Hereditary angioedema is a genetic disease that causes symptoms such as swelling in the body, especially in the face, hands, feet and genitals, and recurrent abdominal pain with nausea and vomiting. In some cases, the swelling can also affect organs such as pancreas, stomach and brain.
These symptoms generally appear before 6 years of age and swelling bouts last about 1 to 2 days, while abdominal pains take up to 5 days. In addition, the disease can stay for long periods without causing problems or discomfort to the patient, until new crises arise.
Hereditary angioedema is a rare disease, which may arise even when there is no history in the family of this problem. It is classified into 3 angiedema type 1, type 2 and type 3, according to the affected protein in the body.
Diagnosis
The diagnosis of the disease is made from the symptoms and a blood test that measures the protein C4 in the body, which is at low levels in cases of hereditary angiedema.
In addition, the physician may also ask for the quantitative and qualitative dosage of C1-INH, and it may be necessary to repeat the tests during a disease crisis.
Treatment
The treatment of hereditary angioedema is done according to the severity and frequency of the symptoms, and hormone-based medications such as danazol, stanazolol and oxandrolone, or antifibrinolytic medicines such as epsilon-aminocaproic acid and tranexamic acid, may be used. prevent new crises.
During crises, the doctor may increase the dose of medications and also recommend the use of medicines to combat abdominal pain and nausea.
However, if the crisis causes swelling in the throat, the patient should be taken immediately to the emergency room, as the swelling can block the passage of air and prevent breathing, which can lead to death.
Complications
The main complication of hereditary angioedema is swelling in the throat, which can cause death by asphyxiation. In addition, when swelling occurs in the pancreas and in the, the disease can also impair the proper functioning of these organs.
Some complications may also occur due to the side effects of the medicines used to control the disease, and problems may arise such as:
- Weight gain;
- Donate head;
- Mood alterations;
- Increased acne;
- Hypertension;
- High cholesterol;
- Menstrual changes;
- Blood in the urine;
- Problems in the liver.
During treatment, patients should have blood tests every 6 months to check liver function, and children should be screened every 2 to 3 months, including abdominal ultrasonography every 6 months.
What to do during pregnancy
During pregnancy, patients with hereditary angiedema should discontinue the use of medications, preferably before becoming pregnant, as they may cause malformations in the fetus. If crises occur, treatment should be done according to your doctor's advice.
During normal birth, the onset of seizures is rare, but when they appear, they are usually rare. In case of cesarean delivery, it is recommended to use local anesthesia only, avoiding general anesthesia.
To help relieve swelling, see also:
- Foods to Fight Puffiness
- Home Remedies for Swelling
