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Record W4403378365 · doi:10.1016/j.clinpr.2024.100393

Somatic STAT3 Gain-of-Function (GOF) syndrome underlying susceptibility to Parvovirus B19, Pseudomonas aeruginosa, and Histoplasma capsulatum infections

2024· article· en· W4403378365 on OpenAlexafffund
Cedric Julien, Stephane Bernier, Denis Cournoyer, Yizhe Sun, Anna Perez, Joy Agbonze, Isabelle Angers, Lucie Roussel, Gizelle Popradi, Donald C. Vinh

Bibliographic record

VenueClinical Infection in Practice · 2024
Typearticle
Languageen
FieldImmunology and Microbiology
TopicImmunodeficiency and Autoimmune Disorders
Canadian institutionsMcGill UniversityMcGill University Health Centre
FundersFonds de Recherche du Québec - Santé
KeywordsParvovirusHistoplasma capsulatumPseudomonas aeruginosaMicrobiologyBiologyVirologyHistoplasmosisImmunologyGeneticsVirusBacteria

Abstract

fetched live from OpenAlex

• Severe infections in healthy hosts may signal an underlying immune deficiency. • Inborn errors of immunity (IEI) are monogenic defects, often germline mutations. • IEI can also arise from autoantibodies or somatic mutations. • Somatic STAT3 gain-of-function (GOF) mutations underlie autoimmunity and cancers. • Somatic STAT3 GOF mutation can present as immunodeficiency with severe infections. Infections in overtly immunocompromised persons (e.g. those living with advanced HIV, receiving malignancy-targeting chemotherapy, or recipients of transplants), may present with severe disease. In the absence of overt immunosuppression, unexplained severe disease due to prevalent microbes may reflect an underlying “inborn error of immunity” (IEI). IEI are monogenic penetrant defects of immunity: Classically, the genetic mutation is germline, that is, derived from germ cells, vertically transmitted to offspring in Mendelian fashion (autosomal dominant, autosomal recessive, or X-linked), and present in all cells of the affected individual. IEI may also be caused by naturally-occurring autoantibodies directed to immunologic components (such as cytokines) ( Casanova et al., 2024 ) or by somatic mutations ( Aluri and Cooper, 2023 ). Somatic mutations occur when a deleterious genetic variant occurs in the post-zygotic stage of development and can affect any body cell, other than germ cells; they are well-known causes of solid and hematologic malignancies. Somatic IEI syndromes are emerging, typically associated with lymphoproliferative diseases or autoinflammatory disorders. We report the case of a young woman with unexplained severe infections in whom a somatic gain-of-function (GOF) mutation in Signal transducer and activator of transcription 3 (STAT3) was found.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.002
metaresearch head score (Gemma)0.004
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.348
Threshold uncertainty score0.982

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0020.004
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.001
Science and technology studies0.0000.000
Scholarly communication0.0000.001
Open science0.0000.000
Research integrity0.0000.001
Insufficient payload (model declined to judge)0.0000.001

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.047
GPT teacher head0.369
Teacher spread0.322 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations0
Published2024
Admission routes2
Has abstractyes

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