Pulmonary veno-occlusive disease: a paradigm of diagnosis and therapeutic challenges in pulmonary hypertension
Bibliographic record
Abstract
PURPOSE OF REVIEW: Pulmonary veno-occlusive disease (PVOD) is a rare and life-threatening form of precapillary pulmonary hypertension. This review aims to outline its genetic and environmental risk factors, highlight key diagnostic challenges, and discuss current treatment options. RECENT FINDINGS: PVOD can occur sporadically or as a hereditary autosomal recessive condition with biallelic eukaryotic translation initiation factor 2 alpha kinase 4 ( EIF2AK4) mutations, leading to nearly complete disease penetrance. Known risk factors include specific drug/toxin and environmental exposures, such as mitomycin C and trichloroethylene, respectively. PVOD is characterized by progressive pulmonary venous and capillary remodelling, severe hypoxemia, and right ventricular failure. Diagnosis remains difficult due to overlapping features with pulmonary arterial hypertension (PAH), but high-resolution computed tomography (HRCT) findings, low lung diffusion capacity for carbon monoxide (DLCO), and genetic testing can aid differentiation. Initiation of PAH-approved drugs in patients with PVOD requires careful consideration due to limited evidence of long-term clinical benefits and the high risk of developing pulmonary oedema in this population. Lung transplantation remains the only curative treatment, with posttransplant survival rates comparable to idiopathic PAH. SUMMARY: PVOD is a progressive and fatal disease requiring early recognition and specific management. Due to its poor prognosis and lack of effective medical therapies, early referral for lung transplantation is crucial. Advances in genetic and molecular research may lead to novel treatment strategies.
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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 | 0.003 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.002 | 0.002 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.002 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
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".