The Complex Interaction Between Polycystic Ovary Syndrome and Hereditary Angioedema: Case Reports and Review of the Literature
| Autor: | Jorge Kalil, Pedro Giavina-Bianchi, Edmund Chada Baracat, Gustavo Arantes Rosa Maciel, Marina Iahn-Aun, Antonio Abilio Motta, Sylvia Asaka Yamashita Hayashida, Marcelo Vivolo Aun |
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| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Abdominal pain medicine.medical_specialty Adolescent medicine.drug_class Mutation Missense Asymptomatic C1-inhibitor Diagnosis Differential 03 medical and health sciences 0302 clinical medicine Internal medicine medicine Humans Ultrasonography biology Angioedema business.industry Hyperandrogenism Ovary Angioedemas Hereditary Obstetrics and Gynecology Estrogens General Medicine medicine.disease Polycystic ovary Dermatology Abdominal Pain Oligomenorrhea 030104 developmental biology Endocrinology 030228 respiratory system Estrogen Hereditary angioedema biology.protein Female medicine.symptom business Complement C1 Inhibitor Protein Polycystic Ovary Syndrome |
| Zdroj: | Obstetricalgynecological survey. 72(7) |
| ISSN: | 1533-9866 |
| Popis: | Importance Hereditary angioedema (HAE) is a rare but severe disease, with high risk of death, and attacks have been associated to high estrogen levels. Polycystic ovary syndrome (PCOS) is a common hyperandrogenic condition, which is frequently treated with combined oral contraceptives. Objective The aim of this study was to describe 2 clinical cases of young women diagnosed as having PCOS who developed HAE attacks after the introduction of combined estrogen-progestin pills to treat PCOS symptoms. Evidence acquisition Literature review of sex hormones' role in genesis of HAE attacks and possible mechanisms involved. Results In the cases reported, after initiation of combined contraceptives, patients presented with facial swelling with airway involvement (laryngeal edema) and abdominal pain. They had a familial history of angioedema and normal C1 inhibitor (C1-INH) levels, leading to the diagnosis of HAE with normal C1-INH (HAEnC1-INH) or HAE type III. After suspension of exogenous estrogen, patients remained asymptomatic from HAE. Conclusions and relevance HAEnC1-INH is an estrogen-dependent form of HAE. It is well established that exogenous estrogen triggers attacks of all types of HAE. However, this is the first description of the association between PCOS and HAE, in which PCOS could be masking HAE symptoms. We propose that PCOS might have a protective role regarding HAE attacks, because of its particular hormonal features, that is, hyperandrogenism and relative stable levels of estradiol. The use of combined estrogen-progestin compounds in women with PCOS and HAE must be avoided, and treatment must be individualized. |
| Databáze: | OpenAIRE |
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