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Hereditary angioedema: Special considerations in children
Douglas T Johnston1, R Christina Smith2
1From the Asthma and Allergy Specialists, Charlotte, North Carolina; and.
Allergy and Asthma Proceedings
|October 28, 2020
Summary
Hereditary angioedema (HAE) attacks in children can be subtle, leading to delayed diagnosis and treatment. Early screening and proactive management are crucial for improving outcomes and reducing the psychosocial burden of HAE in pediatric patients.
Area of Science:
- Pediatric Allergy and Immunology
- Rare Disease Research
- Genetics and Genomics
Background:
- Hereditary angioedema (HAE) attacks can occur at any age, with onset often in childhood.
- Pediatric HAE is uniquely challenging due to subtle symptoms, resemblance to other conditions, and delayed diagnosis.
- Delayed diagnosis leads to undertreatment, increased attack frequency/severity, and significant psychosocial distress.
Purpose of the Study:
- To highlight the diagnostic challenges and unique vulnerabilities of HAE in children.
- To emphasize the impact of HAE on pediatric patients' psychosocial well-being and educational opportunities.
- To recommend strategies for improved HAE management in pediatric populations.
Main Methods:
- Review of clinical presentations and diagnostic considerations for HAE in children.
- Analysis of the psychosocial impact of HAE on pediatric patients and families.
- Evaluation of current treatment limitations for acute HAE attacks in pediatric patients in the US.
Main Results:
- Pediatric HAE attacks are often subtle, mimicking other diseases, leading to diagnostic delays.
- HAE attacks can worsen during and after puberty, impacting school performance and future opportunities.
- Limited availability of self-administered treatments for acute attacks in children complicates early intervention.
Conclusions:
- Physicians should screen children with a family history of HAE.
- Recognizing the evolving nature of HAE during adolescence and its psychosocial impact is essential.
- Continuous reassessment and proactive management plans are vital for optimizing care for pediatric HAE patients.
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