Document Detail


Chapter 22: Hereditary and acquired angioedema.
MedLine Citation:
PMID:  22794695     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Hereditary angioedema (HAE) is an autosomal dominant disorder defined by a deficiency of functional C1 esterase inhibitor (C1-INH). Acquired angioedema (AAE) is caused by either consumption (type 1) or inactivation (type 2) of CI-INH. Both HAE and AAE can be life-threatening. The screening test for both conditions is complement component C4, which is low to absent at times of angioedema or during quiescent periods. A useful test to differentiate HAE from AAE is C1q protein, which is normal in HAE and low in AAE. There are three types of HAE: type 1 HAE is most common, occurring in ∼85% of patients and characterized by decreased production of C1-INH, resulting in reduced functional activity to 5-30% of normal. In type 2, which occurs in 15% of cases, C1-INH is detectable in normal or elevated quantities but is dysfunctional. Finally, type 3, which is rare and almost exclusively occurs in women, is estrogen dependent and associated with normal CI-INH and C4 levels. One-third of these patients have a gain-of-function mutation in clotting factor XII leading to kallikrein-driven bradykinin production. Although the anabolic steroid, danazol, is useful in increasing the concentration of C4 and reducing the episodes of angioedema in HAE and AAE, it has expected adverse effects. Fortunately, disease-specific therapies are available and include C1-INH enzyme for i.v. infusion either acutely or empirically, ecallantide, an inhibitor of kallikrein, and icatibant, a bradykinin B2-receptor antagonist, both approved for acute angioedema and administered, subcutaneously.
Authors:
Mary S Georgy; Jacqueline A Pongracic
Related Documents :
17907435 - Neuromuscular disease as the cause of late clubfoot relapses: report of 4 cases.
8104685 - Soluble intercellular adhesion molecule 1 (sicam-1) and malignant melanoma.
18650185 - Association between renal cell carcinoma and plasma cell dyscrasias: a case series of s...
12732085 - Changes in respiratory condition after thymectomy for patients with myasthenia gravis.
10907675 - Electroencephalogram alterations during treatment with olanzapine.
12402665 - Rendu-osler-weber disease: experience with 56 patients.
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Allergy and asthma proceedings : the official journal of regional and state allergy societies     Volume:  33 Suppl 1     ISSN:  1539-6304     ISO Abbreviation:  Allergy Asthma Proc     Publication Date:    2012 May-Jun
Date Detail:
Created Date:  2012-07-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9603640     Medline TA:  Allergy Asthma Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  73-6     Citation Subset:  IM    
Affiliation:
Division of Allergy-Immunology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

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


Previous Document:  Chapter 21: Urticaria and angioedema.
Next Document:  Chapter 23: Food allergy.