Hereditary angio-oedema: Long-term prophylaxis with danazol

Hereditary angio-oedema is a rare autosomal dominant disease characterized by recurrent attacks of subcutaneous oedema and caused by a deficiency of the plasma protein C1 inhibitor. Hereditary angio-oedema attacks carry a high risk of morbidity or even mortality. In literature, there are only a few...

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Bibliographic Details
Published inRespiratory medicine CME Vol. 4; no. 1; pp. 15 - 16
Main Authors Hammami, S., Harrathi, K., Hadded, S., Besbès, L. Ghédira, Elkorbi, A., Chouchane, S., Meriem, Ch. B., Gassab, A., Guediche, M.N.
Format Journal Article
LanguageEnglish
Published Elsevier Ltd 2011
Subjects
Children
Danazol
Hereditary angio-oedema
Laryngeal oedema
Hereditary angio-oedema
Laryngeal oedema
Children
Danazol
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Summary:Hereditary angio-oedema is a rare autosomal dominant disease characterized by recurrent attacks of subcutaneous oedema and caused by a deficiency of the plasma protein C1 inhibitor. Hereditary angio-oedema attacks carry a high risk of morbidity or even mortality. In literature, there are only a few publications on the treatment and follow-up of paediatric patients. We report the case of a 14-year-old male with known hereditary angio-oedema since the age of six years, revealed by a recurrent acute abdominal pain and oedema in subcutaneous tissues of extremities and face, and he was admitted once for sub mucosal oedema of larynx with suffocation, treated by fresh-frozen plasma with favourable outcome. Danazol was used for long-term prophylaxis. Clinical course improved over a follow-up of 12 months.
ISSN:1755-0017
1755-0017
DOI:10.1016/j.rmedc.2010.01.006