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Record W4399443861 · doi:10.1002/ped4.12429

Low‐dose immune tolerance induction for severe hemophilia A inhibitor patients: Immunosuppressants are generally not necessary for inhibitor‐titer below 200 BU/mL

2024· article· en· W4399443861 on OpenAlexaff
Zhengping Li, Jie Sun, Zekun Li, Zhenping Chen, Guoqing Liu, Wanru Yao, Xiaoling Cheng, Gang Li, Yingzi Zhen, Di Ai, Yaohan Zhou, Qianqian Mao, Man‐Chiu Poon, Runhui Wu

Bibliographic record

VenuePediatric Investigation · 2024
Typearticle
Languageen
FieldMedicine
TopicHemophilia Treatment and Research
Canadian institutionsUniversity of Calgary
FundersBeijing Municipal Science and Technology CommissionNational Natural Science Foundation of China
KeywordsRegimenMedicineRituximabInternal medicineTiterPrednisoneGastroenterologyImmunologyAntibody

Abstract

fetched live from OpenAlex

ABSTRACT Importance It remained unclear that the efficacy comparison between low‐dose immune tolerance induction (LD‐ITI) incorporating immunosuppressants (IS) when severe hemophilia A (SHA) patients had inhibitor‐titer ≥200 Bethesda Units (BU)/mL (LD‐ITI‐IS 200 regimen) and LD‐ITI combining with IS when SHA patients had inhibitor‐titer ≥40 BU/mL (LD‐ITI‐IS 40 regimen). Objective To compare the efficacy of the LD‐ITI‐IS 200 regimen with that of the LD‐ITI‐IS 40 regimen for SHA patients with high‐titer inhibitors. Methods A prospective cohort study on patients receiving LD‐ITI‐IS 200 compared to those receiving LD‐ITI‐IS 40 from January 2021 to December 2023. Both received LD‐ITI [FVIII 50 IU/kg every other day]. IS (rituximab + prednisone) was added when peak inhibitor tier ≥200 BU/mL in the LD‐ITI‐IS 200 regimen and ≥40 BU/mL in the LD‐ITI‐IS 40 regimen. Success is defined as a negative inhibitor plus FVIII recovery ≥66% of the expected. Results We enrolled 30 patients on LD‐ITI‐IS 200 and 64 patients on LD‐ITI‐IS 40 , with similar baseline clinical characteristics. A lower IS‐use rate was discovered in the LD‐ITI‐IS 200 regimen compared to the LD‐ITI‐IS 40 regimen (30.0% vs. 62.5%). The two regimens (LD‐ITI‐IS 200 vs. LD‐ITI‐IS 40 ) had similar success rate (70.0% vs. 79.7%), median time to success (9.4 vs. 10.6 months), and annualized bleeding rate during ITI (3.7 vs. 2.8). The cost to success was lower for LD‐ITI‐IS 200 than for LD‐ITI‐IS 40 (2107 vs. 3256 US Dollar/kg). Among patients with peak inhibitor‐titer 40–199 BU/mL, 10 non‐IS‐using (on LD‐ITI‐IS 200 regimen) and 28 IS‐using (on LD‐ITI‐IS 40 regimen) had similar success rates (70.0% vs. 78.6%) and time to success (9.0 vs. 8.8 months). Interpretation In LD‐ITI, IS are not necessary for inhibitor titer <200 BU/mL.

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 categoriesMeta-epidemiology (narrow)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.288
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.001
Science and technology studies0.0000.000
Scholarly communication0.0000.001
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 head0.024
GPT teacher head0.276
Teacher spread0.252 · 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.

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 routes1
Has abstractyes

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