Acute abdominal attack of hereditary angioneurotic oedema associated with ultrasound abnormalities suggestive of acute hepatitis
2002; Wiley; Volume: 91; Issue: 8 Linguagem: Inglês
10.1111/j.1651-2227.2002.tb02864.x
ISSN1651-2227
AutoresHenriette Farkas, G Harmat, B. Fekete, István Karádi, Beáta Visy, Lilian Varga,
Tópico(s)Enzyme function and inhibition
ResumoHereditary 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.
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