DISTROFIA MUSCULAR DE DUCHENNE: UMA REVISAO DO TRATAMENTO COM CORTICOSTEROIDES





DISTROFIA MUSCULAR DE DUCHENNE: UMA REVISAO DO TRATAMENTO COM CORTICOSTEROIDES

(especial para SIIC © Derechos reservados)
feder9.jpg Autor:
David Feder
Columnista Experto de SIIC

Institución:
Faculdade de Medicina do ABC


Artículos publicados por David Feder
Coautores
Luiza Panosso Macedo* Renata Schewed Razaboni** Helena Wohlers Sabo** Karina Perez Sacardo** 
Aluna do 3º ano do curso de medicina, Faculdade de Medicina do ABC, São Paulo, Brasil*
aluna do 3º ano do curso de medicina, Faculdade de Medicina do ABC, São Paulo, Brasil**
Recepción del artículo
14 de Abril, 2009
Aprobación
27 de Abril, 2009
Primera edición
19 de Octubre, 2009
Segunda edición, ampliada y corregida
7 de Junio, 2021

Resumen
La Distrofia muscular de Duchenne (DMD es una afección hereditaria recesiva ligada al gen X, que afecta a 1 de cada 3.500 hombres y es causada por mutaciones en el gen que codifica la distrofina. La gran mayoría de los pacientes con DMD carecen de la proteína distrofina. Hasta contar con la terapia molecular, los corticosteroides aumentan temporalmente la función muscular. Varios estudios demostraron que la prednisona (0,75 mg / kg) y el deflazacort (0,9 mg / kg) aumentaron la masa muscular y retrasaron el avance de la DMD; el uso de esteroides retrasó la pérdida de la deambulación independiente, disminuyó la velocidad de la degeneración muscular y mejoró la función cardíaca y respiratoria. Además de sus efectos positivos sobre la preservación de la función muscular, la prednisona y el deflazacort se asocian también a efectos secundarios importantes. Deflazacort tiene menos efectos secundarios, pero aumenta el riesgo de catarata. Los efectos beneficiosos y los efectos secundarios de los corticosteroides deben ser monitorizados en forma cuidadosa.

Palabras clave
distrofia muscular de Duchenne, cromossomo X, corticostróides, distrofia muscular de Duchenne, corticosteroides, prednisona, deflazacort


Artículo completo

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Extensión:  +/-5.51 páginas impresas en papel A4
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Abstract
Duchenne muscular dystrophy (DMD) is an inherited X-linked recessive form of muscular dystrophy, which affects 1 in 3.500 men, and is caused by a mutation in the gen coding for the protein dystrophin. Most DMD patients show absence of dystrophin. Until a molecular therapy is available for this condition, corticosteroids temporary increase muscular function. Several studies have demonstrated that both prednisone (0.75 mg/kg) and deflazacort (0.9 mg/kg) increase muscular mass and delay DMD progression. The use of corticosteroids delay the loss of independent ambulation, the rate of muscular degeneration, and it also improves cardiac and respiratory function. Aside from their positive effects on motor function preservation, prednisone and deflazacort are associated with significant side effects. Deflazacort has fewer side effects, but the risk of developing cataracts is higher. The beneficial and side effects of corticosteroids must be carefully monitored.

Key words
Duchenne muscular dystrophy, corticosteroids, prednisone, deflazacort


Clasificación en siicsalud
Artículos originales > Expertos de Iberoamérica >
página   www.siicsalud.com/des/expertocompleto.php/

Especialidades
Principal: Neurología, Pediatría
Relacionadas: Atención Primaria, Farmacología, Fisiatría, Medicina Familiar, Medicina Farmacéutica, Medicina Interna



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Enviar correspondencia a:
David Feder, Faculdade de Medicina do ABC, Rua Thomas Carvalhal, 760 apto 11, São Paulo, Brasil
Bibliografía del artículo

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