Kingdom, 2Instituto de Estudios de Mastocitosis, Hospital Virgen Del. Valle ... of Pediatrics, Division of Allergy-Immunology, National Jewish Health,. Denver, CO ...
AB56 Abstracts
SATURDAY
201
Pediatric Presentation of Hereditary Angioedema (HAE) Due to C1 Inhibitor Deficiency Sandra C. Christiansen, MD, FAAAAI1,2, Donna Davis3, Anthony J. Castaldo3, Bruce L. Zuraw, MD2,4; 1Southern CA Permanente Med Grp, San Diego, CA, 2University of California San Diego, La Jolla, CA, 3 US Hereditary Angioedema Association, Honolulu, HI, 4San Diego VA Healthcare, San Diego, CA. RATIONALE: Several European surveys have reported that symptoms of HAE often begin in childhood. We sought to assess the age of symptom onset in a large population of United States HAE subjects, and determine the implications of age of disease onset. METHODS: We performed an observational IRB approved study on adult subjects with HAE utilizing an anonymous, web-based survey. All subjects reported a history of physician-diagnosed HAE. Age of symptom onset, age of diagnosis, and attack frequency were assessed in 650 subjects. RESULTS: Symptoms of HAE began in the pediatric age range _18 years) in 542 (83.4%) of the 650 subjects, starting by the age of (< 6 in 25.4% and by the age of 12 in 54.8%. Current HAE severity was highest in subjects developing symptoms at the youngest age (p
