title: Hereditary angioedema in Greece: the first results of the greek hereditary angioedema registry.
abstract: BACKGROUND
  No published data presently exist concerning hereditary angioedema (HAE) in Greece. The aim of this study was to present the results from patients recorded by the Greek Hereditary Angioedema Registry over the last 3 years (July 2010 to June 2013).
  
  
  METHODS
  A systematic recording of HAE cases was undertaken following a physician awareness campaign and confirmation of diagnosis. A questionnaire was also used for the assessment of key parameters of the patients' disease-specific quality of life.
  
  
  RESULTS
  One hundred and sixteen patients from 41 non-related families were recorded. There were 33 (80.5%) families with type I HAE, 7 (17%) with type II HAE and 1 (2.5%) with non-C1 inhibitor (C1-INH), non-FXII HAE. Two further non-C1-INH, non-FXII HAE sporadic cases were recorded. An investigation of non-symptomatic family members revealed another 6 asymptomatic individuals with C1-INH deficiency. The average delay in diagnosis was 16.5 years and the incidence of death in the families of patients was 1 for every 2 families. The use of newer therapeutic agents seems to fall significantly short of the existing needs. HAE was found to affect the quality of life slightly in 14%, greatly in 63% and significantly in 23% of the patients.
  
  
  CONCLUSIONS
  Until recently, there has been a significant degree of underdiagnosis of HAE in Greece. Very low compliance with the provisions of the applicable international guidelines and consensus positions, with adverse consequences on the patients' quality of life, was also observed. The centralized model we used to uncover the patients could be effective in other countries presenting with comparable disease characteristics.
authors.affiliation: Allergy Department, Naval Hospital of Athens, Athens, Greece.
authors.forename: Fotis
authors.initials: F
authors.lastname: Psarros
authors.forename: Nick
authors.initials: N
authors.lastname: Koutsostathis
authors.forename: Evangelia
authors.initials: E
authors.lastname: Farmaki
authors.forename: Matthaios G
authors.initials: MG
authors.lastname: Speletas
authors.forename: Anastasios E
authors.initials: AE
authors.lastname: Germenis
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001920:Bradykinin / Q000031:analogs & derivatives / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003613:Danazol / Q000627:therapeutic use; D004965:Estrogen Antagonists / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006115:Greece / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012042:Registries; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D004965:Estrogen Antagonists; C065679:icatibant; D003613:Danazol; D001920:Bradykinin