Pulmonary involvement in patients with Marfan Syndrome.
Bibliographic record
Abstract
AIM: Pulmonary involvement is not generally considered a main feature of Marfan syndrome, an autosomal connective tissue disorder caused by mutations in fibrillin 1. Thanks to the substantial progress in treatments, life expectation of these patients has been dramatically improved determining changes in different organ systems. The number of patients with pulmonary pathology may be higher than expected. Objective of the study was to evaluate the pulmonary involvement in all the patients referring to the largest Italian center for Marfan syndrome, assessing clinical examination and lung function tests. METHODS: Clinical history, spirometry, lung volumes and diffusing capacity have been assessed in 64 patients of our national referral center. RESULTS: None of the patients reported chronic respiratory symptoms. Fourteen percent reported a previous pneumothorax and 3 blebs and 45% had moderate to severe rib cage abnormalities. Twenty-three percent had cardiothoracic surgery. Two of the 19 patients with chest TC performed at our hospital were diagnosed with emphysema and were both non smoker; 7 had subpleural apical blebs. Only 37% of patients had normal lung function; 19% showed a restrictive pattern and 44% an obstructive pattern or an isolated diffusion impairment or an isolated hyperinflation. All patients with pneumothorax showed an obstructive pattern and diffusion impairment. CONCLUSION: In the absence of early respiratory symptoms, pulmonary abnormalities should be detected and monitored before they aggravate. Particular attention should be paid to prevent pneumothorax. Our results support the importance of lung volume determination to identify patients in which pulmonary parenchyma require a careful chest CT evaluation.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot 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.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
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.
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 teacher head, 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".