title: [Recombinant human C1-inhibitor is effective in the treatment of acute attacks of hereditary angioedema--case report].
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.
authors.affiliation: Zaklad Alergologii Klinicznej i Srodowiskowej, Collegium Medicum Uniwersytetu Jagiellońskiego, Kraków. mmobtulo@cyf-kr.edu.pl
authors.forename: Grzegorz
authors.initials: G
authors.lastname: Porebski
authors.forename: Barbara
authors.initials: B
authors.lastname: Bilo
authors.forename: Krystyna
authors.initials: K
authors.lastname: Obtułowicz
authors.forename: Piotr
authors.initials: P
authors.lastname: Obtułowicz
authors.forename: Jan
authors.initials: J
authors.lastname: Nuijens
mesh_terms: D000208:Acute Disease; D000328:Adult; D000799:Angioedema / Q000235:genetics / Q000628:therapy*; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011044:Poland; D011994:Recombinant Proteins / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D050718:Complement C1 Inhibitor Protein; D011994:Recombinant Proteins