Executive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies
Por:
Cordero E, Goycochea-Valdivia W, Mendez-Echevarria A, Allende LM, Alsina L, Bravo García-Morato M, Gil-Herrera J, Gudiol C, Len-Abad O, López-Medrano F, Moreno-Pérez D, Muñoz P, Olbrich P, Sánchez-Ramón S, Soler-Palacín P, Aguilera Cros C, Arostegui-Gorospe JI, Badell Serra I, Carbone J, Fortún J, Gonzalez-Granado LI, López-Granados E, Lucena JM, Parody R, Ramakers J, Regueiro JR, Rivière JG, Roca-Oporto C, Rodríguez Pena R, Santos-Pérez JL, Rodríguez-Gallego C and Neth O
Publicada:
1 nov 2020
Categoría:
Immunology and allergy
Resumen:
Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and children with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available. (C) 2020 American Academy of Allergy, Asthma & Immunology; Sociedad Espanola de Enfermedades Infecciosas y Microbiologia Clinica [Published by Elsevier Inc./Elsevier Espana]. All rights reserved.
Filiaciones:
Cordero E:
Clinical Unit of Infectious Diseases University Hospital Virgen del Rocio, Institute of Biomedicine, CSIC, University of Seville, Seville, Spain
Department of Medicine, University of Seville, Seville, Spain
Goycochea-Valdivia W:
Paediatric Infectious Diseases, Rheumatology and Immunology Unit, University Hospital Virgen del Rocio, Institute of Biomedicine, Seville, Spain
Mendez-Echevarria A:
Servicio de Pediatría y Enfermedades Infecciosas, Hospital Universitario La Paz, Madrid, Spain
Allende LM:
Servicio de Inmunología, Hospital Universitario 12 de Octubre, Instituto de Investigación i+12, Universidad Complutense de Madrid, Madrid, Spain
Alsina L:
Clinical Immunology and Primary Immunodeficiencies Unit, Pediatric Allergy and Clinical Immunology Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Institut de Recerca Sant Joan de Déu, Clinical Immunology Unit Hospital Sant Joan de Déu-Hospital Clínic Barcelona, Barcelona, Spain
Bravo García-Morato M:
Servicio de Inmunología, Hospital Universitario La Paz, Instituto de Investigación Biomédica del Hospital La Paz (IdiPAZ), Centro de Investigación en Red de Enfermedades Raras (CIBERER), Madrid, Spain
Gil-Herrera J:
Department of Immunology, Hospital General Universitario and Health Research Institute Gregorio Marañón, School of Medicine, Univerisdad Complutense, Madrid, Spain
Gudiol C:
Infectious Diseases Department, Hospital Universitari de Bellvitge and Institut Català d'Oncologia (ICO), Hospital Duran i Reynals, IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain
Len-Abad O:
Infectious Diseases Unit, Hospital Universitari Vall d'Hebron, Barcelona, Spain
López-Medrano F:
Infectious Diseases University Unit, Hospital 12 de Octubre, Instituto de Investigación Biomédica i+12, School of Medicine, Universidad Complutense de Madrid, Madrid, Spain
Moreno-Pérez D:
Pediatric Infectology and Immunodeficiencies Unit, Department of Pediatrics, Hospital Regional Universitario de Málaga, IBIMA, RECLIP, University of Malaga, Málaga, Spain
Muñoz P:
Servicio de Microbiología Clínica y Enfermedades Infecciosas, Hospital General Universitario Gregorio Marañón, Instituto de Investigación Sanitaria Gregorio Marañón, CIBER Enfermedades Respiratorias-CIBERES (CB06/06/0058), Facultad de Medicina, Universidad Complutense de Madrid, Madrid, Spain
Olbrich P:
Paediatric Infectious Diseases, Rheumatology and Immunology Unit, University Hospital Virgen del Rocio, Institute of Biomedicine, Seville, Spain
Sánchez-Ramón S:
Department of Immunology, IML and IdSSC, Hospital Clínico San Carlos, Madrid, Spain
Soler-Palacín P:
Pediatric Infectious Diseases and Immunodeficiencies Unit, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona (UAB), Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Barcelona, Spain
Aguilera Cros C:
Department of Rheumatology, University Hospital Virgen del Rocío, Sevilla, Spain
Arostegui-Gorospe JI:
Department of Immunology, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, School of Medicine, Universitat de Barcelona, Barcelona, Spain
Badell Serra I:
Unidad de Hematología, Oncología y Trasplante Hematopoyético, Servicio de Pediatría, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain
Carbone J:
Servicio de Inmunología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
Fortún J:
Servicio de Enfermedades Infecciosas, Hospital Universitario Ramón y Cajal, Madrid, Spain
Gonzalez-Granado LI:
Primary Immunodeficiencies Unit, Pediatrics, Hospital 12 de Octubre, Research Institute Hospital 12 octubre (i+12), School of Medicine, Universidad Complutense de Madrid, Madrid, Spain
López-Granados E:
Servicio de Inmunología, Instituto de Investigación Biomédica del Hospital La Paz (IdiPAZ), Hospital Universitario La Paz, Centro de Investigación en Red de Enfermedades Raras (CIBERER), Madrid, Spain
Lucena JM:
Immunology Unit, University Hospital Virgen del Rocío, Sevilla, Spain
Parody R:
Servicio de Hematología Clínica, Institut Català d'Oncologia H. Duran i Reynals, IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain
Ramakers J:
Department of Pediatrics, Pediatric Rheumatology and Immunology, Son Espases University Hospital, Health Research Institute of the Balearic Islands (IdISBa), Palma de Mallorca, Spain
Regueiro JR:
Department of Immunology, School of Medicine, Universidad Complutense de Madrid, Madrid, Spain
Rivière JG:
Pediatric Infectious Diseases and Immunodeficiencies Unit, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona (UAB), Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Barcelona, Spain
Roca-Oporto C:
Clinical Unit of Infectious Diseases University Hospital Virgen del Rocio, Institute of Biomedicine, CSIC, University of Seville, Seville, Spain
Rodríguez Pena R:
Servicio de Inmunología, Instituto de Investigación Biomédica del Hospital La Paz (IdiPAZ), Hospital Universitario La Paz, Centro de Investigación en Red de Enfermedades Raras (CIBERER), Madrid, Spain
Santos-Pérez JL:
Infectious Diseases and Immunodeficiencies Unit, Service of Pediatrics, University Hospital Virgen de las Nieves, Granada, Spain
Rodríguez-Gallego C:
Department of Immunology, Hospital Universitario de Gran Canaria Dr. Negrín, Las Palmas de Gran Canaria, Spain
University Fernando Pessoa Canarias, Las Palmas de Gran Canaria, Spain
Neth O:
Paediatric Infectious Diseases, Rheumatology and Immunology Unit, University Hospital Virgen del Rocio, Institute of Biomedicine, Seville, Spain
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