We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Treatment for hereditary angioedema with normal C1- INH and specific mutations in the F12 gene ( HAE- FXII).
- Authors
Bork, K.; Wulff, K.; Witzke, G.; Hardt, J.
- Abstract
Hereditary angioedema with normal C1 esterase inhibitor and mutations in the F12 gene ( HAE- FXII) is associated with skin swellings, abdominal pain attacks, and the risk of asphyxiation due to upper airway obstruction. It occurs nearly exclusively in women. We report our experience treating HAE- FXII with discontinuation of potential trigger factors and drug therapies. The study included 72 patients with HAE- FXII. Potential triggers included estrogen-containing oral contraceptives ( eOC), hormonal replacement therapy, or angiotensin-converting enzyme inhibitors. Drug treatment comprised plasma-derived C1 inhibitor (pdC1- INH) for acute swelling attacks and progestins, tranexamic acid, and danazol for the prevention of attacks. Discontinuation of eOC was effective in 25 (89.3%) of 28 women and led to a reduction in the number of attacks (about 90%). After ending hormonal replacement therapy, three of eight women became symptom-free. Three women with exacerbation of HAE- FXII during intake of quinapril or enalapril had no further HAE- FXII attacks after discontinuation of those drugs. Eleven women were treated with pdC1- INH for 143 facial attacks. The duration of the treated facial attacks (mean: 26.6 h; SD: 10.1 h) was significantly shorter than that of the previous 88 untreated facial attacks in the same women (mean: 64.1 h; SD: 28.0 h; P < 0.01). The mean reduction in attack frequency was 99.8% under progestins after discontinuing eOC (16 women), 93.8% under tranexamic acid (four women), and 100% under danazol (three women). For patients with HAE- FXII, various treatment options are available which completely or at least partially reduce the number or duration of attacks.
- Subjects
ANGIONEUROTIC edema; DRUG therapy; THERAPEUTIC use of progestational hormones; TRANEXAMIC acid; DANAZOL; HORMONE therapy; THERAPEUTICS
- Publication
Allergy, 2017, Vol 72, Issue 2, p320
- ISSN
0105-4538
- Publication type
Article
- DOI
10.1111/all.13076