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Utilidad de la prueba de ejercicio cardiopulmonar en pacientes con miopatía mitocondrial
Role of cardiopulmonary exercise test in patients with mitochondrial myopathies
J. Fernández*, T. Montemayor, J. Castillo
Servicio de Neumología. Hospital Virgen del Rocío. Sevilla.
J. Bautistaa, R. Márquezb, L. Jiménezbb, J. Arenasc, Y. Camposc
a Servicio de Neurología Hospital Virgen del Rocío. Sevilla.
b Departamento de Bioquímica.Hospital Virgen del Rocío. Sevilla
c Unidad de Investigación Neuromuscular. Facultad de Medicina. Universidad Complutense de Madrid
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        "resumen" => "<span class="elsevierStyleSectionTitle">Fundamento</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Las pruebas de ejercicio cardiopulmonar han sido utilizadas en el diagn&#243;stico delas miopat&#237;as metab&#243;licas&#46; Se desconoce si existe un patr&#243;n de respuesta anormal en las miopat&#237;asmitocondriales con afectaci&#243;n leve y la utilidad de esta t&#233;cnica en el diagn&#243;stico de sospechainicial&#46;</p> <span class="elsevierStyleSectionTitle">Pacientes y m&#233;todos</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Estudio prospectivo en 26 pacientes con miopat&#237;as mitocondriales &#40;MM&#41;&#40;15 varones y 11 mujeres&#41; y un grupo control de 14 voluntarios sanos &#40;9 varones y 5 mujeres&#41;de caracter&#237;sticas antropom&#233;tricas similares&#46; Realizamos pruebas de funci&#243;n respiratoria yprueba de ejercicio en tapiz rodante con determinaciones venosas seriadas de lactato&#44; piruvato&#44;cuerpos cet&#243;nicos&#44; &#225;cidos grasos libres y creatincinasa&#46;</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Los pacientes con miopat&#237;as mitocondriales presentaban limitaci&#243;n al ejercicio&#44;con valores menores de potencia m&#225;xima &#40;MM &#61; 143 &#91;47&#93; vatios&#44; C &#61; 187 &#91;40&#93; vatios&#44;p &#61; 0&#44;006&#41;&#44; consumo de ox&#237;geno m&#225;ximo &#40;MM &#61; 27 &#91;8&#93; ml&#47;kg&#47;min&#44; C &#61; 40 &#91;7&#93; ml&#47;kg&#47;min&#44; p &#61;0&#44;001&#41; y pulso de ox&#237;geno &#40;MM &#61; 11 &#91;3&#93; ml&#47;latido&#44; C &#61; 14 &#91;3&#93; ml&#47;latido&#44; p &#61; 0&#44;006&#41;&#46; La frecuenciacard&#237;aca en relaci&#243;n con el consumo de ox&#237;geno fue mayor y el umbral anaerobio fuem&#225;s precoz en el grupo MM &#40;MM &#61; 48&#37; &#91;14&#93;&#44; C &#61; 62&#37; &#91;12&#93;&#44; p &#61; 0&#44;01&#41;&#46; Los pacientes conpredominio de l&#237;pidos en la biopsia muscular &#40;n &#61; 15&#41; presentaban un consumo de ox&#237;geno m&#225;ximom&#225;s bajo&#46; Las curvas de cuerpos cet&#243;nicos&#44; &#225;cidos grasos libres y creatincinasa fueron similaresen ambos grupos&#46; La prueba de esfuerzo fue anormal en 17 de 26 pacientes&#46; La sensibilidadde la prueba para el consumo de ox&#237;geno m&#225;ximo fue de 0&#44;65&#46;</p> <span class="elsevierStyleSectionTitle">Conclusiones</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">La mayor&#237;a de los pacientes con miopat&#237;a mitocondrial leve presentan una pruebade ejercicio cardiopulmonar anormal&#44; siendo por tanto una prueba &#250;til en el estudio inicial dedetecci&#243;n de estos pacientes&#46;</p>"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Background</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Exercise tests has been used in the diagnosis of metabolic myopathies&#46; If there isan anormal response pattern in mild mitochondrial myopathy &#40;MM&#41; and her role in the initialsuspect diagnostic is unknown&#46;</p> <span class="elsevierStyleSectionTitle">Subjects and methods</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Prospective study of 26 patients with mitochondrial myopathy &#40;15 men&#44;11 women&#41; and a control group of 14 sedentary volunteers &#40;9 men&#44; 5 woman&#41; with similar antropometriccharacteristics&#46; We have maked pulmonary function tests and treadmill exercisewith serial venous sampling of blood lactate&#44; piruvate&#44; ketone bodies&#44; free fatty acids and creatinkinase&#46;</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Patients with MM showed exercise limitation with lower maximal power &#40;MM &#61; 143&#91;47&#93; vatts&#44; C &#61; 187 &#91;40&#93; vatts&#44; p &#61; 0&#46;006&#41;&#44; maximal oxygen uptake &#40;MM &#61; 27 &#91;8&#93; ml&#47;min&#47;kg&#44; C&#61; 40 &#91;7&#93; ml&#47;min&#47;kg&#44; p &#61; 0&#46;001&#41; and maximal oxygen pulse &#40;MM &#61; 11 &#91;3&#93; ml&#47;beat&#44; C &#61; 14 &#91;3&#93;ml&#47;beat&#44; p &#61; 0&#46;006&#41;&#46; For the same oxygen uptake the heart rate was higher and the anaerobicthreshold was earlier in MM patients &#40;MM &#61; 48&#37; &#91;14&#93;&#44; C &#61; 62&#37; &#91;12&#93;&#44; p &#61; 0&#46;01&#41;&#46; We found aconsiderable slower maximal oxygen uptake in patients with lipid increase in muscle biopsy &#40;n&#61; 15&#41;&#46; Acetoacetate curves&#44; &#223;-hydroxybutirate&#44; free fatty acids and creatinkinase were similar inboth groups&#46; The exercise test was pathologic in 17&#47;26 of the patients&#46; The sensitivity of theexercise test for maximal oxygen uptake was 0&#46;65&#46;</p> <span class="elsevierStyleSectionTitle">Conclusions</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">The cardiopulmonary exercise test is a uselful test in the suspect initial diagnostic ofpatients with mild mitochondrial myopathy because the majority of patients show a pathologic test&#46;</p>"
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ISSN: 00257753
Idioma original: Español
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