Health-related quality of life among children with hereditary angioedema

Batya Engel-Yeger, Henriette Farkas, Shmuel Kivity, Nóra Veszeli, Kinga Viktória Kőhalmi, Aharon Kessel

Research output: Contribution to journalArticlepeer-review


Background: The clinical expressions of hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and its related burden may negatively affect patient quality of life. This study aimed to assess health-related quality of life (HRQoL) in children with C1-INH-HAE. Methods: Children (N = 98: 34 C1-INH-HAE patients, 64 healthy controls) aged 3–18 years were recruited in Israel and Hungary. All individuals completed a demographic questionnaire, a disease activity and site questionnaire, and the Pediatric Quality of Life Inventory (PedsQL™) 4.0 Generic Core Scales (child self-report and maternal proxy report) to assess HRQoL. Results: Among C1-INH-HAE patients, nine (26.5%) had 1–5 attacks/year, six (17.6%) had 6–18 attacks/year, eight (23.5%) had 25–60 attacks/year, and 11 (32.4%) were asymptomatic over the previous year. Children with C1-INH-HAE attacks demonstrated lower HRQoL than healthy control children across the total score, school, and psychosocial dimensions of the PedsQL™. The number of C1-INH-HAE attacks negatively correlated with the total HRQoL score (r = −0.48, p = 0.008), school-related HRQoL (r = −0.39, p = 0.02), and psychosocial HRQoL (r = −0.43, p = 0.01). Patients with multisite laryngeal, abdominal, and peripheral C1-INH-HAE attacks had a lower HRQoL compared with those who experienced solely peripheral attacks across the total score (p = 0.04), physical (p = 0.04), and school (p = 0.02) domains. There was no significant difference between asymptomatic C1-INH-HAE patients and healthy controls. Conclusions: Children with symptomatic C1-INH-HAE demonstrate impaired HRQoL compared with healthy controls. HRQoL was affected by the frequency and site of C1-INH-HAE attacks and mostly in the school and physical domains.

Original languageEnglish
Pages (from-to)370-376
Number of pages7
JournalPediatric Allergy and Immunology
Issue number4
StatePublished - Jun 2017

Bibliographical note

Publisher Copyright:
© 2017 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.


  • C1-inhibitor
  • PedsQL™ 4.0
  • children
  • complement-1 esterase inhibitor defects
  • hereditary angioedema
  • quality of life
  • symptom assessment

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Immunology and Allergy
  • Immunology


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