Effective treatment of hereditary angioedema with fresh frozen plasma in an emergency department.
Hereditary angioedema (HAE) is a rarely seen disorder of C1 inhibitor (C1-INH) deficiency usually manifested by non-pruritic swelling of the skin. Acute exacerbations are not sensitive to conventional medications, and C1-esterase inhibitor concentrates are recommended as the first-line therapy. However, fresh frozen plasma is the main treatment alternative in many centers due to the lack of C1-esterase inhibitor concentrates. In this report, we present 3 patients with acute exacerbations of hereditary angioedema who were effectively and safely treated with fresh frozen plasma.
Pekdemir
Murat
M
Department of Emergency Medicine, Kocaeli University Hospital, Izmit, Turkey.
Ersel
Murat
M
Aksay
Ersin
E
Yanturali
Sedat
S
Akturk
Aysun
A
Kiyan
Selahattin
S
D000328:Adult; D000799:Angioedema / Q000150:complications / Q000628:therapy*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency; D050718:Complement C1 Inhibitor Protein; D004636:Emergency Service, Hospital; D005260:Female; D030342:Genetic Diseases, Inborn / Q000628:therapy; D006801:Humans; D010949:Plasma; D015843:Serpins / Q000172:deficiency; D016896:Treatment Outcome
D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human; D015843:Serpins