Health-related quality of life in children with hereditary angioedema compared with patients with histaminergic angioedema.

Abstract

Background: Hereditary angioedema (HAE) is a potentially life-threatening disease that remarkably impacts patients' health-related quality of life (HRQoL). This study evaluated the HRQoL in children with HAE and compared it with healthy controls and patients with histaminergic angioedema (HA). Methods: Fifty-nine children with HAE (median [interquartile range {IQR}], ages, 8.9 years [5.4-12.6 years]), 60 children with HA (median [IQR] ages, 10.3 years [8-15.4 years]), and 72 healthy controls (median [IQR] ages, 10.3 years [6.6-13.9 years]) were recruited. All the children and their families completed the age-adapted KINDL generic instrument for assessing health-related quality of life in children and adolescents. Results: The total HRQoL score and other subscales scores of both the patients with HAE and patients with HA, and the parent-proxy reports were significantly lower than those of the healthy children (p < 0.05). In the patients with HAE, the age at symptom onset was positively correlated with physical well-being (r = 0.335; p = 0.043) and negatively correlated with self-esteem (r = -0.324; p = 0.049). In addition, the physical well-being scores were affected by the site of attacks, which was significantly lower in the patients with abdominal pain attacks (p = 0.045). The family scores and total scores were statistically significantly higher in the HA group than the HAE group (p < 0.001 and p = 0.009, respectively). A significant correlation was found between self-report and parent-proxy HRQoLs in total and all subscales scores in the patients with HAE. For HA, there were no significant correlations for family scores. Conclusion: HAE caused significant impairment of the HRQoL of children and their families, and affected their lifestyle and quality of life. In pediatric patients with HAE, the HRQoL is mainly related to age at symptom onset and the site of attacks. Although patients with HAE are affected more negatively than the patients with HA, HA affected the quality of life as well.