title: Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults: HAE-QoL.
abstract: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) may affect health-related quality of life (HRQoL). A specific HRQoL questionnaire for adult patients with C1-INH-HAE, the HAE-QoL, has recently been developed in Spain.
  
  
  
  The objective of this study was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting.
  
  
  
  Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire, and Short Form 36-item Health Survey Version 2.0 (SF-36v2) were pilot tested internationally. Item reduction was based on both descriptive and exploratory factor analysis. Psychometric properties were assessed.
  
  
  
  Cross-cultural adaptation of the HAE-QoL was performed in 18 countries. The draft version of the HAE-QoL was pilot tested in 332 patients, and accurate data were obtained from 290 patients from 11 countries. The reduction process resulted in a new version with 25 items and 7 dimensions (treatment difficulties, physical functioning and health, disease-related stigma, emotional role and social functioning, concern about offspring, perceived control over illness, and mental health). Strong psychometric properties were observed (Cronbach's α 0.92; test-retest reliability 0.87). Convergent validity showed mild to moderate correlations with SF-36v2 physical and mental component summaries (0.45 and 0.64, respectively) and with SF-36v2 dimensions (P < .004). HAE-QoL scores discriminated significantly among severity groups (median: asymptomatic 133.5 vs severe 84.0; P < .001); between patients with and without long-term prophylaxis (median: 101 vs 90; P = .001); and between patients with and without psychiatric and/or psychological care (median: 74 vs 103; P ≤ .001).
  
  
  
  The HAE-QoL, currently available in 18 languages, showed good reliability and validity evidence.
authors.affiliation: Allergy Department, Hospital Universitario Severo Ochoa, Leganés, Madrid, Spain. Electronic address: nivprior@gmail.com.
authors.forename: Nieves
authors.initials: N
authors.lastname: Prior
authors.affiliation: Faculty of Psychology, Universidad Autónoma, Madrid, Spain.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Remor
authors.affiliation: Research Unit, Hospital Universitario Fundación Alcorcón, Madrid, Spain.
authors.forename: Elia
authors.initials: E
authors.lastname: Pérez-Fernández
authors.affiliation: Allergy Department, Hospital La Paz Institute for Health Research (IdiPaz), Madrid, Spain.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Caminoa
authors.affiliation: Allergy Department, Hospital La Paz Institute for Health Research (IdiPaz), Madrid, Spain.
authors.forename: Carmen
authors.initials: C
authors.lastname: Gómez-Traseira
authors.affiliation: Research Unit, Hospital La Paz Institute for Health Research Institute (IdiPAZ), Madrid, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Gayá
authors.affiliation: Department of Dermatology and Allergy Centre and OPEN Odense Patient data Explorative Network, Odense University Hospital, Odense, Denmark.
authors.forename: Anne
authors.initials: A
authors.lastname: Aabom
authors.affiliation: Department of Dermatology, Medical University, Graz, Austria.
authors.forename: Werner
authors.initials: W
authors.lastname: Aberer
authors.affiliation: Division of Allergy and Clinical Immunology St. Michael's Hospital, Toronto, Canada.
authors.forename: Stephen
authors.initials: S
authors.lastname: Betschel
authors.affiliation: National Reference Centre for Angioedème (CREAK), Internal Medicine Department, Grenoble University Hospital Joseph Fourier University of Medicine, Grenoble, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Boccon-Gibod
authors.affiliation: National Reference Centre for Angioedème (CREAK), Internal Medicine Department, Grenoble University Hospital Joseph Fourier University of Medicine, Grenoble, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Bouillet
authors.affiliation: Department of Dermatology and Allergy Centre and OPEN Odense Patient data Explorative Network, Odense University Hospital, Odense, Denmark.
authors.forename: Anette
authors.initials: A
authors.lastname: Bygum
authors.affiliation: National Angioedema Center 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
authors.forename: Dorottya
authors.initials: D
authors.lastname: Csuka
authors.affiliation: National Angioedema Center 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.affiliation: Faculty of Medicine ABC, Sao Paulo, Brazil.
authors.forename: Maria
authors.initials: M
authors.lastname: Gomide
authors.affiliation: Faculty of Medicine ABC, Sao Paulo, Brazil.
authors.forename: Anete
authors.initials: A
authors.lastname: Grumach
authors.affiliation: Allergy, Clinical Immunology and Angioedema Unit, Chaim Sheba Medical Center, Tel Hashomer, Israel.
authors.forename: Iris
authors.initials: I
authors.lastname: Leibovich
authors.affiliation: Unidad de Alergia, Asma e Inmunología Clínica, Buenos Aires, Argentina.
authors.forename: Alejandro
authors.initials: A
authors.lastname: Malbran
authors.affiliation: Department of Allergology-Immunology, Mures County Hospital, Tìrgu-Mureş Romania.
authors.forename: Dumitru
authors.initials: D
authors.lastname: Moldovan
authors.affiliation: Department of Allergology-Immunology, Mures County Hospital, Tìrgu-Mureş Romania.
authors.forename: Eniko
authors.initials: E
authors.lastname: Mihaly
authors.affiliation: Department of Clinical and Environmental Allergology, Jagiellonian University, Krakow, Poland.
authors.forename: Krystyna
authors.initials: K
authors.lastname: Obtulowicz
authors.affiliation: Unidad de Alergia, Asma e Inmunología Clínica, Buenos Aires, Argentina.
authors.forename: Cecilia
authors.initials: C
authors.lastname: Perpén
authors.affiliation: Department of Dermatology, University Medical Center Mainz, Mainz, Germany.
authors.forename: Adriane
authors.initials: A
authors.lastname: Peveling-Oberhag
authors.affiliation: Department of Clinical and Environmental Allergology, Jagiellonian University, Krakow, Poland.
authors.forename: Grzegorz
authors.initials: G
authors.lastname: Porebski
authors.affiliation: Division of Allergy and Clinical Immunology St. Michael's Hospital, Toronto, Canada.
authors.forename: Celine Rayonne
authors.initials: CR
authors.lastname: Chavannes
authors.affiliation: Allergy, Clinical Immunology and Angioedema Unit, Chaim Sheba Medical Center, Tel Hashomer, Israel.
authors.forename: Avner
authors.initials: A
authors.lastname: Reshef
authors.affiliation: Department of Dermatology, University Medical Center Mainz, Mainz, Germany.
authors.forename: Petra
authors.initials: P
authors.lastname: Staubach
authors.affiliation: Department of Dermatology, Medical University, Graz, Austria.
authors.forename: Michaela
authors.initials: M
authors.lastname: Wiednig
authors.affiliation: Allergy Department, Hospital La Paz Institute for Health Research (IdiPaz), Madrid, Spain; Biomedical Research Network on Rare Diseases (CIBERER, U754), Madrid, Spain.
authors.forename: Teresa
authors.initials: T
authors.lastname: Caballero
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000523:psychology*; D005260:Female; D006801:Humans; D008297:Male; D011594:Psychometrics; D011788:Quality of Life; D011795:Surveys and Questionnaires
keywords: Adults; C1 inhibitor; Disease-specific; HAE-QoL; Hereditary angioedema; Psychometric; Quality of life; Questionnaire; SF-36v2; Validation studies