Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency

Pediatrics. 2016 Nov;138(5):e20160575. doi: 10.1542/peds.2016-0575.


Hereditary angioedema (HAE) is a potentially life-threatening inherited disease characterized by attacks of skin swelling, severe abdominal pain, and upper airway swelling. Attacks typically begin in childhood, but the appropriate diagnosis is often missed. Attacks do not respond to epinephrine, antihistamines, or glucocorticoids. Recently, many effective drugs have been approved for treatment of adults with HAE, and the Medical Advisory Board of the HAE Patient's Association has developed and reported treatment recommendations for adults. Only 1 medication is approved for treatment of children <12 years of age, and there are no reported consensus recommendations for treatment of young children in the United States. The 11-member Medical Advisory Board, with extensive experience in the treatment of children, in concert with the leaders of the HAE Patient's Association, has developed these consensus recommendations to help in recognition, diagnosis, treatment of attacks, and prophylaxis of children with HAE.

Publication types

  • Consensus Development Conference

MeSH terms

  • Adolescent
  • Angioedemas, Hereditary / diagnosis
  • Angioedemas, Hereditary / drug therapy*
  • Angioedemas, Hereditary / prevention & control*
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Antifibrinolytic Agents / therapeutic use
  • Bradykinin / analogs & derivatives
  • Bradykinin / therapeutic use
  • Child
  • Complement C1 Inactivator Proteins / therapeutic use
  • Complement C1 Inhibitor Protein / therapeutic use
  • Danazol / adverse effects
  • Genetic Testing
  • Humans
  • Patient Care Team
  • Patient Education as Topic
  • Peptides / therapeutic use
  • Recombinant Proteins / therapeutic use
  • Tranexamic Acid / therapeutic use


  • Anti-Inflammatory Agents, Non-Steroidal
  • Antifibrinolytic Agents
  • Complement C1 Inactivator Proteins
  • Complement C1 Inhibitor Protein
  • Peptides
  • Recombinant Proteins
  • SERPING1 protein, human
  • ecallantide
  • conestat alfa
  • Tranexamic Acid
  • icatibant
  • Danazol
  • Bradykinin