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Hereditary Angioedema in Swedish Adults: Report From the National Cohort
Linköping University, Department of Clinical and Experimental Medicine. Linköping University, Faculty of Medicine and Health Sciences. Region Östergötland, Local Health Care Services in East Östergötland. County Hospital Ryhov, Sweden.
Futurum, Sweden.
Linköping University, Department of Clinical and Experimental Medicine, Division of Neuro and Inflammation Science. Linköping University, Faculty of Medicine and Health Sciences. Futurum, Sweden.
Karolinska Institute, Sweden.
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2016 (English)In: Acta Dermato-Venereologica, ISSN 0001-5555, E-ISSN 1651-2057, Vol. 96, no 4, 540-545 p.Article in journal (Refereed) PublishedText
Abstract [en]

Hereditary angioedema (HAE) is rare, disabling and sometimes life-threatening. The aim of this study is to describe its prevalence, symptomatology and treatment in Sweden. A total of 146 patients were identified; 110 adults and 36 children with HAE type I (n = 136) or II (n = 10), giving a minimum HAE prevalence of 1.54/100,000. All patients received a written questionnaire followed by a structured telephone interview. This report focuses on the 102 adults who responded. Females reported 19 attacks in the previous year vs. 9 for males (p < 0.01), and females reported 10 days of sick leave vs. 4 days for males (p < 0.05). For all treated acute attacks, plasma-derived Cl-inhibitor concentrate (pdClINH) (used in 27% of patients) had a good effect. For maintenance treatment, 43% used attenuated androgens and 8% used pdClINH, which reduced their attack rate by more than 50%. In conclusion, the minimum HAE prevalence in Sweden was 1.54/100,000. HAE affected females more severely. Attenuated androgens and pdClINH had a good effect on preventing attacks.

Place, publisher, year, edition, pages
ACTA DERMATO-VENEREOLOGICA , 2016. Vol. 96, no 4, 540-545 p.
Keyword [en]
Clinhibitor deficiency; census; clinical manifestations; epidemiology; hereditary angioedema; prevalence; Sweden
National Category
Health Sciences
URN: urn:nbn:se:liu:diva-128976DOI: 10.2340/00015555-2274ISI: 000375741300022PubMedID: 26540175OAI: diva2:934807

Funding Agencies|Futurum the Academy for Healthcare; Jonkoping County Council; Linkoping University; Karolinska Institutet

Available from: 2016-06-09 Created: 2016-06-07 Last updated: 2016-06-28

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Nordenfelt, PatrikBjörkander, Jan Fredrik
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Department of Clinical and Experimental MedicineFaculty of Medicine and Health SciencesLocal Health Care Services in East ÖstergötlandDivision of Neuro and Inflammation Science
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