Hereditary angioedema: Difference between revisions
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==Diagnosis== | ==Diagnosis== | ||
Hereditary deficiency is characterized by normal levels of [[complement C1q]] and [[complement C1 inhibitor protein]] function.ref name="pmid10644276">{{cite journal |author=Markovic SN, Inwards DJ, Frigas EA, Phyliky RP |title=Acquired C1 esterase inhibitor deficiency |journal=Ann. Intern. Med. |volume=132 |issue=2 |pages=144–50 |year=2000 |month=January |pmid=10644276 |doi= |url=http://www.annals.org/cgi/pmidlookup?view=long&pmid=10644276 |issn=}}</ref> [[complement C1 inhibitor protein]] antigen is low in type I and normal in type II. | Hereditary deficiency is characterized by the absence of [[urticaria]] and by normal levels of [[complement C1q]] and [[complement C1 inhibitor protein]] function.<ref name="pmid10644276">{{cite journal |author=Markovic SN, Inwards DJ, Frigas EA, Phyliky RP |title=Acquired C1 esterase inhibitor deficiency |journal=Ann. Intern. Med. |volume=132 |issue=2 |pages=144–50 |year=2000 |month=January |pmid=10644276 |doi= |url=http://www.annals.org/cgi/pmidlookup?view=long&pmid=10644276 |issn=}}</ref> [[complement C1 inhibitor protein]] antigen is low in type I and normal in type II. | ||
==Treatment== | |||
Options, such as [[complement C1 inhibitor protein]] concentrate, exist for preventing attacks.<ref name="pmid14600687">{{cite journal| author=Maeda S, Miyawaki T, Nomura S, Yagi T, Shimada M| title=Management of oral surgery in patients with hereditary or acquired angioedemas: review and case report. | journal=Oral Surg Oral Med Oral Pathol Oral Radiol Endod | year= 2003 | volume= 96 | issue= 5 | pages= 540-3 | pmid=14600687 | doi=10.1016/S1079210403005018 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14600687 }} </ref> | |||
==References== | ==References== | ||
<references/> | <references/> | ||
Latest revision as of 13:05, 28 March 2011
Hereditary angioedema (hereditary C1 esterase inhibitor deficiency) is an "inherited disorder that is characterized by subcutaneous and submucosal edema in the upper respiratory tract and gastrointestinal tract."[1]
Unregulated kallikrein activation of bradykinin may cause hereditary angioedema:[2]
- Hereditary angioedema types I and II are due to insufficient activity of complement C1 inhibitor protein (C1 esterase inhibitor).[3]
- Hereditary angioedema type III may due to increased activity of coagulation factor XII (Hageman factor).[4][5][6]
Diagnosis
Hereditary deficiency is characterized by the absence of urticaria and by normal levels of complement C1q and complement C1 inhibitor protein function.[7] complement C1 inhibitor protein antigen is low in type I and normal in type II.
Treatment
Options, such as complement C1 inhibitor protein concentrate, exist for preventing attacks.[8]
References
- ↑ Anonymous (2024), Hereditary angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ Schneider L, Lumry W, Vegh A, Williams AH, Schmalbach T (August 2007). "Critical role of kallikrein in hereditary angioedema pathogenesis: a clinical trial of ecallantide, a novel kallikrein inhibitor". J. Allergy Clin. Immunol. 120 (2): 416–22. DOI:10.1016/j.jaci.2007.04.028. PMID 17559913. Research Blogging.
- ↑ Hereditary angioedema. (Online Mendelian Inheritance in Man, OMIM®. Johns Hopkins University, Baltimore, MD. MIM Number: 106100. World Wide Web URL: http://omim.org/.)
- ↑ Hereditary angioedema, type III. (Online Mendelian Inheritance in Man, OMIM®. Johns Hopkins University, Baltimore, MD. MIM Number: 610618. World Wide Web URL: http://omim.org/.)
- ↑ Cichon S, Martin L, Hennies HC, et al (December 2006). "Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III". Am. J. Hum. Genet. 79 (6): 1098–104. DOI:10.1086/509899. PMID 17186468. PMC 1698720. Research Blogging.
- ↑ Angioedema, hereditary. Online Mendelian Inheritance in Man. Retrieved on 2008-06-16.
- ↑ Markovic SN, Inwards DJ, Frigas EA, Phyliky RP (January 2000). "Acquired C1 esterase inhibitor deficiency". Ann. Intern. Med. 132 (2): 144–50. PMID 10644276. [e]
- ↑ Maeda S, Miyawaki T, Nomura S, Yagi T, Shimada M (2003). "Management of oral surgery in patients with hereditary or acquired angioedemas: review and case report.". Oral Surg Oral Med Oral Pathol Oral Radiol Endod 96 (5): 540-3. DOI:10.1016/S1079210403005018. PMID 14600687. Research Blogging.