LOGO
EN

Hereditary angioedema – patient preparation for surgery

Bartosz Rustecki, Anna Rychlik, Agnieszka Żylińska

Affiliacja i adres do korespondencji
Pediatr Med rodz Vol 8 Numer 2, p. 165-167
Streszczenie

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.

Słowa kluczowe
hereditary angioedema, anaesthesia, allergology, C1 inhibitors, premedication

Oświadczam, że posiadam prawo wykonywania zawodu lekarza i jestem uprawniony do otrzymywania specjalistycznych informacji medycznych. Chcę zapoznać się z informacją z serwisu.