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Hereditary angioedema: New therapeutic options for a potentially deadly disorder

Although the biochemistry of hereditary angioedema (HAE) is fairly well understood today, the lag in diagnosis of a decade or more suggests that clinicians have low awareness of this disease. This lag in diagnosis and hence treatment certainly stems from the rarity and complexity of the presentation...

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Autor principal: Eidelman, Frank J
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2881905/
https://www.ncbi.nlm.nih.gov/pubmed/20470390
http://dx.doi.org/10.1186/1471-2326-10-3
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author Eidelman, Frank J
author_facet Eidelman, Frank J
author_sort Eidelman, Frank J
collection PubMed
description Although the biochemistry of hereditary angioedema (HAE) is fairly well understood today, the lag in diagnosis of a decade or more suggests that clinicians have low awareness of this disease. This lag in diagnosis and hence treatment certainly stems from the rarity and complexity of the presentation which can be easily mistaken for allergic and non-allergic reactions alike. The symptoms of the disease include acute swelling of any or multiple parts of the body. The attacks may be frequent or rare, and they may vary substantially in severity, causing stomach discomfort or periorbital swelling in mild cases and hypovolemic shock due to abdominal fluid shift or asphyxiation in the most severe cases. Given that these patients are at significant risk for poor quality of life and death, greater awareness of this disease is needed to ensure that newly available, effective medications are used in these patients. These new medications represent significant advances in HAE therapy because they are targeted at the plasma cascades implicated in the pathophysiology of this disease. The clinical presentation of HAE, overlapping symptoms with other angioedemas, and available therapies are reviewed.
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spelling pubmed-28819052010-06-08 Hereditary angioedema: New therapeutic options for a potentially deadly disorder Eidelman, Frank J BMC Blood Disord Review Although the biochemistry of hereditary angioedema (HAE) is fairly well understood today, the lag in diagnosis of a decade or more suggests that clinicians have low awareness of this disease. This lag in diagnosis and hence treatment certainly stems from the rarity and complexity of the presentation which can be easily mistaken for allergic and non-allergic reactions alike. The symptoms of the disease include acute swelling of any or multiple parts of the body. The attacks may be frequent or rare, and they may vary substantially in severity, causing stomach discomfort or periorbital swelling in mild cases and hypovolemic shock due to abdominal fluid shift or asphyxiation in the most severe cases. Given that these patients are at significant risk for poor quality of life and death, greater awareness of this disease is needed to ensure that newly available, effective medications are used in these patients. These new medications represent significant advances in HAE therapy because they are targeted at the plasma cascades implicated in the pathophysiology of this disease. The clinical presentation of HAE, overlapping symptoms with other angioedemas, and available therapies are reviewed. BioMed Central 2010-05-14 /pmc/articles/PMC2881905/ /pubmed/20470390 http://dx.doi.org/10.1186/1471-2326-10-3 Text en Copyright ©2010 Eidelman; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review
Eidelman, Frank J
Hereditary angioedema: New therapeutic options for a potentially deadly disorder
title Hereditary angioedema: New therapeutic options for a potentially deadly disorder
title_full Hereditary angioedema: New therapeutic options for a potentially deadly disorder
title_fullStr Hereditary angioedema: New therapeutic options for a potentially deadly disorder
title_full_unstemmed Hereditary angioedema: New therapeutic options for a potentially deadly disorder
title_short Hereditary angioedema: New therapeutic options for a potentially deadly disorder
title_sort hereditary angioedema: new therapeutic options for a potentially deadly disorder
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2881905/
https://www.ncbi.nlm.nih.gov/pubmed/20470390
http://dx.doi.org/10.1186/1471-2326-10-3
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