Hereditary angioedema with an estrogen trigger in a 12-year-old

Mowat, Alex E. and Kimble, Rebecca (2014) Hereditary angioedema with an estrogen trigger in a 12-year-old. Journal of Pediatric and Adolescent Gynecology, 27 6: e131-e132. doi:10.1016/j.jpag.2013.10.007

Author Mowat, Alex E.
Kimble, Rebecca
Title Hereditary angioedema with an estrogen trigger in a 12-year-old
Journal name Journal of Pediatric and Adolescent Gynecology   Check publisher's open access policy
ISSN 1873-4332
Publication date 2014-12
Year available 2014
Sub-type Article (original research)
DOI 10.1016/j.jpag.2013.10.007
Open Access Status
Volume 27
Issue 6
Start page e131
End page e132
Total pages 2
Place of publication Philadelphia, PA, United States
Publisher Elsevier
Collection year 2015
Language eng
Formatted abstract
Hereditary angioedema (HAE) is rare autosomal dominant genetic disorder, commonly affecting girls around the menarche, which manifests clinically as recurrent episodes of angioedema. Laryngeal edema can lead to asphyxiation and death. Traditionally hormones have been avoided in the management due a reported association with flares in the literature. This case describes an alternative management with a progestin.

A 12 year old HAE sufferer failed to receive relief from her symptoms of angioedema with standard treatment. A trial of depot medroxyprogesterone acetate has resulted in resolution of her symptoms for the last 14 months.

Although estrogens and progestins have been avoided in the management of HAE in the past, the reasons for this are based only on a small number of case reports. In this case, successful treatment with depot medroxyprogesterone acetate indicates that progestins, as an alternative management for estrogen-triggered HAE, certainly warrants further research.
Keyword Hereditary angioedema
Depot medroxyprogesterone acetate
Q-Index Code CX
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Non HERDC
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