Pediatric Emergency Department Management of C1 Inhibitor Deficiency

C1 inhibitor deficiency is a rare, potentially life-threatening syndrome. Acute attacks of angioedema may occur at any time, so the emergency department (ED) constitutes an indispensable component of its care.

To describe the reasons for consultation by children with C1 inhibitor deficiency at the ED, as well as its management and complications.

This is a longitudinal retrospective study conducted in a pediatric ED of a tertiary care hospital in Madrid. The study includes children with C1 inhibitor deficiency, aged 0 to 16 years, who had consulted the ED for whatever reason, over a span of 9 years (2011-2020). Analyzed data include the following: age, sex, type of disease, reason for query, complementary examinations, established diagnosis, treatment, number of visits to the ED, length of ED stay, and admissions.

Sixteen patients, amounting a total of 83 ED visits, were analyzed. Fifty-six percent were boys, and the median age was 6.9 years (5 months to 15.9 years). The median and mean of number of visits to the ED per patient was 2 (1-22) and 5.2 ± 6.11, respectively. There were 85.5% of the ED visits initially attributed to acute angioedema attacks. Additional tests were conducted in 30.1%, and 31.3% required C1 inhibitor concentrate. Three episodes required hospital admission (3.6%), and there were no complications. The stay in the ED was longer for patients who needed specific intravenous treatment.

C1 inhibitor deficiency is a rare disease that may require ED care. The main reasons for ED visits were respiratory problems, and the main location of the acute attacks of angioedema were abdominal and cutaneous. Almost one third of the ED visits needed specific treatment, resulting in longer stays. A proper management in the ED and the specific treatment with C1 inhibitor concentrate were effective in 96.2% of the acute attacks of angioedema. Knowledge of this disease in the ED is key to prevent complications.