Pediatric Hereditary Angioedema - Symptoms occur earlier than we thought

Published Online: January 19, 2015

Hereditary angioedema (HAE) is a rare disorder that causes episodes of swelling of the face, abdomen, hands, and feet. These swelling attacks were classically reported to begin after age 10 years. It has also been reported that children have less severe swelling attacks. However, the authors’ experience in clinics, as well as some prior studies, suggests that these swelling attacks in children can be more severe, and can occur earlier in life, than previously thought. Thus, in an article recently published in The Journal of Allergy and Clinical Immunology: In Practice, Nanda et al. examine the age of onset and frequency/location of swelling attacks in children with HAE.

The authors of this study reviewed charts of children under 18 years of age with a diagnosis of HAE at Cincinnati Children’s Hospital Medical Center and The Bernstein Allergy Group, obtaining information about each child’s age at diagnosis, age at onset of symptoms, family history, and frequency/location of swelling attacks. Any information missing from the chart was collected by telephone interviews with patient and/or parents.
The majority of the 21 children with HAE identified were male, Caucasian, and had a family history of HAE. Five children diagnosed were attack-free, but 87% of those with swelling attacks started in the first decade of life. The median age of swelling attack onset was 5.7 years and the median age of diagnosis was 5.0 years. Three children without a family history of HAE had a mean delay of diagnosis of six years. Children with a family history were diagnosed 0.9 years prior to symptom onset. The majority of children reported having had an abdominal attack, but attacks involving the hands and feet were more frequent.
The previously often reported mean age of onset in the literature and clinical practice is 11.2 years; however this study shows a much younger onset of swelling attacks. This belief that symptom onset occurs in the second decade of life may delay a diagnosis of HAE in young children, especially in those without a family history. Although abdominal pain is a common pediatric complaint, it is also a presenting feature of HAE. Therefore, clinicians need to maintain a high index of suspicion in younger children and in children with repeated bouts of severe abdominal pain. A screening C4 level should be considered in children with a history of swelling episodes including severe bouts of abdominal pain. As children are presenting with HAE symptoms at a younger age than previously thought, availability of currently FDA approved preventive and on-demand medications for use in children is extremely important.

The Journal of Allergy and Clinical Immunology: In Practice is an official journal of the AAAAI, focusing on practical information for the practicing clinician.

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