# Psychological processes in the experience of hereditary angioedema in adult patients: an observational study.

- **DOI:** [10.1186/s13023-020-01643-x](https://doi.org/10.1186/s13023-020-01643-x)
- **Rating:** Limited (2.6 out of 5)
- **Verdict:** Adult HAE data add a modest stress–engagement signal, but cross-sectional causal interpretation and sparse contingency analysis prevent clinically reliable inference.
- **Framework version:** v5.9
- **Pipeline version:** v0.4
- **Evaluation date:** 2026-09-18
- **Evaluation page:** https://www.nabu.science/paper/10.1186%2Fs13023-020-01643-x

## Key claims

1. Stress correlated with patient health engagement and with psychological discomfort.
   - Support: Tentative
   - Type: Primary finding
   - Basis: small concomitant sample and sparse PHE contingency cells limit confidence in the association
   - Location: Abstract; Results, Tables 2, 4, and 5
2. severity of C1-INH-HAE was not found to be associated with stress nor with health engagement.
   - Support: Tentative
   - Type: Secondary
   - Basis: cross-sectional null tests in 28 patients have limited power to exclude clinically relevant relationships
   - Location: Discussion; Results, Table 4
3. Alexithymia rarely occurred in this group of patients; only three subjects had a high TAS score, and emotion regulation strategies do not differ from the ones of the normative healthy sample’s scores.
   - Support: Supported
   - Type: Secondary
   - Basis: direct sample descriptions support the scoped claim, though normative comparison and sample size constrain generalisation
   - Location: Discussion, alexithymia paragraph; Results, Table 1
4. alexithymia and deficits in emotion regulation characterize the experience of children with chronic diseases due to their developmental stage, whereas adults become able to recognize and regulate their own emotions independently from being affected by the disease
   - Support: Tentative
   - Type: Secondary
   - Basis: a cross-sectional adult sample without a pediatric comparison cannot identify the proposed developmental explanation
   - Location: Discussion, alexithymia paragraph

## Strengths

- **Extends evidence into adult HAE** The study tests psychological patterns in adults with C1-INH-HAE and explicitly contrasts the results with prior pediatric findings, including the absence of the expected severity relationship.
  - Dimension: contribution
  - Location: Background, final paragraph; Discussion, alexithymia and pediatric-comparison paragraphs
- **Uses established psychological assessment tools** Clinical and psychological variables were measured with validated Italian translations of the severity score, TAS-20, ERC, PSS, PHE-S, and SCL-90-R.
  - Dimension: methodological rigour
  - Location: Patients and Methods, Assessments
- **Reports null results alongside associations** Tables 4 and 5 present non-significant severity, engagement, alexithymia, GSI, and psychoticism comparisons rather than limiting reporting to significant findings.
  - Dimension: methodological rigour
  - Location: Results, Tables 4 and 5

## Limitations

- **Cross-sectional data support causal implications** All scales were completed concomitantly, yet the Discussion states that better knowledge causes less stress and proposes engagement as a therapeutic target. The design cannot establish this temporal or causal pathway.
  - Dimension: methodological rigour
  - Location: Patients and Methods, Assessments; Discussion, PHE interpretation and conclusion
- **Sparse tables weaken headline association** The PHE–stress chi-square analysis includes zero and small cells without a reported exact alternative or approximation check, limiting confidence in the principal association.
  - Dimension: methodological rigour
  - Location: Results, Tables 2 and 5; Statistical Analysis
- **Selection and precision remain underexamined** The 28 participants came from three referral centres, but recruitment flow, participation denominators, and sensitivity analyses are not reported. The resulting generalisability and stability of the estimates are uncertain.
  - Dimension: positioning
  - Location: Patients and Methods, Study Design; Discussion, limitations paragraph

## Reliability checks

Caveats (4 of 4 checks).

- **Internal coherence:** Noted. The reported counts generally preserve the direction of the findings, but sparse contingency cells and several percentage errors reduce confidence in the precision of the tabular statistical reporting.
- **Research conduct:** Clean. Ethics approval and informed consent are explicitly declared. No supplied-text evidence indicates a conduct concern; unreported COI, registration, data, and code statements are not treated as adverse findings.
- **Reference integrity:** Clean. 16 of 16 checkable references verified
  - 20 references in manuscript
  - 4 have no canonical index record — counted, but not index-checkable
  - 4 references confirmed by manual review
  - 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