Tratamiento de reposición enzimática en la insuficiencia pancreática exocrina asociada a la fibrosis quística. Grupo de trabajo de Fibrosis Quística de la Sociedad Latinoamericana de Gastroenterología, Hepatología y Nutrición Pediátrica (LASPHGAN)

• Garcete Mañotti, Lidia ^[1] ; Cázares Méndez, Josefina Monserrat ^[4] ; Ortiz Paranza, Lourdes ^[5] ; Rodríguez González, Lorena ^[2] ; Wagener, Marta ^[3]
  1. [1] Universidad Nacional de Asunción

     Universidad Nacional de Asunción

     Paraguay

     
  2. [2] Hospital San Juan de Dios

     Hospital San Juan de Dios

     Santiago, Chile

     
  3. [3] Universidad Nacional de Rosario

     Universidad Nacional de Rosario

     Argentina

     
  4. [4] Star Médica Hospital Infantil Privado, Consulta de gastroenterología. Ciudad de México, México
  5. [5] Ministerio de Salud Pública y Bienestar Social, Hospital General Pediátrico Niños de Acosta Ñu, Unidad de fibrosis quística. Asunción, Paraguay

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• Localización: Anales de la Facultad de Ciencias Médicas, ISSN-e 2313-2841, Vol. 55, Nº. 3, 2022 (Ejemplar dedicado a: ANALES), págs. 76-85
• Idioma: español
• DOI: 10.18004/anales/2022.055.03.76
• Títulos paralelos:
  • Enzyme replacement treatment in exocrine pancreatic insufficiency associated with cystic fibrosis. Cystic fibrosis Working group of the Latin American Society of Pediatric Gastroenterology, Hepatology and Nutrition (LASPHGAN)
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  • Texto completo
• Resumen
  • español

    La fibrosis quística (FQ) es un trastorno hereditario, de las glándulas de secreción exocrina, considerada la causa más frecuente de insuficiencia pancreática exocrina (IPE) en la infancia. Esta revisión resume el panorama del tratamiento de reposición enzimática en la IPE asociada a la FQ, las formulaciones disponibles, su dosificación y modo de administración, así como las limitaciones y desafíos actuales y las posibles áreas del desarrollo futuro

  • English

    Cystic fibrosis (CF) is an inherited disorder of the exocrine secretion glands, considered the most frequent cause of exocrine pancreatic insufficiency (EPI) in childhood. This review summarizes the landscape of enzyme replacement therapy in PEI associated with CF, the available formulations, their dosage and mode of administration, as well as the current limitations and challenges and potential areas for future development

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