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Free Content Hereditary angioedema: Special considerations in children

Patients with hereditary angioedema (HAE) can experience attacks at any age; however, the onset of swelling is typically in childhood. Unlike adults, this population is uniquely vulnerable; attacks in young children may be subtle, resemble other diseases, and often lead to a delay in diagnosis. Misdiagnosis contributes to significant delays in treatment, painful attacks, increased emotional stress, unnecessary procedures, and a potential risk of death. Older children may hide their symptoms due to anxiety or fear of social isolation. Attacks typically become more severe and more frequent during and after puberty. The impact of HAE attacks on school attendance and school performance may prevent future career or education opportunities. Living with HAE poses significant psychosocial stress on children and their families. In the United States, medical treatments for acute attacks in children approved for self-administration are limited to intravenous therapies, which complicates early treatment. To provide optimal care, we suggest that physicians screen all children with a family history of HAE, appreciate the dynamic nature of the disease during adolescence, proactively assess the psychosocial impact of disease, and continually reassess the treatment plan.

Keywords: C1 inhibitor; adolescence; angioedema; attacks; children; deficiency; hereditary; prophylaxis; swelling; treatment

Document Type: Research Article

Affiliations: 1: From the Asthma and Allergy Specialists, Charlotte, North Carolina; and 2: Department of Pediatrics, Levine Children’s Hospital, Atrium Health, Charlotte, North Carolina

Publication date: November 1, 2020

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