Content
Hereditary angioedema is a genetic disease that causes symptoms such as swelling throughout the body, and recurrent abdominal pain that can be accompanied by nausea and vomiting. In some cases, the swelling can also affect organs such as the pancreas, stomach and brain.
In general, these symptoms appear before the age of 6 and swelling attacks last for about 1 to 2 days, while abdominal pain can last for up to 5 days. The disease can remain for long periods without causing problems or inconvenience to the patient, until new crises arise.
Hereditary angiedema is a rare disease, which can arise even when not in the family of this problem, being classified into 3 types of angiedema: type 1, type 2 and type 3, according to the protein affected in the body.
What symptoms
Some of the most common symptoms of angioedema are swelling throughout the body, especially in the face, hands, feet and genitals, abdominal pain, nausea, vomiting and, in more severe cases, swelling of organs such as the pancreas, stomach and brain.
Possible causes
Angioedema is caused by a genetic mutation in a gene that produces a protein related to the immune system, leading to the appearance of swelling whenever the body's immune system is activated.
Crises can also be exacerbated in the event of trauma, stress, or during physical exercise. In addition, women are more susceptible to crises during menstruation and pregnancy.
What complications can arise
The main complication of hereditary angiedema is swelling in the throat, which can cause death from suffocation. In addition, when swelling of certain organs occurs, the disease can also impair its functioning.
Some complications can also occur due to the side effects of the drugs used to control the disease, and problems such as:
- Weight gain;
- Headache;
- Changes in mood;
- Increased acne;
- Hypertension;
- High cholesterol;
- Menstrual changes;
- Blood in the urine;
- Liver problems.
During treatment, patients should have blood tests every 6 months to assess liver function, and children should have tests every 2 to 3 months, including an abdominal ultrasound scan every 6 months.
What is the diagnosis
The diagnosis of the disease is made from the symptoms and a blood test that measures the C4 protein in the body, which is at low levels in cases of hereditary angiedema.
In addition, the doctor may also order the quantitative and qualitative dosage of C1-INH, and it may be necessary to repeat the tests during a crisis of the disease.
How the treatment is done
The treatment of hereditary angiedema is done according to the severity and frequency of symptoms, and hormone-based medications, such as danazol, stanazolol and oxandrolone, or antifibrinolytic remedies, such as epsilon-aminocaproic acid and tranexamic acid, can be used. preventing new crises.
During crises, the doctor may increase the dose of medication and also recommend the use of drugs 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 airway and prevent breathing, which can lead to death.
What to do during pregnancy
During pregnancy, patients with hereditary angiedema should discontinue the use of medications, preferably before becoming pregnant, as they can cause malformations in the fetus. If crises arise, treatment should be done according to the doctor's guidance.
During normal birth, the onset of attacks is rare, but when they do appear, they are usually severe. In the case of cesarean delivery, only the use of local anesthesia is recommended, avoiding general anesthesia.
Created by: Tua Saúde Editorial Team