Hereditary angio-oedema: Long-term prophylaxis with danazol

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• 作者：S. Hammami^a ; ^{hamami_sabeur@yahoo.fr"" rel=""nofollow} ; K. Harrathi^b ; S. Hadded^a ; L. Ghé ; dira Besb&#xe8 ; s^a ; A. Elkorbi^b ; S. Chouchane^a ; Ch. B. Meriem^a ; A. Gassab^b ; M.N. Guediche^a
• 关键词：Hereditary angio-oedema ; Children ; Laryngeal oedema ; Danazol
• 刊名：Respiratory Medicine CME
• 出版年：2011
• 出版时间：2011
• 年：2011
• 卷：4
• 期：1
• 页码：15-16
• 全文大小：92 K

文摘

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.