Mucopolysaccharidosis type I (MPS I). Multidiciplinary aspects and clinical guidelines

  • Imelda Martínez Núñez Facultad de Ciencias Médicas, Universidad Nacional de Asunción, San Lorenzo
  • Zoilo Morel Servicio de Reumatología, Hospital Central, Instituto de Previsión Social, Asunción, Paraguay. Cátedra de Pediatría, Hospital de Clínicas, Asunción
  • Gina Montiel Servicio de Clínica Médica, Hospital Nacional de Itaugua
  • Indianara Vermuth Servicio de Pediatría, Hospital Regional de Ciudad del Este
  • Amalio Benítez Departamento de Cardiología Pediátrica, Hospital de Clínicas, Facultad de Ciencias Médicas, Universidad Nacional de Asunción, Asunción
  • Soledad Álvarez Departamento de Cardiología, Hospital General Pediátrico “Niños de Acosta Ñú”, Ministerio de Salud Pública y Bienestar Social, San Lorenzo
  • Graciela González Servicio de Cardiología, Hospital Central, Instituto de Previsión Social, Asunción
  • Alicia Alcaraz Servicio de Neumología Pediátrica, Hospital Central, Instituto de Previsión Social, Asunción
  • Sonia Sánchez Cátedra de Neumología, Hospital de Clínicas, Facultad de Ciencias Médicas, Universidad Nacional de Asunción
  • Luis Montaner Cátedra de Neumología, Hospital de Clínicas, Facultad de Ciencias Médicas, Universidad Nacional de Asunción
  • Federico Rivarola Servicio de Otorrinolaringología, Hospital Central, Instituto de Previsión Social, Asunción
  • Franck Smith Servicio de Otorrinolaringología, Hospital Central, Instituto de Previsión Social, Asunción
  • Marco Casartelli Departamento de Neurología, Hospital General Pediátrico “Niños de Acosta Ñú”, Ministerio de Salud Pública y Bienestar Social
  • Patricia Arredondo Departamento de Neurología, Hospital General Pediátrico “Niños de Acosta Ñú”, Ministerio de Salud Pública y Bienestar Social
  • Claudia Insfrán Servicio de Clínica Médica, Hospital Central, Instituto de Previsión Social, Asunción
  • Ángeles Martínez Servicio de Clínica Médica, Hospital Central, Instituto de Previsión Social, Asunción
  • Pedro Babak Servicio de Reumatología, Hospital Central, Instituto de Previsión Social, Asunción
  • Rosa Ayala Cátedra de Oftalmología, Hospital de Clínicas, Facultad de Ciencias Médicas, Universidad Nacional de Asunción, San Lorenzo
  • Daniel Sánchez Di Martino Cátedra de Oftalmología, Hospital de Clínicas, Facultad de Ciencias Médicas, Universidad Nacional de Asunción, San Lorenzo
  • Mabel Zacur Unidad de Gastroenterología, Cátedra de Pediatría, Hospital de Clínicas, Facultad de Ciencias Médicas, Universidad Nacional de Asunción, San Lorenzo
  • Romina Sirtori Servicio de Imágenes de Resonancia Magnética, Hospital Central, Instituto de Presión Social, Asunción
Palabras clave: Mucopolisacaridosis tipo I, terapia de reemplazo enzimático, multidisciplinario

Resumen

La existencia de una terapia de reemplazo enzimático específica para el tratamiento de la Mucopolisacaridosis tipo I ha modificado el curso natural de la enfermedad. Dada la naturaleza progresiva y el compromiso multisistémico, es muy importante el trabajo coordinado interdisciplinario, de manera a dar cobertura integral y optimizar resultados de todas las intervenciones hechas en los pacientes. El contenido de esta guía trata sobre conocimientos actuales de las Mucopolisacaridosis (MPS) en general, para luego adentrarse más específicamente en la temática propia de la MPS I, con la contribución de los estudiosos de cada área, y procedentes de diferentes Instituciones del país.Se encontrarán además recomendaciones basadas en evidencias científicas publicadas en la literatura.

Citas

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Publicado
2017-06-30
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1.
Martínez Núñez I, Morel Z, Montiel G, Vermuth I, Benítez A, Álvarez S, González G, Alcaraz A, Sánchez S, Montaner L, Rivarola F, Smith F, Casartelli M, Arredondo P, Insfrán C, Martínez Ángeles, Babak P, Ayala R, Sánchez Di Martino D, Zacur M, Sirtori R. Mucopolysaccharidosis type I (MPS I). Multidiciplinary aspects and clinical guidelines. Rev. Parag. Reumatol. [Internet]. 30 de junio de 2017 [citado 3 de julio de 2020];3(1):2-24. Disponible en: http://revista.spr.org.py/index.php/spr/article/view/49
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