Abstract

Introduction. Hereditary angioedema (HAE) due to C1 inhibitor deficiency or dysfunction (HAE-C1-INH) is a rare, autosomal dominant condition with recurrent swellings, often beginning in childhood. Pediatric data on disease burden and management are limited.

Materials and methods. We conducted a retrospective review of pediatric HAE cases at Barts Health NHS Trust, London, UK, analyzing clinical features, treatment, and outcomes, comparing symptomatic and asymptomatic patients.

Results. Of 33 children (mean age of 12.9 years), 26 were symptomatic. Median age at first attack was 3.8 years. Facial and abdominal attacks were common; laryngeal attacks rare. 88% had type I HAE. 27% received long-term prophylaxis; 97% held on-demand treatment. Mean AECT score was 11.6.

Discussion. Symptoms and treatment responses varied. Many attacks were untreated despite access to treatment. Prophylaxis was underused and often not first-line.

Conclusion. Pediatric HAE is heterogeneous in presentation. Greater access to modern prophylaxis and better symptom recognition may improve outcomes

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Authors

Rhea Bansal - Department of Immunology, Royal London Hospital, Barts Health NHS Trust, Whitechapel Road, London, UK

Lorena Lorenzo - Department of Immunology, Royal London Hospital, Barts Health NHS Trust, Whitechapel Road, London, UK

Mildred Iro - Department of Paediatric Infectious Diseases and Immunology, Royal London Hospital, Barts Health NHS Trust, Whitechapel Road, London, UK

Keith Sacco - Department of Immunology, Royal London Hospital, Barts Health NHS Trust, Whitechapel Road, London, UK

Anthony Dorr - Department of Immunology, Royal London Hospital, Barts Health NHS Trust, Whitechapel Road, London, UK

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Copyright (c) 2025 Italian Journal of Pediatric Allergy and Immunology

How to Cite
Bansal, R., Lorenzo, L. ., Iro, M. ., Sacco, K. ., & Dorr, A. (2025). Demographic and clinical variation in pediatric hereditary angioedema: a single center service evaluation. Italian Journal of Pediatric Allergy and Immunology, 39(2). https://doi.org/10.53151/2531-3916/2025-1263
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