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  Authority of Polish Society of Allergology

vol 4. no 1. January 1999  

 Original articles
Two families with hereditary angioedema - effectiveness of treatment with danazol
Anna Ciebiada1, Mariola Kubicka2 , Marek L. Kowalski2,3

1)Studenckie Koło Naukowe przy Katedrze i Zakładzie Immunologii Klinicznej
2)Poradnia Immunologii Klinicznej i Reumatologii przy Katedrze Immunologii AM
3)Katedra i Zakład Immunologii Klinicznej Akademii Medycznej, ul. Mazowiecka 11, 92-215 Łód

Hereditary angioedema (HAE) is characterized by recurrent epizodes of oedema involving any part of the body resulting from the absence of functional C1 inhibitor. The authors present two families with HAE including 6 and 2 affected members, respectively. A characteristic "family specific" clustering of symptoms were observed in each family. Treatment with danazol on a chronic basis resulted in significant relief of symptoms in 4 patients. No correlation between clinical improvement and serum C1 - inhibitor levels was observed, although C1 - inhibitor levels correlated with serum C4 levels.


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