This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.Background: Hereditary angioedema is a rare inherited disease with symptoms of swelling of your arms, legs, face, throat, and/or abdomen. Hypothesis: An inhibitor of C1 esterase would be expected to reduce symptoms of hereditary angioedema and to stop the progress in patients having acute attacks.
Specific Aims and Procedures (summary): During the study you may receive 1 of 4 doses of DX-88. It will be given through an infusion over 10 minutes. The amount given is based on your body surface area, calculating your height and weight. Experimental Design (summary): Initially, 60 attacks in up to 60 patients will be studied at approx. 50 clinical centers in the United States and Europe. This may be increased to up to 240 attacks. Up to 2 doses of DX-88 will be given per attack. You can be treated for multiple attacks in this study. Significance (summary): There are currently no drugs approved to stop an attack of hereditary angioedema in the US and some European countries. The drugs that are available treat symptoms only.
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