Hereditary angioedema in childhood: a challenging diagnosis you cannot afford to miss

Pediatr Dermatol. 2013 Jan-Feb;30(1):94-6. doi: 10.1111/j.1525-1470.2011.01675.x. Epub 2012 Jan 26.

Abstract

Hereditary angioedema (HAE) is a rare inherited disease that is often difficult to diagnose. We report a case of a 9-year-old boy with a spontaneous mutation causing HAE, diagnosed after a life-threatening episode of angioedema of the head and upper respiratory tract after a 5-year history of recurrent skin swellings and abdominal pain leading to several hospital admissions. The aim of this report is to direct focus on this rare disease, which can be treated effectively, to diminish morbidity and mortality of children suffering from undiagnosed HAE.

Publication types

  • Case Reports

MeSH terms

  • Angioedemas, Hereditary / diagnosis*
  • Angioedemas, Hereditary / drug therapy
  • Angioedemas, Hereditary / genetics*
  • Child
  • Complement C1 Inhibitor Protein / genetics*
  • Critical Illness / therapy
  • Delayed Diagnosis
  • Emergency Service, Hospital
  • Gene Expression Regulation
  • Humans
  • Male
  • Mutation
  • Rare Diseases
  • Recurrence
  • Risk Assessment
  • Tranexamic Acid / therapeutic use

Substances

  • Complement C1 Inhibitor Protein
  • Tranexamic Acid