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, Garcia-Morato, MB, Gil-Herrera, J, Gudiol, C, Len-Abad, O, Lopez-Medrano, F, Moreno-Perez, D, Munoz, P, Olbrich, P, Sanchez-Ramon, S, Soler-Palacin, P, Cros, CA, Arostegui, JI, Serra, IB, Carbone, J, Fortun, J, Gonzalez-Granado, LI, Lopez-Granados, E, Lucena, JM, Parody, R, Ramakers, J, Regueiro, JR, Riviere, JG, Roca-Oporto, C, Pena, RR, Santos-Perez, JL, Rodriguez-Gallego, C, Neth, O
Publicada:
1 nov 2020
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:
Univ Seville, Clin Unit Infect Dis, Univ Hosp Virgen del Rocio, CSIC,Inst Biomed, Seville, Spain
Univ Seville, Dept Med, Seville, Spain
Goycochea-Valdivia, W:
Univ Hosp Virgen del Rocio, Inst Biomed, Paediat Infect Dis Rheumatol & Immunol Unit, Seville, Spain
Mendez-Echevarria, A:
Hosp Univ La Paz, Serv Pediat & Enfermedades Infecciosas, Madrid, Spain
Allende, LM:
Univ Complutense Madrid, Hosp Univ Octubre 12, Serv Inmunol, Inst Invest I 12, Madrid, Spain
Alsina, L:
Univ Barcelona, Pediat Allergy & Clin Immunol Dept, Clin Immunol & Primary Immunodeficiencies Unit,Ho, Hosp St Joan de Deu,Inst Recerca St Joan de Deu,C, Barcelona, Spain
Garcia-Morato, MB:
Hosp Univ La Paz, Inst Invest Biomed Hosp La Paz IdiPAZ, Ctr Invest Red Enfermedades Raras CIBERER, Serv Inmunol, Madrid, Spain
Gil-Herrera, J:
Univ Complutense, Hosp Gen Univ, Dept Immunol, Madrid, Spain
Univ Complutense, Hlth Res Inst Gregorio Maranon, Sch Med, Madrid, Spain
Gudiol, C:
Hosp Univ Bellvitge, Infect Dis Dept, Barcelona, Spain
Hosp Duran & Reynals, Inst Catala Oncol ICO, IDIBELL, Barcelona, Spain
Len-Abad, O:
Hosp Univ Vall dHebron, Infect Dis Unit, Barcelona, Spain
Lopez-Medrano, F:
Univ Complutense Madrid, Infect Dis Univ Unit, Hosp Octubre 12, Inst Invest Biomed I 12,Sch Med, Madrid, Spain
Moreno-Perez, D:
Univ Malaga, Hosp Reg Univ Malaga, Pediat Infectol & Immunodeficiencies Unit, Dept Pediat,IBIMA,RECLIP, Malaga, Spain
Munoz, P:
Univ Complutense Madrid, CIBER Enfermedades Resp CIBERES CB06 06 0058, Serv Microbiol Clin & Enfermedades Infecciosas, Fac Med,Hosp Gen Univ Gregorio Maranon,Inst Inves, Madrid, Spain
Olbrich, P:
Univ Hosp Virgen del Rocio, Inst Biomed, Paediat Infect Dis Rheumatol & Immunol Unit, Seville, Spain
Sanchez-Ramon, S:
Hosp Clin San Carlos, Dept Immunol, IML, Madrid, Spain
Hosp Clin San Carlos, IDISSC, Madrid, Spain
Soler-Palacin, P:
Univ Autonoma Barcelona UAB, Hosp Univ Vall dHebron, Pediat Infect Dis & Immunodeficiencies Unit, Jeffrey Modell Diagnost & Res Ctr Primary Immunod, Barcelona, Spain
Cros, CA:
Univ Hosp Virgen del Rocio, Dept Rheumatol, Seville, Spain
Arostegui, JI:
Univ Barcelona, Inst Invest Biomed August Pi & Sunyer, Sch Med, Dept Immunol,Hosp Clin, Barcelona, Spain
Serra, IB:
Univ Autonoma Barcelona, Serv Pediat, Hosp Santa Creu & St Pau, Unidad Hematol Oncol & Trasplante Hematopoyet, Barcelona, Spain
Carbone, J:
Hosp Gen Univ Gregorio Maranon, Serv Inmunol, Madrid, Spain
Fortun, J:
Hosp Univ Ramon Y Cajal, Serv Enfermedades Infecciosas, Madrid, Spain
Gonzalez-Granado, LI:
Univ Complutense Madrid, Hosp Octubre 12, Primary Immunodeficiencies Unit, Res Inst Hosp Octubre I 12 12,Pediat,Sch Med, Madrid, Spain
Lopez-Granados, E:
Hosp Univ La Paz, Inst Invest Biomed Hosp La Paz IdiPAZ, Ctr Invest Red Enfermedades Raras CIBERER, Serv Inmunol, Madrid, Spain
Lucena, JM:
Univ Hosp Virgen del Rocio, Immunol Unit, Seville, Spain
Parody, R:
Inst Catala Oncol H Duran & Reynals, Serv Hematol Clin, IDIBELL, Barcelona, Spain
Ramakers, J:
Son Espases Univ Hosp, Hlth Res Inst Balearic Isl IdISBa, Dept Pediat Pediat Rheumatol & Immunol, Palma De Mallorca, Spain
Regueiro, JR:
Univ Complutense Madrid, Sch Med, Dept Immunol, Madrid, Spain
Riviere, JG:
Univ Autonoma Barcelona UAB, Hosp Univ Vall dHebron, Pediat Infect Dis & Immunodeficiencies Unit, Jeffrey Modell Diagnost & Res Ctr Primary Immunod, Barcelona, Spain
Roca-Oporto, C:
Univ Seville, Clin Unit Infect Dis, Univ Hosp Virgen del Rocio, CSIC,Inst Biomed, Seville, Spain
Pena, RR:
Hosp Univ La Paz, Inst Invest Biomed Hosp La Paz IdiPAZ, Ctr Invest Red Enfermedades Raras CIBERER, Serv Inmunol, Madrid, Spain
Santos-Perez, JL:
Univ Hosp Virgen de las Nieves, Serv Pediat, Infect Dis & Immunodeficiencies Unit, Granada, Spain
Rodriguez-Gallego, C:
Hosp Univ Gran Canaria Dr Negrin, Dept Immunol, Las Palmas Gran Canaria, Spain
Univ Fernando Pessoa Canarias, Las Palmas Gran Canaria, Spain
Neth, O:
Univ Hosp Virgen del Rocio, Inst Biomed, Paediat Infect Dis Rheumatol & Immunol Unit, Seville, Spain
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