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Analysis of mucolytic therapy use in cystic fibrosis based on national registries

2025· article· W7117585239 on OpenAlexaboutno aff
V. V. Shadrina, E. I. Kondratyeva

Bibliographic record

VenueArchives of Pediatrics and Pediatric Surgery · 2025
Typearticle
Language
FieldMedicine
TopicCystic Fibrosis Research Advances
Canadian institutionsnot available
Fundersnot available
KeywordsCystic fibrosisHypertonic salineInhalationDosingPharmacotherapyDrug

Abstract

fetched live from OpenAlex

An analysis of mucolytic drug use was conducted using data from national cystic fibrosis (CF) patient registries in Europe, the US, Canada, Australia, and Russia. Mucolytic therapy is a key component of CF treatment. Mucolytic drugs are used in patients of all ages. During the study period, dornase alfa was prescribed to the largest number of patients across all registries. In Russia, this drug is available to all patients, largely owing to the “14 High- Cost Nosologies” program and the presence of a dornase alfa biosimilar produced in Russia. According to registry data, dornase alfa was used more frequently in Russia than in other countries – up to 95.8% in 2019, with 25.9% of patients receiving a second dose intranasally (in 2023). In the US, dornase alfa was prescribed to up to 90% of patients, in Australia – up to 60%, in Canada – up to 52% of adult patients, and in the EU, more than 50% of patients received this drug. Among rapid-acting mucolytics, hypertonic saline inhalation was the most commonly prescribed, accounting for up to 77% of patients in the US, up to 72.7% in Russia, up to 52.3% in the EU, up to 45.4% in Australia, and up to 41.6% in Canada among adult CF patients. Mannitol inhalation was used in individual cases, primarily in adolescence (up to 15.7% of adolescents with CF in Australia in 2020). In recent years, a slight decrease in the use of mucolytic medications has been observed across all countries under study.

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 machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.005
metaresearch head score (Gemma)0.012
Version: metacan-v3-hybrid-931329e0061cValidation 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 score0.011
Threshold uncertainty score0.024

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0050.012
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.002
Bibliometrics0.0050.008
Science and technology studies0.0000.000
Scholarly communication0.0010.001
Open science0.0010.001
Research integrity0.0000.001
Insufficient payload (model declined to judge)0.0020.000

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 head0.025
GPT teacher head0.297
Teacher spread0.272 · 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 source (direct Gemma or distilled Codex), 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
Published2025
Admission routes1
Has abstractyes

Explore more

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