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Miositis osificante progresiva: ultraestructura, bioquímica e histoquímica de músculo macroscópicamente sano

Authors: Garmendia, J.; Torres, S.H.; Hernández, N.; Finol, Héctor J.; Martínez Conde, A.;

Miositis osificante progresiva: ultraestructura, bioquímica e histoquímica de músculo macroscópicamente sano

Abstract

Se estudió un caso de miositis osificante progresiva en una niña de 13 años, a la cual se le tomó una muestra de músculo gastronecmio lateral, aparentemente no afectado, en el curso de una intervención quirúrgica ortopédica. La muestra se procesó mediante métodos histológicos, histoquímicos, bioquímicos, inmunocitoquímicos y ultraestructurales. Se encontró un predominio de fibras musculares tipo I (83%) con alta capacidad oxidativa y baja capacidad glicolítica. Las fibras del tipo II eran pequeñas (área promedio 2.084 Um2 ) y mostraron otros signos de atrofia al examen ultraestructural. La densidad capilar fue relativamente alta, (573) siendo normal el índice capilar/fibra (1,76). Sin embargo, algunos capilares se mostraron engrosados y con la luz ocluida, con la tinción de amilasa-PAS, lo cual fue corroborado con la microscopía electrónica, donde se vio la membrana basal engrosada, e inclusive algunos capilares totalmente degenerados. No se encontró reacción de inmunofluorescencia con las globulinas anti-IgG ni anti-IgM en los cortes de músculo. El espacio intersticial se encontró agrandado. Se concluye que no hay evidencias de la participación de un mecanismo autoinmune en la miositis osificante progresiva, que existe un daño capilar y alteración de las fibras musculares, aún en el músculo que no manifiesta a simple vista proceso de osificación.

During an orthopedic operation a sample of the apparently normal lateral gastronecmius muscle was taken from a 13 year old female patient affected by myositis ossificans progressiva. The muscle sample was analyzed by light, electron and fluorescence microscopy, and some enzymes were assayed. Muscle fibers were classified by the adenosintriphosphatase reaction. The percentage of type I fiber was high (83%). Atrophy was found in type II fibers as shown by small mean area (2.084 Um2 ) and some ultrastructural features as infoldings of the sarcolemma. Capillary density was high (573 capillaries/mm2 ), and capillaries per fiber index was normal (1.76), as were oxidative enzymes. However many capillaries were occluded, with thick basal membrane and abnormal endothelial cells and pericytes. No immunofluorescence was found with anti IgG or anti IgM in the muscle fibers. Intersticial spaces in the cross section of the muscle were enlarged. In conclusion, no evidence of autoimmune involvement was found in myositis ossificans progressiva, but alteracions of capillaries and muscle fibers were found in a muscle apparently not affected yet by the ossification process.

Keywords

Medicina clínica, UNESCO::CIENCIAS MÉDICAS, :CIENCIAS MÉDICAS [UNESCO], Ciencias de la salud

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
0
Average
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