1. [Recombinant human C1-inhibitor is effective in the treatment of acute attacks of hereditary angioedema--case report].
- Author
-
Porebski G, Bilo B, Obtułowicz K, Obtułowicz P, and Nuijens J
- Subjects
- Acute Disease, Adult, Angioedema genetics, Complement C1 Inhibitor Protein metabolism, Dose-Response Relationship, Drug, Female, Humans, Male, Middle Aged, Poland, Recombinant Proteins therapeutic use, Treatment Outcome, Angioedema therapy, Complement C1 Inhibitor Protein therapeutic use
- Abstract
Hereditary angioedema (HAE) is a rare condition, resting on attacks of edema in various localizations, potentially life-threatening if in facial, laryngeal, pharyngeal or gastrointestinal area. The disease is caused by deficiency or impaired activity of C1 inhibitor, therefore C1 inhibitor infusion is the the essential treatment and the only efficient method in an acute attack of HAE. Nowadays C1 inhibitor applied in our patients is obtained from human plasma, what restricts the availability of the drug and carries relevant risks. After many years of research it came to the synthesis of the recombinant protein with features of human C1 inhibitor. Its first usage in Poland took place-in two HAE patients with severe edema in 2004. The course and efficiency of this treatment is reported in the paper. Recombinant human C1 inhibitor occurred efficient and safe in presented case of severe angioedema.
- Published
- 2005