Pulmonary and Critical Care
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 these therapies, it becomes difficult to manage this condition effectively. We present a case of hereditary angioedema, who presented with life-threatening laryngeal edema, causing asphyxia, leading to cardiac arrest. Due to a lack of availability of C1-INH concentrate, he was given fresh frozen plasma (FFP). His condition gradually improved, and he was successfully extubated after three days. This is the first time we are reporting a case from Pakistan in which the patient was successfully treated with FFP for an acute attack of hereditary angioedema.
Ahmed, A. S.,
(2020). Novel use of fresh frozen plasma in treating hereditary angioedema: A success story from Pakistan. Cureus, 12(8), e9669.
Available at: https://ecommons.aku.edu/pakistan_fhs_mc_med_intern_med/173
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