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Vol. 42. Issue 11.
Pages 605-607 (November 2006)
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Vol. 42. Issue 11.
Pages 605-607 (November 2006)
Case Reports
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Domiciliary Respiratory Muscle Training in Myotonic Dystrophy
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Guilherme Augusto de Freitas Fregonezi1,
Corresponding author
gfregonezi@msn.com

Correspondence: Dr. G.A. de Freitas Fregonezi Departamento de Neumología. Hospital de la santa Creu i saint Pau Antoni Maria Claret, 167. 08025 Barcelona. España
, Vanessa Regiane Resqueti, Pere Casan
Departamento de Neumología, Hospital de la Santa Creu i Sant Pau, Facultad de Medicina, Universitat Autònoma de Barcelona, Barcelona, Spain
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A 42-year-old man diagnosed with myotonic dystrophy experienced loss of respiratory muscle strength over a period of 6 months. We report the application of a domiciliary training program targeting both inspiratory and expiratory muscles. Maximal inspiratory and expiratory pressures, forced vital capacity, and forced midexpiratory flow rate were measured 6 months before start of training, just before commencement of the program, and immediately after 12 weeks of training. Adherence to the program was satisfactory. inspiratory muscle training was efficacious in increasing respiratory muscle strength. Expiratory muscle training, which made use of the Threshold PEP bronchial hygiene device incorporating an adapted flutter valve, was not efficacious in increasing maximal expiratory pressure or halting its loss. However, decreased obstruction of medium-caliber airways was observed with use of the device.

Key words:
Myotonic dystrophy
Respiratory muscle traning
Maximal respiratory pressures

Se describe el caso de un varón de 42 años de edad con diagnóstico de distrofia muscular miotónica e historia de disminución de la fuerza muscular respiratoria en los últimos 6 meses. Relatamos la aplicación de un programa de entrenamiento muscular respiratorio (inspiratorio e espira torio) domiciliario. En los 6 meses previos al entrenamiento, justo antes del comienzo del programa e inmediatamente después de 12 semanas de su inicio se estudiaron la presión inspiratoria máxima, la presión espiratoria máxima, la Capacidad vital forzada y el flujo mesoespiratorio forzado.

El cumplimento del programa fue satisfactorio. El entrenamiento muscular inspiratorio se mostró efectivo para aumentar la fuerza muscular respiratoria. El entrenamiento muscular espiratorio utilizando la válvula adaptada de higiene bronquial Threshold PEP®, adaptada para el entrenamiento, no fue eficiente para aumentar la presión espiratoria máxima y/o frenar su disminución. Sin embargo, con la utilización de la válvula Threshold PEP® se observó la disminución de la obstrucción de las vías aéreas de medio calibre.

Palabras claves:
Distrofia muscular miotónica
Entrenamiento muscular respiratorio
Presiones respiratorias máximas
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GA de Freitas Fregonezi is a doctoral fellow of CNPq-Brasil (process 2000-05/01-4).

Copyright © 2006. Sociedad Española de Neumología y Cirugía Torácica (SEPAR)
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