Hereditary angioedema: A decade of human C1-inhibitor concentrate therapy

H. Farkas, László Jakab, György Temesszentandrási, B. Visy, G. Harmat, G. Füst, G. Széplaki, B. Fekete, I. Karádi, L. Varga

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Abstract

Background: C1-inhibitor (C1-INH) is a serine protease inhibitor regulating the complement, kinin-kallikrein, coagulation, and fibrinolytic systems. Hereditary angioedema (HAE) is caused by an inherited deficiency of C1-INH characterized by sudden, recurrent edematous swellings of the subcutaneous or submucosal tissues. The optional therapy for the acute management of HAE is administration of human C1-INH (hC1-INH) concentrate. However, hC1-INH is not available in many countries, in which case fresh frozen plasma is an alternative. Objective: To summarize our experience with hC1-INH concentrate in patients with HAE. Methods: Clinical and laboratory information on the effectiveness and safety of hC1-INH administered to relieve 468 acute edematous attacks in 61 patients with HAE was analyzed. Results: Severe abdominal or subcutaneous attacks and laryngeal edema were consistently relieved by the administration of 500 U hC1-INH concentrate. Symptoms improved within 15 to 60 minutes of administration. Progression of the attacks was never observed, and there were no recurrent attacks within 72 hours. hC1-INH concentrate requirements did not change after repeated use. hC1-INH concentrate proved effective in the management of 94 attacks in 22 children and 6 attacks in 4 pregnant women. Adverse reactions, viral infections, and antibody formation against the purified protein did not occur. Conclusion: The administration of hC1-INH concentrate in HAE is highly effective and safe for the treatment of acute attacks and short-term prophylaxis and in pediatric patients and pregnant women. Clinical implications: Human C1-INH concentrate is effective and safe for the treatment of acute HAE attacks as well as for short-term prophylaxis.

Original languageEnglish
Pages (from-to)941-947
Number of pages7
JournalJournal of Allergy and Clinical Immunology
Volume120
Issue number4
DOIs
Publication statusPublished - okt. 2007

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Hereditary Angioedemas
Therapeutics
Pregnant Women
Laryngeal Edema
Viral Antibodies
human SERPING1 protein
Kinins
Kallikreins
Serine Proteinase Inhibitors
Virus Diseases
Antibody Formation
Pediatrics
Safety

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

@article{88bf23b7b3bb430a8eb4edaea68e0ae0,
title = "Hereditary angioedema: A decade of human C1-inhibitor concentrate therapy",
abstract = "Background: C1-inhibitor (C1-INH) is a serine protease inhibitor regulating the complement, kinin-kallikrein, coagulation, and fibrinolytic systems. Hereditary angioedema (HAE) is caused by an inherited deficiency of C1-INH characterized by sudden, recurrent edematous swellings of the subcutaneous or submucosal tissues. The optional therapy for the acute management of HAE is administration of human C1-INH (hC1-INH) concentrate. However, hC1-INH is not available in many countries, in which case fresh frozen plasma is an alternative. Objective: To summarize our experience with hC1-INH concentrate in patients with HAE. Methods: Clinical and laboratory information on the effectiveness and safety of hC1-INH administered to relieve 468 acute edematous attacks in 61 patients with HAE was analyzed. Results: Severe abdominal or subcutaneous attacks and laryngeal edema were consistently relieved by the administration of 500 U hC1-INH concentrate. Symptoms improved within 15 to 60 minutes of administration. Progression of the attacks was never observed, and there were no recurrent attacks within 72 hours. hC1-INH concentrate requirements did not change after repeated use. hC1-INH concentrate proved effective in the management of 94 attacks in 22 children and 6 attacks in 4 pregnant women. Adverse reactions, viral infections, and antibody formation against the purified protein did not occur. Conclusion: The administration of hC1-INH concentrate in HAE is highly effective and safe for the treatment of acute attacks and short-term prophylaxis and in pediatric patients and pregnant women. Clinical implications: Human C1-INH concentrate is effective and safe for the treatment of acute HAE attacks as well as for short-term prophylaxis.",
keywords = "hereditary angioedema, hereditary angioneurotic edema, Human C1-inhibitor concentrate, treatment",
author = "H. Farkas and L{\'a}szl{\'o} Jakab and Gy{\"o}rgy Temesszentandr{\'a}si and B. Visy and G. Harmat and G. F{\"u}st and G. Sz{\'e}plaki and B. Fekete and I. Kar{\'a}di and L. Varga",
year = "2007",
month = "10",
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language = "English",
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pages = "941--947",
journal = "Journal of Allergy and Clinical Immunology",
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TY - JOUR

T1 - Hereditary angioedema

T2 - A decade of human C1-inhibitor concentrate therapy

AU - Farkas, H.

AU - Jakab, László

AU - Temesszentandrási, György

AU - Visy, B.

AU - Harmat, G.

AU - Füst, G.

AU - Széplaki, G.

AU - Fekete, B.

AU - Karádi, I.

AU - Varga, L.

PY - 2007/10

Y1 - 2007/10

N2 - Background: C1-inhibitor (C1-INH) is a serine protease inhibitor regulating the complement, kinin-kallikrein, coagulation, and fibrinolytic systems. Hereditary angioedema (HAE) is caused by an inherited deficiency of C1-INH characterized by sudden, recurrent edematous swellings of the subcutaneous or submucosal tissues. The optional therapy for the acute management of HAE is administration of human C1-INH (hC1-INH) concentrate. However, hC1-INH is not available in many countries, in which case fresh frozen plasma is an alternative. Objective: To summarize our experience with hC1-INH concentrate in patients with HAE. Methods: Clinical and laboratory information on the effectiveness and safety of hC1-INH administered to relieve 468 acute edematous attacks in 61 patients with HAE was analyzed. Results: Severe abdominal or subcutaneous attacks and laryngeal edema were consistently relieved by the administration of 500 U hC1-INH concentrate. Symptoms improved within 15 to 60 minutes of administration. Progression of the attacks was never observed, and there were no recurrent attacks within 72 hours. hC1-INH concentrate requirements did not change after repeated use. hC1-INH concentrate proved effective in the management of 94 attacks in 22 children and 6 attacks in 4 pregnant women. Adverse reactions, viral infections, and antibody formation against the purified protein did not occur. Conclusion: The administration of hC1-INH concentrate in HAE is highly effective and safe for the treatment of acute attacks and short-term prophylaxis and in pediatric patients and pregnant women. Clinical implications: Human C1-INH concentrate is effective and safe for the treatment of acute HAE attacks as well as for short-term prophylaxis.

AB - Background: C1-inhibitor (C1-INH) is a serine protease inhibitor regulating the complement, kinin-kallikrein, coagulation, and fibrinolytic systems. Hereditary angioedema (HAE) is caused by an inherited deficiency of C1-INH characterized by sudden, recurrent edematous swellings of the subcutaneous or submucosal tissues. The optional therapy for the acute management of HAE is administration of human C1-INH (hC1-INH) concentrate. However, hC1-INH is not available in many countries, in which case fresh frozen plasma is an alternative. Objective: To summarize our experience with hC1-INH concentrate in patients with HAE. Methods: Clinical and laboratory information on the effectiveness and safety of hC1-INH administered to relieve 468 acute edematous attacks in 61 patients with HAE was analyzed. Results: Severe abdominal or subcutaneous attacks and laryngeal edema were consistently relieved by the administration of 500 U hC1-INH concentrate. Symptoms improved within 15 to 60 minutes of administration. Progression of the attacks was never observed, and there were no recurrent attacks within 72 hours. hC1-INH concentrate requirements did not change after repeated use. hC1-INH concentrate proved effective in the management of 94 attacks in 22 children and 6 attacks in 4 pregnant women. Adverse reactions, viral infections, and antibody formation against the purified protein did not occur. Conclusion: The administration of hC1-INH concentrate in HAE is highly effective and safe for the treatment of acute attacks and short-term prophylaxis and in pediatric patients and pregnant women. Clinical implications: Human C1-INH concentrate is effective and safe for the treatment of acute HAE attacks as well as for short-term prophylaxis.

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KW - hereditary angioneurotic edema

KW - Human C1-inhibitor concentrate

KW - treatment

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