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Novel Use Of Fresh Frozen Plasma In Treating Hereditary Angioedema: A Success Story From Pakistan, Amber Sabeen Ahmed, Sidra Fayyaz

Section of Internal Medicine

Hereditary angioedema (HAE) due to a C1-esterase inhibitor(C1-INH) deficiency is a rare and potentially life-threatening disorder. It is characterized by an episodic and self-limiting increase in vascular permeability. The condition manifests itself as recurrent attacks of swelling in any part of the body. The angioedema can cause the involvement of the respiratory tract, skin, and gastrointestinal tract. Laryngeal involvement can make the condition life-threatening. It does not respond well to conventional angioedema therapy of steroids, adrenaline, and antihistamines. The targeted therapy for HAE consists of plasma-derived or recombinant C1-INH, ecallantide, and icatibant or bradykinin receptor antagonist. In the absence of …