1. Hereditary Angioedema in Swedish Adults: Report From the National Cohort
- Author
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Mats Nilsson, Patrik Nordenfelt, Jan Fredrik Björkander, Lotus Mallbris, Anders Lindfors, and Carl-Fredrik Wahlgren
- Subjects
Adult ,Male ,Pediatrics ,medicine.medical_specialty ,Time Factors ,Attack rate ,Dermatology ,Complement C1 Inactivator Proteins ,National cohort ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Health Sciences ,Prevalence ,Humans ,Medicine ,Registries ,Aged ,Retrospective Studies ,Aged, 80 and over ,Sweden ,business.industry ,Clinhibitor deficiency ,census ,clinical manifestations ,epidemiology ,hereditary angioedema ,prevalence ,Remission Induction ,Angioedemas, Hereditary ,Retrospective cohort study ,General Medicine ,Hälsovetenskaper ,Middle Aged ,medicine.disease ,Complement Inactivating Agents ,Treatment Outcome ,030228 respiratory system ,Telephone interview ,Health Care Surveys ,Hereditary angioedema ,Sick leave ,Androgens ,Female ,Sick Leave ,business ,Complement C1 Inhibitor Protein ,Biomedical sciences - Abstract
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. Funding Agencies|Futurum the Academy for Healthcare; Jonkoping County Council; Linkoping University; Karolinska Institutet
- Published
- 2016
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