Nergis ATAOL, Murat ÖKSÜZ, Feride KORKMAZ, Alaattin ÖZTÜRK

Herediter anjioödemli hastanın anestezi yönetimi

Herediter anjioödem C1 esteraz inhibitör eksikliğine bağlı olarak gelişen otozomal dominant geçiş gösteren nadir bir hastalıktır. Herediter anjioödemli hastalar klinik olarak yüz, gövde, solunum yolları, abdominal organlar ve ekstremiteleri etkileyen, tekrarlayan ödem atakları ile karakterizedir. Ataklar ya spontan olarak ya da travma, stres, cerrahi, enfeksiyon ve hormon düzeylerindeki dalgalanmaları takiben oluşabilir. Larinks ödemine bağlı asfiksi en sık ölüm sebebidir. Tedavisinde C1 esteraz inhibitörü verilmesi en etkili yöntemdir. Tedavisinde ayrıca taze donmuş plazma, androjen steroidler, kinin yolağı inhibitörleri, antifibrinolitikler ve bradikinin reseptör antagonistleri kullanılabilir. Bu yazımızda inguinal herni onarımı yapılacak herediter anjioödemli bir hastaya anestezi yaklaşımını sunuyoruz.

Anaesthetic management of a patient with hereditary angioedema

Hereditary angioedema is a rare autosomal dominant disorder caused by reduced activity of the C1 esterase inhibitor. Patients with hereditary angioedema are clinically characterized by recurrent episodes of swelling of the extremities, face, trunk, airways and abdominal organs. Attacks may occur either spontaneously or following trauma, stress, surgery, infections and hormonal fluctuations. The most common cause of death is asphyxia related to laryngeal edema. Giving C1 esterase inhibitor is the most effective method of treatment. Also fresh frozen plasma, androgen steroids, quinine pathway inhibitors, antifibrinolytics and bradykinin receptor antagonists can be used as treatment. In this paper, the anesthetic management of a patient with hereditary angioedema undergoing inguinal hernia repair surgery is reported.

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Farkas H, Harmat G, Fust G, et al. Clinical management of hereditary angio-oedema in children. Pediatr Allergy Immunol 2002;13:153-161.
Cicardi M, Agostoni A. Hereditary angioedema. N Engl J Med 1996;334:1666-1667.
Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med 2006;119:267-274.
Bork K, Fischer B, Dewald G. Recurrent episodes of skin angioedema and severe attacks of abdominal pain induced by oral contraceptives or hormone replacement therapy. Am J Med. 2003;114:294-298.
Oyaizu T, Kikuchi A, Minoshima R, et al. Anesthesia for total hip arthroplasty in a patient with C1 inhibitor deficiency. Masui 2014;63:820-822.
Cox M, Holdcroft A. Hereditary anjioneurotic oedema: current management in pregnancy. Anaesthesia 1995;50:547- 549.