Diagnosis and management of hereditary angioedema: an American approach.
Hereditary angioedema (HAE) is an autosomal dominant disease characterized by recurrent episodes of angioedema, and caused by a deficiency of the plasma protein C1 inhibitor. HAE attacks carry a substantial risk of morbidity or even mortality, making it imperative that the correct diagnosis be established and an appropriate management plan be in place. This report reviews the current diagnostic and therapeutic approaches available in the United States. Areas in which the diagnostic or therapeutic tools are deficient are discussed, and the prospects for improved therapeutic modalities highlighted.
Zuraw
Bruce L
BL
Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10666 N. Torrey Pines Road, La Jolla, CA 92037, USA. zuraw@scripps.edu
D000208:Acute Disease; D015119:Aminocaproic Acid / Q000378:metabolism; D000799:Angioedema / Q000175:diagnosis* / Q000235:genetics / Q000517:prevention & control / Q000628:therapy*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D013997:Time Factors; D014481:United States
D003174:Complement C1 Inactivator Proteins; D015119:Aminocaproic Acid