Hereditary Angioedema.

Humans Angioedemas, Hereditary / diagnosis Complement C1 Inhibitor Protein / genetics
Bradykinin C1-inhibitor Complement Difficult airway Hereditary angioedema Quincke disease

Journal

Immunology and allergy clinics of North America
ISSN: 1557-8607
Titre abrégé: Immunol Allergy Clin North Am
Pays: United States
ID NLM: 8805635

Informations de publication

Date de publication:
08 2023
Historique:
medline: 4 7 2023
pubmed: 3 7 2023
entrez: 2 7 2023
Statut: ppublish

Résumé

Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder that usual results from a decreased level of functional C1-INH and clinically manifests with intermittent attacks of swelling of the subcutaneous tissue or submucosal layers of the respiratory or gastrointestinal tracts. Laboratory studies and radiographic imaging have limited roles in evaluation of patients with acute attacks of HAE except when the diagnosis is uncertain and other processes must be ruled out. Treatment begins with assessment of the airway to determine the need for immediate intervention. Emergency physicians should understand the pathophysiology of HAE to help guide management decisions.

Identifiants

DOI: 10.1016/j.iac.2022.10.012 PMID: 37394258
pubmed: 37394258
pii: S0889-8561(22)00909-2
doi: 10.1016/j.iac.2022.10.012
pii:
doi:

Substances chimiques

Complement C1 Inhibitor Protein 0

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

533-552

Informations de copyright

Copyright © 2022 Elsevier Inc. All rights reserved.

Déclaration de conflit d'intérêts

Disclosure The authors have nothing to disclose.

Auteurs

R Gentry Wilkerson (RG)

Department of Emergency Medicine, University of Maryland School of Medicine, 110 South Paca Street, 6th Floor, Suite 200, Baltimore, MD 21201, USA. Electronic address: gwilkerson@som.umaryland.edu.

Joseph J Moellman (JJ)

Department of Emergency Medicine, University of Cincinnati College of Medicine, 231 Albert Sabin Way, MSB 1654, Cincinnati, OH 45267-0769, USA. Electronic address: https://twitter.com/edmojo.

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Classifications MeSH

questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Hereditary Angioedema.
questionsmedicales.fr › Maladies du système immunitaire › Déficits immunitaires › Maladies d'immunodéficience primaire › Déficits héréditaires en complément › Angio-oedèmes héréditaires › Hereditary Angioedema.
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines inhibitrices de la fraction C1 du complément › C1 Inhibiteur › Hereditary Angioedema.