Results 21 to 30 of about 15,070 (233)

Inborn Errors of Immunity: how to diagnose them?

open access: yesJornal de Pediatria, 2021
Objectives: Inborn Errors of Immunity are characterized by infectious conditions and manifestations of immune dysregulation. The diversity of clinical phenotypes can make it difficult to direct the laboratory investigation.
Anete Sevciovic Grumach   +1 more
doaj   +6 more sources

Rheumatological manifestations in inborn errors of immunity [PDF]

open access: bronzePediatric Research, 2019
Rare monogenetic diseases serve as natural models to dissect the molecular pathophysiology of the complex disease traits. Rheumatologic disorders by their nature are considered complex diseases with partially genetic origin, as illustrated by their heterogeneous genetic background and variable phenotypic presentation.
Sevgi Köstel Bal   +3 more
openalex   +4 more sources

Future of Therapy for Inborn Errors of Immunity [PDF]

open access: hybridClinical Reviews in Allergy & Immunology, 2022
Over the past 20 years, the rapid evolution in the diagnosis and treatment of primary immunodeficiencies (PI) and the recognition of immune dysregulation as a feature in some have prompted the use of "inborn errors of immunity" (IEI) as a more encompassing term used to describe these disorders [1, 2] .
Elena Pérez
openalex   +3 more sources

Editorial: Immune dysregulation in inborn errors of immunity

open access: yesFrontiers in Pediatrics, 2023
Eveline Y. Wu   +3 more
doaj   +3 more sources

Novel Discoveries in Immune Dysregulation in Inborn Errors of Immunity [PDF]

open access: goldFrontiers in Immunology, 2021
With the expansion of our knowledge on inborn errors of immunity (IEI), it gradually becomes clear that immune dysregulation plays an important part. In some cases, autoimmunity, hyperinflammation and lymphoproliferation are far more serious than infections. Thus, immune dysregulation has become significant in disease monitoring and treatment.
Anwen Ren   +9 more
openalex   +6 more sources

The Ever-Increasing Array of Novel Inborn Errors of Immunity : an Interim Update by the IUIS Committee [PDF]

open access: yes, 2021
The most recent updated classification of inborn errors of immunity/primary immunodeficiencies, compiled by the International Union of Immunological Societies Expert Committee, was published in January 2020.
Al-Herz, Waleed   +17 more
core   +2 more sources

An ACE2 Triple Decoy that neutralizes SARS-CoV-2 shows enhanced affinity for virus variants

open access: yesScientific Reports, 2021
The SARS-CoV-2 variants replacing the first wave strain pose an increased threat by their potential ability to escape pre-existing humoral protection. An angiotensin converting enzyme 2 (ACE2) decoy that competes with endogenous ACE2 for binding of the ...
Shiho Tanaka   +15 more
doaj   +1 more source

Failure of immunological competence: when to suspect?

open access: yesJornal de Pediatria, 2021
Objectives: To draw physicians’ attention to the different warning signs of diseases of inborn errors of immunity. Data sources: A non-systematic review of the literature was carried out in the PubMed, LILACS, and SciELO databases, in addition to ...
Fernanda Pinto-Mariz
doaj   +1 more source

Monogenic Adult-Onset Inborn Errors of Immunity

open access: yesFrontiers in Immunology, 2021
Inborn errors of immunity (IEI) are a heterogenous group of disorders driven by genetic defects that functionally impact the development and/or function of the innate and/or adaptive immune system.
Frederik Staels   +15 more
doaj   +1 more source

RIPK1-Associated Inborn Errors of Innate Immunity

open access: yesFrontiers in Immunology, 2021
RIPK1 (receptor-interacting serine/threonine-protein kinase 1) is a key molecule for mediating apoptosis, necroptosis, and inflammatory pathways downstream of death receptors (DRs) and pattern recognition receptors (PRRs).
Jiahui Zhang   +6 more
doaj   +1 more source

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