Recent advances in the management of hereditary angioedema

Stephen E. Hemperly, Niti Sardana Agarwal, Ying Yang Xu, Yu Xiang Zhi, Timothy J. Craig

Research output: Contribution to journalReview article

4 Citations (Scopus)

Abstract

Hereditary angioedema (HAE) is a rare genetic condition that manifests as painful and potentially life-threatening episodic attacks of cutaneous and submucosal swelling. It results from functional deficiency of C1 inhibitor (C1 INH), which is a regulator of the complement, fibrinolytic, kinin (contact), and coagulation systems. In patients with HAE, the low plasma concentration of functional C1 INH leads to overactivation of the kinin cascade and local release of bradykinin. Bradykinin is responsible for the pain, vascular permeability changes, and edema associated with HAE. Until recently, therapeutic options for HAE have been very limited. Many new therapies have emerged, however, such as C1 INH replacement drugs and medications aimed at components of the contact system (eg, plasma kallikrein inhibitor and bradykinin B2 receptor antagonist). The authors review current and novel treatments for patients with HAE.

Original languageEnglish (US)
Pages (from-to)546-555
Number of pages10
JournalJournal of the American Osteopathic Association
Volume113
Issue number7
DOIs
StatePublished - Jul 30 2013

Fingerprint

Hereditary Angioedemas
Kinins
Bradykinin
Plasma Kallikrein
Capillary Permeability
Edema
Therapeutics
Pain
Skin
Pharmaceutical Preparations

All Science Journal Classification (ASJC) codes

  • Complementary and alternative medicine

Cite this

Hemperly, Stephen E. ; Sardana Agarwal, Niti ; Xu, Ying Yang ; Zhi, Yu Xiang ; Craig, Timothy J. / Recent advances in the management of hereditary angioedema. In: Journal of the American Osteopathic Association. 2013 ; Vol. 113, No. 7. pp. 546-555.
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Recent advances in the management of hereditary angioedema. / Hemperly, Stephen E.; Sardana Agarwal, Niti; Xu, Ying Yang; Zhi, Yu Xiang; Craig, Timothy J.

In: Journal of the American Osteopathic Association, Vol. 113, No. 7, 30.07.2013, p. 546-555.

Research output: Contribution to journalReview article

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AU - Hemperly, Stephen E.

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AB - Hereditary angioedema (HAE) is a rare genetic condition that manifests as painful and potentially life-threatening episodic attacks of cutaneous and submucosal swelling. It results from functional deficiency of C1 inhibitor (C1 INH), which is a regulator of the complement, fibrinolytic, kinin (contact), and coagulation systems. In patients with HAE, the low plasma concentration of functional C1 INH leads to overactivation of the kinin cascade and local release of bradykinin. Bradykinin is responsible for the pain, vascular permeability changes, and edema associated with HAE. Until recently, therapeutic options for HAE have been very limited. Many new therapies have emerged, however, such as C1 INH replacement drugs and medications aimed at components of the contact system (eg, plasma kallikrein inhibitor and bradykinin B2 receptor antagonist). The authors review current and novel treatments for patients with HAE.

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