title: Hereditary angioedema: quality of life in Brazilian patients.
abstract: OBJECTIVE
  Hereditary angioedema is a serious medical condition caused by a rare autosomal dominant genetic disorder and it is associated with deficient production or dysfunction of the C1 esterase inhibitor. In most cases, affected patients experience unexpected and recurrent crises of subcutaneous, gastrointestinal and laryngeal edema. The unpredictability, intensity and other factors associated with the disease impact the quality of life of hereditary angioedema patients. We evaluated the quality of life in Brazilian hereditary angioedema patients.
  
  
  METHODS
  Patients older than 15 years with any severity of hereditary angioedema and laboratory confirmation of C1 inhibitor deficiency were included. Two questionnaires were used: a clinical questionnaire and the SF-36 (a generic questionnaire). This protocol was approved by the Ethics Committee of Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo.
  
  
  RESULTS
  The SF-36 showed that 90.4% (mean) of all the patients had a score below 70 and 9.6% had scores equal to or higher than 70. The scores of the eight dimensions ranged from 51.03 to 75.95; vitality and social aspects were more affected than other arenas. The internal consistency of the evaluation was demonstrated by a Cronbach's alpha value above 0.7 in seven of the eight domains.
  
  
  CONCLUSIONS
  In this study, Brazilian patients demonstrated an impaired quality of life, as measured by the SF-36. The most affected domains were those related to vitality and social characteristics. The generic SF-36 questionnaire was relevant to the evaluation of quality of life; however, there is a need for more specific instruments for better evaluation.
authors: Faculdade de Medicina da Universidade de São Paul, Department of Dermatology, São Paulo/SP, Brazil.
authors: Maria Abadia Consuelo M S
authors: MA
authors: Gomide
authors: Eliana
authors: E
authors: Toledo
authors: Solange Oliveira Rodrigues
authors: SO
authors: Valle
authors: Regis A
authors: RA
authors: Campos
authors: Alfeu T
authors: AT
authors: França
authors: Nieves Prior
authors: NP
authors: Gomez
authors: Heitor Franco
authors: HF
authors: Andrade
authors: Teresa
authors: T
authors: Caballero
authors: Anete S
authors: AS
authors: Grumach
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000503:physiopathology / Q000523:psychology*; D001938:Brazil; D050718:Complement C1 Inhibitor Protein; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012016:Reference Values; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D050718:Complement C1 Inhibitor Protein