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

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Abstract

Hereditary angioneurotic oedema (HANO) is an autosomal dominant disorder caused by a deficiency of the inhibitor protein C1-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.

Original languageEnglish
Pages (from-to)971-974
Number of pages4
JournalActa Paediatrica, International Journal of Paediatrics
Volume91
Issue number8
DOIs
Publication statusPublished - 2002

Keywords

  • Ascites
  • C1-esterase inhibitor concentrate
  • Hereditary angioneurotic oedema
  • Sonographic liver abnormalities

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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