Management of hereditary angioedema in pediatric patients

Pediatrics. 2007 Sep;120(3):e713-22. doi: 10.1542/peds.2006-3303. Epub 2007 Aug 27.


Hereditary angioneurotic edema is a rare disorder caused by the congenital deficiency of C1 inhibitor. Recurring angioedematous paroxysms that most commonly involve the subcutis (eg, extremities, face, trunk, and genitals) or the submucosa (eg, intestines and larynx) are the hallmarks of hereditary angioneurotic edema. Edema formation is related to reduction or dysfunction of C1 inhibitor, and conventional therapy with antihistamines and corticosteroids is ineffective. Manifestations occur during the initial 2 decades of life, but even today there is a long delay between the onset of initial symptoms and the diagnosis of hereditary angioneurotic edema. Although a variety of reviews have been published during the last 3 decades on the general management of hereditary angioneurotic edema, little has been published regarding management of pediatric hereditary angioneurotic edema. Thus, we review our experience and published data to provide an approach to hereditary angioneurotic edema in childhood.

Publication types

  • Review

MeSH terms

  • Abdomen, Acute / etiology
  • Algorithms
  • Angioedema / diagnosis*
  • Angioedema / genetics
  • Angioedema / metabolism
  • Angioedema / therapy*
  • Child
  • Complement C1 Inhibitor Protein / supply & distribution
  • Complement C1 Inhibitor Protein / therapeutic use
  • Complement C4 / metabolism
  • Complement Inactivating Agents / supply & distribution
  • Complement Inactivating Agents / therapeutic use
  • Early Diagnosis
  • Edema / etiology
  • Humans
  • Laryngeal Edema / etiology
  • Self Administration
  • Subcutaneous Tissue


  • Complement C1 Inhibitor Protein
  • Complement C4
  • Complement Inactivating Agents