Ανασκόπηση: Κληρονομικό αγγειοοίδημα

Cicardi M, Johnston DT.

Hereditary and acquired complement component 1 esterase inhibitor deficiency: a review for the hematologist.

Acta Haematol. 2012;127(4):208-20.

Abstract

Hereditary angioedema (HAE), a rare autosomal dominant disorder, was first described in the late 19th century. The disease remained poorly understood and without therapeutic options until the latter half of the 20th century. Advances in the understanding of immunologic and hematologic pathways have shed light on HAE, a disease characterized by painful and unpredictable recurrent attacks of nonpitting edema without urticaria. Recognition that a deficiency of complement component 1 (C1) esterase inhibitor leads to overproduction of vasoactive kinins that cause angioedema paved the way for the development of early treatments. Increased understanding of the role of bradykinin in hereditary and acquired forms of C1 esterase inhibitor deficiency has led to the development of more targeted treatments for this painful, debilitating and potentially life-threatening disease.

Copyright © 2012 S. Karger AG, Basel.

Μία πολύ ενδιαφέρουσα ανασκόπηση για το κληρονομικό αγγειοίδημα η οποία παρότι απευθύνεται στον αιματολόγο είναι πολύ χρήσιμη και για τον αλλεργιολόγο.

Η πρόσβαση στο άρθρο είναι ελεύθερη από την ιστοσελίδα του εκδότη:

http://content.karger.com/produktedb/produkte.asp?doi=336590