Human pasteurized C1-inhibitor concentrate for the treatment of hereditary angioedema due to C1-inhibitor deficiency.
Hereditary angioedema is a relatively rare genetic disorder affecting between one in 10,000 and one in 50,000 individuals worldwide. The most common clinical symptoms observed are relapsing swelling of the skin and abdominal pain attacks. However, more serious and potentially fatal laryngeal attacks can also occur. Hereditary angioedema is most frequently caused by a deficiency of C1-inhibitor. Replacement therapy with Berinert, an intravenous pasteurized C1-inhibitor concentrate derived from human plasma, is a recommended treatment for rapid resolution of acute attacks of hereditary angioedema due to C1-inhibitor deficiency. Prophylactic therapy with C1-inhibitor is also available. Future advances may improve morbidity and mortality associated with hereditary angioedema.
Department of Dermatology, Johannes Gutenberg University, Langenbeckstr. 1, 55131 Mainz, Germany. bork@hautklinik.klinik.uni-mainz.de
Konrad
K
Bork
D015746:Abdominal Pain / Q000188:drug therapy / Q000235:genetics / Q000401:mortality; D054179:Angioedemas, Hereditary / Q000188:drug therapy* / Q000235:genetics* / Q000401:mortality; D003174:Complement C1 Inactivator Proteins / Q000235:genetics* / Q000627:therapeutic use*; D050718:Complement C1 Inhibitor Protein; D006801:Humans; D007262:Infusions, Intravenous; D061352:Pasteurization
D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human