This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This is an open label study designed to assess the safety and effectiveness of repeated dosing of DX-88 in patients with heriditary angioedema (HAE). The exact pathophysiology of hereditary angioedema (HAE) is unknown. There are two hypotheses for the mechanism of HAE attacks: 1. Attacks are due to unopposed activation of C 1 with attendant cleavage of the next two components of the complement cascade C4 and C2. 2. Attacks are the consequence of activation of the kinin-generating system, with cleavage of high molecular weight kininogen (HMWK) by activating kallikrein with attendant formation of bradykinin. It is believed that bradykinin is responsible for angioedema attacks. Hereditary Angioedema Previously an ascending dose ranging study of DX-88, has been copmpleted in patients experiencing acute attacks of hereditary angioedema. There was a blinded comparison of the effects of each of four dose groups of DX-88 and placebo on the time to resolution of the attack.
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