title: Swedish children with hereditary angioedema report good overall health and quality of life despite symptoms.
abstract: OBJECTIVE
  Few studies have been published on children with hereditary angioedema (HAE), an autosomal dominant disease caused by mutations on chromosome 11. This study explored various aspects of the disease in the Swedish paediatric population.
  
  
  METHODS
  A retrospective questionnaire was sent to all 36 Swedish children known to have HAE, and a physician carried out follow-up telephone interviews.
  
  
  RESULTS
  Most of the questionnaires were completed by the parents of 31 (86%) children with HAE, with or without their input, at a median age of nine years (range 1-17), and the physician also interviewed 29. HAE symptoms were experienced by 23 children, including abdominal attacks (96%), skin swelling (78%) and swelling in the mouth and/or upper airways (52%). Psychological stress was the most common trigger for abdominal attacks and trauma and sports triggered skin swelling. The majority (n = 19) had access to complement-1 esterase inhibitor concentrate at home. Current health and quality of life were generally rated as good, independent of whether the child had experienced HAE symptoms or not.
  
  
  CONCLUSIONS
  Most children with HAE had experienced abdominal attacks and skin swelling, but their overall health and quality of life were generally perceived to be good.
authors.affiliation: Dermatology Unit, Department of Medicine Solna, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Anders
authors.initials: A
authors.lastname: Nygren
authors.affiliation: Department of Clinical and Experimental Medicine, University of Linköping, Linköping, Sweden.|Department of Internal Medicine, County Hospital of Ryhov, Jönköping, Sweden.
authors.forename: Patrik
authors.initials: P
authors.lastname: Nordenfelt
authors.affiliation: Department of Paediatrics, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.|Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Anders
authors.initials: A
authors.lastname: Lindfors
authors.affiliation: Dermatology Unit, Department of Medicine Solna, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Lotus
authors.initials: L
authors.lastname: Mallbris
authors.affiliation: Department of Clinical and Experimental Medicine, University of Linköping, Linköping, Sweden.|Futurum - Academy for Health and Care, Region Jönköping County, Jönköping, Sweden.
authors.forename: Janne
authors.initials: J
authors.lastname: Björkander
authors.affiliation: Dermatology Unit, Department of Medicine Solna, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Carl-Fredrik
authors.initials: CF
authors.lastname: Wahlgren
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000188:drug therapy / Q000523:psychology; D002648:Child; D002675:Child, Preschool; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use; D051056:Complement Inactivating Agents / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006305:Health Status Indicators; D006801:Humans; D007223:Infant; D008297:Male; D011788:Quality of Life / Q000523:psychology; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D013548:Sweden
chemical_list: D050718:Complement C1 Inhibitor Protein; D051056:Complement Inactivating Agents
keywords: Children; Complement-1 esterase inhibitor defects; Epidemiology; Hereditary angioedema; Symptoms