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Reviews

Current treatment options for hereditary angioedema due to C1 inhibitor deficiency

Maddalena Alessandra WuDepartment of Biomedical and Clinical Sciences “Luigi Sacco”, Luigi Sacco Hospital, University of Milan, Milan, Italy
,
Andrea ZanichelliDepartment of Biomedical and Clinical Sciences “Luigi Sacco”, Luigi Sacco Hospital, University of Milan, Milan, Italy
,
Marta MansiDepartment of Biomedical and Clinical Sciences “Luigi Sacco”, Luigi Sacco Hospital, University of Milan, Milan, Italy
&
Marco CicardiDepartment of Biomedical and Clinical Sciences “Luigi Sacco”, Luigi Sacco Hospital, University of Milan, Milan, ItalyCorrespondence[email protected]
Pages 27-40 | Published online: 29 Oct 2015

Bibliography

  • Papers of special note have been highlighted as either of importance (*) or of considerable importance (**) to readers.
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** It is the first classification of angioedema

** A comprehensive review of the topic

  • Bafunno V, Bova M, Loffredo S, et al. Mutational spectrum of the c1 inhibitor gene in a cohort of Italian patients with hereditary angioedema: description of nine novel mutations. Ann Hum Genet. 2014;78:73–82.
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* A review providing deep insights into the physiopathology of C1-INH

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* First evidence of presence of bradykinin in plasma during angioedema

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* A comprehensive review on the interaction among these systems

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* Evidence of plasma bradykinin in different forms of angioedema

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** First therapeutic guidelines based on evidence

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** DBPC trial for efficacy of pdC1-INH in acute attacks

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** DBPC trial for efficacy of pdC1-INH in acute attacks and prophylaxis

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** DBPC trial for efficacy of recombinant C1-INH in acute attacks

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** DBPC trial for efficacy of icatibant in acute attacks

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  • Cicardi M, Levy RJ, McNeil DL, et al. Ecallantide for the treatment of acute attacks in hereditary angioedema. N Engl J Med. 2010;363:523–531.

** DBPC trial for efficacy of ecallantide in acute attacks

  • Craig TJ, Li HH, Riedl M, et al. Characterization of anaphylaxis after ecallantide treatment of hereditary angioedema attacks. J Allergy Clin Immunol Pract. 2015;3:206–12.e4.
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* Guidelines on HAE

* Guidelines on HAE

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** Evidence of clinical and biochemical efficacy of danazol

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