CURSO ORTOPEDIA PEDIATRICA - .D) ESCLERODERMA H. Unverricht ... Enf. Intestino delgado Poco común

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  • CURSO ORTOPEDIA PEDIATRICA

    PROFESORES: Dr. Jos Fernando de la Garza Salazar

    Dr. Aurelio G. Martnez Lozano

    Dr. Jos Alberto Moreno Gonzlez

    ASESORA: Dra. Nadina Eugenia Rubio Prez

    PONENTE: Dr. Jorge Luis de la Vega Barraza

    Residente 3er ao Ortopedia24 de julio de 2009

  • A) DERMATOMIOSITIS

    B) POLIMIOSITIS

    C) LUPUS ERITEMATOSO SISTMICO

    D) ESCLERODERMA

  • H. Unverricht 1891

    0.5 por 100,000

    80% de miopatas en

    nios

    2-15 aos

    Mujer 3:1 Hombre

  • ETIOLOGIA:

    Microangiopata

    autoinmune

    Susceptibilidad

    gentica

    Agente viral ??

    Harati, Y, Niakan, E, Bergman, EW. Childhooddermatomyositis in monozygotic twins. Neurology 1986;36:721.Leonhardt, T. Familial occurrence of collagen diseases. II.Progressive systemic sclerosisand dermatomyositis. Acta

    Med Scand 1961; 169:735.

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  • MANIFESTACIONES

    CLINICAS:

    Debilidad muscular

    Sntomas

    constitutivos

    Manifestaciones

    drmicas

    Mialgias/Artralgias

    Disfona/Disfagia

    Calcinosis

    Ramanan, AV, Feldman, BM. Clinical features and outcomes of

    juvenile dermatomyositis and other childhood onset myositis

    syndromes. Rheum Dis Clin North Am 2002; 28:833.

  • CRITERIO DEFINICINDebilidad muscular Proximal de la cintura escapular o plvica

    Enzimas

    sarcoplsmicas

    elevadas

    CPK, Aldolasa, Transaminasas elevadas

    Cambios

    electromiogrficos

    Pequeas unidades polifsicas motoras, fibrilacin y

    descargas repetitivas

    Biopsia muscular Infiltrado perivascular alrededor de fascculos, fibras

    necrticas y atrofia. rgano blanco: vasos

    sanguneos.

    Cambios drmicos Ppulas de Gottron o Heliotropo

    Bohan A, Peter JB: Polymyositis and dermatomyositis (first of two

    parts). N Engl J Med 1975; 292:344.

  • DIAGNSTICO:

    EXAMEN FSICO:

    Rash

    Ppulas de Gottron

    (91%)

    Heliotropo (83%)

    Signo de Mascada

    Signo en V

    Manos de mecnico

    Poiquilodermia vascular atrfica

  • DIAGNSTICO:

    EXAMEN FSICO:

    Fuerza muscular

    Signo de Gower

  • DIAGNSTICO:

    EXAMEN FSICO:

    Calcinosis

    Anasarca

    lceras drmicas

  • FASES CLINICAS DE LA DERMATOMIOSITIS

    1. Periodo prodrmico con sntomas

    inespecficos (semanas a meses)

    2. Debilidad muscular progresiva y rash (das a

    semanas)

    3. Debilidad persistente, rash y miositis activa

    (ms de 2 aos)

    4. Recuperacin con o sin atrofia muscular

    residual, contracturas y calcinosis

    Spencer, CH, Hanson, V, Singsen, BH, et al. Course of treated

    juvenile dermatomyositis. J Pediatr 105: 399-408, 1984.

  • TRATAMIENTO:

    Reposo, calor hmedo,

    movilidad

    Protector solar

    Prednisona 60 mg/da

    Metotrexate 10 mg/sem

    Azatioprina 2 mg/kg/da

    Ciclosporina 3mg/kg/da

    Hidroxicloroquina

    200-400mg/da

  • PRONOSTICO:

    Mortalidad menor a 15%

    Sobrevida a 5 aos del 95%

    50-75% remisin

  • E. Wagner en 188