BENEFITS OF CHRONIC PLASMAPHERESIS AND INTRAVENOUS HEME-ALBUMIN IN ERYTHROPOIETIC PROTOPORPHYRIA AFTER ORTHOTOPIC LIVER TRANSPLANTATION12
Bibliographic record
Abstract
Erythropoietic protoporphyria (EPP) is characterized by a deficiency of ferrochelatase the final enzyme of the heme biosynthetic pathway. Patients with EPP may overproduce protoporphyrin IX, chiefly in developing erythrocytes. In some, protoporphyrin accumulates and causes toxicity, particularly to the skin and liver. Orthotopic liver transplantation (OLT) treats the severe liver disease that sometimes occurs in EPP; however, it does not correct the underlying metabolic disorder. We recently reported a patient with EPP who was improved with plasmapheresis and i.v. heme-albumin before OLT. Subsequently he developed histological and biochemical evidence of recurrent hepatotoxicity from protoporphyrin in the graft liver. We now report successful treatment of the patient with additional plasmapheresis and heme-albumin with improvement of hepatic histological and biochemical abnormalities. We conclude that plasmapheresis and heme-albumin are of benefit in EPP complicated by hepatotoxicity before and after liver transplantation.
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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 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 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 | 0.001 | 0.001 |
| 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".