ArticleOrphanet journal of rare diseases2018
Emotional processes and stress in children affected by hereditary angioedema with C1-inhibitor deficiency: a multicenter, prospective study.
Article in Orphanet journal of rare diseases, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 2 of them syntheses that pooled it.
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Who cites it
22 citing papers in PubMed, 2 syntheses or guidelines pooled it, 32 citations in OpenAlex.
- International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema.Allergy · 2026Guideline
- Psychology and hereditary angioedema: A systematic review.Allergy and asthma proceedings · 2021Pooled it
- The 2025 WAO Guidelines for the classification, diagnosis, and treatment of hereditary angioedema, with consideration of worldwide disparities.The World Allergy Organization journal · 2026Article
- Is hereditary angioedema associated with deficits in emotion regulation? A quantitative study in adult patients.Orphanet journal of rare diseases · 2026Article
- Physiological evaluation of the emotional regulation of patients with hereditary angioedema.BioPsychoSocial medicine · 2026Article
- Frailty in elderly with a rare genetic disease: a geriatric score analysis in C1 inhibitor Hereditary Angioedema.Frontiers in medicine · 2026Article
- Hereditary angioedema in children: Review and practical perspective for clinical management.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2024Review
- Implications of Family-Oriented Psychotherapy for Hereditary Angioedema Management: A Case Series.Cureus · 2024Article
- Neurologic and Psychiatric Manifestations of Bradykinin-Mediated Angioedema: Old and New Challenges.International journal of molecular sciences · 2023Review
- The multifactorial impact of receiving a hereditary angioedema diagnosis.The World Allergy Organization journal · 2023Review
- The impact of puberty on the onset, frequency, location, and severity of attacks in hereditary angioedema due to C1-inhibitor deficiency: A survey from the Italian Network for Hereditary and Acquired Angioedema (ITACA).Frontiers in pediatrics · 2023Article
- Pregnancy in women with Hereditary Angioedema due to C1-inhibitor deficiency: Results from the ITACA cohort study on outcome of mothers and children withFrontiers in medicine · 2022Article
- Psychological processes in the experience of hereditary angioedema in adult patients: an observational study.Orphanet journal of rare diseases · 2021Observational
- Prophylactic Treatment in Hereditary Angioedema Is Associated with Reduced Anxiety in Patients in Leipzig, Germany.International archives of allergy and immunology · 2021Article
- Going Beyond the Visible in Type 2 Diabetes Mellitus: Defense Mechanisms and Their Associations With Depression and Health-Related Quality of Life.Frontiers in psychology · 2020Article
- How Closely Related Are Parent and Child Reports of Child Alexithymia?Frontiers in psychology · 2020Article
- The Semiotic Construction of the Sense of Agency. The Modal Articulation in Narrative Processes.Integrative psychological & behavioral science · 2019Article
- Increased fibrinolysis-induced bradykinin formation in hereditary angioedema confirmed using stored plasma and biotechnological inhibitors.BMC research notes · 2019Article
- Serum fetuin-A, tumor necrosis factor alpha and C-reactive protein concentrations in patients with hereditary angioedema with C1-inhibitor deficiency.Orphanet journal of rare diseases · 2019Article
- Caregivers' Sensemaking of Children's Hereditary Angioedema: A Semiotic Narrative Analysis of the Sense of Grip on the Disease.Frontiers in psychology · 2019Article
Corrections and comments
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Authors and funding
19 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is characterized by recurrent edema of unpredictable frequency and severity. Stress, anxiety, and low mood are among the triggering factors most frequently reported. Impaired regulation and processing of emotions, also known as alexithymia, may influence outcomes. The aim of this study was to confirm the presence of alexithymia and stress in children with C1-INH-HAE, to determine whether they are also present in children affected by other chronic diseases, and to investigate their relationship with C1-INH-HAE severity. Data from children with C1-INH-HAE (n = 28) from four reference centers in Italy were compared with data from children with type 1 diabetes (T1D; n = 23) and rheumatoid arthritis (RA; n = 25). Alexithymia was assessed using the Alexithymia Questionnaire for Children scale; perceived stress was assessed using the Coddington Life Event Scale for Children (CLES-C).
resultsMean age (standard deviation [SD]) in the C1-INH-HAE, T1D, and RA groups was 11.8 (3.3), 11.7 (2.9), and 11.1 (2.6) years, respectively. Mean C1-INH-HAE severity score was 5.9 (2.1), indicating moderate disease. Alexithymia scores were similar among disease groups and suggestive of difficulties in identifying and describing emotions; CLES-C scores tended to be worse in C1-INH-HAE children. C1-INH-HAE severity was found to correlate significantly and positively with alexithymia (p = 0.046), but not with perceived stress. Alexithymia correlated positively with perceived stress.
conclusionsAlexithymia is common in children with chronic diseases. In C1-INH-HAE, it may result in increased perceived stress and act as a trigger of edema attacks. Comprehensive management of C1-INH-HAE children should consider psychological factors.
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