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Every year, CREAK organizes a medical science day for network professionals to keep abreast of the latest developments.
Read theAngioedema is a sudden swelling of the skin. There are several types of angioedema, each with a different cause and expression. Depending on its etiology, angioedema may be benign or more severe.
Very common
90% of cases are mild mast cell angioedema. These angioedemas are usually spontaneous and benign. They may be associated with urticaria (red, itchy patches). In very rare cases, they are allergic.
Diagnosis and management is often the responsibility of an angioedema expert.
Rare
They may be genetic in origin or the result of medication. There are different types, including hereditary angioedema. C1-Inhibitor is involved, and is the subject of a special assay
Management is carried out by a specialized center.
Hereditary angioedema is a rare genetic disorder characterized by recurrent attacks of edema (swelling) affecting various parts of the body, such as the face, hands, feet, respiratory tract and digestive tract. The condition is linked to dysfunction or deficiency of C1 esterase inhibitor (C1-INH), a protein that regulates inflammation and vascular permeability.
Symptoms vary from person to person, but the most common are:
- Sudden, painful swellings (e.g. in the face, lips, eyelids, hands, feet).
- Intense abdominal pain, which may be accompanied by nausea, vomiting or diarrhea, when the swelling affects the digestive tract.
- Breathing difficulties (rare but severe), if the larynx or upper airways are affected.
The frequency and severity of attacks can also vary over the course of a lifetime.
Diagnosis is based on:
- Clinical examination: family history of recurrent swelling and absence of itching (unlike allergic reactions).
- Biological tests: determination of the level and function of C1 esterase inhibitor, C4 complement, and sometimes other components of the complement system.
- Genetic testing: can confirm mutation in the gene responsible for C1-INH protein production (mainly the SERPING1 gene).
Early diagnosis is important to adapt follow-up and avoid serious complications, particularly respiratory.
The main aims of AEH treatment are as follows:
- Preventing attacks:Using C1 esterase inhibitor concentrates (long-term prophylaxis in some cases).Using kallikrein inhibitors or bradykinin receptor antagonists.
- Managing acute attacks:Using C1 esterase inhibitor concentrates, kallikrein inhibitors or icatibant (bradykinin receptor antagonist). Manage acute attacks:Administration of C1 esterase inhibitor concentrates, kallikrein inhibitors or icatibant (bradykinin receptor antagonist).
Emergency treatment can be administered at home, after training, to intervene rapidly in the event of severe laryngeal or abdominal oedema.
In addition to drug treatment, regular medical follow-up and information for the patient and family are essential.
In some cases, psychological care can help cope with the emotional and social impact of the disease.
