Pulmonary arterial hypertension and targeting pulmonary vascular remodelling: are we there yet?
Bibliographic record
Abstract
Extract Pulmonary arterial hypertension (PAH) is a rare, progressive disorder of the pre-capillary pulmonary circulation, characterised by progressive pulmonary vascular remodelling and vasoconstriction, increased pulmonary vascular resistance, right ventricular hypertrophy, right heart failure and premature death, despite the development of several effective therapies [1–3]. Several factors have been implicated in the pulmonary vascular remodelling, including inflammatory, immunological, epigenetic and genetic factors, such as bone morphogenetic protein receptor type 2 (BMPR2) genetic variants, the most prevalent genetic variant identified in hereditable PAH [4]. Strong associations have been identified between the development of PAH, and 1) aberrant bone morphogenetic protein (BMP) signalling via BMPR2 receptors, 2) dysregulated growth factors, manifesting an imbalance in proliferative and anti-proliferative transforming growth factor (TGF)-β signalling and upregulation of the activin pathway regulated by ActRIIA, 3) progressive endothelial cell dysfunction, 4) pathological distal fibro-proliferation, and 5) plexogenic pulmonary vascular remodelling [4, 5].
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot 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.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.006 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.003 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.014 | 0.012 |
| Insufficient payload (model declined to judge) | 0.012 | 0.008 |
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.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
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".