From the Department of Pediatric Allergy, Hacettepe University Faculty of Medicine, Ankara, Turkey; and.
Department of Pediatric Allergy, Sanliurfa Training and Research Hospital, Sanliurfa, Turkey.
Allergy Asthma Proc. 2021 Jul 1;42(4):325-332. doi: 10.2500/aap.2021.42.210019.
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). 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. 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; = 0.043) and negatively correlated with self-esteem ( = -0.324; = 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 ( = 0.045). The family scores and total scores were statistically significantly higher in the HA group than the HAE group ( < 0.001 and = 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. 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.
遗传性血管性水肿（HAE）是一种潜在危及生命的疾病，极大地影响患者的健康相关生活质量（HRQoL）。本研究评估了 HAE 患儿的 HRQoL，并与健康对照和组胺能血管性水肿（HA）患者进行了比较。共招募了 59 例 HAE 患儿（中位数[四分位距{IQR}]，年龄 8.9 岁[5.4-12.6 岁]）、60 例 HA 患儿（中位数[IQR]，年龄 10.3 岁[8-15.4 岁]）和 72 名健康对照（中位数[IQR]，年龄 10.3 岁[6.6-13.9 岁]）。所有儿童及其家属均完成了适合年龄的 KINDL 通用量表，用于评估儿童和青少年的健康相关生活质量。HAE 患儿和 HA 患儿的总 HRQoL 评分和其他各分量表评分以及父母代理报告均显著低于健康儿童（p < 0.05）。在 HAE 患儿中，症状发作年龄与身体幸福感呈正相关（r = 0.335；p = 0.043），与自尊呈负相关（ = -0.324；p = 0.049）。此外，攻击部位会影响身体幸福感评分，腹痛发作的患儿评分显著较低（p = 0.045）。HA 组的家庭评分和总分均显著高于 HAE 组（p < 0.001 和 p = 0.009）。HAE 患儿的自我报告和父母代理 HRQoL 总分及各分量表评分均存在显著相关性。HA 组在家庭评分方面无显著相关性。HAE 导致儿童及其家属的 HRQoL 显著受损，并影响其生活方式和生活质量。在 HAE 儿科患者中，HRQoL 主要与症状发作年龄和攻击部位有关。虽然 HAE 患者受影响比 HA 患者更严重，但 HA 也会影响生活质量。
