TY - JOUR
T1 - Health-related quality of life among children with hereditary angioedema
AU - Engel-Yeger, Batya
AU - Farkas, Henriette
AU - Kivity, Shmuel
AU - Veszeli, Nóra
AU - Kőhalmi, Kinga Viktória
AU - Kessel, Aharon
N1 - Publisher Copyright:
© 2017 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.
PY - 2017/6
Y1 - 2017/6
N2 - 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.
AB - 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.
KW - C1-inhibitor
KW - PedsQL™ 4.0
KW - children
KW - complement-1 esterase inhibitor defects
KW - hereditary angioedema
KW - quality of life
KW - symptom assessment
UR - http://www.scopus.com/inward/record.url?scp=85017411263&partnerID=8YFLogxK
U2 - 10.1111/pai.12712
DO - 10.1111/pai.12712
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AN - SCOPUS:85017411263
SN - 0905-6157
VL - 28
SP - 370
EP - 376
JO - Pediatric Allergy and Immunology
JF - Pediatric Allergy and Immunology
IS - 4
ER -