# Short-term prophylaxis in patients with angioedema due to C1-inhibitor deficiency undergoing dental procedures: An observational study.

- **DOI:** [10.1371/journal.pone.0230128](https://doi.org/10.1371/journal.pone.0230128)
- **Rating:** Limited (2.5 out of 5)
- **Verdict:** A useful rare-disease case series reinforces dental prophylaxis practice, but its uncontrolled design cannot establish the causal efficacy claims it makes.
- **Framework version:** v5.9
- **Pipeline version:** v0.4
- **Evaluation date:** 2026-09-18
- **Evaluation page:** https://www.nabu.science/paper/10.1371%2Fjournal.pone.0230128

## Key claims

1. STP with pdC1-INH was effective in preventing post-procedural attacks.
   - Support: Tentative
   - Type: Primary finding
   - Basis: definitive efficacy language rests on non-comparable prophylaxis groups and only two wholly unprotected procedures
   - Location: Conclusions, paragraph 2
2. Most C1-INH-HAE patients reported hurdles in receiving dental care.
   - Support: Supported
   - Type: Secondary
   - Basis: directly reported by 17 of 27 patients with available access data in one specialist-centre cohort
   - Location: Abstract Conclusions; Conclusions, paragraph 1
3. Treating oral diseases results in improvement in the frequency of attacks.
   - Support: Tentative
   - Type: Secondary
   - Basis: causal framing rests on an uncontrolled pre/post comparison with improvement observed in four patients
   - Location: Abstract Conclusions

## Strengths

- **Concrete rare-disease access finding** The records show that 17 of 27 patients with available data reported hurdles in obtaining dental care, identifying a specific problem for dentists, referral centres, and affected patients.
  - Dimension: contribution
  - Location: Results, paragraph 2; Conclusions, paragraph 1
- **Transparent procedural outcome documentation** The Methods specify STP drugs, doses, timing, long-term prophylaxis, and a 48-hour post-procedure attack window. Results distinguish the unprotected HAE extraction attack from the AAE breakthrough attack after pdC1-INH.
  - Dimension: methodological rigour
  - Location: Methods, Study Population and Endpoints; Results, prophylaxis and attack paragraphs
- **Relevant linkage to existing evidence** The Discussion compares the cohort with Bork's extraction data, the Berinert Registry, Italian guidance, infection-trigger research, and prior retrospective analyses. This places the observations within the existing clinical evidence trajectory.
  - Dimension: positioning
  - Location: Discussion, STP, Bork, Magerl, Farkas, and guideline paragraphs

## Limitations

- **Causal efficacy remains unidentified** Only two procedures received neither STP nor LTP, and prophylaxis status was intertwined with procedure and clinical context. The resulting groups cannot establish that STP caused the observed difference in attacks.
  - Dimension: methodological rigour
  - Location: Results, prophylaxis distribution; Discussion, STP effectiveness paragraphs
- **Uncontrolled dental-care comparison** The reported improvement occurred in four of 19 patients after dental care, without statistical testing or control for concurrent prophylaxis, natural fluctuation, selection, or regression to the mean. This concern materially affects the paper's causal interpretation.
  - Dimension: methodological rigour
  - Location: Results, final paragraph; Discussion, oral-pathology impact paragraph; Conclusions, paragraph 3
- **Conclusions contradict stated caution** The limitations say retrospective data require interventional confirmation, yet the Abstract and Conclusions state that STP protects and oral treatment produces improvement. This leaves the central claims more definitive than the acknowledged evidence permits.
  - Dimension: positioning
  - Location: Discussion, limitations paragraph 3; Abstract Conclusions; Full-text Conclusions, paragraphs 2–3

## Reliability checks

Concerns (4 of 4 checks).

- **Internal coherence:** Concern. The reported observations are broadly coherent, but the paper interprets them as causal evidence despite an uncontrolled design with only two procedures receiving no prophylaxis and no adjustment for clinical selection or concurrent prophylaxis. The dental-care improvement claim likewise relies on an uncontrolled pre/post comparison.
- **Research conduct:** Clean. Ethics approval, Helsinki compliance, and written informed consent are declared. No research-conduct concern is established from the supplied text; the referenced S1 Data file remains an assessability gap only.
- **Reference integrity:** Clean. 14 of 14 checkable references verified
  - 14 references in manuscript
  - References not verified automatically are resolved manually within 24 hours.

## Post-publication record

- **Status:** Clean
- **Summary:** No retraction or expression of concern found in Crossref (includes Retraction Watch).
- **Last checked:** 2026-10-17