Document Detail


Successful management with C1-inhibitor concentrate of hereditary angioedema attacks during two successive pregnancies: a case report.
MedLine Citation:
PMID:  17653743     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Cedric Hermans
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2007-02-15
Journal Detail:
Title:  Archives of gynecology and obstetrics     Volume:  276     ISSN:  0932-0067     ISO Abbreviation:  Arch. Gynecol. Obstet.     Publication Date:  2007 Sep 
Date Detail:
Created Date:  2007-07-26     Completed Date:  2008-03-25     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  8710213     Medline TA:  Arch Gynecol Obstet     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  271-6     Citation Subset:  IM    
Affiliation:
Haemostasis and Thrombosis Unit, Department of Haematology, Cliniques Universitaires Saint-Luc, Catholic University of Louvain, Avenue Hippocrate 10, 1200, Brussels, Belgium. hermans@sang.ucl.ac.be
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MeSH Terms
Descriptor/Qualifier:
Adult
Angioedemas, 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
Chemical
Reg. No./Substance:
0/Complement C1 Inactivator Proteins; 0/SERPING1 protein, human; 0/Serpins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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