FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4242706780> ?p ?o ?g. }

• W4242706780 endingPage "443" @default.
• W4242706780 startingPage "438" @default.
• W4242706780 abstract "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. Las inmunodeficiencias primarias (IDP) son unas enfermedades raras, frecuentemente infradiagnosticadas y potencialmente fatales. Las manifestaciones clínicas de las IDP pueden ser muy graves y ocasionar secuelas que empeoran la calidad de vida de los pacientes. Tradicionalmente, el tratamiento de las IDP ha sido fundamentalmente de soporte, con excepción del trasplante de progenitores hematopoyéticos y, más recientemente, la terapia génica. El descubrimiento de nuevos mecanismos patogénicos, el desarrollo de nuevas moléculas y fármacos biológicos y los avances en el conocimiento de las bases moleculares de estas enfermedades han abierto oportunidades para el tratamiento de esta afección. El objetivo de este documento es revisar el conocimiento actual y aportar recomendaciones para el diagnóstico y el tratamiento clínico de los pacientes adultos y pediátricos con IDP basado en la evidencia científica disponible y teniendo en cuenta la actual práctica y los retos futuros. Se realizó una revisión sistemática, que justifica los niveles de evidencia para cada recomendación." @default.
• W4242706780 created "2022-05-12" @default.
• W4242706780 creator A5002831959 @default.
• W4242706780 creator A5003442720 @default.
• W4242706780 creator A5004127149 @default.
• W4242706780 creator A5006027109 @default.
• W4242706780 creator A5007452395 @default.
• W4242706780 creator A5008240549 @default.
• W4242706780 creator A5009198871 @default.
• W4242706780 creator A5009832583 @default.
• W4242706780 creator A5010673451 @default.
• W4242706780 creator A5011027535 @default.
• W4242706780 creator A5020417323 @default.
• W4242706780 creator A5021918368 @default.
• W4242706780 creator A5023026686 @default.
• W4242706780 creator A5027142622 @default.
• W4242706780 creator A5035683885 @default.
• W4242706780 creator A5035897626 @default.
• W4242706780 creator A5039723546 @default.
• W4242706780 creator A5041818001 @default.
• W4242706780 creator A5046600042 @default.
• W4242706780 creator A5054374372 @default.
• W4242706780 creator A5061334675 @default.
• W4242706780 creator A5062503120 @default.
• W4242706780 creator A5066261398 @default.
• W4242706780 creator A5067139338 @default.
• W4242706780 creator A5067861619 @default.
• W4242706780 creator A5070209864 @default.
• W4242706780 creator A5071487615 @default.
• W4242706780 creator A5071542553 @default.
• W4242706780 creator A5071719503 @default.
• W4242706780 creator A5075936171 @default.
• W4242706780 creator A5077220786 @default.
• W4242706780 creator A5078199034 @default.
• W4242706780 date "2020-11-01" @default.
• W4242706780 modified "2023-10-18" @default.
• W4242706780 title "Executive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies" @default.
• W4242706780 doi "https://doi.org/10.1016/j.eimce.2020.07.004" @default.
• W4242706780 hasPublicationYear "2020" @default.
• W4242706780 type Work @default.
• W4242706780 citedByCount "0" @default.
• W4242706780 crossrefType "journal-article" @default.
• W4242706780 hasAuthorship W4242706780A5002831959 @default.
• W4242706780 hasAuthorship W4242706780A5003442720 @default.
• W4242706780 hasAuthorship W4242706780A5004127149 @default.
• W4242706780 hasAuthorship W4242706780A5006027109 @default.
• W4242706780 hasAuthorship W4242706780A5007452395 @default.
• W4242706780 hasAuthorship W4242706780A5008240549 @default.
• W4242706780 hasAuthorship W4242706780A5009198871 @default.
• W4242706780 hasAuthorship W4242706780A5009832583 @default.
• W4242706780 hasAuthorship W4242706780A5010673451 @default.
• W4242706780 hasAuthorship W4242706780A5011027535 @default.
• W4242706780 hasAuthorship W4242706780A5020417323 @default.
• W4242706780 hasAuthorship W4242706780A5021918368 @default.
• W4242706780 hasAuthorship W4242706780A5023026686 @default.
• W4242706780 hasAuthorship W4242706780A5027142622 @default.
• W4242706780 hasAuthorship W4242706780A5035683885 @default.
• W4242706780 hasAuthorship W4242706780A5035897626 @default.
• W4242706780 hasAuthorship W4242706780A5039723546 @default.
• W4242706780 hasAuthorship W4242706780A5041818001 @default.
• W4242706780 hasAuthorship W4242706780A5046600042 @default.
• W4242706780 hasAuthorship W4242706780A5054374372 @default.
• W4242706780 hasAuthorship W4242706780A5061334675 @default.
• W4242706780 hasAuthorship W4242706780A5062503120 @default.
• W4242706780 hasAuthorship W4242706780A5066261398 @default.
• W4242706780 hasAuthorship W4242706780A5067139338 @default.
• W4242706780 hasAuthorship W4242706780A5067861619 @default.
• W4242706780 hasAuthorship W4242706780A5070209864 @default.
• W4242706780 hasAuthorship W4242706780A5071487615 @default.
• W4242706780 hasAuthorship W4242706780A5071542553 @default.
• W4242706780 hasAuthorship W4242706780A5071719503 @default.
• W4242706780 hasAuthorship W4242706780A5075936171 @default.
• W4242706780 hasAuthorship W4242706780A5077220786 @default.
• W4242706780 hasAuthorship W4242706780A5078199034 @default.
• W4242706780 hasConcept C138885662 @default.
• W4242706780 hasConcept C141071460 @default.
• W4242706780 hasConcept C15708023 @default.
• W4242706780 hasConcept C177713679 @default.
• W4242706780 hasConcept C187212893 @default.
• W4242706780 hasConcept C2911091166 @default.
• W4242706780 hasConcept C2994180119 @default.
• W4242706780 hasConcept C71924100 @default.
• W4242706780 hasConceptScore W4242706780C138885662 @default.
• W4242706780 hasConceptScore W4242706780C141071460 @default.
• W4242706780 hasConceptScore W4242706780C15708023 @default.
• W4242706780 hasConceptScore W4242706780C177713679 @default.
• W4242706780 hasConceptScore W4242706780C187212893 @default.
• W4242706780 hasConceptScore W4242706780C2911091166 @default.
• W4242706780 hasConceptScore W4242706780C2994180119 @default.
• W4242706780 hasConceptScore W4242706780C71924100 @default.
• W4242706780 hasIssue "9" @default.
• W4242706780 hasLocation W42427067801 @default.
• W4242706780 hasOpenAccess W4242706780 @default.
• W4242706780 hasPrimaryLocation W42427067801 @default.
• W4242706780 hasRelatedWork W2410491650 @default.
• W4242706780 hasRelatedWork W2582335907 @default.
• W4242706780 hasRelatedWork W2590434509 @default.
• W4242706780 hasRelatedWork W3028728276 @default.

• next