Acquired C1 Esterase Inhibitor Deficiency
Authors: Allen P. Kaplan
Journal: Annals of Internal Medicine (2000)

Letters21 November 2000Acquired C1 Esterase Inhibitor DeficiencyAllen P. Kaplan, MDAllen P. Kaplan, MDMedical University of South Carolina; Charleston, SC 29425 (Kaplan)Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-133-10-200011210-00023 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail TO THE EDITOR:The review on acquired C1 esterase inhibitor deficiency (1) reports that C2 kinin is the cause of the swelling. This peptide was originally described as a plasmin-cleavage product of C2b, and a synthetic peptide appears to possess such activity (2). However, it has not been possible to activate human plasma that is deficient in C1 inhibitor to yield a kinin other than bradykinin (3, 4), and bradykinin levels are elevated in plasma of patients with hereditary angioedema during attacks of swelling. Furthermore, swelling has never occurred in members of one unique family with a dysfunctional C1 inhibitor ...References1. Markovic SN, Inwards DJ, Frigas EA, Phyliky RP. Acquired C1 esterase inhibitor deficiency. Ann Intern Med. 2000;132:144-50. LinkGoogle Scholar2. Strang CJ, Cholin S, Spragg J, Davis AE, Schneeberger E, Donaldson VH, et al . Angioedema induced by a peptide derived from complement component C2. J Exp Med. 1988;168:1685-98. CrossrefMedlineGoogle Scholar3. Fields T, Ghebrehiwet B, Kaplan AP. Kinin formation in hereditary angioedema plasma: evidence against kinin derivation from C2 and in support of “spontaneous” formation of bradykinin. J Allergy Clin Immunol. 1983;72:54-60. CrossrefMedlineGoogle Scholar4. Nussberger J, Cugno M, Amstutz C, Cicardi M, Pellacani A, Agostoni A. Plasma bradykinin in angio-oedema. Lancet. 1998;351:1693-7. CrossrefMedlineGoogle Scholar5. Zahedi R, Wisnieski J, Davis AE. Role of the P2 residue of complement 1 inhibitor (Ala443) in determination of target protease specificity. Inhibition of complement and contact system proteases. J Immunol. 1997;159:983-8. MedlineGoogle Scholar Author, Article, and Disclosure InformationAuthors: Allen P. Kaplan, MDAffiliations: Medical University of South Carolina; Charleston, SC 29425 (Kaplan) PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoAcquired C1 Esterase Inhibitor Deficiency Svetomir N. Markovic , David J. Inwards , Evangelos A. Frigas , and Robert P. Phyliky Acquired C1 Esterase Inhibitor Deficiency Antal Csepregi and Elemér Nemesánszky Acquired C1 Esterase Inhibitor Deficiency Svetomir N. Markovic , David J. Inwards , and Robert P. Phyliky Acquired C1 Esterase Inhibitor Deficiency Teresa Caballero , Maria Concepcion Lopez-Serrano , and Margarita Lopez-Trascasa Acquired C1 Esterase Inhibitor Deficiency Thomas Grünewald Acquired C1 Esterase Inhibitor Deficiency Marco Cicardi , Angelo Agostoni , and Alvin E. Davis III Metrics Cited byProtease inhibitors in the treatment of hereditary angioedema 21 November 2000Volume 133, Issue 10Page: 838KeywordsBlood plasmaEsterasesHereditary angioedemaInflammationPlasma proteinsPlasmins ePublished: 21 November 2000 Issue Published: 21 November 2000 Copyright & PermissionsCopyright © 2000 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...