What is the treatment for WAS syndrome

WAS syndrome is still a very rare disease with a very low incidence rate. However, although the incidence rate is very low, it does not mean that it does not exist. If it occurs, there is no good treatment method at all. This is why many patients suffer from various complications. These symptoms, to a certain extent, cause considerable harm to the patient's body. Therefore, antibacterial treatment must be taken in time after diagnosis.

IVIG

Intravenous immunoglobulin (IVIG)

The purity of immunoglobulin G is over 98%, with the main component being IgG monomer molecules and a trace amount of IgA components. Used to treat XLA, common variable immunodeficiency, immunoglobulin G subclass deficiency, various secondary immunoglobulin deficiencies and certain autoimmune diseases.

Selection Principles

1. Typical WAS patients need to start using antibiotics to prevent Pneumocystis infection after diagnosis.

2. IVIG can be used in cases of frequent or severe infections to maintain serum IgG at normal levels.

3. When an active infection exists, sensitive antibiotics should be used to eliminate the infection as quickly as possible.

Liquid or lyophilized powder injection: 2.5g or 5g/bottle.

Usage: The lyophilized powder injection should be dissolved in water for injection to the specified volume (dissolve to 50ml for 2.5g/bottle and dissolve to 100ml for 5g/bottle). Generally, it is infused once every 3-4 weeks, 400-600 mg per kilogram of body weight. The dosage should be individualized to control the infection, relieve the child's symptoms, and achieve normal growth and development.

Side effects and precautions:

1. Some patients experience transient headaches, palpitations, nausea and other reactions during infusion, which may be related to too fast infusion or individual differences. Most of these reactions are mild and often occur within 1 hour after infusion. Therefore, the patient should be closely observed and the infusion rate should be slowed down or suspended if necessary. The symptoms will generally resolve on their own without any treatment.

2. This product is for intravenous infusion only and should be infused alone. It is prohibited to mix it with other drugs for infusion.

3. The product should be a colorless clear liquid after dissolution. If there are foreign matter, flocs or precipitation, it should not be used. After dissolution, it should be infused all at once and should not be divided into several times or given to others.

4. Children with severe acid-base metabolism disorders should use this medicine with caution.

5. Store below 10°C away from light.

6. It is contraindicated for those who are allergic to human immunoglobulin or have a history of other severe allergies. It is not suitable for those with selective IgA deficiency or IgA antibody deficiency.

Usage: Generally, 400 mg per kg is used for the first time, and the dose can be adjusted according to serum IgG levels and clinical improvement.