Therapeutic management of hereditary angioedema due to C1 inhibitor deficiency. 2013

Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
Dipartimento di Scienze Biomediche e Cliniche Luigi Sacco, Università degli Studi di Milano, Ospedale Luigi Sacco, Milan 20157, Italy. andrea.zanichelli@unimi.it

Hereditary angioedema (HAE) due to C1 inhibitor (C1-INH) deficiency is a rare genetic disease characterized by recurrent swellings of the subcutaneous and submucosal tissues that can manifest as cutaneous edema, abdominal pain and laryngeal edema with airway obstruction. These symptoms have a significant impact on patients' quality of life. The reduction in C1-INH function leads to uncontrolled activation of the contact system and generation of bradykinin, the mediator of increased vascular permeability and edema formation. In the past, few treatment options were available; however, several new therapies with proven efficacy have recently become available to treat and prevent HAE attacks, such as plasma-derived and recombinant C1-INHs that replace the deficient protein, bradykinin receptor antagonist (icatibant) that blocks bradykinin activity and kallikrein inhibitor (ecallantide) that prevents bradykinin release. Such therapies can improve disease outcome. This article reviews the therapeutic management of HAE, which involves the treatment of acute attacks and prophylaxis.

UI MeSH Term Description Entries
D010455 Peptides Members of the class of compounds composed of AMINO ACIDS joined together by peptide bonds between adjacent amino acids into linear, branched or cyclical structures. OLIGOPEPTIDES are composed of approximately 2-12 amino acids. Polypeptides are composed of approximately 13 or more amino acids. PROTEINS are considered to be larger versions of peptides that can form into complex structures such as ENZYMES and RECEPTORS. Peptide,Polypeptide,Polypeptides
D001920 Bradykinin A nonapeptide messenger that is enzymatically produced from KALLIDIN in the blood where it is a potent but short-lived agent of arteriolar dilation and increased capillary permeability. Bradykinin is also released from MAST CELLS during asthma attacks, from gut walls as a gastrointestinal vasodilator, from damaged tissues as a pain signal, and may be a neurotransmitter. Arg-Pro-Pro-Gly-Phe-Ser-Pro-Phe-Arg,Bradykinin Acetate, (9-D-Arg)-Isomer,Bradykinin Diacetate,Bradykinin Hydrochloride,Bradykinin Triacetate,Bradykinin, (1-D-Arg)-Isomer,Bradykinin, (2-D-Pro)-Isomer,Bradykinin, (2-D-Pro-3-D-Pro-7-D-Pro)-Isomer,Bradykinin, (2-D-Pro-7-D-Pro)-Isomer,Bradykinin, (3-D-Pro)-Isomer,Bradykinin, (3-D-Pro-7-D-Pro)-Isomer,Bradykinin, (5-D-Phe)-Isomer,Bradykinin, (5-D-Phe-8-D-Phe)-Isomer,Bradykinin, (6-D-Ser)-Isomer,Bradykinin, (7-D-Pro)-Isomer,Bradykinin, (8-D-Phe)-Isomer,Bradykinin, (9-D-Arg)-Isomer,Arg Pro Pro Gly Phe Ser Pro Phe Arg
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D016896 Treatment Outcome Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series. Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes
D054179 Angioedemas, Hereditary Inherited disorders that are characterized by subcutaneous and submucosal EDEMA in the upper RESPIRATORY TRACT and GASTROINTESTINAL TRACT. Angioedema, Hereditary,Angioneurotic Edema, Hereditary,C1 Esterase Inhibitor Deficiency,C1 Inhibitor Deficiency,Hereditary Angioedema,Hereditary Angioedemas,Hereditary Angioneurotic Edema,Angioneurotic Edemas, Hereditary,Edema, Hereditary Angioneurotic,Edemas, Hereditary Angioneurotic,Hereditary Angioneurotic Edemas
D056829 Hereditary Angioedema Types I and II Forms of hereditary angioedema that occur due to mutations in the gene for COMPLEMENT C1 INHIBITOR PROTEIN. Type I hereditary angioedema is associated with reduced serum levels of complement C1 inhibitor protein. Type II hereditary angioedema is associated with the production of a non-functional complement C1 inhibitor protein. Angioedema, Hereditary, Type I,Angioedema, Hereditary, Type II,Angioedema, Hereditary, Types I and II,C1 Esterase Inhibitor, Deficiency Of,Deficiency of C1 Esterase Inhibitor,Hereditary Angioedema Type 1,Hereditary Angioedema Type I,Hereditary Angioedema Type II
D019468 Disease Management A broad approach to appropriate coordination of the entire disease treatment process that often involves shifting away from more expensive inpatient and acute care to areas such as preventive medicine, patient counseling and education, and outpatient care. This concept includes implications of appropriate versus inappropriate therapy on the overall cost and clinical outcome of a particular disease. (From Hosp Pharm 1995 Jul;30(7):596) Disease Managements,Management, Disease,Managements, Disease

Related Publications

Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
January 1989, Revista de medicina interna, neurologe, psihiatrie, neurochirurgie, dermato-venerologie. Medicina interna,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
July 2010, Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
November 2016, Pediatrics,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
August 2015, Current opinion in allergy and clinical immunology,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
October 2012, Medicina clinica,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
January 2008, Journal of investigational allergology & clinical immunology,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
November 2023, Wiener klinische Wochenschrift,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
April 2020, Revue medicale suisse,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
March 2007, La Revue du praticien,
Andrea Zanichelli, and Marta Mansi, and Giulia Periti, and Marco Cicardi
October 2023, Journal of investigational allergology & clinical immunology,
Copied contents to your clipboard!