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Hereditary angioedema with a focus on the child
Gregory Bennett1, Timothy Craig
1Department of Pediatrics, Penn State Children's Hospital, Penn State University, Hershey, Pennsylvania, USA.
Allergy and Asthma Proceedings
|January 7, 2015
Summary
Hereditary angioedema (HAE) in children is understudied, with swelling being the most common symptom. Early diagnosis of HAE is crucial for better pediatric patient outcomes.
Area of Science:
- Pediatric Allergy and Immunology
- Rare Diseases
- Genetics
Background:
- Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent swelling.
- Pediatric HAE data and treatment options are limited in the United States.
- HAE symptoms can lead to significant morbidity and mortality, often appearing in childhood.
Purpose of the Study:
- To summarize existing data on pediatric HAE.
- To conduct a retrospective chart review of children diagnosed with HAE.
- To improve the clinical care and management of HAE in pediatric patients.
Main Methods:
- Institutional Review Board approval was obtained.
- A retrospective chart review of electronic medical records from 2001 to 2011.
- Identified pediatric patients (1-18 years) with a confirmed HAE diagnosis.
Main Results:
- Twenty-five pediatric patients with HAE were identified (13 female, 12 male).
- The median age at diagnosis was seven years.
- Common initial symptoms included extremity swelling, abdominal pain, facial swelling, and scrotal swelling; 84% had a family history.
Conclusions:
- Accurate and timely diagnosis of HAE in children is essential.
- Improved understanding of pediatric HAE can reduce morbidity and mortality.
- Further research is needed to address the paucity of data and treatment options for pediatric HAE.
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