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Record W2601358985 · doi:10.1111/hae.13205

Severe bleeding diatheses in an elderly patient with combined type autoantibody against factor <scp>XIII</scp> A subunit; novel approach to the diagnosis and classification of anti‐factor XIII antibodies

2017· article· en· W2601358985 on OpenAlexaff
Natasha Szuber, Ëva Katona, Krisztina Pénzes, Arnaud Bonnefoy, Bálint Bécsi, Ferenc Erdődi, Georges‐Étienne Rivard, László Muszbek

Bibliographic record

VenueHaemophilia · 2017
Typearticle
Languageen
FieldMedicine
TopicBlood properties and coagulation
Canadian institutionsCentre Hospitalier Universitaire Sainte-Justine
FundersMagyar Tudományos Akadémia
KeywordsAutoantibodyFactor XIIIMedicineAntibodyImmunologyAntigenMolecular biologyFibrinBiology

Abstract

fetched live from OpenAlex

Introduction Acquired factor XIII ( FXIII ) deficiency due to autoantibody is a rare, severe bleeding diathesis. Its laboratory diagnosis and classification represents a difficult task. Aim Introduction of novel approaches into the diagnosis and characterization of anti‐ FXIII autoantibody and demonstration of their use in the diagnosis of a patient with autoimmune FXIII deficiency. Methods Factor XIII activity, FXIII antigen levels and the titre of anti‐ FXIII ‐A antibody were monitored throughout the course of the disease. FXIII activity was measured by ammonia release assay; FXIII ‐A 2 B 2 complex, total and free FXIII ‐B concentrations were determined by ELISA s. The binding constant for the interaction of the autoantibody with recombinant FXIII ‐A 2 ( rFXIII ‐A 2 ) and FXIII ‐A 2 B 2 was determined by surface plasmon resonance ( SPR ). The inhibitory capacity of IgG was expressed as the concentration exerting 50% inhibition of FXIII activation/activity ( IC 50). The truncation of FXIII ‐A by thrombin was monitored by western blotting. The inhibition of Ca 2+ ‐induced FXIII activation and active FXIII ( FXIII a) were assessed by FXIII activity assay. Results The antibody bound to rFXIII ‐A 2 and FXIII ‐A 2 B 2 with high affinity and accelerated the decay of supplemented FXIII concentrate. An IC 50 value of 170.1 μg IgG·mL −1 indicated effective FXIII neutralization. The main neutralizing effect of the autoantibody was the inhibition of FXIII a. After 2 months, due to combined therapeutic modalities, the autoantibody disappeared and FXIII activity significantly elevated. Conclusion The anti‐ FXIII ‐A autoantibody exerted a combined effect including inhibition of FXIII a and acceleration of FXIII decay in the plasma. IC 50 and binding constant determinations added important information to the characterization of the autoantibody.

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)0.000
metaresearch head score (Gemma)0.000
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 score0.040
Threshold uncertainty score0.578

Codex and Gemma teacher scores by category

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

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 head0.074
GPT teacher head0.285
Teacher spread0.211 · 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

Citations5
Published2017
Admission routes1
Has abstractyes

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