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- Title
Health-related quality of life among children with hereditary angioedema.
- Authors
Engel‐Yeger, Batya; Farkas, Henriette; Kivity, Shmuel; Veszeli, Nóra; Kőhalmi, Kinga Viktória; Kessel, Aharon
- Abstract
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 (Peds QL™) 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 Peds QL™. 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.
- Subjects
QUALITY of life; ANGIONEUROTIC edema; GENETIC disorders in children; ENZYME inhibitors; ESTERASES
- Publication
Pediatric Allergy & Immunology, 2017, Vol 28, Issue 4, p370
- ISSN
0905-6157
- Publication type
Article
- DOI
10.1111/pai.12712