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Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency

Background Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by relapsing skin swellings, abdominal pain attacks, and, less frequently, potentially life-threatening laryngeal attacks. Objective This study determined the mortality of patients with and without the diag... Full description

Journal Title: Journal of allergy and clinical immunology 2012, Vol.130 (3), p.692-697
Main Author: Bork, Konrad, MD
Other Authors: Hardt, Jochen, PhD , Witzke, Günther, PhD
Format: Electronic Article Electronic Article
Language: English
Subjects:
Age
Quelle: Alma/SFX Local Collection
Publisher: New York, NY: Mosby, Inc
ID: ISSN: 0091-6749
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title: Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency
format: Article
creator:
  • Bork, Konrad, MD
  • Hardt, Jochen, PhD
  • Witzke, Günther, PhD
subjects:
  • Abridged Index Medicus
  • Adult
  • Age
  • Aged
  • Aged, 80 and over
  • Allergic diseases
  • Allergy and Immunology
  • Angioedema
  • Angioneurotic edema
  • Antigens
  • Asphyxia - etiology
  • asphyxiation
  • Biological and medical sciences
  • C1 inhibitor deficiency
  • Complement C1 Inhibitor Protein - physiology
  • complement component C1
  • Consciousness
  • Dyspnea
  • Family medical history
  • Female
  • Fundamental and applied biological sciences. Psychology
  • Fundamental immunology
  • Health aspects
  • hereditary angioedema
  • Hereditary Angioedema Types I and II - complications
  • Hereditary Angioedema Types I and II - mortality
  • Humans
  • Immunopathology
  • laryngeal attacks
  • Life span
  • Male
  • Medical sciences
  • Medical treatment
  • Middle Aged
  • Mortality
  • Pain
  • Respiration
  • Retrospective Studies
  • Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
  • Skin
  • Skin allergic diseases. Stinging insect allergies
  • Time Factors
  • upper airway obstruction
ispartof: Journal of allergy and clinical immunology, 2012, Vol.130 (3), p.692-697
description: Background Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by relapsing skin swellings, abdominal pain attacks, and, less frequently, potentially life-threatening laryngeal attacks. Objective This study determined the mortality of patients with and without the diagnosis of HAE-C1-INH and analyzed fatal laryngeal attacks. Methods A cohort of 728 patients from 182 families with HAE-C1-INH was evaluated for death cases by analyzing pedigrees. Detailed information on fatal laryngeal attacks in 36 patients was obtained by questioning relatives and treating physicians. Results Of the 214 patients who had died, 70 asphyxiated during a laryngeal attack. Mortality by asphyxiation was higher in patients with undiagnosed HAE-C1-INH (63 cases) than in patients with diagnosed HAE-C1-INH (7 cases). The lifespan of asphyxiated patients with undiagnosed HAE-C1-INH was on average ∼31 years shorter than patients with undiagnosed HAE-C1-INH who died of other causes. Three phases were distinguished in the fatal laryngeal attacks. Phase 1, the predyspnea phase, lasted on average for 3.7 ± 3.2 hours (range, 0-11 hours). Phase 2, the dyspnea phase, lasted on average for 41 ± 49 minutes (range, 2 minutes to 4 hours). Phase 3, the loss of consciousness phase, lasted on average for 8.9 ± 5.1 minutes (range, 2-20 minutes). Conclusions The high mortality in patients with undiagnosed HAE-C1-INH underscores the need to identify these patients and diagnose their condition. The analysis of fatal laryngeal attacks gives further insight into their course, thus helping to avoid fatalities in the future.
language: eng
source: Alma/SFX Local Collection
identifier: ISSN: 0091-6749
fulltext: fulltext
issn:
  • 0091-6749
  • 1097-6825
url: Link


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titleFatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency
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descriptionBackground Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by relapsing skin swellings, abdominal pain attacks, and, less frequently, potentially life-threatening laryngeal attacks. Objective This study determined the mortality of patients with and without the diagnosis of HAE-C1-INH and analyzed fatal laryngeal attacks. Methods A cohort of 728 patients from 182 families with HAE-C1-INH was evaluated for death cases by analyzing pedigrees. Detailed information on fatal laryngeal attacks in 36 patients was obtained by questioning relatives and treating physicians. Results Of the 214 patients who had died, 70 asphyxiated during a laryngeal attack. Mortality by asphyxiation was higher in patients with undiagnosed HAE-C1-INH (63 cases) than in patients with diagnosed HAE-C1-INH (7 cases). The lifespan of asphyxiated patients with undiagnosed HAE-C1-INH was on average ∼31 years shorter than patients with undiagnosed HAE-C1-INH who died of other causes. Three phases were distinguished in the fatal laryngeal attacks. Phase 1, the predyspnea phase, lasted on average for 3.7 ± 3.2 hours (range, 0-11 hours). Phase 2, the dyspnea phase, lasted on average for 41 ± 49 minutes (range, 2 minutes to 4 hours). Phase 3, the loss of consciousness phase, lasted on average for 8.9 ± 5.1 minutes (range, 2-20 minutes). Conclusions The high mortality in patients with undiagnosed HAE-C1-INH underscores the need to identify these patients and diagnose their condition. The analysis of fatal laryngeal attacks gives further insight into their course, thus helping to avoid fatalities in the future.
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publisherNew York, NY: Mosby, Inc
subjectAbridged Index Medicus ; Adult ; Age ; Aged ; Aged, 80 and over ; Allergic diseases ; Allergy and Immunology ; Angioedema ; Angioneurotic edema ; Antigens ; Asphyxia - etiology ; asphyxiation ; Biological and medical sciences ; C1 inhibitor deficiency ; Complement C1 Inhibitor Protein - physiology ; complement component C1 ; Consciousness ; Dyspnea ; Family medical history ; Female ; Fundamental and applied biological sciences. Psychology ; Fundamental immunology ; Health aspects ; hereditary angioedema ; Hereditary Angioedema Types I and II - complications ; Hereditary Angioedema Types I and II - mortality ; Humans ; Immunopathology ; laryngeal attacks ; Life span ; Male ; Medical sciences ; Medical treatment ; Middle Aged ; Mortality ; Pain ; Respiration ; Retrospective Studies ; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis ; Skin ; Skin allergic diseases. Stinging insect allergies ; Time Factors ; upper airway obstruction
ispartofJournal of allergy and clinical immunology, 2012, Vol.130 (3), p.692-697
rights
0American Academy of Allergy, Asthma & Immunology
12012 American Academy of Allergy, Asthma & Immunology
22015 INIST-CNRS
3Copyright © 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.
4COPYRIGHT 2012 Elsevier B.V.
5Copyright Elsevier Limited Sep 2012
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descriptionBackground Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by relapsing skin swellings, abdominal pain attacks, and, less frequently, potentially life-threatening laryngeal attacks. Objective This study determined the mortality of patients with and without the diagnosis of HAE-C1-INH and analyzed fatal laryngeal attacks. Methods A cohort of 728 patients from 182 families with HAE-C1-INH was evaluated for death cases by analyzing pedigrees. Detailed information on fatal laryngeal attacks in 36 patients was obtained by questioning relatives and treating physicians. Results Of the 214 patients who had died, 70 asphyxiated during a laryngeal attack. Mortality by asphyxiation was higher in patients with undiagnosed HAE-C1-INH (63 cases) than in patients with diagnosed HAE-C1-INH (7 cases). The lifespan of asphyxiated patients with undiagnosed HAE-C1-INH was on average ∼31 years shorter than patients with undiagnosed HAE-C1-INH who died of other causes. Three phases were distinguished in the fatal laryngeal attacks. Phase 1, the predyspnea phase, lasted on average for 3.7 ± 3.2 hours (range, 0-11 hours). Phase 2, the dyspnea phase, lasted on average for 41 ± 49 minutes (range, 2 minutes to 4 hours). Phase 3, the loss of consciousness phase, lasted on average for 8.9 ± 5.1 minutes (range, 2-20 minutes). Conclusions The high mortality in patients with undiagnosed HAE-C1-INH underscores the need to identify these patients and diagnose their condition. The analysis of fatal laryngeal attacks gives further insight into their course, thus helping to avoid fatalities in the future.
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5Allergic diseases
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8Angioneurotic edema
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19Female
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29Life span
30Male
31Medical sciences
32Medical treatment
33Middle Aged
34Mortality
35Pain
36Respiration
37Retrospective Studies
38Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
39Skin
40Skin allergic diseases. Stinging insect allergies
41Time Factors
42upper airway obstruction
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titleFatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency
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abstractBackground Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by relapsing skin swellings, abdominal pain attacks, and, less frequently, potentially life-threatening laryngeal attacks. Objective This study determined the mortality of patients with and without the diagnosis of HAE-C1-INH and analyzed fatal laryngeal attacks. Methods A cohort of 728 patients from 182 families with HAE-C1-INH was evaluated for death cases by analyzing pedigrees. Detailed information on fatal laryngeal attacks in 36 patients was obtained by questioning relatives and treating physicians. Results Of the 214 patients who had died, 70 asphyxiated during a laryngeal attack. Mortality by asphyxiation was higher in patients with undiagnosed HAE-C1-INH (63 cases) than in patients with diagnosed HAE-C1-INH (7 cases). The lifespan of asphyxiated patients with undiagnosed HAE-C1-INH was on average ∼31 years shorter than patients with undiagnosed HAE-C1-INH who died of other causes. Three phases were distinguished in the fatal laryngeal attacks. Phase 1, the predyspnea phase, lasted on average for 3.7 ± 3.2 hours (range, 0-11 hours). Phase 2, the dyspnea phase, lasted on average for 41 ± 49 minutes (range, 2 minutes to 4 hours). Phase 3, the loss of consciousness phase, lasted on average for 8.9 ± 5.1 minutes (range, 2-20 minutes). Conclusions The high mortality in patients with undiagnosed HAE-C1-INH underscores the need to identify these patients and diagnose their condition. The analysis of fatal laryngeal attacks gives further insight into their course, thus helping to avoid fatalities in the future.
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doi10.1016/j.jaci.2012.05.055