Management of hereditary angioedema in pediatric patients

Research output: Contribution to journalReview article

109 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)e713-e722
JournalPediatrics
Volume120
Issue number3
DOIs
Publication statusPublished - Sep 1 2007

Keywords

  • C1 inhibitor
  • Danazol
  • Hereditary angioedema
  • Pediatrics
  • Tranexamic acid

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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