Hereditary angioedema – patient preparation for surgery
Klinika Anestezjologii i Intensywnej Terapii, Wojskowy Instytut Medyczny w Warszawie.
Kierownik Kliniki: dr n. med. Andrzej Truszczyński
Adres do korespondencji: Klinika Anestezjologii i Intensywnej Terapii CSK MON WIM w Warszawie, ul. Szaserów 128, 04‑141 Warszawa
Praca finansowana ze środków własnych
Pediatr Med rodz Vol 8 Numer 2, p. 165-167
ABSTRACT

Hereditary angioedema (HAE) is a rare condition, first described by Quincke in 1882. Diminished levels of the C1 inhibitor protein of dysfunctional forms of the same protein are basis of this disease pathophysiology. We mark out three types of HAE. Type I, most common, is related with C1 INH insufficiency, type II – with its abnormal function. Mucous oedemas are characteristic for these diseases, including face and larynx oedema. Oedema involving larynx can be life‑threatening condition. HAE swelling can be caused by some drugs, especially from angiotensin convertase inhibitors group, stress, trauma, infections. Surgical procedures can predispose to HAE related larynx swelling. Surgery preparation can involve continuous intubation tube cuff pressure control, doubling androgen based drugs doses as well as transfusion of fresh frozen plasma. Acute HAE swelling management is recently based on cause treatment covering substitution C1 INH. We describeV46 years old patient ongoing gynaecologic surgery with prophylactic C1 INH concentrate application (Cinryze). No complications were observed during surgery, subarachnoid anaesthesia or post‑surgery period.

Keywords: hereditary angioedema, anaesthesia, allergology, C1 inhibitors, premedication