SAT-251 Massive Tissue Deposition Of Cholesterol Leading To Multi-organ Failure In Cholestatic Hypercholesterolemia
Bibliographic record
Abstract
Abstract Disclosure: D.R. Barros: None. B.G. Ballios: None. Y. Patoida: None. S. Singer: None. C.F. Rosen: None. M.A. Seidman: None. R.A. Hegele: None. G.F. Lewis: None. Background: Cholestasis-induced severe hypercholesterolemia can result in various complications, including xanthomas, although atherosclerosis is not a consistent feature. However, deposition of lipids in organs beyond the skin and tendons secondary to hepatobiliary dysfunction, to the point of eliciting organ dysfunction or failure, has not previously been reported. Case Presentations: Here we present two cases of marked hypercholesterolemia resulting from hepatobiliary disease. Patient 1 presented at a later stage of disease progression, with markedly elevated plasma cholesterol at 62.2 mmol/L (N<5.2 mmol/L) and triglycerides at 3.83 mmol/L (N<1.7 mmol/L). Massive cholesterol deposition in his retinas led to profound vision loss, while myocardium and kidney lipid infiltration ultimately resulted in fatal cardiorenal failure. Patient 2 presented with a lesser degree of hypercholesterolemia, with plasma cholesterol at 18.4 mmol/L. He was noted to have retinal cholesterol deposition without vision loss, along with extensive pruritic and painful tuberoeruptive xanthomas. Management and Outcome: Both patients were treated with weekly or biweekly plasmapheresis to prevent further disease progression, which successfully lowered plasma cholesterol to 6.92 and 5.88 mmol/L in patients 1 and 2, respectively. The multi-organ lipid deposition of patient 1 proved to be irremediable despite plasma exchange therapy. In contrast, six months of biweekly plasmapheresis in patient 2 yielded marked resolution of xanthomas and halted progression of retinal disease and vision loss. These cases reveal previously unappreciated non-atherosclerotic clinical consequences of severe hypercholesterolemia secondary to cholestatic liver disease, which can be ameliorated if detected early and treated with serial plasma exchange. Presentation: Saturday, July 12, 2025
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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) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| 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".