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Successful management with C1-inhibitor concentrate of hereditary angioedema attacks during two successive pregnancies: a case report.

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Hermans, Cédric [UCL]

BACKGROUND: [corrected] Hereditary angioedema (HAE) is a rare genetic disorder caused by a deficiency of the plasma protein C1 inhibitor (C1-INH). HAE is characterised by the onset of angioedema, which may develop in one or several organs, and may last from a few hours to several days. Oedema of the upper airway can be life-threatening. As a result of hormonal changes, some women experience more frequent angioedema attacks during pregnancy. During pregnancy, antifibrinolytic agents should only be used with caution, and attenuated androgens are contraindicated; therefore, replacement therapy with C1-INH concentrate represents one of few therapeutic options, but it is not widely documented. CASE STUDY: We report the first case study of the successful management with regular infusions of C1-INH concentrate, of two successive pregnancies in a patient with HAE. During the second half of the first pregnancy, C1-INH was administered on demand at home. For the second pregnancy, on demand treatment was intensified to prophylactic therapy, with once or twice weekly infusions from the middle of the second trimester in order to efficiently control the frequent attacks. CONCLUSIONS: This report illustrates that HAE can be successfully managed during pregnancy with C1-INH infusions at home. Since the number of crises may vary between pregnancies, the treatment regimen must be adapted to the patient's need.

Document type Article de périodique (Journal article) – Case Reports, Journal Article
Access type Accès restreint
Publication date 2007
Journal information "Archives of gynecology and obstetrics" - Vol. 276, no. 3, p. 271-6 (2007)
Peer reviewed yes
issn 0932-0067
Publication status Publié
Affiliation UCL - MD/MINT - Département de médecine interne
MESH Subject Adult ; Angioedema, Hereditary - drug therapy ; Complement C1 Inactivator Proteins - therapeutic use ; Female ; Humans ; Infant, Newborn ; Male ; Pregnancy ; Pregnancy Complications, Hematologic - drug therapy ; Pregnancy Outcome ; Recurrence ; Reproductive History ; Serpins - therapeutic use ; Treatment Outcome
Links
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Bibliographic reference Hermans, Cédric. Successful management with C1-inhibitor concentrate of hereditary angioedema attacks during two successive pregnancies: a case report.. In: Archives of gynecology and obstetrics, Vol. 276, no. 3, p. 271-6 (2007)
Permanent URL http://hdl.handle.net/2078.1/23104