Cardiopulmonary Testing and Pulmonary Hypertension in Patients with Sickle Cell Anemia
Bibliographic record
Abstract
Abstract Objectives: Sickle cell disease (SCD) is responsible for multiple complications that can lead to dyspnea, including pulmonary hypertension (PAH). PAH is a common cause of death among these patients and represents a prognosis feature, but can be clinically difficult to detect. We reviewed the charts of patients with SCD and analyse correlation between hemoglobin level, EKG, chest X-ray, pulmonary function tests, and cardiopulmonary exercise test (CPET) with echocardiography. Methods: We performed a retrospective chart review of 25 patients with SCD (11 patients with SC, 12 patients with SS and 2 patients with S-β thalassemia haplotype). All patients had pulmonary functions testing, CPET and resting echocardiography in the same period. PAH was defined as tricuspid regurgitant jet velocity (TRJV) >2,5 m/s on cardiac echography. Subjects underwent symptom-limited CPET using cycle ergometry and individualized ramp protocol. Exercise was stopped when subject showed criteria of maximality or inability to keep the pace. Breath-by-breath analysis was done. Results: Only 3 patients had PAH. All these patients were SS and suffered numerous acute thoracic syndrome. No correlation were made between lower hemoglobin level and higher TRJV; no patients had EKG or CXR anomaly. DLCO/VA appeared to be a independent risk factor: DLCO/VA < 75 % correlated with a TRJV > 2,5. CPET was abnormal in all patients, but were limited by patient fatigue and patterns were consistent with peripheral muscle disease except for one patient who had PAH; this patient showed a higher VE/VCO2 and a slower pulse kinetics, compatible with pulmonary vasculopathy. Conclusions: Decrease DLCO/VA may indicate a sickle pulmonary vasculopathy. and this factor appears to play a significant role in detecting PAH in SCD patients. CPET was a safe procedure and gave details about global aerobic performance and exercise tolerance. Because of peripheral muscle limitations, CPET still an unreliable diagnostic test for PAH. Peripheral muscle dysfunction could explain a large part of exercise intolerance, but physiopathologic mechanisms still underexplored.
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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 | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 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) | 0.001 | 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 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".