Background: The severe life-threatening characteristics of hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE) can affect anxiety levels among pediatric patients. This emotional burden together with the physical restrictions of C1-INH-HAE may decrease children's health-related quality of life (HRQoL). Objectives: (i) To compare anxiety state and trait between children with C1-INH-HAE and healthy controls; (ii) to examine the relationship between the level of anxiety of children with C1-INH-HAE, their disease activity/affected sites and their HRQoL; and (iii) to predict the HRQoL of children with C1-INH-HAE based on their anxiety level and disease activity/affected sites. Methods: Thirty-three children with C1-INH-HAE (aged 5-18 years) and 52 healthy controls were recruited from Israel and Hungary. All children completed the State-Trait Anxiety Inventory for Children (STAIC), the Pediatric Quality of Life Inventory (Peds-QL) demographic questionnaire and a disease activity and site questionnaire. Disease activity was defined as the number of attacks in last year. Results: Both anxiety state and trait were significantly higher among children with C1-INH-HAE as compared to the controls (44.74±10.56 vs 38.76±10.67, P<.01, 29.21±5.16 vs 25.23±4.09, P<.001 in comparison). Significant differences were found between C1-INH-HAE patients with HAE attacks, asymptomatic C1-INH-HAE patients, and healthy controls in both anxiety state (F56,2=4.69, P=.001) and trait (F56,2=9.06, P<.0001). A higher anxiety trait was correlated with the number of angioedema-affected sites (r=.52, P=.003). The presence of HAE attacks and higher anxiety trait predicted a lower HRQoL in children with C1-INH-HAE. Conclusions: C1-INH-HAE children have higher anxiety trait and state, which correlate with reduced HRQoL domains.
- hereditary angioedema with C1-inhibitor deficiency
- pedsQL™ 4.0
- quality of life
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Immunology and Allergy