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Management of hereditary angioedema in pediatric patients
Henriette Farkas1, Lilian Varga, Gábor Széplaki
13rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4, H-1125, Budapest, Hungary. farkash@kut.sote.hu
Insights
Hereditary angioneurotic edema, a rare genetic disorder, involves C1 inhibitor deficiency causing swelling. This review focuses on effective management strategies for pediatric hereditary angioneurotic edema, addressing a gap in current literature.
Area of Science:
- Immunology
- Genetics
- Pediatrics
Background:
- Hereditary angioneurotic edema (HAE) is a rare genetic disorder characterized by recurrent swelling episodes.
- It results from a deficiency or dysfunction of the C1 inhibitor (C1-INH).
- Conventional treatments like antihistamines and corticosteroids are often ineffective for HAE.
Purpose of the Study:
- To review the experience and published data on managing pediatric HAE.
- To provide a comprehensive approach to HAE in childhood.
- To address the limited literature specifically on pediatric HAE management.
Main Methods:
- Review of institutional experience with HAE.
- Systematic review of published literature on pediatric HAE.
- Analysis of diagnostic and therapeutic strategies for HAE in children.
Main Results:
- HAE manifestations typically appear within the first two decades of life.
- There is a significant delay between symptom onset and HAE diagnosis.
- Current literature on pediatric HAE management is scarce.
Conclusions:
- A specialized approach is needed for managing HAE in children.
- Early diagnosis and appropriate treatment are crucial for improving outcomes in pediatric HAE.
- Further research and guidelines for pediatric HAE are warranted.
Abstract:
Hereditary angioneurotic edema is a rare disorder caused by the congenital deficiency of C1 inhibitor. Recurring angioedematous paroxysms that most commonly involve the subcutis (eg, extremities, face, trunk, and genitals) or the submucosa (eg, intestines and larynx) are the hallmarks of hereditary angioneurotic edema. Edema formation is related to reduction or dysfunction of C1 inhibitor, and conventional therapy with antihistamines and corticosteroids is ineffective. Manifestations occur during the initial 2 decades of life, but even today there is a long delay between the onset of initial symptoms and the diagnosis of hereditary angioneurotic edema. Although a variety of reviews have been published during the last 3 decades on the general management of hereditary angioneurotic edema, little has been published regarding management of pediatric hereditary angioneurotic edema. Thus, we review our experience and published data to provide an approach to hereditary angioneurotic edema in childhood.
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