidade de Campinas, efetuando-se exame ultra-sonográfico obstétrico de rotina com medida da biometria fetal e utilizando-se, para o cálculo do peso fetal, a fórmula de Hadlock et al. (1991). Calcularam-se os valores dos percentis 10, 50 e 90 ...

  3. Ratos tratados com dieta cetogênica apresentam aumento de tecido adiposo mediado pela elevação da atividade da fosfoenolpiruvato carboxicinase

    OpenAIRE

    Leticia Carina Ribeiro

    2005-01-01

    A Dieta Cetogênica é caracterizada pelo alto teor de gordura e baixo teor de carboidratos e proteínas, e tem sido proposta como benéfica em crianças com desordens epiléticas que não respondem ao tratamento convencional de drogas anti-epiléticas. O retardo de crescimento é um importante inconveniente desta dieta e as causas metabólicas ainda não estão bem caracterizadas. O objetivo desse estudo é examinar a variação de peso corporal e de tecido adiposo de ratos Wistar tratados com a dieta ceto...

  4. Limb-Girdle Muscular Dystrophy (LGMD)

    Science.gov (United States)

    ... Marie-Tooth Disease (CMT) Congenital Muscular Dystrophy (CMD) Duchenne Muscular Dystrophy (DMD) Emery-Dreifuss Muscular Dystrophy Endocrine Myopathies Metabolic Diseases of Muscle Mitochondrial Myopathies (MM) Myotonic Dystrophy (DM) Spinal-Bulbar ...

  5. Evaluation of Limb-Girdle Muscular Dystrophy

    Science.gov (United States)

    2014-03-06

    Becker Muscular Dystrophy; Limb-Girdle Muscular Dystrophy, Type 2A (Calpain-3 Deficiency); Limb-Girdle Muscular Dystrophy, Type 2B (Miyoshi Myopathy, Dysferlin Deficiency); Limb-Girdle Muscular Dystrophy, Type 2I (FKRP-deficiency)

  6. Papel de la vitamina D y su receptor (VDR) en tejido adiposo y su relación con el metabolismo de los hidratos de carbono

    OpenAIRE

    Muñoz Garach, Araceli

    2014-01-01

    El déficit de vitamina D es una patología frecuente que se relaciona con el desarrollo de diabetes y con las alteraciones de los sujetos con obesidad. La asociación de estas tres entidades no esta claramente establecida. Objetivos Cuantificar los niveles plasmáticos de vitamina D y la expresión de su receptor VDR en tejido adiposo de sujetos clasificados según su índice de masa corporal (IMC) y su perfil glucémico. Pacientes y métodos Se analizaron 119 sujetos y clasificados según...

  7. Muscular dystrophy in a dog resembling human becker muscular dystrophy.

    Science.gov (United States)

    Baroncelli, A B; Abellonio, F; Pagano, T B; Esposito, I; Peirone, B; Papparella, S; Paciello, O

    2014-05-01

    A 3-year-old, male Labrador retriever dog was presented with clinical signs of progressive exercise intolerance, bilateral elbow extension, rigidity of the forelimbs, hindlimb flexion and kyphosis. Microscopical examination of muscle tissue showed marked variability in myofibre size, replacement of muscle with mature adipose tissue and degeneration/regeneration of muscle fibres, consistent with muscular dystrophy. Immunohistochemical examination for dystrophin showed markedly reduced labelling with monoclonal antibodies specific for the rod domain and the carboxy-terminal of dystrophin, while expression of β-sarcoglycan, γ-sarcoglycan and β-dystroglycan was normal. Immunoblotting revealed a truncated dystrophin protein of approximately 135 kDa. These findings supported a diagnosis of congenital canine muscular dystrophy resembling Becker muscular dystrophy in man. Copyright © 2014 Elsevier Ltd. All rights reserved.

  8. Protocolo de extracción y procesamiento de células madre adultas del tejido adiposo abdominal: coordenadas del cirujano plástico en la investigación traslacional

    Directory of Open Access Journals (Sweden)

    E. Serna-Cuéllar

    2013-12-01

    Full Text Available La terapia basada en células madre adultas constituye la forma de tratamiento más innovadora para la regeneración de los tejidos patológicos, ausentes o deficitarios; también de relleno estético apropiado para variedad de indicaciones cosméticas y reconstructivas, y una de las líneas prioritarias de investigación traslacional en todo el mundo, evitando los problemas de rechazo inmune de los homotrasplantes, las complicaciones inherentes a la implantación de otros materiales aloplásticos, las secuelas estéticas o funcionales de las zonas donantes de los autotrasplantes de mayor morbilidad, y los problemas éticos del uso de células madre embrionarias. El cirujano plástico tiene un papel protagonista tanto en la extracción del injerto de células madre del abdomen mediante una liposucción manual tumescente, como en la aplicación clínica rápida sobre distintos defectos, deformidades o pérdidas de sustancia. Presentamos el protocolo de extracción y procesamiento de células madre derivadas del tejido adiposo (ADSC aprobado por el Comité Ético de Investigación Clínica, que resulta ser factible, seguro y reproducible, pudiendo servir de guía de actuación para el diseño de nuevos estudios clínicos controlados sobre la eficacia del autotrasplante de células madre aplicados a muchas otras patologías en el universo de la Cirugía Plástica en el que esté comprometida la cicatrización.

  9. Rhabdomyolysis featuring muscular dystrophies.

    Science.gov (United States)

    Lahoria, Rajat; Milone, Margherita

    2016-02-15

    Rhabdomyolysis is a potentially life threatening condition of various etiology. The association between rhabdomyolysis and muscular dystrophies is under-recognized in clinical practice. To identify muscular dystrophies presenting with rhabdomyolysis at onset or as predominant feature. We retrospectively reviewed clinical and laboratory data of patients with a genetically confirmed muscular dystrophy in whom rhabdomyolysis was the presenting or main clinical manifestation. Thirteen unrelated patients (males=6; females=7) were identified. Median age at time of rhabdomyolysis was 18 years (range, 2-47) and median duration between the first episode of rhabdomyolysis and molecular diagnosis was 2 years. Fukutin-related protein (FKRP) muscular dystrophy (n=6) was the most common diagnosis, followed by anoctaminopathy-5 (n=3), calpainopathy-3 (n=2) and dystrophinopathy (n=2). Four patients experienced recurrent rhabdomyolysis. Eight patients were asymptomatic and 3 reported myalgia and exercise intolerance prior to the rhabdomyolysis. Exercise (n=6) and fever (n=4) were common triggers; rhabdomyolysis was unprovoked in 3 patients. Twelve patients required hospitalization. Baseline CK levels were elevated in all patients (median 1200 IU/L; range, 600-3600). Muscular dystrophies can present with rhabdomyolysis; FKRP mutations are particularly frequent in causing such complication. A persistently elevated CK level in patients with rhabdomyolysis warrants consideration for underlying muscular dystrophy. Copyright © 2015 Elsevier B.V. All rights reserved.

  10. Força e arquitetura muscular do gémeo interno na bomba muscular venosa

    OpenAIRE

    Peixoto, Flávia; Pinto, Ângela; Kozlova, Veronika; Crisóstomo, Rute

    2015-01-01

    Objetivo: Avaliar e comparar a Força Muscular (FM), Amplitude de Movimento (ADM) e Arquitetura Muscular da bomba muscular venosa em sujeitos com e sem Insuficiência Venosa Crónica (IVC). Relevância: A IVC provoca alterações na função da bomba muscular venosa, no entanto, pouco se conhece acerca das suas repercussões físicas e funcionais. Amostra: Sujeitos com IVC (alterações da tróficas, e úlcera ativa/cicatrizada) e saudáveis. Foram avaliados 33 sujeitos dos quais foram analis...

  11. Influência do processo de familiarização para avaliação da força muscular em testes de 1-RM Influencia del proceso de familiarizacion para evaluación de la fuerza muscular en tests de 1-RM Influence of familiarization process on muscular strength assessment in 1-RM tests

    Directory of Open Access Journals (Sweden)

    Raphael Mendes Ritti Dias

    2005-02-01

    Full Text Available Embora testes de uma repetição máxima (1-RM sejam freqüentemente utilizados para a avaliação da força muscular, acredita-se que os resultados obtidos possam ser afetados pela falta de familiarização prévia, até mesmo em sujeitos com experiência em exercícios com pesos. Assim, o objetivo deste estudo foi investigar o impacto do processo de familiarização para avaliação da força muscular em testes de 1-RM. Para tanto, 21 homens (24,5 ± 3,8 anos, aparentemente saudáveis, com experiência prévia de pelo menos seis meses em treinamento com pesos, foram submetidos a testes repetitivos de 1-RM nos exercícios supino em banco horizontal, agachamento e rosca direta de bíceps. Os testes foram executados em quatro sessões, intervaladas a cada 48-72 horas. Um número máximo de três tentativas, com intervalo de três a cinco minutos para recuperação, foi utilizado em cada exercício, nas quatro sessões de testagem. ANOVA para medidas repetidas, seguida pelo teste post hoc de Tukey, quando p 0,05, bem como entre a terceira e a quarta sessão no supino em banco horizontal e no agachamento (p > 0,05. Os resultados indicam que a falta de familiarização prévia com testes de 1-RM pode comprometer a avaliação da força muscular. Portanto, sugere-se, para avaliação mais acurada da força muscular mediante testes de 1-RM, a execução de duas a três sessões de familiarização em homens adultos com experiência em exercícios com pesos.Embora la utilización de tests de una repetición máxima (1-RM es frecuente para la evaluación de la fuerza muscular, se acredita que los resultados obtenidos puedan ser afectados por la falta de familiarización previa, hasta mismo en sujetos con experiencia en ejercicios con pesos. Asi, el objetivo de este estudio fué investigar el impacto del proceso de familiarización para evaluación de la fuerza muscular en tests de 1-RM. Por lo tanto, 21 hombres (24,5 ± 3,8 años, aparentemente

  12. Structural Organization of Muscular Elements of a Skin-Muscular Sac of Trematodes: Literature Survey

    OpenAIRE

    Kanat Kambarovich Akhmetov; Irina Yurievna Chidunchi

    2015-01-01

    The issue of structural organization of muscular elements of a trematodes’ skin-muscular sac is considered in the study. Special attention is paid to an analysis of materials of preceding researches, study of foreign authors and also to additional literature reflecting peculiarities of structure of a trematodes’ body muscular system. The stated issue is insufficiently studied and calls for further researches. A comparative analysis of places of trematodes’ localization, taking into considerat...

  13. Spinal Muscular Atrophy FAQ

    Science.gov (United States)

    ... as ALS (Lou Gehrig’s Disease), cystic fibrosis and Duchenne muscular dystrophy. Approximately 1 in 50 Americans, or about 6 ... Pediatric Neuromuscular Clinical Research Network ( PNCR ) and the Muscular ... is the SMN2 gene? Muscle weakness and atrophy in SMA results from the ...

  14. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... with mental retardation Muscular dystrophy, congenital, Fukuyama type Muscular dystrophy, congenital, with central nervous system involvement Polymicrogyria with muscular dystrophy Related Information How ...

  15. Distrofia muscular de Emery-Dreifuss: relato de caso Emery-Dreifuss muscular dystrophy: case report

    Directory of Open Access Journals (Sweden)

    Ana Lucila Moreira Carsten

    2006-06-01

    Full Text Available A distrofia muscular de Emery-Dreifuss é uma forma de distrofia muscular freqüentemente associada a contraturas articulares e defeitos de condução cardíaca, que pode ser causada pela deficiência da proteína emerina na membrana nuclear interna das fibras musculares. Descrevemos o caso de um homem de 19 anos com diminuição de força muscular, hipotrofia nas cinturas escapular e pélvica, disfagia, contraturas articulares em cotovelos e tornozelos, apresentando história familiar compatível com herança ligada ao cromossomo X. A investigação mostrou creatinaquinase sérica elevada, eletrocardiograma com bloqueio atrioventricular de primeiro grau e bloqueio de ramo direito, eletroneuromiografia normal, biópsia muscular com alterações miopáticas e a análise por imuno-histoquímica mostrou deficiência de emerina. São discutidas as manifestações clínicas e genéticas, alterações laboratoriais e eletroneuromiográficas, bem como, a importância do estudo do padrão de herança no aconselhamento genético destas famílias.The Emery-Dreifuss muscular dystrophy is a form of muscular dystrophy that frequently presents early contractures and cardiac conduction defects, caused by emerin deficiency in the inner nuclear membrane of the muscular fibers. A 19-years-old man it presented muscle weakness and hypotrophy in the proximal upper and lower limbs, dysphagia and early contractures in elbows and ankles, with familiar history compatible with X-linked inheritance form. The investigation showed increased serum creatinekinase levels electrocardiogram had a first degree atrioventricular block and right bundle branch block normal electromyography and nerve conduction study muscle biopsy disclosed myopathic characteristics and nuclear protein immunohystochemical analysis showed deficiency of emerin. The clinical and genetics manifestations, laboratorial and electromyography changes, as well as, the study of the pattern of inheritance for

  16. Relaci??n entre la masa muscular, la densidad mineral ??sea, la fuerza muscular, la aptitud funcional y la calidad muscular en personas mayores

    OpenAIRE

    Pati??o Villada, Fredy Alonso

    2015-01-01

    294 p. La tesis pretende determinar la frecuencia de la sarcopenia y osteporosis , problemas que afectan a la salud de las personas mayores, y analizar la relaci??n entre la masa muscular, la densidad mineral ??sea (DMO), la fuerza muscular, la aptitud funcional y la calidad muscular (CM). La muestra estudiada fue la formada por un grupo de 83 hombres y 175 mujeres mayores de Le??n (Espa??a). El estudio transversal eval??a ??ndices de masa grasa y densidad mineral ??sea y niveles d...

  17. Para-muscular and trans-muscular approaches to the lumbar inter-vertebral foramen: an anatomical comparison.

    Science.gov (United States)

    Poetscher, Arthur Werner; Ribas, Guilherme Carvalhal; Yasuda, Alexandre; Nishikuni, Koshiro

    2005-03-01

    Foraminal and extra-foraminal disc herniations comprise up to 11.7% of all lumbar disc herniations. Facetectomy, which had been the classic approach, is now recognized as cause of pain and instability after surgery. Otherwise, posterior lateral approaches through a trans-muscular or a para-muscular technique offer no significant damage to key structures for spinal stability. The surgical anatomy of these approaches has already been described, but they were not compared. In order to quantify the angle of vision towards the intervertebral foramen offered by each technique, 12 fresh cadavers were dissected and studied regarding these approaches. The angle presented by trans-muscular approach was wider in all studied lumbar levels. Surgery through the trans-muscular approach is performed with a better working angle, requiring a smaller resection of surrounding tissues. Therefore, minor surgical trauma can be expected. Our measurements support previously published data that point the trans-muscular approach as the best surgical option.

  18. Orocaecal transit time in Duchenne muscular dystrophy.

    OpenAIRE

    Korman, S H; Bar-Oz, B; Granot, E; Meyer, S

    1991-01-01

    Smooth muscle degeneration may occur in Duchenne muscular dystrophy. We measured fasting orocaecal transit time in patients with advanced Duchenne muscular dystrophy and other muscular dystrophies and in healthy controls. No significant differences were found. In contrast to reports of gastric hypomotility in Duchenne muscular dystrophy, we found no evidence of impaired small intestinal motility.

  19. Estimated saving potential and efficient use of energy in air conditioning by means of the solar protection; Potencial estimado de ahorro y uso eficiente de energia en aires acondicionados mediante la proteccion solar

    Energy Technology Data Exchange (ETDEWEB)

    Mejia D, David; Morillon G, David; Rodriguez V, Luis [Universidad Nacional Autonoma de Mexico, Mexico, D.F. (Mexico)

    2001-07-01

    In here it is presented the analysis of the impact of the solar gains through the transparent parts of a prototype house of social interest, located in a dry warm climate. This analysis was made to determine the gains through windows for the summer time and under the following conditions: without outside solar protections, with the use of eaves, solar breakers and finally with the use of both systems of solar protection. The results were compared to obtain the considered potential of energy saving in cooling equipment, that could be obtained in future houses to build. [Spanish] Se presenta el analisis del impacto de las ganancias solares a traves de las partes transparentes de un prototipo de vivienda de interes social, ubicado en un clima calido seco. Dicho analisis se realizo para determinar las ganancias a traves de ventanas para la epoca de verano y bajo las siguientes condiciones: sin protecciones solares exteriores, con el empleo de aleros, con quiebrasoles y finalmente con el empleo de ambos sistemas de proteccion solar. Se compararon los resultados para obtener el potencial estimado de ahorro de energia en equipo de enfriamiento, que se tendria en las futuras viviendas por construir.

  20. Prevalence of muscular dystrophy in patients with muscular disorders in Tehran, Iran

    Directory of Open Access Journals (Sweden)

    Khadijeh Hajinaghi Tehrani

    2018-05-01

    Full Text Available Muscular dystrophy is a group of diseases that is characterized by progressive muscle wasting and the weakness of variable distribution and severity. On the basis of the distribution of predominant muscle weakness, there are many different kinds of muscular dystrophy. Some dystrophies are especially frequent in certain populations. There are no studies on the prevalence of muscular dystrophy in Iran. This study was aimed to survey the prevalence of muscular dystrophy among Iranian patients with muscular disorders. This analytical cross-sectional study was conducted on 1000 patients with musculoskeletal disorders who visited the dystrophy association of Bou-Ali Hospital (Tehran from June 2014 to June 2016. Patients’ data were extracted using a checklist that included age, gender, age of onset, family history, findings from clinical diagnostic tests and types of muscular dystrophy. The clinical findings were the results of genetic tests; EMG-NCV; para-clinical findings, including LDH and CPK; and pathological findings. All data were analyzed by SPSS V.22 (IBM Inc., NY with Chi Square and One way ANOVA tests. All analyses were performed with P = 0.05 considered as the threshold of statistical significant. Out of the 337 patients studied, 262 (77.7% were male and 75 (22.3% were female. Subjects had a mean (± SD age of 26.08 (± 11.86 years with an age range of 3 to 59 years. The most common types of muscular dystrophy were found to be Duchenne dystrophy (131 cases, 38.9%, limb-girdle dystrophy (91 cases, 27%, Becker dystrophy (58 cases, 17.2%, FSHD dystrophy (31 cases, 9.2%, and SMA (26 cases, 7.7%, respectively. The results showed that a statistically significant relationship between dystrophy types and gender, age, family history, age of diagnosis, CPK and LDH levels (P < 0.001. There were no statistical relationship between dystrophy types and pathological findings (P = 0.57, EMG-NCV test results (P = 0.062, and genetic findings (P = 0

  1. Dismorfia muscular Muscle dysmorphia

    Directory of Open Access Journals (Sweden)

    Sheila Seleri Marques Assunção

    2002-12-01

    Full Text Available Preocupações mórbidas com a imagem corporal eram tidas até recentemente como problemas eminentemente femininos. Atualmente estas preocupações também têm sido encontradas no sexo masculino. A dismorfia muscular é um subtipo do transtorno dismórfico corporal que ocorre principalmente em homens que, apesar da grande hipertrofia muscular, consideram-se pequenos e fracos. Além de estar associada a prejuízos sociais, ocupacionais, recreativos e em outras áreas do funcionamento do indivíduo, a dismorfia muscular é também um fator de risco para o abuso de esteróides anabolizantes. Este artigo aborda aspectos epidemiológicos, etiológicos e padrões clínicos da dismorfia muscular, além de tecer comentários sobre estratégias de tratamento para este transtorno.Morbid concern over body image was considered, until recently, a female issue. Nowadays, it has been viewed as a common male disorder. Muscle dysmorphia, a subtype of a body dysmorphic disorder, affects men who, despite having clear muscular hypertroph,y see themselves as frail and small. Besides being associated to major social, leisure and occupational dysfunction, muscle dysmorphia is also a risk factor for the abuse of steroids. This article describes epidemiological, etiological and clinical characteristics of muscle dysmorphia and comments on its treatment strategy.

  2. A study of atriphos (ATP) action on muscular circulation in progressive muscular dystrophy by the radioactive xenon clearance technique

    International Nuclear Information System (INIS)

    Chakyrov, B.; Samardzhiev, A.

    1977-01-01

    The effect of intramuscularly and intravenously adminostered atriphos on the muscular circulation was studied with radioactive xenon in 12 children with progressive muscular dystrophy. After combined local intramuscular injection of ATP (atriphos) with the radioactive marker a 12-fold increment of muscular circulation ensues, lasting about 15 minutes. No vasodilatating effect on the muscular flow was oberved after intravenous injection of 20-40 mg of atriphos. It is believed that intramuscular administration of atriphos produced dilatation of capillaries and of the venous part of the muscular circulation. (author)

  3. ENVEJECIMIENTO, MASA MUSCULAR Y ENTRENAMIENTO DE LA FUERZA: UNA REVISIÓN

    Directory of Open Access Journals (Sweden)

    Santiago Adolfo Arboleda Franco

    2014-11-01

    Full Text Available En este artículo se revisan los fundamentos sobre los cuales se ha reconocido la fuerza como elemento esencial en las prescripciones de ejercicio físico para la salud, específicamente en las personas mayores. Se describen las implicaciones de la sarcopenia como síndrome propio de este periodo vital y los hallazgos sobre la importancia de la masa muscular en el equilibrio homeostático mediante su función endocrina. También se examina lo propuesto desde consensos y declaraciones de asociaciones científicas sobre el entrenamiento de distintas manifestaciones de la fuerza en un contexto de salud y se revisa lo hallado en algunos meta–análisis y revisiones sistemáticas recientes sobre los efectos de este entrenamiento en distintas variables relacionadas con la capacidad funcional. Por último, se señalan algunos horizontes de investigación en el tema.

  4. A comparison of swallowing dysfunction in Becker muscular dystrophy and Duchenne muscular dystrophy.

    Science.gov (United States)

    Yamada, Yuka; Kawakami, Michiyuki; Wada, Ayako; Otsuka, Tomoyoshi; Muraoka, Kaori; Liu, Meigen

    2018-06-01

    Swallowing dysfunction has been reported in Duchenne muscular dystrophy (DMD), but has not been studied in Becker muscular dystrophy (BMD). The aims of this study were to report the characteristics of swallowing dysfunction in BMD compared with DMD. The study participants were 18 patients with BMD and 18 patients with DMD. All the patients were examined using videofluorography during swallowing of 5 mL of fluid. The penetration-aspiration scale (P-A scale) and the videofluorographic dysphagia scale (VDS) were used to evaluate dysphagia. Swinyard functional ability stage was not significantly different between the BMD and DMD groups. Rate of aspiration, P-A scale score, and total VDS score did not differ across groups, but the VDS item score for laryngeal elevation was lower in the BMD group than in the DMD group (median scores 4.5 and 9, respectively; p Becker muscular dystrophy (BMD) was not well known. Eighteen patients with BMD and 18 patients with Duchenne muscular dystrophy were examined with videofluorography. Patients with BMD have swallowing problems similar to those observed in patients with DMD.

  5. Morphologic imaging in muscular dystrophies and inflammatory myopathies

    International Nuclear Information System (INIS)

    Degardin, Adrian; Lacour, Arnaud; Vermersch, Patrick; Morillon, David; Cotten, Anne; Stojkovic, Tanya

    2010-01-01

    To determine if magnetic resonance imaging (MR imaging) is useful in the diagnostic workup of muscular dystrophies and idiopathic inflammatory myopathies for describing the topography of muscle involvement. MR imaging was performed in 31 patients: 8 with dystrophic myotony types 1 (n = 4) or 2 (n = 4); 11 with limb-girdle muscular dystrophy, including dysferlinopathy, calpainopathy, sarcoglycanopathy, and dystrophy associated with fukutin-related protein mutation; 3 with Becker muscular dystrophy; and 9 with idiopathic inflammatory myopathies, including polymyositis, dermatomyositis, and sporadic inclusion body myositis. Analysis of T1 images enabled us to describe the most affected muscles and the muscles usually spared for each muscular disease. In particular, examination of pelvis, thigh, and leg muscles demonstrated significant differences between the muscular diseases. On STIR images, hyperintensities were present in 62% of our patients with muscular dystrophies. A specific pattern of muscular involvement was established for each muscular disease. Hyperintensities observed on STIR images precede fatty degeneration and are not specific for inflammatory myopathies. (orig.)

  6. Morphologic imaging in muscular dystrophies and inflammatory myopathies

    Energy Technology Data Exchange (ETDEWEB)

    Degardin, Adrian; Lacour, Arnaud; Vermersch, Patrick [CHU de Lille, Clinique neurologique, Lille (France); Morillon, David; Cotten, Anne [CHRU de Lille, Service de Radiologie Osteoarticulaire, Hopital Roger Salengro, Lille (France); Stojkovic, Tanya [G-H Pitie-Salpetriere, Institut de Myologie, Paris (France)

    2010-12-15

    To determine if magnetic resonance imaging (MR imaging) is useful in the diagnostic workup of muscular dystrophies and idiopathic inflammatory myopathies for describing the topography of muscle involvement. MR imaging was performed in 31 patients: 8 with dystrophic myotony types 1 (n = 4) or 2 (n = 4); 11 with limb-girdle muscular dystrophy, including dysferlinopathy, calpainopathy, sarcoglycanopathy, and dystrophy associated with fukutin-related protein mutation; 3 with Becker muscular dystrophy; and 9 with idiopathic inflammatory myopathies, including polymyositis, dermatomyositis, and sporadic inclusion body myositis. Analysis of T1 images enabled us to describe the most affected muscles and the muscles usually spared for each muscular disease. In particular, examination of pelvis, thigh, and leg muscles demonstrated significant differences between the muscular diseases. On STIR images, hyperintensities were present in 62% of our patients with muscular dystrophies. A specific pattern of muscular involvement was established for each muscular disease. Hyperintensities observed on STIR images precede fatty degeneration and are not specific for inflammatory myopathies. (orig.)

  7. Learning about Duchenne Muscular Dystrophy

    Science.gov (United States)

    ... protein. Often these boys are classified as having Becker muscular dystrophy. Genetic testing (looking at the body's genetic instructions) ... National Library of Medicine Web site Duchenne and Becker muscular dystrophy [ghr.nlm.nih.gov] From Genetics Home Reference ...

  8. A lesão muscular na miastenia grave: estudo de 17 casos com histoquimica muscular

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1982-03-01

    Full Text Available Estudo de 17 biópsias musculares de pacientes com miastenia grave, utilizando técnicas de coloração a fresco e histoquímica muscular. Foram encontradas 15 biópsias musculares anormais, sendo que as principais alterações foram fibras musculares angulares escuras atróficas, excesso de gotículas de gordura na membrana externa das fibras, variação no diâmetro das fibras e atrofia de fibras do tipo II. Os achados foram interpretados como denervação em 11 biópsias, atrofia de fibras do tipo II em 7, infiltrado linfocitário em 4, necrose de fibras musculares com fagocitose em 1 e em 2 biópsias não foi encontrada qualquer anormalidade. Quanto maior o tempo de doença, mais severa foi a anormalidade encontrada. Dois pacientes apresentavam timoma, um miastenia grave congênita, um artrite reumatoide, um neurite hipertrófica intersticial, um tireoidite de Hashimoto e um com síndrome miastênica concomitante. São discutidos os achados anatomopatológicos e sua possível explicação.

  9. Modelamiento de la marcha humana con prótesis de miembro inferior mediante herramientas de simulación dinámica (“una aplicación en opensim”)

    OpenAIRE

    Silva Castellanos, Christian Augusto

    2015-01-01

    Se presenta el proceso que se siguió para simular la marcha de un paciente con amputación transfemoral mediante la captura de movimiento en un laboratorio de marcha y su correspondiente simulación con el software OpenSim de la Universidad de Stanford, con el cual se pudo simular la cinemática inversa, fuerzas de reacción tridimensional y activaciones musculares del mismo en contraste con los datos capturados de un paciente con marcha normal, para así identificar las contribuciones de la prót...

  10. Asociación del tejido adiposo epicárdico con la fibrilación auricular y su pronóstico posterior a la ablación de venas pulmonares

    Directory of Open Access Journals (Sweden)

    Viviana Quintero-Yepes

    2017-05-01

    Conclusiones: el aumento del tejido adiposo epicárdico se relaciona de manera independiente con el sobrepeso y la obesidad, así como con la severidad y el pronóstico de la fibrilación auricular. Deben hacerse estudios prospectivos de cohortes en los que se evalúe y establezcan valores de corte normales para nuestra población, al igual que seguimiento a largo plazo de la evolución posterior a ablación de venas pulmonares y a intervenciones como disminución de peso.

  11. Efecto comparativo de una dieta rica en grasa monoinsaturada sobre la disfunción del tejido adiposo en pacientes con síndrome metabólico en estado posprandial

    OpenAIRE

    Meneses Álvarez, María Eugenia

    2013-01-01

    Introducción: Una de las características más comunes de los pacientes con síndrome metabólico (SMet) es la obesidad abdominal. El aumento y expansión que experimenta el tejido adiposo (TA) en personas obesas provoca una serie de alteraciones fisiopatológicas entre las que destaca la disfunción del TA. La exposición a un balance positivo de energía durante un largo periodo de tiempo produce fenómenos de hiperplasia (incrementa el número de adipocitos) e hipertrofia (incrementa e...

  12. Obtención y criopreservación de células madre del tejido graso mediante liposucción Collecting and criopreservation of stem cells obtained by liposuction from fat tissue

    Directory of Open Access Journals (Sweden)

    J. Planas Ribo

    2011-12-01

    Full Text Available Existen diferentes técnicas para la obtención directa de células madre embrionarias a través de embriones criopreservados en nitrógeno líquido (-196 ºC, blastómeros individuales, activación de ovocitos por transferencia nuclear y células madre del líquido amniótico. También es posible obtener células madre adultas hematopoyéticas y de la médula ósea, más conocidas y empleadas en la clínica desde hace tiempo, así como también de la sangre del cordón umbilical en recién nacidos y de la grasa corporal en adultos. La grasa autóloga corporal extraída mediante liposucción se reutiliza en ocasiones para injertar o infiltrar otras zonas corporales y faciales como material de relleno, pero en la mayoría de los casos es desechada. Por lo general, para obtener un resultado satisfactorio en el trasplante de grasa autóloga, son necesarios varios tiempos quirúrgicos por lo que sería muy útil contar con un banco de tejido adiposo personalizado. Existen diferentes estudios que demuestran que el tejido adiposo aspirado por liposucción convencional puede preservarse y almacenarse con éxito a baja temperatura (-85 ºC en banco de tejido para futuras aplicaciones. Las células madre mesenquimales (MSCs poseen gran capacidad de proliferación y diferenciación con un alto valor terapéutico. El tejido graso es un reservorio importante de MSCs capaces de diferenciarse en tejido óseo, cartilaginoso, muscular, etc. Si reservamos una pequeña cantidad del tejido graso lipoaspirado, podemos obtener una cantidad suficiente de MSCs y conservarlas mediante un método óptimo de criopreservación. En 2007 realizamos un estudio de la grasa lipoaspirada en 36 pacientes para demostrar y validar el método de obtención de tejido graso con su posterior traslado, manipulación y criopreservación en banco de células madre. Los resultados fueron satisfactorios y en junio del 2010 se validó el método de recogida, procesamiento y almacenamiento

  13. Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy

    DEFF Research Database (Denmark)

    Sveen, Marie-Louise; Thune, Jens Jakob; Køber, Lars

    2008-01-01

    OBJECTIVE: To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD). DESIGN: Prospective screening. SETTING: Neuromuscular Clinic and Department of Cardiology...

  14. Muscular dystrophy

    Science.gov (United States)

    ... are no known cures for the various muscular dystrophies. The goal of treatment is to control symptoms. Physical therapy may help maintain muscle strength and function. Leg braces and a wheelchair ...

  15. Muscular Oxygen Uptake Kinetics in Aged Adults.

    Science.gov (United States)

    Koschate, J; Drescher, U; Baum, K; Eichberg, S; Schiffer, T; Latsch, J; Brixius, K; Hoffmann, U

    2016-06-01

    Pulmonary oxygen uptake (V˙O2) kinetics and heart rate kinetics are influenced by age and fitness. Muscular V˙O2 kinetics can be estimated from heart rate and pulmonary V˙O2. In this study the applicability of a test using pseudo-random binary sequences in combination with a model to estimate muscular V˙O2 kinetics was tested. Muscular V˙O2 kinetics were expected to be faster than pulmonary V˙O2 kinetics, slowed in aged subjects and correlated with maximum V˙O2 and heart rate kinetics. 27 elderly subjects (73±3 years; 81.1±8.2 kg; 175±4.7 cm) participated. Cardiorespiratory kinetics were assessed using the maximum of cross-correlation functions, higher maxima implying faster kinetics. Muscular V˙O2 kinetics were faster than pulmonary V˙O2 kinetics (0.31±0.1 vs. 0.29±0.1 s; p=0.004). Heart rate kinetics were not correlated with muscular or pulmonary V˙O2 kinetics or maximum V˙O2. Muscular V˙O2 kinetics correlated with maximum V˙O2 (r=0.35; p=0.033). This suggests, that muscular V˙O2 kinetics are faster than estimates from pulmonary V˙O2 and related to maximum V˙O2 in aged subjects. In the future this experimental approach may help to characterize alterations in muscular V˙O2 under various conditions independent of motivation and maximal effort. © Georg Thieme Verlag KG Stuttgart · New York.

  16. Genetics Home Reference: spinal muscular atrophy

    Science.gov (United States)

    ... difficulty breathing. Children with this type often have joint deformities (contractures) that impair movement. In severe cases, ... Proximal spinal muscular atrophy Washington University, St. Louis: Neuromuscular Disease Center: Spinal Muscular Atrophy Patient Support and ...

  17. Genetics Home Reference: Duchenne and Becker muscular dystrophy

    Science.gov (United States)

    ... Conditions Duchenne and Becker muscular dystrophy Duchenne and Becker muscular dystrophy Printable PDF Open All Close All Enable Javascript ... dystrophy occur almost exclusively in males. Duchenne and Becker muscular dystrophies have similar signs and symptoms and are caused ...

  18. Cardiac involvement in children with neuro-muscular disorders

    Directory of Open Access Journals (Sweden)

    E. N. Arkhipova

    2015-01-01

    Full Text Available Many inherited neuromuscular disorders include cardiac involvement as a typical clinical feature. Among the most common of them is the group of muscular dystrophies. Dilated cardiomyopathy, ventricular arrhythmias, atrial fibrillations, atrioventricular and intraventricular conduction abnormalities, and sudden cardiac death are well known pathological findings in Duchenne muscular dystrophies, myotonic dystrophy type I and 2, Emery-Dreifuss muscular dystrophies and different types of limb-girdle muscular dystrophies and other disorders. Detection of cardiac pathology in patients with different muscular dystrophies is possible with ECG, echocardiography and cardiovascular magnetic resonance imaging, which are recommended for screening and early cardioprotective treatment.

  19. Caracterización fenotípica de células madre mesenquimales humanas de médula ósea y tejido adiposo. Resultados preliminares

    Directory of Open Access Journals (Sweden)

    Consuelo Macías-Abraham

    Full Text Available Introducción:las células madre mesenquimales (CMM poseen características fenotípicas y funcionalesque les confieren un amplio potencial terapéutico por su posible uso en la terapia celular regenerativa, en el rechazo del trasplante alogénico y en enfermedades inflamatorias crónicas. Objetivo: evaluar la expresión de moléculas de membrana que permiten identificar la expresión de patrones moleculares característicos de CMM humanas mantenidas en cultivo. Métodos: se estudió la expresión fenotípica de células mononucleares procedentes de médula ósea obtenidas mediante aspiración medular, separadas por gradiente de Ficoll y cultivadas ex vivo entre los pases o subcultivos 3 y 16; y adipocitos cultivados procedentes de la extracción enzimática de tejido adiposo de donantes sanos. Se realizó doble marcaje para las moléculas CD34/CD45, CD34/CD90, CD34/CD117, y CD34/CD44. Resultados: en los resultados preliminares obtenidos se observó que las células cultivadas procedentes de médula ósea, entre los pases 4 y 8 de cultivo expresaron 45,13 % de células CD34-/CD45- (doblemente negativas, lo que correspondió con el 25,24 % de células CD34-/CD90+ y el 96,90 % de CD34-/CD117-. En las células procedentes de cultivo de adipocitos se observó el52,3 % de CD34-/CD45- (doblemente negativas,12,31 % de CD34-/CD90+,43,31 % de CD34-/CD117- y 64,68 % de CD34-/CD44+.Estos resultados sugieren que ambos cultivos se diferenciaron a CMM. Las CMM procedentes de adipocitos mostraron el 64,68 % de células con expresión de la molécula de adhesión CD44 a la que se atribuyen propiedades funcionalescomo el asentamiento tisular. Conclusiones: estos resultados preliminares permiten corroborar que ambos métodos experimentales de cultivo son efectivos para la obtención de CMM con fines terapéuticos.

  20. Muscular Imbalance Correction in the Power Fitness Training

    OpenAIRE

    Olga E. Aftimichuk; Alexander V. Varvarich

    2013-01-01

    Muscular imbalance is one of the manifestations of pathological-biomechanical changes in muscular-skeletal system. It is the result of tonus-power imbalance of short and relaxed muscles. Muscle shortening is the most striking sign of muscular imbalance. Hypodynamia and passive lifestyle can cause such results. The paper justifies the experimental technique of women muscular imbalances correction by means of power training. Selection of exercises, weights and machines was made, taking into acc...

  1. Avaliação histológica do tecido adiposo da pele de ratas sob ação de cafeína e Cafeisilane® C

    OpenAIRE

    Clara Tsugumi Nakamura Tano

    2003-01-01

    Hidrolipodistrofia ginóide é uma alteração genuína e loco-regional do panículo adiposo subcutâneo determinante do formato corporal característico da mulher. Várias substâncias ativas podem ser empregadas como anticelulíticos, sendo selecionado para esse trabalho a cafeína e o silício orgânico da cafeína (Cafeisilane® C). Foram desenvolvidas formulações cosméticas (9 emulsões empregando como base cera emulsionante não iônica e 3 géis utilizando polímero carboxivinílico e 1 gel a base de ...

  2. Tratamiento Manual Postcirugía en un caso Clínico de Transposición muscular del Romboides y Angular de la Escápula

    OpenAIRE

    Gómez García, Ana

    2009-01-01

    Descripción de un caso clínico en la que se secciona el nervio espinal derecho (neurotmesis) tras la exéresis de un quiste en el cuello. Debido a la falta de transmisión neuronal se produce una parálisis funcional en el músculo trapecio y en el músculo esternocleidomastoideo derecho y se suple sus funciones mediante una reinserción del músculo elevador de la escápula y del músculo romboides de dicho lado que recibe el nombre de transposición muscular. Se realiza tratamiento de fisioter...

  3. Muscular Dystrophy

    Science.gov (United States)

    ... Surveillance Tracking and Research Network , known as MD STAR net . Learn more about CDC’s other muscular dystrophy ... for Disease Control and Prevention Email Recommend Tweet YouTube Instagram Listen Watch RSS ABOUT About CDC Jobs ...

  4. Composición corporal de escolares pertenecientes a diferentes contextos socioeconómicos de Puerto Madryn, Chubut, Argentina

    Directory of Open Access Journals (Sweden)

    Dahinten, Silvia Lucrecia

    2007-01-01

    Full Text Available El desigual desarrollo del tejido muscular y de la distribución de tejido adiposo que tiene lugar durante el crecimiento humano presenta dimorfismo sexual (DS, en condiciones de vida saludables. Esta variación ha sido traducida en algoritmos utilizando la circunferencia y pliegues subcutáneos del brazo y se han elaborado ecuaciones para calcular áreas. El objetivo fue evaluar el DS observado en el crecimiento corporal de escolares de 6 a 14 años de hogares con diferente NBI -(NBI alto y (NBI bajo-. Se estudió transversalmente una muestra de 500 mujeres y 570 varones de la ciudad de Puerto Madryn, Chubut. Se relevaron: perímetro braquial y pliegues subcutáneos tricipital y subescapular y se calcularon las áreas adiposa (AAB y muscular (AMB. Se utilizaron pruebas de Wilcoxon, p= 0.05. El AAB de las mujeres fue a casi todas las edades superior a la de los varones (NBI bajo p=0.09; NBI alto p=0.02. A edades tempranas, el AMB mostró que los varones presentaron cifras similares a las mujeres. A partir de los 12 años, sin embargo, los varones comenzaron a incrementar la masa muscular. En las niñas este aumento se hizo mucho más lento (NBI alto p=0.01; NBI bajo p=0.03. La ausencia de DS en el NBI bajo, para los caracteres considerados, se relaciona con el aumento de tejido adiposo en los varones de este sector que muestra la adopción dentro de la comunidad, de dietas de alto contenido en grasas y carbohidratos acompañadas de altas tasas de obesidad.

  5. Entrenamiento y adaptación muscular: sustratos y vías metabólicas para la producción de energía

    Directory of Open Access Journals (Sweden)

    Federico Martín Boffi

    2008-07-01

    Full Text Available Tres isoformas de MyHC han sido caracterizadas en músculo esquelético de equinos adultos a nivel proteico: tipo I, IIA y IIX. La distribución de estas MyHCs definen tres tipos puros de fibras que contienen una sola isoforma (tipo I, IIA y IIX y dos tipos de fibras híbridas que expresan dos isoformas (I+IIA y IIAX. Las fibras híbridas IIAX existen en los músculos locomotores como una población estable y significativa. Dependiendo de la naturaleza del estímulo, la respuesta adaptativa puede tomar diferentes formas: hipertrofia, remodelación sin hipertrofia y respuesta mixta, cuando se combina la remodelación con la hipertrofia. Los equinos pueden aprovechar diferentes sustratos energéticos, algunos de los cuales son utilizados en forma inmediata tras la ingesta, mientras que otros son almacenados en el hígado, músculos y tejido adiposo para ser usados en otro momento. El glucógeno muscular y la glucosa pueden ser degradados por la vía aeróbica (oxidativa o la anaeróbica lactacida (glucolítica, mientras que en ejercicios de resistencia la utilización de ácidos grasos se realiza a partir de la degradación de triglicéridos almacenados en el tejido celular subcutáneo. El metabolismo oxidativo mide la función de los sistemas cardiovascular y respiratorio. En cambio, el metabolismo anaeróbico solo involucra al tejido muscular.Three isoforms of MyHc have been characterized in the skeletal muscle of adult equines at the proteins level: types I, IIA and IIX. The distribution of these MyHCs defines three pure kinds of fibre which contain a single isoform (I+IIA and IIAX. The hybrid fibres IIAX exist in the locomotive muscles as a permanent and significant population. The adjusting response may take different forms depending on the nature of the stimulus: hypertrophy, reorganization (restructuring without hypertrophy and a combined answer, when restructuring joins hypertrophy. The equines may take the most of different energetic

  6. Caffeine ingestion acutely enhances muscular strength and power but not muscular endurance in resistance-trained men.

    Science.gov (United States)

    Grgic, Jozo; Mikulic, Pavle

    2017-09-01

    The goal of this randomized, double-blind, cross-over study was to assess the acute effects of caffeine ingestion on muscular strength and power, muscular endurance, rate of perceived exertion (RPE), and pain perception (PP) in resistance-trained men. Seventeen volunteers (mean ± SD: age = 26 ± 6 years, stature = 182 ± 9 cm, body mass = 84 ± 9 kg, resistance training experience = 7 ± 3 years) consumed placebo or 6 mg kg -1 of anhydrous caffeine 1 h before testing. Muscular power was assessed with seated medicine ball throw and vertical jump exercises, muscular strength with one-repetition maximum (1RM) barbell back squat and bench press exercises, and muscular endurance with repetitions of back squat and bench press exercises (load corresponding to 60% of 1RM) to momentary muscular failure. RPE and PP were assessed immediately after the completion of the back squat and bench press exercises. Compared to placebo, caffeine intake enhanced 1RM back squat performance (+2.8%; effect size [ES] = 0.19; p = .016), which was accompanied by a reduced RPE (+7%; ES = 0.53; p = .037), and seated medicine ball throw performance (+4.3%, ES = 0.32; p = .009). Improvements in 1RM bench press were not noted although there were significant (p = .029) decreases in PP related to this exercise when participants ingested caffeine. The results point to an acute benefit of caffeine intake in enhancing lower-body strength, likely due to a decrease in RPE; upper-, but not lower-body power; and no effects on muscular endurance, in resistance-trained men. Individuals competing in events in which strength and power are important performance-related factors may consider taking 6 mg kg -1 of caffeine pre-training/competition for performance enhancement.

  7. CONSIDERAÇÕES SOBRE A OXIDAÇÃO LIPÍDICA E SUAS LIMITAÇÕES: o possível papel do fracionamento de carboidratos

    OpenAIRE

    Smolarek, André de Camargo; Gomes, Fernanda dos Santos; Souza Junior, Tácito Pessoa de

    2012-01-01

    Os ácidos graxos estocados principalmente na forma de triacilglicerois no tecido adiposo ou em reduzidas quantidades no tecido muscular representam uma fundamental via energética em exercícios de intensidade leve a moderada. Caracterizam-se pela alta disponibilidade de trifosfato de adenosina (ATP) possuindo relevância em termos quantitativos nos exercícios de longa duração (aeróbios). De forma simplificada, a oxidação lipídica divide-se em mobilização e transporte dos ácidos graxos, β-oxida...

  8. Three novel serum biomarkers, miR-1, miR-133a, and miR-206 for Limb-girdle muscular dystrophy, Facioscapulohumeral muscular dystrophy, and Becker muscular dystrophy.

    Science.gov (United States)

    Matsuzaka, Yasunari; Kishi, Soichiro; Aoki, Yoshitsugu; Komaki, Hirofumi; Oya, Yasushi; Takeda, Shin-Ichi; Hashido, Kazuo

    2014-11-01

    Muscular dystrophies are a clinically and genetically heterogeneous group of inherited myogenic disorders. In clinical tests for these diseases, creatine kinase (CK) is generally used as diagnostic blood-based biomarker. However, because CK levels can be altered by various other factors, such as vigorous exercise, etc., false positive is observed. Therefore, three microRNAs (miRNAs), miR-1, miR-133a, and miR-206, were previously reported as alternative biomarkers for duchenne muscular dystrophy (DMD). However, no alternative biomarkers have been established for the other muscular dystrophies. We, therefore, evaluated whether these miR-1, miR-133a, and miR-206 can be used as powerful biomarkers using the serum from muscular dystrophy patients including DMD, myotonic dystrophy 1 (DM1), limb-girdle muscular dystrophy (LGMD), facioscapulohumeral muscular dystrophy (FSHD), becker muscular dystrophy (BMD), and distal myopathy with rimmed vacuoles (DMRV) by qualitative polymerase chain reaction (PCR) amplification assay. Statistical analysis indicated that all these miRNA levels in serum represented no significant differences between all muscle disorders examined in this study and controls by Bonferroni correction. However, some of these indicated significant differences without correction for testing multiple diseases (P < 0.05). The median values of miR-1 levels in the serum of patients with LGMD, FSHD, and BMD were approximately 5.5, 3.3 and 1.7 compared to that in controls, 0.68, respectively. Similarly, those of miR-133a and miR-206 levels in the serum of BMD patients were about 2.5 and 2.1 compared to those in controls, 1.03 and 1.32, respectively. Taken together, our data demonstrate that levels of miR-1, miR-133a, and miR-206 in serum of BMD and miR-1 in sera of LGMD and FSHD patients showed no significant differences compared with those of controls by Bonferroni correction. However, the results might need increase in sample sizes to evaluate these three miRNAs as

  9. Atrofia muscular espinal en el niño Spinal muscular atrophy present in children

    Directory of Open Access Journals (Sweden)

    Nicolás Garófalo Gómez

    2009-09-01

    Full Text Available INTRODUCCIÓN. Las atrofias musculares espinales en la infancia (AME son trastornos genéticos autosómicos recesivos, caracterizados por degeneración de las motoneuronas espinales y bulbares. El presente estudio tuvo el objetivo principal de describir las principales características clínicas en una serie de niños con AME. MÉTODOS. Se realizó un estudio retrospectivo de los pacientes con AME atendidos en el Instituto de Neurología y Neurocirugía de Cuba, entre enero de 1997 y diciembre de 2001. Se recopilaron los datos de 35 pacientes, 4 de ellos, fetos con confirmación prenatal de AME. Se precisaron las principales características clínicas, electromiográficas, de la biopsia muscular y de los estudios genéticos moleculares realizados en cada caso. RESULTADOS. La AME de tipo II resultó la forma clínica más frecuente (58 %, seguida por la AME de tipo I (42 %. Las principales manifestaciones clínicas resultaron la debilidad muscular generalizada con predominio proximal en extremidades, asociada a hipotonía y arreflexia osteotendinosa. La deleción de los exones 7 y 8 del gen SMN1 se detectó en 20 de 23 casos estudiados (87 %.INTRODUCTION: Spinal muscular atrophies (SMA in childhood are autosomal recessive genetic disorders, characterized by spinal and bulbar motoneurons degenerations. Aim of present paper was to describe the main clinical features in a series of children presenting SMA. METHODS: A retrospective study of patients with SMA seen in the Neurology and Neurosurgery Institute of Cuba from January, 2997 and December, 2001 was made. Data from 35 patients were available; four of them were fetus with prenatal confirmation of SMA. Main clinical, electromyography, muscular biopsy, and of molecular genetic studies performed in each case were determined. RESULTS: Type II SMA was the more frequent clinical presentation (58%, followed by type I SMA (42,%. Main clinical manifestations were a systemic muscular weakness with

  10. Radiographic features of Golden Retriever muscular dystrophy.

    Science.gov (United States)

    Brumitt, Jason W; Essman, Stephanie C; Kornegay, Joe N; Graham, John P; Weber, William J; Berry, Clifford R

    2006-01-01

    Golden Retriever muscular dystrophy is an inherited, degenerative myopathy due to the absence of dystrophin and is used as a model of Duchenne muscular dystrophy of young boys. This report describes the radiographic abnormalities of Golden Retriever muscular dystrophy in 26 dogs. The thoracic abnormalities included diaphragmatic asymmetry (18/26), diaphragmatic undulation (18/26), and gastro-esophageal hiatal hernia (6/26). Pelvic abnormalities included narrowing of the body of the ilia (14/19), ventral deviation and curvature of the tuber ischii (14/19), elongation of the obturator foramen with a decrease in opacity of the surrounding bone (12/19), and lateral flaring of the wings of the ilia (12/19). Abdominal abnormalities consisted of hepatomegaly (14/22) and poor serosal detail (12/22). The unique thoracic abnormalities were a consistent finding in affected Golden Retriever muscular dystrophy dogs. The diagnosis of muscular dystrophy should be included in the differential list if the combination of diaphragm undulation and asymmetry, and gastro-esophageal hiatal hernia are identified. These diaphragmatic abnormalities are related to hypertrophy and hyperplasia of the diaphragm. Additionally, the skeletal changes of pelvic tilt, elongation of the pelvis, widening of the obturator foramina and thinning of the ischiatic tables appear to be specific to Golden Retriever muscular dystrophy in dogs. These pelvic abnormalities are most likely secondary to bone remodeling associated with the progressive skeletal myopathy and subsequent contracture/fibrosis.

  11. Viabilidade celular da fração mononuclear da medula óssea e fração vascular estromal do tecido adiposo de equinos após o processo de congelamento e descongelamento Viability of equine bone marrow mononuclear fraction and adipose tissue-derived stromal vascular fraction after freezing and thawing process

    Directory of Open Access Journals (Sweden)

    Gesiane Ribeiro

    2012-12-01

    Full Text Available Cinco cavalos adultos foram submetidos à coleta de medula óssea do esterno e de tecido adiposo da região glútea. As amostras foram processadas para obtenção da fração mononuclear da medula óssea e fração vascular estromal do tecido adiposo, o número de células obtidas e a viabilidade celular foram determinados. Em seguida, realizou-se o congelamento das amostras em solução contendo 20% de soro fetal bovino e 10% de dimetilsulfóxido. Depois de um mês, realizou-se o descongelamento das amostras e a viabilidade celular foi novamente mensurada. Os resultados revelaram que as técnicas utilizadas tanto para coleta de medula óssea quanto de tecido adiposo em equinos são simples, rápidas e seguras. As metodologias adotadas para o processamento das amostras foram eficientes, obtendo-se aproximadamente 95% de viabilidade celular. Após o descongelamento, a viabilidade média das amostras de células mononucleares da medula óssea foi de 86% e da fração vascular estromal do tecido adiposo de 64%. Frente à importância da terapia celular na clínica médica de equinos, concluiu-se que é necessária a realização de mais estudos, visando padronizar uma técnica de criopreservação que mantenha a integridade das células da fração mononuclear da medula óssea e da fração vascular estromal do tecido adiposo de equinos.In five adult horses, bone marrow was aspirated from the sternum and adipose tissue extracted from the gluteal region. The samples were processed to obtain the mononuclear fraction of bone marrow and stromal vascular fraction of adipose tissue, and the number of cells obtained and cell viability were determined. Next, the cell samples were frozen in medium containing 20% fetal bovine serum and 10% dimethylsulfoxide. After one month, the cells were thawed and cell viability was again determined. The results revealed that the techniques for collecting both bone marrow and adipose tissue in horses are simple, rapid and

  12. Tratamiento alternativo de la obesidad exógena

    OpenAIRE

    Ramos Padilla, Kenia; Ramos Padilla, Katia; Ledesma Neyra, Arelys; Camacho Díaz, René

    2013-01-01

    Introducción: la obesidad es una de origen multifactorial caracterizada por acumulación excesiva de grasa o hipertrofia general del tejido adiposo en el cuerpo; factor de riesgo conocido para múltiples enfermedades, con repercusión estética y social. Objetivo: obtener el peso ideal en pacientes obesos mediante la combinación de dieta, homeopatía e implantación de catgut en puntos de acupuntura, tratando de equilibrar la energía y cambiar estilos de vida. Material y método: se realizó un estud...

  13. What Are the Types of Muscular Dystrophy?

    Science.gov (United States)

    ... muscular dystrophy? There are more than 30 forms of muscular dystrophy (MD), with information on the primary types included in the table below. 1 Duchenne (DMD) What It Is Common Symptoms How It ...

  14. Parental attitudes toward newborn screening for Duchenne/Becker muscular dystrophy and spinal muscular atrophy.

    Science.gov (United States)

    Wood, Molly F; Hughes, Sarah C; Hache, Lauren P; Naylor, Edwin W; Abdel-Hamid, Hoda Z; Barmada, M Michael; Dobrowolski, Steven F; Stickler, David E; Clemens, Paula R

    2014-06-01

    Disease inclusion in the newborn screening (NBS) panel should consider the opinions of those most affected by the outcome of screening. We assessed the level and factors that affect parent attitudes regarding NBS panel inclusion of Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and spinal muscular atrophy (SMA). The attitudes toward NBS for DMD, BMD, and SMA were surveyed and compared for 2 categories of parents, those with children affected with DMD, BMD, or SMA and expectant parents unselected for known family medical history. The level of support for NBS for DMD, BMD, and SMA was 95.9% among parents of children with DMD, BMD, or SMA and 92.6% among expectant parents. There was strong support for NBS for DMD, BMD, and SMA in both groups of parents. Given advances in diagnostics and promising therapeutic approaches, discussion of inclusion in NBS should continue. Copyright © 2013 Wiley Periodicals, Inc.

  15. Mitochondrial disorders in progressive muscular dystrophies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of different progressive muscular dystrophies. It describes changes in Duchenne, limb-girdle, facial scapulohumeral (Landuzi—Degerina muscular dystrophies. The review is based on both clinical and experimental animal studies. Along with the implication of mitochondria in the pathogenesis of the diseases, it describes muscular dystrophy treatment options compensating for energy disorders and overcoming oxidative stress and mitochondrial dysfunction. Mitochondrial studies in different muscle diseases hand physicians treatment modalities that fail to lead to recovery, but compensate for disorders caused by mutations in the genetic apparatus. 

  16. Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): case definition in surveillance for childhood-onset Duchenne/Becker muscular dystrophy.

    Science.gov (United States)

    Mathews, Katherine D; Cunniff, Chris; Kantamneni, Jiji R; Ciafaloni, Emma; Miller, Timothy; Matthews, Dennis; Cwik, Valerie; Druschel, Charlotte; Miller, Lisa; Meaney, F John; Sladky, John; Romitti, Paul A

    2010-09-01

    The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in 1 of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by >4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either ''definite'' or ''probable'' Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982-1987) to 94% in the cohort born 2004 to 2009.

  17. Becker muscular dystrophy: an unusual presentation.

    OpenAIRE

    Thakker, P B; Sharma, A

    1993-01-01

    A 15 year old boy who presented with passing painless dark urine was found to have myoglobinuria. His creatine phosphokinase was raised, and a muscle biopsy specimen showed non-specific dystrophic changes. Subsequent DNA analysis led to the diagnosis of Becker muscular dystrophy. Myoglobinuria may be a presenting symptom of Becker muscular dystrophy.

  18. Oxidative muscular injury and its relevance to hyperthyroidism.

    Science.gov (United States)

    Asayama, K; Kato, K

    1990-01-01

    In experimental hyperthyroidism, acceleration of lipid peroxidation occurs in heart and slow-oxidative muscles, suggesting the contribution of reactive oxygen species to the muscular injury caused by thyroid hormones. This article reviews various models of oxidative muscular injury and considers the relevance of the accompanying metabolic derangements to thyrotoxic myopathy and cardiomyopathy, which are the major complications of hyperthyroidism. The muscular injury models in which reactive oxygen species are supposed to play a role are ischemia/reperfusion syndrome, exercise-induced myopathy, heart and skeletal muscle diseases related to the nutritional deficiency of selenium and vitamin E and related disorders, and genetic muscular dystrophies. These models provide evidence that mitochondrial function and the glutathione-dependent antioxidant system are important for the maintenance of the structural and functional integrity of muscular tissues. Thyroid hormones have a profound effect on mitochondrial oxidative activity, synthesis and degradation of proteins and vitamin E, the sensitivity of the tissues to catecholamine, the differentiation of muscle fibers, and the levels of antioxidant enzymes. The large volume of circumstantial evidence presented here indicates that hyperthyroid muscular tissues undergo several biochemical changes that predispose them to free radical-mediated injury.

  19. Estudio del balance energético en velocistas de alto rendimiento

    OpenAIRE

    Lorente Gutiérrez, Jesús

    2015-01-01

    El objetivo de este estudio fue evaluar el balance energético en tres atletas de alto rendimiento durante 28 días, que coincidieron con el periodo competitivo de pista cubierta. La ingesta energética fue estudiada a partir de registros alimentarios durante los 28 días. Del mismo modo, el gasto energético fue estimado por tres métodos, mediante registros de actividad durante 28 días, mediante el estudio del ritmo metabólico basal estudiado por calorimetría indirecta, aplicándole el fa...

  20. Muscular Dystrophy (MD)

    Science.gov (United States)

    ... patients may need assisted ventilation to treat respiratory muscle weakness and a pacemaker for cardiac abnormalities. View Full Treatment Information Definition The muscular dystrophies (MD) are a group of more than 30 ...

  1. Respiratory function in facioscapulohumeral muscular dystrophy 1

    NARCIS (Netherlands)

    Wohlgemuth, M.; Horlings, G.C.; Kooi, E.L. van der; Gilhuis, H.J.; Hendriks, J.C.M.; Maarel, S.M. van der; Engelen, B.G.M. van; Heijdra, Y.F.; Padberg, G.W.A.M.

    2017-01-01

    To test the hypothesis that wheelchair dependency and (kypho-)scoliosis are risk factors for developing respiratory insufficiency in facioscapulohumeral muscular dystrophy, we examined 81 patients with facioscapulohumeral muscular dystrophy 1 of varying degrees of severity ranging from ambulatory

  2. [Human myopathy and animal muscular dystrophy].

    Science.gov (United States)

    Schapira, G; Dreyfus, J C; Schapira, F

    1977-08-01

    Two hereditary muscular dystrophies similar to human progressive muscular dystrophy (P.M.D. Duchenne type) have been isolated in animals, one in mouse, the other in chicken. The decrease in the activity of glycogenolytic enzymes is similar to that observed in denervated muscle. Isozymic fetal types for several muscular enzymes have been observed as well in chicken as in man, but this fetal type may also be found in neurogenic atrophy. The release in circulation of muscle enzymes seems more specific. But the origin of the genetic lesion is still unknown. We describe here the three different theories about this problem: i.e. neurogenic, vascular, or myogenic. This last theory implies a trouble of membrane permeability.

  3. Recent developments in the treatment of Duchenne muscular dystrophy and spinal muscular atrophy

    Science.gov (United States)

    Liew, Wendy K. M.

    2013-01-01

    Pediatric neuromuscular disorders comprise a large variety of disorders that can be classified based on their neuroanatomical localization, patterns of weakness, and laboratory test results. Over the last decade, the field of translational research has been active with many ongoing clinical trials. This is particularly so in two common pediatric neuromuscular disorders: Duchenne muscular dystrophy and spinal muscular atrophy. Although no definitive therapy has yet been found, numerous active areas of research raise the potential for novel therapies in these two disorders, offering hope for improved quality of life and life expectancy for affected individuals. PMID:23634188

  4. Genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies.

    Science.gov (United States)

    Hightower, Rylie M; Alexander, Matthew S

    2018-01-01

    Muscular dystrophy is defined as the progressive wasting of skeletal muscles that is caused by inherited or spontaneous genetic mutations. Next-generation sequencing has greatly improved the accuracy and speed of diagnosis for different types of muscular dystrophy. Advancements in depth of coverage, convenience, and overall reduced cost have led to the identification of genetic modifiers that are responsible for phenotypic variability in affected patients. These genetic modifiers have been postulated to explain key differences in disease phenotypes, including age of loss of ambulation, steroid responsiveness, and the presence or absence of cardiac defects in patients with the same form of muscular dystrophy. This review highlights recent findings on genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies based on animal and clinical studies. These genetic modifiers hold great promise to be developed into novel therapeutic targets for the treatment of muscular dystrophies. Muscle Nerve 57: 6-15, 2018. © 2017 Wiley Periodicals, Inc.

  5. Macrosísmica de Cuba: su aplicación en los estimados de peligrosidad sísmica

    Directory of Open Access Journals (Sweden)

    Tomás J. Chuy-Rodríguez

    2003-03-01

    Full Text Available Se presenta una síntesis del catálogo de sismos perceptibles (1528 - 1990, obtenido y preparado al mismo tiempo como base de datos para PC. Este catálogo está suficientemente documentado y permite extender los períodos de tiempo que se analizan, limitados normalmente por la información instrumental. Complementan este resultado ejemplos del Atlas de Isosistas de Terremotos perceptibles con epicentro en el archipiélago cubano y cuya utilización para caracterizar la atenuación sísmica en el territorio nacional, en cuanto a intensidad, es prácticamente imprescindible para la realización de estimados de Peligrosidad Sísmica. En este sentido, la interpretación espacio - temporal de los sismos perceptibles considerados, contribuye a precisar las evaluaciones sismotectónicas de las Zonas Sismogénicas relacionadas con ellos. Se muestra también, el análisis teórico - experimental de la Ley de Atenuación de la Intensidad Sísmica de las Antillas Mayores y sus principales parámetros asociados a las Zonas Sismogénicas del país, los cuales se modelaron a partir de los mapas de isosistas de terremotos perceptibles y fuertes. Todos estos resultados se utilizan en los estimados de Peligrosidad Sísmica determinísticos y probabilísticos, realizados para diferentes regiones del país y que en este trabajo se unifican. Esto permitió que, a partir del Mapa de Peligrosidad Sísmica obtenido, en términos de la aceleración horizontal máxima, pudiera proponerse el Mapa de Zonación Sísmica con fines de Ingeniería utilizado actualmente en la Nueva Norma Sísmica Cubana. Como contribución a la caracterización de nuestra sismicidad, se presenta el análisis espacio - temporal - energético de las réplicas perceptibles de los terremotos más documentados ocurridos en Cuba, y se relacionan con las Zonas Sismogénicas que les dieron origen. En consecuencia, se obtuvo una relación empírica entre la Longitud de Ruptura y el valor de Magnitud

  6. BEEF CATTLE MUSCULARITY CANDIDATE GENES

    Directory of Open Access Journals (Sweden)

    Irida Novianti

    2010-04-01

    Full Text Available Muscularity is a potential indicator for the selection of more productive cattle. Mapping quantitative trait loci (QTL for traits related to muscularity is useful to identify the genomic regions where the genes affecting muscularity reside. QTL analysis from a Limousin-Jersey double backcross herd was conducted using QTL Express software with cohort and breed as the fixed effects. Nine QTL suggested to have an association with muscularity were identified on cattle chromosomes BTA 1, 2, 3, 4, 5, 8, 12, 14 and 17. The myostatin gene is located at the centromeric end of chromosome 2 and not surprisingly, the Limousin myostatin F94L variant accounted for the QTL on BTA2. However, when the myostatin F94L genotype was included as an additional fixed effect, the QTL on BTA17 was also no longer significant. This result suggests that there may be gene(s that have epistatic effects with myostatin located on cattle chromosome 17. Based on the position of the QTL in base pairs, all the genes that reside in the region were determined using the Ensembl data base (www.ensembl.org. There were two potential candidate genes residing within these QTL regions were selected. They were Smad nuclear interacting protein 1 (SNIP1 and similar to follistatin-like 5 (FSTL5. (JIIPB 2010 Vol 20 No 1: 1-10

  7. Lesiones musculares en el deporte. Muscular injuries in sport.

    Directory of Open Access Journals (Sweden)

    Jiménez Díaz, José Fernando

    2006-04-01

    Full Text Available ResumenDurante la práctica de la actividad física hay una gran incidencia de lesiones musculares, si bien se han llevado a cabo pocos estudios clínicos sobre el tratamiento y la resolución de las mismas. Desde el punto de vista etiopatogénico, hay que señalar que la incidencia de lesión es mayor en aquellos músculos poliarticulares en condiciones de acumulación de fatiga y con condiciones ambientales desfavorables. La clasificación de las lesiones musculares permite distinguir entre aquellas que no afectan a la fascia produciéndose un sangrado dentro del mismo (intramuscular o bien si la fascia también se rompe, el sangrado se sitúa entre los diferentes músculos (intermuscular. El tratamiento de estas lesiones se realizará combinando reposo, compresión, aplicación de frío y elevación del área lesionada así como el desarrollo de un adecuado programa de readaptación funcional que permita al jugador incorporarse lo antes posible a la dinámica del equipo. En la actualidad se está llevando a cabo opciones terapéuticas con factores de crecimiento, terapia génica y células madre, si bien todavía no están lo suficientemente desarrolladas.AbstractDuring the practice of the physical activity there is a great effect of muscular injuries, though few clinical studies have been carried out on the treatment and the resolution of the same ones. Inside the reasons it is necessary to indicate that the effect of injury is major in those muscles you will polyarticulate in situation of fatigue and with environmental unfavorable conditions.The classification of the muscular injuries allows to distinguish between those that do not affect the fascia producing the bled intramuscular or if the fascia also breaks, the bled one places between the different muscles (intermuscular.The treatment will be realized combining rest, compression, application of cold and elevation of these injuries as well as the development of a program of functional

  8. Modificación en la valoración de la resistencia y reactancia del tejido adiposo corporal con las nuevas vías de administración de anticoncepción hormonal

    OpenAIRE

    Encinas Pardilla, María Begoña

    2013-01-01

    En este trabajo se han estudiado las posibles modificaciones que los contraceptivos hormonales vía oral, transdérmica y transvaginal podrían tener en la composición del tejido adiposo corporal durante un año, así como sus efectos en el perfil lipídico y el índice de masa corporal. Conociendo los efectos que las formulaciones hormonales orales tienen en el perfil lipídico, cabría esperar que también pudiera alterarse en alguna medida el porcentaje de grasa corporal o al menos su densidad, de...

  9. Resistance training in patients with limb-girdle and becker muscular dystrophies

    DEFF Research Database (Denmark)

    Sveen, Marie-Louise; Andersen, Søren P; Ingelsrud, Lina H

    2013-01-01

    In this study we investigated the effect of strength training in patients with limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD).......In this study we investigated the effect of strength training in patients with limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD)....

  10. Fatores maternos associados ao peso fetal estimado pela ultra-sonografia Maternal factors associated with fetal weight estimated by ultrasonography

    Directory of Open Access Journals (Sweden)

    Adriana Suely de Oliveira Melo

    2008-09-01

    Full Text Available OBJETIVO: avaliar o efeito de variáveis maternas, socioeconômicas e obstétricas, assim como a presença de incisuras na 20ª e na 24ª semana, sobre o peso fetal estimado no final da gravidez (36ª semana em gestantes atendidas pelo Programa Saúde da Família em uma cidade do interior do Nordeste do Brasil. MÉTODOS: estudo longitudinal incluindo 137 gestantes. As gestantes foram acompanhadas a cada quatro semanas para aferição das condições clínicas, socioeconômicas e obstétricas, incluindo o peso materno. As artérias uterinas foram avaliadas pelo Doppler na 20ª e 24ª semana, o peso fetal e o índice de líquido amniótico (ILA foram determinados na 36ª semana. O estado nutricional materno inicial foi determinado pelo índice de massa corpórea (IMC, classificando-se as gestantes como com baixo peso, eutróficas, com sobrepeso e obesas. O ganho ponderal durante a gestação foi avaliado de acordo com o estado nutricional inicial, sendo ao final do segundo e terceiro trimestre classificado em ganho ponderal insuficiente, adequado e excessivo. Foi realizada análise de variância para avaliar a associação do peso fetal estimado na 36ª semana com as variáveis preditoras, ajustada por regressão linear múltipla. RESULTADOS: observou-se associação entre peso fetal estimado na 36ª semana e idade da mãe (p=0,02, trabalho materno (p=0,02, estado nutricional inicial (p=0,04, ganho ponderal no segundo trimestre (p=0,01, presença de incisuras nas artérias uterinas (p=0,02 e ILA (p=0,007. Os principais fatores associados ao peso fetal estimado na 36ª semana, após a análise de regressão múltipla, foram: IMC no início da gravidez, ganho ponderal no segundo trimestre, ILA e tabagismo. CONCLUSÕES: o peso fetal no presente estudo associou-se positivamente ao estado nutricional materno inicial, ao ganho ponderal no segundo trimestre, ao volume do líquido amniótico e negativamente ao hábito de tabagismo.PURPOSE: to evaluate the

  11. Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in the Netherlands : a cohort study

    NARCIS (Netherlands)

    Hoogerwaard, EM; Bakker, E; Ippel, PF; Oosterwijk, JC; Majoor-Krakauer, DF; Leschot, NJ; Van Essen, AJ; Brunner, HG; van der Wouw, PA; Wilde, AAM; de Visser, Marianne

    1999-01-01

    Background Carriers of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) may show muscle weakness or dilated cardiomyopathy. Studies focusing on skeletal-muscle involvement were done before DNA analysis was possible. We undertook a cross-sectional study in a population of

  12. Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in The Netherlands: a cohort study

    NARCIS (Netherlands)

    Hoogerwaard, E. M.; Bakker, E.; Ippel, P. F.; Oosterwijk, J. C.; Majoor-Krakauer, D. F.; Leschot, N. J.; van Essen, A. J.; Brunner, H. G.; van der Wouw, P. A.; Wilde, A. A.; de Visser, M.

    1999-01-01

    BACKGROUND: Carriers of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) may show muscle weakness or dilated cardiomyopathy. Studies focusing on skeletal-muscle involvement were done before DNA analysis was possible. We undertook a cross-sectional study in a population of

  13. Pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy.

    Science.gov (United States)

    Lager, Christina; Kroksmark, Anna-Karin

    2015-09-01

    The purpose of this study was to explore the prevalence, nature and scope of pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy and whether the pain differs between diagnostic groups or between adolescents with different ambulation status. Furthermore to study the consequences of pain and to identify pain-exacerbating and pain-relieving factors. In a national survey, fifty-five adolescents with spinal muscular atrophy and dystrophinopathy completed a questionnaire assessing pain frequency, duration, location using a body map, intensity and discomfort using visual analogue scales, pain interference using a modified version of Brief Pain Inventory and factors exacerbating and relieving pain. Sixty-nine per cent of the adolescents reported pain during the past three months and 50% reported chronic pain. The pain prevalence did not differ significantly between diagnostic groups or between ambulators and non-ambulators. The average pain intensity was graded as mild and the worst pain as moderate. The pain typically occurred weekly, most frequently in the neck/back or legs. General activity and mood were the areas that were most affected by pain. Common pain-exacerbating factors were sitting, too much movement/activity and being lifted or transferred. Pain is a frequent problem in adolescents with spinal muscular atrophy and dystrophinopathy. The assessments used enable an understanding both of the nature and scope of pain and of the impact of pain in everyday life. The study highlights the importance of assessing pain in a systematic manner and offering an individual approach to interventions designed to reduce pain in this population. Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  14. Clinico-epidemiologic characteristics of spinal muscular atrophy ...

    African Journals Online (AJOL)

    Rabah M. Shawky

    Deletion;. Chromosome 5;. Mutations. Abstract Spinal muscular atrophy (SMA) is characterized by progressive hypotonia and muscular weakness because of progressive degeneration of alpha motor neuron from anterior horn cells in the spinal cord. It is inherited by an autosomal recessive pattern. The precise frequency of ...

  15. Preimplantation genetic diagnosis of spinal muscular atrophy

    NARCIS (Netherlands)

    Dreesen, JCFM; Bras, M; de Die-Smulders, C; Dumoulin, JCM; Cobben, JM; Evers, JLH; Smeets, HJM; Geraedts, JPM

    After Duchenne muscular dystrophy, spinal muscular atrophy (SMA) is the most common severe neuromuscular disease in childhood. Since 1995, homozygous deletions in exon 7 of the survival motor neuron (SMN) gene have been described in >90-95% of SMA patients. However, the presence of a highly

  16. Respuesta hemodinámica con el entrenamiento en resistencia y fuerza muscular de miembros superiores en rehabilitación cardiaca Hemodynamic response to training in resistance and muscular strength of upper limbs in cardiac rehabilitation

    Directory of Open Access Journals (Sweden)

    Diana M Camargo

    2007-08-01

    Full Text Available Antecedentes: el entrenamiento de la fuerza muscular en pacientes con eventos cardiovasculares, estaba totalmente contraindicado por el riesgo de incrementar las cifras de tensión arterial sistólica y diastólica, así como someter al miocardio a una sobrecarga. Objetivo: valorar la respuesta hemodinámica (frecuencia cardiaca, tensión arterial durante el entrenamiento de resistencia a la fuerza muscular en los pacientes que asisten a un programa de rehabilitación cardiaca. Diseño: cuasi experimental prospectivo. Excenario: se realizó un entrenamiento de resistencia a la fuerza muscular en miembros superiores a pacientes durante la fase II de rehabilitación cardiaca. Sujetos: se incluyeron 175 pacientes, 135 hombres y 40 mujeres con un promedio de edad de 58,79 años, los cuales presentaron enfermedad coronaria con y sin procedimientos de revascularización, cirugía de cambios valvulares, síncope y corrección quirúrgica de anomalías congénitas. Procedimiento: previo entrenamiento aeróbico (en banda sinfín o bicicleta estática, se realizó la valoración de fuerza muscular mediante una máxima repetición y se inició el entrenamiento de fuerza a 30%-50% de ésta, en circuito de tres ejercicios diferentes en miembros superiores, 10 repeticiones en tres series y frecuencia de dos veces por semana. Se realizó, además, monitoría de la frecuencia cardiaca y electrocardiograma por telemetría (V5, toma de presión arterial antes, durante y después de la sesión, y percepción subjetiva del esfuerzo por escala de Borg, y se observaron signos y síntomas de intolerancia a la actividad o descompensación. Para el análisis estadístico, se utilizaron medidas de tendencia central con los cambios cardiovasculares, y para comparar el cambio en la fuerza muscular, se utilizó una prueba de t pareada con un nivel de significancia de 0,05. Resultados: la fuerza muscular medida con la máxima repetición, evidenció un aumento significativo

  17. Muscular atrophy in diabetic neuropathy

    DEFF Research Database (Denmark)

    Andersen, H; Gadeberg, P C; Brock, B

    1997-01-01

    Diabetic patients with polyneuropathy develop motor dysfunction. To establish whether motor dysfunction is associated with muscular atrophy the ankle dorsal and plantar flexors of the non-dominant leg were evaluated with magnetic resonance imaging in 8 patients with symptomatic neuropathy, in 8 non...... confirmed that the atrophy predominated distally. We conclude that muscular atrophy underlies motor weakness at the ankle in diabetic patients with polyneuropathy and that the atrophy is most pronounced in distal muscles of the lower leg indicating that a length dependent neuropathic process explains...

  18. Monitorización ambulatoria de la presión arterial en niños con obesidad

    OpenAIRE

    García-Vao Bel, Carlos María

    2015-01-01

    Tesis Doctoral leída en la Universidad Rey Juan Carlos de Madrid en 2015. Directores de la Tesis: Ángel Gil de Miguel y María José Rivero Martín Se define la obesidad como un exceso de masa grasa con relación a la masa corporal total, esta acumulación de grasa es excesiva y generalizada tanto en el tejido subcutáneo como en el resto de los tejidos y se asocia a un desequilibrio en las proporciones de los componentes magro o muscular, óseo, visceral y adiposo. Por tanto no se pu...

  19. Facioscapulohumeral muscular dystrophy

    Science.gov (United States)

    ... There is no known cure for facioscapulohumeral muscular dystrophy. Treatments are given to control symptoms and improve quality of life. Activity is encouraged. Inactivity such as bedrest can make the muscle disease worse. Physical therapy may help maintain muscle ...

  20. The Effect of Aging on Muscular Dynamics Underlying Movement Patterns Changes.

    Science.gov (United States)

    Vernooij, Carlijn A; Rao, Guillaume; Berton, Eric; Retornaz, Frédérique; Temprado, Jean-Jacques

    2016-01-01

    Introduction: Aging leads to alterations not only within the complex subsystems of the neuro-musculo-skeletal system, but also in the coupling between them. Here, we studied how aging affects functional reorganizations that occur both within and between the behavioral and muscular levels, which must be coordinated to produce goal-directed movements. Using unimanual reciprocal Fitts' task, we examined the behavioral and muscular dynamics of older adults (74.4 ± 3.7 years) and compared them to those found for younger adults (23.2 ± 2.0 years). Methods: To achieve this objective, we manipulated the target size to trigger a phase transition in the behavioral regime and searched for concomitant signatures of a phase transition in the muscular coordination. Here, muscular coordination was derived by using the method of muscular synergy extraction. With this technique, we obtained functional muscular patterns through non-negative matrix factorization of the muscular signals followed by clustering the resulting synergies. Results: Older adults showed a phase transition in behavioral regime, although, in contrast to young participants, their kinematic profiles did not show a discontinuity. In parallel, muscular coordination displayed two typical signatures of a phase transition, that is, increased variability of coordination patterns and a reorganization of muscular synergies. Both signatures confirmed the existence of muscular reorganization in older adults, which is coupled with change in dynamical regime at behavioral level. However, relative to young adults, transition occurred at lower index of difficulty (ID) in older participants and the reorganization of muscular patterns lasted longer (over multiple IDs). Discussion: This implies that consistent changes occur in coordination processes across behavior and muscle. Furthermore, the repertoire of muscular patterns was reduced and somewhat modified for older adults, relative to young participants. This suggests that

  1. Estruturas elásticas e fadiga muscular

    OpenAIRE

    Kronbauer, Gláucia Andreza; Castro, Flávio Antônio de Souza

    2013-01-01

    A fadiga muscular pode ser definida pela incapacidade de manter certa tarefa ao longo do tempo; os mecanismos neuromusculares e metabólicos envolvidos na contração muscular estão diretamente associados a esse fenômeno. Este estudo bibliográfico busca descrever as alterações nos elementos contráteis e elásticos envolvidos na contração muscular e sua relação com o desempenho na locomoção. As estruturas contráteis são aquelas que desenvolvem força ativa com gasto de energia metabólica - mecanism...

  2. Experimental investigation of muscular neurotization in the rat.

    Science.gov (United States)

    Must, R

    1987-01-01

    Reinnervation of a free muscle graft by nerves from an adjacent intact muscle is called muscular neurotization. This paper investigates the mechanisms and stimuli responsible for muscular neurotization in the rat. Sternohyoid or sternomastoid muscles were transplanted as free muscle grafts to the ventral surface of an intact sternohyoid muscle (feeder muscle). After several weeks the graft and underlying feeder muscle were removed together, frozen, serially sectioned, stained, and carefully examined for the presence or absence of nerves. It was concluded from a series of experiments that in this model muscular neurotization is a form of nerve regeneration. In order for muscular neurotization to occur, it is necessary to have (1) injury to the nerves of the intact feeder muscle and (2) a pathway upon which the regenerating nerves may grow into the graft.

  3. Metabolismo muscular en el ejercicio

    OpenAIRE

    Martín Martín, Laura

    2017-01-01

    Fundamentos: Cada vez son más las personas que realizan algún tipo de actividad física, pero pocas son las que poseen un verdadero conocimiento de los procesos que se desencadenan a nivel muscular y la influencia de la alimentación en la misma. El objetivo de este trabajo es ofrecer información de manera general sobre el metabolismo muscular. Métodos: Revisión bibliográfica de artículos y documentos consultando bases de datos y libros. La mayor parte del análisis ha sido ext...

  4. Influencia del polimorfismo del receptor de andrógenos en la masa ósea, masa muscular y condición física en niños y niñas

    OpenAIRE

    Rodríguez García, Lorena

    2015-01-01

    Programa de doctorado: Actividad Física, Salud y Rendimiento Deportivo Premio Extraordinario de Doctorado. Rama Ciencias Sociales y Jurídicas [ES]Para determinar el efecto de ciertos polimorfismos del gen del receptor de andrógenos sobre la masa muscular y la masa ósea, se estudió una muestra poblacional de 152 niños y 116 niñas sanos y físicamente activos (estudios I y II). La composición corporal se determinó mediante absorciometría fotónica dual de rayos X (DXA) y antropometría. La c...

  5. Nuevo dispositivo para el aislamiento de la fracción estromal vascular a partir de lipoaspirados humanos: método de obtención y análisis de calidad celular

    OpenAIRE

    S. Dos-Anjos; J. Mercader; A.J. Katz.; R. Llull

    2013-01-01

    El tejido adiposo obtenido mediante liposucción es una fuente idónea para aislar células con potencial terapéutico, las denominadas células de la fracción estromal vascular (FEV), que incluyen células madre mesenquimales. Estas células se han convertido en una de las principales herramientas de terapia celular autóloga para diversas aplicaciones médicas, y en los últimos años se han ido desarrollando diversas tecnologías para su aislamiento y uso clínico. En este trabajo presentamos un nuevo ...

  6. Investigación comparativa entre un sistema de bombeo fotovoltaico y un sistema de bombeo eléctrico para implementarse en Lago Agrio

    OpenAIRE

    Tubón Guevara, Juan Paúl

    2015-01-01

    La situación que enfrenta la ciudad de Lago Agrio por problemas de agua potable, ha obligado a sus habitantes a utilizar bombas eléctricas para abastecerse del líquido vital. La mayoría de domicilios cuenta con tanques o cisternas de almacenamiento y bombas eléctricas para distribución de agua. Otros domicilios cuentan con pozos de agua y mediante bombas eléctricas se proveen del líquido vital. Se ha estimado mediante información local, que en Lago Agrio existen 4733 bombas domésticas tradici...

  7. Validação da curva normal de peso fetal estimado pela ultra-sonografia para o diagnóstico do peso neonatal

    OpenAIRE

    Cecatti José Guilherme; Machado Maria Regina Marrocos; Krupa Fabiana da Graça; Figueiredo Priscila Garcia; Pires Helaine Maria Besteti

    2003-01-01

    OBJETIVO: avaliar a concordância entre o peso fetal estimado (PFE) por ultra-sonografia e o neonatal, o desempenho da curva normal de PFE por idade gestacional no diagnóstico de desvios do peso fetal/neonatal e fatores associados. MÉTODOS: participaram do estudo 186 grávidas atendidas de novembro de 1998 a janeiro de 2000, com avaliação ultra-sonográfica até 3 dias antes do parto, determinação do PFE e do índice de líquido amniótico e parto na instituição. O PFE foi calculado e classificado d...

  8. Roentgenological findings in muscular alterations of extremities

    International Nuclear Information System (INIS)

    Palvoelgyi, R.

    1978-01-01

    A survey of roentgenological findings in muscular alterations of extremities based on the author's experiences and on the literature is presented. Following a description of the normal roentgen anatomy, the alterations in different diseases of interstitial lipomatosis are demonstrated. By roentgenological examinations differt muscular lesions of the extremities can be differentiated and the clinical follow-up verified. (orig.) [de

  9. Becker muscular dystrophy-like myopathy regarded as so-called "fatty muscular dystrophy" in a pig: a case report and its diagnostic method.

    Science.gov (United States)

    Horiuchi, Noriyuki; Aihara, Naoyuki; Mizutani, Hiroshi; Kousaka, Shinichi; Nagafuchi, Tsuneyuki; Ochiai, Mariko; Ochiai, Kazuhiko; Kobayashi, Yoshiyasu; Furuoka, Hidefumi; Asai, Tetsuo; Oishi, Koji

    2014-03-01

    We describe a case of human Becker muscular dystrophy (BMD)-like myopathy that was characterized by the declined stainability of dystrophin at sarcolemma in a pig and the immunostaining for dystrophin on the formalin-fixed, paraffin-embedded (FFPE) tissue. The present case was found in a meat inspection center. The pig looked appeared healthy at the ante-mortem inspection. Muscular abnormalities were detected after carcass dressing as pale, discolored skeletal muscles with prominent fat infiltrations and considered so-called "fatty muscular dystrophy". Microscopic examination revealed following characteristics: diffused fat infiltration into the skeletal muscle and degeneration and regeneration of the remaining skeletal muscle fibers. Any lesions that were suspected of neurogenic atrophy, traumatic muscular degeneration, glycogen storage disease or other porcine muscular disorders were not observed. The immunostaining for dystrophin was conducted and confirmed to be applicable on FFPE porcine muscular tissues and revealed diminished stainability of dystrophin at the sarcolemma in the present case. Based on the histological observations and immunostaining results, the present case was diagnosed with BMD-like myopathy associated with dystrophin abnormality in a pig. Although the genetic properties were not clear, the present BMD-like myopathy implied the occurrence of dystrophinopathy in pigs. To the best of our knowledge, this is the first report of a natural case of myopathy associated with dystrophin abnormalities in a pig.

  10. Influencia de habitos alimentarios sobre las concentraciones de pesticidas organoclorados en tejido adiposo Influence of alimentary habits on organochlorine concentrations in adipose tissue

    Directory of Open Access Journals (Sweden)

    Ijoni Costabeber

    2002-01-01

    Full Text Available Fueron evaluados los niveles de residuos de pesticidas organoclorados (p,p'-DDE, HCB, gamma-HCH, aldrín presentes en 123 muestras de tejido adiposo mamario obtenidas de mujeres residentes en Córdoba (España. Además, se investigó la influencia de hábitos alimentarios y edad de las donantes sobre los niveles de organoclorados encontrados en el tejido. El p,p'-DDE fue el compuesto encontrado en concentraciones más altas (1,848myg/g, seguido de HCB (0,235mg/g, aldrín (0,005myg/g y gamma-HCH (0,003myg/g. Las donantes mayores presentaron niveles de p,p'-DDE, aldrín y HCB superiores que las más jóvenes. Sin embargo, la edad no influyó en los niveles de gamma-HCH. La mayoría de las donantes (40,65% indicaron las frutas como el alimento más consumido, seguido de la alimentación variada (34,15%, los legumbres (6,50%, los guisos y potajes (6,50%, las verduras (4,88%, la leche (3,25%, el pescado (2,44% y la carne (1,63%. Una correlación no significativa fue observada entre los niveles de organoclorados en el tejido adiposo y los hábitos alimentarios de las donantes.The levels of organochlorine pesticide residues (p,p'-DDE, HCB, gamma-HCH, aldrin present in adipose tissue samples from mammary glands of 123 women living in Cordoba (Spain were evaluated. The influence of alimentary habits and age of donors on organochlorine tissue levels was also investigated. The compound found at the highest concentration was p,p'-DDE (1.848mug/g, followed by HCB (0.235mug/g, aldrin (0.005mug/g and gamma-HCH (0.003mg/g. The older donors presented tissue levels of p,p'-DDE, aldrin and HCB higher than those found in younger donors. Conversely, age had no influence on tissue levels of gamma-HCH. The majority of donors (40.65% indicated fruits as the most consumed food, followed by mixed food (34.15%, non-green vegetables (6.50%, stewed meat and vegetables (6.50%, green vegetables (4.88%, milk (3.25%, fish (2.44% and meat (1.63%. No significant correlation was

  11. [Supplementary device for a dynamometer to evaluate and register muscular endurance indices].

    Science.gov (United States)

    Timoshenko, D A; Bokser, O Ia

    1986-01-01

    In practice of psychophysiologic research muscular endurance index is used for estimation of CNS function. Muscular endurance index is defined as relative time needed for maintaining the preset muscular effort. The described device widens the possibilities of a digital dynamometer for automatic estimation and recording of muscular endurance index in real time.

  12. Muscular pathology: echographic and NMR imaging aspects

    International Nuclear Information System (INIS)

    Pascal-Suisse, P.; Beaurain, P.; Mougniot, C.

    1995-01-01

    A comparison of echographic techniques and NMR imaging has been done for the diagnosis of muscular trauma and tumor pathologies. In traumatic pathology, the echographic analysis allows to determine the complete assessment of recent muscular injuries. NMR imaging can be used in granuloma or fibrous callosity appreciation and for the analysis of deep injury (muscles and muscles-tendon junctions) and of muscular aponeurosis. Echography must be used together with color coding Doppler technique in the diagnosis of tumor pathology and for the study of slow fluxes. The recently available energy Doppler technique seems to be powerful in the study of vascularization of small expansive formations, but their extension to adjacent bone or tissue can only be appreciated using NMR imaging. (J.S.)

  13. Is drive for muscularity related to body checking behaviors in men athletes?

    Directory of Open Access Journals (Sweden)

    Leonardo de Sousa Fortes

    Full Text Available Abstract The aim of this study was to analyze the relationship between drive for muscularity and body checking behaviors in men athletes. Two hundred and twelve Brazilian athletes over 15 years of age participated. We used the Drive for Muscularity Scale (DMS to evaluate the drive for muscularity. The Male Body Checking Questionnaire was used to assess body checking behaviors. The findings demonstrated a relationship between the "body image-oriented muscularity" subscale of the DMS and body checking behaviors (p = 0.001. The results indicated differences in body checking among athletes with high and low levels of drive for muscularity. We concluded that drive for muscularity was related to body checking behaviors in men athletes.

  14. Stem cell transplantation for treating Duchenne muscular dystrophy

    Science.gov (United States)

    Yang, Xiaofeng

    2012-01-01

    OBJECTIVE: To identify global research trends in stem cell transplantation for treating Duchenne muscular dystrophy using a bibliometric analysis of Web of Science. DATA RETRIEVAL: We performed a bibliometric analysis of studies on stem cell transplantation for treating Duchenne muscular dystrophy from 2002 to 2011 retrieved from Web of Science. SELECTION CRITERIA: Inclusion criteria: (a) peer-reviewed published articles on stem cell transplantation for treating Duchenne muscular dystrophy indexed in Web of Science; (b) original research articles, reviews, meeting abstracts, proceedings papers, book chapters, editorial material, and news items; and (c) publication between 2002 and 2011. Exclusion criteria: (a) articles that required manual searching or telephone access; (b) documents that were not published in the public domain; and (c) corrected papers. MAIN OUTCOME MEASURES: (1) Annual publication output; (2) distribution according to subject areas; (3) distribution according to journals; (4) distribution according to country; (5) distribution according to institution; (6) distribution according to institution in China; (7) distribution according to institution that cooperated with Chinese institutions; (8) top-cited articles from 2002 to 2006; (9) top-cited articles from 2007 to 2011. RESULTS: A total of 318 publications on stem cell transplantation for treating Duchenne muscular dystrophy were retrieved from Web of Science from 2002 to 2011, of which almost half derived from American authors and institutes. The number of publications has gradually increased over the past 10 years. Most papers appeared in journals with a focus on gene and molecular research, such as Molecular Therapy, Neuromuscular Disorders, and PLoS One. The 10 most-cited papers from 2002 to 2006 were mostly about different kinds of stem cell transplantation for muscle regeneration, while the 10 most-cited papers from 2007 to 2011 were mostly about new techniques of stem cell transplantation

  15. Cognitive and Neurobehavioral Profile in Boys With Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Banihani, Rudaina; Smile, Sharon; Yoon, Grace; Dupuis, Annie; Mosleh, Maureen; Snider, Andrea; McAdam, Laura

    2015-10-01

    Duchenne muscular dystrophy is a progressive neuromuscular condition that has a high rate of cognitive and learning disabilities as well as neurobehavioral disorders, some of which have been associated with disruption of dystrophin isoforms. Retrospective cohort of 59 boys investigated the cognitive and neurobehavioral profile of boys with Duchenne muscular dystrophy. Full-scale IQ of Duchenne muscular dystrophy. © The Author(s) 2015.

  16. Determinación del esfuerzo de muestreo requerido para el estimado de procucción de huevos de anchoveta Engraulis ringens, frente al Perú

    OpenAIRE

    Santander, Haydeé; Smith, Paul E.; Alheit, Juergen

    1982-01-01

    Para obtener una primera aproximación del número de muestras requerido para un determinado nivel de precisión del estimado de producción de huevos de anchoveta por unidad de área superficial del mar, se han utilizado datos existentes de muestras de huevos provenientes de exploraciones efectuadas frente al Perú en los últimos 20 años. La anchoveta parece desovar en cardúmenes, produciendo una gran can­ tidad de huevos, de los que las muestras toman una pequeña proporción. Aproximadamente e...

  17. Muscle Dysmorphia and the Perception of Men's Peer Muscularity Preferences.

    Science.gov (United States)

    Lin, Linda; DeCusati, Frank

    2016-11-01

    Research suggests that peer muscularity norms preferences are related to men's body image, but little information is known about how perceptions of specific peer group norms preferences are related to men's body image disturbances and specific health behaviors. This study investigated how men perceived the muscularity preferences of male, female, close, and distant peers and whether the perceptions of specific peer preferences were related to muscle dysmorphia and steroid use. Data on muscle dysmorphia and the perceptions of peer muscularity norms were collected from 117 male college students. Results indicated that men perceived distant and male peers as having the most exaggerated preferences for muscularity and that those perceptions were not an accurate reflection of their distant male peers' reported preferences. Results also indicated that perceptions of close female peer muscularity preferences were predictive of symptoms of muscle dysmorphia, but this relationship did not exist for other peer groups, suggesting that the perceptions of close female peer preferences may play a role in the development of muscle dysmorphia. No relationship was found between perceptions of peer muscularity preferences and steroid use. © The Author(s) 2015.

  18. Preimplantation genetic diagnosis associated to Duchenne muscular dystrophy.

    Science.gov (United States)

    Bianco, Bianca; Christofolini, Denise Maria; Conceição, Gabriel Seixas; Barbosa, Caio Parente

    2017-01-01

    Duchenne muscular dystrophy is the most common muscle disease found in male children. Currently, there is no effective therapy available for Duchenne muscular dystrophy patients. Therefore, it is essential to make a prenatal diagnosis and provide genetic counseling to reduce the birth of such boys. We report a case of preimplantation genetic diagnosis associated with Duchenne muscular dystrophy. The couple E.P.R., 38-year-old, symptomatic patient heterozygous for a 2 to 47 exon deletion mutation in DMD gene and G.T.S., 39-year-old, sought genetic counseling about preimplantation genetic diagnosis process. They have had a 6-year-old son who died due to Duchenne muscular dystrophy complications. The couple underwent four cycles of intracytoplasmic sperm injection (ICSI) and eight embryos biopsies were analyzed by polymerase chain reaction (PCR) for specific mutation analysis, followed by microarray-based comparative genomic hybridisation (array CGH) for aneuploidy analysis. Preimplantation genetic diagnosis revealed that two embryos had inherited the maternal DMD gene mutation, one embryo had a chromosomal alteration and five embryos were normal. One blastocyst was transferred and resulted in successful pregnancy. The other embryos remain vitrified. We concluded that embryo analysis using associated techniques of PCR and array CGH seems to be safe for embryo selection in cases of X-linked disorders, such as Duchenne muscular dystrophy.

  19. Muscular hamartoma of the breast: a case report

    International Nuclear Information System (INIS)

    Kang, Byeong Seong; Park, Jeong Mi

    2002-01-01

    Muscular hamartoma is a variant of breast hamartoma shown at microscopic examination to be composed of abundant smooth muscle cells. We report a case of muscular hamartoma occurring in a 35-year-old woman with an incidentally discovered breast mass, and confirmed by excisional biopsy. We also describe the radiologic and pathologic findings

  20. Avaliação dos efeitos da orientação da bobina de estimulação magnética transcraniana nos potenciais evocados motores do músculo abdutor curto do polegar por eletromiografia de alta densidade

    OpenAIRE

    Victor Hugo de Oliveira e Souza

    2014-01-01

    A estimulação magnética transcraniana (EMT) aplicada com diferentes orientações da bobina causa variações na amplitude e na latência dos potenciais evocados motores (PEM) dos músculos da mão. No entanto, as propriedades dos PEM são afetadas também pelos sistemas de detecção, e.g. localização e tamanho dos eletrodos de aquisição, e pela anatomia do músculo analisado, e.g. arquitetura dos tecidos muscular e adiposo. Neste estudo, verificamos como variam a distribuição espacial, a amplitude pico...

  1. Why is muscularity sexy? Tests of the fitness indicator hypothesis.

    Science.gov (United States)

    Frederick, David A; Haselton, Martie G

    2007-08-01

    Evolutionary scientists propose that exaggerated secondary sexual characteristics are cues of genes that increase offspring viability or reproductive success. In six studies the hypothesis that muscularity is one such cue is tested. As predicted, women rate muscular men as sexier, more physically dominant and volatile, and less committed to their mates than nonmuscular men. Consistent with the inverted-U hypothesis of masculine traits, men with moderate muscularity are rated most attractive. Consistent with past research on fitness cues, across two measures, women indicate that their most recent short-term sex partners were more muscular than their other sex partners (ds = .36, .47). Across three studies, when controlling for other characteristics (e.g., body fat), muscular men rate their bodies as sexier to women (partial rs = .49-.62) and report more lifetime sex partners (partial rs = .20-.27), short-term partners (partial rs = .25-.28), and more affairs with mated women (partial r = .28).

  2. Guidelines for the Perianesthesia Care of the Duchenne Muscular Dystrophy/Becker Muscular Dystrophy Patient.

    Science.gov (United States)

    Alliod, Barbara A; Ash, Rebecca A

    2016-12-01

    More patients suffering with Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are presenting to perianesthesia settings for emergent and nonemergent treatment and care. A group of collaborative health care providers at Rush University Medical Center in Chicago developed a multidisciplinary DMD/BMD Task Force to study this disorder and create a set of guidelines to aid those engaging in the planning, execution of care, and recovery of this unique population in the perianesthesia setting. Attention to detail, well-executed preplanning, meticulous awareness of the patient, and prearranged implementation and intervention has proven to offset potential problems and complications and is the key to a successful perianesthesia period. Copyright © 2016 American Society of PeriAnesthesia Nurses. Published by Elsevier Inc. All rights reserved.

  3. A Study of CAP-1002 in Ambulatory and Non-Ambulatory Patients With Duchenne Muscular Dystrophy

    Science.gov (United States)

    2018-04-17

    Muscular Dystrophies; Muscular Dystrophy, Duchenne; Muscular Disorders, Atrophic; Muscular Diseases; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, X-Linked; Genetic Diseases, Inborn

  4. Computed tomography of skeletal muscles in childhood spinal progressive muscular atrophies

    International Nuclear Information System (INIS)

    Arai, Yumi; Osawa, Makiko; Sumida, Sawako; Shishikura, Keiko; Suzuki, Haruko; Fukuyama, Yukio; Kohno, Atsushi

    1992-01-01

    Computed tomographic (CT) scanning of skeletal muscles was performed in patients with type 1 and type 2 spinal progressive muscular atrophy (SPMA) and Kugelberg-Welander disease (K-W) to delineate the characteristic CT features of each category. Marked muscular atrophy was observed in type 1 SPMA, and both muscular atrophy and intramuscular low density areas in type 2 SPMA, changes being more pronounced in older patients. In contrast, in K-W, muscular atrophy was slight, and intramuscular low density areas constituted the most prominent findings. These observations indicate that SPMA and K-W are each characterized by distinct CT findings. (author)

  5. Duchenne muscular dystrophy carriers

    International Nuclear Information System (INIS)

    Matsumura, K.; Nakano, I.

    1989-01-01

    By means of magnetic resonance imaging (MRI), the proton spin-lattice relaxation times (T1 values) of the skeletal muscles were measured in Duchenne muscular dystrophy (DMD) carriers and normal controls. The bound water fraction (BWF) was calculated from the T1 values obtained, according to the fast proton diffusion model. In the DMD carriers, T1 values of the gluteus maximus and quadriceps femoris muscles were significantly higher, and BWFs of these muscles were significantly lower than in normal control. Degenerative muscular changes accompanied by interstitial edema were presumed responsible for this abnormality. No correlation was observed between the muscle T1 and serum creatine kinase values. The present study showed that MRI could be a useful method for studying the dynamic state of water in both normal and pathological skeletal muscles. Its possible utility for DMD carrier detection was discussed briefly. (orig.)

  6. Upper limb function in adults with Duchenne muscular dystrophy

    NARCIS (Netherlands)

    B. Bartels (Bart); R.F. Pangalila (Robert); M.P. Bergen (Michael); N.A.M. Cobben (Nicolle); H.J. Stam (Henk); M.E. Roebroeck (Marij)

    2011-01-01

    textabstractTo determine upper limb function and associated factors in adults with Duchenne muscular dystrophy. Design: Cross-sectional study. Subjects: A sample of 70 men with Duchenne muscular dystrophy (age range 20-43 years). Methods: General motor function and, in particular, upper limb distal

  7. Duchenne muscular dystrophy: the management of scoliosis

    Science.gov (United States)

    Gardner, Adrian C.; Roper, Helen P.; Chikermane, Ashish A.; Tatman, Andrew J.

    2016-01-01

    This study summaries the current management of scoliosis in patients with Duchenne Muscular Dystrophy. A literature review of Medline was performed and the collected articles critically appraised. This literature is discussed to give an overview of the current management of scoliosis within Duchenne Muscular Dystrophy. Importantly, improvements in respiratory care, the use of steroids and improving surgical techniques have allowed patients to maintain quality of life and improved life expectancy in this patient group. PMID:27757431

  8. Dysphagia in Duchenne Muscular Dystrophy Assessed by Validated Questionnaire

    Science.gov (United States)

    Archer, Sally K.; Garrod, Rachel; Hart, Nicholas; Miller, Simon

    2013-01-01

    Background: Duchenne muscular dystrophy (DMD) leads to progressive muscular weakness and death, most typically from respiratory complications. Dysphagia is common in DMD; however, the most appropriate swallowing assessments have not been universally agreed and the symptoms of dysphagia remain under-reported. Aims: To investigate symptoms of…

  9. Rare muscular variations identified in a single cadaveric upper limb: a four-headed biceps brachii and muscular elevator of the latissimus dorsi tendon.

    Science.gov (United States)

    Moore, Colin W; Rice, Charles L

    2018-03-01

    Supernumerary or accessory heads of the biceps brachii are persistent muscular structures which can vary in number and location in the arm. Variations in other arm muscles, such as the coracobrachialis, can accompany supernumerary biceps brachii musculature in the upper limb. In this case report, we describe two rare muscular variants in a single adult male: a four-headed biceps brachii and the muscular elevator of the latissimus dorsi tendon. Additionally, accessory muscles of the brachialis and flexor digiti minimi brevis were identified in the upper limb. To our knowledge, the muscular variants identified here are considered rare, and their co-occurrence in a single upper limb has not been described previously. Also, a four-headed biceps brachii consisting of both the infero-medial and infero-lateral humeral heads has not been described previously to our knowledge. We postulate that the simultaneous appearance of several muscular variations may indicate a signaling disruption in embryogenesis during muscle patterning of the ventral limb bud. Knowledge of variant musculature in the arm is important for surgeons and clinicians as these muscles and their aberrant innervation patterns can complicate surgical procedures and may compress arteries and nerves producing upper limb pain and paresthesia. The clinical, functional and embryological implications of the upper limb variants are discussed.

  10. Muscle-Eye-Brain Disease; a Rare Form of Syndromic Congenital Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Gosal Gurinder S

    2011-03-01

    Full Text Available Congenital muscular dystrophy (CMD is a heterogeneous group of disorders characterized by muscular hypotonia since birth and the histologic features of muscular dystrophy. Syndromic congenital muscular dystrophies are clinically similar autosomal recessive disorders characterized by congenital muscular dystrophy, lissencephaly, and eye anomalies. We present a case of a rare form of syndromic congenital muscular dystrophy in an eight year old girl, born of first- degree consanguinity. She had: global developmental delay; a seizure disorder; hypotonia; progressive muscle contractures including bilateral symmetrical flexion contractures of hips, knees, equinus contracture and thoracolumbar scoliosis; diminished deep tendon reflexes: bilateral premature cataract; pseudophakia; and nystagmus. The patient was also highly myopic. Based on clinical features, muscle biopsy and MRI of the brain, a diagnosis of muscle- eye- brain disease was made. Identification of these patients may help to prevent this crippling disorder in the future siblings of probands by utilizing genetic counselling and mutation analysis.

  11. Regeneración de las lesiones críticas del nervio periférico con factores de crecimiento: Estudio experimental

    OpenAIRE

    García Medrano, Belén

    2013-01-01

    Introducción:El objetivo del proyecto es estudiar la regeneración de lesiones no reparables de nervio periférico mediante un injerto muscular enriquecido con factores de crecimiento. Material y método: Estudio experimental controlado, nivel I de evidencia cientifica. Comparamos diez ratas con reparación de lesiones de 15 mm del nervio ciático mediante injerto muscular acelular, con un grupo de diez años en los que se añade la inyección de 2 cc de IGF-l (10 mg/ml de me

  12. Common recessive limb girdle muscular dystrophies differential diagnosis: why and how?

    Directory of Open Access Journals (Sweden)

    Ana Cotta

    2014-09-01

    Full Text Available Limb girdle muscular dystrophies are heterogeneous autosomal hereditary neuromuscular disorders. They produce dystrophic changes on muscle biopsy and they are associated with mutations in several genes involved in muscular structure and function. Detailed clinical, laboratorial, imaging, diagnostic flowchart, photographs, tables, and illustrated diagrams are presented for the differential diagnosis of common autosomal recessive limb girdle muscular dystrophy subtypes diagnosed nowadays at one reference center in Brazil. Preoperative image studies guide muscle biopsy site selection. Muscle involvement image pattern differs depending on the limb girdle muscular dystrophy subtype. Muscle involvement is conspicuous at the posterior thigh in calpainopathy and fukutin-related proteinopathy; anterior thigh in sarcoglycanopathy; whole thigh in dysferlinopathy, and telethoninopathy. The precise differential diagnosis of limb girdle muscular dystrophies is important for genetic counseling, prognostic orientation, cardiac and respiratory management. Besides that, it may probably, in the future, provide specific genetic therapies for each subtype.

  13. Genetics Home Reference: spinal muscular atrophy with progressive myoclonic epilepsy

    Science.gov (United States)

    ... myoclonic epilepsy Spinal muscular atrophy with progressive myoclonic epilepsy Printable PDF Open All Close All Enable Javascript ... boxes. Description Spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME) is a neurological condition that causes ...

  14. Dismorfia muscular: A busca pelo corpo hiper musculoso

    Directory of Open Access Journals (Sweden)

    Andréa Pires Azevedo

    2012-03-01

    Full Text Available A dismorfia muscular tem sido identificada tanto em homens quanto em mulheres, provocando alterações da perceção da autoimagem e prejuízos socioculturais, e na saúde e bem-estar dos indivíduos. Nesse sentido, o objetivo deste estudo é analisar os aspetos socioculturais, psicológicos e o uso de recursos ergogênicos relacionados à dismorfia muscular, bem como, identificar os riscos promovidos pelo transtorno. Participaram 20 indivíduos, inscritos na rede mundial de computadores. Utilizou-se um questionário para coleta de dados e a análise documental para interpretação das respostas. Os resultados demonstram que as preocupações com a imagem corporal geram insegurança social, baixa autoestima e sentimentos de inferioridade, que seriam resolvidos se a pessoa tivesse corpos belos e fortes. A dismorfia muscular pode aumentar o risco de uso dos esteroides anabolizantes e o uso indiscriminado de suplementos alimentares. Conclui-se que a dismorfia muscular causa sofrimentos e prejuízos psicológicos, socioculturais e, desse modo, compromete a saúde das pessoas.

  15. Spirometry improvement after muscular exercise in elite swimmers.

    Science.gov (United States)

    Rubini, Alessandro; Rizzato, Alex; Fava, Simone; Olivato, Nicola; Mangar, Devanand; Camporesi, Enrico M; Bosco, Gerardo

    2017-12-01

    An increased sympathetic activity during muscular effort is a well established physiological response, whose intensity is known to increase with the muscular load. Spirometry was described to improve as an effect of swimming training in healthy and asthmatic subjects, suggesting a decrease in airway resistance The aim was to investigate the possible effect of muscular exercise (swimming) on spirometry, in particular searching for possible differences because of different swimming times. The measurements were performed on 9 highly trained male competitive swimmers (age: 41±12.79 years, height: 1.69±0.06 meters, weight: 66.14±14.28 kg, BMI: 22.8±3.61 kg/m2) during an official competition. The data were collected at the border of the swimming-pool before (control, C) and few minutes after the swimming sessions (exercise, E), which consisted either of 800 meter (7 subjects) or 1500 meter (5 subjects) free style. A general trend indicating a postexercise increase in spirometry was observed. We found post-exercise significant increments in FEV1 and in MEF75 for both the 800 and 1500 meter swimming sessions, and in FEF25-75 and in MEF25 for the shorter distance. We conclude that, as it may be expected, muscular exercise induces an improvement of spirometry both because of a smooth muscle relaxation-induced modulation of airway diameter and resistance to airflow, and because of an enhanced expiratory muscle contraction strength. Both of these mechanisms are related to an increased sympathetic activity which is well known to accompany muscular exercise.

  16. Duchenne muscular dystrophy with associated growth hormone deficiency

    International Nuclear Information System (INIS)

    Ghafoor, T.; Mahmood, A.; Shams, S.

    2003-01-01

    A patient with duchenne muscular dystrophy (DMD) and growth hormone (GH) deficiency is described who had no clinical evidence of muscular weakness before initiation of GH replacement therapy. Treatment with human GH resulted in appearance of symptoms of easy fatigability and muscle weakness. Thorough investigations including serum creating phosphokinase (CK) levels in recommended in every patient with GH deficiency before starting GH replacement therapy. (author)

  17. CT finding and cerebrospinal fluid proteins in muscular dystrophy patients

    Energy Technology Data Exchange (ETDEWEB)

    Hirase, Tsutomu; Ide, Masami; Araki, Shukuro; Okamoto, Hiroshi (Kumamoto Univ. (Japan). School of Medicine); Kawasaki, Shoichiro; Imamura, Shigehiro

    1983-06-01

    We analyzed the microcomponents of protein fractions in the cerebrospinal fluid of patients with various types of muscular dystrophy. The degenerative pattern is characterized by an increase in the prealbumin and a decrease in the ..gamma..-globulin fraction is shown in the Duchenne and congenital muscular dystrophy. The increase in CSF IgG, ..gamma..-globulin fraction is shown in the myotonic dystrophy. In addition to the abnormality of IQ, EEG, and brain CT, abnormal CSF proteins obviously suggest the presence of CNS involvement in muscular dystrophy.

  18. CT finding and cerebrospinal fluid proteins in muscular dystrophy patients

    International Nuclear Information System (INIS)

    Hirase, Tsutomu; Ide, Masami; Araki, Shukuro; Okamoto, Hiroshi; Kawasaki, Shoichiro; Imamura, Shigehiro.

    1983-01-01

    We analyzed the microcomponents of protein fractions in the cerebrospinal fluid of patients with various types of muscular dystrophy. The degenerative pattern is characterized by an increase in the prealbumin and a decrease in the γ-globulin fraction is shown in the Duchenne and congenital muscular dystrophy. The increase in CSF IgG, γ-globulin fraction is shown in the myotonic dystrophy. In addition to the abnormality of IQ, EEG, and brain CT, abnormal CSF proteins obviously suggest the presence of CNS involvement in muscular dystrophy. (author)

  19. Relação entre a freqüência de consumo de carne e pescado e os níveis de hexaclorobenzeno, lindano, aldrin e 4,4' - diclorodifenil - 1,1' dicloroetileno, em tecido adiposo de glândulas mamárias de mulheres espanholas Relationship between the frequency of meat and fish intake and the levels of hexaclorobenzene, lindane, aldrin e 4,4' dichloro diphenil 1,1' dichloroethylene, present in mamary adipose tissue samples from spanish women

    Directory of Open Access Journals (Sweden)

    Ijoni Costabeber

    2003-02-01

    Full Text Available Níveis de resíduos dos pesticidas organoclorados, hexaclorobenzeno (HCB, lindano gama-HCH, aldrin e 4,4'-diclorodifenil-1,1'dicloroetileno (p,p'-DDE foram determinados em tecido adiposo mamário de mulheres residentes em Córdoba, Espanha. A maior concentração média foi detectada para o p,p'-DDE (1,869 mig/g de tecido adiposo, que estava presente em 100% das amostras. As concentrações médias de HCB, gama-HCH e aldrin encontrados foram 0,236, 0,003 e 0,006mig/g de tecido adiposo, respectivamente. Os níveis dos resíduos detectados nas amostras foram relacionados ao consumo semanal de carne e pescado pelas doadoras. O único composto cujos níveis variaram significativamente (p£0,05 em função da freqüência de consumo de carne foi o p,p'-DDE, tendo apresentado concentração média de 2,833 mig/g de tecido adiposo quando as doadoras relataram uma freqüência de consumo de carne superior a quatro vezes por semana.The levels of organochlorine pesticide residues ¾ hexaclorobenzene (HCB, lindane (gamma-HCH, aldrin and 4,4'dichloro diphenil 1,1'dichloroethylene (p,p'-DDE - present in mammary adipose tissue samples from women living in Cordoba (Spain were evaluated. The compound found at the highest concentration was p,p´-DDE (1.869(mug/g of adipose tissue, which was present in 100% of the analyzed samples. The average levels of HCB, gamma-HCH and aldrin were 0.236, 0.003 and 0.006mug/g of adipose tissue, respectively. The tissue levels of organochlorine residues were correlated with the weekly intake of meat and fish by donors. Only the tissue levels of p-p´-DDE were significantly (p£0.05 affected by the frequency of meat intake. Donors with a meat intake higher than four times a week presented an average level of 2.833mug/g of adipose tissue.

  20. Emerging strategies for cell and gene therapy of the muscular dystrophies

    OpenAIRE

    Muir, Lindsey A.; Chamberlain, Jeffrey S.

    2009-01-01

    The muscular dystrophies are a heterogeneous group of over 40 disorders that are characterised by muscle weakness and wasting. The most common are Duchenne muscular dystrophy and Becker muscular dystrophy, which result from mutations within the gene encoding dystrophin; myotonic dystrophy type 1, which results from an expanded trinucleotide repeat in the myotonic dystrophy protein kinase gene; and facioscapulohumeral dystrophy, which is associated with contractions in the subtelomeric region ...

  1. Muscular Dystrophy: Hope Through Research

    Science.gov (United States)

    ... of muscular dystrophy appeared in 1830, when Sir Charles Bell wrote an essay about an illness that ... linked disorder to their sons but their daughters will be carriers of that disorder. Carrier females occasionally ...

  2. Meaning of Muscular Dystrophy

    Science.gov (United States)

    ... is very similar to Duchenne, except kids with Becker MD may not have problems until much later, when they're teenagers or adults. It takes a long time for their muscles to become weak. How Does a Kid Get Muscular Dystrophy? MD is not contagious (say: con-TAY-juss), ...

  3. Neurocognitive Profiles in Duchenne Muscular Dystrophy and Gene Mutation Site

    Science.gov (United States)

    D’Angelo, Maria Grazia; Lorusso, Maria Luisa; Civati, Federica; Comi, Giacomo Pietro; Magri, Francesca; Del Bo, Roberto; Guglieri, Michela; Molteni, Massimo; Turconi, Anna Carla; Bresolin, Nereo

    2011-01-01

    The presence of nonprogressive cognitive impairment is recognized as a common feature in a substantial proportion of patients with Duchenne muscular dystrophy. To investigate the possible role of mutations along the dystrophin gene affecting different brain dystrophin isoforms and specific cognitive profiles, 42 school-age children affected with Duchenne muscular dystrophy, subdivided according to sites of mutations along the dystrophin gene, underwent a battery of tests tapping a wide range of intellectual, linguistic, and neuropsychologic functions. Full-scale intelligence quotient was approximately 1 S.D. below the population average in the whole group of dystrophic children. Patients with Duchenne muscular dystrophy and mutations located in the distal portion of the dystrophin gene (involving the 140-kDa brain protein isoform, called Dp140) were generally more severely affected and expressed different patterns of strengths and impairments, compared with patients with Duchenne muscular dystrophy and mutations located in the proximal portion of the dystrophin gene (not involving Dp140). Patients with Duchenne muscular dystrophy and distal mutations demonstrated specific impairments in visuospatial functions and visual memory (which seemed intact in proximally mutated patients) and greater impairment in syntactic processing. PMID:22000308

  4. Upper Body Muscular Endurance Among Children 2-5 Years.

    Science.gov (United States)

    Gabbard, Carl P.; And Others

    The upper body muscular endurance of males and females 2-5 years of age was assessed, and relationships relative to sex, age, endurance and selected anthropometric measures were investigated. None of the relationships were found to be of practical predicative value; while upper body muscular strength increased with age, no significant differences…

  5. MR imaging of fukuyama congenital muscular dystrophy; a case report

    International Nuclear Information System (INIS)

    Yoo, Jeong Hyun; Kim, Yoo Kyung; Koo, Hae Soo; Park, Ki Deuk

    2000-01-01

    Fukuyama congenital muscular dystrophy is a genetic disease and common in Japan. The typical clinical features are hypotonia with an early infantile onset and severe developmental delay. The diagnosis is based on pathologic evidence of muscular dystrophy revealed by biopsy or an increased serum creatine kinase levels. Involvement of the brain is characterized by abnormal cerebral cortical dysplasia, cerebellar dysplasia, and white matter changes. We encountered a case of Fukuyama congenital muscular dystrophy in which brain MRI findings were typical, and present this case together with a review of the literature

  6. Duchenne and Becker muscular dystrophy: a molecular and immunohistochemical approach Distrofia muscular de Duchenne e Becker: abordagem molecular e imuno-histoquímica

    Directory of Open Access Journals (Sweden)

    Aline Andrade Freund

    2007-03-01

    Full Text Available Duchenne muscular dystrophy (DMD and Becker muscular dystrophy (BMD are caused by mutations in the dystrophin gene. We studied 106 patients with a diagnosis of probable DMD/BMD by analyzing 20 exons of the dystrophin gene in their blood and, in some of the cases, by immunohistochemical assays for dystrophin in muscle biopsies. In 71.7% of the patients, deletions were found in at least one of the exons; 68% of these deletions were in the hot-spot 3' region. Deletions were found in 81.5% of the DMD cases and in all the BMD cases. The cases without deletions, which included the only woman in the study with DMD, had dystrophin deficiency. The symptomatic female carriers had no deletions but had abnormal dystrophin distribution in the sarcolemma (discontinuous immunostains. The following diagnoses were made for the remaining cases without deletions with the aid of a muscle biopsy: spinal muscular atrophy, congenital myopathy; sarcoglycan deficiency and unclassified limb-girdle muscular dystrophy. Dystrophin analysis by immunohistochemistry continues to be the most specific method for diagnosis of DMD/BMD and should be used when no exon deletions are found in the dystrophin gene in the blood.As distrofias musculares de Duchenne (DMD e de Becker (DMB são doenças causadas por mutação no gene da distrofina. Foram estudados 106 casos com a suspeita diagnóstica de DMD/BMD com a analise de 20 exons do gene da distrofina no sangue e biópsia muscular com imuno-histoquímica para distrofina em alguns casos. Em 71,7% dos casos foi encontrada deleção em pelo menos um dos exons, sendo que 68% das deleções localizam-se na região 3' hot spot. Foram encontradas deleções em 81,5% dos DMD e em todos os BMD, sendo que os sem deleção tinham deficiência de distrofina, incluindo a mulher com DMD. As portadoras sintomáticas não tinham deleções mas anormalidades na distribuição da distrofina no sarcolema. Os outros casos sem deleção, com auxilio da

  7. Muscular system in interna of Peltogaster paguri (Rhizocephala: Peltogastridae).

    Science.gov (United States)

    Miroliubov, Aleksei A

    2017-03-01

    Rhizocephalan parasites have a peculiar life cycle, and their adults lost almost all traits found usually in Crustacea. Despite some data on anatomy and ultrastructure of interna of Peltogastridae, some crucial aspects of morphology are still unknown. For example, there is only one mentioning of myocytes found in interna of Rhizocephalans (Sacculina carcini). So we aimed at studying the muscular system of the interna of Peltogaster paguri using serial histological sectioning and fluorescent staining (TRITC-labeled phalloidin) with confocal microscopy. Within the wall of the main trunk we found striated muscular fibers. The majority of these fibers form a unidirectional single spiral. There are additional small fibers that connect the coils of the large spiral. The density of muscular fibers is highest near the externa stalk, and the number of muscle fibers decreases towards the distal part of the main trunk. We suggest that such a muscular system could provide peristaltic movements of the main trunk and the transport of nutrients through the interna. Copyright © 2016 Elsevier Ltd. All rights reserved.

  8. Dasatinib as a treatment for Duchenne muscular dystrophy.

    Science.gov (United States)

    Lipscomb, Leanne; Piggott, Robert W; Emmerson, Tracy; Winder, Steve J

    2016-01-15

    Identification of a systemically acting and universal small molecule therapy for Duchenne muscular dystrophy would be an enormous advance for this condition. Based on evidence gained from studies on mouse genetic models, we have identified tyrosine phosphorylation and degradation of β-dystroglycan as a key event in the aetiology of Duchenne muscular dystrophy. Thus, preventing tyrosine phosphorylation and degradation of β-dystroglycan presents itself as a potential therapeutic strategy. Using the dystrophic sapje zebrafish, we have investigated the use of tyrosine kinase and other inhibitors to treat the dystrophic symptoms in this model of Duchenne muscular dystrophy. Dasatinib, a potent and specific Src tyrosine kinase inhibitor, was found to decrease the levels of β-dystroglycan phosphorylation on tyrosine and to increase the relative levels of non-phosphorylated β-dystroglycan in sapje zebrafish. Furthermore, dasatinib treatment resulted in the improved physical appearance of the sapje zebrafish musculature and increased swimming ability as measured by both duration and distance of swimming of dasatinib-treated fish compared with control animals. These data suggest great promise for pharmacological agents that prevent the phosphorylation of β-dystroglycan on tyrosine and subsequent steps in the degradation pathway as therapeutic targets for the treatment of Duchenne muscular dystrophy. © The Author 2015. Published by Oxford University Press.

  9. Urological manifestations of Duchenne muscular dystrophy.

    Science.gov (United States)

    Askeland, Eric J; Arlen, Angela M; Erickson, Bradley A; Mathews, Katherine D; Cooper, Christopher S

    2013-10-01

    Duchenne muscular dystrophy is a dystrophinopathy affecting males that is associated with multiple organ system complications. To our knowledge urological complications of Duchenne muscular dystrophy have been described only anecdotally to date. We reviewed the medical charts of 135 patients with Duchenne or Duchenne-Becker muscular dystrophy for demographics and disease progression, urological diagnoses, intervention and followup. Of 135 patients 67 (50%) had at least 1 documented urological diagnosis and 38 (28%) had multiple manifestations. Lower urinary tract symptoms were the most common urological diagnosis (32% of patients). Survival analysis revealed a median age at onset of lower urinary tract symptoms of 23 years (95% CI 17.7-23.9). Intervention was required in 12 patients (9%), most commonly due to nephrolithiasis. Urological morbidity increased with Duchenne muscular dystrophy progression when stratified by clinical progression. Lower urinary tract symptoms were more common in nonambulatory patients (40.7% vs 19%, p = 0.007), those with a diagnosis of scoliosis (44% vs 19.7%, p = 0.003) and/or scoliosis spine surgery (60% vs 22%, p <0.001), and those on invasive respiratory support (53% vs 29%, p = 0.046). Likewise, nephrolithiasis was more common in nonambulatory patients (10% vs 0%, p = 0.017), those with scoliosis (12% vs 0%, p = 0.004) and/or scoliosis spine surgery (20% vs 1%, p <0.001), and those on invasive respiratory support (29% vs 3%, p <0.001). Only 28% of patients with a urological manifestation were referred to urology. As these patients transition into adolescence and adulthood, the increased prevalence of urological manifestations warrants increased awareness and referral to urologists. Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  10. Respuesta al entrenamiento en EPOC: Diferencia entre limitación por fatiga muscular y por disnea

    Directory of Open Access Journals (Sweden)

    Martín Sívori

    2011-04-01

    Full Text Available Este estudio comparó la respuesta post entrenamiento al ejercicio en pacientes limitados por fatiga muscular (LF vs. disnea (LD. Se incluyeron pacientes con EPOC moderada y grave (definición GOLD, clasificándolos en LF si la respuesta a cicloergometría máxima tenía ≥ 2 puntos en la escala de Borg para fatiga muscular vs. disnea; LD a la inversa. Se realizaron ergometría submáxima, 6 minutos y pruebas de calidad de vida mediante cuestionario. Fueron entrenados 3 veces/semana, 90 min/sesión con ejercicios de fuerza y aeróbicos por 8 semanas, evaluándolos de la misma manera. Fueron estudiados 14 pacientes del grupo LF y 11 del LD. El promedio de edad fue 69 y 66 años respectivamente. Presentaban grave obstrucción bronquial (FEV1: 49%. No existían diferencias basales entre ambos grupos, excepto en el índice masa-corporal (IMC, menor en los LF. Luego del entrenamiento, ambos grupos mejoraron significativamente en variables de ejercicio y calidad de vida, excepto carga máxima en los LD. Comparando ambos grupos, se observó mejor respuesta en los LF en carga máxima (48.7 ± 9.2 vs. 40.04 ± 15.48 watts, p = 0.033, prueba de 6 minutos (505.42 ± 50.75 vs. 454.9 ± 64.3 metros, p = 0.048 y ergometría submáxima (14.57 ± 9.55 vs. 6.71 ± 4.18min, p = 0.025, respectivamente. Como conclusión, los pacientes LF tuvieron mayor respuesta al entrenamiento en ejercicios submáximos y carga máxima, presentando menor IMC. Posiblemente, deberían instrumentarse diferentes estrategias de entrenamiento para diferentes fenotipos de pacientes con EPOC.

  11. Prevalence of cardiomyopathy in duchenne and becker's muscular dystrophy

    International Nuclear Information System (INIS)

    Sultan, A.; Fayaz, M.

    2008-01-01

    Cardiac assessment was not done routinely in Duchenne (DMD) and Becker muscular dystrophy (BMD) patients in Northern region of England while evidence was gathering on progressive cardiomyopathy in these patients. We wanted to find out the prevalence, progression and clinical features of cardiac involvement in Duchenne and Becker muscular dystrophy. Methods: It is a retrospective review of clinical, electrocardiographic and echocardiographic assessments. The notes of 52 Duchenne and Becker muscular dystrophy patients were reviewed out of which 32 had DMD, 6 had Intermediate muscular dystrophy (IMD) and 14 had BMD. Prevalence of preclinical and clinically evident cardiac involvement was 88.4% in DMD and BMD patients. Sixty nine% of patients had clinically evident cardiac involvement but only four patients had cardiac symptoms in the form of palpitations, out of which two were due to respiratory dysfunction and others was due to cardiac failure. Clinical examination of the rest of all of the patients was unremarkable. Electrocardiogram was abnormal in 88.4% of patients. Conduction defects were found in 19.4% of patients. Echocardiogram was abnormal in 80.7% of patients but all were poor echo subjects including those who had normal echocardiogram. Though most patients were asymptomatic, a high percentage had evidence of preclinical and clinically evident cardiac involvement. So in all patients with Xp21 linked muscular dystrophy a routine baseline cardiac assessment should be done at the age of 10 years and reviewed after intervals of one to two years. (author)

  12. PREDICCION DE LA ALTURA DE SALTO VERTICAL. IMPORTANCIA DEL IMPULSO MECÁNICO Y DE LA MASA MUSCULAR DE LAS EXTREMIDADES INFERIORES

    Directory of Open Access Journals (Sweden)

    C. Ferragut

    2010-09-01

    Full Text Available

     

    RESUMEN

    El objetivo de este estudio ha sido determinar si es posible predecir la altura de vuelo en el salto vertical a partir de variables cinemáticas, dinamométricas y antropométricas, mediante un modelo de regresión múltiple lineal. Participaron en el estudio 53 sujetos, 21 hombres jugadores de voleibol de categorías nacionales (División de Honor y Primera División y 9 mujeres jugadoras de voleibol de División de Honor, así como 23 estudiantes de Educación Física, de los cuales 12 eran hombres y 11 mujeres. Inicialmente se determinó la altura de vuelo en saltos efectuados sin contramovimiento o "squat jumps" (SJ y en saltos precedidos por un contramovimiento o "countermovement jumps" (CMJ. Además, se determinó la fuerza isométrica máxima (FIM en posición de semisentadillla, con las rodillas flexionadas a 90º, 120º y 140º , simultáneamente se tomaron medidas de la actividad electromiográfica del vasto externo del cuádriceps. La masa muscular de las extremidades inferiores se midió mediante absociometría fotónica dual de rayos X (DEXA. El impulso positivo explicó por sí solo un 77% de la variabilidad en altura de vuelo. La variable anterior combinada con el porcentaje de masa corporal representado por la masa muscular de las extremidades inferiores permitió explicar un 82% de la variabilidad de la altura de vuelo en el CMJ. Al añadir a la ecuación anterior la masa muscular de las extremidades inferiores se pudo explicar un 98% de la variabilidad en altura de vuelo. En los saltos sin contramovimiento, también fue posible explicar un porcentaje similar de la variabilidad de la altura de vuelo utilizando las mismas variables.
    PALABRAS CLAVE: Salto vertical, DEXA, masa muscular, fuerza

    ABSTRACT

  13. Biological, Psychological, and Sociocultural Factors Contributing to the Drive for Muscularity in Weight-Training Men

    Science.gov (United States)

    Schneider, Catharina; Rollitz, Laura; Voracek, Martin; Hennig-Fast, Kristina

    2016-01-01

    The drive for muscularity and associated behaviors (e.g., exercising and dieting) are of growing importance for men in Western societies. In its extreme form, it can lead to body image concerns and harmful behaviors like over-exercising and the misuse of performance-enhancing substances. Therefore, investigating factors associated with the drive for muscularity, especially in vulnerable populations like bodybuilders and weight trainers can help identify potential risk and protective factors for body image problems. Using a biopsychosocial framework, the aim of the current study was to explore different factors associated with drive for muscularity in weight-training men. To this purpose, German-speaking male weight trainers (N = 248) completed an online survey to determine the extent to which biological, psychological, and sociocultural factors contribute to drive for muscularity and its related attitudes and behaviors. Using multiple regression models, findings showed that media ideal body internalization was the strongest positive predictor for drive for muscularity, while age (M = 25.9, SD = 7.4) held the strongest negative association with drive for muscularity. Dissatisfaction with muscularity, but not with body fat, was related to drive for muscularity. The fat-free mass index, a quantification of the actual degree of muscularity of a person, significantly predicted drive for muscularity-related behavior but not attitudes. Body-related aspects of self-esteem, but not global self-esteem, were significant negative predictors of drive for muscularity. Since internalization of media body ideals presented the highest predictive value for drive for muscularity, these findings suggest that media body ideal internalizations may be a risk factor for body image concerns in men, leading, in its most extreme form to disordered eating or muscle dysmorphia. Future research should investigate the relations between drive for muscularity, age, body composition

  14. CT findings of muscular dystrophy

    International Nuclear Information System (INIS)

    Saitoh, Hiroshi

    1991-01-01

    CT scans of muscles in patients with limb girdle type (LG), myotonic type (MYD) and Duchenne type (DMD) dystrophies were obtained at five different body levels: the neck, L3 vertebral body, pelvic girdle, thigh and lower leg. CT numbers, cross sectional areas (CSA) and %CSA of muscle or fat were evaluated in each muscle. The characteristic CT patterns for each type of muscular dystrophy were obtained. Compared with DMD, the gracilis and soleus were more severely damaged in LG and the biceps femoris remained relatively preserved among the hamstrings. In addition, the multifidus of the neck and sternocleidomastoid also were more severely damaged in MYD. This study suggests that CT scan will be useful in the differential diagnosis of these types of muscular dystrophy as well as in planning appropriate rehabilitation and detecting damaged muscles. (author)

  15. The Role of Adiposity in the Association between Muscular Fitness and Cardiovascular Disease.

    Science.gov (United States)

    Pérez-Bey, Alejandro; Segura-Jiménez, Víctor; Fernández-Santos, Jorge Del Rosario; Esteban-Cornejo, Irene; Gómez-Martínez, Sonia; Veiga, Oscar L; Marcos, Ascensión; Castro-Piñero, José

    2018-05-11

    To test the associations of muscular fitness and body mass index (BMI), individually and combined, with clustered cardiovascular disease risk factors in children and adolescents and to analyze the mediator role of BMI in the association between muscular fitness and clustered cardiovascular disease risk factors. In total, 239 children (113 girls) and 270 adolescents (128 girls) participated in this cross-sectional study. Height and weight were assessed, and BMI was calculated. A cardiovascular disease risk factors index (CVDRF-I) was created from the combination of the following variables: waist circumference, systolic blood pressure, triglycerides, high-density lipoprotein cholesterol, and glucose. Handgrip strength/weight and standing long jump tests were used to assess muscular fitness. A muscular fitness index was computed from the combination of both tests. Muscular fitness index was associated with CVDRF-I in children of both sexes and adolescent boys; however, these associations disappeared after accounting for BMI. BMI was associated with CVDRF-I in both children and adolescents, even after adjusting for muscular fitness (all P < .001). In male and female children and in adolescent boys, the association between muscular fitness and CVDRF-I was mediated by BMI (all P < .001). Because there was no association between muscular fitness and CVDRF-I in adolescent girls, the mediation hypothesis was discarded. BMI is an independent predictor of CVDRF-I in children and adolescents of both sexes. Conversely, the effect of muscular fitness on CVDRF-I seems to be fully mediated by BMI levels in male and female children and in adolescent boys. Copyright © 2018 Elsevier Inc. All rights reserved.

  16. Development of Non-Hormonal Steroids for the Treatment of Duchenne Muscular Dystrophy

    Science.gov (United States)

    2013-02-01

    constructs envisioned in gene therapy, are also expressed in Becker muscular dystrophy (alleles of dystrophinopathy leading to milder disease). In other words...the Treatment of Duchenne Muscular Dystrophy PRINCIPAL INVESTIGATOR: Terence Partridge, PhD CONTRACTING ORGANIZATION: Children’s...Duchenne Muscular Dystrophy 5a. CONTRACT NUMBER 5b. GRANT NUMBER W81XWH-11-1-0754 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Terence Partridge

  17. How Physicians Support Mothers of Children with Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Fujino, Haruo; Saito, Toshio; Matsumura, Tsuyoshi; Shibata, Saki; Iwata, Yuko; Fujimura, Harutoshi; Shinno, Susumu; Imura, Osamu

    2015-09-01

    Communicating about Duchenne muscular dystrophy and its prognosis can be difficult for affected children and their family. We focused on how physicians provide support to the mothers of children with Duchenne muscular dystrophy who have difficulty communicating about the condition with their child. The eligible participants were certified child neurologists of the Japanese Society of Child Neurology. Participants responded to questionnaires consisting of free descriptions of a vignette of a child with Duchenne muscular dystrophy and a mother. We analyzed 263 responses of the participants. We found 4 themes on advising mothers, involving encouraging communication, family autonomy, supporting family, and considering the child's concerns. These results provide a better understanding of the communication between physicians and family members who need help sharing information with a child with Duchenne muscular dystrophy. These findings will assist clinical practitioners in supporting families and the affected children throughout the course of their illness. © The Author(s) 2015.

  18. Serum creatinine level: a supplemental index to distinguish Duchenne muscular dystrophy from Becker muscular dystrophy.

    Science.gov (United States)

    Zhang, Huili; Zhu, Yuling; Sun, Yiming; Liang, Yingyin; Li, Yaqin; Zhang, Yu; Deng, Langhui; Wen, Xingxuan; Zhang, Cheng

    2015-01-01

    To improve assessment of dystrophinopathy, the aim of this study was to identify whether serum creatinine (Crn) level reflects disease severity. Biochemical, Vignos score, and genetic data were collected on 212 boys with dystrophinopathy. Serum Crn level had a strong inverse correlation with Vignos score by simple correlation (r = -0.793) and partial correlation analysis after adjustment for age, height, and weight (r = -0.791; both P Becker muscular dystrophy (BMD) patients than Duchenne muscular dystrophy (DMD) patients at ages 4, 5, 7, and 9 yr (all P < 0.0125). After adjusting for age, height, and weight, BMD patients still had a significantly higher serum Crn level than DMD patients (β = 7.140,  t = 6.277,  P < 0.01). Serum Crn level reflected disease severity and may serve as a supplemental index to distinguish DMD from BMD in clinical practice.

  19. The drive for muscularity in men: media influences and objectification theory.

    Science.gov (United States)

    Daniel, Samantha; Bridges, Sara K

    2010-01-01

    Presently, objectification theory has yielded mixed results when utilized to explain body image concerns in men. An online survey assessing internalization of media ideals, self-objectification, body surveillance, body shame, the drive for muscularity, and body mass index (BMI) was completed by 244 predominantly college-aged males. Path analyses were used to investigate relationships among these variables where it was hypothesized that objectification variables would mediate the relationship between internalization of media ideals and the drive for muscularity. Internalization of media ideals was the strongest predictor of the drive for muscularity, followed by BMI, though variables of objectification theory had no impact on the drive for muscularity contrary to hypotheses. The results suggest that objectification theory may not be applicable to men as it is currently measured. Copyright 2009 Elsevier Ltd. All rights reserved.

  20. Tratamento da distrofia muscular progressiva com lactato de sódio Treatment of progressive muscular dystrophy with sodium lactate

    Directory of Open Access Journals (Sweden)

    José Antonio Levy

    1969-12-01

    Full Text Available Com base em trabalhos anteriores, 13 casos de distrofia muscular progressiva foram tratados com lactato de sódio 1/6 molar associado a ATP e complexo B. O exame da força muscular, realizado antes e após o tratamento — salvo em dois casos nos quais ocorreram melhoras muito discretas — não mostrou qualquer efeito favorável da medicação. Os autores sugerem a verificação de possíveis alterações enzimáticas provocadas pelo lactato de sódio, o que serviria para melhor avaliação do efeito terapêutico.Thirteen cases of progressive muscular dystrophy were treated with 1/6 M. sodium lactate plus ATP and B complex. Examinations of muscle strength, before and after the treatment, did not show any favourable effects, except in two of the cases which showed slight improvement. The authors suggest that possible enzimatic alterations caused by the sodium lactate be checked up on, since this checking could be employed in the evaluation of the therapeutic effects.

  1. Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials.

    Science.gov (United States)

    Anthony, Karen; Cirak, Sebahattin; Torelli, Silvia; Tasca, Giorgio; Feng, Lucy; Arechavala-Gomeza, Virginia; Armaroli, Annarita; Guglieri, Michela; Straathof, Chiara S; Verschuuren, Jan J; Aartsma-Rus, Annemieke; Helderman-van den Enden, Paula; Bushby, Katherine; Straub, Volker; Sewry, Caroline; Ferlini, Alessandra; Ricci, Enzo; Morgan, Jennifer E; Muntoni, Francesco

    2011-12-01

    Duchenne muscular dystrophy is caused by mutations in the DMD gene that disrupt the open reading frame and prevent the full translation of its protein product, dystrophin. Restoration of the open reading frame and dystrophin production can be achieved by exon skipping using antisense oligonucleotides targeted to splicing elements. This approach aims to transform the Duchenne muscular dystrophy phenotype to that of the milder disorder, Becker muscular dystrophy, typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin. There is ongoing debate regarding the functional properties of the different internally deleted dystrophins produced by exon skipping for different mutations; more insight would be valuable to improve and better predict the outcome of exon skipping clinical trials. To this end, we have characterized the clinical phenotype of 17 patients with Becker muscular dystrophy harbouring in-frame deletions relevant to on-going or planned exon skipping clinical trials for Duchenne muscular dystrophy and correlated it to the levels of dystrophin, and dystrophin-associated protein expression. The cohort of 17 patients, selected exclusively on the basis of their genotype, included 4 asymptomatic, 12 mild and 1 severe patient. All patients had dystrophin levels of >40% of control and significantly higher dystrophin (P = 0.013), β-dystroglycan (P = 0.025) and neuronal nitric oxide synthase (P = 0.034) expression was observed in asymptomatic individuals versus symptomatic patients with Becker muscular dystrophy. Furthermore, grouping the patients by deletion, patients with Becker muscular dystrophy with deletions with an end-point of exon 51 (the skipping of which could rescue the largest group of Duchenne muscular dystrophy deletions) showed significantly higher dystrophin levels (P = 0.034) than those with deletions ending with exon 53. This is the first quantitative study on both

  2. X-rays computed tomographic scans of lower limb and trunk muscles in facioscapulohumeral muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Horikawa, Hirosei; Mano, Yukio; Takayanagi, Tetsuya [Nara Medical Univ., Kashihara (Japan); Takahashi, Keiichi; Nishio, Hisahide

    1992-10-01

    X-rays computed tomographic (CT) scans of muscles of the lower limbs and the trunk in 14 patients with facioscapulohumeral muscular dystrophy (FSH) were studied. The CT scans showed that the affected muscles were decreased in density and size. The laterality of muscular involvement was sometimes observed. The muscular lesions in the lower limbs showed proximal distribution. In the thigh, the hamstrings were affected first, the adductor muscles second, and then the muscular involvement progressed to the quadriceps femoris muscle. In the lower leg, the gastrocnemius and soleus muscles were relatively spared as compared with the tibialis anterior muscle. In the lumbar girdle, the abdominal muscles were involved first, the gluteal muscles second, the back muscles third, and the psoas major muscle were relatively spared. The muscular weakness of this distribution exacerbated lumbar lordosis. The neck muscles were less affected than those of the lumbar girdle. The CT scans in FSH demonstrated the characteristic pattern of muscular involvement, which differed from the inherited muscular diseases such as Duchenne muscular dystrophy, myotonic dystrophy, and others. (author).

  3. X-rays computed tomographic scans of lower limb and trunk muscles in facioscapulohumeral muscular dystrophy

    International Nuclear Information System (INIS)

    Horikawa, Hirosei; Mano, Yukio; Takayanagi, Tetsuya; Takahashi, Keiichi; Nishio, Hisahide.

    1992-01-01

    X-rays computed tomographic (CT) scans of muscles of the lower limbs and the trunk in 14 patients with facioscapulohumeral muscular dystrophy (FSH) were studied. The CT scans showed that the affected muscles were decreased in density and size. The laterality of muscular involvement was sometimes observed. The muscular lesions in the lower limbs showed proximal distribution. In the thigh, the hamstrings were affected first, the adductor muscles second, and then the muscular involvement progressed to the quadriceps femoris muscle. In the lower leg, the gastrocnemius and soleus muscles were relatively spared as compared with the tibialis anterior muscle. In the lumbar girdle, the abdominal muscles were involved first, the gluteal muscles second, the back muscles third, and the psoas major muscle were relatively spared. The muscular weakness of this distribution exacerbated lumbar lordosis. The neck muscles were less affected than those of the lumbar girdle. The CT scans in FSH demonstrated the characteristic pattern of muscular involvement, which differed from the inherited muscular diseases such as Duchenne muscular dystrophy, myotonic dystrophy, and others. (author)

  4. A case of muscular sarcoidosis diagnosed by gallium-67 scintigraphy and magnetic resonance imaging

    International Nuclear Information System (INIS)

    Sohn, Hyung Sun; Kim, Euy Neyng

    1999-01-01

    Gallium-67 scintigraphy is helpful in the assessment of active extrapulmonary sarcoidosis. Muscular involvement of sarcoidosis is often asymptomatic or nonspecific, and laboratory examinations do not provide convincing evidence of muscular involvement. We report a case of muscular sarcoidosis, which was detected by gallium-67 scintigraphy. In a patient who was suffering from fever and arthalgia of knee joint, gallium-67 scintigraphy showed mediastinal and hilar involvement of sarcoidosis with unexpected extensive muscular uptake. Magnetic resonance imaging revealed the detailed depiction of intramuscular infiltration of sarcoid granuloma. Gallium-67 scintigraphy is useful in detecting inflammatory muscular involvement of sarcoidosis as well as other multiorgan involvement

  5. Inherited myopathies and muscular dystrophies

    NARCIS (Netherlands)

    Cardamone, Michael; Darras, Basil T.; Ryan, Monique M.

    The inherited myopathies and muscular dystrophies are a diverse group of muscle diseases presenting with common complaints and physical signs: weakness, motor delay, and respiratory and bulbar dysfunction. The myopathies are caused by genetic defects in the contractile apparatus of muscle, and

  6. Genetics and emerging treatments for Duchenne and Becker muscular dystrophy.

    Science.gov (United States)

    Wein, Nicolas; Alfano, Lindsay; Flanigan, Kevin M

    2015-06-01

    Mutations in the DMD gene result in Duchenne or Becker muscular dystrophy due to absent or altered expression of the dystrophin protein. The more severe Duchenne muscular dystrophy typically presents around ages 2 to 5 with gait disturbance, and historically has led to the loss of ambulation by age 12. It is important for the practicing pediatrician, however, to be aware of other presenting signs, such as delayed motor or cognitive milestones, or elevated serum transaminases. Becker muscular dystrophy is milder, often presenting after age 5, with ambulation frequently preserved past 20 years and sometimes into late decades. Copyright © 2015 Elsevier Inc. All rights reserved.

  7. Limb girdle muscular dystrophy due to mutations in POMT2

    DEFF Research Database (Denmark)

    Østergaard, Sofie Thurø; Johnson, Katherine; Stojkovic, Tanya

    2018-01-01

    BACKGROUND: Mutations in the gene coding for protein O-mannosyl-transferase 2 (POMT2) are known to cause severe congenital muscular dystrophy, and recently, mutations in POMT2 have also been linked to a milder limb-girdle muscular dystrophy (LGMD) phenotype, named LGMD type 2N (LGMD2N). Only four...

  8. Primary muscular hydatid: preoperative diagnosis Throught computerized tomography and ultrasonography

    International Nuclear Information System (INIS)

    Macho Fernandez, J.M.; Marin Cardenas, M.A.; Mazas Artasona, L.; Lample Lacasa, C.; Otero Sierra, C.; Hernandez Navarrete, M.J.; Gomez-Pereda, R.

    1995-01-01

    Primary muscular hydatid disease, is extremely rare,- but not exceptional-, comparatively with other atypical localization. In this article the authors revised 474 patients with hydatid disease over a ten years period. Three cases of primary muscular localization were found. The ultrasonography and computed tomography facilitates the preoperative diagnosis. (Author) 40 refs

  9. Disparities in the diagnostic process of Duchenne and Becker muscular dystrophy.

    Science.gov (United States)

    Holtzer, Caleb; Meaney, F John; Andrews, Jennifer; Ciafaloni, Emma; Fox, Deborah J; James, Katherine A; Lu, Zhenqiang; Miller, Lisa; Pandya, Shree; Ouyang, Lijing; Cunniff, Christopher

    2011-11-01

    To determine whether sociodemographic factors are associated with delays at specific steps in the diagnostic process of Duchenne and Becker muscular dystrophy. We examined abstracted medical records for 540 males from population-based surveillance sites in Arizona, Colorado, Georgia, Iowa, and western New York. We used linear regressions to model the association of three sociodemographic characteristics with age at initial medical evaluation, first creatine kinase measurement, and earliest DNA analysis while controlling for changes in the diagnostic process over time. The analytical dataset included 375 males with information on family history of Duchenne and Becker muscular dystrophy, neighborhood poverty levels, and race/ethnicity. Black and Hispanic race/ethnicity predicted older ages at initial evaluation, creatine kinase measurement, and DNA testing (P Becker muscular dystrophy predicted younger ages at initial evaluation, creatine kinase measurement and DNA testing (P Becker muscular dystrophy are evident even after adjustment for family history of Duchenne and Becker muscular dystrophy and changes in the diagnostic process over time. Black and Hispanic children are initially evaluated at older ages than white children, and the gap widens at later steps in the diagnostic process.

  10. FDG-PET imaging of lower extremity muscular activity during level walking

    International Nuclear Information System (INIS)

    Oi, Naoyuki; Iwaya, Tsutomu; Tobimatsu, Yoshiko; Fujimoto, Toshihiko; Itoh, Masatoshi; Yamaguchi, Keiichiro

    2003-01-01

    We analyzed muscular activity of the lower extremities during level walking using positron emission tomography (PET) with 18 F-fluorodeoxyglucose ( 18 F-FDG). We examined 17 healthy male subjects; 11 were assigned to a walking group and 6 to a resting group. After 18 F-FDG injection, the walking group subjects walked at a free speed for 15 min. A whole-body image was then obtained by a PET camera, and the standardized uptake ratio (SUR) was computed for each muscle. The SUR for each muscle of the walking group was compared with that for the corresponding muscles in the resting group. The level of muscular activity of all the muscles we examined were higher during level walking than when resting. The activity of the lower leg muscles was higher than that of the thigh muscles during level walking. The muscular activity of the soleus was highest among all the muscles examined. Among the gluteal muscles, the muscular activity of the gluteus minimus was higher than that of the gluteus maximus and gluteus medius. The concurrent validity of measuring muscular activity of the lower extremity during level walking by the PET method using 18 F-FDG was demonstrated. (author)

  11. Immunohistochemical alterations of dystrophin in congenital muscular dystrophy Alterações imuno-hístoquímicas da distrofina na distrofia muscular congênita

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1995-09-01

    Full Text Available The dystrophin distribution in the plasma muscle membrane using immunohystochemistry was studied in 22 children with congenital muscular dystrophy. The dystrophin was detected by immunofluorescence in muscle biopsy through a polyclonal antibody. All the cases had patchy interruptions of the fluorescence in the plasma membrane. A large patchy interruption of the sarcolemma was found in 17 cases, small interruption in 12, and a combination of large and small patchy discontinuity in 7. Small gaps around the fiber like a rosary were found in 15 cases. The frequency of these abnormalities ranged cases from: all fibers in 5 cases, frequent in 8, occasional in 5, and rare in 4. Five cases had total absence of immunofluorescence. These results suggest that the dystrophin expression is abnormal in this group of children and that this type of abnormalities can not be differentiated from early Becker muscular dystrophy nor childhood autosomal recessive muscular dystrophy through immunohystochemistry alone.Foi estudada a distribuição da distrofina na membrana plasmática das fibras musculares em 22 crianças com distrofia muscular congênita, através de técnicas de imuno-histoquímica. A distrofina foi identificada nas biópsias musculares processadas a fresco, por técnicas de imunofluorescência utilizando anticorpos policlonais. Todos os casos tinham interrupções da imunofluorescência na membrana plasmática. Em 17 elas eram grandes, em 12 eram pequenas e em 7 eram de ambos os tipos. Fibras com interrupções pequenas e constantes, como um rosário, foram vistas em 15 casos. Essas anormalidades estavam presentes em todas as fibras em 5 casos, eram frequentes em 8, ocasionais em 5 e raras em 4. Cinco casos mostraram fibras sem distrofina. Esses dados sugerem que a expressão da distrofina é anormal nesse grupo de crianças. Essas anormalidades podem também ser encontradas em casos precoces de distrofia muscular de Becker e distrofia autoss

  12. Caracterización a impacto de caucho reciclado mediante elementos finitos

    OpenAIRE

    Escribano Castro, Ane

    2015-01-01

    Análisis de caucho reciclado de manera hiperelástica mediante métodos de ajuste de Mínimos Cuadrados con programa MATLAB y Curve fitting mediante ANSYS. Para la parte viscoelástica se usa Algoritmo de Optimicación con MATLAB. Comprobación de resultados y fiabilidad.

  13. Men, Muscles, and Eating Disorders: an Overview of Traditional and Muscularity-Oriented Disordered Eating.

    Science.gov (United States)

    Lavender, Jason M; Brown, Tiffany A; Murray, Stuart B

    2017-06-01

    There is growing recognition that eating disorder (ED) symptoms, particularly those of a muscularity-oriented nature, are more common in men than previously understood. The purpose of the current review is to describe contemporary directions and implications of research on traditional and muscularity-oriented ED symptoms among males. Evidence indicates that ED symptoms occur in a substantial minority of men. Importantly, recent research has focused on muscularity-oriented body image and disordered eating in males, demonstrating the prevalence, correlates, and consequences of maladaptive muscularity-oriented attitudes and behaviors. A growing number of assessments are available to measure these constructs in males, and preliminary treatment considerations have begun to be addressed in the literature. Research on male EDs and body image is increasingly focusing on muscularity-oriented manifestations. Continued empirical work will be critical to improve our understanding of the onset, maintenance, and treatment of muscularity-oriented disordered eating in males.

  14. Dysphagia in facioscapulohumeral muscular dystrophy.

    NARCIS (Netherlands)

    Wohlgemuth, M.; Swart, B.J.M. de; Kalf, J.G.; Joosten, F.B.M.; Vliet, A.M. van der; Padberg, G.W.A.M.

    2006-01-01

    Dysphagia is not considered a symptom of facioscapulohumeral muscular dystrophy (FSHD). In this study, the authors found that dysphagia does occur in patients with advanced FSHD showing mild involvement of the jaw and lingual muscles. Dysphagia is seldom life threatening in these patients. The

  15. Lamin A/C mutations with lipodystrophy, cardiac abnormalities, and muscular dystrophy

    NARCIS (Netherlands)

    van der Kooi, A. J.; Bonne, G.; Eymard, B.; Duboc, D.; Talim, B.; van der Valk, M.; Reiss, P.; Richard, P.; Demay, L.; Merlini, L.; Schwartz, K.; Busch, H. F. M.; de Visser, M.

    2002-01-01

    Mutations in the lamin A/C gene are found in Emery-Dreifuss muscular dystrophy, limb girdle muscular dystrophy with cardiac conduction disturbances, dilated cardiomyopathy with conduction system disease, and familial partial lipodystrophy. Cases with lamin A/C mutations presenting with lipodystrophy

  16. Quantification of muscle strength and motor ability in patients with Duchenne muscular dystrophy on steroid therapy Quantificação da força muscular e habilidades motoras em pacientes com distrofia muscular de Duchenne em corticoterapia

    Directory of Open Access Journals (Sweden)

    Samara Lamounier Santana Parreira

    2007-06-01

    Full Text Available OBJECTIVE: An assessment protocol was applied to quantify and describe muscular strength and motor abilities of 32 patients with Duchenne muscular dystrophy (DMD, aged between 5 and 12 years on steroid therapy. METHOD: Assessments were made monthly for the first six months and with intervals of two months thereafter until the 14-month end point. The tests employed included: the Medical Research Council (MRC scale; the Hammersmith motor ability score; maximum weight lift; timed rise from floor and nine-meter walk. RESULTS: The results showed that loss of muscular strength and motor abilities were slowed in comparison to that observed in the natural evolution of the disease according to the literature. CONCLUSION: We conclude that a swift and objective assessment may be performed using the MRC scale for lower limbs and trunk, the Hammersmith motor ability score, timed nine-meter walk and weight lifts.OBJETIVO: Um protocolo de avaliação foi aplicado com o objetivo de quantificar e descrever evolutivamente a força muscular e as habilidades motoras de 32 pacientes com distrofia muscular de Duchenne (DMD, com idades variando de 5 a 12 anos, em corticoterapia. MÉTODO: As avaliações foram aplicadas mensalmente durante os primeiros seis meses e bimensais até completar um período de 14 meses. Os testes empregados foram: escala da "Medical Research Council" (MRC; Hammersmith "motor ability score"; levantamento da carga máxima de peso; cronometragem do tempo para levantar-se do chão e percorrer nove metros. RESULTADOS: Os resultados demonstraram que a perda da força muscular e das habilidades motoras foi mais lenta do que a observada na evolução natural da doença, como descrito na literatura internacional. CONCLUSÃO: Concluímos que uma rápida e objetiva avaliação pode ser executada utilizando a escala MRC para membros inferiores e tronco, Hammersmith motor ability score, cronometragem do tempo para percorrer 9 metros.e o levantamento de

  17. Predicting muscularity-related behavior, emotions, and cognitions in men: The role of psychological need thwarting, drive for muscularity, and mesomorphic internalization.

    Science.gov (United States)

    Edwards, Christian; Tod, David; Molnar, Gyozo; Markland, David

    2016-09-01

    We examine the relationships that internalization, need thwarting (NT), and drive for muscularity (DFM), along with their interactions, had with weightlifting, muscle dissatisfaction (MD), and muscle-related-worry (MRW). A sample of 552 men (MAge=20.5 years, SD=3.1) completed the Psychological Need Thwarting Scale, the Internalization subscale of the male version of the Sociocultural Attitudes Towards Appearance Questionnaire, the Drive for Muscularity Scale-Attitudes subscale, the Male Body Attitudes Scale-Muscularity subscale, the Body Change Inventory-Worry subscale, and an inventory assessing weightlifting behavior. DFM significantly predicted weightlifting, MRW, and MD. Internalization significantly predicted weightlifting and MRW. NT significantly predicted weightlifting and MD, and its relationship with MRW approached significance. The interaction terms did not predict weightlifting or MRW. The NT/DFM and NT/Internalization interaction terms predicted MD. These results highlight the role of NT in predicting appearance variables in men. Copyright © 2016 Elsevier Ltd. All rights reserved.

  18. Pulmonary Endpoints in Duchenne Muscular Dystrophy. A Workshop Summary.

    Science.gov (United States)

    Finder, Jonathan; Mayer, Oscar Henry; Sheehan, Daniel; Sawnani, Hemant; Abresch, R Ted; Benditt, Joshua; Birnkrant, David J; Duong, Tina; Henricson, Erik; Kinnett, Kathi; McDonald, Craig M; Connolly, Anne M

    2017-08-15

    Development of novel therapeutics for treatment of Duchenne muscular dystrophy (DMD) has led to clinical trials that include pulmonary endpoints that allow assessment of respiratory muscle status, especially in nonambulatory subjects. Parent Project Muscular Dystrophy (PPMD) convened a workshop in Bethesda, Maryland, on April 14 and 15, 2016, to summarize published respiratory data in DMD and give guidance to clinical researchers assessing the effect of interventions on pulmonary outcomes in DMD.

  19. Thinness and muscularity internalization: Associations with disordered eating and muscle dysmorphia in men.

    Science.gov (United States)

    Klimek, Patrycja; Murray, Stuart B; Brown, Tiffany; Gonzales Iv, Manuel; Blashill, Aaron J

    2018-04-01

    The tripartite influence model of body image identifies internalization of societal body ideals as a risk factor for developing body dissatisfaction, and subsequent disordered eating behavior. In men, internalization of two dimensions of body image ideals, thinness and muscularity, is associated with body dissatisfaction and eating concerns. However, it is unknown how thinness and muscularity internalization interact in predicting muscle dysmorphia and disordered eating in men. Data were collected online from 180 undergraduate men, with ages ranging from 18 to 33 years (19.6, SD = 2.6). Regression models were used to test the interactive effects of thinness and muscularity internalization on (a) muscle dysmorphia symptoms and (b) disordered eating. Subsequent simple slope analyses probed effects at the mean, and ±1 standard deviation of thinness internalization. Muscularity and thinness internalization were independently positively related to muscle dysmorphia symptoms and disordered eating. Additionally, a significant interaction revealed that muscularity internalization was increasingly related to muscle dysmorphia symptoms as thinness internalization decreased. Men who internalized the muscular ideal had higher levels of muscle dysmorphia when they did not highly internalize the thin ideal. However, greater internalization of both the muscularity and thin ideal independently may be most relevant in the development of disordered eating in men. Future research is needed to explore variability in experiences of muscle dysmorphia compared with disordered eating in males. © 2018 Wiley Periodicals, Inc.

  20. Determinants of the epithelial-muscular axis on embryonic stem cell-derived gut-like structures.

    Science.gov (United States)

    Luo, Yi; Takaki, Miyako; Misawa, Hiromi; Matsuyoshi, Hiroko; Sasahira, Tomonori; Chihara, Yoshitomo; Fujii, Kiyomu; Ohmori, Hitoshi; Kuniyasu, Hiroki

    2010-01-01

    Dome-like structures with epithelial-muscular layers resembling the gut have been derived from mouse embryonic stem (ES) cells. These domes have been reported to show spontaneous contractions and are called ES gut. In the present study, we examined the epithelial-muscular axis of these domes by detecting differentiation markers. A normal epithelial-muscular axis was exhibited in the domes with spontaneous motility, whereas the domes without spontaneous motility showed either an inverted or obscure axis. To investigate the factors affecting the epithelial-muscular axis, we examined the expression of hedgehog signaling factors in the domes. Expression of hedgehog family factors was detected in the epithelial components of the domes with motility, whereas this expression was inverted or obscure in the domes without motility. Out of the 25 domes, 10 of the 10 motility (+) domes showed a normal epithelial-muscular axis, whereas 14 of the 15 motility (-) domes lacked a normal epithelial-muscular axis. This implies that activin A upregulated the expression of sonic hedgehog and intestinal alkaline phosphatase in the embryoid bodies. These findings suggest that the motility of the ES gut depends on the domes' epithelial-muscular axis. Copyright © 2010 S. Karger AG, Basel.

  1. Predicting drive for muscularity behavioural engagement from body image attitudes and emotions.

    Science.gov (United States)

    Tod, David; Edwards, Christian

    2013-01-01

    We examined the potential moderating effects of appearance investment, body image disturbance, and situational body image dysphoria on the drive for muscularity attitude-behaviour relationship. Participants (339 British college men, M(AGE)=20.00 years, SD=2.59) completed drive for muscularity attitude and behaviour, appearance investment, body image disturbance, and situational body image dysphoria measures. Results indicated higher levels of appearance investment, body image disturbance, and situational body image dysphoria were associated with increases in the drive for muscularity attitude's relationship with physique-enhancing behavioural engagement. Results help extend recent research that has moved beyond identifying correlates to examining ways that groups of variables interact to predict drive for muscularity behavioural engagement and may contribute to theory development. Copyright © 2012 Elsevier Ltd. All rights reserved.

  2. Prevention of pectus excavatum for children with spinal muscular atrophy type 1.

    Science.gov (United States)

    Bach, John R; Bianchi, Carlo

    2003-10-01

    To demonstrate the elimination of pectus excavatum and promotion of more normal lung growth and chest wall development by the use of high-span positive inspiratory pressure plus positive end-expiratory pressure (PIP+PEEP), patients with spinal muscular atrophy type 1 with paradoxical breathing were placed on high-span PIP+PEEP when sleeping from the point of diagnosis of spinal muscular atrophy. Although the appearance of pectus excavatum is ubiquitous in untreated infants with spinal muscular atrophy type 1, after institution of high-span PIP+PEEP, pectus resolves and lungs and chest walls grow more normally. High-span PIP+PEEP is indicated for all infants diagnosed with spinal muscular atrophy who demonstrate paradoxical breathing for the purpose of promoting more normal lung and chest development.

  3. Dominant inherited distal spinal muscular atrophy with atrophic and hypertrophic calves

    NARCIS (Netherlands)

    Groen, R J; Sie, O G; van Weerden, T W

    The clinical, electrophysiological, radiological and morphological data of 3 members of a family with autosomal dominant distal spinal muscular atrophy (DSMA) are reported. One patient has the clinical picture of peroneal muscular atrophy with atrophic calves. His father and sister suffer from

  4. Some Dynamics of Personality Development in Boys Suffering from Muscular Dystrophy

    Science.gov (United States)

    Mearig, Judith S.

    1973-01-01

    Discussed are personality aspects of Duchenne or pseudohypertrophic muscular dystrophy, a progressive wasting of muscular tissue, which afflicts only boys, and usually has its noticeable onset before the age of 6 years; and described is the development of three male dystrophic siblings. (DB)

  5. Você conhece esta síndrome? Do you know this syndrome?

    Directory of Open Access Journals (Sweden)

    Júnia Bicalho de Sousa

    2006-02-01

    Full Text Available A lipodistrofia generalizada congênita (síndrome de Berardinelli-Seip, doença autossômica recessiva, caracteriza-se por escassez do tecido subcutâneo. A falta de tecido adiposo propicia disfunção metabólica dos lípides e carboidratos, resistência periférica à insulina, hipertrigliceridemia e hipermetabolismo. Outros achados são acantose nigricante, acromegalia, hepatomegalia e alterações musculares, ósseas, cardiovasculares e neurológicas. Relata-se o caso de paciente com essa síndrome, cujo diagnóstico foi realizado em um serviço de dermatologia.Congenital generalized lipodystrophy (Berardinelli-Seip syndrome is a recessive autossomic disease characterized by absence of subcutaneous tissue. The fat tissue absence leads to metabolic dysfunction of lipids and carbohydrates, peripheral insulin resistance and increased seric levels of triglycerides and also a higher metabolic rate. Other findings are acanthosis nigricans, acromegaly, hepatomegaly and muscular, bony, cardiovascular and neurological abnormalities. A case of a patient with this syndrome is reported, whose diagnosis was made in a dermatology ambulatory.

  6. Deficiência muscular de carnitina: relato de 8 casos com estudo clínico, eletromiográfico, histoquímico e bioquímico muscular

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1985-09-01

    Full Text Available São relatados os casos de 8 pacientes, sendo 7 do sexo masculino, cuja idade variou entre 5 dias e 64 anos. Sete pacientes apresentavam diminuição da força muscular e todos apresentavam, nas biópsias musculares, acúmulo de lipídios. Os sintomas iniciaram nos primeiros dias de vida em três pacientes, na infância em dois, na idade adulta em dois; um dos casos apresentava-se assinto-mático aos 64 anos de idade (heterozigoto?. Em graus variáveis os pacientes apresentavam dificuldades na deglutição, hipotonia, atrofia muscular, dificuldades na mastigação, parestesias em membros inferiores, hepatomegalia e esplenome-galia. Cinco casos tinham história familiar e um relatava recorrências dos sintomas. Todos apresentavam aumento dos enzimas séricos, principalmente da creatinoquinase. A eletromiografia foi compatível a envolvimento muscular primário em um caso, desenervação em dois e neuromiopático em dois, não tendo sido realizada em três casos. Na biópsia muscular, em todos os casos, além do acúmulo de lipídios, ocorriam: componente de desenervação em 4, miopa-tia crônica em 4, atrofia de fibras do tipo II em um. Em dois casos, as alterações histológicas eram sugestivas de atrofia espinhal infantil. Um dos casos, possivelmente pertencente à forma sistêmica de deficiência de carnitina, possuía importante envolvimento miocárdico, vindo a falecer. São discutidos aspectos clínicos, metabólicos e terapêuticos das deficiências musculares de carnitina.

  7. [Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy].

    Science.gov (United States)

    Silva, Helga Cristina Almeida da; Hiray, Marcia; Vainzof, Mariz; Schmidt, Beny; Oliveira, Acary Souza Bulle; Amaral, José Luiz Gomes do

    2017-05-31

    Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia. Male patient, 14 years old, referred for hyperCKemia (chronic increase of serum creatine kinase levels - CK), with CK values of 7,779-29,040IU.L -1 (normal 174IU.L -1 ). He presented with a discrete delay in motor milestones acquisition (sitting at 9 months, walking at 18 months). He had a history of liver transplantation. In the neurological examination, the patient showed difficulty in walking on one's heels, myopathic sign (hands supported on the thighs to stand), high arched palate, calf hypertrophy, winged scapulae, global muscle hypotonia and arreflexia. Spirometry showed mild restrictive respiratory insufficiency (forced vital capacity: 77% of predicted). The in vitro muscle contracture test in response to halothane and caffeine was normal. Muscular dystrophy analysis by Western blot showed reduced dystrophin (20% of normal) for both antibodies (C and N-terminal), allowing the diagnosis of Becker muscular dystrophy. On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia. Copyright © 2017 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  8. Muscular Dystrophies at Different Ages: Metabolic and Endocrine Alterations

    Directory of Open Access Journals (Sweden)

    Oriana del Rocío Cruz Guzmán

    2012-01-01

    Full Text Available Common metabolic and endocrine alterations exist across a wide range of muscular dystrophies. Skeletal muscle plays an important role in glucose metabolism and is a major participant in different signaling pathways. Therefore, its damage may lead to different metabolic disruptions. Two of the most important metabolic alterations in muscular dystrophies may be insulin resistance and obesity. However, only insulin resistance has been demonstrated in myotonic dystrophy. In addition, endocrine disturbances such as hypogonadism, low levels of testosterone, and growth hormone have been reported. This eventually will result in consequences such as growth failure and delayed puberty in the case of childhood dystrophies. Other consequences may be reduced male fertility, reduced spermatogenesis, and oligospermia, both in childhood as well as in adult muscular dystrophies. These facts all suggest that there is a need for better comprehension of metabolic and endocrine implications for muscular dystrophies with the purpose of developing improved clinical treatments and/or improvements in the quality of life of patients with dystrophy. Therefore, the aim of this paper is to describe the current knowledge about of metabolic and endocrine alterations in diverse types of dystrophinopathies, which will be divided into two groups: childhood and adult dystrophies which have different age of onset.

  9. X-ray diagnostic sign for the differentiation of neurogenic and primary muscular diseases

    International Nuclear Information System (INIS)

    Palvoelgyi, R.; Gallai, M.

    1981-01-01

    The authors give an account of X-ray examinations of the limb musculature of 70 patients suffering from neurogenic muscular diseases, 42 suffering from primary muscular diseases and 45 suffering from senile degeneration of the muscles. Different degree of damage to different parts of the same muscle could only been observed in one case of neurogenic atrophy (in the postpoliomyelitic states) and in two cases of senile degeneration, while it was found in 11 cases (20%) for the other muscular diseases. In the latter cases the more severe muscle damage, which could be demonstrated radiographically, was always found in the part of the muscle adjacent to a tendon. On the above reasons the authors consider that radiographically demonstrable partial or uneven damage to any particular muscle can be used as a new diagnostical information in distinguishing muscular diseases from neurogenic muscular atrophy. (orig.) [de

  10. Nodular type of muscular sarcoidosis : a case report

    International Nuclear Information System (INIS)

    Chae, Soo Hyun; Kim, Hong Soo; Park, Sook Hee; Kim, Sung Me

    1999-01-01

    Muscular involvement of sarcoidosis is rare and occurs in two forms:nodular and myopathic. In the nodular variety, lesions are long and extend along muscle fibers. Axial MR imaging reveals a star-shaped central structure of decreased signal intensity. Sagittal and coronal MR images show three stripes:an inner stripe of decreased signal intensity and outer stripes of increased signal intensity. Longitudinal sonography shows an echogenic inner stripe and hypoechoic outer stripes. We report a case of nodular- type muscular sarcoidosis in a 53-year-old man, describing the findings of MRI and ultrasonography

  11. Muscular forearm activation in hand-grip tasks with superimposition of mechanical vibrations.

    Science.gov (United States)

    Fattorini, L; Tirabasso, A; Lunghi, A; Di Giovanni, R; Sacco, F; Marchetti, E

    2016-02-01

    The purpose of this paper is to evaluate the muscular activation of the forearm, with or without vibration stimuli at different frequencies while performing a grip tasks of 45s at various level of exerted force. In 16 individuals, 9 females and 7 males, the surface electromyogram (EMG) of extensor carpi radialis longus and the flexor carpi ulnari muscles were assessed. At a short latency from onset EMG, RMS and the level of MU synchronization were assessed to evaluate the muscular adaptations. Whilst a trend of decay of EMG Median frequency (MDFd) was employed as an index of muscular fatigue. Muscular tasks consists of the grip of an instrumented handle at a force level of 20%, 30%, 40%, 60% of the maximum voluntary force. Vibration was supplied by a shaker to the hand in mono-frequential waves at 20, 30, 33 and 40Hz. In relation to EMG, RMS and MU synchronization, the muscular activation does not seem to change with the superimposition of the mechanical vibrations, on the contrary a lower MDFd was observed at 33Hz than in absence of vibration. This suggests an early muscular fatigue induced by vibration due to the fact that 33Hz is a resonance frequency for the hand-arm system. Copyright © 2015 Elsevier Ltd. All rights reserved.

  12. Bortezomib partially improves laminin α2 chain-deficient muscular dystrophy.

    Science.gov (United States)

    Körner, Zandra; Fontes-Oliveira, Cibely C; Holmberg, Johan; Carmignac, Virginie; Durbeej, Madeleine

    2014-05-01

    Congenital muscular dystrophy, caused by mutations in LAMA2 (the gene encoding laminin α2 chain), is a severe and incapacitating disease for which no therapy is yet available. We have recently demonstrated that proteasome activity is increased in laminin α2 chain-deficient muscle and that treatment with the nonpharmaceutical proteasome inhibitor MG-132 reduces muscle pathology in laminin α2 chain-deficient dy(3K)/dy(3K) mice. Here, we explore the use of the selective and therapeutic proteasome inhibitor bortezomib (currently used for treatment of relapsed multiple myeloma and mantle cell lymphoma) in dy(3K)/dy(3K) mice and in congenital muscular dystrophy type 1A muscle cells. Outcome measures included quantitative muscle morphology, gene and miRNA expression analyses, proteasome activity, motor activity, and survival. Bortezomib improved several histological hallmarks of disease, partially normalized miRNA expression (miR-1 and miR-133a), and enhanced body weight, locomotion, and survival of dy(3K)/dy(3K) mice. In addition, bortezomib reduced proteasome activity in congenital muscular dystrophy type 1A myoblasts and myotubes. These findings provide evidence that the proteasome inhibitor bortezomib partially reduces laminin α2 chain-deficient muscular dystrophy. Investigation of the clinical efficacy of bortezomib administration in congenital muscular dystrophy type 1A clinical trials may be warranted. Copyright © 2014 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  13. A preliminary study of muscular artifact cancellation in single-channel EEG.

    Science.gov (United States)

    Chen, Xun; Liu, Aiping; Peng, Hu; Ward, Rabab K

    2014-10-01

    Electroencephalogram (EEG) recordings are often contaminated with muscular artifacts that strongly obscure the EEG signals and complicates their analysis. For the conventional case, where the EEG recordings are obtained simultaneously over many EEG channels, there exists a considerable range of methods for removing muscular artifacts. In recent years, there has been an increasing trend to use EEG information in ambulatory healthcare and related physiological signal monitoring systems. For practical reasons, a single EEG channel system must be used in these situations. Unfortunately, there exist few studies for muscular artifact cancellation in single-channel EEG recordings. To address this issue, in this preliminary study, we propose a simple, yet effective, method to achieve the muscular artifact cancellation for the single-channel EEG case. This method is a combination of the ensemble empirical mode decomposition (EEMD) and the joint blind source separation (JBSS) techniques. We also conduct a study that compares and investigates all possible single-channel solutions and demonstrate the performance of these methods using numerical simulations and real-life applications. The proposed method is shown to significantly outperform all other methods. It can successfully remove muscular artifacts without altering the underlying EEG activity. It is thus a promising tool for use in ambulatory healthcare systems.

  14. Distrofia muscular progressiva: alguns aspectos do diagnõstico diferencial

    Directory of Open Access Journals (Sweden)

    Sylvio Saraiva

    1960-09-01

    Full Text Available The authors call attention to some clinical entities which are less known and more difficult to recognize and with which differential diagnosis of progressive muscular dystrophy should be made (infantile spinal muscular atrophy, amyotonia congenita, congenital acute anterior poliomyelitis, anthro-griposis multiplex, von Gierke's disease, central core disease, chronical polymyositis and dermatomyositis, thyrotoxic myopathy and menopausal dys- trophy. The importance of muscle biopsy in the differential diagnosis is emphasized.

  15. Distrofia muscular congênita e deficiência de merosina Congenital muscular dystrophy and merosin deficiency

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1997-01-01

    Full Text Available Uma proporção variável de pacientes com distrofia muscular congênita (DMC da forma clássica ou ocidental apresenta deficiência da cadeia α2 da merosina, uma proteína da matriz extracelular. Foi realizado estudo das características clínicas, laboratoriais e histopatológicas de 18 pacientes com DMC, relacionadas com o padrão de merosina encontrado na biópsia muscular. Estudo imuno-histoquímico demonstrou que 11 pacientes eram merosina-deficiente (MD e sete pacientes eram merosina-positiva (MP. Nenhum dos nove pacientes MD com idade suficiente para serem avaliados alcançaram a capacidade de deambulação, enquanto quatro dos sete pacientes MP atingiram deambulação sem auxílio. Os níveis de creatinoquinase estavam mais aumentados nos pacientes MD, mas a diferença entre os dois grupos não foi estatisticamente significativa. Estudo da condução nervosa motora foi realizado em 12 pacientes. Todos os quatro pacientes MP apresentaram exames normais, enquanto dois de oito pacientes MD apresentaram diminuição da velocidade de condução nervosa motora. Entre 69 parâmetros de biópsia muscular avaliados, não foi encontrada diferença estatisticamente significativa entre os grupos MP e MD. Esses resultados sugerem que a diferenciação entre os casos MP e MD serve para fins de prognóstico, pois os pacientes MP chegam a deambular. Além disso, este estudo indica que não existe relação entre a ausência de merosina e as alterações histológicas encontradas na biópsia muscular.Merosin α2 chain, an extracellular matrix protein, is deficient in a proportion of patients with classical congenital muscular dystrophy (CMD. A study of clinical, laboratory and histopathological features of 18 patients with CMD was performed in relation to the merosin expression in muscle biopsy. Immunohistochemistry study showed that merosin was deficient in 11 patients and present in 7. None of the 9 merosin-deficient patient: evaluated achieved

  16. Miyoshi-type distal muscular dystrophy. Clinical spectrum in 24 Dutch patients

    NARCIS (Netherlands)

    Linssen, W. H.; Notermans, N. C.; van der Graaf, Y.; Wokke, J. H.; van Doorn, P. A.; Höweler, C. J.; Busch, H. F.; de Jager, A. E.; de Visser, M.

    1997-01-01

    Miyoshi-type distal muscular dystrophy has now been found to be more frequent outside Japan than was previously thought. We studied 24 Dutch patients with Miyoshi-type distal muscular dystrophy and focused on its clinical expression and natural history, muscle CT-scans and muscle biopsy findings.

  17. Miyoshi-type distal muscular dystrophy. Clinical spectrum in 24 Dutch patients

    NARCIS (Netherlands)

    W.H.J.P. Linssen (Wim); N.C. Notermans (Nicolette); Y. van der Graaf (Yolanda); J.H.J. Wokke (John); P.A. van Doorn (Pieter); C.J. Höweler (Chris); H.F.M. Busch (Herman); A.E.J. de Jager (Aeiko); M. de Visser (Marianne)

    1997-01-01

    textabstractMiyoshi-type distal muscular dystrophy has now been found to be more frequent outside Japan than was previously thought. We studied 24 Dutch patients with Miyoshi-type distal muscular dystrophy and focused on its clinical expression and natural history muscle CT-scans and muscle biopsy

  18. Miyoshi-type distal muscular dystrophy - Clinical spectrum in 24 Dutch patients

    NARCIS (Netherlands)

    Linssen, WHJP; Notermans, NC; VanderGraaf, Y; Wokke, JHJ; VanDoorn, PA; Howeler, CJ; Busch, HFM; DeJager, AEJ; DeVisser, M

    1997-01-01

    Miyoshi-type distal muscular dystrophy has now been found to be more frequent outside Japan than was previously thought. We studied 24 Dutch patients with Miyoshi-type distal muscular dystrophy and focused on its clinical expression and natural history, muscle CT-scans and muscle biopsy findings.

  19. Sex differences in muscular load among house painters performing identical work tasks

    DEFF Research Database (Denmark)

    Meyland, Jacob; Heilskov-Hansen, Thomas; Alkjær, Tine

    2014-01-01

    PURPOSE: The present study aimed to estimate possible differences in upper body muscular load between male and female house painters performing identical work tasks. Sex-related differences in muscular load may help explain why women, in general, have more musculoskeletal complaints than men....... METHODS: In a laboratory setting, 16 male and 16 female house painters performed nine standardised work tasks common to house painters. Unilateral electromyography (EMG) recordings were obtained from the supraspinatus muscle by intramuscular electrodes and from the trapezius, extensor and flexor carpi...... radialis muscles by surface electrodes. Relative muscular loads in %EMGmax as well as exerted force in Newton, based on ramp calibrations, were assessed. Sex differences were tested using a mixed model approach. RESULTS: Women worked at about 50% higher relative muscular loads than men in the supraspinatus...

  20. Cardio-Muscular Conditioner

    Science.gov (United States)

    1993-01-01

    In the mid-sixties, Gary Graham, a Boeing designer, developed a cardiovascular conditioner for a planned Air Force orbiting laboratory. After the project was cancelled, Graham participated in space station conditioning studies for the Skylab program. Twenty years later, he used this expertise to develop the Shuttle 2000-1, a physical therapy and athletic development conditioner, available through Contemporary Designs. The machine is used by football teams, sports clinics and medical rehabilitation centers. Cardiovascular fitness and muscular strength development are promoted through both kinetic and plyometric exercises.

  1. Trends with corticosteroid use in males with Duchenne muscular dystrophy born 1982-2001.

    Science.gov (United States)

    Fox, Deborah J; Kumar, Anil; West, Nancy A; DiRienzo, A Gregory; James, Katherine A; Oleszek, Joyce

    2015-01-01

    This study examines trends in corticosteroid use for males with Duchenne muscular dystrophy by birth year, race/ethnicity, and knowledge of Duchenne muscular dystrophy family history. Firstborn males (n = 521) selected from a population-based surveillance system of Duchenne muscular dystrophy were analyzed using Kaplan Meier and regression methods. Comparing males born 1982 to 1986 with males born 1997 to 2001, steroid use increased from 54% to 72% and mean age at steroid initiation decreased from 8.2 to 7.1 years. Hispanics and non-Hispanic Black males used steroids less frequently and delayed initiation compared to white males. Compared to males without a Duchenne muscular dystrophy family history, males with known family history were half as likely to use steroids. Duration of steroid use increased over time and age at initiation decreased. Racial/ethnic disparities exist for steroid use and should be addressed to improve outcome and quality of life for boys with Duchenne muscular dystrophy. © The Author(s) 2014.

  2. Quantitative analysis of muscular wastings of lower limbs in Duchenne muscular dystrophy by computed tomography

    International Nuclear Information System (INIS)

    Horikawa, Hirosei; Konagaya, Masaaki; Takayanagi, Tetsuya; Otsuji, Hideaki

    1985-01-01

    We quantitatively evaluated the muscular wastings of lower extremities in Duchenne muscular dystrophy (DMD) by computed tomography (CT). The subjects were 21 cases of DMD (an ambulant case and 20 wheelchair-ridden cases, ages ranging from 10 to 21 years old) and 4 control males. The CT scan was carried out at the mid-level between lesser trochanter and medial condyle of femur and the largest diameter level of lower leg. The density and the cross-sectional area of each muscle were measured on the CT image. The average CT number of normal muscle was varying from 40 to 60, as well as that of fat was -115. Then we calculated CT index of each muscle denoted as follows: CT index = [average CT number of muscle-(-115)] X(cross-sectional area of each muscle). The measurements of muscle strength and serum CK level were performed and their relationships to CT index were examined. The results were achieved as follows: 1) Wheelchair-ridden cases with DMD showed severe decrease in the average CT number and the CT index of each muscle with normal controls. With progression, the average CT number and the CT index were reduced. But gracilis muscle and sartorius muscle were relatively spared in comparison with other muscles. 2) There was positive correlation between the CT index and the muscle strength in triceps surae muscle, hamstrings muslce and quardriceps femoris muscle. 3) The CT index of whole thigh muscles and that of whole lower leg muscles were highly correlated to serum CK level. These results suggest that the quantitative analysis of muscle CT is an useful measurement for assessement of muscular wastings in DMD. (author)

  3. Distrofia oftalmoplégica progressiva. Forma de inicio ocular precoce e comprometimento muscular universal tardio Progressive ophthalmoplegic dystrophy

    Directory of Open Access Journals (Sweden)

    João P. Rodrigues

    1968-03-01

    Full Text Available É estudada uma forma particular de distrofia muscular progressiva — forma óculo-faríngea — cujo diagnóstico foi comprovado mediante traçados eletromiográficos e biópsia do músculo orbicular da pálpebra. São analisadas as duas formas de distrofias oftalmoplégicas: 1 miopatia ocular; 2 miopatia óculo-faríngea. A forma óculo-faríngea é mais rara, incide em idades mais avançadas (média de 40 anos em comparação com a forma ocular pura (média de 23 anos; além disso, é ressaltada a alta incidência familiar daquela forma de miopatia. O caso ora registrado parece ser um élo entre as duas formas, pois o início foi precoce, sem incidência familiar e com comprometimento da deglutição e de músculos da face, pescoço, tronco, cintura escapular, pélvica e dos membros.A case of peculiar form of progressive muscular dystrophy — the oculopharyngeal one — is reported. The diagnosis was ascertained by electromiography and biopsy of orbicularis palpebrae muscle. Two forms of ophthalmoplegic dystrophy are analysed: 1 ocular myopathy and 2 oculopharyngeal miopathy. The latter is less frequent, occurring on patients in the fourth decade, in comparison with the pure ocular form affecting patients in the second decade. Besides this, the high familial incidence of the oculo-pharyngeal form is stressed. The reported case seems to be an intermediate form between them. The onset occurred at 11 years of age; there was no other case in the family, the disease involving swallowing, face, neck, trunk, shoulder, pelvic and members muscles.

  4. Equiprobable Growth Model and Growth Cycles: An Approximation to Persistence and Volatility

    Directory of Open Access Journals (Sweden)

    Zeus Salvador Hernández Veleros

    2008-01-01

    Full Text Available Este artículo presenta una aproximación novedosa al estudio de los ciclos de crecimiento, para lo cual se emplea la distribucion de probabilidad multinomial y el denominado modelo de crecimiento equiprobable. El estudio considera información de 142 economías durante el periodo 1950-2000, las cuales son agrupadas en siete clusters. El análisis planteado define tres regímenes de crecimiento y sus características (duración, volatilidad y correlación entre tales regímenes. A partir del ciclo de crecimiento estimado para cada cluster se obtiene una tendencia de largo plazo mediante el uso del valor esperado. Las hipótesis del artículo son examinadas mediante bootstrap.

  5. Muscular strength is associated with self-esteem in college men but not women.

    Science.gov (United States)

    Ciccolo, Joseph T; SantaBarbara, Nicholas J; Dunsiger, Shira I; Busch, Andrew M; Bartholomew, John B

    2016-12-01

    Muscular strength is a well-known predictor of morbidity and mortality. Similarly, self-esteem is a predictor of health and well-being. The relationship between these two variables, however, is currently unknown. This study examined the cross-sectional relationship between maximal muscular strength (i.e. handgrip and one-repetition-maximum (1-RM) squat) and global self-esteem in 126 college students. Significant correlations were found between both measures of muscular strength and self-esteem. Further analyses revealed that these relationships were only significant for men. Based on these results, additional research is needed to further explore the relationship between muscular strength and self-esteem, especially in other demographic groups and longitudinally. © The Author(s) 2015.

  6. Autonomic Dysfunction in Muscular Dystrophy: A Theoretical Framework for Muscle Reflex Involvement

    Directory of Open Access Journals (Sweden)

    Scott Alan Smith

    2014-02-01

    Full Text Available Muscular dystrophies are a heterogeneous group of genetically inherited disorders whose most prominent clinical feature is progressive degeneration of skeletal muscle. In several forms of the disease, the function of cardiac muscle is likewise affected. The primary defect in this group of diseases is caused by mutations in myocyte proteins important to cellular structure and/or performance. That being stated, a growing body of evidence suggests that the development of autonomic dysfunction may secondarily contribute to the generation of skeletal and cardio-myopathy in muscular dystrophy. Indeed, abnormalities in the regulation of both sympathetic and parasympathetic nerve activity have been reported in a number of muscular dystrophy variants. However, the mechanisms mediating this autonomic dysfunction remain relatively unknown. An autonomic reflex originating in skeletal muscle, the exercise pressor reflex, is known to contribute significantly to the control of sympathetic and parasympathetic activity when stimulated. Given the skeletal myopathy that develops with muscular dystrophy, it is logical to suggest that the function of this reflex might also be abnormal with the pathogenesis of disease. As such, it may contribute to or exacerbate the autonomic dysfunction that manifests. This possibility along with a basic description of exercise pressor reflex function in health and disease are reviewed. A better understanding of the mechanisms that possibly underlie autonomic dysfunction in muscular dystrophy may not only facilitate further research but could also lead to the identification of new therapeutic targets for the treatment of muscular dystrophy.

  7. Intraoperative closure of infant multiple muscular ventricular septal defects with Amplatzer occluder

    International Nuclear Information System (INIS)

    Liu Jinfen; Gao Wei; Zhu Zhongqun; Chen Huiwen; Zhang Yuqi

    2005-01-01

    Objective: To report the preliminary experience of intraoperative hybrid therapy for closure of multiple muscular ventricular septal defects (VSD) in a small infant. Methods: After median sternotomy, a AGA Amplatzer occluder was introduced through right ventricular surface to close 2 muscular ventricular septal defects under transesophageal echocardiographic guidance. Results: The infant survived after the treatment without residual shunting, and rehabilitated rapidly. Conclusions: Intraoperative hybrid therapy with combined surgical technique and interventional procedure for closure of multiple muscular VSD in small infant is a safe and effective method. (authors)

  8. Análise da expressão do colágeno VI na distrofia muscular congênita Analysis of the expression of collagen VI in congenital muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Regina Toni Loureiro de Freitas

    2005-06-01

    Full Text Available A distrofia muscular congênita (DMC compõe um grupo de miopatias caracterizadas por hipotonia e fraqueza muscular notadas já no primeiro ano de vida. A forma de Ullrich é caracterizada por retrações musculares proximais e hiperextensibilidade distal. Cerca de 40% destes pacientes apresentam mutações em um dos genes que codificam as três sub-unidades do colágeno VI (COL6, acarretando deficiência total ou parcial na marcação da proteína. Analisamos, através de imunofluorescência, a marcação do COL6 em fragmentos musculares de 50 pacientes com DMC, 20 deles com ausência da marcação para merosina. Identificamos 4 casos com deficiência total da marcação do COL6 (8% do total, representando 13% dos casos com marcação normal para merosina. As alterações histológicas musculares dos pacientes com COL6 deficiente eram indistinguíveis das outras formas de DMC, porém mais brandas que as observadas na DMC com deficiência de merosina. Em três dos pacientes com COL6 deficiente observou-se hipotonia e fraqueza muscular, notadas já no período neonatal, atraso do desenvolvimento motor, retrações musculares em joelhos e cotovelos, hiperextensibilidade distal e luxação congênita do quadril (dois pacientes. Um paciente perdeu a capacidade para a marcha, e outro faleceu por problemas respiratórios. A análise da marcação do COL6, assim como da merosina, no tecido muscular de pacientes com DMC pode auxiliar na identificação e caracterização fenotípica dos diversos subtipos de DMC.Congenital muscular dystrophy (CMD composes a group of disorders characterized by hypotonia and muscular weakness noticed in the first year of life. The Ullrich's form is characterized by proximal joint contractures and distal hiperextensibility. About 40% of these patients present mutations in one of the genes that codify the sub-units of the collagen VI protein (COL6, producing total or partial deficiency of the protein expression. We analyzed

  9. MR and ultrasound findings in a case of cerebro-oculo-muscular-syndrome

    International Nuclear Information System (INIS)

    Simma, B.; Maurer, H.; Gassner, I.; Krassnitzer, S.; Felber, S.

    1990-01-01

    We report on a boy with type II lissencephaly and congenital muscular dystrophy. The patient presented with the features of a cerebro-oculo-muscular-syndrome (COMS). We describe the clinical presentations and the characteristic sonographic and MR findings. (orig.)

  10. Intramuscular renin-angiotensin system is activated in human muscular dystrophy.

    Science.gov (United States)

    Sun, Guilian; Haginoya, Kazuhiro; Dai, Hongmei; Chiba, Yoko; Uematsu, Mitsugu; Hino-Fukuyo, Naomi; Onuma, Akira; Iinuma, Kazuie; Tsuchiya, Shigeru

    2009-05-15

    To investigate the role of the muscular renin-angiotensin system (RAS) in human muscular dystrophy, we used immunohistochemistry and Western blotting to examine the cellular localization of angiotensin-converting enzyme (ACE), the angiotensin II type 1 receptor (AT1) and the angiotensin II type 2 receptor (AT2) in muscle biopsies from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and congenital muscular dystrophy (CMD). In normal muscle, ACE was expressed in vascular endothelial cells and neuromuscular junctions (NMJs), whereas AT1 was immunolocalized to the smooth muscle cells of blood vessels and intramuscular nerve twigs. AT2 was immunolocalized in the smooth muscle cells of blood vessels. These findings suggest that the RAS has a functional role in peripheral nerves and NMJs. ACE and AT1, but AT2 immunoreactivity were increased markedly in dystrophic muscle as compared to controls. ACE and the AT1 were strongly expressed in the cytoplasm and nuclei of regenerating muscle fibers, fibroblasts, and in macrophages infiltrating necrotic fibers. Double immunolabeling revealed that activated fibroblasts in the endomysium and perimysium of DMD and CMD muscle were positive for ACE and AT1. Triple immunolabeling demonstrated that transforming growth factor-beta1 (TGF-beta1) and ACE were colocalized on the cytoplasm of activated fibroblasts in dystrophic muscle. Furthermore, Western blotting showed increases in the expression of AT1 and TGF-beta1 protein in dystrophic muscle, which coincided with our immunohistochemical results. The overexpression of ACE and AT1 in dystrophic muscle would likely result in the increased production of Ang II, which may act on these cells in an autocrine manner via AT1. The activation of AT1 may induce fibrous tissue formation through overexpression of TGF-beta1, which potently activates fibrogenesis and suppresses regeneration. In conclusion, our results imply that the intramuscular RAS-TGF-beta1 pathway

  11. Nuevo dispositivo para el aislamiento de la fracción estromal vascular a partir de lipoaspirados humanos: método de obtención y análisis de calidad celular

    Directory of Open Access Journals (Sweden)

    S. Dos-Anjos

    2013-12-01

    Full Text Available El tejido adiposo obtenido mediante liposucción es una fuente idónea para aislar células con potencial terapéutico, las denominadas células de la fracción estromal vascular (FEV, que incluyen células madre mesenquimales. Estas células se han convertido en una de las principales herramientas de terapia celular autóloga para diversas aplicaciones médicas, y en los últimos años se han ido desarrollando diversas tecnologías para su aislamiento y uso clínico. En este trabajo presentamos un nuevo método rápido, sencillo y eficiente para el aislamiento de células de la FEV mediante un dispositivo médico cerrado que permite recoger y procesar lipoaspirados humanos en el mismo procedimiento quirúrgico de manera coste-efectiva. Además describimos los métodos llevados a cabo para cuantificar la calidad, seguridad y eficacia del inóculo celular obtenido.

  12. Hereditary muscular dystrophies and the heart

    NARCIS (Netherlands)

    Hermans, M. C. E.; Pinto, Y. M.; Merkies, I. S. J.; de Die-Smulders, C. E. M.; Crijns, H. J. G. M.; Faber, C. G.

    2010-01-01

    Cardiac disease is a common clinical manifestation of neuromuscular disorders, particularly of muscular dystrophies. Heart muscle cells as well as specialized conducting myocardial fibres may be affected by the dystrophic process. The incidence and nature of cardiac involvement vary with different

  13. Effect of sildenafil on skeletal and cardiac muscle in Becker muscular dystrophy

    DEFF Research Database (Denmark)

    Witting, Nanna; Kruuse, Christina; Nyhuus, Bo

    2014-01-01

    OBJECTIVE: Patients with Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy lack neuronal nitric oxide synthase (nNOS). nNOS mediates physiological sympatholysis, thus ensuring adequate blood supply to working muscle. In mice lacking dystrophin, restoration of nNOS effects...

  14. Investigation of Poor Academic Achievement in Children with Duchenne Muscular Dystrophy

    Science.gov (United States)

    Hinton, V. J.; De Vivo, D. C.; Fee, R.; Goldstein, E.; Stern, Y.

    2004-01-01

    Duchenne Muscular Dystrophy (DMD) is a neurogenetic developmental disorder that presents with progressive muscular weakness. It is caused by a mutation in a gene that results in the absence of specific products that normally localize to muscle cells and the central nervous system (CNS). The majority of affected individuals have IQs within the…

  15. Meeting the Assistive Technology Needs of Students with Duchenne Muscular Dystrophy

    Science.gov (United States)

    Heller, Kathryn Wolff; Mezei, Peter J.; Avant, Mary Jane Thompson

    2009-01-01

    Students with Duchenne muscular dystrophy (DMD) have a degenerative disease that requires ongoing changes in assistive technology (AT). The AT team needs to be knowledgeable about the disease and its progression in order to meet these students' changing needs in a timely manner. The unique needs of students with Duchenne muscular dystrophy in…

  16. Dystrophin Immunity in Duchenne’s Muscular Dystrophy

    Science.gov (United States)

    Mendell, Jerry R.; Campbell, Katherine; Rodino-Klapac, Louise; Sahenk, Zarife; Shilling, Chris; Lewis, Sarah; Bowles, Dawn; Gray, Steven; Li, Chengwen; Galloway, Gloria; Malik, Vinod; Coley, Brian; Clark, K. Reed; Li, Juan; Xiao, Xiao; Samulski, Jade; McPhee, Scott W.; Samulski, R. Jude; Walker, Christopher M.

    2010-01-01

    SUMMARY We report on delivery of a functional dystrophin transgene to skeletal muscle in six patients with Duchenne’s muscular dystrophy. Dystrophin-specific T cells were detected after treatment, providing evidence of transgene expression even when the functional protein was not visualized in skeletal muscle. Circulating dystrophin-specific T cells were unexpectedly detected in two patients before vector treatment. Revertant dystrophin fibers, which expressed functional, truncated dystrophin from the deleted endogenous gene after spontaneous in-frame splicing, contained epitopes targeted by the autoreactive T cells. The potential for T-cell immunity to self and nonself dystrophin epitopes should be considered in designing and monitoring experimental therapies for this disease. (Funded by the Muscular Dystrophy Association and others; ClinicalTrials.gov number, NCT00428935.) PMID:20925545

  17. Treatment of muscular rheumatism with 99Tc-MDP (one case report)

    International Nuclear Information System (INIS)

    Wang Jincheng

    2001-01-01

    Objective: To observe the value of using Yun Ke ( 99 Tc-MDP) to cure muscular rheumatism. Methods: A 10 years old male patient was diagnosed with muscular rheumatism. His symptoms were wandering and muscular pain and tenderness at lumbosacral region, abdomen and double crus. He was given therapy with Yun Ke everyday, intravenous injection of 5 mg for 30 days. Results: After intravenous injection of Yun Ke within 8 to 72 hours, the pain in the left crus, the lumbosacral portion and the right crus was reduced in respective order. Finally, the pain disappeared, and only the abdominal pain remained. Between 6 and 30 days, the area of abdominal pain reduced little by little to the size of 1.0 x 1.0 cm 2 , which was beside the navel. On the 33 rd day after the initial treatment, we injected him in the previous mentioned area with prednisone and the pain disappeared. Conclusion: Yun Ke ( 99 Tc-MDP) is a new medicine for the treatment of rheumatism, and we believe muscular rheumatism is a new indication of Yun Ke

  18. Zebrafish models flex their muscles to shed light on muscular dystrophies.

    Science.gov (United States)

    Berger, Joachim; Currie, Peter D

    2012-11-01

    Muscular dystrophies are a group of genetic disorders that specifically affect skeletal muscle and are characterized by progressive muscle degeneration and weakening. To develop therapies and treatments for these diseases, a better understanding of the molecular basis of muscular dystrophies is required. Thus, identification of causative genes mutated in specific disorders and the study of relevant animal models are imperative. Zebrafish genetic models of human muscle disorders often closely resemble disease pathogenesis, and the optical clarity of zebrafish embryos and larvae enables visualization of dynamic molecular processes in vivo. As an adjunct tool, morpholino studies provide insight into the molecular function of genes and allow rapid assessment of candidate genes for human muscular dystrophies. This unique set of attributes makes the zebrafish model system particularly valuable for the study of muscle diseases. This review discusses how recent research using zebrafish has shed light on the pathological basis of muscular dystrophies, with particular focus on the muscle cell membrane and the linkage between the myofibre cytoskeleton and the extracellular matrix.

  19. SIRT1: A Novel Target for the Treatment of Muscular Dystrophies

    Directory of Open Access Journals (Sweden)

    Atsushi Kuno

    2016-01-01

    Full Text Available Muscular dystrophies are inherited myogenic disorders accompanied by progressive skeletal muscle weakness and degeneration. Duchenne muscular dystrophy (DMD is the most common and severe form of muscular dystrophy and is caused by mutations in the gene that encodes the cytoskeletal protein dystrophin. The treatment for DMD is limited to glucocorticoids, which are associated with multiple side effects. Thus, the identification of novel therapeutic targets is urgently needed. SIRT1 is an NAD+-dependent histone/protein deacetylase that plays roles in diverse cellular processes, including stress resistance and cell survival. Studies have shown that SIRT1 activation provides beneficial effects in the dystrophin-deficient mdx mouse, a model of DMD. SIRT1 activation leads to the attenuation of oxidative stress and inflammation, a shift from the fast to slow myofiber phenotype, and the suppression of tissue fibrosis. Although further research is needed to clarify the molecular mechanisms underlying the protective role of SIRT1 in mdx mice, we propose SIRT1 as a novel therapeutic target for patients with muscular dystrophies.

  20. Rimmed vacuoles in Becker muscular dystrophy have similar features with inclusion myopathies.

    Science.gov (United States)

    Momma, Kazunari; Noguchi, Satoru; Malicdan, May Christine V; Hayashi, Yukiko K; Minami, Narihiro; Kamakura, Keiko; Nonaka, Ikuya; Nishino, Ichizo

    2012-01-01

    Rimmed vacuoles in myofibers are thought to be due to the accumulation of autophagic vacuoles, and can be characteristic in certain myopathies with protein inclusions in myofibers. In this study, we performed a detailed clinical, molecular, and pathological characterization of Becker muscular dystrophy patients who have rimmed vacuoles in muscles. Among 65 Becker muscular dystrophy patients, we identified 12 patients who have rimmed vacuoles and 11 patients who have deletions in exons 45-48 in DMD gene. All patients having rimmed vacuoles showed milder clinical features compared to those without rimmed vacuoles. Interestingly, the rimmed vacuoles in Becker muscular dystrophy muscles seem to represent autophagic vacuoles and are also associated with polyubiquitinated protein aggregates. These findings support the notion that rimmed vacuoles can appear in Becker muscular dystrophy, and may be related to the chronic changes in muscle pathology induced by certain mutations in the DMD gene.

  1. Rimmed vacuoles in Becker muscular dystrophy have similar features with inclusion myopathies.

    Directory of Open Access Journals (Sweden)

    Kazunari Momma

    Full Text Available Rimmed vacuoles in myofibers are thought to be due to the accumulation of autophagic vacuoles, and can be characteristic in certain myopathies with protein inclusions in myofibers. In this study, we performed a detailed clinical, molecular, and pathological characterization of Becker muscular dystrophy patients who have rimmed vacuoles in muscles. Among 65 Becker muscular dystrophy patients, we identified 12 patients who have rimmed vacuoles and 11 patients who have deletions in exons 45-48 in DMD gene. All patients having rimmed vacuoles showed milder clinical features compared to those without rimmed vacuoles. Interestingly, the rimmed vacuoles in Becker muscular dystrophy muscles seem to represent autophagic vacuoles and are also associated with polyubiquitinated protein aggregates. These findings support the notion that rimmed vacuoles can appear in Becker muscular dystrophy, and may be related to the chronic changes in muscle pathology induced by certain mutations in the DMD gene.

  2. Brain Function in Duchenne Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    J. Gordon Millichap

    2002-02-01

    Full Text Available The role of dystrophin disorders in the CNS function of boys with Duchenne muscular dystrophy (DMD and the dystrophin-deficient mdx mouse, an animal model of DMD, is reviewed at the University of New South Wales, University of Sydney, Australia.

  3. Serum Creatinine Level: A Supplemental Index to Distinguish Duchenne Muscular Dystrophy from Becker Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Huili Zhang

    2015-01-01

    Full Text Available Background. To improve assessment of dystrophinopathy, the aim of this study was to identify whether serum creatinine (Crn level reflects disease severity. Methods. Biochemical, Vignos score, and genetic data were collected on 212 boys with dystrophinopathy. Results. Serum Crn level had a strong inverse correlation with Vignos score by simple correlation (r=-0.793 and partial correlation analysis after adjustment for age, height, and weight (r=-0.791; both P<0.01. Serum Crn level was significantly higher in patients with in-frame than out-of-frame mutations (Z=-4.716, P<0.01 and in Becker muscular dystrophy (BMD patients than Duchenne muscular dystrophy (DMD patients at ages 4, 5, 7, and 9 yr (all P<0.0125. After adjusting for age, height, and weight, BMD patients still had a significantly higher serum Crn level than DMD patients (β=7.140, t=6.277, P<0.01. Conclusions. Serum Crn level reflected disease severity and may serve as a supplemental index to distinguish DMD from BMD in clinical practice.

  4. Insuficiência cardíaca congestiva na distrofia muscular de Duchenne: um relato de caso

    Directory of Open Access Journals (Sweden)

    Paula de Souza Dias Lopes

    2012-07-01

    Full Text Available A distrofia muscular de Duchenne é uma doença neuromuscular, hereditária, recessiva, causada por mutações no gene da distrofina. Clinicamente, caracteriza-se por fraqueza muscular progressiva e simétrica. O diagnóstico é feito pela biópsia muscular com imuno-histoquímica e o tratamento tem a finalidade de retardar a progressão da disfunção muscular, já que uma causa frequente de óbito é a insuficiência cardiorrespiratória.

  5. O retardo mental na distrofia muscular de Duchenne

    Directory of Open Access Journals (Sweden)

    Flávia Nardes

    2012-02-01

    Full Text Available OBJETIVO: Fazer um levantamento da literatura médica destinada ao estudo das disfunções cognitivas nos pacientes com distrofia muscular de Duchenne, através da descrição dos marcos do desenvolvimento neuropsicomotor e dos testes psicométricos para quantificação da inteligência. FONTES DOS DADOS: Revisão não sistemática sobre os aspectos da cognição na distrofia muscular de Duchenne nas principais bases médicas científicas: MEDLINE, LILACS, Biblioteca Cochrane e SciELO. SÍNTESE DOS DADOS: Os pacientes com distrofia muscular de Duchenne apresentaram atraso para marcha e desenvolvimento da linguagem, os quais se correlacionaram a menores pontuações nos testes de inteligência no futuro. Há marcante disfunção nos subtestes das habilidades verbais. CONCLUSÕES: A média do coeficiente de inteligência encontra-se com um desvio padrão abaixo da média populacional. Quanto maior a disfunção cognitiva, piores serão os aspectos relacionados à morbidade e mortalidade na doença.

  6. ATROFIA MUSCULAR ESPINAL: MANEJO RESPIRATORIO EN LA PERSPECTIVA DE LOS RECIENTES AVANCES TERAPÉUTICOS

    Directory of Open Access Journals (Sweden)

    Dra. María Angélica Palomino

    2017-01-01

    Full Text Available La atrofia muscular espinal (AME es la primera causa genética de mortalidad en lactantes. La severidad de las manifestaciones clínicas es un continuo, con tres principales subtipos en pediatría: AME1 que se presenta en recién nacidos y no logran sentarse, AME2 en niños que no logran caminar pero sí sentarse y AME3 que logran caminar. La complicación más seria es la insuficiencia respiratoria. El enfoque del manejo respiratorio es preventivo, con toma de decisiones anticipadas por parte de los cuidadores, que incluye optimizar el manejo de la tos, evitar la deformación de la caja torácica y permitir un desarrollo pulmonar adecuado, tratar la hipoventilación, manejar oportunamente las infecciones respiratorias, el trastorno de deglución, el reflujo gastroesofágico y la malnutrición. A las puertas del desarrollo de tratamientos específicos modificadores de la enfermedad, mediante oligonucleótidos antisentido o vectores genéticos entre otros, los cuidados en AME con enfoque multidisciplinario nos imponen nuevos desafíos donde los cuidados respiratorios deberían estar de acuerdo a lo sugerido en consensos de estándar de manejo, optimizando sus condiciones globales a la espera de tratamientos más específicos.

  7. Estimulação elétrica neuromuscular de média freqüência (russa em cães com atrofia muscular induzida Medium frequency neuromuscular electrical stimulation (russian in dogs with induced muscle atrophy

    Directory of Open Access Journals (Sweden)

    Charles Pelizzari

    2008-06-01

    Full Text Available A estimulação elétrica neuromuscular (EENM de média freqüência (Russa ou de Kotz pode ser empregada para a recuperação de massa muscular em animais apresentando atrofia muscular por desuso. Assim, o objetivo deste trabalho foi empregar a EENM de média freqüência no quadríceps femoral de cães com atrofia muscular induzida, avaliando-se a ocorrência de ganho de massa. Foram utilizados oito cães em dois grupos denominados de GI ou controle e de GII ou tratado. Para a indução da atrofia muscular, a articulação fêmoro-tíbio-patelar esquerda foi imobilizada por 30 dias. Após 48 horas da remoção, foi realizada a EENM nos cães do grupo II, três vezes por semana, com intervalo de 48 horas cada sessão, pelo período de 60 dias. Foram avaliadas a mensuração da perimetria da coxa, da goniometria do joelho, as enzimas creatina-quinase (CK e morfometria das fibras musculares em cortes transversais do músculo vasto lateral, colhido mediante a biópsia muscular. A EENM foi empregada no músculo quadríceps femoral numa freqüência de 2.500Hz, largura de pulso de 50% e relação de tempo on/off de 1:2. Não houve diferença significativa quanto aos valores de perimetria da coxa e a atividade da enzima CK entre os grupos I e II. Na goniometria, houve diminuição significativa (PThe medium frequency neuromuscular electrical stimulation (NMES (Russa or Kotz is designed for recuperation of muscle mass in dogs with muscular atrophy in disuse. This study aims to utilize medium frequency NMES on the femoral quadriceps of dogs with induced muscular atrophy and evaluate the occurrence of gain in mass. Eight dogs in two groups denominated GI, or control, and GII, or treated were used. For the induction of muscular atrophy, the left femoral-tibial-patellar joint was immobilized for 30 days. NMES treatment began 48 hours after the removal of the immobilization device on dogs from group II and was carried out three times per week, with an

  8. Duchenne and Becker muscular dystrophy in adolescents: current perspectives

    Directory of Open Access Journals (Sweden)

    Andrews JG

    2018-03-01

    Full Text Available Jennifer G Andrews, Richard A Wahl Department of Pediatrics, University of Arizona, Tucson, AZ, USA Abstract: Duchenne muscular dystrophy (DMD and Becker muscular dystrophy (BMD are life-limiting and progressive neuromuscular conditions with significant comorbidities, many of which manifest during adolescence. BMD is a milder presentation of the condition and much less prevalent than DMD, making it less represented in the literature, or more severely affected individuals with BMD may be subsumed into the DMD population using clinical cutoffs. Numerous consensus documents have been published on the clinical management of DMD, the most recent of which was released in 2010. The advent of these clinical management consensus papers, particularly respiratory care, has significantly increased the life span for these individuals, and the adolescent years are now a point of transition into adult lives, rather than a period of end of life. This review outlines the literature on DMD and BMD during adolescence, focusing on clinical presentation during adolescence, impact of living with a chronic illness on adolescents, and the effect that adolescents have on their chronic illness. In addition, we describe the role that palliative-care specialists could have in improving outcomes for these individuals. The increasing proportion of individuals with DMD and BMD living into adulthood underscores the need for more research into interventions and intracacies of adolescence that can improve the social aspects of their lives. Keywords: adolescent health, review, Duchenne muscular dystrophy, Becker muscular dystrophy, dystrophinopathy, palliative care

  9. Proximal spinal muscular atrophy: current orthopedic perspective

    Directory of Open Access Journals (Sweden)

    Haaker G

    2013-11-01

    Full Text Available Gerrit Haaker, Albert Fujak Department of Orthopaedic Surgery, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany Abstract: Spinal muscular atrophy (SMA is a hereditary neuromuscular disease of lower motor neurons that is caused by a defective "survival motor neuron" (SMN protein that is mainly associated with proximal progressive muscle weakness and atrophy. Although SMA involves a wide range of disease severity and a high mortality and morbidity rate, recent advances in multidisciplinary supportive care have enhanced quality of life and life expectancy. Active research for possible treatment options has become possible since the disease-causing gene defect was identified in 1995. Nevertheless, a causal therapy is not available at present, and therapeutic management of SMA remains challenging; the prolonged survival is increasing, especially orthopedic, respiratory and nutritive problems. This review focuses on orthopedic management of the disease, with discussion of key aspects that include scoliosis, muscular contractures, hip joint disorders, fractures, technical devices, and a comparative approach of conservative and surgical treatment. Also emphasized are associated complications including respiratory involvement, perioperative care and anesthesia, nutrition problems, and rehabilitation. The SMA disease course can be greatly improved with adequate therapy with established orthopedic procedures in a multidisciplinary therapeutic approach. Keywords: spinal muscular atrophy, scoliosis, contractures, fractures, lung function, treatment, rehabilitation, surgery, ventilation, nutrition, perioperative management

  10. Management of myocardial damage in muscular dystrophy

    International Nuclear Information System (INIS)

    Tamura, Takuhisa

    2011-01-01

    Heart failure (HF) is a fatal complication in many muscular dystrophy cases and has become the most common cause of death in Duchenne muscular dystrophy (DMD) since 2001. HF deaths in DMD occur in young patients and increase, along with respiratory failure, in older patients. Managing HF, therefore, is the most important component of DMD treatment. Management of HF is necessary in DMD patients of all ages because myocardial damage progresses regardless of age and disability. Electrocardiography, echocardiography, myocardial single-photon emission computed tomography (SPECT), and natriuretic peptides are used for the diagnosis of myocardial damage and chronic HF. Tissue Doppler echocardiography is in particularly useful for early detection of minute myocardial damage and dysfunction in DMD. The first-line drugs for chronic HF are angiotensin-converting enzyme inhibitors, and the prognosis of DMD patients has been improved using these drugs and beta-blockers. Diuretics are added in the presence of pulmonary congestion. Digoxin is most effective at a blood level of 0.5-0.8 ng/mL because of its pharmacokinetics in DMD. Surgical treatment may be necessary in cases of intractable HF. Cardiac resynchronization therapy (biventricular pacing), a treatment with an artificial pacemaker, is indicated for cases that meet specific criteria, including HF with ventricular dyssynchrony. Applications of partial left ventriculectomy (Batista procedure) and left ventricular assist devices in muscular dystrophy are likely in the near future. (author)

  11. Case of early pelviolumeral progressive muscular dystrophy associated with marked heart affection

    International Nuclear Information System (INIS)

    Gor'kova, N.B.; Starykh, L.M.; Karpova, L.E.

    1991-01-01

    A case of early pelviolumeral progressive muscular dystrophy detected in childhood and associated with marked heart affection is described. Patient underwent multimodality examination, including ECG, ultrasonography, roentgenography. It is shown that patients with progressive muscular dystrophy should receive medical supervision and treatment of both neuropathologist and therapist

  12. Atrofia muscular espinal tipo 1: enfermedad de Werdnig-Hoffmann

    OpenAIRE

    Zárate-Aspiros, Romeo; Rosas-Sumano, Ana Beatriz; Paz-Pacheco, Alberto; Fenton-Navarro, Patricia; Chinas-López, Silvet; López-Ríos, José Antonio

    2013-01-01

    Introducción. Las atrofias musculares espinales de la infancia son enfermedades neuromusculares hereditarias, autosómicas, recesivas, caracterizadas por la degeneración de las neuronas motoras del asta anterior de la médula espinal. La atrofia muscular espinal tipo I (enfermedad de Werdnig-Hoffmann) es la forma más severa. Se inicia in útero o durante los primeros meses de vida. La muerte suele ocurrir antes de los dos años de edad. Caso clínico. Lactante de 6 meses de edad que ingresa al Ser...

  13. Magnetic resonance imaging of children with Duchenne muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Schreiber, A.; Smith, W.L.; Franken, E.A.; Dunn, V.; Ehrhardt, J.; Ionasescu, V.; Zellweger, H.

    1987-10-01

    Eight children representing a spectrum of clinical states of biopsy-proven Duchenne muscular dystrophy (DMD) underwent magnetic resonance (MR) scans to assess the degree of muscular involvement and disease progression. Five muscle groups (neck, shoulder girdle, pelvic girdle, thigh and calf) were evaluated. In each case, involved muscles were clearly demarcated. Image estimates of disease severity by degree of muscle involvement correlated well with clinical staging. In our experience MR is useful for assessment of disease stage, selection of appropriate muscles for biopsy and planning for courses of physical and rehabilitation therapy.

  14. Magnetic resonance imaging of children with Duchenne muscular dystrophy

    International Nuclear Information System (INIS)

    Schreiber, A.; Smith, W.L.; Franken, E.A.; Dunn, V.; Ehrhardt, J.; Ionasescu, V.; Zellweger, H.

    1987-01-01

    Eight children representing a spectrum of clinical states of biopsy-proven Duchenne muscular dystrophy (DMD) underwent magnetic resonance (MR) scans to assess the degree of muscular involvement and disease progression. Five muscle groups (neck, shoulder girdle, pelvic girdle, thigh and calf) were evaluated. In each case, involved muscles were clearly demarcated. Image estimates of disease severity by degree of muscle involvement correlated well with clinical staging. In our experience MR is useful for assessment of disease stage, selection of appropriate muscles for biopsy and planning for courses of physical and rehabilitation therapy. (orig.)

  15. Creatine kinase response to high-intensity aerobic exercise in adult-onset muscular dystrophy

    DEFF Research Database (Denmark)

    Andersen, Søren P; Sveen, Marie-Louise; Hansen, Regitze S

    2013-01-01

    We investigated the effect of high-intensity exercise on plasma creatine kinase (CK) in patients with muscular dystrophies.......We investigated the effect of high-intensity exercise on plasma creatine kinase (CK) in patients with muscular dystrophies....

  16. Muscle-Derived Proteins as Serum Biomarkers for Monitoring Disease Progression in Three Forms of Muscular Dystrophy

    Science.gov (United States)

    Burch, Peter M.; Pogoryelova, Oksana; Goldstein, Richard; Bennett, Donald; Guglieri, Michela; Straub, Volker; Bushby, Kate; Lochmüller, Hanns; Morris, Carl

    2015-01-01

    Abstract Background: Identifying translatable, non-invasive biomarkers of muscular dystrophy that better reflect the disease pathology than those currently available would aid the development of new therapies, the monitoring of disease progression and the response to therapy. Objective: The goal of this study was to evaluate a panel of serum protein biomarkers with the potential to specifically detect skeletal muscle injury. Method: Serum concentrations of skeletal troponin I (sTnI), myosin light chain 3 (Myl3), fatty acid binding protein 3 (FABP3) and muscle-type creatine kinase (CKM) proteins were measured in 74 Duchenne muscular dystrophy (DMD), 38 Becker muscular dystrophy (BMD) and 49 Limb-girdle muscular dystrophy type 2B (LGMD2B) patients and 32 healthy controls. Results: All four proteins were significantly elevated in the serum of these three muscular dystrophy patient populations when compared to healthy controls, but, interestingly, displayed different profiles depending on the type of muscular dystrophy. Additionally, the effects of patient age, ambulatory status, cardiac function and treatment status on the serum concentrations of the proteins were investigated. Statistical analysis revealed correlations between the serum concentrations and certain clinical endpoints including forced vital capacity in DMD patients and the time to walk ten meters in LGMD2B patients. Serum concentrations of these proteins were also elevated in two preclinical models of muscular dystrophy, the mdx mouse and the golden-retriever muscular dystrophy dog. Conclusions: These proteins, therefore, are potential muscular dystrophy biomarkers for monitoring disease progression and therapeutic response in both preclinical and clinical studies. PMID:26870665

  17. THE INFLUENCE OF HANDEDNESS ON THE DISTRIBUTION OF MUSCULAR WEAKNESS OF THE ARM IN FACIOSCAPULOHUMERAL MUSCULAR-DYSTROPHY

    NARCIS (Netherlands)

    BROUWER, OF; PADBERG, GW; VANDERPLOEG, RJO; RUYS, CJM; BRAND, R

    1992-01-01

    The strength of 10 muscle groups in both arms was measured using hand-held myometry to determine the influence of handedness on left-right differences of muscle strength in facioscapulohumeral muscular dystrophy (FSHD). Two groups of subjects were studied: 24 healthy volunteers (19 right-handed),

  18. How Do People Cope with Muscular Dystrophy?

    Science.gov (United States)

    ... topic are answered in this section. How do people cope with muscular dystrophy (MD)? Although MD presents ... improve health and quality of life. Almost all people with any form of MD experience a worsening ...

  19. [Muscular disorders associated with ankylosing spondylitis and their correction with the help of whole body cryotherapy].

    Science.gov (United States)

    Kulikov, A G; Tabiev, V I; Rassulova, M A

    2015-01-01

    The objective of the present study was to evaluate the possibilities for the correction of muscular disorders associated with ankylosing spondylitis and their correction with the help of whole body cryotherapy. The study included 55 patients randomly allocated to two groups. Group 1 was comprised of the patients treated with the use of the common mineral baths, physiotherapy, therapeutic physical exercises, spinal massage, and whole body air-cryotherapy. Group 2 contained the patients who were treated in a similar way with the exception of whole body cryotherapy; they served as controls. Muscular disorders were diagnosed by means of functional muscular testing. The study has demonstrated the high prevalence of muscular disorders in the patients suffering from ankylosing spondylitis. Moreover, it revealed the profile of such disorders associated with ankylosing spondylitis and showed significant correlation between the results of functional muscular testing, BASMI and BASFI indices as well as characteristics of chest excursions (pcryotherapy in comparison with the alternative therapeutic modalities employed in the present study. This therapeutic modality ensured the statistically more pronounced improvement of functional muscular testing parameters (pcryotherapy accounting for its corrective influence on the muscular disorders in the patients presenting with ankylosing spondylitis. It is concluded that the proposed approach can be recommended for the introduction in the combined therapeutic and rehabilitative treatment of muscular disorders associated with ankylosing spondylitis.

  20. Glucocorticoids for Duchenne Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2013-07-01

    Full Text Available Investigators at the Dubowitz Neuromuscular Centre, Great Ormond Street Hospital, and other centers in the UK, conducted a prospective longitudinal study across 17 neuromuscular centers in the UK of 360 boys aged 3-15 years with Duchenne muscular dystrophy who were treated with daily or intermittent (10 days on/10 days off prednisolone for a mean duration of 4 years.

  1. Atrofia muscular espinhal: diagnóstico, tratamento e perspectivas futuras

    Directory of Open Access Journals (Sweden)

    Mariana T. C. Baioni

    2010-08-01

    Full Text Available OBJETIVO: Relatar as recentes descobertas genéticas e moleculares, juntamente com as perspectivas futuras, para o tratamento da atrofia muscular espinhal, auxiliando, dessa forma, os profissionais da área da saúde a fazerem um rápido diagnóstico e proporcionarem um suporte terapêutico correto e precoce. FONTES DOS DADOS: As informações foram coletadas a partir de artigos científicos publicados nas duas últimas décadas, pesquisados nas bases de dados SciELO, PubMed e MEDLINE. SÍNTESE DOS DADOS: A atrofia muscular espinhal é uma doença neurodegenerativa com herança genética autossômica recessiva. É causada por uma deleção homozigótica do gene de sobrevivência do motoneurônio. Essa alteração genética resulta na redução dos níveis da proteína de sobrevivência do motoneurônio, levando à degeneração de motoneurônios alfa da medula espinhal, o que resulta em fraqueza e paralisia muscular proximal progressiva simétrica. Sabe-se que alguns cuidados básicos referentes à nutrição, respiração e fisioterapia podem ser importantes para retardar o progresso da doença e prolongar a vida dos pacientes. Vários medicamentos estão sendo testados, alguns novos, outros já conhecidos, como o ácido valproico, sendo que a paralisia pode ser estacionada, mas não revertida. CONCLUSÕES: A atrofia muscular espinhal é uma desordem de difícil diagnóstico, por ser pouco conhecida, e de tratamento ainda incerto. Os tratamentos farmacológicos e as terapias de suporte existentes ainda não são capazes de recuperar os motoneurônios ou as células musculares que já foram perdidos, mas têm o objetivo de retardar o progresso da doença e melhorar a função muscular residual dos pacientes, bem como oferecer uma melhor qualidade e expectativa de vida.

  2. Muscular sarcoidosis involving the chest and abdominal walls: case report with MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seunghyun; Lee, In Sook; Song, You Seon [Pusan National University Hospital, Biomedical Research Institute, Department of Radiology, Busan (Korea, Republic of); Pusan National University School of Medicine, Busan (Korea, Republic of); Mok, Jeongha [Pusan National University Hospital, Biomedical Research Institute, Department of Internal Medicine, Busan (Korea, Republic of); Choi, Kyung-Un [Pusan National University Hospital, Biomedical Research Institute, Department of Pathology, Busan (Korea, Republic of)

    2018-03-15

    Sarcoidosis is an inflammatory disorder that is characterized by the presence of noncaseating granulomas in tissues, involving many organs and tissues. Extra-pulmonary, especially muscular sarcoidosis is a rare condition. The most common location of the muscular sarcoidosis is known to be the proximal muscles of the extremities; however, there have been no cases of diffuse involvement of the chest and abdominal wall muscles. Here, we report a rare muscular sarcoidosis with infiltrative pattern in the chest and abdominal wall muscles and describe the MR imaging findings that were mistaken as lymphoma at initial diagnosis. Although our case did not show characteristic MR findings of muscular sarcoidosis, clinicians or radiologists who are aware of these imaging features can perform early systemic survey for sarcoidosis. Also muscle biopsy is very important to confirm the sarcoidosis and distinguish it from other tumors. (orig.)

  3. Muscular sarcoidosis involving the chest and abdominal walls: case report with MR imaging

    International Nuclear Information System (INIS)

    Lee, Seunghyun; Lee, In Sook; Song, You Seon; Mok, Jeongha; Choi, Kyung-Un

    2018-01-01

    Sarcoidosis is an inflammatory disorder that is characterized by the presence of noncaseating granulomas in tissues, involving many organs and tissues. Extra-pulmonary, especially muscular sarcoidosis is a rare condition. The most common location of the muscular sarcoidosis is known to be the proximal muscles of the extremities; however, there have been no cases of diffuse involvement of the chest and abdominal wall muscles. Here, we report a rare muscular sarcoidosis with infiltrative pattern in the chest and abdominal wall muscles and describe the MR imaging findings that were mistaken as lymphoma at initial diagnosis. Although our case did not show characteristic MR findings of muscular sarcoidosis, clinicians or radiologists who are aware of these imaging features can perform early systemic survey for sarcoidosis. Also muscle biopsy is very important to confirm the sarcoidosis and distinguish it from other tumors. (orig.)

  4. Impact of a supervised worksite exercise program on back and core muscular endurance in firefighters.

    Science.gov (United States)

    Mayer, John M; Quillen, William S; Verna, Joe L; Chen, Ren; Lunseth, Paul; Dagenais, Simon

    2015-01-01

    Low back pain is a leading cause of disability in firefighters and is related to poor muscular endurance. This study examined the impact of supervised worksite exercise on back and core muscular endurance in firefighters. A cluster randomized controlled trial was used for this study. The study occurred in fire stations of a municipal fire department (Tampa, Florida). Subjects were 96 full-duty career firefighters who were randomly assigned by fire station to exercise (n = 54) or control (n = 42) groups. Exercise group participants completed a supervised exercise targeting the back and core muscles while on duty, two times per week for 24 weeks, in addition to their usual fitness regimen. Control group participants continued their usual fitness regimen. Back and core muscular endurance was assessed with the Biering-Sorensen test and plank test, respectively. Changes in back and core muscular endurance from baseline to 24 weeks were compared between groups using analysis of covariance and linear mixed effects models. After 24 weeks, the exercise group had 12% greater (p = .021) back muscular endurance and 21% greater (p = .0006) core muscular endurance than did the control group. The exercise intervention did not disrupt operations or job performance. A supervised worksite exercise program was safe and effective in improving back and core muscular endurance in firefighters, which could protect against future low back pain.

  5. Kinematics, muscular activity and propulsion in gopher snakes

    Science.gov (United States)

    Moon; Gans

    1998-10-01

    Previous studies have addressed the physical principles and muscular activity patterns underlying terrestrial lateral undulation in snakes, but not the mechanism by which muscular activity produces curvature and propulsion. In this study, we used synchronized electromyography and videography to examine the muscular basis and propulsive mechanism of terrestrial lateral undulation in gopher snakes Pituophis melanoleucus affinis. Specifically, we used patch electrodes to record from the semispinalis, longissimus dorsi and iliocostalis muscles in snakes pushing against one or more pegs. Axial bends propagate posteriorly along the body and contact the pegs at or immediately posterior to an inflection of curvature, which then reverses anterior to the peg. The vertebral column bends broadly around a peg, whereas the body wall bends sharply and asymmetrically around the anterior surface of the peg. The epaxial muscles are always active contralateral to the point of contact with a peg; they are activated slightly before or at the point of maximal convexity and deactivated variably between the inflection point and the point of maximal concavity. This pattern is consistent with muscular shortening and the production of axial bends, although variability in the pattern indicates that other muscles may affect the mechanics of the epaxial muscles. The kinematic and motor patterns in snakes crawling against experimentally increased drag indicated that forces are produced largely by muscles that are active in the axial bend around each peg, rather than by distant muscles from which the forces might be transmitted by connective tissues. At each point of force exertion, the propulsive mechanism of terrestrial lateral undulation may be modeled as a type of cam-follower, in which continuous bending of the trunk around the peg produces translation of the snake.

  6. Repair of an inguinoscrotal hernia in a patient with Becker muscular dystrophy.

    Science.gov (United States)

    Tatulli, F; Caraglia, A; Delcuratolo, A; Cassano, S; Chetta, G S

    2017-01-01

    Inguinal hernia repairs are routinely performed as outpatient procedures in most patients, whereas a few require admission due to clinical or social peculiarities. Muscular dystrophies are inherited disorders characterized by progressive muscle wasting and weakness. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards a 48 y. o. male patient diagnosed with Becker Muscular Dystrophy by muscle biopsy 10 years earlier. He had a left-sided sizable inguinoscrotal hernia with repeat episodes of incarceration. An elective mesh repair with suction drainage was accomplished under selective spinal anesthesia. The post-operative course was uneventful. A few inguinal hernia repairs require admission due to peculiarities such as extensive scrotal hernias requiring suction drainage. Muscular dystrophies are inherited disorders with no cure and no two dystrophy patients are exactly alike, therefore the health issues will be different for each individual. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards the successful elective mesh repair with suction drainage of a large left-sided inguino-scrotal hernia in a 48 y. o. male patient affected by Becker muscular dystrophy by selective spinal anesthesia obtained by 10 milligrams of hyperbaric bupivacaine. Effective mesh repair with suction drainage of large inguinal hernias under spinal anesthesia can be achieved in patients affected by muscular dystrophy.

  7. Vibration therapy tolerated in children with Duchenne muscular dystrophy: a pilot study.

    Science.gov (United States)

    Myers, Kenneth A; Ramage, Barbara; Khan, Aneal; Mah, Jean K

    2014-07-01

    Duchenne muscular dystrophy is an X-linked recessive muscular dystrophy. Clinical management primarily involves rehabilitation strategies aimed at preserving functional mobility as long as possible. Side-alternating vibration therapy is a rehabilitation intervention that has shown promise in a number of different neuromuscular disorders, and has the potential to preserve strength, functional mobility, and bone mass. There has been little research regarding the tolerance to side-alternating vibration therapy in muscle diseases such as Duchenne muscular dystrophy. Four patients were recruited for a pilot study assessing the safety and tolerance of side-alternating vibration therapy in individuals with Duchenne muscular dystrophy. All patients participated in a 4-week training period involving side-alternating vibration therapy sessions three times per week. Serum creatine kinase was measured, and adverse effects reviewed at each session with functional mobility assessed before and after the training period. All patients tolerated the training protocol well, and there were no major changes in functional mobility. One patient had a transient increase in creatine kinase during the study; however, levels of this enzyme were stable overall when comparing the pretraining and posttraining values. Some patients reported subjective improvement during the training period. Side-alternating vibration therapy is well tolerated in children with Duchenne muscular dystrophy and may have potential to improve or maintain functional mobility and strength in these patients. Copyright © 2014 Elsevier Inc. All rights reserved.

  8. Childhood cardiorespiratory fitness, muscular fitness and adult measures of glucose homeostasis.

    Science.gov (United States)

    Fraser, Brooklyn J; Blizzard, Leigh; Schmidt, Michael D; Juonala, Markus; Dwyer, Terence; Venn, Alison J; Magnussen, Costan G

    2018-02-14

    To assess whether childhood cardiorespiratory fitness (CRF) and muscular fitness phenotypes (strength, power, endurance) predict adult glucose homeostasis measures. Prospective longitudinal study. Study examining participants who had physical fitness measured in childhood (aged 7-15 years) and who attended follow-up clinics approximately 20 years later and provided a fasting blood sample which was tested for glucose and insulin. Physical fitness measurements included muscular strength (right and left grip, shoulder flexion, shoulder and leg extension), power (standing long jump distance) and endurance (number of push-ups in 30s), and CRF (1.6km run duration). In adulthood, fasting glucose and insulin levels were used to derive glucose homeostasis measures of insulin resistance (HOMA2-IR) and beta cell function (HOMA2-β). A standard deviation increase in childhood CRF or muscular strength (males) was associated with fasting glucose (CRF: β=-0.06mmol/L), fasting insulin (CRF: β=-0.73mU/L; strength: β=-0.40mU/L), HOMA2-IR (CRF: β=-0.06; strength: β=-0.05) and HOMA2-β (CRF: β=-3.06%; strength: β=-2.62%) in adulthood, independent of the alternative fitness phenotype (all p0.06). CRF and muscular fitness in childhood were inversely associated with measures of fasting insulin, insulin resistance and beta cell function in adulthood. Childhood CRF and muscular fitness could both be potential independent targets for strategies to help reduce the development of adverse glucose homeostasis. Copyright © 2018 Sports Medicine Australia. Published by Elsevier Ltd. All rights reserved.

  9. Nanolipodendrosome-loaded glatiramer acetate and myogenic differentiation 1 as augmentation therapeutic strategy approaches in muscular dystrophy.

    Science.gov (United States)

    Afzal, Ehsan; Zakeri, Saba; Keyhanvar, Peyman; Bagheri, Meisam; Mahjoubi, Parvin; Asadian, Mahtab; Omoomi, Nogol; Dehqanian, Mohammad; Ghalandarlaki, Negar; Darvishmohammadi, Tahmineh; Farjadian, Fatemeh; Golvajoee, Mohammad Sadegh; Afzal, Shadi; Ghaffari, Maryam; Cohan, Reza Ahangari; Gravand, Amin; Ardestani, Mehdi Shafiee

    2013-01-01

    [Corrected] Muscular dystrophies consist of a number of juvenile and adult forms of complex disorders which generally cause weakness or efficiency defects affecting skeletal muscles or, in some kinds, other types of tissues in all parts of the body are vastly affected. In previous studies, it was observed that along with muscular dystrophy, immune inflammation was caused by inflammatory cells invasion - like T lymphocyte markers (CD8+/CD4+). Inflammatory processes play a major part in muscular fibrosis in muscular dystrophy patients. Additionally, a significant decrease in amounts of two myogenic recovery factors (myogenic differentation 1 [MyoD] and myogenin) in animal models was observed. The drug glatiramer acetate causes anti-inflammatory cytokines to increase and T helper (Th) cells to induce, in an as yet unknown mechanism. MyoD recovery activity in muscular cells justifies using it alongside this drug. In this study, a nanolipodendrosome carrier as a drug delivery system was designed. The purpose of the system was to maximize the delivery and efficiency of the two drug factors, MyoD and myogenin, and introduce them as novel therapeutic agents in muscular dystrophy phenotypic mice. The generation of new muscular cells was analyzed in SW1 mice. Then, immune system changes and probable side effects after injecting the nanodrug formulations were investigated. The loaded lipodendrimer nanocarrier with the candidate drug, in comparison with the nandrolone control drug, caused a significant increase in muscular mass, a reduction in CD4+/CD8+ inflammation markers, and no significant toxicity was observed. The results support the hypothesis that the nanolipodendrimer containing the two candidate drugs will probably be an efficient means to ameliorate muscular degeneration, and warrants further investigation.

  10. Sarcopenia and sarcopenic obesity in patients with muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Luciano eMerlini

    2014-10-01

    Full Text Available Aging sarcopenia and muscular dystrophy are two conditions characterized by lower skeletal muscle quantity, lower muscle strength, and lower physical performance. Aging is associated with a peculiar alteration in body composition called sarcopenic obesity characterized by a decrease in lean body mass and increase in fat mass. To evaluate the presence of sarcopenia and obesity in a cohort of adult patients with muscular dystrophy we have used the measurement techniques considered golden standard for sarcopenia that is for muscle mass dual energy X-ray absorptiometry (DXA, for muscle strength hand held dynamometry, and for physical performance gait speed. The study involved 14 adult patients with different types of muscular dystrophy. We were able to demonstrate that all patient were sarcopenic-obese. We showed in fact that all were sarcopenic based on appendicular lean, fat & bone free, mass index (ALMI. In addition all resulted obese according to the % of body fat determined by DXA in contrast with their body mass index ranging from underweight to obese. Skeletal muscle mass determined by DXA was markedly reduced in all patients and correlated with residual muscle strength determined by hand held dynamometry, and physical performances determined by gait speed and respiratory function. Finally we showed that ALMI was the best linear explicator of muscle strength and physical function. Altogether, our study suggest the relevance of a proper evaluation of body composition in muscular dystrophy and we propose to use, both in research and practice, the measurement techniques that has already been demonstrated effective in aging sarcopenia.

  11. Muscular involvement by malignant lymphoma: CT and MR findings

    International Nuclear Information System (INIS)

    Kim, Baek Hyun

    2000-01-01

    To investigate the CT and MR findings of muscular involvement by malignant lymphoma. Thirteen patients with biopsy-proved muscular involvement by malignant lymphoma were included in this study. Two patients were primary muscle lymphoma and 11 patients were muscle lymphoma by secondary involvement of malignant lymphoma. CT of 10 patients (6 pre-contrast CT and 9 postcontrast CT) and MRI of 6 patients (all with pre a nd post-contrast studies) were retrospectively analyzed. In the majority of patients (84.6%, 11/13), the appearance of muscular involvement was the diffuse enlargement of several muscles as like as a group. The muscles involved by malignant lymphoma showed iso-attenuation (5/6) and homogeneity (6/6) on pre-contrast CT scan, and high attenuation (5/9) or iso-attenuation (4/9) and homogeneity (7/9) on post-contrast CT scan. The signal intensity of involved muscle showed slightly hyper- (4/6) or iso-intense (2/6) and homogeneous (6/6) on T1-weighted images, and hyper-intense (6/6) and homogeneous (4/6) on T2- and Gadolinium-enhanced T1-weighted images. Adjacent bone change was demonstrated in 69.2% (9/13), subcutaneous fat change in 61.5% (8/13), and neurovascular encasement within involved muscle in 53.8% (7/13). The CT and MR findings of muscular involvement by malignant lymphoma were diffuse enlargement of several muscles with homogeneous attenuation or signal intensity, and frequent changes in adjacent bones and subcutaneous fat, or neurovascular encasement. (author)

  12. Physical Therapy and Facioscapulohumeral Muscular Dystrophy (FSHD)

    Science.gov (United States)

    Physical Therapy & FSHD Facioscapulohumeral Muscular Dystrophy A Guide for Patients & Physical Therapists Authors: Wendy M. King, P.T., Assistant ... Shree Pandya, P.T., M.S., Assistant Professor, Neurology & Physical Medicine and Rehabilitation A publication of the FSH ...

  13. Influence of Manual Labor at Work on Muscular Fitness and Its Relationship With Work Performance.

    Science.gov (United States)

    Ryan, Eric D; Thompson, Brennan J; Sobolewski, Eric J

    2016-10-01

    The present study examined the influence of workplace manual labor on measures of muscular fitness, with a secondary aim to investigate the relationship between muscular fitness and work performance in blue-collar (BC) workers. Leg extension isokinetic strength at slow and fast velocities, hamstring and hip-flexor flexibility, and low back muscular endurance were examined in young and older BC workers and white-collar (WC) controls, while work performance was examined in the BC cohort. There were no differences in muscular fitness variables between BC and WC groups; however, the older men had lower low back muscular endurance (-43.0%) and strength at slow (-9.4%) and fast (-12.7%) velocities. Work performance was associated with strength at fast velocities (r = 0.633) in the older BC workers. Leg strength may influence work performance, with higher velocities becoming more important in older workers.

  14. Limb-girdle muscular dystrophy: an immunohistochemical diagnostic approach Distrofias musculares de cinturas: uma abordagem diagnóstica imuno-histoquímica

    Directory of Open Access Journals (Sweden)

    Enio Alberto Comerlato

    2005-06-01

    Full Text Available The limb-girdle muscle dystrophy (LGMD represents a heterogeneous group of muscular diseases with dominant and recessive inheritance, individualized by gene mutation. A group of 56 patients, 32 males and 24 females, with suggestive LGMD diagnosis were submitted to clinical evaluation, serum muscle enzymes, electromyography, muscle biopsy, and the immunoidentification (ID of sarcoglycans (SG alpha, beta, gamma and delta, dysferlin and western blot for calpain-3. All the patients had normal ID for dystrophin (rod domain, carboxyl and amine terminal. The alpha-SG was normal in 42 patients, beta-SG in 28, beta-SG in 45, delta-SG in 32, dysferlin in 37 and calpain-3 in 9. There was a reduction in the alpha-SG in 7 patients, beta-SG in 4, gamma-SG in 2, and delta-SG in 8. There was deficiency of alpha-SG in 7 patients, beta-SG in 6, gamma-SG in 9, delta-SG in 5, dysferlin in 8, and calpain-3 in 5. The patients were grouped according the ID as sarcoglycans deficiency 18 cases, dysferlin deficiency 8 cases and calpain-3 deficiency 5 cases. Only the sarcoglycans deficiency group showed calf hypertrophy. The dysferlin deficiency group was more frequent in females and the onset was later than sarcoglycan and calpain-3 deficiency groups. The calpain-3 deficiency group occurred only in males and showed an earlier onset and weaker muscular strength.As distrofias musculares de cinturas (DMC representam grupo heterogêneo de doenças musculares com heranças autossômicas dominante ou recessivas, caracterizadas geneticamente por mutações gênicas específicas. Cinqüenta e seis pacientes, 32 masculinos e 24 femininos, com diagnóstico sugestivo de DMC, foram submetidos a avaliação clínica, dosagem séricas das enzimas musculares, eletromiografia, biópsia muscular e imunoidentificação (ID das proteínas sarcoglicanas (SG alfa, beta, gama e delta, disferlina e calpaína-3. A ID da distrofina (domínio rod e terminais carboxila e amino era normal em todos

  15. Emery-Dreifuss muscular dystrophy: anatomical-clinical correlation (case report Distrofia muscular de Emery-Dreifuss: correlação anátomo-clínica (relato de caso

    Directory of Open Access Journals (Sweden)

    ALZIRA ALVES DE SIQUEIRA CARVALHO

    2000-12-01

    Full Text Available We report on a man that had weakness of humeroperoneal distribution associated with limited range of motion of the cervical spine and elbows since he was 5 years old . At age 26 he developed tachycardia episodes. A complex arrhythmia was discovered, and a nodal ablation was done with a cardiac pacemaker implanted. The patient had an arrhythmia and sudden death followed this. Emery-Dreifuss muscular dystrophy is a rare recessive X-linked muscular disorder where mixed patterns in electromyography and muscle histology (neurogenic and/or myopathic have caused nosological confusion. The autopsy findings are here described and correlated to the clinical features in an attempt to better understand the ambiguous findings concerning the process etiology .Relatamos o caso de um paciente com fraqueza muscular de distribuição úmero-peroneal associada a limitação de movimentos da coluna cervical e cotovelos. Aos 26 anos, ele desenvolveu episódios de taquicardia. Uma arritmia complexa foi descoberta sendo feito ablação nodal seguida por implante de marcapasso cardíaco. O paciente evoluiu com arritmia e morte. A distrofia de Emery-Dreifuss é desordem muscular rara, recessiva, ligada ao cromossomo X cujo aspectos mistos (neurogênico e/ou miopático na eletroneuromiografia e biopsia muscular tem provocado dúvidas na nosologia. Os achados de autopsia são descritos e correlacionados aos critérios clínicos na tentativa de melhor compreender os achados ambíguos em relação à etiologia.

  16. Emerging genetic therapies to treat Duchenne muscular dystrophy

    Science.gov (United States)

    Nelson, Stanley F.; Crosbie, Rachelle H.; Miceli, M. Carrie; Spencer, Melissa J.

    2010-01-01

    Purpose of review Duchenne muscular dystrophy is a progressive muscle degenerative disease caused by dystrophin mutations. The purpose of this review is to highlight two emerging therapies designed to repair the primary genetic defect, called `exon skipping' and `nonsense codon suppression'. Recent findings A drug, PTC124, was identified that suppresses nonsense codon translation termination. PTC124 can lead to restoration of some dystrophin expression in human Duchenne muscular dystrophy muscles with mutations resulting in premature stops. Two drugs developed for exon skipping, PRO051 and AVI-4658, result in the exclusion of exon 51 from mature mRNA. They can restore the translational reading frame to dystrophin transcripts from patients with a particular subset of dystrophin gene deletions and lead to some restoration of dystrophin expression in affected boys' muscle in vivo. Both approaches have concluded phase I trials with no serious adverse events. Summary These novel therapies that act to correct the primary genetic defect of dystrophin deficiency are among the first generation of therapies tailored to correct specific mutations in humans. Thus, they represent paradigm forming approaches to personalized medicine with the potential to lead to life changing treatment for those affected by Duchenne muscular dystrophy. PMID:19745732

  17. Congenital contractural arachnodactyly with neurogenic muscular atrophy: case report

    Directory of Open Access Journals (Sweden)

    Scola Rosana Herminia

    2001-01-01

    Full Text Available We report the case of a 3-1/2-year-old girl with hypotonia, multiple joint contractures, hip luxation, arachnodactyly, adducted thumbs, dolichostenomelia, and abnormal external ears suggesting the diagnosis of congenital contractural arachnodactyly (CCA. The serum muscle enzimes were normal and the needle electromyography showed active and chronic denervation. The muscle biopsy demonstrated active and chronic denervation compatible with spinal muscular atrophy. Analysis of exons 7 and 8 of survival motor neuron gene through polymerase chain reaction did not show deletions. Neurogenic muscular atrophy is a new abnormality associated with CCA, suggesting that CCA is clinically heterogeneous.

  18. Duchenne muscular dystrophy - a molecular service

    African Journals Online (AJOL)

    In 1987 a carrier detection and prenatal diagnostic service for. Duchenne muscular dystrophy using molecular technology was instituted at the Department of Human Genetics, Uni- versity of Cape Town, to serve affe.cted families in southern. Africa. DNA samples from 100 affected male subjects and. 350 of their relatives ...

  19. Distinctive serum miRNA profile in mouse models of striated muscular pathologies.

    Directory of Open Access Journals (Sweden)

    Nicolas Vignier

    Full Text Available Biomarkers are critically important for disease diagnosis and monitoring. In particular, close monitoring of disease evolution is eminently required for the evaluation of therapeutic treatments. Classical monitoring methods in muscular dystrophies are largely based on histological and molecular analyses of muscle biopsies. Such biopsies are invasive and therefore difficult to obtain. The serum protein creatine kinase is a useful biomarker, which is however not specific for a given pathology and correlates poorly with the severity or course of the muscular pathology. The aim of the present study was the systematic evaluation of serum microRNAs (miRNAs as biomarkers in striated muscle pathologies. Mouse models for five striated muscle pathologies were investigated: Duchenne muscular dystrophy (DMD, limb-girdle muscular dystrophy type 2D (LGMD2D, limb-girdle muscular dystrophy type 2C (LGMD2C, Emery-Dreifuss muscular dystrophy (EDMD and hypertrophic cardiomyopathy (HCM. Two-step RT-qPCR methodology was elaborated, using two different RT-qPCR miRNA quantification technologies. We identified miRNA modulation in the serum of all the five mouse models. The most highly dysregulated serum miRNAs were found to be commonly upregulated in DMD, LGMD2D and LGMD2C mouse models, which all exhibit massive destruction of striated muscle tissues. Some of these miRNAs were down rather than upregulated in the EDMD mice, a model without massive myofiber destruction. The dysregulated miRNAs identified in the HCM model were different, with the exception of one dysregulated miRNA common to all pathologies. Importantly, a specific and distinctive circulating miRNA profile was identified for each studied pathological mouse model. The differential expression of a few dysregulated miRNAs in the DMD mice was further evaluated in DMD patients, providing new candidates of circulating miRNA biomarkers for DMD.

  20. Association of physical activity with muscular strength and fat-free mass in adolescents: the HELENA study.

    Science.gov (United States)

    Moliner-Urdiales, Diego; Ortega, Francisco B; Vicente-Rodriguez, Germán; Rey-Lopez, Juan P; Gracia-Marco, Luis; Widhalm, Kurt; Sjöström, Michael; Moreno, Luis A; Castillo, Manuel J; Ruiz, Jonatan R

    2010-08-01

    The objective of this study is to analyse the association of objectively assessed physical activity (PA) with muscular strength and fat-free mass in adolescents, and to determine whether meeting the current PA recommendations is associated with higher levels of muscular strength and fat-free mass. The present cross-sectional study comprised 363 Spanish adolescents (180 females) aged 12.5-17.5 years. PA was assessed by accelerometry and expressed as average PA (counts/min), and min/day of inactive, light, moderate, vigorous and moderate to vigorous PA (MVPA). MVPA was dichotomized into or = 60. Upper body muscular strength was measured with the handgrip strength test, and lower body muscular strength was measured with the standing broad jump, squat jump, counter movement jump and Abalakov tests. Fat-free mass was measured by DXA. We observed positive associations between vigorous PA and all the lower body muscular strength tests except for the counter movement jump in males. PA was not associated with fat-free mass in both males and females. Male adolescents engaged in at least 60 min/day MVPA performed better in the standing broad jump test. In conclusion, the findings of the present study suggest that only vigorous PA is associated with muscular strength, particularly lower-body muscular strength in male adolescents.

  1. The Clinical Observation on one case of Patient with Progressive Muscular Dystrophies

    Directory of Open Access Journals (Sweden)

    Yoo, Chang-Kil

    2000-12-01

    Full Text Available After observing a patient diagnosed with Progressive Muscular Dystropies from the August, 31, 2000 to the January, 2001, the following results are obtained. Method and Result: Under our assumption that the Korean Bee Venom Therapy is a good method to treat Progress Muscular Dystropies. Korean Bee Venom Therapy was applied on the following acupuncture points: BL23(Shinsu:腎兪, BL26(Guanyuanshu:關元兪, ST36(Chok-Samni:足三里, LI4(Hapkok:合谷, LV3(Taechung:太, SP10(Hyolhae:血海, SI9(Sojang-Kyonjong:貞. In addition CFC(Carthami Flos;紅花: and Cervi Pantotrichum Cornu; Herbal-Acupuncture is also treated on the other acupuncture points. For herbal medication was given to the patient based on the Sasang Constitution, Taeyangin Ogapijangchuk-tang. Following these treatments in this case of Progressive Muscular Dystropies, the skeletal muscle functions made remarkable improvement. Conclusion: Based on the clinical results, traditional Korean Medical treatment is believed to be effective for treating Progressive Muscular Dystropies, and further studies should be conducted to provide more valuable information.

  2. Secondary Conditions Among Males With Duchenne or Becker Muscular Dystrophy.

    Science.gov (United States)

    Latimer, Rebecca; Street, Natalie; Conway, Kristin Caspers; James, Kathy; Cunniff, Christopher; Oleszek, Joyce; Fox, Deborah; Ciafaloni, Emma; Westfield, Christina; Paramsothy, Pangaja

    2017-06-01

    Duchenne and Becker muscular dystrophy are X-linked neuromuscular disorders characterized by progressive muscle degeneration. Despite the involvement of multiple systems, secondary conditions among affected males have not been comprehensively described. Two hundred nine caregivers of affected males (aged 3-31 years) identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network completed a mailed survey that included questions about secondary conditions impacting multiple body functions. The 5 most commonly reported conditions in males with Duchenne were cognitive deficits (38.4%), constipation (31.7%), anxiety (29.3%), depression (27.4%), and obesity (19.5%). Higher frequencies of anxiety, depression, and kidney stones were found among nonambulatory males compared to ambulatory males. Attention-deficit hyperactivity disorder (ADHD) was more common in ambulatory than nonambulatory males. These data support clinical care recommendations for monitoring of patients with Duchenne or Becker muscular dystrophy by a multidisciplinary team to prevent and treat conditions that may be secondary to the diagnosis.

  3. Drive for muscularity and social physique anxiety mediate the perceived ideal physique muscle dysmorphia relationship.

    Science.gov (United States)

    Thomas, Adam; Tod, David A; Edwards, Christian J; McGuigan, Michael R

    2014-12-01

    This study examined the mediating role of drive for muscularity and social physique anxiety (SPA) in the perceived muscular male ideal physique and muscle dysmorphia relationship in weight training men. Men (N = 146, mean ± SD; age, 22.8 ± 5.0 years; weight, 82.0 ± 11.1 kg; height, 1.80 ± 0.07 m; body mass index, 25.1 ± 3.0) who participated in weight training completed validated questionnaires measuring drive for muscularity, SPA, perceived muscular male ideal physique, global muscle dysmorphia, and several characteristics of muscle dysmorphia (exercise dependence, diet manipulation, concerns about size/symmetry, physique protection behavior, and supplementation). Perceived ideal physique was an independent predictor of muscle dysmorphia measures except physique protection (coefficients = 0.113-0.149, p ≤ 0.05). Perceived ideal physique also predicted muscle dysmorphia characteristics (except physique protection and diet) through the indirect drive for muscularity pathway (coefficients = 0.055-0.116, p ≤ 0.05). Perceived ideal physique also predicted size/symmetry concerns and physique protection through the indirect drive for muscularity and SPA pathway (coefficients = 0.080-0.025, p ≤ 0.05). These results extend current research by providing insights into the way correlates of muscle dysmorphia interact to predict the condition. The results also highlight signs (e.g., anxiety about muscularity) that strength and conditioning coaches can use to identify at-risk people who may benefit from being referred for psychological assistance.

  4. Hipertrofia muscular e nutrição : Monografia : Muscule Hypertrophy and Nutrition

    OpenAIRE

    Moreira, Bruna Isabel Araújo

    2010-01-01

    Resumo da tese: A hipertrofia muscular é o processo através do qual se dá o aumento da massa muscular. O exercício de resistência é considerado um estímulo eficaz para a ocorrência da hipertrofia muscular. No entanto, tal não é suficiente para a promoção da deposição proteica no músculo, sendo essencial a interacção do exercício com a adequada ingestão alimentar. Apesar da tão badalada necessidade da ingestão proteica, factores como a ingestão energética ou o timing de ingestão têm-se mostrad...

  5. A Preliminary Study of Muscular Artifact Cancellation in Single-Channel EEG

    OpenAIRE

    Chen, Xun; Liu, Aiping; Peng, Hu; Ward, Rabab K.

    2014-01-01

    Electroencephalogram (EEG) recordings are often contaminated with muscular artifacts that strongly obscure the EEG signals and complicates their analysis. For the conventional case, where the EEG recordings are obtained simultaneously over many EEG channels, there exists a considerable range of methods for removing muscular artifacts. In recent years, there has been an increasing trend to use EEG information in ambulatory healthcare and related physiological signal monitoring systems. For pra...

  6. The Intriguing Regulators of Muscle Mass in Sarcopenia and Muscular Dystrophy

    OpenAIRE

    Sakuma, Kunihiro; Aoi, Wataru; Yamaguchi, Akihiko

    2014-01-01

    Recent advances in our understanding of the biology of muscle have led to new interest in the pharmacological treatment of muscle wasting. Loss of muscle mass and increased intramuscular fibrosis occur in both sarcopenia and muscular dystrophy. Several regulators (mammalian target of rapamycin, serum response factor, atrogin-1, myostatin, etc.) seem to modulate protein synthesis and degradation or transcription of muscle-specific genes during both sarcopenia and muscular dystrophy. This revie...

  7. Venous pump of the calf: a study of venous and muscular pressures.

    Science.gov (United States)

    Alimi, Y S; Barthelemy, P; Juhan, C

    1994-11-01

    Little data are available concerning the relation between the muscular pumping mechanism and the variation of superficial and deep venous pressure during normal action of the calf pump; therefore we undertook this study to determine the pressure values in three compartments of the calf and in the deep and the superficial venous system and to establish correlation between muscular and venous pressure. Nine healthy young women with a mean age of 23 years (range 19 to 28 years) were examined. In the same calf, a muscular catheter was placed in the deep posterior compartment (DPC), in the superficial posterior compartment (SPC), and in the anterior tibial compartment (ATC), and a vascular catheter was placed in the popliteal vein and in the greater saphenous vein (GSV). The five lines of pressure were simultaneously recorded in the following situations: at rest, during Valsalva maneuver, foot flexion, and foot extension. The situation was studied with the patient in the following positions: decubitus, sitting, standing, and squatting. A final continuous recording was carried out after the patient had been walking for 5 minutes. Mean values with standard errors of muscular and venous pressure were established in each situation. At rest and during Valsalva maneuver, the muscular pressures did not vary, whereas venous pressures increased significantly when the patient was sitting and standing. On the other hand, squatting was associated with a rise in the muscular and vein pressures. Foot flexion entailed a significant increase in the ATC pressure and a rise in the GSV pressure, whereas foot extension caused the DPC pressure to rise without venous pressure modifications. Walking was associated with an alternating increase in the DPC, SPC, GSV and popliteal vein pressures when the foot was compressed to floor followed by a significant decrease when the foot pressure was released. The variations in the deep and superficial venous pressures when the patient is sitting and

  8. Cardiomyopathy in becker muscular dystrophy: Overview.

    Science.gov (United States)

    Ho, Rady; Nguyen, My-Le; Mather, Paul

    2016-06-26

    Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed.

  9. Malocclusion prevalence and its relation to facial type and muscular activity in school students from 7 to 12 years of age in Bahia, Brazil = Malocusão, tipo facial e avaliação muscular em escolares de 7 a 12 anos de idade na Bahia, Brasil

    Directory of Open Access Journals (Sweden)

    Ribeiro, Camila Oliveira

    2015-01-01

    Full Text Available Objetivo: Avaliar a oclusão de escolares através do Índice de Estética Dentária (IED, do tipo facial utilizando antropometria, e a eletromiografia como recurso para avaliar a função muscular. Metodologia: A amostra constou de 324 escolares do gênero masculino e feminino, com idades de 7 a 12 anos, residentes em São Francisco do Conde, Bahia. A oclusão foi avaliada por intermédio do IED conforme a metodologia proposta pela Organização Mundial de Saúde. Com a cabeça na posição natural, o padrão facial foi analisado mediante a determinação da distância entre a altura facial morfológica e a largura bizigomática através de um paquímetro digital. A avaliação muscular foi tomada a partir da aquisição de registros eletromiográficos. Resultados: Verificaram-se diferenças estatisticamente significantes entre a severidade da oclusão e o tipo facial (p=0,039. A comparação com o potencial elétrico dos músculos não mostrou diferenças significantes. De acordo com a classificação do IED, 22,8% dos pacientes apresentaram oclusão normal ou leve alteração, 21% maloclusão definida, 20,1% maloclusão severa e 36,1% maloclusão muito severa ou deformadora. Conclusão: Este estudo revelou alto percentual de maloclusões de variados graus, reforçando a importância da atenção a ser dada a esta patologia nos programas institucionais de saúde bucal

  10. Lesão muscular nos atletas Muscle injuries in athletes

    Directory of Open Access Journals (Sweden)

    Guilherme Campos Barroso

    2011-01-01

    Full Text Available Este artigo tem por objetivo demonstrar a fisiologia, o diagnóstico e o tratamento das lesões musculares com foque nos atletas, suas demandas e expectativas. As lesões musculares estão entre as queixas mais comuns no atendimento ortopédico, ocorrendo tanto em atletas como em não atletas. Estas lesões caracterizam um desafio para os especialistas, haja vista a lenta recuperação que afasta o atleta dos treinamentos e competições, as frequentes sequelas e a recorrência das lesões. A maior parte das lesões musculares ocorre durante atividade desportiva, correspondendo de 10 a 55% de todas as lesões. Os músculos mais comumente afetados são os isquiotibiais, quadríceps e gastrocnêmios. Músculos estes biarticulares que estão mais sujeitos a forças de aceleração e desaceleração. O tratamento da lesão muscular varia desde o tratamento conservador até o tratamento cirúrgico. Novos procedimentos estão sendo utilizados, como a câmara hiperbárica e o uso de fatores de crescimento. No entanto, ainda é grande o número de recidivas de lesões. A lesão muscular continua sendo um tema com várias controvérsias. Novos tratamentos estão sendo pesquisados e desenvolvidos. A prevenção com fortalecimento muscular, o alongamento e o equilíbrio muscular continuam sendo o melhor "tratamento".This article had the aim of demonstrating the physiology, diagnosis and treatment of muscle injuries, focusing on athletes and their demands and expectations. Muscle injuries are among the most common complaints in orthopedic practice, occurring both among athletes and among non-athletes. These injuries present a challenge for specialists, due to the slow recovery, during which time athletes are unable to take part in training and competitions, and due to frequent sequelae and recurrences of the injuries. Most muscle injuries (between 10% and 55% of all injuries occur during sports activities. The muscles most commonly affected are the

  11. Complementary and alternative medicine for Duchenne and Becker muscular dystrophies: characteristics of users and caregivers.

    Science.gov (United States)

    Zhu, Yong; Romitti, Paul A; Conway, Kristin M; Andrews, Jennifer; Liu, Ke; Meaney, F John; Street, Natalie; Puzhankara, Soman; Druschel, Charlotte M; Matthews, Dennis J

    2014-07-01

    Complementary and alternative medicine is frequently used in the management of chronic pediatric diseases, but little is known about its use by those with Duchenne or Becker muscular dystrophy. Complementary and alternative medicine use by male patients with Duchenne or Becker muscular dystrophy and associations with characteristics of male patients and their caregivers were examined through interviews with 362 primary caregivers identified from the Muscular Dystrophy Surveillance, Tracking, and Research Network. Overall, 272 of the 362 (75.1%) primary caregivers reported that they had used any complementary and alternative medicine for the oldest Muscular Dystrophy Surveillance, Tracking, and Research Network male in their family. The most commonly reported therapies were from the mind-body medicine domain (61.0%) followed by those from the biologically based practice (39.2%), manipulative and body-based practice (29.3%), and whole medical system (6.9%) domains. Aquatherapy, prayer and/or blessing, special diet, and massage were the most frequently used therapies. Compared with nonusers, male patients who used any therapy were more likely to have an early onset of symptoms and use a wheel chair; their caregivers were more likely to be non-Hispanic white. Among domains, associations were observed with caregiver education and family income (mind-body medicines [excluding prayer and/or blessing only] and whole medical systems) and Muscular Dystrophy Surveillance, Tracking, and Research Network site (biologically based practices and mind-body medicines [excluding prayer and/or blessing only]). Complementary and alternative medicine use was common in the management of Duchenne and Becker muscular dystrophies among Muscular Dystrophy Surveillance, Tracking, and Research Network males. This widespread use suggests further study to evaluate the efficacy of integrating complementary and alternative medicine into treatment regimens for Duchenne and Becker muscular

  12. An unusual variant of Becker muscular dystrophy

    NARCIS (Netherlands)

    de Visser, M.; Bakker, E.; Defesche, J. C.; Bolhuis, P. A.; van Ommen, G. J.

    1990-01-01

    We report on 5 brothers with slowly progressive limbgirdle weakness. Calf hypertrophy was absent. The levels of creatine kinase, electromyography, and findings from a muscle biopsy specimen were compatible with muscular dystrophy. The propositus's biopsy specimen also showed numerous rimmed

  13. What Are the Treatments for Muscular Dystrophy?

    Science.gov (United States)

    ... Child Neurology Society. (2005). Practice parameter: Corticosteroid treatment of Duchenne dystrophy. Neurology, 64 , 13-20. Retrieved June 22, 2012, ... Statement. (2004). Respiratory care of the patient with Duchenne muscular ... American Journal of Respiratory and Critical Care Medicine, 170, 456-465. ...

  14. Progress study of the cardiac damage in Duchenne muscular dystrophy

    Directory of Open Access Journals (Sweden)

    ZHANG Yao

    2013-05-01

    Full Text Available Duchenne muscular dystrophy (DMD is a fatal muscular disease with rapid progression in children. Most patients die of respiratory and circulatory failure before the age of 20 if there is no systematic treatment. Now the heart problem in this disease has become increasingly prominent, and is thought to be closely associated with certain dystrophin exon deletion. We would like to review the epidemiology, relevance of dystrophin, pathogenesis, clinical manifestations and pathological features, as well as early prevention and treatment of DMD.

  15. Tratamiento fisioterápico en la distrofia muscular de Duchenne

    OpenAIRE

    Burgos González, Sara

    2014-01-01

    la Distrofia Muscular de Duchenne es una enfermedad neuromuscular hereditaria de carácter recesivo ligada al cromosoma X. Afecta aproximadamente a 1 de cada 3500 niños varones nacidos vivos. Se caracteriza por cursar con una debilidad progresiva como resultado de una degeneración de los músculos, iniciando en piernas y pelvis y posteriormente abarcando todo el cuerpo. Este trastorno se debe a una mutación que es culpable de la ausencia de una proteína muscular, la distrofina...

  16. The effects of exposure to slender and muscular images on male body dissatisfaction.

    Science.gov (United States)

    Galioto, Rachel; Crowther, Janis H

    2013-09-01

    This research examined the effects of appearance-based comparisons to muscular and slender idealized male bodies and the contribution of internalization and social comparison to change in body dissatisfaction. Participants were 111 male undergraduates who completed measures of body dissatisfaction, internalization, and social comparison and viewed images of either muscular or slender men in advertisements or product-only advertisements. Results indicated that exposure to both muscular and slender images was associated with an increase in body dissatisfaction, with no significant differences in the change in body dissatisfaction between the two image conditions. Internalization and trait social comparison were each associated with an increase in body dissatisfaction; however, upward social comparison was only a significant predictor of a change in body dissatisfaction for the males who viewed muscular images. These results highlight the impact of slender models on young men's body dissatisfaction and support the examination of media literacy interventions with this population. Copyright © 2013 Elsevier Ltd. All rights reserved.

  17. Late-onset Becker-type muscular dystrophy in a Border terrier dog.

    Science.gov (United States)

    Jeandel, A; Garosi, L S; Davies, L; Guo, L T; Salgüero, R; Shelton, G D

    2018-01-29

    A 9-year-old Border terrier was presented to a referral hospital after a 1-year history of progressive stiffness and exercise intolerance. Neurological examination was consistent with a neuromuscular disorder. Serum creatine kinase activity was mildly elevated. A myopathy was suspected based on MRI findings and electrophysiological examination. Muscle histopathology was consistent with a severe non-inflammatory myopathy of a dystrophic type. Immunofluorescence and western blotting confirmed a dystrophinopathy with an 80-kDa truncated dystrophin fragment similar to Becker muscular dystrophy in people. To our knowledge, this is the first description of a late-onset Becker-type muscular dystrophy in a dog, and the first description of a dystrophinopathy in a Border terrier. Muscular dystrophy in dogs should not be ruled out based on late onset clinical signs and only mildly elevated creatine kinase. © 2018 British Small Animal Veterinary Association.

  18. Sparks, signals and shock absorbers: how dystrophin loss causes muscular dystrophy.

    Science.gov (United States)

    Batchelor, Clare L; Winder, Steve J

    2006-04-01

    The dystrophin-glycoprotein complex (DGC) can be considered as a specialized adhesion complex, linking the extracellular matrix to the actin cytoskeleton, primarily in muscle cells. Mutations in several components of the DGC lead to its partial or total loss, resulting in various forms of muscular dystrophy. These typically manifest as progressive wasting diseases with loss of muscle integrity. Debate is ongoing about the precise function of the DGC: initially a strictly mechanical role was proposed but it has been suggested that there is aberrant calcium handling in muscular dystrophy and, more recently, changes in MAP kinase and GTPase signalling have been implicated in the aetiology of the disease. Here, we discuss new and interesting developments in these aspects of DGC function and attempt to rationalize the mechanical, calcium and signalling hypotheses to provide a unifying hypothesis of the underlying process of muscular dystrophy.

  19. Nose muscular dynamics: the tip trigonum.

    Science.gov (United States)

    Figallo, E E; Acosta, J A

    2001-10-01

    In 1995, the senior author (E.E.F.) published an article in which he described the musculus digastricus septi nasi labialis. In the article presented here, work carried out by anatomists and other researchers who, over the last two centuries, studied nose muscular dynamics is described. The present study is based on Gray's Anatomy, which, in 1858, first described the nasal tip muscles, along with the other nasal muscles. Later works not only used different terminology for these muscles but also ignored some, creating tremendous confusion. The study presented here provides an update of the exact terms, location, insertions, and muscle functions of the muscles of the nose. Each nose muscle is described with regard to the two portions able to produce separate contractions. In this study, the term "dual function" is used and characterizes the nasal mimetic muscles that do not have well-defined fascia. Therefore, there is doubt about the existence of a real nasal superficial muscle aponeurotic system. The musculus myrtiformis seems to have a dual function, inserting in the canine fosse and in the periosteum of the central incisors, forming two portions-one to the septum and the other to the nostril-each of which has specific functions. This study has been based on research in physiognomy, the science of expression. With regard to the basis for nose expressions, common anatomical research is excluded because it provides a different view of the dynamics studied to date. The term trigonum musculare apicis nasi defines the interaction of the musculi compressor narium minor and dilator naris anterior, connecting with the columellar bundle of the musculus digastricus and levering the nasal spine. This muscular trigone creates circular concentric and eccentric movements of the nasal tip.

  20. Muscular subunits transplantation for facial reanimation

    Directory of Open Access Journals (Sweden)

    Hazan André Salo Buslik

    2006-01-01

    Full Text Available PURPOSE: To present an alternative technique for reconstruction of musculocutaneous damages in the face transferring innervated subsegments(subunits of the latissimus dorsi flap for replacement of various facial mimetic muscles. METHODS: One clinical case of trauma with skin and mimetic muscles damage is described as an example of the technique. The treatment was performed with microsurgical transfer of latissimus dorsi muscle subunits. Each subunit present shape and dimensions of the respective mimetic muscles replaced. The origin, insertions and force vectors for the mimicmuscle lost were considered. Each subsegment has its own arterial and venous supply with a motor nerve component for the muscular unit. RESULTS: Pre and one year postoperative photos registration of static and dynamic mimic aspects, as well as digital electromyography digital data of the patients were compared. The transplanted muscular units presented myoeletric activity, fulfilling both the functional and cosmetic aspect. CONCLUSION: This technique seems to be a promising way to deal with the complex musculocutaneous losses of the face as well as facial palsy.

  1. Histopathological findings in Becker-type muscular dystrophy

    NARCIS (Netherlands)

    ten Houten, R.; de Visser, M.

    1984-01-01

    Muscle biopsy specimens from 14 patients with Becker-type muscular dystrophy were analyzed to investigate possible neurogenic factors underlying the histopathological changes. Group atrophy, pyknotic nuclear clumps, and angular small fibers were seen respectively in 71%, 85%, and 100% of the cases.

  2. La fatiga muscular en los deportistas: métodos físicos, nutricionales y farmacológicos para combatirla

    OpenAIRE

    Urdampilleta, Aritz; Armentia, Ivan; Gómez-Zorita, Saioa; Martínez Sanz, José Miguel; Mielgo-Ayuso, Juan

    2015-01-01

    Antecedentes: Una rápida recuperación en los deportistas es un aspecto fundamental para continuar entrenando a intensidades elevadas y seguir progresando más, especialmente en deportes en los que se compite todos los días. Los ejercicios excéntricos producen rupturas de miofibrillas musculares, sobre todo si se llevan a cabo de forma intensa y no habitual provocando daño muscular. Este daño muscular produce una fatiga muscular que limita el rendimiento muscular, disminuyendo la fuerza, el pic...

  3. Oxidative stress and pathology in muscular dystrophies: focus on protein thiol oxidation and dysferlinopathies.

    Science.gov (United States)

    Terrill, Jessica R; Radley-Crabb, Hannah G; Iwasaki, Tomohito; Lemckert, Frances A; Arthur, Peter G; Grounds, Miranda D

    2013-09-01

    The muscular dystrophies comprise more than 30 clinical disorders that are characterized by progressive skeletal muscle wasting and degeneration. Although the genetic basis for many of these disorders has been identified, the exact mechanism for pathogenesis generally remains unknown. It is considered that disturbed levels of reactive oxygen species (ROS) contribute to the pathology of many muscular dystrophies. Reactive oxygen species and oxidative stress may cause cellular damage by directly and irreversibly damaging macromolecules such as proteins, membrane lipids and DNA; another major cellular consequence of reactive oxygen species is the reversible modification of protein thiol side chains that may affect many aspects of molecular function. Irreversible oxidative damage of protein and lipids has been widely studied in Duchenne muscular dystrophy, and we have recently identified increased protein thiol oxidation in dystrophic muscles of the mdx mouse model for Duchenne muscular dystrophy. This review evaluates the role of elevated oxidative stress in Duchenne muscular dystrophy and other forms of muscular dystrophies, and presents new data that show significantly increased protein thiol oxidation and high levels of lipofuscin (a measure of cumulative oxidative damage) in dysferlin-deficient muscles of A/J mice at various ages. The significance of this elevated oxidative stress and high levels of reversible thiol oxidation, but minimal myofibre necrosis, is discussed in the context of the disease mechanism for dysferlinopathies, and compared with the situation for dystrophin-deficient mdx mice. © 2013 The Authors Journal compilation © 2013 FEBS.

  4. Living with illness and self-transcendence: the lived experience of patients with spinal muscular atrophy.

    Science.gov (United States)

    Ho, Hsin-Mei; Tseng, Ying-Hua; Hsin, Yu-Mei; Chou, Fan-Hao; Lin, Wei-Ting

    2016-11-01

    The aim of this study was to explore the lived experiences of patients afflicted with spinal muscular atrophy. Existing research studies on spinal muscular atrophy address the physical and psychological effects and complications of the disease; they also provide suggestions for how to improve the current management of this disease. However, information is limited on the disease process and the lived experience of spinal muscular atrophy patients. A phenomenological approach was conducted. Through 18 in-depth interviews recorded by a pen voice recorder, this study collected data obtained from a purposive sample of nine patients from the, 'Taiwan spinal muscular atrophy Families,' between November 2010-August 2011. The audio recordings were transcribed verbatim and data were analysed using Colaizzi's steps. Four themes and eight subthemes were identified: a loss of control (loss of muscular strength and independence), breaking limitations (assistive device use and mobility design), transcending limitations (independence/autonomy and social development) and living with hope (cherishing life and self-control). The results showed that the lived experiences of the spinal muscular atrophy patients involved living with illness, transcending the self and pursuing the meaning of life. Facing a life-threatening illness, these patients made self-adjustments in their lifestyles and exerted themselves to positively cope with hardships and maintain dignity and self-control. These findings could serve as evidence-based practice resources for healthcare professionals in helping individuals and their family members gain an in-depth understanding of spinal muscular atrophy's progression and life course and assist individuals in improving self-integrity to with hope. © 2016 John Wiley & Sons Ltd.

  5. Aerobic fitness, muscular strength and obesity in relation to risk of heart failure.

    Science.gov (United States)

    Crump, Casey; Sundquist, Jan; Winkleby, Marilyn A; Sundquist, Kristina

    2017-11-01

    Low physical fitness and obesity have been associated with higher risk of developing heart failure (HF), but their interactive effects are unknown. Elucidation of interactions among these common modifiable factors may help facilitate more effective primary prevention. We conducted a national cohort study to examine the interactive effects of aerobic fitness, muscular strength and body mass index (BMI) among 1 330 610 military conscripts in Sweden during 1969-1997 (97%-98% of all 18-year-old men) on risk of HF identified from inpatient and outpatient diagnoses through 2012 (maximum age 62 years). There were 11 711 men diagnosed with HF in 37.8 million person-years of follow-up. Low aerobic fitness, low muscular strength and obesity were independently associated with higher risk of HF, after adjusting for each other, socioeconomic factors, other chronic diseases and family history of HF. The combination of low aerobic fitness and low muscular strength (lowest vs highest tertiles) was associated with a 1.7-fold risk of HF (95% CI 1.6 to 1.9; pfitness, low muscular strength and obesity at the age of 18 years were independently associated with higher risk of HF in adulthood, with interactive effects between aerobic fitness and muscular strength. These findings suggest that early-life interventions may help reduce the long-term risk of HF and should include both aerobic fitness and muscular strength, even among persons with normal BMI. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  6. [DIAGNOSTIC VARIATIONS OF X-LINKED MUSCULAR DYSTROPHY WITH CONTRACTURES].

    Science.gov (United States)

    Kvirkvelia, N; Shakarishvili, R; Gugutsidze, D; Khizanishvili, N

    2015-01-01

    Case report with review describes X-linked muscular dystrophy with contractures in 28 years old man and his cousin. The disease revealed itself in an early stage (age 5-10), the process was progressing with apparent tendons retraction and contraction, limited movement in the areas of the neck and back of spine, atrophy of shoulder and pelvic yard and back muscles. Intellect was intact. Cardyomyopathy was exhibited. CK was normal. EMG showed classic myopathic features. Muscle biopsy showed different caliber groups of muscle fibers, growth of endo-perimesial connective tissue. Clinical manifestations together with electrophysiological and histological data suggest consistency with Rotthauwe-Mortier-Bayer X-linked muscular dystrophy.

  7. Structure and function of masticatory muscles in a case of muscular dystrophy

    DEFF Research Database (Denmark)

    Bakke, M; Kirkeby, S; Jensen, B L

    1990-01-01

    Histologic examination of muscle biopsies and functional examination comprising electromyography and force measurements in a 19-yr-old boy with muscular dystrophy showed different wasting patterns of mandibular elevator and depressor muscles. Pronounced histopathologic changes were present...... depressor strength corresponded more to reference values. This difference of muscular wasting might be caused by protective enzymes in the digastric muscle and/or functionally induced damage of the masseter. As affection from muscular dystrophy may vary greatly between the masticatory muscles, structural...... in the masseter muscle, whereas pathologic findings in the anterior digastric muscle were limited to increased number of cells in slightly enlarged interfiber connective tissue. The masticatory pattern was distorted, and strength of mandibular elevator muscles was less than one third of the norm, whereas...

  8. Promoción de la salud en adolescentes mediante un programa de intervención de mejora de la condición física y del estilo de vida

    Directory of Open Access Journals (Sweden)

    Eva Clemente Bosch

    2012-12-01

    Full Text Available Dado que el estilo de vida se condiciona en gran medida durante la adolescencia, es una etapa clave para la promoción de estilos de vida saludables, tales como el incremento de la actividad física y la mejora de la condición física. El objetivo de este estudio ha sido conocer el nivel de práctica de actividad física habitual en adolescentes y analizar su influencia en la salud, especialmente cardiovascular. Así como, diseñar y llevar a cabo un programa de intervención de mejora de la condición física y del estilo de vida en esta misma población. Se evaluó, pre-intervención, nivel de práctica de actividad física y tiempo dedicado a actividades sedentarias, mediante cuestionario; y pre- y posintervención, presión arterial y frecuencia cardiaca en reposo, mediante tensiómetro digital; composición corporal, mediante antropometría (peso, talla, pliegues cutáneos y perímetros corporales y la condición física orientada a la salud, mediante ocho pruebas para medir diferentes capacidades (resistencia aeróbica, fuerza y resistencia muscular, flexibilidad y velocidad-agilidad. A partir de las anteriores, se calculó Índice de masa corporal, porcentaje de grasa, masa libre de grasa y capacidad aeróbica. Concluimos que el nivel de práctica de actividad física de los adolescentes residentes en el área metropolitana de Valencia es bajo, asociándose los chicos a un nivel de práctica muy superior al de las chicas (57,6 % de chicos y 14 % de chicas cumplen las recomendaciones al respecto; datos que son preocupantes por las repercusiones que puedan tener en la salud. El sexo tiene una alta influencia en los hábitos de práctica de actividad física, por lo que se debería tener muy en cuenta a la hora de diseñar y llevar a cabo programas de intervención. Los adolescentes que practican más actividad física tienen mayor probabilidad de presentar mejor salu-d cardiovascular y la prevalencia de adolescentes que posee una

  9. Muscle regeneration and inflammation in patients with facioscapulohumeral muscular dystrophy

    DEFF Research Database (Denmark)

    Hauerslev, S; Ørngreen, M C; Hertz, J M

    2013-01-01

    The aim of this study was to investigate whether inflammation and regeneration are prominent in mildly affected muscles of patients with facioscapulohumeral muscular dystrophy type 1A (FSHD1A). Inflammation in muscle has been suggested by MRI studies in patients with FSHD1A.......The aim of this study was to investigate whether inflammation and regeneration are prominent in mildly affected muscles of patients with facioscapulohumeral muscular dystrophy type 1A (FSHD1A). Inflammation in muscle has been suggested by MRI studies in patients with FSHD1A....

  10. Anti-gravity training improves walking capacity and postural balance in patients with muscular dystrophy

    DEFF Research Database (Denmark)

    Berthelsen, Martin Peter; Husu, Edith; Christensen, Sofie Bouschinger

    2014-01-01

    of their weakness. We investigated the functional effects of combined aerobic and strength training in patients with Becker and limb-girdle muscular dystrophies with knee muscle strength levels as low as 3% of normal strength. Eight patients performed 10weeks of aerobic and strength training on an anti...... affected patients with Becker and limb-girdle muscular dystrophies.......Recent studies in patients with muscular dystrophies suggest positive effects of aerobic and strength training. These studies focused training on using bicycle ergometers and conventional strength training, which precludes more severely affected patients from participating, because...

  11. BIOCHEMICAL MECHANISM OF AUTOLYTIC PROCESSES OF MUSCULAR TISSUE OF FISHES

    Directory of Open Access Journals (Sweden)

    L. V. Antipova

    2015-01-01

    Full Text Available The conducted researches allowed to establish that intensive disintegration of a muscular glycogen leads to sharp decrease in size рН muscular tissue in the sour party that in turn affects a chemical composition and physic-colloidal structure of proteins therefore: resistance of meat of fish to action of putrefactive microorganisms increases; solubility of muscle proteins, level of their hydration which is water connecting abilities decreases; there is a swelling of collagen of connecting fabric; activity of the cathepsin (an optimum рН 5,3 causing hydrolysis of proteins at later stages of an autolysis increases; the bicarbonate system of muscular tissue with release of carbon dioxide collapses; predecessors of taste and aroma of meat are formed; process of oxidation of lipids becomes more active. As a result of accumulation dairy, phosphoric and other acids in meat of fish concentration of hydrogen ions of that decrease рН is result increases. Sharply shown sour environment and availability of inorganic phosphorus is considered the reason of disintegration of an actin-myosin complex on actin and a myosin which begins after 8 hours of storage, i.e. there comes the period of relaxation of muscle fibers and the period of permission of an numbness, and then the last stage of maturing of meat – deep autolysis. Thus, on the basis of classical ideas of biochemical changes of meat of land animals and summarizing the obtained data on posthumous changes in muscular tissue of fishes, it is possible to draw a conclusion that they have similar nature of regularity in comparison with muscular tissue of land animals, but their main difference is higher speed of course of autolytic transformations. It in turn leads to faster change of FTS of meat of fishes who are the defining indicators when developing assortment groups of products taking into account stages of an autolysis in meat.

  12. Adaptive characteristics of main muscular groups’ static endurance in 6 years children in initial school period

    Directory of Open Access Journals (Sweden)

    S.V. Bondarenko

    2017-08-01

    Full Text Available Purpose: to study adaptation of 6 years children’s muscular skeletal apparatus to learning work by indicators of static endurance dynamic. Material: in the research 6 years children participated (n=64, boys - n=36, girls- n=28. Indicators of main muscular groups’ static endurance were studied. Results: we determined comparative topography of 13 muscular groups’ static endurance and substantial sex dimorphism was found. In 9 muscular groups the boys advantage was 11.7 - 50.2% (р < 0.05 ÷ 0.01. The level and correlation of muscles-antagonists’ static endurance from the point of evolutionary and ontogeny development of muscular-skeletal apparatus were substantiated. At the end of semester we found significant (р < 0.001 reduction of static endurance indicators as well as the fact that torso muscles were the most sensitive to influence of learning work’s static component. The level of static endurance weakening, under which syndrome of static over-tension appears, was determined. Conclusions: the research results permit to balance the volumes of learning load at initial stage of school work. Organizational-methodic principles of learning and physical education system for children shall be oriented on overcoming muscular-skeletal apparatus’s dysfunctions.

  13. Procedimiento de estabilizacion de mercurio liquido mediante cemento polimerico de azufre,via sulfuro de mercurio

    OpenAIRE

    López Gómez, Félix Antonio; López-Delgado, Aurora; Alguacil, Francisco José; Alonso Gámez, Manuel

    2009-01-01

    Procedimiento para la estabilización de mercurio líquido mediante la obtención de cementos poliméricos de azufre que comprende: (a) transformación del mercurio líquido en sulfuro de mercurio (metacinabrio) mediante reacción química, en condiciones estequiométricas, entre el mercurio y el azufre elemental; y (b) obtención de cemento polimérico de azufre mediante la incorporación el sulfuro de mercurio obtenido en la etapa anterior, en una mezcla estable constituida por áridos, azufre elemental...

  14. Duchenne muscular dystrophy: High-resolution melting curve ...

    African Journals Online (AJOL)

    Duchenne muscular dystrophy: High-resolution melting curve analysis as an affordable diagnostic mutation scanning tool in a South African cohort. ... Genetic screening for D/BMD in South Africa currently includes multiple ligase-dependent probe amplification (MLPA) for exonic deletions and duplications and linkage ...

  15. Temporalis muscle hypertrophy and reduced skull eccentricity in Duchenne muscular dystrophy.

    Science.gov (United States)

    Straathof, C S M; Doorenweerd, N; Wokke, B H A; Dumas, E M; van den Bergen, J C; van Buchem, M A; Hendriksen, J G M; Verschuuren, J J G M; Kan, H E

    2014-10-01

    Muscle hypertrophy and muscle weakness are well known in Duchenne muscular dystrophy. Decreased muscle force can have secondary effects on skeletal growth and development such as facial and dental morphology changes. In this study, we quantified temporal muscle thickness, circumference, and eccentricity of the skull and the head on T1-weighted magnetic resonance imaging (MRI) scans of the head of 15 Duchenne muscular dystrophy patients and 15 controls. Average temporal muscle thickness was significantly increased in patients (12.9 ± 5.2 mm) compared to controls (6.8 ± 1.4 mm) (P muscle thickness and skull eccentricity were significantly negatively correlated in patients, and positively in controls. Hypertrophy of the temporal muscles and changes in skull eccentricity appear to occur early in the course of Duchenne muscular dystrophy. Further studies in younger patients are needed to confirm a causal relationship. © The Author(s) 2014.

  16. Weekly Time Course of Neuro-Muscular Adaptation to Intensive Strength Training.

    Science.gov (United States)

    Brown, Niklas; Bubeck, Dieter; Haeufle, Daniel F B; Weickenmeier, Johannes; Kuhl, Ellen; Alt, Wilfried; Schmitt, Syn

    2017-01-01

    Detailed description of the time course of muscular adaptation is rarely found in literature. Thus, models of muscular adaptation are difficult to validate since no detailed data of adaptation are available. In this article, as an initial step toward a detailed description and analysis of muscular adaptation, we provide a case report of 8 weeks of intense strength training with two active, male participants. Muscular adaptations were analyzed on a morphological level with MRI scans of the right quadriceps muscle and the calculation of muscle volume, on a voluntary strength level by isometric voluntary contractions with doublet stimulation (interpolated twitch technique) and on a non-voluntary level by resting twitch torques. Further, training volume and isokinetic power were closely monitored during the training phase. Data were analyzed weekly for 1 week prior to training, pre-training, 8 weeks of training and 2 weeks of detraining (no strength training). Results show a very individual adaptation to the intense strength training protocol. While training volume and isokinetic power increased linearly during the training phase, resting twitch parameters decreased for both participants after the first week of training and stayed below baseline until de-training. Voluntary activation level showed an increase in the first 4 weeks of training, while maximum voluntary contraction showed only little increase compared to baseline. Muscle volume increased for both subjects. Especially training status seemed to influence the acute reaction to intense strength training. Fatigue had a major influence on performance and could only be overcome by one participant. The results give a first detailed insight into muscular adaptation to intense strength training on various levels, providing a basis of data for a validation of muscle fatigue and adaptation models.

  17. Cardiac function associated with home ventilator care in Duchenne muscular dystrophy.

    Science.gov (United States)

    Lee, Sangheun; Lee, Heeyoung; Eun, Lucy Youngmin; Gang, Seung Woong

    2018-02-01

    Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non-ventilator-assisted. The patients in the home ventilator group were older (16.25±1.85 years) than those in the nonventilator group (14.73±1.36 years) ( P =0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity (1,038±620.41 mL) than the nonventilator group (1,455±603.12 mL). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio (1.7±0.44) was lower in the home ventilator group than in the nonventilator group (2.02±0.62). The mitral valve annular systolic velocity was higher in the home ventilator group (estimated β, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.

  18. A method to accurately estimate the muscular torques of human wearing exoskeletons by torque sensors.

    Science.gov (United States)

    Hwang, Beomsoo; Jeon, Doyoung

    2015-04-09

    In exoskeletal robots, the quantification of the user's muscular effort is important to recognize the user's motion intentions and evaluate motor abilities. In this paper, we attempt to estimate users' muscular efforts accurately using joint torque sensor which contains the measurements of dynamic effect of human body such as the inertial, Coriolis, and gravitational torques as well as torque by active muscular effort. It is important to extract the dynamic effects of the user's limb accurately from the measured torque. The user's limb dynamics are formulated and a convenient method of identifying user-specific parameters is suggested for estimating the user's muscular torque in robotic exoskeletons. Experiments were carried out on a wheelchair-integrated lower limb exoskeleton, EXOwheel, which was equipped with torque sensors in the hip and knee joints. The proposed methods were evaluated by 10 healthy participants during body weight-supported gait training. The experimental results show that the torque sensors are to estimate the muscular torque accurately in cases of relaxed and activated muscle conditions.

  19. A Method to Accurately Estimate the Muscular Torques of Human Wearing Exoskeletons by Torque Sensors

    Directory of Open Access Journals (Sweden)

    Beomsoo Hwang

    2015-04-01

    Full Text Available In exoskeletal robots, the quantification of the user’s muscular effort is important to recognize the user’s motion intentions and evaluate motor abilities. In this paper, we attempt to estimate users’ muscular efforts accurately using joint torque sensor which contains the measurements of dynamic effect of human body such as the inertial, Coriolis, and gravitational torques as well as torque by active muscular effort. It is important to extract the dynamic effects of the user’s limb accurately from the measured torque. The user’s limb dynamics are formulated and a convenient method of identifying user-specific parameters is suggested for estimating the user’s muscular torque in robotic exoskeletons. Experiments were carried out on a wheelchair-integrated lower limb exoskeleton, EXOwheel, which was equipped with torque sensors in the hip and knee joints. The proposed methods were evaluated by 10 healthy participants during body weight-supported gait training. The experimental results show that the torque sensors are to estimate the muscular torque accurately in cases of relaxed and activated muscle conditions.

  20. [Specific features of Becker Muscular Dystrophy patients and female carriers of Duchenne Muscular Dystrophy].

    Science.gov (United States)

    Magot, A; Mercier, S; Péréon, Y

    2015-12-01

    Becker muscular dystrophy (BMD) was first described in 1955 and linked to the DMD gene in 1987. Compared to Duchenne muscular dystrophy (DMD), clinical onset of BMD usually occurs after the age of 12 and wheelchair is required after the age of 16. BMD is characterized by generalized weakness first affecting limb girdle muscles, hypertrophy of the calves and cardiomyopathy in males. Some patients have only mild symptoms such as cramps or elevated serum creatine kinases (SCK) throughout all their lives. SCK levels are usually elevated. Muscle biopsy (immunohistochemistry or immunoblotting) shows a dystrophic pattern with abnormal dystrophin staining. Diagnosis is confirmed by DMD gene sequencing. Deletions or duplications of one or several exons are identified in the majority of cases. A multidisciplinary approach is recommended for the care management of these patients with a particular attention to the cardiomyopathy, which is typically responsible for death but can be prevented by specific treatment. X-linked dilated cardiomyopathies linked to DMD gene are a phenotypic continuum of BMD. Some female carriers of DMD mutations exhibit clinical symptoms of variable severity, often milder and beginning later than in males. The cardiomyopathy is the most frequent feature that should be especially monitored in these patients. Genetic counselling should be systematically proposed. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  1. Skeletal Muscle Metabolism in Duchenne and Becker Muscular Dystrophy—Implications for Therapies

    Directory of Open Access Journals (Sweden)

    Ahlke Heydemann

    2018-06-01

    Full Text Available The interactions between nutrition and metabolism and skeletal muscle have long been known. Muscle is the major metabolic organ—it consumes more calories than other organs—and therefore, there is a clear need to discuss these interactions and provide some direction for future research areas regarding muscle pathologies. In addition, new experiments and manuscripts continually reveal additional highly intricate, reciprocal interactions between metabolism and muscle. These reciprocal interactions include exercise, age, sex, diet, and pathologies including atrophy, hypoxia, obesity, diabetes, and muscle myopathies. Central to this review are the metabolic changes that occur in the skeletal muscle cells of muscular dystrophy patients and mouse models. Many of these metabolic changes are pathogenic (inappropriate body mass changes, mitochondrial dysfunction, reduced adenosine triphosphate (ATP levels, and increased Ca2+ and others are compensatory (increased phosphorylated AMP activated protein kinase (pAMPK, increased slow fiber numbers, and increased utrophin. Therefore, reversing or enhancing these changes with therapies will aid the patients. The multiple therapeutic targets to reverse or enhance the metabolic pathways will be discussed. Among the therapeutic targets are increasing pAMPK, utrophin, mitochondrial number and slow fiber characteristics, and inhibiting reactive oxygen species. Because new data reveals many additional intricate levels of interactions, new questions are rapidly arising. How does muscular dystrophy alter metabolism, and are the changes compensatory or pathogenic? How does metabolism affect muscular dystrophy? Of course, the most profound question is whether clinicians can therapeutically target nutrition and metabolism for muscular dystrophy patient benefit? Obtaining the answers to these questions will greatly aid patients with muscular dystrophy.

  2. Skeletal Muscle Metabolism in Duchenne and Becker Muscular Dystrophy-Implications for Therapies.

    Science.gov (United States)

    Heydemann, Ahlke

    2018-06-20

    The interactions between nutrition and metabolism and skeletal muscle have long been known. Muscle is the major metabolic organ—it consumes more calories than other organs—and therefore, there is a clear need to discuss these interactions and provide some direction for future research areas regarding muscle pathologies. In addition, new experiments and manuscripts continually reveal additional highly intricate, reciprocal interactions between metabolism and muscle. These reciprocal interactions include exercise, age, sex, diet, and pathologies including atrophy, hypoxia, obesity, diabetes, and muscle myopathies. Central to this review are the metabolic changes that occur in the skeletal muscle cells of muscular dystrophy patients and mouse models. Many of these metabolic changes are pathogenic (inappropriate body mass changes, mitochondrial dysfunction, reduced adenosine triphosphate (ATP) levels, and increased Ca 2+ ) and others are compensatory (increased phosphorylated AMP activated protein kinase (pAMPK), increased slow fiber numbers, and increased utrophin). Therefore, reversing or enhancing these changes with therapies will aid the patients. The multiple therapeutic targets to reverse or enhance the metabolic pathways will be discussed. Among the therapeutic targets are increasing pAMPK, utrophin, mitochondrial number and slow fiber characteristics, and inhibiting reactive oxygen species. Because new data reveals many additional intricate levels of interactions, new questions are rapidly arising. How does muscular dystrophy alter metabolism, and are the changes compensatory or pathogenic? How does metabolism affect muscular dystrophy? Of course, the most profound question is whether clinicians can therapeutically target nutrition and metabolism for muscular dystrophy patient benefit? Obtaining the answers to these questions will greatly aid patients with muscular dystrophy.

  3. Estimulação elétrica neuromuscular em cães com atrofia muscular induzida Neuromuscular electric stimulation in dogs with induced muscle atrophy

    Directory of Open Access Journals (Sweden)

    C. Pelizzari

    2008-02-01

    Full Text Available Empregou-se a estimulação elétrica neuromuscular (EENM de baixa freqüência no músculo quadríceps femoral de cães com atrofia induzida e avaliou-se a ocorrência de ganho de massa nessa musculatura. Foram utilizados oito cães com pesos entre 15 e 30kg, distribuídos aleatoriamente em dois grupos denominados de I ou controle e II ou tratado. A articulação femorotibiopatelar esquerda foi imobilizada por 30 dias pelo método de transfixação percutânea tipo II, com retirada de aparelho de imobilização após esse período. Decorridas 48 horas da remoção, foi realizada a EENM nos cães do grupo II, cinco vezes por semana, com intervalo de 24 horas cada sessão, pelo período de 60 dias. Foram avaliadas a circunferência da coxa, a goniometria do joelho, a análise clínica da marcha, as enzimas creatina-quinase (CK e aspartato-amino-transferase (AST e a morfometria das fibras musculares em cortes transversais do músculo vasto lateral colhido mediante biópsia muscular. A EENM foi empregada no músculo quadríceps femoral na freqüência de 50Hz, duração de pulso de 300 milisegundos e relação de tempo on/off de 1:2. Quanto à morfometria das fibras do músculo vasto lateral, no grupo tratado houve aumento significativo (PLow frequency neuromuscular electrical stimulation (NMES was used on the femoral quadriceps of dogs with induced muscular atrophy and the occurrence of gain in mass in these muscles was evaluated. Eight dogs from 15 to 30kg were randomly distributed in two groups named I, or control; and II, or treated. For the induction of muscular atrophy, the left femoral-tibial-patellar joint was immobilized for 30 days by percutaneous transfixation type II. After 30 days, the immobilization device was removed. The NMES treatment began 48 hours after the removal of the immobilization device of the dogs of group II, and it was carried out five times per week with an interval of 24 hours between each session, for 60 days. The

  4. Impact of muscular uptake and statistical noise on tumor quantification based on simulated FDG-PET studies

    International Nuclear Information System (INIS)

    Silva-Rodríguez, Jesús; Domínguez-Prado, Inés; Pardo-Montero, Juan; Ruibal, Álvaro

    2017-01-01

    Purpose: The aim of this work is to study the effect of physiological muscular uptake variations and statistical noise on tumor quantification in FDG-PET studies. Methods: We designed a realistic framework based on simulated FDG-PET acquisitions from an anthropomorphic phantom that included different muscular uptake levels and three spherical lung lesions with diameters of 31, 21 and 9 mm. A distribution of muscular uptake levels was obtained from 136 patients remitted to our center for whole-body FDG-PET. Simulated FDG-PET acquisitions were obtained by using the Simulation System for Emission Tomography package (SimSET) Monte Carlo package. Simulated data was reconstructed by using an iterative Ordered Subset Expectation Maximization (OSEM) algorithm implemented in the Software for Tomographic Image Reconstruction (STIR) library. Tumor quantification was carried out by using estimations of SUV max , SUV 50 and SUV mean from different noise realizations, lung lesions and multiple muscular uptakes. Results: Our analysis provided quantification variability values of 17–22% (SUV max ), 11–19% (SUV 50 ) and 8–10% (SUV mean ) when muscular uptake variations and statistical noise were included. Meanwhile, quantification variability due only to statistical noise was 7–8% (SUV max ), 3–7% (SUV 50 ) and 1–2% (SUV mean ) for large tumors (>20 mm) and 13% (SUV max ), 16% (SUV 50 ) and 8% (SUV mean ) for small tumors (<10 mm), thus showing that the variability in tumor quantification is mainly affected by muscular uptake variations when large enough tumors are considered. In addition, our results showed that quantification variability is strongly dominated by statistical noise when the injected dose decreases below 222 MBq. Conclusions: Our study revealed that muscular uptake variations between patients who are totally relaxed should be considered as an uncertainty source of tumor quantification values. - Highlights: • Distribution of muscular uptake from 136 PET

  5. Impacto de oito semanas de treinamento com pesos sobre a força muscular de homens e mulheres Impacto de ocho semanas de entrenamiento con pesos sobre la fuerza muscular de hombres y mujeres Impact of an eight-week weight training program on the muscular strength of men and women

    Directory of Open Access Journals (Sweden)

    Raphael Mendes Ritti Dias

    2005-08-01

    Full Text Available O objetivo do presente estudo foi verificar o impacto de oito semanas de treinamento com pesos (TP sobre a força muscular. Para tanto, 23 homens (20,7 ± 1,7 anos e 15 mulheres (20,9 ± 2,1 anos, aparentemente saudáveis e moderadamente ativos (atividade física regular El objetivo del presente estudio fué el de verificar el impacto de ocho semanas de entrenamiento con pesos (TP sobre la fuerza muscular. Para ello, 23 hombres (20,7 ± 1,7 años y 15 mujeres (20,9 ± 2,1 años, aparentemente saludables y moderadamente activos (atividad física regular The objective of the present study was to verify the impact of an eight-week weight training program (WT on the muscular strength. To do so, 23 men (20.7 ± 1.7 years and 15 women (20.9 ± 2.1 years, apparently healthy and moderately active (regular physical activity < 2 times a week were submitted to a WT program composed of 10 exercises for the different muscular groups during eight consecutive weeks. Three series of 8-12 RM were performed in each exercise in three weekly sessions with intervals each 48 hours. The pre and post-training muscular strength was determined by means of 1-RM tests in bench press, squat and arm curl exercises after five familiarization sessions. Increases on the muscular strength were verified in both genders in all exercises investigated as well as in the total amount of load lifted (P < 0.01. Although men presented higher absolute strength when compared to women in all exercises evaluated, the gains observed along time were higher for women (14.7 vs. 7.6% in squat; 17.2 vs. 11.0% bench press; 20.4 vs. 14.0% in arm curl. The results suggest that the eight-week WT period seems to be sufficient to promote significant modifications in the muscular strength of men and women in different body segments (lower limbs, trunk and upper limbs.

  6. Clinical and molecular characterization of limb-girdle muscular dystrophy due to LAMA2 mutations

    DEFF Research Database (Denmark)

    Gavassini, Bruno F; Carboni, Nicola; Nielsen, Jørgen E

    2011-01-01

    In this study we describe the clinical and molecular characteristics of limb-girdle muscular dystrophy (LGMD) due to LAMA2 mutations.......In this study we describe the clinical and molecular characteristics of limb-girdle muscular dystrophy (LGMD) due to LAMA2 mutations....

  7. Muscular and Cardiorespiratory Fitness in Homeschool versus Public School Children.

    Science.gov (United States)

    Kabiri, Laura S; Mitchell, Katy; Brewer, Wayne; Ortiz, Alexis

    2017-08-01

    The growth and unregulated structure of homeschooling creates an unknown population in regard to muscular and cardiorespiratory fitness. The purpose of this research was to compare muscular and cardiorespiratory fitness between elementary school aged homeschool and public school children. Homeschool children ages 8-11 years old (n = 75) completed the curl-up, 90° push-up, and Progressive Aerobic Capacity Endurance Run (PACER) portions of the FitnessGram to assess abdominal and upper body strength and endurance as well as cardiorespiratory fitness. Comparisons to public school children (n = 75) were made using t tests and chi-square tests. Homeschool children showed significantly lower abdominal (t(148) = -11.441, p fitness by total PACER laps (t(108) = 0.879, p = .381) or estimated VO 2max (t(70) = 1.187, p = .239; χ 2 (1) = 1.444, p = .486). Homeschool children showed significantly lower levels of both abdominal and upper body muscular fitness compared with their age and gender matched public school peers but no difference in cardiorespiratory fitness.

  8. Gay male attraction toward muscular men: does mating context matter?

    Science.gov (United States)

    Varangis, Eleanna; Lanzieri, Nicholas; Hildebrandt, Tom; Feldman, Matthew

    2012-03-01

    The purpose of this study was to examine gay men's perceived attractiveness of male figures based on short-term and long-term partner contexts. A sample of 190 gay adult men rated the attractiveness of line-drawings depicting male figures varying systematically in muscularity and body fat percentage in both short-term and long-term dating contexts. Mixed effects modeling was used to estimate the effects of figure (muscularity and body fat), dating context (short-term vs. long-term), and individual rater characteristics on attractiveness ratings. Results indicated that figure muscularity and body-fat had significant non-linear (i.e., quadratic) relationships with attractiveness ratings, and short-term dating context was associated with more discriminating ratings of attractiveness. Interactions between individual characteristics and figure characteristics indicated that the more available the individual and lower body fat, the more discriminating they were in ratings of attractiveness. The implications for future investigations considering both object and observer characteristics of attractiveness preferences are discussed. Copyright © 2012 Elsevier Ltd. All rights reserved.

  9. Effects of operative treatment for muscular asthenopia caused by esophoria

    Directory of Open Access Journals (Sweden)

    Tao Zeng

    2016-05-01

    Full Text Available AIM:To observe the effects of operative treatment for muscular asthenopia caused by esophoria. METHODS:Twenty-one cases(42 eyeswith muscular asthenopia caused by esophoria were included. After examination of refraction, phoria and fusion function, all cases were given operative treatment to correct esophoria. RESULTS:At 1wk after surgery, clinical symptoms were improved evidently in all cases, such as headache, diplopia and eye swelling. After followed up for 1a, no recrudescence was found. Before surgery, at 1wk and 1a after surgery, esophoria in the distance were 20.3△±6.3△,-3.1△±1.4△,0.7△±1.6△ respectively and esophoria in the near distance were 10.5△±3.1△,-1.3△±0.6△,1.5△±0.8△ respectively. By comparison of esophoria before and after surgery in different stages separately, the differences were statistically(PP>0.05. CONCLUSION:Esophoria operation may relieve obviously muscular asthenopia caused by esophoria without changing fusion function.

  10. Adaptability and Prediction of Anticipatory Muscular Activity Parameters to Different Movements in the Sitting Position.

    Science.gov (United States)

    Chikh, Soufien; Watelain, Eric; Faupin, Arnaud; Pinti, Antonio; Jarraya, Mohamed; Garnier, Cyril

    2016-08-01

    Voluntary movement often causes postural perturbation that requires an anticipatory postural adjustment to minimize perturbation and increase the efficiency and coordination during execution. This systematic review focuses specifically on the relationship between the parameters of anticipatory muscular activities and movement finality in sitting position among adults, to study the adaptability and predictability of anticipatory muscular activities parameters to different movements and conditions in sitting position in adults. A systematic literature search was performed using PubMed, Science Direct, Web of Science, Springer-Link, Engineering Village, and EbscoHost. Inclusion and exclusion criteria were applied to retain the most rigorous and specific studies, yielding 76 articles, Seventeen articles were excluded at first reading, and after the application of inclusion and exclusion criteria, 23 were retained. In a sitting position, central nervous system activity precedes movement by diverse anticipatory muscular activities and shows the ability to adapt anticipatory muscular activity parameters to the movement direction, postural stability, or charge weight. In addition, these parameters could be adapted to the speed of execution, as found for the standing position. Parameters of anticipatory muscular activities (duration, order, and amplitude of muscle contractions constituting the anticipatory muscular activity) could be used as a predictive indicator of forthcoming movement. In addition, this systematic review may improve methodology in empirical studies and assistive technology for people with disabilities. © The Author(s) 2016.

  11. Síndrome pulmonar por hantavirus en población infantil. Chile: Regiones IX y X. 1998-2000

    OpenAIRE

    SOZA C., GUILLERMO; LORCA O., PEDRO; PUEBLA M., SERGIO; WENZEL M., MARISOL; NAVARRETE C., MARITZA; VILLAGRA C., ELIECER; MORA R., JUDITH; LEVIS C., SILVANA; AVILES A., GABRIELA

    2000-01-01

    El síndrome pulmonar por hantavirus (SPH) ha estado presente en Chile desde 1993 y ha sido detectado desde 1997 en la IX Región. Es una grave zoonosis con alta mortalidad, que afecta a gente joven incluyendo niños. Se ha estimado oportuno dar a conocer nuestra experiencia en la atención de 6 pacientes pediátricos, atendidos en las unidades de Cuidados Intensivos y Aislamiento en el Hospital Regional de Temuco, entre enero de 1998 y enero de 2000 mediante un estudio descriptivo de la experienc...

  12. Skull development in the muscular dystrophic mouse

    DEFF Research Database (Denmark)

    Vilmann, H; Kirkeby, S; Moss, M L

    1989-01-01

    Roentgencephalometric tracings of skulls of 7-week-old normal and muscular dystrophic mice were compared. A marked size reduction of the dystrophic skulls relative to the normal ones was observed. However, the visceral parts of the dystrophic skull were more reduced in size than the neural parts....

  13. A Drosophila model for Duchenne muscular dystrophy

    NARCIS (Netherlands)

    Plas, Mariska Cathelijne van der

    2008-01-01

    Duchenne Muscular Dystrophy (DMD) is a severe X-linked disease characterized by progressive muscle wasting and sometimes mild mental retardation. The disease is caused by mutations in the dystrophin gene. DMD is correlated with the absence of Dp427, which is located along the sarcolemma in skeletal

  14. Cold modalities with different thermodynamic properties have similar effects on muscular performance and activation.

    Science.gov (United States)

    Vieira, A; Oliveira, A B; Costa, J R; Herrera, E; Salvini, T F

    2013-10-01

    Although tissue cooling is widely used in the treatment of musculoskeletal injuries there is still controversy about its effects on muscular performance. The combination of cooling and exercise justifies the study of this topic. The aim was to compare the effects of ice pack and cold-water immersion on the muscular performance parameters of plantar flexors and muscular activation of the triceps surae. 41 healthy men (mean age: 22.1 years, SD: 2.9) were randomly assigned to cooling with either ice pack (n=20) or cold-water immersion (n=21). Independent variables were cold modality (ice pack or cold-water immersion) and pre- and post-cooling measurement time. Dependent variables were muscular performance (measured during isometric and concentric contractions of plantar flexors) and electromyography parameters of the triceps surae (median frequency and root mean square amplitude). Dependent-samples t-tests were used to compare pre- and post-cooling data and independent-samples t-tests were used to compare the difference (pre- and post-cooling) between groups. Ice pack increased isometric peak torque (mean: 9.00 Nm, P=0.01) and both cold modalities reduced muscular activation in triceps surae (Pimmersion and ice pack reduced peak torque and total work during dynamic isokinetic contraction at both velocities (mean: -11,00 Nm, Pimmersion decrease concentric muscular performance. These results indicate that these cooling methods should be chosen with caution, considering the type of task required during training or rehabilitation. New studies investigating other muscle groups and joints are necessary. © Georg Thieme Verlag KG Stuttgart · New York.

  15. Toxic myopathies: muscle biopsy features Miopatia tóxica: biópsia muscular

    Directory of Open Access Journals (Sweden)

    Rosana Herminia Scola

    2007-03-01

    Full Text Available Several drugs and toxic substances can cause muscular abnormalities and are frequent causes of acquired myopathies. We present a series of 32 patients, predominance of young adult patients, diagnosed with toxic myopathy. The most common substances inducing myopathy were corticosteroids (56.2% followed by the propoxyphene, neuroleptics, zidovudine and drug-induced hypokalemia. The investigation showed normal serum creatine kinase levels in 65.4%, myopathic pattern of the needle electromyography in 40% and the more frequent histological diagnosis of the muscle biopsy was type 2 fiber atrophy (59.3%. Clinical features, etiology, course of the disease, serum levels of muscular enzymes, electromyographic features and, especially, muscle biopsy features are discussed.Diversos medicamentos e substâncias tóxicas podem causar alterações musculares e são causas freqüentes de miopatia adquirida. Apresentamos uma série de 32 pacientes, predomínio de pacientes adulto jovens, com miopatia tóxica. As substâncias mais relacionadas com a miopatia foram os corticosteróides (56,2% seguidos pelo propoxifeno, neurolépticos, zidovudina e drogas indutoras de hipocalemia. A investigação mostrou níveis normais de creatino quinase sérica em 65,4%, eletromiografia de agulha com padrão miopático em 40% e o mais freqüente diagnóstico histológico da biópsia muscular foi atrofia de fibras do tipo 2 (59,3%. As manifestações clínicas, etiologia, tempo de evolução, nível sérico das enzimas musculares, alterações da eletroneuromiografia e, especialmente, da biópsia muscular são discutidos.

  16. Next Generation Sequencing approach to molecular diagnosis of Duchenne muscular dystrophy; identification of a novel mutation.

    Science.gov (United States)

    Ebrahimzadeh-Vesal, Reza; Teymoori, Atieh; Azimi-Nezhad, Mohsen; Hosseini, Forough Sadat

    2018-02-20

    Duchenne Muscular Dystrophy (DMD; MIM 310200) is one of the most common and severe type of hereditary muscular dystrophies. The disease is caused by mutations in the dystrophin gene. The dystrophin gene is associated with X-linked recessive Duchenne and Becker muscular dystrophy. This disease occurs almost exclusively in males. The clinical symptoms of muscle weakness usually begin at childhood. The main symptoms of this disorder are gradually muscular weakness. The affected patients have inability to standing up and walking. Death is usually due to respiratory infection or cardiomyopathy. In this article, we have reported the discovery of a new nonsense mutation that creates abnormal stop codon in the dystrophin gene. This mutation was detected using Next Generation Sequencing (NGS) technique. The subject was a 17-year-old male with muscular dystrophy that who was suspected of having DMD. He was referred to Hakim medical genetics center of Neyshabur, IRAN. Copyright © 2017. Published by Elsevier B.V.

  17. Antimyosin scintigraphy in patients with acquired and hereditary muscular disorders

    International Nuclear Information System (INIS)

    Loefberg, M.; Liewendahl, K.; Savolainen, S.; Nikkinen, P.; Lamminen, A.; Tiula, E.; Somer, H.

    1994-01-01

    Scintigraphy with indium-111 labelled antimyosin has an established role in the evaluation of cardiac muscle damage. This antibody has been shown to cross-react with myosin in skeletal muscle. We therefore studied the usefulness of this method for the detection of skeletal muscle lesions in rhabdomyolysis, myositis and hereditary muscular dystrophies. All nine patients with rhabdomyolysis had focal uptake of antimyosin antibody which correlated with the clinical findings of soft tissue damage. However, a number of symptomless lesions were also detected by immunoscintigraphy. In rhabdomyolysis the target to non-target uptake ratios varied from 1.3 to 7.6. Diffuse uptake of antibody in skeletal muscle was observed in all three patients with polymyositis-dermatomyositis and in 12 out of 13 patients with muscular dystrophies. In myositis the intensity of antibody accumulation correlated reasonably well with the magnitude of oedema detected by magnetic resonance imaging (MRI). Most patients with Becker type or non-X-chromosomal muscular dystrophies showed slight or moderate uptake of antibody, mainly in the lower extremities. In these patients more antibody accumulated in the calves than in the thighs, whereas the findings on MRI were more prominent in the thighs than in the calves, presumably because of the better preserved muscle bulk in the calves. We conclude that antimyosin scintigraphy can be used for the detection of muscle lesions not only in acquired muscle diseases but also in hereditary muscular disorders, and that immunoscintigraphy provides information on muscle disease activity not obtainable with MRI. (orig.)

  18. Muscular strength and incident hypertension in normotensive and prehypertensive men.

    Science.gov (United States)

    Maslow, Andréa L; Sui, Xuemei; Colabianchi, Natalie; Hussey, Jim; Blair, Steven N

    2010-02-01

    The protective effects of cardiorespiratory fitness (CRF) on hypertension (HTN) are well known; however, the association between muscular strength and incidence of HTN has yet to be examined. This study evaluated the strength-HTN association with and without accounting for CRF. Participants were 4147 men (age = 20-82 yr) in the Aerobics Center Longitudinal Study for whom an age-specific composite muscular strength score was computed from measures of a one-repetition maximal leg and a one-repetition maximal bench press. CRF was quantified by maximal treadmill exercise test time in minutes. Cox proportional hazards regression analysis was used to estimate hazard ratios (HR) and 95% confidence intervals of incident HTN events according to exposure categories. During a mean follow-up of 19 yr, there were 503 incident HTN cases. Multivariable-adjusted (excluding CRF) HR of HTN in normotensive men comparing middle- and high-strength thirds to the lowest third were not significant at 1.17 and 0.84, respectively. Multivariable-adjusted (excluding CRF) HR of HTN in baseline prehypertensive men comparing middle- and high-strength thirds to the lowest third were significant at 0.73 and 0.72 (P = 0.01 each), respectively. The association between muscular strength and incidence of HTN in baseline prehypertensive men was no longer significant after control for CRF (P = 0.26). The study indicated that middle and high levels of muscular strength were associated with a reduced risk of HTN in prehypertensive men only. However, this relationship was no longer significant after controlling for CRF.

  19. Antimyosin scintigraphy in patients with acquired and hereditary muscular disorders

    Energy Technology Data Exchange (ETDEWEB)

    Loefberg, M. (Dept. of Neurology, Helsinki Univ. Central Hospital (Finland)); Liewendahl, K. (Dept. of Clinical Chemistry, Helsinki Univ. Central Hospital (Finland)); Savolainen, S. (Dept. of Clinical Chemistry, Helsinki Univ. Central Hospital (Finland)); Nikkinen, P. (Dept. of Clinical Chemistry, Helsinki Univ. Central Hospital (Finland)); Lamminen, A. (Dept. of Radiology, Helsinki Univ. Central Hospital (Finland)); Tiula, E. (First Dept. of Internal Medicine, Helsinki Univ. Central Hospital (Finland)); Somer, H. (Dept. of Neurology, Helsinki Univ. Central Hospital (Finland))

    1994-10-01

    Scintigraphy with indium-111 labelled antimyosin has an established role in the evaluation of cardiac muscle damage. This antibody has been shown to cross-react with myosin in skeletal muscle. We therefore studied the usefulness of this method for the detection of skeletal muscle lesions in rhabdomyolysis, myositis and hereditary muscular dystrophies. All nine patients with rhabdomyolysis had focal uptake of antimyosin antibody which correlated with the clinical findings of soft tissue damage. However, a number of symptomless lesions were also detected by immunoscintigraphy. In rhabdomyolysis the target to non-target uptake ratios varied from 1.3 to 7.6. Diffuse uptake of antibody in skeletal muscle was observed in all three patients with polymyositis-dermatomyositis and in 12 out of 13 patients with muscular dystrophies. In myositis the intensity of antibody accumulation correlated reasonably well with the magnitude of oedema detected by magnetic resonance imaging (MRI). Most patients with Becker type or non-X-chromosomal muscular dystrophies showed slight or moderate uptake of antibody, mainly in the lower extremities. In these patients more antibody accumulated in the calves than in the thighs, whereas the findings on MRI were more prominent in the thighs than in the calves, presumably because of the better preserved muscle bulk in the calves. We conclude that antimyosin scintigraphy can be used for the detection of muscle lesions not only in acquired muscle diseases but also in hereditary muscular disorders, and that immunoscintigraphy provides information on muscle disease activity not obtainable with MRI. (orig.)

  20. Rapidly worsening bulbar symptoms in a patient with spinobulbar muscular atrophy

    Directory of Open Access Journals (Sweden)

    Montserrat Diaz-Abad

    2013-12-01

    Full Text Available X-linked spinobulbar muscular atrophy (Kennedy’s disease affects muscles and motor neurons, manifesting as weakness and wasting of bulbar, facial, and proximal limb muscles due to loss of anterior horn cells in the brain and spinal cord. We present the case of a patient with X-linked spinobulbar muscular atrophy with rapidly worsening bulbar symptoms caused by laryngopharyngeal irritation associated with a viral upper respiratory tract infection, seasonal allergies and laryngopharyngeal reflux, who dramatically improved with multimodality therapy.

  1. Método de eliminación de trihalometanos y/o contaminantes emergentes mediante plasma

    OpenAIRE

    Erra Serrabasa, Pilar; Jover Comas, Eric; Molina Mansilla, Ricardo; Bertrán Serra, Enric; Bayona Termens, Josep María; Reyes Contreras, Carolina

    2009-01-01

    Método de eliminación de trihalometanos y/o contaminantes emergentes mediante plasma. Se describe un método de eliminación de trihalometanos y contaminantes refractarios en medios acuosos mediante la aplicación directa de plasma para conseguir la degradación de los compuestos contaminantes presentes en el agua.

  2. Muscular dystrophies: key elements for everyday diagnosis and management

    Directory of Open Access Journals (Sweden)

    Alberto Palladino

    2013-12-01

    Full Text Available Muscular dystrophies are a heterogeneous group of inherited disorders that share similar clinical features and dystrophic changes on muscle biopsy, associated with progressive weakness. Weakness may be noted at birth or develop in late adult life. In recent years, cardiac involvement has been observed in a growing number of genetic muscle diseases, and considerable progress has been made in understanding the relationships between disease skeletal muscle and cardiac muscle disease. This review will focus on the skeletal muscle diseases most commonly associated with cardiac complications that can be diagnosed by echocardiography, such as dystrophinopathies including Duchenne (DMD and Becker (BMD muscular dystrophies, cardiomyopathy of DMD/BMD carriers and X-L dilated cardiomyopathy.

  3. Nonmuscular involvement in merosin-negative congenital muscular dystrophy.

    NARCIS (Netherlands)

    Gilhuis, H.J.; Donkelaar, H.J. ten; Tanke, R.B.; Vingerhoets, D.M.; Zwarts, M.J.; Verrips, A.; Gabreëls, F.J.M.

    2002-01-01

    The spectrum of nonmuscular involvement in six children with merosin-negative congenital muscular dystrophy is described. In all children, biochemical, neuroradiologic, cardiac, and neurophysiologic studies were performed. Cerebral structures that were myelinated at gestation, including internal

  4. Modelado de un amortiguador magneto-reológico mediante EcosimPro

    OpenAIRE

    Rodríguez Cadenas, Rubén

    2012-01-01

    El objetivo de este proyecto es la creación de una librería en la herramienta de modelado y simulación EcosimPro enfocada a amortiguadores magneto‐reológicos. El modelado y simulación mediante cualquier herramienta informática permite la obtención de datos y el desarrollo de componentes con un coste inferior al que habría que invertir mediante una experimentación real. Además, permite llevar el componente hasta el límite sin el riesgo de romperlo o dejarlo inutilizable. Por tanto, se puede de...

  5. Intra-muscular hemangioma: A review

    Directory of Open Access Journals (Sweden)

    Shruti Nayak

    2014-01-01

    Full Text Available Intra-muscular hemangiomas (IMH are relatively uncommon benign vascular tumors, which account for less than 1% of all hemangiomas. IMH may be presented as a perceived sporting injury. Diagnosis of this lesion is important not only because of its rarity, but also due to dangers posed by misdiagnosis and mismanagement. They must be considered in the differential diagnosis of unexplained pain and swelling in muscles. IMH occurring in the oral cavity is reviewed below.

  6. Fracture in Duchenne Muscular Dystrophy: Natural History and Vitamin D Deficiency.

    Science.gov (United States)

    Perera, Nadia; Sampaio, Hugo; Woodhead, Helen; Farrar, Michelle

    2016-08-01

    The present study examined the natural history of fracture and vitamin D levels in Duchenne muscular dystrophy patients, who are vulnerable to osteoporosis and fractures. Retrospective analysis of a cohort of 48 Duchenne muscular dystrophy patients revealed that 43% of patients experienced ≥1 fracture. Fracture probabilities at ages 6, 9, 12, and 15 years were 4%, 9%, 31%, and 60% respectively, accelerating around the time of ambulation loss (mean age 11.8 ± 2.7 years). Chronic corticosteroid therapy was utilized in 69% of patients and was associated with all vertebral fractures. A history of vitamin D deficiency occurred in 84%, and 35% were currently deficient. Despite chronic vitamin D supplementation, 38% remained deficient. These results demonstrate that osteoporosis and fracture remain major concerns in Duchenne muscular dystrophy. Bone health should be optimized well before loss of ambulation, however current levels of vitamin D supplementation may be inadequate given high levels of deficiency. © The Author(s) 2016.

  7. Prenatal molecular diagnosis of inherited neuromuscular diseases: Duchenne/Becker muscular dystrophy, myotonic dystrophy type 1 and spinal muscular atrophy.

    Science.gov (United States)

    Esposito, Gabriella; Ruggiero, Raffaella; Savarese, Maria; Savarese, Giovanni; Tremolaterra, Maria Roberta; Salvatore, Francesco; Carsana, Antonella

    2013-12-01

    Neuromuscular disease is a broad term that encompasses many diseases that either directly, via an intrinsic muscle disorder, or indirectly, via a nerve disorder, impairs muscle function. Here we report the experience of our group in the counselling and molecular prenatal diagnosis of three inherited neuromuscular diseases, i.e., Duchenne/Becker muscular dystrophy (DMD/BMD), myotonic dystrophy type 1 (DM1), spinal muscular atrophy (SMA). We performed a total of 83 DMD/BMD, 15 DM1 and 54 SMA prenatal diagnoses using a combination of technologies for either direct or linkage diagnosis. We identified 16, 5 and 10 affected foetuses, respectively. The improvement of analytical procedures in recent years has increased the mutation detection rate and reduced the analytical time. Due to the complexity of the experimental procedures and the high, specific professional expertise required for both laboratory activities and the related counselling, these types of analyses should be preferentially performed in reference molecular diagnostic centres.

  8. CABLES MUSCULARES

    Directory of Open Access Journals (Sweden)

    Alejandro Gómez

    Full Text Available Los cables musculares o fibras de nitinol presentan una excelente alternativa a los actuadores convencionales, con una fuerza de actuación muy alta, equivalente a la de los actuadores hidráulicos, proporcionalmente a su peso, además de su acción silenciosa. Este material, inventado en 1963, aún no es muy conocido y de ahí que se haya realizado una recopilación de sus propiedades. Entre ellas, la temperatura de transición es la más importante, por ser la que activa la aleación. Muchos sistemas se han creado para alcanzar adecuadamente la temperatura de transición, y también se continúa en la investigación de métodos que ayuden a lograr un control preciso del movimiento de la aleación con memoria de forma (SMA.

  9. Becker′s Muscular Dystrophy-A Case Report

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    Rajendran P

    1998-01-01

    Full Text Available A case of Becker′s Muscular dystrophy (BMD in a 26-year-old male is reported. Muscle biopsy immunohistochemical staining showed absence of labelling for dystrophin along the sacrolemmal membrane in majority of the fibres. Antibodies to adhalin and merosin showed normal localisation along the sacrolemma.

  10. Muscular Activities Measurements of Forward Lean and Upright Sitting Motorcycling Postures via Surface Electromyography (sEMG

    Directory of Open Access Journals (Sweden)

    Ma’arof Muhammad Izzat Nor

    2017-01-01

    Full Text Available Motorcycling postures are generically speculated to be physical and physiologically demanding – which in-turn may lead to motorcycling fatigue, and then becoming a possible factor to road accident. The objective of this study was to measure the muscular activities of various motorcycling postures. High muscular activity reading will signifies that motorcycling is indeed physically and physiologically demanding to the motorcyclist. For this particular study, the following postures were tested: i forward lean, ii upright sitting, and iii neutral sitting (as control. Surface electromyography (sEMG measurement was conducted on the following muscles: i extensor carpi radialis, ii upper trapezius iii latissimus dorsi, and iv erector spinae. The results showed that for all test subjects, the muscular activities readings for the forward lean posture was actually close to neutral sitting’s. Whilst, the upright sitting had showed much higher muscular activities measurement instead. Conclusively, this study had proven that any types of discomforts associated with the forward lean posture is not originated from muscular activities. Whereas, confirming that any discomforts in regards to the upright sitting is indeed related to muscular activities. Further studies are warranted to discover the actual risk factors that causes physical and physiological discomforts for the forward lean motorcycling posture.

  11. Fat embolism after fractures in Duchenne muscular dystrophy: an underdiagnosed complication? A systematic review

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    Feder D

    2017-10-01

    Full Text Available David Feder,1 Miriam Eva Koch,1 Beniamino Palmieri,2 Fernando Luiz Affonso Fonseca,1 Alzira Alves de Siqueira Carvalho3 1Pharmacology Department, Faculdade de Medicina do ABC, Santo André, São Paulo, Brazil; 2Department of General Surgery and Surgical Specialties, University of Modena and Reggio Emilia Medical School, Surgical Clinic, Modena, Italy; 3Neuroscience Department, Faculdade de Medicina do ABC, Santo André, São Paulo, Brazil Abstract: Duchenne muscular dystrophy is the most frequent lethal genetic disease. Several clinical trials have established both the beneficial effect of steroids in Duchenne muscular dystrophy and the well-known risk of side effects associated with their daily use. For many years it has been known that steroids associated with ambulation loss lead to obesity and also damage the bone structure resulting in the bone density reduction and increased incidence of bone fractures and fat embolism syndrome, an underdiagnosed complication after fractures. Fat embolism syndrome is characterized by consciousness disturbance, respiratory failure and skin rashes. The use of steroids in Duchenne muscular dystrophy may result in vertebral fractures, even without previous trauma. Approximately 25% of patients with Duchenne muscular dystrophy have a long bone fracture, and 1% to 22% of fractures have a chance to develop fat embolism syndrome. As the patients with Duchenne muscular dystrophy have progressive cardiac and respiratory muscle dysfunction, the fat embolism may be unnoticed clinically and may result in increased risk of death and major complications. Different treatments and prevention measures of fat embolism have been proposed; however, so far, there is no efficient therapy. The prevention, early diagnosis and adequate symptomatic treatment are of paramount importance. The fat embolism syndrome should always be considered in patients with Duchenne muscular dystrophy presenting with fractures, or an unexplained and

  12. Muscular condition and trunk stability in judoka of national and international level

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    Casto Juan-Recio

    2013-12-01

    Full Text Available Background: It is theorized that the development of the ability to stabilize the trunk may improve the performance of a judoka because it improves body balance control and optimizes force transmission from the lower extremities to the upper limbs. However, there is a lack of scientific evidence to establish a clear relationship between trunk stability and performance in judo.Aim: The purpose of this study was to determine whether the quantification of trunk stability and muscular strength and endurance allowed differentiation between national level (n = 7 and international level judoka (n = 6. In addition, the relationship between trunk stability and muscular strength and endurance of the muscles involved in trunk stability control was analyzed.Method: To assess trunk stability, trunk responses to sudden loads applied by a pneumatic mechanism were analyzed, as well as trunk postural control through an unstable sitting paradigm. Muscular strength and endurance were assessed via a flexion and extension trunk test using an isokinetic dynamometer.Results/Conclusions: International level judokas showed lower CoP displacement in the most complex task in unstable seat (7.00 ± 1.19 vs 8.93 ± 1.45 mm, T = .025 and higher absolute and relative peak torque in extensor muscles (7.05 ± 0.87 vs 5.74 ± 0.72 Nm, T = .013 than national level judoka. According to these results, core stability and trunk muscular condition are important qualities in the physical training of elite judoka. Correlational analysis found no relation between the analyzed variables, thus muscular strength and endurance appear to have a non-significant effect on performance in the trunk stability tests.

  13. Inter-rater reliability of the evaluation of muscular chains associated with posture alterations in scoliosis

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    Fortin Carole

    2012-05-01

    Full Text Available Abstract Background In the Global postural re-education (GPR evaluation, posture alterations are associated with anterior or posterior muscular chain impairments. Our goal was to assess the reliability of the GPR muscular chain evaluation. Methods Design: Inter-rater reliability study. Fifty physical therapists (PTs and two experts trained in GPR assessed the standing posture from photographs of five youths with idiopathic scoliosis using a posture analysis grid with 23 posture indices (PI. The PTs and experts indicated the muscular chain associated with posture alterations. The PTs were also divided into three groups according to their experience in GPR. Experts’ results (after consensus were used to verify agreement between PTs and experts for muscular chain and posture assessments. We used Kappa coefficients (K and the percentage of agreement (%A to assess inter-rater reliability and intra-class coefficients (ICC for determining agreement between PTs and experts. Results For the muscular chain evaluation, reliability was moderate to substantial for 12 PI for the PTs (%A: 56 to 82; K: 0.42 to 0.76 and perfect for 19 PI for the experts. For posture assessment, reliability was moderate to substantial for 12 PI for the PTs (%A > 60%; K: 0.42 to 0.75 and moderate to perfect for 18 PI for the experts (%A: 80 to 100; K: 0.55 to 1.00. The agreement between PTs and experts was good for most muscular chain evaluations (18 PI; ICC: 0.82 to 0.99 and PI (19 PI; ICC: 0.78 to 1.00. Conclusions The GPR muscular chain evaluation has good reliability for most posture indices. GPR evaluation should help guide physical therapists in targeting affected muscles for treatment of abnormal posture patterns.

  14. Radiographic and ultrasonographic features of hypertrophic feline muscular dystrophy in two cats

    International Nuclear Information System (INIS)

    Berry, C.R.; Gaschen, F.P.; Ackerman, N.

    1992-01-01

    Hypertrophic fellne musculer dystrophy has been reported as an X-linked inherited deficiency of a cytoskeletal myofiber protein called dystrophin. This report deserlbes the radiographic and ultrasonographic abnormalities of two male littermate domestic short-hair cats and reviews the previous reported findings assoclated with hypertrophic feline muscular dystrophy. The thoracic radiographic abnormalities included: progressive cardiomegaly, large convex, scalloped irregularities associated with the vetral aspect of the diaphragm, and variable degrees of esophageal dilation (megaesophagus) with associated cranioventral aspiration pneumonia. Echocardiographic features included: concentric left vetricular wall thickening, increased left ventricular and diastolic and systolic dimensions, and an increase in endocardial echogenicity. Abdominal radiographic abnormalities included: hepatosplenomegaly, peritoneal effusion, renomegaly, adrenal gland mineralization, and paralumbar and diaphragmatic musculature enlargement. Abdomlnal ultrasonographic abnormalities included: irregularly thickened muscular portion of the diaphragm; hypoechogenicity of the liver; peritoneal effusion; hepatosplenomegaly; renomegaly with hyperechoic cortex and medulla; and adrenal gland mineralization. The irregular scalloped appearance of the diaphragm (particularly along the ventral/sternal margin) was a consistenl radiographic abnormlity in the two cats with hypertrophic feline muscular dystrophy after the age of 7 months. This finding was confirmed by ultrasound as a thickened irregular, hyperechoic diaphragm. A diagnosis of hypertrophic feline muscular dystrophy should be strongly suspected if this abnormality is identified

  15. Emery-Dreifuss muscular dystrophy: the most recognizable laminopathy

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    Agnieszka Madej-Pilarczyk

    2016-03-01

    Full Text Available Emery-Dreifuss muscular dystrophy (EDMD, a rare inherited disease, is characterized clinically by humero-peroneal muscle atrophy and weakness, multijoint contractures, spine rigidity and cardiac insufficiency with conduction defects. There are at least six types of EDMD known so far, of which five have been associated with mutations in genes encoding nuclear proteins. The majority of the EDMD cases described so far are of the emerinopathy (EDMD1 kind, with a recessive X-linked mode of inheritance, or else laminopathy (EDMD2, with an autosomal dominant mode of inheritance. In the work described here, the authors have sought to describe the history by which EDMD came to be distinguished as a separate entity, as well as the clinical and genetic characteristics of the disease, the pathophysiology of lamin-related muscular diseases and, finally, therapeutic issues, prevention and ethical aspects.

  16. Psychometric Properties of the Drive for Muscularity Attitudes Questionnaire Among Irish Men

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    Travis A. Ryan

    2014-09-01

    Full Text Available The Drive for Muscularity Attitudes Questionnaire (DMAQ was developed to measure men’s desire to attain an idealized muscular body. To date, the cross-cultural suitability of this measure has received limited attention. The current study addressed this omission by testing the psychometric properties of the DMAQ using an online sample of Irish men (N = 327. Confirmatory factor analysis revealed that a unidimensional model adequately matched observed data (i.e., fit indices suggested acceptable model fit. Analyses also showed that the DMAQ yielded reliable and construct valid scores, suggesting that the scale holds promise as an indicant of the drive for muscularity among Irish men. Strengths and limitations associated with this study are discussed, such as advantages and disadvantages of Internet research. Directions for future research are given, including the need for more psychometric work.

  17. Respiratory muscle training in Duchenne muscular dystrophy.

    OpenAIRE

    Rodillo, E; Noble-Jamieson, C M; Aber, V; Heckmatt, J Z; Muntoni, F; Dubowitz, V

    1989-01-01

    Twenty two boys with Duchenne muscular dystrophy were entered into a randomised double blind crossover trial to compare respiratory muscle training with a Triflow II inspirometer and 'placebo' training with a mini peak flow meter. Supine posture was associated with significantly impaired lung function, but respiratory muscle training showed no benefit.

  18. Cardiac Complications of Fukuyama-Type Congenital Muscular Dystrophy

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    J Gordon Millichap

    2006-07-01

    Full Text Available The course of left ventricular function was evaluated using M-mode and Doppler echocardiography in 34 patients with Fukuyama-type congenital muscular dystrophy (FCMD, in a study at the Tokyo Women’s Medical University, Tokyo, Japan.

  19. Strength training and albuterol in facioscapulohumeral muscular dystrophy

    NARCIS (Netherlands)

    van der Kooi, EL; Vogels, OJM; van Asseldonk, RJGP; Lindeman, E; Hendriks, JCM; Wohlgemuth, M; van der Maarel, SM; Padberg, GW

    2004-01-01

    Background: In animals and healthy volunteers beta2-adrenergic agonists increase muscle strength and mass, in particular when combined with strength training. In patients with facioscapulohumeral muscular dystrophy (FSHD) albuterol may exert anabolic effects. The authors evaluated the effect of

  20. Composición corporal en escolares de General Alvear

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    Sucre, María Leonor

    2007-01-01

    Full Text Available El objetivo del presente trabajo es analizar la composición corporal en niños con diferente estado nutricional. Se realizó un estudio transversal en 2997 escolares de ambos sexos comprendidos entre los 5 y los 12 años residentes en la ciudad de General Alvear (Mendoza. Se relevaron peso corporal, estatura total y sentado, perímetro braquial y pliegues subescapular y tricipital. Se calcularon las prevalencias de desnutrición y sobrepeso, los índices de masa corporal, subescapular/tricipital (IST y las áreas muscular y adiposa. Las prevalencias obtenidas indicaron que, mientras la desnutrición tuvo porcentajes cercanos al 8%, el sobrepeso alcanzó el 14%. El 62% de los escolares desnutridos y el 37% con sobrepeso presentaron déficit de tejido muscular. En el grupo de niños desnutridos no hubo exceso de tejido adiposo, en tanto que en aquellos con sobrepeso alcanzó el 41% con un patrón de distribución predominantemente centralizado. El 45% de ellos registró adiposidad abdominal y el 20% riesgo de padecerlo. Los resultados obtenidos permiten identificar dos situaciones problemáticas: la reducción del tejido muscular en ambos extremos de la malnutrición y la distribución centralizada de la adiposidad en el grupo con sobrepeso que puede asociarse a un mayor riesgo de padecer enfermedades crónicas no transmisibles.

  1. Terapia em paciente com disfunção temporomandibular muscular

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    Aline Avrella

    2015-11-01

    Full Text Available Objetivo. Relatar um caso clínico de uma paciente jovem com disfunção temporomandibular muscular (DTM. Materiais e métodos. O estudo foi realizado em uma clínica odontológica de ensino superior, utilizando a aplicação de calor úmido, exercícios ativos e agulhamento seco. Resultados. Paciente feminina, 31 anos, relatando dor na região parotídea-massetérica bilateral, sendo diagnosticada com DTM muscular bilateral em masseter com presença de pontos gatilho. Para o tratamento foi realizado a termoterapia pela utilização do calor úmido, fisioterapia com exercícios ativos de relaxamento, agulhamento seco utilizando agulhas filiformes sólidas e, Terapia Cognitiva Comportamental, com melhora importante referida pela paciente. Discussão. Dentre as formas de tratamento para DTM muscular encontram-se as técnicas utilizadas com a paciente em estudo, as quais proporcionando uma melhora biopsicossocial. Conclusão. As técnicas de tratamento realizadas foram efetivas para o tratamento da paciente, proporcionando alívio da dor e melhora nos sintomas.

  2. Composite biomarkers for assessing Duchenne muscular dystrophy: an initial assessment.

    Science.gov (United States)

    Shklyar, Irina; Pasternak, Amy; Kapur, Kush; Darras, Basil T; Rutkove, Seward B

    2015-02-01

    Compared with individual parameters, composite biomarkers may provide a more effective means for monitoring disease progression and the effects of therapy in clinical trials than single measures. In this study, we built composite biomarkers for use in Duchenne muscular dystrophy by combining values from two objective measures of disease severity: electrical impedance myography and quantitative ultrasound and evaluating how well they correlated to standard functional measures. Using data from an ongoing study of electrical impedance myography and quantitative ultrasound in 31 Duchenne muscular dystrophy and 26 healthy boys aged 2-14 years, we combined data sets by first creating z scores based on the normal subject data and then using simple mathematical operations (addition and multiplication) to create composite measures. These composite scores were then correlated to age and standard measures of function including the 6-minute walk test, the North Star Ambulatory Assessment, and handheld dynamometry. Combining data sets resulted in stronger correlations with all four outcomes than for either electrical impedance myography or quantitative ultrasound alone in six of eight instances. These improvements reached statistical significance (P Duchenne muscular dystrophy clinical trials is warranted. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Amiloidose manifestando-se com pseudo-hipertrofia muscular Amyloidosis presenting as muscle pseudohypertrophy

    Directory of Open Access Journals (Sweden)

    Rodrigo Bortoli

    2007-12-01

    Full Text Available Amiloidose tipo AL é uma doença rara causada pela deposição extracelular de fragmentos de cadeias leves monoclonais em órgãos e tecidos. Pode apresentar-se com uma ampla variedade de sinais e sintomas, e o acometimento cutâneo-muscular, simulando pseudo-hipertrofia muscular, é um achado muito raro. São descritos dois casos que apresentaram tal manifestação. CASO 1 - Mulher, 61 anos, há quatro meses com história de mialgia e aumento da massa muscular nas cinturas pélvica, escapular e região cervical. Não havia alterações significativas ao exame físico, exceto aparente hipertrofia muscular difusa e discreta macroglossia. CASO 2 - Homem, 51 anos, há dois anos com cansaço e espessamento cutâneo progressivo do dorso, pescoço e braços. Em outros serviços levantou suspeitas diagnósticas de esclerodermia ou de escleredema de Buschke; desde fevereiro de 2007 passou a ser acompanhado nesse serviço e referia, havia cerca de um ano, disfagia para sólidos, disartria e dificuldade para movimentar a língua. Chamava atenção em seu exame o porte físico atlético com musculatura torácica proeminente, porém referia não fazer exercícios físicos. Em ambos os casos, a biópsia cutânea foi realizada com identificação do depósito amilóide por meio da coloração de vermelho congo.AL amyloidosis is a rare disease secondary to extracellular deposition of light chains fragments in organs and tissues. It can cause a wide variety of signs and symptoms, being the muscular pseudohypertrophy form a very rare finding. CASE 1 - a 61-year-old female had a history of myalgia and increase of muscular mass on pelvic and scapular girdle and cervical region. Besides the generalized muscular hypertrophy and discrete macroglossia, the rest of physical examination was normal. CASE 2 - a 51-year-old male complained of tiredness and progressive cutaneous thickening on his thorax, neck and arms for the last two years. Initially, he was misdiagnosed

  4. Visuospatial Attention Disturbance in Duchenne Muscular Dystrophy

    Science.gov (United States)

    De Moura, Maria Clara Drummond Soares; do Valle, Luiz Eduardo Ribeiro; Resende, Maria Bernadete Dutra; Pinto, Katia Osternack

    2010-01-01

    Aim: The cognitive deficits present in the Duchenne muscular dystrophy (DMD) are not yet well characterized. Attention, considered to be the brain mechanism responsible for the selection of sensory stimuli, could be disturbed in DMD, contributing, at least partially, to the observed global cognitive deficit. The aim of this study was to…

  5. Duchenne muscular dystrophy models show their age

    OpenAIRE

    Chamberlain, Jeffrey S.

    2010-01-01

    The lack of appropriate animal models has hampered efforts to develop therapies for Duchenne muscular dystrophy (DMD). A new mouse model lacking both dystrophin and telomerase (Sacco et al., 2010) closely mimics the pathological progression of human DMD and shows that muscle stem cell activity is a key determinant of disease severity.

  6. Unusual Respiratory Manifestations in Two Young Adults with Duchenne Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Julie Lemay

    2012-01-01

    Full Text Available Adult respirologists are often involved in the evaluation and treatment of young adult patients with Duchenne muscular dystrophy. In this context, the most frequent respiratory complication is nocturnal and daytime hypoventilation related to respiratory muscle weakness. The present article describes cases of Duchenne muscular dystrophy involving two brothers, 17 and 19 years of age, respectively, who presented with less frequently reported respiratory complications of their disease: obstructive sleep apnea and Cheyne-Stokes respiration with central apnea, which were believed to be partially or completely related to congestive cardiomyopathy.

  7. Imaging of muscular denervation secondary to motor cranial nerve dysfunction

    International Nuclear Information System (INIS)

    Connor, S.E.J.; Chaudhary, N.; Fareedi, S.; Woo, E.K.

    2006-01-01

    The effects of motor cranial nerve dysfunction on the computed tomography (CT) and magnetic resonance imaging (MRI) appearances of head and neck muscles are reviewed. Patterns of denervation changes are described and illustrated for V, VII, X, XI and XII cranial nerves. Recognition of the range of imaging manifestations, including the temporal changes in muscular appearances and associated muscular grafting or compensatory hypertrophy, will avoid misinterpretation as local disease. It will also prompt the radiologist to search for underlying cranial nerve pathology, which may be clinically occult. The relevant cranial nerve motor division anatomy will be described to enable a focussed search for such a structural abnormality

  8. Imaging of muscular denervation secondary to motor cranial nerve dysfunction

    Energy Technology Data Exchange (ETDEWEB)

    Connor, S.E.J. [Neuroradiology Department, Kings College Hospital, Denmark Hill, London SE5 9RS (United Kingdom)]. E-mail: sejconnor@tiscali.co.uk; Chaudhary, N. [Neuroradiology Department, Kings College Hospital, Denmark Hill, London SE5 9RS (United Kingdom); Fareedi, S. [Neuroradiology Department, Kings College Hospital, Denmark Hill, London SE5 9RS (United Kingdom); Woo, E.K. [Neuroradiology Department, Kings College Hospital, Denmark Hill, London SE5 9RS (United Kingdom)

    2006-08-15

    The effects of motor cranial nerve dysfunction on the computed tomography (CT) and magnetic resonance imaging (MRI) appearances of head and neck muscles are reviewed. Patterns of denervation changes are described and illustrated for V, VII, X, XI and XII cranial nerves. Recognition of the range of imaging manifestations, including the temporal changes in muscular appearances and associated muscular grafting or compensatory hypertrophy, will avoid misinterpretation as local disease. It will also prompt the radiologist to search for underlying cranial nerve pathology, which may be clinically occult. The relevant cranial nerve motor division anatomy will be described to enable a focussed search for such a structural abnormality.

  9. Crecimiento y desarrollo de una población infanto juvenil de la provincia de Chubut

    Directory of Open Access Journals (Sweden)

    Torres, María Fernanda

    1999-01-01

    Full Text Available La diferenciación y adaptación ontogenética son temas centrales en antropología biológica por cuanto su modificación puede afectar la dinámica evolutiva poblacional. Numerosos factores estresantes actúan provocando variación en la expresión fenotípica y nuevas respuestas adaptativas. En el presente trabajo se determinó el crecimiento de niños de 5 a 14 años contrastando con estándares internacionales. La muestra se constituyó con 473 escolares de Río Mayo (Chubut, de ambos sexos. Se relevaron y calcularon variables corporales. Para la comparación con los estándares los datos fueron convertidos a escores z y contrastados además mediante pruebas de Wilcoxon. La estadística empleada consistió en análisis multifactorial de la varianza y pruebas de LSD. Si bien hubo crecimiento en todos los grupos etáreos analizados, los valores obtenidos difirieron significativamente de los correspondientes al estándar. En varones, a excepción del pliegue subescapular, que presentó escores z superiores al estándar las restantes variables se ubicaron entre 0 y -2 DS, siendo más afectadas las estaturas total y sentado y el área muscular. En mujeres, se observó una situación similar a excepción del área adiposa y la masa corporal, con valores mayores al estándar. Estos resultados evidencian la presencia de una desnutrición que abarca toda la franja etárea. La disminución de la talla y el aumento del tejido adiposo constituyen sin duda, respuestas adaptativas que permiten la supervivencia de los individuos frente a la injuria nutricional.

  10. Estudo do metabolismo energético muscular em atletas por 31P-ERM Muscular energetic metabolism study in athletes by 31P-MRS

    Directory of Open Access Journals (Sweden)

    Maria Gisele dos Santos

    2004-04-01

    Full Text Available OBJETIVO: Caracterizar as reservas energéticas de metabólitos fosforilados no músculo esquelético de atletas mediante 31P-ERM. MÉTODOS: Amostra deste estudo foi formada por 14 atletas de alto nível do Centro de Alto Rendimento Esportivo (CAR, Sant Cugat del Vallés, Espanha. O padrão de metabólitos fosforilados foi medido no músculo vasto medial por 31P-ERM. A suplementação oral foi realizada durante 14 dias, na forma de 20g de monohidrato de creatina. Os atletas foram determinados conforme as suas características físicas (peso, altura, índice de massa corporal (IMC, consumo máximo de oxigênio (VO2 Max. em dois grupos: placebo (maltodextrina e suplementação com creatina. O protocolo de exercício foi realizado no interior do túnel de ressonância (160 x 52 cm, a 60 ciclos por minuto para ambas as pernas. RESULTADOS: Os resultados demonstraram um aumento significativo da fosfocreatina (PCr durante o exercício, após o período de suplementação, denotando uma redução do seu consumo no grupo que recebeu suplementação com creatina; não houve diminuição significativa do pH intracelular e fosfato inorgânico após a suplementação. CONCLUSÃO: O protocolo de exercício realizado pelos fundistas no Centro de Diagnóstico de Pedralbes permitiu detectar mediante 31P-ERM, no grupo que foi suplementado com creatina, uma diminuição do consumo de PCr durante os períodos de exercício.BACKGROUND: The aim of this study was to characterize the muscular reservoirs of phosphorilated energetic components of athletes using 31P-MRS. METHODS: The sample was formed by 14 elite athletes from the Center for High Sportive Performance (CAR, Sant Cugat del Vallés, Spain. The pattern of the phosphorilated metabolites was measured from the muscle vastus medialis by 31P-MRS. Oral supplementation of 20 g of Creatine monohydrate was given during 14 days. Two groups of athletes were formed according to their physical characteristics (weight

  11. A Human Pluripotent Stem Cell Model of Facioscapulohumeral Muscular Dystrophy-Affected Skeletal Muscles.

    Science.gov (United States)

    Caron, Leslie; Kher, Devaki; Lee, Kian Leong; McKernan, Robert; Dumevska, Biljana; Hidalgo, Alejandro; Li, Jia; Yang, Henry; Main, Heather; Ferri, Giulia; Petek, Lisa M; Poellinger, Lorenz; Miller, Daniel G; Gabellini, Davide; Schmidt, Uli

    2016-09-01

    : Facioscapulohumeral muscular dystrophy (FSHD) represents a major unmet clinical need arising from the progressive weakness and atrophy of skeletal muscles. The dearth of adequate experimental models has severely hampered our understanding of the disease. To date, no treatment is available for FSHD. Human embryonic stem cells (hESCs) potentially represent a renewable source of skeletal muscle cells (SkMCs) and provide an alternative to invasive patient biopsies. We developed a scalable monolayer system to differentiate hESCs into mature SkMCs within 26 days, without cell sorting or genetic manipulation. Here we show that SkMCs derived from FSHD1-affected hESC lines exclusively express the FSHD pathogenic marker double homeobox 4 and exhibit some of the defects reported in FSHD. FSHD1 myotubes are thinner when compared with unaffected and Becker muscular dystrophy myotubes, and differentially regulate genes involved in cell cycle control, oxidative stress response, and cell adhesion. This cellular model will be a powerful tool for studying FSHD and will ultimately assist in the development of effective treatments for muscular dystrophies. This work describes an efficient and highly scalable monolayer system to differentiate human pluripotent stem cells (hPSCs) into skeletal muscle cells (SkMCs) and demonstrates disease-specific phenotypes in SkMCs derived from both embryonic and induced hPSCs affected with facioscapulohumeral muscular dystrophy. This study represents the first human stem cell-based cellular model for a muscular dystrophy that is suitable for high-throughput screening and drug development. ©AlphaMed Press.

  12. Changes in muscular fitness and its association with blood pressure in adolescents.

    Science.gov (United States)

    Agostinis-Sobrinho, César; Ruiz, Jonatan R; Moreira, Carla; Lopes, Luís; Ramírez-Vélez, Robinson; García-Hermoso, Antonio; Mota, Jorge; Santos, Rute

    2018-05-08

    The aims of this study were to examine the longitudinal association between muscular fitness (MF) and blood pressure (BP) 2 years later, and to determine whether changes in MF over a 2-year period were associated with BP at follow-up, in adolescents. The sample comprised 734 youths (349 girls) aged from 12 to 18 years. MF was assessed with the standing long jump and handgrip tests. Socioeconomic status, pubertal stage, waist circumference, resting BP, and cardiorespiratory fitness were measured according to standard procedures. Regression analyses showed a significant inverse association between MF at baseline and systolic BP (β = - 0.072; p = 0.032) and rate pressure product (β = - 0.124; p fitness, these associations did not remain significant. Adolescents with persistently high and increasing MF exhibited the lowest levels of diastolic BP (F (3, 721)  = 3.814, p = 0.018) and systolic BP (F (3, 721)  = 3.908, p = 0.014) when compared to those with persistent low MF after adjustment for age, sex, height, socioeconomic status, cardiorespiratory fitness, and waist circumference. This study suggests that persistent greater and increasing MF in youth are associated with lower levels of BP across the adolescence. What is Known: • Currently, there is a growing interest on the health benefits of muscular fitness. • Cross-sectional studies have identified an association between muscular fitness and blood pressure in adolescents. What is New: • Changes in muscular fitness during adolescence were associated with systolic and diastolic BP over a 2-year period. • Adolescents with persistently low muscular fitness exhibited the highest levels of diastolic and systolic BP.

  13. Distribuição de gordura corporal, pressão arterial e níveis de lipídios-lipoproteínas plasmáticas Body fat distribution, blood pressure and plasma lipids and lipoprotein levels

    Directory of Open Access Journals (Sweden)

    Dartagnan Pinto Guedes

    1998-02-01

    Full Text Available OBJETIVO: Investigar associações entre distribuição do tecido adiposo e níveis de pressão arterial e concentrações de lipídios-lipoproteínas plasmáticas, mediante controle de indicadores, quanto à quantidade de gordura corporal e à prática da atividade física. MÉTODOS: Estudo de 62 indivíduos com idades entre 20 e 45 anos. A distribuição do tecido adiposo foi determinada baseando-se na relação circunferência de cintura/quadril (CCQ, e como indicador da quantidade de gordura corporal recorreu-se às informações do índice de massa corporal (IMC, enquanto o nível de prática da atividade física foi estabelecido mediante estimativas do consumo máximo de oxigênio (VO2max. As associações entre CCQ e níveis de pressão arterial e de lipídios-lipoproteínas plasmáticas, com os efeitos do IMC e do VO2max controlados estatisticamente, foram estabelecidas pelo coeficiente de correlação parcial. RESULTADOS: Após correção pelo IMC verificou-se significativa correlação parcial entre a distribuição centrípeta do tecido adiposo e os níveis de pressão arterial, LDL-C e triglicerídios plasmáticos. Entretanto, controlando-se o VO2max, não foram constatadas associações significativas entre CCQ e qualquer variável sangüínea e pressão arterial.CONCLUSÃO: A distribuição centrípeta do tecido adiposo, independente da quantidade de gordura corporal, foi relacionada com concentrações de lipídios-lipoproteínas plasmáticas e níveis de pressão arterial em ambos os sexos. A prática da atividade física parece ser um importante modulador dessa associação, enfatizando seu papel no controle dos fatores de risco predisponentes às doenças cardiovasculares.PURPOSE: To study associations between FAT distribution and blood pressure levels and concentrations of lipids and lipoproteins, irrespective of body fat content and physical activity. METHODS: A sample of 62 subjects of both genders aging 20-45 years-old was

  14. Avaliação da função motora em crianças com distrofia muscular congênita com deficiência da merosina Motor function evaluation in merosin-deficient congenital muscular dystrophy children

    Directory of Open Access Journals (Sweden)

    Fernanda M. Rocco

    2005-06-01

    Full Text Available A distrofia muscular congênita (DMC compõe um grupo de miopatias caracterizadas por hipotonia e fraqueza muscular notadas até o primeiro ano de vida. Em torno de 40% a 50% dos casos são decorrentes de deficiência primária da proteína merosina (DM, os quais apresentam um fenótipo mais homogêneo, com grave comprometimento motor e respiratório. Foram avaliadas neste estudo onze crianças com diagnóstico clínico e histológico de DMC-DM, com idade de 3 a 15 anos, através de exame de força muscular ("Medical Research Council", análise goniométrica, avaliação das habilidades motoras e das atividades de vida diária (AVDs (indicador de Barthel, com o objetivo de caracterizar as principais limitações funcionais motoras. Os grupos musculares mais comprometidos foram os flexores cervicais, paravertebrais e proximais dos membros. Os grupos musculares dos membros superiores estavam tão comprometidos quanto os dos membros inferiores, enquanto que os extensores encontravam-se mais comprometidos que os flexores. Todas as crianças apresentavam importantes retrações musculares nos quadris, joelhos e cotovelos. Outras deformidades freqüentes foram escoliose e pés eqüino-varo. Nenhuma criança possuía a habilidade motora necessária para engatinhar, ficar de pé ou andar; e todas foram classificadas como dependentes ou semidependentes para a maioria das AVDs estudadas. Nossos achados confirmam o envolvimento difuso e intenso da musculatura esquelética na DMC-DM, acarretando graves limitações funcionais motoras e deformidades músculo-esqueléticas.Congenital muscular dystrophy (CMD is a heterogeneous group of disorders characterized by early onset of hypotonia and weakness. Almost 50% of the cases are caused by primary deficiency of a protein named merosin (MD, and present a homogenous phenotype with a severe motor and respiratory involvement. Eleven children with clinical and histological diagnosis of CMD-MD, aged of 3 to 15 years

  15. Muscular anatomy of the Podocoryna carnea hydrorhiza.

    Science.gov (United States)

    Buss, Leo W; Anderson, Christopher; Bolton, Edward W

    2013-01-01

    The muscular anatomy of the athecate hydroid Podocoryna carnea hydrorhiza is elucidated. The polyp-stolon junction is characterized by an opening, here called the chloe, in the otherwise continuous hydrorhizal perisarc. The chloe is elliptical when the polyp first arises, but takes on a more complex outline as multiple stolons anastomose to communicate with that polyp. Surrounding the polyp base are spots, here called anchors, which autofluoresce at the same wavelengths as perisarc and which, like perisarc, contain chitin as assessed by Calcofluor White, Congo Red and wheat germ agglutinin staining. Anchors remain after living tissues are digested using KOH. Collagen IV staining indicates that the mesoglea is pegged to the anchors and rhodamine phallodin staining detects cytoskeletal F-actin fibers of the basal epidermis surrounding the anchors. Longitudinal muscle fibers of the polyp broaden at the polyp base and are inserted into the mesoglea of the underlying stolon, but were neither observed to extend along the stolonal axis nor to attach to the anchors. Circular muscular fibers of the polyp extend into stolons as a dense collection of strands running along the proximal-distal axis of the stolon. These gastrodermal axial muscular fibers extend to the stolon tip. Epidermal cells at the stolon tip and the polyp bud display a regular apical latticework of F-actin staining. A similar meshwork of F-actin staining was found in the extreme basal epidermis of all stolons. Immunohistochemical staining for tubulin revealed nerves at stolon tips, but at no other hydrorhizal locations. These studies bear on the mechanisms by which the stolon tip and polyp bud pulsate, the manner in which the stolon lumen closes, and on the developmental origin of the basal epidermis of the hydrorhiza.

  16. Cardiac abnormalities in a follow-up study on carriers of Duchenne and Becker muscular dystrophy

    NARCIS (Netherlands)

    van Westrum, S. M. Schade; Hoogerwaard, E. M.; Dekker, L.; Standaar, T. S.; Bakker, E.; Ippel, P. F.; Oosterwijk, J. C.; Majoor-Krakauer, D. F.; van Essen, A. J.; Leschot, N. J.; Wilde, A. A. M.; de Haan, R. J.; de Visser, M.; van der Kooi, A. J.

    Objectives: Cardiac involvement has been reported in carriers of dystrophin mutations giving rise to Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). The progress of these abnormalities during long-term follow-up is unknown. We describe the long-term follow-up of dilated

  17. Cardiac abnormalities in a follow-up study on carriers of Duchenne and Becker muscular dystrophy

    NARCIS (Netherlands)

    Schade van Westrum, S. M.; Hoogerwaard, E. M.; Dekker, L.; Standaar, T. S.; Bakker, E.; Ippel, P. F.; Oosterwijk, J. C.; Majoor-Krakauer, D. F.; van Essen, A. J.; Leschot, N. J.; Wilde, A. A. M.; de Haan, R. J.; de Visser, M.; van der Kooi, A. J.

    2011-01-01

    Objectives: Cardiac involvement has been reported in carriers of dystrophin mutations giving rise to Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). The progress of these abnormalities during long-term follow-up is unknown. We describe the long-term follow-up of dilated

  18. Evaluation of myocardial and skeletal muscular involvement with thallium-201 myocardial emission computed tomography and whole body scintigraphy

    International Nuclear Information System (INIS)

    Yamamoto, Shuhei; Matsushima, Hideo; Sotobata, Iwao; Suzuki, Akio; Indo, Toshikatsu; Matsuoka, Yukihiko

    1986-01-01

    Thallium-201 (Tl-201) myocardial emission computed tomography and whole body scintigraphy were performed using a rotating gamma camera in 64 patients with neurologic disease and 14 normal subjects. Thallium-201 myocardial perfusion defects were seen in 40 % of the muscular involvement in 47 patients with muscular dystrophy (MD), in whom morphological abnormality of the heart was common. There was strong relationship between the degree of left ventricular perfusion defects and the degree of pulmonary uptake of Tl-201. Thallium-201 whole body scintigraphy showed homogeneous distribution of Tl-201 in the extremities in normal subjects, and perfusion defects in 73 % of the muscular lesions in MD patients. Muscular and skeletal lesions for MD appear to progress independently. Thallium-201 imaging seems to be of clinical value in assessing the muscular and skeletal lesions. (Namekawa, K.)

  19. Sensado de variables mediante terminal Android

    OpenAIRE

    Altaba Rosas, Mar

    2017-01-01

    El presente documento describe los procesos de diseño y desarrollo de un sistema que, a través de una aplicación móvil, sirve como dispositivo para el registro de la actividad cardíaca del paciente, mediante la obtención del electrocardiograma (ECG), y que permite detectar irregularidades para posteriormente, en caso que fuera necesario, poder enviar los datos adquiridos al profesional sanitario pertinente para que éste los analice. El sistema tiene dos componentes diferenciados, por un lado,...

  20. Variables de la desnutrición en pacientes en diálisis

    Directory of Open Access Journals (Sweden)

    Luis Garrido Pérez

    Full Text Available Introducción: El paciente en diálisis va a sufrir una desnutrición proteico-calórica, con diferentes factores implicados en su aparición, lo cual se asocia con una elevadísima morbilidad cardiovascular y mortalidad. Se ha estimado una prevalencia de desnutrición en la población en hemodiálisis del 18-75%, siendo por tanto, un problema de especial relevancia en este tipo de pacientes. Objetivo: Realizar una revisión bibliográfica de los artículos científicos existentes sobre las variables que intervienen en la desnutrición del paciente en diálisis. Metodología: Se ha realizado una revisión bibliográfica mediante las bases de datos PubMed, Scielo, Pro- Quest. La búsqueda se ha realizado con términos Mesh, con una antigüedad no mayor de 5 años y con distintas palabras clave. Resultados: Se han revisado 19 artículos. La mayoría de los artículos fueron estudios observacionales y de revisión. Los factores que se asocian con desnutrición son la edad, pérdida de masa muscular, baja actividad física y dieta pobre en micronutrientes. Otro factor muy importante, es la inflamación. En cuanto a los métodos diagnósticos, son variados y diferentes, debido a la gran cantidad de variables que influyen en la desnutrición. Conclusiones: La desnutrición en pacientes en diálisis depende de distintas variables y no solamente de la dieta. Los factores que se asocian con desnutrición son mayor edad, pérdida de masa muscular, baja actividad física y dieta pobre en micronutrientes. Además, habría que añadir el doble papel que juega la inflamación en este proceso, pues puede ser tanto consecuencia como factor predisponente a la desnutrición.

  1. Media exposure, mediated social comparison to idealized images of muscularity, and anabolic steroid use.

    Science.gov (United States)

    Melki, Jad P; Hitti, Eveline A; Oghia, Michael J; Mufarrij, Afif A

    2015-01-01

    This study examined the association between anabolic-androgenic steroid (AAS) use and dominant sociocultural factors, specifically media exposure to idealized images of male muscularity, and mediated social comparison trends among a sample of young Arab adults. The study found evidence that participants more exposed to content that promotes muscularity and those who idealize images of muscularity and perceive them as motivators for achieving muscularity are more likely to be AAS users. It also found that a significant percentage of participants used at least one kind of dietary supplement and that the level of AAS use among health club participants indicates it is a significant public health problem in Lebanon. The study suggests that dealing with this problem requires a unique approach, beyond the typical awareness of risks strategy, since some users were well aware of the risks yet continue to use AAS, and their motivations pertain more to body image and sexuality. A stronger approach that utilizes critical media literacy teaching that ingrains these issues into school and university curricula will have a more lasting impact.

  2. Imagem radiográfica da cavidade torácica de cães Golden Retriever acometidos pela distrofia muscular Radiologic images of the thoracic cavity of Golden Retriever dogs affected by muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Flávio R. Alves

    2009-02-01

    Full Text Available A distrofia muscular de Duchenne (DMD é uma doença de origem genética, cuja principal manifestação clínica é enfraquecimento e atrofia progressiva dos músculos. Os cães da raça Golden Retriever podem apresentar distrofia muscular, com características genotípicas e fenotípicas muito próximas à distrofia muscular humana, sendo considerado o modelo animal mais apropriado para o estudo da DMD. Foram realizadas radiografias torácicas látero-laterais e dorsoventrais de 10 cães Golden Retriever afetados pela distrofia muscular, com o objetivo de relatar as alterações radiográficas associadas a essa patologia. O exame radiográfico da cavidade torácica evidenciou: (a padrão pulmonar intersticial e alveolar predominante, (b um quadro de pneumonia e edema pulmonar em fase inicial, (c a cardiomegalia como o principal achado de comprometimento circulatório na cavidade torácica, (d O megaesôfago torácico foi observado deslocando a traquéia e silhueta cardíaca ventralmente e, (e a cúpula diafragmática apresentou modificação morfológica, mostrando protrusão para o interior da cavidade torácica e hérnia hiatal, com deslocamento do estômago para o espaço mediastino caudal. Os achados de necropsia evidenciaram efusão pleural e enfisema pulmonar e lesões compatíveis com processos degenerativos e metaplásicos da musculatura diafragmática e intercostal. A avaliação radiográfica constituiu-se como um meio diagnóstico auxiliar essencial na identificação de doença cardíaca e respiratória em cães Golden Retriever acometidos pela Distrofia Muscular, capaz de identificar processos pneumônicos primários, permitindo o estabelecimento de terapêutica adequada de tratamento, com prognóstico reservado nos estágios mais avançados desta alteração.Duchenne Muscular Dystrophy (DMD is a genetic disorder with clinical signs of muscular weaknesses and progressive atrophy. Golden Retriever dogs show similar genotypic and

  3. Gradual nerve elongation affects nerve cell bodies and neuro-muscular junctions.

    Science.gov (United States)

    Kazuo Ikeda, K I; Masaki Matsuda, M M; Daisuke Yamauchi, D Y; Katsuro Tomita, K T; Shigenori Tanaka, S T

    2005-07-01

    The purpose of this study is to clarify the reactions of the neuro-muscular junction and nerve cell body to gradual nerve elongation. The sciatic nerves of Japanese white rabbits were lengthened by 30 mm in increments of 0.8 mm/day, 2.0 mm/day and 4.0 mm/day. A scanning electron microscopic examination showed no degenerative change at the neuro-muscular junction, even eight weeks after elongation in the 4-mm group. Hence, neuro-muscular junction is not critical for predicting damage from gradual nerve elongation. There were no axon reaction cells in the 0.8-mm group, a small amount in the 2-mm group, and a large amount in the 4-mm group. The rate of growth associated protein-43 positive nerve cells was significant in the 4-mm group. Hence, the safe speed for nerve cells appeared to be 0.8-mm/day, critical speed to be 2.0-mm/day, and dangerous speed to be 4.0-mm/day in this elongation model.

  4. Regulatory T cells suppress muscle inflammation and injury in muscular dystrophy

    Science.gov (United States)

    Villalta, S. Armando; Rosenthal, Wendy; Martinez, Leonel; Kaur, Amanjot; Sparwasser, Tim; Tidball, James G.; Margeta, Marta; Spencer, Melissa J.; Bluestone, Jeffrey A.

    2016-01-01

    We examined the hypothesis that regulatory T cells (Tregs) modulate muscle injury and inflammation in the mdx mouse model of Duchenne muscular dystrophy (DMD). Although Tregs were largely absent in the muscle of wildtype mice and normal human muscle, they were present in necrotic lesions, displayed an activated phenotype and showed increased expression of interleukin (IL)-10 in dystrophic muscle from mdx mice. Depletion of Tregs exacerbated muscle injury and the severity of muscle inflammation, which was characterized by an enhanced interferon-gamma (IFNγ) response and activation of M1 macrophages. To test the therapeutic value of targeting Tregs in muscular dystrophy, we treated mdx mice with IL-2/anti-IL-2 complexes (IL-2c), and found that Tregs and IL-10 concentrations were increased in muscle, resulting in reduced expression of cyclooygenase-2 and decreased myofiber injury. These findings suggest that Tregs modulate the progression of muscular dystrophy by suppressing type 1 inflammation in muscle associated with muscle fiber injury, and highlight the potential of Treg-modulating agents as therapeutics for DMD. PMID:25320234

  5. Muscular system in the pacific bluefin tuna Thunnus orientalis (Teleostei: Scombridae).

    Science.gov (United States)

    Nakae, Masanori; Sasaki, Kunio; Shinohara, Gento; Okada, Tokihiko; Matsuura, Keiichi

    2014-02-01

    The muscular system in the Pacific bluefin tuna Thunnus orientalis is studied in detail. For the first time, a complete description of the muscular anatomy of a thunnid is provided here. Eighty-two elements including subdivisions of components of the muscular system are identified. This is less than found in a basal perciform and two other investigated scombrid species, owing mainly to the absence or fusion of pectoral, pelvic and caudal fin muscles. The absence of elements of the basal perciform pattern was most prominent in the caudal fin, which includes only the flexor dorsalis, flexor ventralis, hypochordal longitudinalis, and interradialis. In the caudal fin, the medial fan-shaped ray was identified as the first dorsal ray, judging from myological and neuroanatomical characters. The highly developed gill filament muscles in Thunnus orientalis and sheet-like rectus communis control gill ventilation. Long body muscle tendons reduce the metabolic energy needed during rapid and continuous swimming. These characters are interpreted as adaptations in the context of the oceanic life style of the species. Copyright © 2013 Wiley Periodicals, Inc.

  6. Evaluacion del componente central y periferico de fatiga muscular en pacientes neuropaticos y miopaticos

    Directory of Open Access Journals (Sweden)

    L. G. Cohen

    1983-09-01

    Full Text Available Utilizando un método de cuantificación del electromiograma, se investigaron parte de los mecanismos centrales y periféricos responsables de la fatiga muscular en enfermos crónicamente denervados y en pacientes con compromiso muscular primitivo. Se observó en los denervados que los mecanismos de fatiga muscular, no difieren mayormente de los observados en el grupo de sujetos sanos, por el contrario, en los miopáticos a más del componente central, existen otros de orden periférico, tales como la pérdida de unidades motoras funcionantes durante el esfuerzo y el bloqueo temporario de la transmisión neuromuscular, que contribuye al desarrollo de la fatiga.

  7. Avaliação morfológica do oviduto e qualidade de ovos de poedeiras comerciais (Gallus gallus) em diferentes fases de produção.

    OpenAIRE

    Fátima Naomi Takata

    2006-01-01

    As linhagens de poedeiras comerciais existentes atualmente no mercado foram melhoradas geneticamente para produzir o máximo de ovos a um custo mínimo. Um fato importante para a continuidade desta evolução, é o conhecimento celular do oviduto ("fabrica do ovo"). Na galinha (Gallus gallus) o oviduto é descrito como sendo um tubo muscular tortuoso, cujo comprimento é estimado entre 14 e 19 cm na fase de não postura e de 42 a 86 cm durante a fase de postura. Abre-se próximo ao ovário e termina na...

  8. Comparação entre diferentes modelos de periodização sobre a força e espessura muscular em uma sequência dos menores para os maiores grupamentos musculares

    Directory of Open Access Journals (Sweden)

    Juliano Spineti

    2013-08-01

    Full Text Available INTRODUÇÃO: Estudos comparando modelos de periodização em sequências de treinamento resistido (TR realizadas do menor para o maior grupo muscular sobre as alterações musculares em indivíduos destreinados em TR são escassos. OBJETIVO: Comparar o efeito da periodização ondulatória (PO e da periodização linear (PL sobre a força máxima e hipertrofia muscular em uma sequência de execução dos exercícios dos menores para os maiores grupamentos musculares. MÉTODOS: Vinte e nove homens não experientes em (TR foram distribuídos aleatoriamente em três grupos: PO (n =10, PL (n = 13 e grupo controle (GC, n = 9. Os indivíduos realizaram o teste de uma repetição máxima (1RM nos exercícios rosca bíceps (RB, rosca tríceps (RT, puxada aberta (PA e supino reto (SR, contração isométrica voluntária máxima (CIVM e espessura muscular (EM para flexores de cotovelo (FC e extensores de cotovelo (EC antes e após o período de 12 semanas de treinamento. O grupo PO variou o volume e a intensidade do treinamento diariamente, e o grupo PL a cada quatro semanas. O GC não realizou TR. Foi realizada uma ANOVA de dois caminhos com medidas repetidas, e cálculo do tamanho do efeito (TE, nas cargas obtidas para analisar o efeito do tratamento sobre as variações pré- e pós-período de TR. RESULTADOS: Os principais achados do estudo foram: 1 o grupo PO apresentou maior TE para 1RM dos exercícios RT e RB e para EM dos FC e EC quando comparado ao grupo PL; 2 não houve diferença no TE para os exercícios SR e PA que finalizavam a sessão. CONCLUSÃO: Ambos os modelos de periodização adotados foram eficientes para promover aumentos de força e hipertrofia muscular. Contudo, segundo o cálculo do TE, a PO promoveu maior incremento da força máxima nos exercícios que iniciaram a sessão e hipertrofia muscular.

  9. Confiabilidade da medida de espessuras musculares pela ultrassonografia Reliability of muscle thickness measurements using ultrasound

    Directory of Open Access Journals (Sweden)

    Paulo Sergio Chagas Gomes

    2010-02-01

    Full Text Available OBJETIVO: Determinar a confiabilidade das medidas de espessuras dos músculos flexores e extensores do cotovelo e joelho pela ultrassonografia (US, quantificando o erro típico associado a essas medidas (ETM. MÉTODOS: A confiabilidade (duas medidas interdias foi determinada em 15 voluntários aparentemente saudáveis (oito mulheres, 33,9 ± 11,4 anos, 76 ± 21kg, 170 ± 10cm. As imagens da musculatura flexora (FC e extensora do cotovelo (EC e flexora (FJ e extensora do joelho (EJ foram obtidas pela US bidimensional no modo B, utilizando transdutor de 7,5MHz. As espessuras do tecido muscular compreendidas entre as interfaces com o osso e com o tecido adiposo foram medidas em sítios anatômicos identificados e registrados para ser repetidos na segunda medida. RESULTADOS: A ANOVA não identificou diferenças significativas entre as medidas repetidas. Os coeficientes de correlação intraclasse foram FC = 0,970, EC = 0,971, FJ = 0,555 e EJ = 0,929 (P PURPOSE: To determine the reliability of muscle thickness measurements of elbow and knee flexors and extensors using ultrasound, and to quantify the typical error associated to the measurements (TEM. METHODS: The test-retest reliability was determined in 15 apparently healthy volunteers (8 women, 34 ± 11 years, 76 ± 21 kg, 170 ± 10 cm. The images of elbow flexors (EF and extensors (EE and knee flexors (KF and extensors (KE were obtained using a two dimensional mode B ultrasound instrument with a 7.5 MHz transducer. Muscle thickness between the adipose tissue and bone interfaces were measured at anatomical landmarks previously identified and recorded to assure the exact site for the retest. RESULTS: ANOVA did not identify any significant differences between the repeated measurements. Intraclass correlation coefficients (ICC of each pair of measure were EF = 0.970, EE = 0.971, KF = 0.555 e KE = 0.929 (P < 0.05 for all. The coefficients of variation were 3.9 %, 6.1 %, 6.6 % e 4.6 %, and TEM 1.3 mm, 1

  10. Muscular visualisation on a bone scan in paraneoplastic ...

    African Journals Online (AJOL)

    Immunosuppressive therapy based on oral prednisone and endoxan followed by molecular targeted therapy (Herceptin and taxotere) improved the myositis and cutaneous eruption. Tree months later, creatine kinase level and muscular uptake of 99mTc-MDP dramatically decreased (B). Pan African Medical Journal 2016; ...

  11. Age-related differences in muscular capacity among workers

    NARCIS (Netherlands)

    Hamberg-van Reenen, H.H.; Beek, A.J. van der; Blatter, B.M.

    2009-01-01

    Purpose: To quantify the age-related changes in muscular capacity in a working population, and to investigate whether these changes are dependent on sports participation. Methods: Data were used from the longitudinal study on musculoskeletal disorders, absenteeism, stress and health (n = 1,800). At

  12. Co-incidence of Turner syndrome and Duchenne muscular dystrophy - an important problem for the clinician.

    Science.gov (United States)

    Kaczorowska, Ewa; Zimowski, Janusz; Cichoń-Kotek, Monika; Mrozińska, Agnieszka; Purzycka, Joanna; Wierzba, Jolanta; Limon, Janusz; Lipska-Ziętkiewicz, Beata S

    Turner syndrome is a relatively common chromosomal disorder which affects about one in 2000 live born females. Duchenne muscular dystrophy is an X-linked recessive disorder affecting 1:3600 live born males. Considering the above, the coexistence of these two diseases may occur only anecdotally. Here, we report a 4 ½ year-old female with classical 45,X Turner syndrome who also had Duchenne muscular dystrophy caused by a point mutation in the dystrophin gene (c.9055delG). The patient showed the typical phenotype of Turner syndrome including distinctive dysmorphic features (short neck, low posterior hairline, wide position of nipples), aortic coarctation and feet lymphedema. Besides, she presented with an unusually early beginning of muscular dystrophy symptoms with infantile-onset motor developmental delay, intellectual disability and early calf muscular hypertrophy. The coexistence of an X-linked recessive disorder should be considered in women affected by Turner syndrome presenting with additional atypical clinical features.

  13. Anestesia em paciente com Distrofia Muscular de Duchenne: relato de caso

    Directory of Open Access Journals (Sweden)

    Tonelli Deoclécio

    2003-01-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A distrofia muscular de Duchenne é uma afecção recessiva ligada ao cromossomo X, geralmente diagnosticada na infância, acentuando-se progressivamente até agravar a função respiratória. O objetivo deste relato é apresentar um caso de um paciente com distrofia muscular de Duchenne diagnosticada há 2 anos, submetido à postectomia, sob anestesia geral com cetamina S. RELATO DO CASO: Paciente com 9 anos de idade com Distrofia Muscular de Duchenne diagnosticada há 2 anos, submetido à anestesia geral com levo-cetamina (1,5 mg.kg-1, por via venosa, sob ventilação espontânea assistida manualmente por sistema de Baraka (Mapleson A e bloqueio peniano com bupivacaína a 0,5% (25 mg. Foram usados monitores de pressão arterial não invasiva, oximetria de pulso, cardioscopia e temperatura esofagiana. No decorrer da cirurgia, o caso evoluiu sem intercorrências, sendo que no período pós-operatório o paciente apresentou alguns episódios de vômitos sem outras alterações significativas. Permaneceu internado por 24 horas, tendo alta hospitalar assintomático. CONCLUSÕES: A avaliação pré-anestésica cuidadosa, o uso de monitorização adequada e medicações que não predisponham o aparecimento de complicações tornam seguro o procedimento em pacientes portadores de Distrofia Muscular de Duchenne e seu pós-operatório.

  14. Dismorfia muscular e o uso de suplementos ergogênicos em desportistas

    Directory of Open Access Journals (Sweden)

    Litiane Dorneles de Lima

    2010-12-01

    Full Text Available A dismorfia muscular (DM é um transtorno da imagem corporal que acomete principalmente homens que, apesar da grande hipertrofia muscular, consideram-se pequenos e fracos. Tendo em vista o crescente uso de substâncias ergogênicas para o aumento de massa muscular e o elevado número de indivíduos com transtornos corporais, o objetivo deste trabalho foi avaliar a presença de DM e o uso de suplementos ergogênicos em desportistas frequentadores de academia. Foram avaliados 23 indivíduos do sexo masculino com média de idade de 24 ± 3,8 anos, praticantes de treinamento de força, com idades entre 18 e 35 anos. Para a avaliação corporal foi realizado o teste de bioimpedância, com o qual se obteve os dados de massa magra e gorda, respectivamente, de 66,5 ± 10,1kg e 10,5 ± 3,1kg. Para detectar o risco de DM, foi usada uma escala (MASS - Muscle Appearance Satisfaction Scale; se o resultado desta fosse acima de 52 pontos, estes apresentavam o risco de DM. Juntamente, foi respondido outro questionário para o conhecimento do uso de suplementos. A análise dos resultados identificou que, dos avaliados, 17,4% (n = 4 apresentaram risco positivo para o desenvolvimento deste transtorno corporal; porém, destes, apenas 25% declararam fazer uso de suplementos alimentares. Conclui-se que não houve relação entre o uso de suplementos e a presença de dismorfia muscular.

  15. Naturally Protected Muscle Phenotypes: Development of Novel Treatment Strategies for Duchenne Muscular Dystrophy

    OpenAIRE

    Dowling, Paul; Doran, Philip; Lohan, James; Culligan, Kevin; Ohlendieck, Kay

    2004-01-01

    Primary abnormalities in the dystrophin gene underlie x-linked muscular dystrophy. However, the absence of the dystrophin isoform Dp427 does not necessarily result in a severe dystrophic phenotype in all muscle groups. Distal mdx muscles, namely extraocular and toe fibres, appear to represent a protected phenotype in muscular dystrophy. Thus, a comparative analysis of affected versus naturally protected muscle cells should lead to a greater knowledge of the molecular pathogenes...

  16. Radionuclide study for cardiac lesion in Duchenne muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Oguni, Hirokazu; Osawa, Makiko; Shishikura, Keiko

    1985-12-01

    Tl-201 myocardial scintigraphy and radionuclide ventriculography with Tc-99m were performed in 10 patients with Duchenne muscular dystropohy (DMD) and 2 siblings with Becker muscular dystrophy (BMD). Perfusion defect especially in the left ventricular posterolateral wall (LVPLW) and cardiac apex was seen on Tl-201 imaging in 6 of the DMD patients and one of the BMD patients. For these patients, Tc-99m imaging also showed left ventricular local wall motion abnormality in 5 patients and a decreased left ventricular ejection fraction in 4 patients. These findings coincided well with fibrosis of the LVPLW found on autopsy. There were individual differences regarding the occurrence of cardiac complications. One of the BMD patients, as well as DMD patients, had also cardiac complications which have long been considered less common. (Namekawa, K.).

  17. Radionuclide study for cardiac lesion in Duchenne muscular dystrophy

    International Nuclear Information System (INIS)

    Oguni, Hirokazu; Osawa, Makiko; Shishikura, Keiko

    1985-01-01

    Tl-201 myocardial scintigraphy and radionuclide ventriculography with Tc-99m were performed in 10 patients with Duchenne muscular dystropohy (DMD) and 2 siblings with Becker muscular dystrophy (BMD). Perfusion defect especially in the left ventricular posterolateral wall (LVPLW) and cardiac apex was seen on Tl-201 imaging in 6 of the DMD patients and one of the BMD patients. For these patients, Tc-99m imaging also showed left ventricular local wall motion abnormality in 5 patients and a decreased left ventricular ejection fraction in 4 patients. These findings coincided well with fibrosis of the LVPLW found on autopsy. There were individual differences regarding the occurrence of cardiac complications. One of the BMD patients, as well as DMD patients, had also cardiac complications which have long been considered less common. (Namekawa, K.)

  18. The new frontier in muscular dystrophy research: booster genes

    DEFF Research Database (Denmark)

    Engvall, Eva; Wewer, Ulla M

    2003-01-01

    More than 30 different forms of muscular dystrophy (MD) have been molecularly characterized and can be diagnosed, but progress toward treatment has been slow. Gene replacement therapy has met with great difficulty because of the large size of the defective genes and because of difficulties...... of the boosters are better understood, drugs may be developed to provide the boost to muscle. Some of the experiences in models of muscular dystrophy may inspire new approaches in other genetic degenerative diseases as well....... in delivering a gene to all muscle groups. Cell replacement therapy has also been difficult to realize. Will it even be possible to design specific therapy protocols for all MDs? Or is a more realistic goal to treat some of the secondary manifestations that are common to several forms of MD, such as membrane...

  19. A bedside measure of body composition in Duchenne muscular dystrophy.

    Science.gov (United States)

    Elliott, Sarah A; Davidson, Zoe E; Davies, Peter S W; Truby, Helen

    2015-01-01

    In clinical practice, monitoring body composition is a critical component of nutritional assessment and weight management in boys with Duchenne muscular dystrophy. We aimed to evaluate the accuracy of a simple bedside measurement tool for body composition, namely bioelectrical impedance analysis, in boys with Duchenne muscular dystrophy. Measures of fat-free mass were determined using a bioelectrical impedance analysis machine and compared against estimations obtained from a reference body composition model. Additionally, the use of raw impedance values was analyzed using three existing predictive equations for the estimation of fat-free mass. Accuracy of bioelectrical impedance analysis was assessed by comparison against the reference model by calculation of biases and limits of agreement. Body composition was measured in 10 boys with Duchenne muscular dystrophy, mean age 9.01 ± 2.34 years. The bioelectrical impedance analysis machine values of fat-free mass were on average 2.3 ± 14.1 kg higher than reference values. Limits of agreement (based on 95% confidence interval of the mean) were -7.4 to 2.9 kg. There was a significant correlation between the mean fat-free mass and difference in fat-free mass between the bioelectrical impedance analysis machine and the reference model (r = -0.86; P = 0.02) suggesting that the bias was not consistent across the range of measurements. The most accurate predictive equation for the estimation of fat-free mass using raw impedance values was the equation by Pietrobelli et al. (mean difference, -0.7 kg; 95% limits of agreement, -3.5 to 2.0 kg). In a clinical setting, where a rapid assessment of body composition is advantageous, the use of raw impedance values, combined with the equation by Pietrobelli et al., is recommended for the accurate estimation of fat-free mass, in boys with Duchenne muscular dystrophy. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. [Percutaneous closure of ductus arteriosus and muscular ventricular defect with amplatzer occluder in a patient with severe pulmonary hypertension].

    Science.gov (United States)

    García-Montes, José Antonio; Zabal Cerdeira, Carlos; Calderón-Colmenero, Juan; Espínola, Nilda; Fernández de la Reguera, Guillermo; Buendía Hernández, Alfonso

    2005-01-01

    Surgical treatment of multiple muscular ventricular septal defects with associated lesions and severe pulmonary hypertension has a high morbility and mortality. Closure of these defects by the Amplatzer muscular VSD occluder is an alternative to surgery, avoiding the need of cardiopulmonary bypass. We present the case of a 38 year-old woman with signs of heart failure in NYHA functional class IV, with two muscular ventricular septal defects, patent ductus arteriosus and severe pulmonary hypertension, that were treated with three Amplatzer muscular VSD occluders, with significant reduction of pulmonary pressure and functional class improvement.

  1. Muscular response to the first three months of deflazacort treatment in boys with Duchenne muscular dystrophy

    DEFF Research Database (Denmark)

    Jensen, L; Petersson, S J; Illum, N O

    2017-01-01

    OBJECTIVE: Duchenne muscular dystrophy (DMD) patients are often treated with glucocorticoids; yet their precise molecular action remains unknown. METHODS: We investigated muscle biopsies from nine boys with DMD (aged: 7,6±2,8 yrs.) collected before and after three months of deflazacort treatment...... into the molecular actions of glucocorticoids in DMD at the mRNA level, and we show that multiple regulatory pathways are influenced. This information can be important in the development of new treatments....

  2. Prevalence and Characteristics of Chinese Patients With Duchenne and Becker Muscular Dystrophy

    Science.gov (United States)

    Lo, Ivan F. M.; Cherk, Sharon W. W.; Cheng, Wai Wai; Fung, Eva L. W.; Yeung, Wai Lan; Ngan, Mary; Lee, Wing Cheong; Kwong, Ling; Wong, Suet Na; Ma, Che Kwan; Tai, Shuk Mui; Ng, Grace S. F.; Wu, Shun Ping; Wong, Virginia C. N.

    2015-01-01

    The aim of this collaborative study on Duchenne muscular dystrophy and Becker muscular dystrophy is to determine the prevalence and to develop data on such patients as a prelude to the development of registry in Hong Kong. Information on clinical and molecular findings, and patient care, was systematically collected in 2011 and 2012 from all Pediatric Neurology Units in Hong Kong. Ninety patients with dystrophinopathy were identified, and 83% has Duchenne muscular dystrophy. The overall prevalence of dystrophinopathy in Hong Kong in 2010 is 1.03 per 10 000 males aged 0 to 24 years. Among the Duchenne group, we observed a higher percentage (40.6%) of point mutations with a lower percentage (45.3%) of exon deletions in our patients when compared with overseas studies. Although we observed similar percentage of Duchenne group received scoliosis surgery, ventilation support, and cardiac treatment when compared with other countries, the percentage (25%) of steroid use is lower. PMID:28503591

  3. Efeito da erva-mate (Ilex paraguariensis) sobre o metabolismo de ratos tratados com dieta hiperlipídica

    OpenAIRE

    Luana Ferreira Gomes

    2012-01-01

    A obesidade é definida por um acúmulo excessivo de gordura na forma de tecido adiposo. É um problema crescente, resultando em significativa morbidade e mortalidade por doenças relacionadas, bem como uma redução da qualidade de vida. O acúmulo excessivo de tecido adiposo que caracteriza a obesidade está diretamente relacionado ao desenvolvimento patologias que, coletivamente, compõem a chamada síndrome metabólica. O tecido adiposo é o principal reservatório energético do organismo, mas também ...

  4. Reconciliando modularidad y eficiencia mediante atajos

    OpenAIRE

    Marco Gómez, Jordi; Franch Gutiérrez, Javier

    1997-01-01

    Se presenta en este artículo una propuesta para el desarrollo de programas eficientes en el marco de la programación con tipos abstractos de datos (TAD), con el objetivo de respetar la estructura modular de los programas propia de este ámbito. La propuesta se centra en el concepto de atajo como camino eficiente de acceso a los datos, alternativo al acceso mediante las operaciones propias del TAD, y se desarrolla sobre un TAD concreto, el almacén de elementos. La definición de los atajos es al...

  5. OPTIMIZING THE MUSCULAR TRAINING THROUGH ALTERNATED CONTRACTION REGYMES AT JUNIOR FEMALE PLAYERS I

    Directory of Open Access Journals (Sweden)

    VLADU Larisa

    2010-04-01

    Full Text Available The muscular training is amongst the most important factors and, in some cases, indeed the primordial ingredient of the sport training with a view to reaching high performance results, for it influences the manner in which the training is organized throughout the macro-cycle stages. It must be done organically and well balancedfor each motrical aptitude solicited by the practice of the performance volleyball.The selection of the muscular groups must be done in accordance with the technical requirements imposed by that sport branch, with the purpose of highly training the muscular groups implied in the specificmovements, allowing the techno-tactical actions to be executed at high potential.The physical training of sportsmen implies best establishing the relation in between the physical preparation, techno-tactical training and psychological training. The interdependency of the sport training factors is of major importance, as is the dominant physical training part. The need to continuously grow the specific andpredominant motric qualities is one of the conditions of raising the players’ efficiency. The muscular training is accomplished both by separate trainings inside the force room as through training sessions specific to volleyball, by appealing to muscle exercises. It is very important to alternate these training throughout the preparation, inorder to obtain the desired effects, while the used means must be in accordance with the game pre-requisites.When it comes to the muscular volleyball training, it is imperative that we consider the following aspects: the game specifics, the game concept, the players’ specificities, the post characteristics, the game evolution tendencies, as well as optimizing the preparation

  6. Estado actual de las terapias con células madre derivadas de tejido adiposo en el ámbito de la Cirugía Plástica The state of the adipose-derived stem cell therapies in Plastic Surgery

    Directory of Open Access Journals (Sweden)

    J.M. Lasso

    2010-09-01

    Full Text Available Durante la última década, la terapia celular ha emergido como una estrategia útil en el tratamiento de diversas enfermedades como la isquemia miocárdica y las fístulas en la enfermedad de Crohn; pero últimamente, hay también ya líneas de investigación centradas en su uso en reconstrucción mamaria, cuyos resultados van siendo publicados paulatinamente. Existen varios tipos de células madre adultas que han sido investigadas en estudios preclínicos y clínicos diseñados para este propósito: células de medula ósea, células del sistema circulatorio y mioblastos y, recientemente se está trabajando en una población de células madre en el tejido adiposo, que presentan una fácil extracción y manipulación. Estas células son capaces de diferenciarse en múltiples líneas celulares, como los adipocitos y las células endoteliales entre otras. En el presente artículo, trataremos de hacer una revisión de los principios básicos de las células madre derivadas del tejido adiposo (tipos, características, procesos de obtención y multiplicación, los primeros estudios experimentales y los ensayos clínicos que están siendo realizados en la actualidad.Over the past decade, cell therapy has emerged as a new approach to reverse several diseases as myocardial ischemia and fistula in Crohn disease; but lately new efforts are centered in breast reconstruction. Several types of adult stem cell have been studied in both preclinical and clinical condition for this purpose: bone marrow cells, circulating cells, and myoblasts. Recently the existence of a population of stem cells located in the adipose tissue has been observed. These cells are able to differentiate into multiple cell lineage including adipocytes and endothelial cells. In this review we discuss the basic principle of adipose-derived stem cell (types, characteristic, harvesting and expansion, the initial experimental studies and the currently ongoing clinical trials.

  7. Swallow Characteristics in Patients with Oculopharyngeal Muscular Dystrophy

    Science.gov (United States)

    Palmer, Phyllis M.; Neel, Amy T.; Sprouls, Gwyneth; Morrison, Leslie

    2010-01-01

    Purpose: This prospective investigation evaluates oral weakness and its impact on swallow function, weight, and quality of life in patients with oculopharyngeal muscular dystrophy (OPMD). Method: Intraoral pressure, swallow pressure, and endurance were measured using an Iowa Oral Performance Instrument in participants with OPMD and matched…

  8. Motor assessment in patients with Duchenne muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Gabriela Palhares Campolina Diniz

    2012-06-01

    Full Text Available OBJECTIVE: Evaluate muscle force and motor function in patients with Duchenne muscular dystrophy (DMD in a period of six months. METHOD: Twenty children and adolescents with diagnosis of DMD were evaluated trough: measurement of the strength of the flexors and extensors of the shoulder, elbow, wrist, knee and ankle through the Medical Research Council (MRC, and application of the Motor Function Measure (MFM. The patients were evaluated twice within a six-month interval. RESULTS: Loss of muscle strength was identified in the MRC score for upper proximal members (t=-2.17, p=0.04. In the MFM, it was noted significant loss in the dimension 1 (t=-3.06, p=0.006. Moderate and strong correlations were found between the scores for muscular strength and the MFM dimensions. CONCLUSION: The MFM scale was a useful instrument in the follow up of patients with DMD. Moreover, it is a more comprehensive scale to assess patients and very good for conducting trials to evaluate treatment.

  9. Exon Deletion Pattern in Duchene Muscular Dystrophy in North West of Iran

    OpenAIRE

    BARZEGAR, Mohammad; HABIBI, Parinaz; BONYADY, Mortaza; TOPCHIZADEH, Vahideh; SHIVA, Shadi

    2015-01-01

    How to Cite This Article: Barzegar M, Habibi P, Bonyady M, Topchizadeh V, Shiva Sh. Exon Deletion Pattern in Duchene Muscular Dystrophy in North West of Iran. Iran J Child Neurol. 2015 Winter; 9(1): 42-48.AbstractObjectiveDuchene and Becker Muscular Dystrophy (DMD/ BMD) are x-linked disorders that both are the result of heterogeneous mutations in the dystrophin gene. The frequency and distribution of dystrophin gene deletions in DMD/ BMD patients show different patterns among different popula...

  10. Ejercicio de resistencia muscular en adultos con diabetes mellitus tipo 2 Exercício de resistência muscular em adultos com diabetes mellitus tipo 2 Endurance training in adults with diabetes mellitus type 2

    Directory of Open Access Journals (Sweden)

    Karina Asunción Muñoz Canché

    2005-02-01

    Full Text Available Objetivos. Probar la efectividad del ejercicio de resistencia muscular en las cifras de hemoglobina glucosilada (HbA1c, en la fuerza muscular y en la fortaleza muscular percibida; explorar la influencia de la dieta, otros ejercicios, hipoglucemiantes y manifestaciones asociadas a episodios de hipoglucemia o hiperglucemia sobre el control glucémico de adultos con diabetes mellitus tipo 2 provenientes de los consultorios de endocrinología de dos hospitales públicos de la Ciudad de Monterrey, México. Procedimientos básicos. Se usó un diseño de 12 semanas de grupo control no equivalente con dos grupos, experimental (n1 = 14 y control (n2 = 11. Los participantes contaron con la recomendación de su médico para participar. Las sesiones de ejercicio fueron de una hora, dos veces por semana. Resultados. El grupo experimental mostró decremento significativo en el porcentaje de HbA1c, e incremento en la fuerza muscular y fortaleza muscular percibida (p Objetivos: Provar a efetividade do exercício de resistência muscular nas taxas de hemoglobina A glicosilada (HbA1c, na força muscular e no fortalecimento muscular percebido, explorar a influência da dieta, outros exercícios, hipoglicemiantes e manifestações associadas a episódios de hipoglicemia ou hiperglicemia sobre o controle glicêmico de adultos com diabetes mellitus tipo 2, provenientes dos consultórios de endocrinologia de dois hospitales públicos da Cidade de Monterrey, México. Procedimentos básicos: Foi utilizado um desenho de 12 semanas de grupo controle não equivalente com dois grupos, experimental (n1=14 e controle (n2=11. Os participantes tiveram recomendação de seu médico para participar. As sessões de exercício foram de uma hora, duas vezes por semana. Resultados: O grupo experimental apresentou decréscimo significativo nas taxas de HbA1c, incremento na força muscular e fortalecimento muscular percebido (pObjectives. Test the effects of an endurance training

  11. A Cross-Sectional Study of School Experiences of Boys with Duchenne and Becker Muscular Dystrophy

    Science.gov (United States)

    Soim, Aida; Lamb, Molly; Campbell, Kimberly; Pandya, Shree; Peay, Holly; Howard, James F., Jr.; Fox, Deborah

    2016-01-01

    The objectives of this study were to investigate types of supportive school services received and factors related to provision of these services. We conducted a cross-sectional study to describe the school experience of males with Duchenne and Becker muscular dystrophies. Study subjects were identified through the Muscular Dystrophy Surveillance,…

  12. Associations between aerobic and muscular fitness and cardiovascular disease risk : the northern Ireland young hearts study

    NARCIS (Netherlands)

    Hoekstra, T.; Boreham, Colin A; Murray, Liam J; Twisk, Jos W R

    2008-01-01

    BACKGROUND: It is not clear what the relative contribution is of specific components of physical fitness (aerobic and muscular) to cardiovascular disease (CVD) risk. We investigated associations between aerobic fitness (endurance) and muscular fitness (power) and CVD risk factors. METHODS: Data were

  13. Vascular anatomy of the medial sural artery perforator flap: a new classification system of intra-muscular branching patterns.

    Science.gov (United States)

    Dusseldorp, Joseph R; Pham, Quy J; Ngo, Quan; Gianoutsos, Mark; Moradi, Pouria

    2014-09-01

    The medial sural artery perforator (MSAP) flap is a versatile fasciocutaneous flap. The main difficulty encountered when raising the MSAP flap is in obtaining adequate pedicle length during intra-muscular dissection. The objective of this study was to determine the pattern of intra-muscular course of the MSAP flap pedicle. 14 cadaveric specimens were dissected and CT angiograms of 84 legs were examined. The intra-muscular branching pattern and depths of the medial sural artery branches were analyzed. The number of perforators, position of the dominant perforator and both intra-muscular and total pedicle length were also recorded and compared to existing anatomical data. Three types of arterial branching pattern were identified within the medial gastrocnemius, demonstrating one (31%), two (59%) or three or more (10%) main branches. A dominant perforator from the medial sural artery was present in 92% of anatomical specimens (13/14). Vertically, the location of the perforator from the popliteal crease was on average 13 cm (±2 cm). Transversely, the perforator originated 2.5 cm (±1 cm) from the posterior midline. Using CT angiography it was possible in 10 consecutive patients to identify a more superficial intra-muscular branch and determine the leg with the optimal branching pattern type for flap harvest. This study is the first to describe the variability of the intra-muscular arterial anatomy of the medial head of gastrocnemius muscle. Surgeons utilizing the MSAP flap option should be aware of the possible branching pattern types and consequently the differing perforator distribution and depths of intra-muscular branches. Routine use of pre-operative CT angiogram may help determine which leg has the most favorable branching pattern type and intra-muscular course for flap harvest. Copyright © 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  14. Mild and severe muscular dystrophy caused by a single {gamma}-sarcoglycan mutation

    Energy Technology Data Exchange (ETDEWEB)

    McNally, E.M.; Boennemann, C.G.; Lidov, H.G.W. [Brigham and Women`s Hospital, Boston, MA (United States)] [and others

    1996-11-01

    Autosomal recessive muscular dystrophy is genetically heterogeneous. One form of this disorder, limb-girdle muscular dystrophy type 2C (LGMD 2C), is prevalent in northern Africa and has been shown to be associated with a single mutation in the gene encoding the dystrophin-associated protein {gamma}-sarcoglycan. The previous mutation analysis of {gamma}-sarcoglycan required the availability of muscle biopsies. To establish a mutation assay for genomic DNA, the intron-exon structure of the {gamma}-sarcoglycan gene was determined, and primers were designed to amplify each of the exons encoding {gamma}-sarcoglycan. We studied a group of Brazilian muscular dystrophy patients for mutations in the {gamma}-sarcoglycan gene. These patients were selected on the basis of autosomal inheritance and/or the presence of normal dystrophin and/or deficiency of {alpha}-sarcoglycan immunostaining. Four of 19 patients surveyed had a single, homozygous mutation in the {gamma}-sarcoglycan gene. The mutation identified in these patients, all of African-Brazilian descent, is identical to that seen in the North African population, suggesting that even patients of remote African descent may carry this mutation. The phenotype in these patients varied considerably. Of four families with an identical mutation, three have a severe Duchenne-like muscular dystrophy. However, one family has much milder symptoms, suggesting that other loci may be present that modify the severity of the clinical course resulting from {gamma}-sarcoglycan gene mutations. 19 refs., 5 figs., 3 tabs.

  15. Sarcospan Regulates Cardiac Isoproterenol Response and Prevents Duchenne Muscular Dystrophy-Associated Cardiomyopathy.

    Science.gov (United States)

    Parvatiyar, Michelle S; Marshall, Jamie L; Nguyen, Reginald T; Jordan, Maria C; Richardson, Vanitra A; Roos, Kenneth P; Crosbie-Watson, Rachelle H

    2015-12-23

    Duchenne muscular dystrophy is a fatal cardiac and skeletal muscle disease resulting from mutations in the dystrophin gene. We have previously demonstrated that a dystrophin-associated protein, sarcospan (SSPN), ameliorated Duchenne muscular dystrophy skeletal muscle degeneration by activating compensatory pathways that regulate muscle cell adhesion (laminin-binding) to the extracellular matrix. Conversely, loss of SSPN destabilized skeletal muscle adhesion, hampered muscle regeneration, and reduced force properties. Given the importance of SSPN to skeletal muscle, we investigated the consequences of SSPN ablation in cardiac muscle and determined whether overexpression of SSPN into mdx mice ameliorates cardiac disease symptoms associated with Duchenne muscular dystrophy cardiomyopathy. SSPN-null mice exhibited cardiac enlargement, exacerbated cardiomyocyte hypertrophy, and increased fibrosis in response to β-adrenergic challenge (isoproterenol; 0.8 mg/day per 2 weeks). Biochemical analysis of SSPN-null cardiac muscle revealed reduced sarcolemma localization of many proteins with a known role in cardiomyopathy pathogenesis: dystrophin, the sarcoglycans (α-, δ-, and γ-subunits), and β1D integrin. Transgenic overexpression of SSPN in Duchenne muscular dystrophy mice (mdx(TG)) improved cardiomyofiber cell adhesion, sarcolemma integrity, cardiac functional parameters, as well as increased expression of compensatory transmembrane proteins that mediate attachment to the extracellular matrix. SSPN regulates sarcolemmal expression of laminin-binding complexes that are critical to cardiac muscle function and protects against transient and chronic injury, including inherited cardiomyopathy. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

  16. Pérdida auditiva por contaminación acústica laboral en Santiago de Chile

    OpenAIRE

    Salazar Bugueño, Ana María

    2013-01-01

    [spa] La Organización Mundial de la Salud (OMS) ha estimado que, aproximadamente, 278 millones de personas presentan déficit auditivo en el mundo; que el 50% de las pérdidas auditivas podrían evitarse mediante prevención, un diagnóstico precoz y una gestión eficaz y que, más de 4.000.000 de años de vida saludable se perdieron debido a las pérdidas auditivas inducidas por ruido. Lo anterior hace necesario establecer un modelo para predecir la pérdida auditiva por contaminación acústica laboral...

  17. Impacto de herramientas de mejora en la eficiencia operacional y responsabilidad ambiental en plantas de manufactura de México

    Directory of Open Access Journals (Sweden)

    Carlos Monge

    2016-03-01

    Full Text Available se propone un modelo matemático para interrelacionar empíricamente las variables manufactura esbelta, la manufactura sustentable y la mejora continua con la eficiencia operacional y responsabilidad ambiental en plantas de manufactura discreta de apodaca, nl, México. Dada la complejidad de las interrelaciones del modelo propuesto, se establece que éste sea estimado mediante la técnica estadística de modelación con ecuaciones estructurales basadas en mínimos cuadrados parciales, utilizando indicadores formativos, y empleando el instrumento de medición propuesto por los autores en un estudio ya publicado.

  18. Overexpression of Latent TGFβ Binding Protein 4 in Muscle Ameliorates Muscular Dystrophy through Myostatin and TGFβ.

    Science.gov (United States)

    Lamar, Kay-Marie; Bogdanovich, Sasha; Gardner, Brandon B; Gao, Quan Q; Miller, Tamari; Earley, Judy U; Hadhazy, Michele; Vo, Andy H; Wren, Lisa; Molkentin, Jeffery D; McNally, Elizabeth M

    2016-05-01

    Latent TGFβ binding proteins (LTBPs) regulate the extracellular availability of latent TGFβ. LTBP4 was identified as a genetic modifier of muscular dystrophy in mice and humans. An in-frame insertion polymorphism in the murine Ltbp4 gene associates with partial protection against muscular dystrophy. In humans, nonsynonymous single nucleotide polymorphisms in LTBP4 associate with prolonged ambulation in Duchenne muscular dystrophy. To better understand LTBP4 and its role in modifying muscular dystrophy, we created transgenic mice overexpressing the protective murine allele of LTBP4 specifically in mature myofibers using the human skeletal actin promoter. Overexpression of LTBP4 protein was associated with increased muscle mass and proportionally increased strength compared to age-matched controls. In order to assess the effects of LTBP4 in muscular dystrophy, LTBP4 overexpressing mice were bred to mdx mice, a model of Duchenne muscular dystrophy. In this model, increased LTBP4 led to greater muscle mass with proportionally increased strength, and decreased fibrosis. The increase in muscle mass and reduction in fibrosis were similar to what occurs when myostatin, a related TGFβ family member and negative regulator of muscle mass, was deleted in mdx mice. Supporting this, we found that myostatin forms a complex with LTBP4 and that overexpression of LTBP4 led to a decrease in myostatin levels. LTBP4 also interacted with TGFβ and GDF11, a protein highly related to myostatin. These data identify LTBP4 as a multi-TGFβ family ligand binding protein with the capacity to modify muscle disease through overexpression.

  19. Overexpression of Latent TGFβ Binding Protein 4 in Muscle Ameliorates Muscular Dystrophy through Myostatin and TGFβ.

    Directory of Open Access Journals (Sweden)

    Kay-Marie Lamar

    2016-05-01

    Full Text Available Latent TGFβ binding proteins (LTBPs regulate the extracellular availability of latent TGFβ. LTBP4 was identified as a genetic modifier of muscular dystrophy in mice and humans. An in-frame insertion polymorphism in the murine Ltbp4 gene associates with partial protection against muscular dystrophy. In humans, nonsynonymous single nucleotide polymorphisms in LTBP4 associate with prolonged ambulation in Duchenne muscular dystrophy. To better understand LTBP4 and its role in modifying muscular dystrophy, we created transgenic mice overexpressing the protective murine allele of LTBP4 specifically in mature myofibers using the human skeletal actin promoter. Overexpression of LTBP4 protein was associated with increased muscle mass and proportionally increased strength compared to age-matched controls. In order to assess the effects of LTBP4 in muscular dystrophy, LTBP4 overexpressing mice were bred to mdx mice, a model of Duchenne muscular dystrophy. In this model, increased LTBP4 led to greater muscle mass with proportionally increased strength, and decreased fibrosis. The increase in muscle mass and reduction in fibrosis were similar to what occurs when myostatin, a related TGFβ family member and negative regulator of muscle mass, was deleted in mdx mice. Supporting this, we found that myostatin forms a complex with LTBP4 and that overexpression of LTBP4 led to a decrease in myostatin levels. LTBP4 also interacted with TGFβ and GDF11, a protein highly related to myostatin. These data identify LTBP4 as a multi-TGFβ family ligand binding protein with the capacity to modify muscle disease through overexpression.

  20. Evidence for linkage disequilibrium in chromosome 13-linked Duchenne-like muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Othmane, K.B.; Speer, M.C.; Stauffer, J. [Duke Univ. Medical Center, Durham, NC (United States)] [and others

    1995-09-01

    Duchenne-like muscular dystrophy (DLMD) is an autosomal recessive Limb Girdle muscular dystrophy (LGMD2C) characterized by late age of onset, proximal muscle weakness leading to disability, high creatine kinase values, normal intelligence and normal dystrophin in muscle biopsy. We have shown previously that three DLMD families from Tunisia are linked to chromosome 13q12. To further localize the LGMD2C gene, we have investigated seven additional families (119 individuals). Both genotyping and two-point linkage analysis were performed as described elsewhere. 7 refs., 1 fig., 1 tab.

  1. Advances in gene therapy for muscular dystrophies [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Hayder Abdul-Razak

    2016-08-01

    Full Text Available Duchenne muscular dystrophy (DMD is a recessive lethal inherited muscular dystrophy caused by mutations in the gene encoding dystrophin, a protein required for muscle fibre integrity. So far, many approaches have been tested from the traditional gene addition to newer advanced approaches based on manipulation of the cellular machinery either at the gene transcription, mRNA processing or translation levels. Unfortunately, despite all these efforts, no efficient treatments for DMD are currently available. In this review, we highlight the most advanced therapeutic strategies under investigation as potential DMD treatments.

  2. Modulación del crecimiento vertebral mediante electrocoagulación hemicircunferencial vertebral asistida

    OpenAIRE

    Caballero García, Alberto

    2011-01-01

    Nuestro trabajo está basado en la posibilidad de controlar el desarrollo asimétrico de los cartílagos de crecimiento vertebral, mediante la realización de una fisiodesis hemivertebral, con electrocoagulación, videoasistida por toracoscópica. Se realizará en cinco niveles torácicos, con un abordaje anterior mínimamente invasivo. Por lo tanto, planteamos como hipótesis de trabajo que La destrucción de las fisis de crecimiento vertebral mediante electrocoagulación, videoasistida por vía toracosc...

  3. Quality of life of adult men with Duchenne muscular dystrophy in the Netherlands : Implications for care

    NARCIS (Netherlands)

    Pangalila, Robert F.; Van Den Bos, Geertrudis A M; Bartels, Bart; Bergen, Michael P.; Kampelmacher, Mike J.; Stam, Henk J.; Roebroeck, Marij E.

    2015-01-01

    Objective: To assess quality of life of adults with Duchenne muscular dystrophy in the Netherlands and to identify domains and major problems influencing quality of life. Design: Cross-sectional. Subjects: Seventy-nine men aged ≥ 20 years with Duchenne muscular dystrophy. Methods: The Medical

  4. EFEITOS DA CRIO E TERMOTERAPIA CONTRA O DANO OXIDATIVO INDUZIDO POR DISTENSÃO MUSCULAR EM RATOS

    OpenAIRE

    Nélson Rodrigues de Carvalho

    2012-01-01

    Lesões musculares esqueléticas estão entre as causas mais frequentes de comprometimento funcional do tecido muscular, acometendo a qualidade de vida e são as principais responsáveis pela perda do ritmo de treinamento no caso de atletas. As lesões mais frequentes são as contusões e distensões musculares. Assim, o desenvolvimento de terapias que amenizem e possam acelerar o processo de reparo celular e reabilitação tecidual são de grande importância. Desta forma, aplicações terapêuticas de agen...

  5. OPTIMIZING THE MUSCULAR TRAINING THROUGH ALTERNATED CONTRACTION REGYMES AT JUNIOR FEMALE PLAYERS I

    OpenAIRE

    VLADU Larisa

    2010-01-01

    The muscular training is amongst the most important factors and, in some cases, indeed the primordial ingredient of the sport training with a view to reaching high performance results, for it influences the manner in which the training is organized throughout the macro-cycle stages. It must be done organically and well balancedfor each motrical aptitude solicited by the practice of the performance volleyball.The selection of the muscular groups must be done in accordance with the technical re...

  6. Peripheral nerve blocks as the sole anesthetic technique in a patient with severe Duchenne muscular dystrophy.

    Science.gov (United States)

    Bang, Seung Uk; Kim, Yee Suk; Kwon, Woo Jin; Lee, Sang Mook; Kim, Soo Hyang

    2016-04-01

    General anesthesia and central neuraxial blockades in patients with severe Duchenne muscular dystrophy are associated with high risks of complications, including rhabdomyolysis, malignant hyperthermia, hemodynamic instability, and postoperative mechanical ventilation. Here, we describe peripheral nerve blocks as a safe approach to anesthesia in a patient with severe Duchenne muscular dystrophy who was scheduled to undergo surgery. A 22-year-old male patient was scheduled to undergo reduction and internal fixation of a left distal femur fracture. He had been diagnosed with Duchenne muscular dystrophy at 5 years of age, and had no locomotive capability except for that of the finger flexors and toe extensors. He had developed symptoms associated with dyspnea 5 years before and required intermittent ventilation. We blocked the femoral nerve, lateral femoral cutaneous nerve, and parasacral plexus under ultrasound on the left leg. The patient underwent a successful operation using peripheral nerve blocks with no complications. In conclusion general anesthesia and central neuraxial blockades in patients with severe Duchenne muscular dystrophy are unsafe approaches to anesthesia because of hemodynamic instability and respiratory depression. Peripheral nerve blocks are the best way to reduce the risks of critical complications, and are a safe and feasible approach to anesthesia in patients with severe Duchenne muscular dystrophy.

  7. Programación por metas Energía alternativa mediante biomasa.

    Directory of Open Access Journals (Sweden)

    Guerrero Casas, Flor María

    2003-01-01

    Full Text Available En este trabajo se presenta un modelo multicriterio de localización de centrales de generación de energía eléctrica mediante biomasa. Los objetivos considerados son: (1 minimizar el coste total de la operación, (2 maximizar la producción de electricidad obtenida, (3 maximizar la distancia entre plantas, (4 maximizar la aceptación social y (5 establecer las plantas o ampliaciones en aquellos lugares donde exista una mayor predisposición por parte de las administraciones locales. Finalmente, se concluye con una aplicación práctica mediante programación por metas ponderadas para la región andaluza, considerando los residuos procedentes del olivar como fuente de energía.

  8. Body Composition, Muscular Strength and Bone Status among Undernourished Children in Malaysia

    International Nuclear Information System (INIS)

    Chong, Kar Hau; Poh, Bee Koon

    2014-01-01

    Full text: Despite significant advances in social and economic development, undernutrition remains a devastating public health problem that affects millions of children across the globe, particularly in developing nations. It is important to understand how changes in nutritional status affect physical health and function, so that undernutrition-related alterations can be identified and interpreted correctly. This paper aimed to determine the impact of undernutrition in children through the assessment of three nutrition-related indicators: body composition, muscular strength and bone status. This study is part of the Nutrition Survey of Malaysian Children, which is part of the four-country South East Asian Nutrition Surveys (SEANUTS). A total of 208 school children (102 boys, 106 girls) in the age range of 7 to 10 years were included in this analysis, of which 104 were underweight (WAZ<-2SD) and 104 were normal-weight group (-2SD≤WAZ≤+2SD), individually-matched for sex, age, and ethnicity. Anthropometric measurements included weight and height; and body composition was measured by bioelectrical impedance analysis. Muscular strength of both hands was assessed independently by hand-held dynamometer. Bone status was evaluated using a radial quantitative ultrasound system at one-third distal radius of the non-dominant hand. Anthropometric measurements and bone status were not significantly different between the sexes. Boys had significantly higher muscular strength and lean mass (p<0.05), but lower fat mass when compared to the girls (p<0.01). In both sexes, the undernourished group presented significantly lower anthropometric and body composition measurements and muscular strength than their normal-weight counterparts (p<0.001). However, no significant differences were observed for bone status between the two weight groups in boys (p = 0.09) and girls (p = 0.98). These findings imply that undernutrition can have profound negative impact on body composition as well

  9. Influence of bench angle on upper extremity muscular activation during bench press exercise.

    Science.gov (United States)

    Lauver, Jakob D; Cayot, Trent E; Scheuermann, Barry W

    2016-01-01

    This study compared the muscular activation of the pectoralis major, anterior deltoid and triceps brachii during a free-weight barbell bench press performed at 0°, 30°, 45° and -15° bench angles. Fourteen healthy resistance trained males (age 21.4 ± 0.4 years) participated in this study. One set of six repetitions for each bench press conditions at 65% one repetition maximum were performed. Surface electromyography (sEMG) was utilised to examine the muscular activation of the selected muscles during the eccentric and concentric phases. In addition, each phase was subdivided into 25% contraction durations, resulting in four separate time points for comparison between bench conditions. The sEMG of upper pectoralis displayed no difference during any of the bench conditions when examining the complete concentric contraction, however differences during 26-50% contraction duration were found for both the 30° [122.5 ± 10.1% maximal voluntary isometric contraction (MVIC)] and 45° (124 ± 9.1% MVIC) bench condition, resulting in greater sEMG compared to horizontal (98.2 ± 5.4% MVIC) and -15 (96.1 ± 5.5% MVIC). The sEMG of lower pectoralis was greater during -15° (100.4 ± 5.7% MVIC), 30° (86.6 ± 4.8% MVIC) and horizontal (100.1 ± 5.2% MVIC) bench conditions compared to the 45° (71.9 ± 4.5% MVIC) for the whole concentric contraction. The results of this study support the use of a horizontal bench to achieve muscular activation of both the upper and lower heads of the pectoralis. However, a bench incline angle of 30° or 45° resulted in greater muscular activation during certain time points, suggesting that it is important to consider how muscular activation is affected at various time points when selecting bench press exercises.

  10. Muscle MRI findings in facioscapulohumeral muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Gerevini, Simonetta; Caliendo, Giandomenico; Falini, Andrea [IRCCS San Raffaele Scientific Institute, Neuroradiology Unit, Head and Neck Department, Milan (Italy); Scarlato, Marina; Previtali, Stefano Carlo [IRCCS San Raffaele Scientific Institute, Department of Neurology, INSPE and Division of Neuroscience, Milan (Italy); Maggi, Lorenzo; Pasanisi, Barbara; Morandi, Lucia [Fondazione IRCCS Istituto Neurologico ' ' Carlo Besta' ' , Neuromuscular Diseases and Neuroimmunology Unit, Milan (Italy); Cava, Mariangela [IRCCS San Raffaele Scientific Institute, Department of Radiology and Center for Experimental Imaging, Milan (Italy)

    2016-03-15

    Facioscapulohumeral muscular dystrophy (FSHD) is characterized by extremely variable degrees of facial, scapular and lower limb muscle involvement. Clinical and genetic determination can be difficult, as molecular analysis is not always definitive, and other similar muscle disorders may have overlapping clinical manifestations. Whole-body muscle MRI examination for fat infiltration, atrophy and oedema was performed to identify specific patterns of muscle involvement in FSHD patients (30 subjects), and compared to a group of control patients (23) affected by other myopathies (NFSHD). In FSHD patients, we detected a specific pattern of muscle fatty replacement and atrophy, particularly in upper girdle muscles. The most frequently affected muscles, including paucisymptomatic and severely affected FSHD patients, were trapezius, teres major and serratus anterior. Moreover, asymmetric muscle involvement was significantly higher in FSHD as compared to NFSHD patients. In conclusion, muscle MRI is very sensitive for identifying a specific pattern of involvement in FSHD patients and in detecting selective muscle involvement of non-clinically testable muscles. Muscle MRI constitutes a reliable tool for differentiating FSHD from other muscular dystrophies to direct diagnostic molecular analysis, as well as to investigate FSHD natural history and follow-up of the disease. (orig.)

  11. Carrier screening for spinal muscular atrophy in Italian population

    Indian Academy of Sciences (India)

    SMA is necessary for effective clinical/prenatal diagnosis ... of SMA critical region in the group of 450 normal controls. ... izing and quality test are as described in Calì et al. .... nosis for spinal muscular atrophy: clinical laboratory analysis of.

  12. Predictive factors for masticatory performance in Duchenne muscular dystrophy

    NARCIS (Netherlands)

    Bruggen, H.W. van; Engel-Hoek, L. van den; Steenks, M.H.; Bronkhorst, E.M.; Creugers, N.H.; Groot, I.J.M. de; Kalaykova, S.

    2014-01-01

    Patients with Duchenne muscular dystrophy (DMD) report masticatory and swallowing problems. Such problems may cause complications such as choking, and feeling of food sticking in the throat. We investigated whether masticatory performance in DMD is objectively impaired, and explored predictive

  13. Physical complaints in ageing persons with spinal muscular atrophy.

    NARCIS (Netherlands)

    Groot, I.J.M. de; Witte, L.P de

    2005-01-01

    OBJECTIVE: While life expectancy is improving for persons with spinal muscular atrophy, new physical complaints may arise. To investigate this, we studied persons with a long duration and severe course (high functional limitations) of the disease. DESIGN: Cross-sectional descriptive study.

  14. The relationship between the drive for muscularity and muscle dysmorphia in male and female weight trainers.

    Science.gov (United States)

    Robert, Courtney A; Munroe-Chandler, Krista J; Gammage, Kimberley L

    2009-09-01

    Muscle dysmorphia is a form of body dysmorphic disorder in which individuals have a pathological preoccupation with their muscularity and, more specifically, an extreme fear that their bodies are too small. Relatively few empirical studies have been completed on muscle dysmorphia, and even fewer studies on the relationship between the drive for muscularity and muscle dysmorphia in men and women. The purpose of this research was to examine the relationship between the drive for muscularity and muscle dysmorphia in male (n = 55) and female (n = 59) recreational weight trainers. Results revealed that the behavior and diet subscales of the drive for muscularity significantly predicted muscle dysmorphia in males and females accounting for 69% and 46% of the total variance, respectively. Although the overall scores of muscle dysmorphia do not indicate clinical levels, these findings suggest that behaviors such as arranging one's schedule around his/her training regimen and dieting in order to gain muscle predict characteristics of muscle dysmorphia in men and women.

  15. Validation of the Spanish version of the Drive for Muscularity Scale (DMS) among males: Confirmatory factor analysis.

    Science.gov (United States)

    Sepulveda, Ana R; Parks, Melissa; de Pellegrin, Yolanda; Anastasiadou, Dimitra; Blanco, Miriam

    2016-04-01

    Drive for Muscularity (DM) has been shown to be a relevant construct for measuring and understanding male body image. For this reason, it is important to have reliable and valid instruments with which to measure DM, and to date no such instruments exist in Spain. This study analyzes the psychometric and structural properties of the Drive for Muscularity Scale (DMS) in a sample of Spanish adolescent males (N=212), with the aim of studying the structural validity of the scale by using a confirmatory factor analysis (CFA), as well as analyzing the internal consistency and construct (convergent and discriminant) and concurrent validity of the instrument. After testing three models, results indicated that the best structure was a two-dimensional model, with the factors of muscularity-oriented body image (MBI) and muscularity behavior (MB). The scale showed good internal consistency (α=.90) and adequate construct validity. Furthermore, significant associations were found between DM and increased difficulties in emotional regulation (rho=.37) and low self-esteem (rho=-.19). Findings suggest that the two-factor structure may be used when assessing drive for muscularity among adolescent males in Spain. Copyright © 2016 Elsevier Ltd. All rights reserved.

  16. Diferenciação de células-tronco mesenquimais derivadas do tecido adiposo em cardiomiócitos

    Directory of Open Access Journals (Sweden)

    Pablo Herthel Carvalho

    2013-01-01

    Full Text Available FUNDAMENTO: O potencial de renovação e proliferação dos cardiomiócitos, in vivo, é pequeno, e por isso, o músculo cardíaco apresenta limitada capacidade de repor células perdidas. Na tentativa de minimizar os danos oriundos de lesões hipóxico-isquêmicas e daquelas que acometem o sistema de condução do coração, a terapia celular com células-tronco mesenquimais (MSC vem sendo utilizada, inclusive com cardiomiócitos diferenciados a partir de MSC. OBJETIVO: O presente trabalho comparou três protocolos distintos de indução de diferenciação objetivando a sugestão de um método viável para a diferenciação de maior número de células funcionais que expressem fenótipo cardiomiogênico. MÉTODOS: Culturas de MSC obtidas de tecido adiposo de ratos jovens da linhagem Lewis transgênicos para proteína verde fluorescente (GFP foram submetidos a três diferentes meios de diferenciação cardiogênica: Planat-Bérnard, 5-azacitidina e meio Planat-Bérnard + 5-azacitidina e observadas quanto a expressão de marcadores celulares cardíacos. RESULTADOS: Nos três protolocos utilizados observou-se formação da proteína alfa-actinina sarcomérica no citoesqueleto das células submetidas à diferenciação, expressão de conexina 43 na membrana nuclear e citoplasmática e formação de gap junctions, necessárias para a propagação do impulso elétrico no miocárdio, contudo, em nenhum protocolo foi observada contração espontânea das células submetidas à diferenciação cardiogênica. CONCLUSÃO: A indução com 5-azacitidina proporcionou diferenciação celular cadiomiogênica efetiva e similar à encontrada com o meio Planat-Bénard e, por ser um protocolo mais simples, rápido e com menor custo torna-se o método de eleição.

  17. EEG windowed statistical wavelet scoring for evaluation and discrimination of muscular artifacts

    International Nuclear Information System (INIS)

    Vialatte, François-Benoit; Cichocki, Andrzej; Solé-Casals, Jordi

    2008-01-01

    EEG recordings are usually corrupted by spurious extra-cerebral artifacts, which should be rejected or cleaned up by the practitioner. Since manual screening of human EEGs is inherently error prone and might induce experimental bias, automatic artifact detection is an issue of importance. Automatic artifact detection is the best guarantee for objective and clean results. We present a new approach, based on the time–frequency shape of muscular artifacts, to achieve reliable and automatic scoring. The impact of muscular activity on the signal can be evaluated using this methodology by placing emphasis on the analysis of EEG activity. The method is used to discriminate evoked potentials from several types of recorded muscular artifacts—with a sensitivity of 98.8% and a specificity of 92.2%. Automatic cleaning of EEG data is then successfully realized using this method, combined with independent component analysis. The outcome of the automatic cleaning is then compared with the Slepian multitaper spectrum based technique introduced by Delorme et al (2007 Neuroimage 34 1443–9)

  18. Primary muscular hydatidosis. US, CT and MR findings

    Energy Technology Data Exchange (ETDEWEB)

    Alexiadis, G.; Deftereos, S.; Manavis, J. [Democritus Univ. of Thrace, Alexandroupolis (Greece). Dept. of Radiology; Lambropoulou, M.; Papadopoulos, N. [Democritus Univ. of Thrace, Alexandroupolis (Greece). Dept. of Pathology

    2002-07-01

    We present a rare case of primary muscular hydatidosis in the left thigh of a 40-year-old female patient. US, CT and MR imaging showed a typical multilocular hydatid cyst deep in the vastus intermedius and vastus medialis muscles. Histopathological examination, which followed surgical excision, established the diagnosis of echinococcus cyst.

  19. Primary muscular hydatidosis. US, CT and MR findings

    International Nuclear Information System (INIS)

    Alexiadis, G.; Deftereos, S.; Manavis, J.; Lambropoulou, M.; Papadopoulos, N.

    2002-01-01

    We present a rare case of primary muscular hydatidosis in the left thigh of a 40-year-old female patient. US, CT and MR imaging showed a typical multilocular hydatid cyst deep in the vastus intermedius and vastus medialis muscles. Histopathological examination, which followed surgical excision, established the diagnosis of echinococcus cyst

  20. Phosphorylation of intact erythrocytes in human muscular dystrophy

    International Nuclear Information System (INIS)

    Johnson, R.M.; Nigro, M.

    1986-01-01

    The uptake of exogenous 32 Pi into the membrane proteins of intact erythrocytes was measured in 8 patients with Duchenne muscular dystrophy. No abnormalities were noted after autoradiographic analysis. This contrasts with earlier results obtained when isolated membranes were phosphorylated with gamma-[ 32 P]ATP, and suggests a possible reinterpretation of those experiments

  1. Muscular Calf Injuries in Runners.

    Science.gov (United States)

    Fields, Karl B; Rigby, Michael D

    2016-01-01

    Calf pain is a common complaint among runners of all ages but is most frequent in masters athletes. This article focuses on injuries to the triceps surae or true 'calf muscles.' The most common calf injury is a tear of the medial gastrocnemius muscle (Tennis Leg) but other structures including the lateral gastrocnemius, plantaris and soleus also may be the cause of muscular pain. This article looks at the presentation, evaluation, and treatment of these injuries. We also highlight some examples of musculoskeletal ultrasound which is a valuable tool for rapid diagnosis of the cause and extent of injury.

  2. Eficacia do metodo Meir Schneider de autocuidado em pessoas com distrofias musculares progressivas : ensaio clinico fase II

    OpenAIRE

    Lea Beatriz Teixeira Soares

    1999-01-01

    Resumo: As pessoas com distrofias musculares progressivas vivem o limite do conhecimento médico e aguardam resultados de pesquisa da prevenção, reabilitação e mesmo a cura dos indivíduos portadores. Nesse contexto discute-se a eficácia do método Meir Schneider de autocuidado (self-healing) baseado em ensaio clínico fase II em 12 pessoas com distrofias musculares progressivas, sob tratamento ambulatorial intensivo e não intensivo. Foram atendidos 7 portadores de Distrofia Muscular Duchenne- DM...

  3. A new chart for weight control in Duchenne muscular dystrophy.

    OpenAIRE

    Griffiths, R D; Edwards, R H

    1988-01-01

    Weight control is desirable in the muscle wasting conditions. A new chart is presented to allow the prediction of an ideal weight, free of excess fat, specifically for boys with Duchenne muscular dystrophy.

  4. Effect of the bitterness of food on muscular activity and masticatory movement.

    Science.gov (United States)

    Okada, Yamato; Shiga, Hiroshi

    2017-10-01

    The purpose of this study was to clarify the effect of the bitterness of food on muscular activity and masticatory movement. Twenty healthy subjects were asked to chew a non-bitter gummy jelly and a bitter gummy jelly on their habitual chewing side. The masseter muscular activity and the movement of mandibular incisal point were recorded simultaneously. For all cycles excluding the first cycle, parameters representing the muscular activity (total integral value and integral value per cycle) and masticatory movement (path, rhythm, and stability) were calculated and compared between the two types of gummy jellies. The total integral value of masseter muscular activity during the chewing of bitter gummy jelly was significantly smaller than during the chewing of non-bitter gummy jelly, however, no definite trends in the integral value per cycle and the stability of movement were observed. The parameters representing the movement path tended to be small during the chewing of bitter gummy jelly than during the chewing of non-bitter gummy jelly. The masticatory width was significantly smaller during the chewing of bitter gummy jelly. The parameters representing the rhythm of movement were significantly longer during the chewing of bitter gummy jelly than during the chewing of non-bitter gummy jelly. From these results it was suggested that the bitterness of food does not affect the integral value per cycle or the stability of the masticatory movement, but it does affect the movement path and rhythm, with narrowing of the path and slowing of the rhythm. Copyright © 2017 Japan Prosthodontic Society. Published by Elsevier Ltd. All rights reserved.

  5. A human in vitro model of Duchenne muscular dystrophy muscle formation and contractility.

    Science.gov (United States)

    Nesmith, Alexander P; Wagner, Matthew A; Pasqualini, Francesco S; O'Connor, Blakely B; Pincus, Mark J; August, Paul R; Parker, Kevin Kit

    2016-10-10

    Tongue weakness, like all weakness in Duchenne muscular dystrophy (DMD), occurs as a result of contraction-induced muscle damage and deficient muscular repair. Although membrane fragility is known to potentiate injury in DMD, whether muscle stem cells are implicated in deficient muscular repair remains unclear. We hypothesized that DMD myoblasts are less sensitive to cues in the extracellular matrix designed to potentiate structure-function relationships of healthy muscle. To test this hypothesis, we drew inspiration from the tongue and engineered contractile human muscle tissues on thin films. On this platform, DMD myoblasts formed fewer and smaller myotubes and exhibited impaired polarization of the cell nucleus and contractile cytoskeleton when compared with healthy cells. These structural aberrations were reflected in their functional behavior, as engineered tongues from DMD myoblasts failed to achieve the same contractile strength as healthy tongue structures. These data suggest that dystrophic muscle may fail to organize with respect to extracellular cues necessary to potentiate adaptive growth and remodeling. © 2016 Nesmith et al.

  6. Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I

    DEFF Research Database (Denmark)

    Løkken, Nicoline; Hedermann, Gitte; Thomsen, Carsten

    2016-01-01

    We investigated whether a linear relationship between muscle strength and cross-sectional area (CSA) is preserved in calf muscles of patients with Becker muscular dystrophy (BMD, n = 14) and limb-girdle type 2I muscular dystrophy (LGMD2I, n = 11), before and after correcting for muscle fat...

  7. Muscular fatigue in response to different modalities of CrossFit sessions.

    Directory of Open Access Journals (Sweden)

    José Luis Maté-Muñoz

    Full Text Available CrossFit is a new strength and conditioning regimen involving short intense daily workouts called workouts of the day (WOD. This study assesses muscular fatigue levels induced by the three modalities of CrossFit WOD; gymnastics (G, metabolic conditioning (M and weightlifting (W.34 healthy subjects undertook three WOD (one per week: a G WOD consisting of completing the highest number of sets of 5 pull-ups, 10 push-ups and 15 air squats in 20 min; an M WOD, in which the maximum number of double skipping rope jumps was executed in 8 sets (20 s, resting (10 s between sets; and finally, a W WOD in which the maximum number of power cleans was executed in 5 min, lifting a load equivalent to 40% of the individual's 1RM. Before and after each WOD, blood lactate concentrations were measured. Also, before, during, and after each WOD, muscular fatigue was assessed in a countermovement jump test (CMJ.Significant reductions were produced in the mechanical variables jump height, average power and maximum velocity in response to G; and in jump height, mean and peak power, maximum velocity and maximum force in response to W (P<0.01. However, in M, significant reductions in mechanical variables were observed between pre- and mid session (after sets 2, 4, 6 and 8, but not between pre- and post session.Muscular fatigue, reflected by reduced CMJ variables, was produced following the G and W sessions, while recovery of this fatigue was observed at the end of M, likely attributable to rest intervals allowing for the recovery of phosphocreatine stores. Our findings also suggest that the high intensity and volume of exercise in G and W WODs could lead to reduced muscular-tendon stiffness causing a loss of jump ability, related here to a longer isometric phase during the CMJ.

  8. Muscular fatigue in response to different modalities of CrossFit sessions.

    Science.gov (United States)

    Maté-Muñoz, José Luis; Lougedo, Juan H; Barba, Manuel; García-Fernández, Pablo; Garnacho-Castaño, Manuel V; Domínguez, Raúl

    2017-01-01

    CrossFit is a new strength and conditioning regimen involving short intense daily workouts called workouts of the day (WOD). This study assesses muscular fatigue levels induced by the three modalities of CrossFit WOD; gymnastics (G), metabolic conditioning (M) and weightlifting (W). 34 healthy subjects undertook three WOD (one per week): a G WOD consisting of completing the highest number of sets of 5 pull-ups, 10 push-ups and 15 air squats in 20 min; an M WOD, in which the maximum number of double skipping rope jumps was executed in 8 sets (20 s), resting (10 s) between sets; and finally, a W WOD in which the maximum number of power cleans was executed in 5 min, lifting a load equivalent to 40% of the individual's 1RM. Before and after each WOD, blood lactate concentrations were measured. Also, before, during, and after each WOD, muscular fatigue was assessed in a countermovement jump test (CMJ). Significant reductions were produced in the mechanical variables jump height, average power and maximum velocity in response to G; and in jump height, mean and peak power, maximum velocity and maximum force in response to W (P<0.01). However, in M, significant reductions in mechanical variables were observed between pre- and mid session (after sets 2, 4, 6 and 8), but not between pre- and post session. Muscular fatigue, reflected by reduced CMJ variables, was produced following the G and W sessions, while recovery of this fatigue was observed at the end of M, likely attributable to rest intervals allowing for the recovery of phosphocreatine stores. Our findings also suggest that the high intensity and volume of exercise in G and W WODs could lead to reduced muscular-tendon stiffness causing a loss of jump ability, related here to a longer isometric phase during the CMJ.

  9. The effects of exposure to muscular male models among men: exploring the moderating role of gym use and exercise motivation.

    Science.gov (United States)

    Halliwell, Emma; Dittmar, Helga; Orsborn, Amber

    2007-09-01

    This study examines the effects of exposure to the muscular male body ideal on body-focused negative affect among male gym users and non-exercisers. As hypothesized, the impact of media exposure depended on men's exercise status. Non-exercisers (n = 58) reported greater body-focused negative affect after exposure to images of muscular male models than after neutral images (no model controls), whereas gym users (n = 58) showed a tendency for less body-focused negative affect after the model images than after the control images. Furthermore, the extent to which gym users were motivated to increase strength and muscularity moderated these exposure effects; men who reported stronger strength and muscularity exercise motivation reported a greater degree of self-enhancement after exposure to the muscular ideal. The findings are interpreted with respect to likely differences in motives for social comparisons.

  10. A study of muscular tissue of animal origin by reflection-spectroscopy methods

    Science.gov (United States)

    Plotnikova, L. V.; Nechiporenko, A. P.; Orekhova, S. M.; Plotnikov, P. P.; Ishevskii, A. L.

    2017-06-01

    A comparative analysis of the spectral characteristics of the surface of muscular tissue of animal origin (pork) and its main components has been performed by the methods of diffuse reflection electronic spectroscopy (DRES) and frustrated total internal reflection IR spectroscopy. The experiments have shown that the application of the DRES method makes it possible to detect more pronounced changes in the surface optical characteristics of muscular tissue and obtain electronic spectra containing information about the component composition of its main parts under successive extraction of sarcoplasmic materials, myofibrillar proteins of the actomyosin complex, and stroma mucopolysaccharides.

  11. Exercise dependence and the drive for muscularity in male bodybuilders, power lifters, and fitness lifters.

    Science.gov (United States)

    Hale, Bruce D; Roth, Andrew D; DeLong, Ryan E; Briggs, Michael S

    2010-06-01

    Researchers have hypothesized differences in exercise dependence and drive for muscularity between bodybuilders and power lifters, while others have not found the predicted differences. This study assessed 146 weight lifters (bodybuilders, n=59; power lifters, n=47; fitness lifters, n=40) on the Exercise Dependence Scale, Bodybuilding Dependence Scale, and the Drive for Muscularity Scale. Results showed that bodybuilders and power lifters were significantly higher than fitness lifters on EDS Total, 7 EDS scales, and the 3 BDS scales. In contrast, power lifters were found to be significantly higher on DMS Total and DMS Behavior scales than bodybuilders. The regression results suggest that exercise dependence may be directly related to the drive for muscularity. 2010 Elsevier Ltd. All rights reserved.

  12. The potential of sarcospan in adhesion complex replacement therapeutics for the treatment of muscular dystrophy

    Science.gov (United States)

    Marshall, Jamie L.; Kwok, Yukwah; McMorran, Brian; Baum, Linda G.; Crosbie-Watson, Rachelle H.

    2013-01-01

    Three adhesion complexes span the sarcolemma and facilitate critical connections between the extracellular matrix and the actin cytoskeleton: the dystrophin- and utrophin-glycoprotein complexes and α7β1 integrin. Loss of individual protein components results in a loss of the entire protein complex and muscular dystrophy. Muscular dystrophy is a progressive, lethal wasting disease characterized by repetitive cycles of myofiber degeneration and regeneration. Protein replacement therapy offers a promising approach for the treatment of muscular dystrophy. Recently, we demonstrated that sarcospan facilitates protein-protein interactions amongst the adhesion complexes and is an important therapeutic target. Here, we review current protein replacement strategies, discuss the potential benefits of sarcospan expression, and identify important experiments that must be addressed for sarcospan to move to the clinic. PMID:23601082

  13. [Optimal solution and analysis of muscular force during standing balance].

    Science.gov (United States)

    Wang, Hongrui; Zheng, Hui; Liu, Kun

    2015-02-01

    The present study was aimed at the optimal solution of the main muscular force distribution in the lower extremity during standing balance of human. The movement musculoskeletal system of lower extremity was simplified to a physical model with 3 joints and 9 muscles. Then on the basis of this model, an optimum mathematical model was built up to solve the problem of redundant muscle forces. Particle swarm optimization (PSO) algorithm is used to calculate the single objective and multi-objective problem respectively. The numerical results indicated that the multi-objective optimization could be more reasonable to obtain the distribution and variation of the 9 muscular forces. Finally, the coordination of each muscle group during maintaining standing balance under the passive movement was qualitatively analyzed using the simulation results obtained.

  14. Physical Activity in Boys With Duchenne Muscular Dystrophy Is Lower and Less Demanding Compared to Healthy Boys

    NARCIS (Netherlands)

    Heutinck, L.B.; Kampen, N. van; Jansen, M.; Groot, I.J.M. de

    2017-01-01

    This study describes the amount of physical activity and perception of physical activity in boys with Duchenne muscular dystrophy (DMD) compared to healthy boys. A questionnaire described 6 domains of physical activity. Four Duchenne muscular dystrophy subgroups were made: early and late ambulatory,

  15. Fighting against disuse of the masticatory system in duchenne muscular dystrophy : A pilot study using chewing gum

    NARCIS (Netherlands)

    Van Bruggen, H. Willemijn; Van Den Engel-Hoek, Lenie; Steenks, Michel H.; Van Der Bilt, Andries; Bronkhorst, Ewald M.; Creugers, Nico H J; De Groot, Imelda J M; Kalaykova, Stanimira I.

    2015-01-01

    Duchenne muscular dystrophy patients report masticatory problems. The aim was to determine the efficacy of mastication training in Duchenne muscular dystrophy using chewing gum for 4 weeks. In all, 17 patients and 17 healthy age-matched males participated. The masticatory performance was assessed

  16. Instructions to Adopt an External Focus Enhance Muscular Endurance

    Science.gov (United States)

    Marchant, David C.; Greig, Matt; Bullough, Jonathan; Hitchen, Daniel

    2011-01-01

    The influence of internal (movement focus) and external (outcome focus) attentional-focusing instructions on muscular endurance were investigated using three exercise protocols with experienced exercisers. Twenty-three participants completed a maximal repetition, assisted bench-press test on a Smith's machine. An external focus of attention…

  17. Genetic diagnosis of Duchenne and Becker muscular dystrophy using multiplex ligation-dependent probe amplification in Rwandan patients.

    Science.gov (United States)

    Uwineza, Annette; Hitayezu, Janvier; Murorunkwere, Seraphine; Ndinkabandi, Janvier; Kalala Malu, Celestin Kaputu; Caberg, Jean Hubert; Dideberg, Vinciane; Bours, Vincent; Mutesa, Leon

    2014-04-01

    Duchenne and Becker muscular dystrophies are the most common clinical forms of muscular dystrophies. They are genetically X-linked diseases caused by a mutation in the dystrophin (DMD) gene. A genetic diagnosis was carried out in six Rwandan patients presenting a phenotype of Duchenne and Becker muscular dystrophies and six asymptomatic female carrier relatives using multiplex ligation-dependent probe amplification (MLPA). Our results revealed deletion of the exons 48-51 in one patient, an inherited deletion of the exons 8-21 in two brothers and a de novo deletion of the exons 46-50 in the fourth patient. No copy number variation was found in two patients. Only one female carrier presented exon deletion in the DMD gene. This is the first cohort of genetic analysis in Rwandan patients affected by Duchenne and Becker muscular dystrophies. This report confirmed that MLPA assay can be easily implemented in low-income countries.

  18. Programa de entrenamiento de hipertrofia muscular para un jugador de baloncesto en postemporada

    OpenAIRE

    Sol Plaza, Javier del

    2013-01-01

    El trabajo pretende mostrar la planificación, programación y periodización de un programa de entrenamiento para el desarrollo de la hipertrofia muscular en un jugador de baloncesto en postemporada. El objeto para el desarrollo de esta propuesta teórica es un jugador con un perfil joven, cuya experiencia en la liga ACB de baloncesto es de aproximadamente un año. El objetivo para este jugador es la ganancia de masa muscular durante la postemporada, es decir, durante el peri...

  19. Dystrophin Immunity in Duchenne’s Muscular Dystrophy

    OpenAIRE

    Mendell, Jerry R.; Campbell, Katherine; Rodino-Klapac, Louise; Sahenk, Zarife; Shilling, Chris; Lewis, Sarah; Bowles, Dawn; Gray, Steven; Li, Chengwen; Galloway, Gloria; Malik, Vinod; Coley, Brian; Clark, K. Reed; Li, Juan; Xiao, Xiao

    2010-01-01

    We report on delivery of a functional dystrophin transgene to skeletal muscle in six patients with Duchenne’s muscular dystrophy. Dystrophin-specific T cells were detected after treatment, providing evidence of transgene expression even when the functional protein was not visualized in skeletal muscle. Circulating dystrophin-specific T cells were unexpectedly detected in two patients before vector treatment. Revertant dystrophin fibers, which expressed functional, truncated dystrophin from th...

  20. Ensayo no destructivo de soldaduras en pernos conectores mediante inspección acústica

    OpenAIRE

    Aznar, A.; Cervera, J.; Ortiz, J.; Hernando, J. I.

    2012-01-01

    Los pernos conectores aportan múltiples ventajas de uso, entre las que se encuentra el elevado margen de seguridad que ofrecen sus soldaduras ejecutadas mediante arco eléctrico. Estas soldaduras, aunque ampliamente fiables, son difícilmente comprobadas mediante ensayos no destructivos. Aparte de la inspección visual, que aporta gran información sobre la calidad de ejecución de la soldadura, el resto de ensayos no destructivos (líquidos penetrantes, partículas magnéticas, ultrasonidos, radiogr...

  1. A case report of cardia cancer complicated with idiopathic muscular hypertrophy of the oesophagus treated with thoracoscopic surgery.

    Science.gov (United States)

    Ren, Jun; Hao, Yingtao; Peng, Chuanliang

    2018-01-01

    The incidence of idiopathic muscular hypertrophy of oesophagus (IMHE) is low, and cancer with IMHE, showing significant hypertrophy of muscular layer of middle part of the oesophagus and successfully treated with minimally invasive thoracoscopic surgery.

  2. Acute irradiation and muscular fibrosis. Development and characteristics in the pig

    International Nuclear Information System (INIS)

    Lefaix, J.L.; Daburon, F.; Remy, J.

    1989-01-01

    This study was performed in an experimental porcin model of acute local irradiation chosen to simulate human accidents. It enabled to determine the development and the physiopathological characteristics of the fibrous tissue which developed in skeletal muscle. In the first month after irradiation the strong inflammatory reaction initiating the radiation induced fibrosis was characterized by edema as visualized on NMR imaging and by acute phase reactant protein changes, associated with elevations of local and general temperatures in irradiated animals. At the margin of the irradiated tissue, atypical fibroblasts isolated among collagen bundles or bunched in nodullary reinforcement were seen associated with intense capillary neogenesis. Several months after irradiation normal skeletal muscle was replaced by atrophic fibrosis delimited by an inflammatory perifibrotic tissue. The muscular fibrosis was characterized by a high atypical fibroblasts density and by an inflammatory distribution pattern of collagen types I, III, IV, laminin, fibronectin and fibrinogen as visualized by immunohistochemical methods. Biochemical results showed an increase in collagen content and synthesis in fibrotic tissue whereas perifibrotic zone synthesized more non collagenous proteins compared with the normal muscle. The contributions of granulation tissue, cellular mediators and inhibition of muscular regeneration to maintain the atrophic character of the muscular radiation induced fibrosis are discussed [fr

  3. From proteins to genes: immunoanalysis in the diagnosis of muscular dystrophies

    Directory of Open Access Journals (Sweden)

    Barresi Rita

    2011-06-01

    Full Text Available Abstract Muscular dystrophies are a large heterogeneous group of inherited diseases that cause progressive muscle weakness and permanent muscle damage. Very few muscular dystrophies show sufficient specific clinical features to allow a definite diagnosis. Because of the currently limited capacity to screen for numerous genes simultaneously, muscle biopsy is a time and cost-effective test for many of these disorders. Protein analysis interpreted in correlation with the clinical phenotype is a useful way of directing genetic testing in many types of muscular dystrophies. Immunohistochemistry and western blot are complementary techniques used to gather quantitative and qualitative information on the expression of proteins involved in this group of diseases. Immunoanalysis has a major diagnostic application mostly in recessive conditions where the absence of labelling for a particular protein is likely to indicate a defect in that gene. However, abnormalities in protein expression can vary from absence to very subtle reduction. It is good practice to test muscle biopsies with antibodies for several proteins simultaneously and to interpret the results in context. Indeed, there is a degree of direct or functional association between many of these proteins that is reflected by the presence of specific secondary abnormalities that are of value, especially when the diagnosis is not straightforward.

  4. MRI for the demonstration of subclinical muscle involvement in muscular dystrophy

    International Nuclear Information System (INIS)

    Sookhoo, S.; MacKinnon, I.; Bushby, K.; Chinnery, P.F.; Birchall, D.

    2007-01-01

    Aim: To compare magnetic resonance imaging (MRI) with clinical examination for the detection of muscle abnormality in patients with muscular dystrophy. Methods: Muscle power in 20 patients with a variety of forms of muscular dystrophy was examined clinically using the Medical Research Council (MRC) grading scale, and patients were subsequently imaged with MRI. MRI and clinical examination for the detection of muscle normality and abnormality were compared using a McNemar chi-squared test to examine differences between the two methods. Results: MRI demonstrated radiological evidence of muscle abnormality more often than clinical examination; 50% of movements assessed as normal on clinical examination were associated with muscle abnormalities on MRI, including a significant proportion where there was severe radiological abnormality, indicating that focally advanced disease may be undetectable clinically. Conclusion: The combination of clinical examination and MRI could improve the accuracy of phenotypic characterization of patients with muscular dystrophy, and this in turn could allow a more focussed molecular analysis through muscle biopsy or genetic investigation. This may also be very helpful in the assessment of the degree of muscle compromise not only in the early phases of the disease but especially during follow-up and can be used in therapeutic trials

  5. Dystrophin analysis in carriers of Duchenne and Becker muscular dystrophy

    NARCIS (Netherlands)

    Hoogerwaard, Edo M.; Ginjaar, Ieke B.; Bakker, Egbert; de Visser, Marianne

    2005-01-01

    Associations between clinical phenotype (muscle weakness, dilated cardiomyopathy) and dystrophin abnormalities in muscle tissue among definite carriers of Duchenne (DMD) and Becker muscular dystrophy (BMD) were investigated. No associations between dystrophin abnormalities and clinical variables in

  6. Orthodontic treatment in a patient with unilateral open-bite and Becker muscular dystrophy. A 5-year follow-up

    Directory of Open Access Journals (Sweden)

    Juan Fernando Aristizabal

    2014-12-01

    Full Text Available INTRODUCTION: Becker muscular dystrophy is an X-chromosomal linked anomaly characterized by progressive muscle wear and weakness. This case report shows the orthodontic treatment of a Becker muscular dystrophy patient with unilateral open bite.METHODS: To correct patient's malocclusion, general anesthesia and orthognathic surgery were not considered as an option. Conventional orthodontic treatment with intermaxillary elastics and muscular functional therapy were employed instead.RESULTS: After 36 months, open bite was corrected. The case remains stable after a 5-year post-treatment retention period.

  7. Serum Osteopontin as a Novel Biomarker for Muscle Regeneration in Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Kuraoka, Mutsuki; Kimura, En; Nagata, Tetsuya; Okada, Takashi; Aoki, Yoshitsugu; Tachimori, Hisateru; Yonemoto, Naohiro; Imamura, Michihiro; Takeda, Shin'ichi

    2016-05-01

    Duchenne muscular dystrophy is a lethal X-linked muscle disorder. We have already reported that osteopontin (OPN), an inflammatory cytokine and myogenic factor, is expressed in the early dystrophic phase in canine X-linked muscular dystrophy in Japan, a dystrophic dog model. To further explore the possibility of OPN as a new biomarker for disease activity in Duchenne muscular dystrophy, we monitored serum OPN levels in dystrophic and wild-type dogs at different ages and compared the levels to other serum markers, such as serum creatine kinase, matrix metalloproteinase-9, and tissue inhibitor of metalloproteinase-1. Serum OPN levels in the dystrophic dogs were significantly elevated compared with those in wild-type dogs before and 1 hour after a cesarean section birth and at the age of 3 months. The serum OPN level was significantly correlated with the phenotypic severity of dystrophic dogs at the period corresponding to the onset of muscle weakness, whereas other serum markers including creatine kinase were not. Immunohistologically, OPN was up-regulated in infiltrating macrophages and developmental myosin heavy chain-positive regenerating muscle fibers in the dystrophic dogs, whereas serum OPN was highly elevated. OPN expression was also observed during the synergic muscle regeneration process induced by cardiotoxin injection. In conclusion, OPN is a promising biomarker for muscle regeneration in dystrophic dogs and can be applicable to boys with Duchenne muscular dystrophy. Copyright © 2016 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  8. ESTUDIO FISICOQUÍMICO DE LA SOLUBILIDAD DEL EUDRAGIT S100® EN ALGUNOS MEDIOS ACUOSOS Y ORGÁNICOS

    Directory of Open Access Journals (Sweden)

    Laura Villamizar

    2009-04-01

    Full Text Available En este trabajo se estudió la solubilidad del Eudragit S100®, respecto al pH en medios acuosos, y a la polaridad en solventes orgánicos, además se estimó el parámetro de solubilidad del polímero d, de forma experimental y teórica, mediante los métodos de contribución de grupos de Fedors y de van Krevelen, estableciendo su mapa de solubilidad. Los resultados mostraron que la solubilidad del polímero depende del pH siendo soluble a partir de pH 6,0. El valor d estimado experimentalmente fue de 12,9 ± 2,4 cal1/2 cm-3/2 siendo casi concordante con los obtenidos mediante los métodos de Fedors y de van Krevelen. Adicionalmente, se construyó el diagrama de Teas para la identificación de solventes y no-solventes para el polímero.

  9. Trainability of muscular activity level during maximal voluntary co-contraction: comparison between bodybuilders and nonathletes.

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    Sumiaki Maeo

    Full Text Available Antagonistic muscle pairs cannot be fully activated simultaneously, even with maximal effort, under conditions of voluntary co-contraction, and their muscular activity levels are always below those during agonist contraction with maximal voluntary effort (MVE. Whether the muscular activity level during the task has trainability remains unclear. The present study examined this issue by comparing the muscular activity level during maximal voluntary co-contraction for highly experienced bodybuilders, who frequently perform voluntary co-contraction in their training programs, with that for untrained individuals (nonathletes. The electromyograms (EMGs of biceps brachii and triceps brachii muscles during maximal voluntary co-contraction of elbow flexors and extensors were recorded in 11 male bodybuilders and 10 nonathletes, and normalized to the values obtained during the MVE of agonist contraction for each of the corresponding muscles (% EMGMVE. The involuntary coactivation level in antagonist muscle during the MVE of agonist contraction was also calculated. In both muscles, % EMGMVE values during the co-contraction task for bodybuilders were significantly higher (P<0.01 than those for nonathletes (biceps brachii: 66±14% in bodybuilders vs. 46±13% in nonathletes, triceps brachii: 74±16% vs. 57±9%. There was a significant positive correlation between a length of bodybuilding experience and muscular activity level during the co-contraction task (r = 0.653, P = 0.03. Involuntary antagonist coactivation level during MVE of agonist contraction was not different between the two groups. The current result indicates that long-term participation in voluntary co-contraction training progressively enhances muscular activity during maximal voluntary co-contraction.

  10. Trainability of Muscular Activity Level during Maximal Voluntary Co-Contraction: Comparison between Bodybuilders and Nonathletes

    Science.gov (United States)

    Maeo, Sumiaki; Takahashi, Takumi; Takai, Yohei; Kanehisa, Hiroaki

    2013-01-01

    Antagonistic muscle pairs cannot be fully activated simultaneously, even with maximal effort, under conditions of voluntary co-contraction, and their muscular activity levels are always below those during agonist contraction with maximal voluntary effort (MVE). Whether the muscular activity level during the task has trainability remains unclear. The present study examined this issue by comparing the muscular activity level during maximal voluntary co-contraction for highly experienced bodybuilders, who frequently perform voluntary co-contraction in their training programs, with that for untrained individuals (nonathletes). The electromyograms (EMGs) of biceps brachii and triceps brachii muscles during maximal voluntary co-contraction of elbow flexors and extensors were recorded in 11 male bodybuilders and 10 nonathletes, and normalized to the values obtained during the MVE of agonist contraction for each of the corresponding muscles (% EMGMVE). The involuntary coactivation level in antagonist muscle during the MVE of agonist contraction was also calculated. In both muscles, % EMGMVE values during the co-contraction task for bodybuilders were significantly higher (Pbodybuilders vs. 46±13% in nonathletes, triceps brachii: 74±16% vs. 57±9%). There was a significant positive correlation between a length of bodybuilding experience and muscular activity level during the co-contraction task (r = 0.653, P = 0.03). Involuntary antagonist coactivation level during MVE of agonist contraction was not different between the two groups. The current result indicates that long-term participation in voluntary co-contraction training progressively enhances muscular activity during maximal voluntary co-contraction. PMID:24260233

  11. Muscular strength and endurance and cardio-metabolic health in disadvantaged Hispanic children from the U.S.

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    Ryan D. Burns

    2017-03-01

    Full Text Available The predictive relationship between muscular strength and endurance and cardio-metabolic health, independent from aerobic fitness, is not clear in disadvantaged Hispanic children. The purpose of this study was to examine the predictive relationship between muscular strength and endurance and clustered cardio-metabolic risk, controlling for aerobic fitness, in Hispanic children from low-income schools. Participants were 320 Hispanic children (Mean age = 10.1 ± 1.1 years; 164 girls, 156 boys recruited during the 2014–2015 and 2015–2016 academic years from five low-income schools from the state of Utah in the U.S. Muscular strength and endurance was assessed using the push-up and curl-up tests and estimated VO2 Peak was calculated from the Progressive Aerobic Cardiovascular Endurance Run. A clustered metabolic syndrome composite score (MetS was calculated from cardio-metabolic health measurements consisting of HDL cholesterol, triglycerides, waist circumference, blood glucose, and mean arterial pressure (MAP. Multi-level general linear mixed effects models were used to examine the predictive relationship between muscular strength and endurance and MetS, controlling for the effect of aerobic fitness and the clustering of children within classrooms and schools. Children who were in the middle and upper tertiles for muscular strength and endurance associated with a lower (more favorable MetS score (middle tertile: β = −2.59, 95% C.I. [−4.23, −0.95], p < 0.05; upper tertile: β = −1.57, 95% C.I. [−3.20, −0.16], p < 0.05. The results suggest that higher levels of muscular strength and endurance relate to lower cardio-metabolic risk, independent of aerobic fitness, in Hispanic children from low-income schools.

  12. Immunological identification of a high molecular weight protein as a condidate for the product of the Duchenne muscular dystrophy gene

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    Kao, L.; Krstenansky, J.; Mendell, J.; Rammohan, K.W.; Gruenstein, E. (Univ. of Cincinnati College of Medicine, OH (USA))

    1988-06-01

    An oligopeptide was synthesized based on translation of the nucleotide sequence of the putative exon region of clone pERT87-25 from the gene for Duchenne muscular dystrophy. Immunization of rabbits with this oligopeptide induced the formation of antibodies directed against a protein present in human, rat, and rabbit skeletal muscle. This protein, which is missing in the skeletal muscle of two patients with Duchenne muscular dystrophy, has a molecular mass of {approx}320-420 kDa and is clearly different from the putative Duchenne muscular dystrophy-related protein nebulin. The data suggest that this 320-420-kDa protein is produced by the Duchenne muscular dystrophy gene.

  13. Immunological identification of a high molecular weight protein as a condidate for the product of the Duchenne muscular dystrophy gene

    International Nuclear Information System (INIS)

    Kao, L.; Krstenansky, J.; Mendell, J.; Rammohan, K.W.; Gruenstein, E.

    1988-01-01

    An oligopeptide was synthesized based on translation of the nucleotide sequence of the putative exon region of clone pERT87-25 from the gene for Duchenne muscular dystrophy. Immunization of rabbits with this oligopeptide induced the formation of antibodies directed against a protein present in human, rat, and rabbit skeletal muscle. This protein, which is missing in the skeletal muscle of two patients with Duchenne muscular dystrophy, has a molecular mass of ∼320-420 kDa and is clearly different from the putative Duchenne muscular dystrophy-related protein nebulin. The data suggest that this 320-420-kDa protein is produced by the Duchenne muscular dystrophy gene

  14. Why short stature is beneficial in Duchenne muscular dystrophy.

    Science.gov (United States)

    Bodor, Marko; McDonald, Craig M

    2013-09-01

    Duchenne muscular dystrophy (DMD) is caused by a genetic defect resulting in absent dystrophin, yet children are able to walk when small and young but lose this ability as they grow. The mdx mouse has absent dystrophin yet does not exhibit significant disability. Allometric modeling of linearly increasing load per muscle fiber and stress on the sarcolemma with growth and exponential decline associated with loss of muscle fibers correlated with case studies and animal models of DMD. Smaller species or breeds are predictably less affected than large as follows: mdx mice muscular dystrophy (GRMD) dogs < large GRMD dogs < humans. Case reports of combined growth hormone and dystrophin deficiency show a relatively benign course of disease. Future therapeutic trials in DMD might include specific growth inhibitors in combination with standard of care treatments to delay the clinical onset and reduce the severity of disease and disability. Copyright © 2013 Wiley Periodicals, Inc.

  15. Mechanisms and assessment of statin-related muscular adverse effects

    Science.gov (United States)

    Moßhammer, Dirk; Schaeffeler, Elke; Schwab, Matthias; Mörike, Klaus

    2014-01-01

    Statin-associated muscular adverse effects cover a wide range of symptoms, including asymptomatic increase of creatine kinase serum activity and life-threatening rhabdomyolysis. Different underlying pathomechanisms have been proposed. However, a unifying concept of the pathogenesis of statin-related muscular adverse effects has not emerged so far. In this review, we attempt to categorize these mechanisms along three levels. Firstly, among pharmacokinetic factors, it has been shown for some statins that inhibition of cytochrome P450-mediated hepatic biotransformation and hepatic uptake by transporter proteins contribute to an increase of systemic statin concentrations. Secondly, at the myocyte membrane level, cell membrane uptake transporters affect intracellular statin concentrations. Thirdly, at the intracellular level, inhibition of the 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase results in decreased intracellular concentrations of downstream metabolites (e.g. selenoproteins, ubiquinone, cholesterol) and alteration of gene expression (e.g. ryanodine receptor 3, glycine amidinotransferase). We also review current recommendations for prescribers. PMID:25069381

  16. Challenges to oligonucleotides-based therapeutics for Duchenne muscular dystrophy

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    Goyenvalle Aurélie

    2011-02-01

    Full Text Available Abstract Antisense oligonucleotides are short nucleic acids designed to bind to specific messenger RNAs in order to modulate splicing patterns or inhibit protein translation. As such, they represent promising therapeutic tools for many disorders and have been actively developed for more than 20 years as a form of molecular medicine. Although significant progress has been made in developing these agents as drugs, they are yet not recognized as effective therapeutics and several hurdles remain to be overcome. Within the last few years, however, the prospect of successful oligonucleotides-based therapies has moved a step closer, in particular for Duchenne muscular dystrophy. Clinical trials have recently been conducted for this myopathy, where exon skipping is being used to achieve therapeutic outcomes. In this review, the recent developments and clinical trials using antisense oligonucleotides for Duchenne muscular dystrophy are discussed, with emphasis on the challenges ahead for this type of therapy, especially with regards to delivery and regulatory issues.

  17. Clinical-Diagnostic Features of Duchenne Muscular Dystrophy in Children

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    Umida T. Omonova

    2013-12-01

    Full Text Available Duchenne Muscular Dystrophy (DMD is a severe, progressive disease that affects about 1 out of every 5,000 male infants; this is the most destructive of all muscular dystrophies, which worsens rapidly. In this study, we performed a clinical analysis of 37 children with DMD. They ranged in age from 3 to 15 years, mean age being 7.8±0.48 years. The mean age at onset was 4.3±0.36 years and ranged from birth to 8 years. The biochemical examination included the determination of the serum levels of the following enzymes, AST, ALT, CPK-MM, and LDH. A genealogical analysis was conducted among 240 first-degree relatives of children with DMD. Electroneuromyography examination included registration of the biopotentials of the hand and foot muscles, measurement of the muscle response (M-wave and the late-evoked responses. The clinical-diagnostic features of DMD in children were characterized.

  18. Using hegemonic masculinity to explain gay male attraction to muscular and athletic men.

    Science.gov (United States)

    Lanzieri, Nicholas; Hildebrandt, Tom

    2011-01-01

    This article reviews relevant research on male homosexual attraction. Utilizing masculinity as its theoretical frame, the authors use childhood experiences with both fathers and peers, the gay community's inculcation of heteronormative ideologies, and the gay media's adherence to masculine prototypes, to provide causal explanations for the appeal of muscular, lean, and athletic physiques. While the authors acknowledge that not all individuals within the gay community look toward muscularity and athleticism as the primary components of attractiveness, it nonetheless remains important to examine the theoretical perspectives that may explain the appeal of this specific aesthetic.

  19. The potential of sarcospan in adhesion complex replacement therapeutics for the treatment of muscular dystrophy.

    Science.gov (United States)

    Marshall, Jamie L; Kwok, Yukwah; McMorran, Brian J; Baum, Linda G; Crosbie-Watson, Rachelle H

    2013-09-01

    Three adhesion complexes span the sarcolemma and facilitate critical connections between the extracellular matrix and the actin cytoskeleton: the dystrophin- and utrophin-glycoprotein complexes and α7β1 integrin. Loss of individual protein components results in a loss of the entire protein complex and muscular dystrophy. Muscular dystrophy is a progressive, lethal wasting disease characterized by repetitive cycles of myofiber degeneration and regeneration. Protein-replacement therapy offers a promising approach for the treatment of muscular dystrophy. Recently, we demonstrated that sarcospan facilitates protein-protein interactions amongst the adhesion complexes and is an important potential therapeutic target. Here, we review current protein-replacement strategies, discuss the potential benefits of sarcospan expression, and identify important experiments that must be addressed for sarcospan to move to the clinic. © 2013 FEBS.

  20. TNF-α-Induced microRNAs Control Dystrophin Expression in Becker Muscular Dystrophy.

    Science.gov (United States)

    Fiorillo, Alyson A; Heier, Christopher R; Novak, James S; Tully, Christopher B; Brown, Kristy J; Uaesoontrachoon, Kitipong; Vila, Maria C; Ngheim, Peter P; Bello, Luca; Kornegay, Joe N; Angelini, Corrado; Partridge, Terence A; Nagaraju, Kanneboyina; Hoffman, Eric P

    2015-09-08

    The amount and distribution of dystrophin protein in myofibers and muscle is highly variable in Becker muscular dystrophy and in exon-skipping trials for Duchenne muscular dystrophy. Here, we investigate a molecular basis for this variability. In muscle from Becker patients sharing the same exon 45-47 in-frame deletion, dystrophin levels negatively correlate with microRNAs predicted to target dystrophin. Seven microRNAs inhibit dystrophin expression in vitro, and three are validated in vivo (miR-146b/miR-374a/miR-31). microRNAs are expressed in dystrophic myofibers and increase with age and disease severity. In exon-skipping-treated mdx mice, microRNAs are significantly higher in muscles with low dystrophin rescue. TNF-α increases microRNA levels in vitro whereas NFκB inhibition blocks this in vitro and in vivo. Collectively, these data show that microRNAs contribute to variable dystrophin levels in muscular dystrophy. Our findings suggest a model where chronic inflammation in distinct microenvironments induces pathological microRNAs, initiating a self-sustaining feedback loop that exacerbates disease progression. Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.