부산대학교 도서관

Introduction: Hereditary angioedema is a rare autosomal dominant inherited disease characterized by recurrent episodes of angioedema involving the skin, gastrointestinal tract and upper respiratory tract mucosa, often without urticaria. Case: A 36-year-old female patient, known to have hereditary angioedema type-3, presented to the emergency department with abdominal pain, urticaria starting from the lower extremities and non-existing edema. Patient was taken to the intensive care unit due to the development of edema in the face, regression in consciousness, and respiratory distress. patient started with recurrent urticaria and then hiccups developed in the follow-up. 1 mg/kg methylprednisolone and tranexamic acid were administered. Due to the lack of C1 esterase inhibitor solution in the hospital, 2 units of fresh frozen plasma (FFP) was administered to the patient. Attacks regressed after FFP and steroid applications. On the 3rd day of the follow-up, the patient without symptoms was discharged. DISCUSSION: Angioedema attacks may occur spontaneously, but trauma, anxiety, menstruation, infections, alcohol, pregnancy, or medications such as estrogen and ACE inhibitors may also trigger attacks. Data on the efficacy of relapse treatment in HAE with normal C1-INH are limited by studies and case reports. Icatabant acts by antagonizing the bradykinin-2 receptor. C1-INH concentrate acts by raising the threshold for kallikrein activation and bradykinin formation. In the absence of C1-INH concentrate, it is recommended to give 1-2 units of FFP within 1-2 hours. Antifibrinolytic drugs also have a place in the short- and long-term profile axis of hereditary angioedema. For this purpose, tranexamic acid and epsilon amino caproic acid are used. However, the mechanisms of action are not fully known. HAE patients with normal C1-INH are not expected to respond to epinephrine, glucocorticoids, and antihistamines. In cases histaminergic angioedema is suspected to accompany the picture, they are recommended to be applied without delay [ABSTRACT FROM AUTHOR]