Type III hereditary angioedema: diagnostic and management challenges (P3173)
Bibliographic record
Abstract
Abstract INTRODUCTION: Type III hereditary angioedema (HAE-III) is a heterogeneous entity presenting with normal C1-INH levels and is often difficult to diagnose. CASE PRESENTATION:A 13-year-old previously healthy girl presented to our emergency department with facial angiodema. Medications included Naproxen for tooth pain and oral combined contraceptives for menometrorrhagia that she had been taking for 6 months. She initially developed pruritic plaques on her legs that resolved within 5 days. On the day of presentation, she developed progressively painful lips’ swelling. There were no systemic symptoms. Angioedema resolved within 24 hours and did not recur over a three-month follow-up period. Family history was notable for recurrent angioedema in her mother and two maternal aunts. RESULTS:Investigations revealed normal tryptase level, C3 and C4 levels, C1 esterase inhibitor level and functional protein assay. DISCUSSION:Given the family history and clinical presentation, the diagnosis is consistent with HAE-III. In contrast to other forms of HAE, normal C4 levels do not rule out this entity. NSAIDs have also been implicated in its pathogenesis and could have contributed to her symptoms. Recent studies suggest that HAE-III may be associated with urticaria. In this case, prophylactic medication such as tranexamic acid or bradykinin inhibitors will be determined according to attacks’ frequency and severity. Clinicians should maintain high level of awareness of this rare disease.
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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 | 0.001 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.002 |
| Insufficient payload (model declined to judge) | 0.003 | 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".