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Record W4415696079 · doi:10.1183/23120541.00581-2025

Unmasking the true value of cystic fibrosis transmembrane conductance regulator modulators

2025· article· en· W4415696079 on OpenAlexaboutno aff
Aqeem Azam, Andrew Jones, Peter Barry

Bibliographic record

VenueERJ Open Research · 2025
Typearticle
Languageen
FieldMedicine
TopicCystic Fibrosis Research Advances
Canadian institutionsnot available
Fundersnot available
KeywordsCystic fibrosis transmembrane conductance regulatorCystic fibrosisRegulatorTransmembrane proteinConductanceValue (mathematics)

Abstract

fetched live from OpenAlex

<title>Extract</title> The licensing of the triple combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) in late 2019 in the United States (US) was the translation of impressive science with the potential to change the disease course for the approximately 90% of people with cystic fibrosis (pwCF) who have responsive CFTR gene variants. However, the true impact of the therapy was obscured by the SARS-CoV-2 pandemic in early 2020, that prompted global public health restrictions that abated some of the main drivers of infective respiratory exacerbations in pwCF including a reduction in all circulating respiratory viruses. It is therefore welcome to see this report by S<sc>tephenson</sc> <italic>et al.</italic> [1] who utilise CF patient registry data in the US and Canada to attempt to disentangle the relative impact of these two major events on the health of pwCF. This elegantly conducted study concludes that ETI had twice the effect of public health interventions in reducing pulmonary exacerbations.

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)0.004
metaresearch head score (Gemma)0.003
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Bench or experimental · Consensus signal: Bench or experimental
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.209
Threshold uncertainty score0.568

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0040.003
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0000.002
Science and technology studies0.0000.001
Scholarly communication0.0000.000
Open science0.0020.001
Research integrity0.0000.001
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 head0.059
GPT teacher head0.429
Teacher spread0.369 · 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 designBench or experimental
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
Published2025
Admission routes1
Has abstractyes

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