Hereditary angioedema was on the agenda of MasterChef viewers. MasterChef competitor Alican Sabunsoy, who was diagnosed with congenital hereditary angioedema, stated that he had attacks from time to time and that his nose affected him. After this development, the curiosity about hereditary angioedema began to be investigated. Here is detailed information about hereditary angioedema.
WHAT IS HERDITARY ANGIOEDEMA?
Hereditary angioedema is a disease that is located asymmetrically and progresses with swelling (edema) in our skin and internal organs without urticaria (hives). Instead of itching, the feeling of pain and tension is in the foreground. Hereditary angioedema occurs by mechanisms different from the development of allergic diseases such as urticaria (hives). Hereditary angioedema occurs when the amount of “C1 esterase inhibitor” in the blood is low or its function is impaired. In the absence of C1 inhibitor or when it cannot function well, bradykinin, which has a very effective vasodilating effect, increases in the body.
Hereditary Angioedema is a hereditary disease. While the C1 inhibitor level is low in the majority of the patients (type I), the C1 inhibitor is functionally inadequate in a few patients (type II). Although a new type (C1 inhibitor is normal) associated with the mutation of the coagulation system Factor XII and some other genes (plasminogen, kininogen, etc.) has been defined in recent years, its mechanisms have not been fully elucidated.
WHAT ARE THE SYMPTOMS OF HEREDITER ANGIOEDEMA?
Hereditary Angioedema findings usually appear in the first years. Recurrent edema is usually more common in the face, lips, mouth, throat, trachea, hand-arm-leg, genital area. The frequency, severity, and affected organs of attacks may differ between patients. The attacks of patients with early symptoms may be more severe. In some patients, the severity of hereditary angioedema findings may increase during adolescence. In some patients, the attacks can last for several days and go away on their own even if untreated, in some patients they can be severe enough to require treatment in the emergency room. The severity of attacks can vary even in the same patient. Attacks may be more severe in the first and last stages of pregnancy.
Hereditary angioedema findings occur depending on the organs affected. Swelling in different parts of our body (face, lips, mouth, throat, hands, arms, legs and genitals) may occur. Nausea and vomiting, abdominal pain (cramp-like) without skin manifestations may complicate the diagnosis. It can be confused with appendicitis and familial Mediterranean fever. Difficulty in breathing and edema of the upper respiratory tract (when the windpipe is blocked) can threaten your life.
IS THERE A TREATMENT FOR HERDITARY ANGIOEDEMA?
Antihistamines, corticosteroids and adrenaline have no place in the treatment of hereditary angioedema.
Prevention of disease attacks;
It is to explain the precautions to prevent the factors that trigger the attacks to the patients and their relatives. The danger of tracheal edema that may develop and pharyngeal interventions such as tooth extraction should be clearly explained. It should be emphasized that birth control pills containing estrogen-containing hormones should be avoided, as some drugs can trigger attacks.