Evidence map›Paper›PMID 37074537›Full record

ArticleJournal of clinical immunology2023

Inborn Error of STAT2-Dependent IFN-I Immunity in a Patient Presented with Hemophagocytic Lymphohistiocytosis and Multisystem Inflammatory Syndrome in Children.

Marta López-Nevado, Julián Sevilla, Patricia Almendro-Vázquez, Francisco J Gil-Etayo, Sara Garcinuño, Antonio Serrano-Hernández, Estela Paz-Artal, Luis I González-Granado, Luis M Allende

Open access · bronzeAbstract read
In one paragraph

Article in Journal of clinical immunology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
2.9field-weighted citation impact, top 9% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

7 citing papers in PubMed, 10 citations in OpenAlex.

  1. Article
  2. Article
  3. Article
  4. Review
  5. Article
  6. HLH Syndrome in a Community Hospital: The Challenge of an Early Diagnosis.Pediatric health, medicine and therapeutics · 2024
    Article
  7. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors at 3 institutions in 1 country.

Marta López-NevadoImmunology Department, University Hospital, 12 de Octubre, Av de Córdoba S/N 28041, Madrid, Spain. martalopezne@gmail.com.ORCID 0000-0002-1236-9281
Julián SevillaHematology and Hemotherapy Unit, University Children's Hospital Niño Jesus, Madrid, Spain.
Patricia Almendro-VázquezImmunology Department, University Hospital, 12 de Octubre, Av de Córdoba S/N 28041, Madrid, Spain.
Francisco J Gil-EtayoImmunology Department, University Hospital, 12 de Octubre, Av de Córdoba S/N 28041, Madrid, Spain.
Sara GarcinuñoImmunology Department, University Hospital, 12 de Octubre, Av de Córdoba S/N 28041, Madrid, Spain.
Antonio Serrano-HernándezImmunology Department, University Hospital, 12 de Octubre, Av de Córdoba S/N 28041, Madrid, Spain.
Estela Paz-ArtalImmunology Department, University Hospital, 12 de Octubre, Av de Córdoba S/N 28041, Madrid, Spain.
Luis I González-GranadoResearch Institute Hospital, 12 Octubre (imas12), Madrid, Spain.
Luis M AllendeImmunology Department, University Hospital, 12 de Octubre, Av de Córdoba S/N 28041, Madrid, Spain. luis.allende@salud.madrid.org.
Research Institute Hospital 12 de Octubre · ESUniversidad Complutense de Madrid · ESHospital Infantil Universitario Niño Jesús · ES

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Human inborn errors of immunity (IEI) affecting the type I interferon (IFN-I) induction pathway have been associated with predisposition to severe viral infections. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening systemic hyperinflammatory syndrome that has been increasingly associated with inborn errors of IFN-I-mediated innate immunity. Here is reported a novel case of complete deficiency of STAT2 in a 3-year-old child that presented with typical features of HLH after mumps, measles, and rubella vaccination at the age of 12 months. Due to the life-threatening risk of viral infection, she received SARS-CoV-2 mRNA vaccination. Unfortunately, she developed multisystem inflammatory syndrome in children (MIS-C) after SARS-CoV-2 infection, 4 months after the last dose. Functional studies showed an impaired IFN-I-induced response and a defective IFNα expression at later stages of STAT2 pathway induction. These results suggest a possible more complex mechanism for hyperinflammatory reactions in this type of patients involving a possible defect in the IFN-I production. Understanding the cellular and molecular links between IFN-I-induced signaling and hyperinflammatory syndromes can be critical for the diagnosis and tailored management of these patients with predisposition to severe viral infection.

Indexed as

COVID-19Interferon Type ILymphohistiocytosis, HemophagocyticAntibodiesChild, PreschoolFemaleHumansInfantSARS-CoV-2STAT2 Transcription FactorSystemic Inflammatory Response SyndromeAntibodiesInterferon Type ISTAT2 protein, humanSTAT2 Transcription FactorIFNαInnate immunityInterferonsSARS-CoV-2STAT2 deficiencyViral susceptibility

Identifiers

PMID37074537
PMCPMC10113994
OpenAlexW4366352016

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.