Recurrent abdominal pain as the sole manifestation of hereditary angioedema in multiple family members

Gastroenterology. 1987 Nov;93(5):1116-8. doi: 10.1016/0016-5085(87)90576-2.

Abstract

This paper describes a previously unreported finding of abdominal pain as the only lifelong manifestation of hereditary angioedema in multiple family members. This diagnosis was obscured by the absence of cutaneous, oropharyngeal, and respiratory involvement. Barium studies performed during painful attacks showed transient intestinal wall edema which, along with abnormalities in the C4 level and C1 esterase inhibitor activity, confirmed the diagnosis. It is important that hereditary angioedema be recognized in its various forms so that invasive procedures can be avoided and prophylactic therapy can be administered.

Publication types

  • Case Reports

MeSH terms

  • Abdomen*
  • Adult
  • Angioedema / complications
  • Angioedema / genetics*
  • Complement C1 Inactivator Proteins / deficiency
  • Complement C4 / deficiency
  • Genes, Dominant
  • Humans
  • Male
  • Pain / etiology*
  • Recurrence

Substances

  • Complement C1 Inactivator Proteins
  • Complement C4