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Recombinant human C1 esterase inhibitor for the treatment of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE).
Expert Rev Clin Immunol 2015; 11(3):319-27ER

Abstract

The lack of C1 inhibitor function that results in excessive production of bradykinin causing the angioedema seen in hereditary angioedema (HAE) is well established. Several drugs have been developed to treat and prevent attacks in patients suffering from HAE due to C1 inhibitor deficiency (C1-INH-HAE). Plasma-derived C1INH has been used to replace the deficiency of C1 inhibitor (C1INH) and has been approved for both treatment of attacks and for prophylactic therapy to prevent attacks. Plasma kallikrein inhibitor (ecallantide) and bradykinin receptor antagonist (icatibant) are both effective for treatment of acute attacks, but their short half-life limits the use for prophylaxis. Androgens, in particular danazol, are effective for long-term prophylaxis, but adverse event profile can limit its use. Recombinant C1 inhibitor derived from transgenic rabbits has recently been approved for use in treatment of C1-INH-HAE attacks and is effective and appears safe with minimal adverse event profile.

Authors+Show Affiliations

Department of Pediatrics, Penn State University, 500 University Drive, 17033 Hershey, USA.No affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

25669442

Citation

Sabharwal, Geetika, and Timothy Craig. "Recombinant Human C1 Esterase Inhibitor for the Treatment of Hereditary Angioedema Due to C1 Inhibitor Deficiency (C1-INH-HAE)." Expert Review of Clinical Immunology, vol. 11, no. 3, 2015, pp. 319-27.
Sabharwal G, Craig T. Recombinant human C1 esterase inhibitor for the treatment of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE). Expert Rev Clin Immunol. 2015;11(3):319-27.
Sabharwal, G., & Craig, T. (2015). Recombinant human C1 esterase inhibitor for the treatment of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE). Expert Review of Clinical Immunology, 11(3), pp. 319-27. doi:10.1586/1744666X.2015.1012502.
Sabharwal G, Craig T. Recombinant Human C1 Esterase Inhibitor for the Treatment of Hereditary Angioedema Due to C1 Inhibitor Deficiency (C1-INH-HAE). Expert Rev Clin Immunol. 2015;11(3):319-27. PubMed PMID: 25669442.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Recombinant human C1 esterase inhibitor for the treatment of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE). AU - Sabharwal,Geetika, AU - Craig,Timothy, Y1 - 2015/02/10/ PY - 2015/2/12/entrez PY - 2015/2/12/pubmed PY - 2015/11/18/medline KW - C1 inhibitor KW - C1-esterase inhibitor KW - hereditary angioedema KW - recombinant KW - recombinant C1 inhibitor KW - treatment SP - 319 EP - 27 JF - Expert review of clinical immunology JO - Expert Rev Clin Immunol VL - 11 IS - 3 N2 - The lack of C1 inhibitor function that results in excessive production of bradykinin causing the angioedema seen in hereditary angioedema (HAE) is well established. Several drugs have been developed to treat and prevent attacks in patients suffering from HAE due to C1 inhibitor deficiency (C1-INH-HAE). Plasma-derived C1INH has been used to replace the deficiency of C1 inhibitor (C1INH) and has been approved for both treatment of attacks and for prophylactic therapy to prevent attacks. Plasma kallikrein inhibitor (ecallantide) and bradykinin receptor antagonist (icatibant) are both effective for treatment of acute attacks, but their short half-life limits the use for prophylaxis. Androgens, in particular danazol, are effective for long-term prophylaxis, but adverse event profile can limit its use. Recombinant C1 inhibitor derived from transgenic rabbits has recently been approved for use in treatment of C1-INH-HAE attacks and is effective and appears safe with minimal adverse event profile. SN - 1744-8409 UR - https://www.unboundmedicine.com/medline/citation/25669442/Recombinant_human_C1_esterase_inhibitor_for_the_treatment_of_hereditary_angioedema_due_to_C1_inhibitor_deficiency__C1_INH_HAE__ L2 - http://www.tandfonline.com/doi/full/10.1586/1744666X.2015.1012502 DB - PRIME DP - Unbound Medicine ER -