Recurrent angioedema - a case report

  • Sandrina Martins Paediatric Service of Hospital Santa Luzia, ULS Alto Minho
  • Miguel Salgado Paediatric Service of Hospital Santa Luzia, ULS Alto Minho
  • Filipa Raposo Paediatric Service of Hospital Santa Luzia, ULS Alto Minho
  • Diana Pinto Paediatric Service of Centro Hospitalar do Porto
  • Isabel Martinho Paediatric Service of Hospital Santa Luzia, ULS Alto Minho
  • Ana Rita Araújo Paediatric Service of Hospital Santa Luzia, ULS Alto Minho
Keywords: C1 -INH, children, hereditary angioedema, recurrence

Abstract

Introduction: Hereditary angioedema (HA) is a rare cause of recurrent angioedema caused by a default in the gene that encodes the C1 esterase inhibitor (C1 -INH). The oedema involves predominantly the face, limbs and genital and gastrointestinal tract. The involvement of the larynx, although less frequent, is the most severe clinical expression of HA and is potentially fatal.

Case report: Clinical report of an eight-year-old female with multiple episodes of angioedema. The laboratory study confirmed the diagnosis of HA.

Discussion: HA diagnosis is established based on the clinical history, family history and complements testing. Its documentation is extremely important because it is potentially fatal and needs specific therapy.

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Published
2016-02-23
Section
Case Reports