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00925njm 22002777a 4500 dc Cordero, Elisa author Goycochea Valdivia, Walter author Mendez Echevarría, Ana author Allende, Luis author Alsina, Laia author Bravo García Morato, María author Gil Herrera, Juana author Gudiol, Carlota author Len Abad, Óscar author López Medrano, Francisco author Moreno Pérez, David author Muñoz, Patricia author Olbrich, Peter author Sánchez Ramón, Silvia María author Soler Paracín, Pere author Aguilera Cros, Clara author Arostegui, Juan Ignacio author Badell Serra, Isabel author Carbone Campoverde, Javier Alberto author Fortún, Jesús author González Granado, Luis Ignacio author López Granados, Eduardo author Lucena, José Manuel author Parody, Rocío author Ramakers, Jan author Regueiro González-Barros, José Ramón author Rivière, Jacque G author Roca Oporto, Cristina author Rodríguez Pena, Rebeca author Santos Pérez, Juan Luis author Rodríguez Gallego, Carlos author Neth, Olaf author 2020-12 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 childre 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. � 2020 American Academy of Allergy, Asthma & Immunology; Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica [Published by Elsevier Inc./Elsevier Espana]. All rights reserved. Cordero E, Goycochea-Valdivia W, Mendez-Echevarria A, Allende LM, Alsina L, Bravo García-Morato M, et al. Executive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies. The Journal of Allergy and Clinical Immunology: In Practice 2020;8:3342–7. https://doi.org/10.1016/j.jaip.2020.05.008. 2213-2198 10.1016/j.jaip.2020.05.008 https://hdl.handle.net/20.500.14352/8576 https://doi.org/10.1016/j.jaip.2020.05.008 Executive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies