Batya Engel-Yeger1, Henriette Farkas2, Shmuel Kivity3, Nóra Veszeli2, Kinga Viktória Kőhalmi2, Aharon Kessel4. 1. Department Faculty of Social Welfare and Health Sciences, University of Haifa Occupational Therapy, Haifa, Israel. 2. 3rd Department of Internal Medicine, Hungarian Angioedema Center, Semmelweis University, Budapest, Hungary. 3. Allergy Department, Tel-Aviv Sourasky Medical Center, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel. 4. Division of Allergy and Clinical Immunology, Technion Faculty of Medicine, Bnai Zion Medical Center, Haifa, Israel.
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 (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.
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-HAEpatients, 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-HAEpatients, 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-HAEpatients 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.
Authors: Aleena Banerji; Yu Li; Paula Busse; Marc A Riedl; Nicole S Holtzman; Huamin Henry Li; Mark Davis-Lorton; Jonathan A Bernstein; Michael Frank; Anthony J Castaldo; Janet Long; Bruce Zuraw; William Lumry; Sandra Christiansen Journal: Allergy Asthma Proc Date: 2018-05-01 Impact factor: 2.587
Authors: Emel Aygören-Pürsün; Daniel F Soteres; Sandra A Nieto-Martinez; Jim Christensen; Kraig W Jacobson; Dumitru Moldovan; Arthur Van Leerberghe; Yongqiang Tang; Peng Lu; Moshe Vardi; Jennifer Schranz; Inmaculada Martinez-Saguer Journal: Pediatr Allergy Immunol Date: 2019-05-29 Impact factor: 6.377
Authors: Donald Levy; Teresa Caballero; Iftikhar Hussain; Avner Reshef; John Anderson; James Baker; Lawrence B Schwartz; Marco Cicardi; Subhransu Prusty; Henrike Feuersenger; Ingo Pragst; Michael E Manning Journal: Pediatr Allergy Immunol Pulmonol Date: 2020-09-16 Impact factor: 1.349