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Background  The consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1‐ INH ‐ HAE ) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1‐ INH ‐ HAE .    Methods  During an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 ( www.haenet.hu ), pediatric data were presented and discussed and a consensus was developed by voting.    Results  The symptoms of C1‐ INH ‐ HAE  often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1‐ INH ‐ HAE , but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1‐ INH  levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1‐ INH ‐ HAE  family member should be screened for C1‐ INH  deficiency. Pediatric patients should always carry a C1‐ INH ‐ HAE  information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma‐derived C1‐ INH , recombinant C1‐ INH , and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an  HAE  comprehensive care center.    Conclusions  The pediatric‐focused international consensus for the diagnosis and management of C1‐ INH ‐ HAE  patients was created.

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency