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JACI Highlights - April 2006
Levi et al – Self-administration of C1-inhibitor concentrate in patients with hereditary of acquired angio-edema due to C1-inhibitor deficiency
C1-inhibitor is a protein which inhibits the aspect of the immune system that causes inflammation. Deficiency of C1-inhibitor leads to recurrent angio-edema attacks (swelling of tissue due to massive leakage of blood vessels) and can be an incapacitating disorder, and may even result in life-threatening situations. The deficiency may be due to an inherited or spontaneously occurring genetic defect. The attacks may occur at various sites of the body but in particular, angio-edema attacks occurring in the orofacial region, the upper airways, and the abdomen need immediate medical attention. Administration of C1-inhibitor concentrate is effective for treatment of severe attacks due to C1-inhibitor deficiency. The concentration is administered intravenously and therefore should be given by a health care professional, which may cause considerable delay in treatment and inconvenience for patients. In the April 2006 issue of the Journal of Allergy and Clinical Immunology, Levi et al investigate the feasibility, efficacy, and safety of on-demand and prophylactic self-administration of C1-inhibitor concentrate in patients with frequent attacks of angio-edema. They observed that patients are capable of self-administration and it resulted in a decreased time between the onset of the attack and the administration of the concentrate. Also, the amount of time for the symptoms to completely disappear was greatly reduced with self-administration. They concluded that intravenous self-administration of C1-inhibitor concentrate is a feasible option for patients who frequently need this treatment and results in more rapid, and potentially more effective, treatment of severe angio-edema attacks in patients with C1-inhibitor deficiency.
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