SP149ECULIZUMAB TREATMENT OF SHIGA TOXIN ESCHERICHIA COLI HEMOLYTIC UREMIC SYNDROME
Bibliographic record
Abstract
INTRODUCTION AND AIMS: Hemolytic uremic syndrome (HUS) has two forms: Shiga toxin-producing Escherichia coli HUS (STEC-HUS) and atypical HUS (aHUS). The underlying common pathophysiology is thrombotic microangiopathy (TMA), triggering endothelial damage. Complement activation has been reported in both diseases. In STEC-HUS, Shiga toxin upregulates P-selectin activating the alternate complement pathway. In aHUS, genetic mutations produce dysregulated alternate complement pathway activation. Traditionally, STEC-HUS treatment is supportive. In aHUS, eculizumab, a monoclonal antibody to C5, blocks formation of C5b-9, the membrane attack complex. Selected case reports have shown improvements in STEC-HUS patients treated with eculizumab. Herein, we describe a case of a 17 year old female with STEC-HUS treated with eculizumab. METHODS: Retrospective case study. RESULTS: The patient presented with bloody diarrhea, gross hematuria, thrombocytopenia hemolytic anemia, acute kidney injury (with macro-proteinuria) requiring dialysis. She was empirically treated with plasmapheresis. Stool PCR was positive for STEC, confirming STEC-HUS. ADAMTS-13 activity was normal and serum C5b-9 levels were elevated. She did not improve with plasmapheresis, therefore eculizumab was started. Within 48 hours, she developed visual loss, hemiparesis and seizures in the context of severe hypertension. MRI head showed posterior reversible encephalopathy syndrome. Continued eculizumab therapy resulted in complete resolution of TMA and normalization of kidney function. Subsequent testing of this patient's serum for complement activation by quantifying C5b-9 deposition on endothelial cells demonstrated elevated C5b-9 deposition at initiation of eculizumab, which normalized with eculizumab treatment. CONCLUSIONS: Few cases of STEC-HUS have been treated with eculizumab. This case is unusual given its severity of complement activation resulting from STEC-HUS. This raises the possibility of an underlying genetic predisposition to aHUS in this patient, but also suggests that eculizumab therapy might be beneficial in severe STEC-HUS.
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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".