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A Comprehensive Management Approach in Pediatric and Adolescent Patients With Hereditary Angioedema
Hereditary angioedema (HAE) is a rare autosomal-dominant disorder; most cases are characterized by low plasma levels of C1 esterase inhibitor (C1-INH). Clinical manifestations of HAE due to C1-INH deficiency include unpredictable, acute, recurrent episodes of nonpruritic swelling that can affect the...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10478326/ https://www.ncbi.nlm.nih.gov/pubmed/36908071 http://dx.doi.org/10.1177/00099228231155703 |
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author | Tachdjian, Raffi Kaplan, Allen P. |
author_facet | Tachdjian, Raffi Kaplan, Allen P. |
author_sort | Tachdjian, Raffi |
collection | PubMed |
description | Hereditary angioedema (HAE) is a rare autosomal-dominant disorder; most cases are characterized by low plasma levels of C1 esterase inhibitor (C1-INH). Clinical manifestations of HAE due to C1-INH deficiency include unpredictable, acute, recurrent episodes of nonpruritic swelling that can affect the face, trunk, limbs, and the respiratory, gastrointestinal, and genitourinary tracts. Attacks can be disfiguring, disabling, painful, and even life-threatening if laryngeal swelling occurs. Symptoms of HAE generally manifest in childhood. Effective medications are available and approved to treat HAE in children. However, evidence informing use of these medications in pediatric clinical practice is limited. Hereditary angioedema management plans are critical to optimize outcomes and should address on-demand treatment for acute attacks and plans to prevent potentially fatal laryngeal attacks. The plan should also comprise a holistic approach to address nonclinical aspects of HAE, including quality of life (QoL) and psychological issues. This article provides an overview of HAE management principles that health care providers can apply to treat pediatric patients to improve their QoL. |
format | Online Article Text |
id | pubmed-10478326 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-104783262023-09-06 A Comprehensive Management Approach in Pediatric and Adolescent Patients With Hereditary Angioedema Tachdjian, Raffi Kaplan, Allen P. Clin Pediatr (Phila) Review Article Hereditary angioedema (HAE) is a rare autosomal-dominant disorder; most cases are characterized by low plasma levels of C1 esterase inhibitor (C1-INH). Clinical manifestations of HAE due to C1-INH deficiency include unpredictable, acute, recurrent episodes of nonpruritic swelling that can affect the face, trunk, limbs, and the respiratory, gastrointestinal, and genitourinary tracts. Attacks can be disfiguring, disabling, painful, and even life-threatening if laryngeal swelling occurs. Symptoms of HAE generally manifest in childhood. Effective medications are available and approved to treat HAE in children. However, evidence informing use of these medications in pediatric clinical practice is limited. Hereditary angioedema management plans are critical to optimize outcomes and should address on-demand treatment for acute attacks and plans to prevent potentially fatal laryngeal attacks. The plan should also comprise a holistic approach to address nonclinical aspects of HAE, including quality of life (QoL) and psychological issues. This article provides an overview of HAE management principles that health care providers can apply to treat pediatric patients to improve their QoL. SAGE Publications 2023-03-12 2023-10 /pmc/articles/PMC10478326/ /pubmed/36908071 http://dx.doi.org/10.1177/00099228231155703 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Review Article Tachdjian, Raffi Kaplan, Allen P. A Comprehensive Management Approach in Pediatric and Adolescent Patients With Hereditary Angioedema |
title | A Comprehensive Management Approach in Pediatric and Adolescent Patients With Hereditary Angioedema |
title_full | A Comprehensive Management Approach in Pediatric and Adolescent Patients With Hereditary Angioedema |
title_fullStr | A Comprehensive Management Approach in Pediatric and Adolescent Patients With Hereditary Angioedema |
title_full_unstemmed | A Comprehensive Management Approach in Pediatric and Adolescent Patients With Hereditary Angioedema |
title_short | A Comprehensive Management Approach in Pediatric and Adolescent Patients With Hereditary Angioedema |
title_sort | comprehensive management approach in pediatric and adolescent patients with hereditary angioedema |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10478326/ https://www.ncbi.nlm.nih.gov/pubmed/36908071 http://dx.doi.org/10.1177/00099228231155703 |
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