ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume: 14 Issue: 2 Year: 2022
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Abstracting & Indexing
Turkish Society for Pediatric Endocrinology and Diabetes
A Possibly Fatal Outcome of Oral Contraceptive Therapy: Estrogen Triggered Hereditary Angioedema Attack in An Adolescent [J Clin Res Pediatr Endocrinol]
J Clin Res Pediatr Endocrinol. Ahead of Print: JCRPE-96636 | DOI: 10.4274/jcrpe.galenos.2021.2021.0053

A Possibly Fatal Outcome of Oral Contraceptive Therapy: Estrogen Triggered Hereditary Angioedema Attack in An Adolescent

Ugur Berkay Balkanci1, Demet Demirkol2, Gul Yesiltepe Mutlu4, Esra Birben5, Ozge Soyer5, Ozlem Yilmaz6, Cansin Sackesen
1Koc University, School of Medicine, Istanbul, Turkey
2Koc University, School of Medicine, Division of Pediatric Intensive Care, Davutpasa Cad. Istanbul, Turkey
3Istanbul University, Istanbul School of Medicine, Division of Pediatric Intensive Care, Istanbul, Turkey; and
4Koc University, School of Medicine, Division of Pediatric Endocrinology, Istanbul, Turkey
5Hacettepe University, School of Medicine, Division of Pediatric Allergy, Ankara, Turkey
6Koc University, School of Medicine, Division of Pediatric Allergy, Istanbul, Turkey

Hereditary angioedema (HAE) is characterized by recurrent angioedema attacks with no urticaria. This disease has a high mortality due to asphyxia. Level of complement 4 (C4), C1 esterase inhibitor (C1-INH) level and function, and genetic mutations determine different endotypes of HAE. Clinical presentation and the triggers of vasogenic edema may change according to the endotypes. An adolescent girl with oligomenorrhea, obesity, hirsutism, and acanthosis nigricans was diagnosed with polycystic ovary syndrome (PCOS) and prescribed ethinyl estradiol & cyproterone acetate containing oral contraceptive (OC). On the 16th day of treatment, she developed angioedema on the face, neck, and chest leading to dyspnea. Adrenaline, antihistamine, and corticosteroid treatments were ineffective. In the family history, the patient’s mother and two cousins had angioedema attacks. C1-INH concentrate was administered with a diagnosis of HAE. C4, and C1-INH level and activity were normal. Genetic analysis identified a mutation of Factor XII (F12) gene, and the diagnosis of Factor 12 (F12)-
related HAE was made. OC treatment discontinued. She has had no additional angioedema attacks in the follow-up period of two years. OC containing estrogen may induce the life-threatening first attack of F12-related HAE even in children. Recurring angioedema attacks in the family should be asked before prescribing estrogen-containing OC pills.

Keywords: Hereditary Angioedema Type III, Hereditary Angioedema, Angioedema, Factor XII, Polycystic Ovary Syndrome



Corresponding Author: Cansin Sackesen, Türkiye
Manuscript Language: English
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