A systematic review of the efficacy and safety of a purified, pasteurized C1 inhibitor concentrate for the treatment of patients with type I or II hereditary angioedema
Bibliographic record
Abstract
Hereditary angioedema (HAE) is a rare disorder characterized by a congenital deficiency of the C1-inhibitor. It is a potentially fatal illness as laryngeal edema may lead to asphyxiation. Treatment of HAE is mainly performed with C1-inhibitor concentrate, icatibant, attenuated androgens, and antifibrinolytic agents. We conducted a systematic review to evaluate the efficacy and safety of a purified, pasteurized C1-inhibitor concentrate in the treatment of patients with type I or II HAE. An electronic search of five databases (Medline, EMBASE, Biosis Previews, CINAHL and the Cochrane Library) was performed with no language or date restrictions applied. Two reviewers used pre-defined criteria for inclusion of studies and the data were tabulated and analyzed. The data were found to be inappropriate for meta-analysis, and thus a qualitative synthesis was performed. We found a total of 63 studies that met our inclusion criteria. Treatment with the C1-inhibitor concentrate was consistently associated with clinically significant reductions in both the time to onset of symptom relief and duration of acute attacks. There were no cases of angioedema in patients who received the C1-inhibitor concentrate as short-term prophylaxis, and use of the C1-inhibitor concentrate as a long-term prophylactic agent resulted in a significant decrease in the frequency of attacks. No serious AEs were reported in any patients who were treated with the C1-inhibitor concentrate, and there were no treatment-related AEs as far as the injections were performed correctly. Treatment with the C1-inhibitor concentrate was not associated with transmission of viruses or development of autoantibodies. Results of this systematic review found the C1-inhibitor concentrate to be an effective option in the treatment of acute HAE attacks, and as a short- and long-term prophylactic agent.
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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 | 0.003 | 0.010 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.006 | 0.004 |
| Bibliometrics | 0.005 | 0.006 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.005 | 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".