Acute abdominal attack of hereditary angioneurotic oedema associated with ultrasound abnormalities suggestive of acute hepatitis

Acta Paediatr. 2002;91(8):971-4. doi: 10.1080/080352502760148720.

Abstract

Hereditary angioneurotic oedema (HANO) is an autosomal dominant disorder caused by a deficiency of the inhibitor protein Cl-esterase. Recurrent subcutaneous and/or submucosal oedema formation is a hallmark of this disease. HANO is a rare, but potentially life-threatening disorder with a mortality around 20-30%. Acute oedematous abdominal attacks of HANO can mimic a surgical emergency; this is exemplified by the case of a 14-y-old male patient with HANO admitted for such clinical manifestations.

Conclusion: Diagnostic clues include ascites and abnormalities of hepatic structure visible with ultrasound during the oedematous attack. The importance of appropriate treatment is emphasized.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abdomen, Acute / diagnostic imaging*
  • Abdomen, Acute / etiology*
  • Abdomen, Acute / genetics
  • Acute Disease
  • Adolescent
  • Angioedema / complications*
  • Angioedema / diagnostic imaging*
  • Angioedema / genetics
  • Hepatitis / diagnostic imaging*
  • Hepatitis / etiology*
  • Hepatitis / genetics
  • Humans
  • Male
  • Ultrasonography