is a dysfunction of the immune system, namely the low capacity of the formation of blood clots. This leads to a decrease in the number and size of blood cells involved in blood clotting. This anomaly of the blood is manifested in the body from birth and often affects only the male gender.
The main cause of disease is mutation in the WASp gene, which is located in the short arm of the X chromosome. Because of this mutation, in the human body is not produced or is produced in a very small amount of the protein WASp. Its absence leads to unpleasant symptoms, as protein is an important part in the interaction between cells of the immune system.
Fortunately, the percentage to the disease at birth is small enough. The syndrome occurs in 5-10 births per 1 million people. The geographical factor is not affected. And as noted earlier, women are not affects of this disease. However, they can be heterozygous carriers for life no single symptom does not manifest itself, but in the following generations affected.
The most frequently manifested skin problems in a variety of dermatitis and eczema. Due to lower levels of platelets can cause bleeding. It can be as external (cuts, abrasions, bleeding gums) and internal (diarrhea with blood, subcutaneous hemorrhage, hematomas). Because of the small number of lymphocytes, children become susceptible to frequent colds, viruses and infections.
The first point from which to start is insulation. The child must be placed in a chamber box, which is under the supervision of doctors, he will be in an aseptic atmosphere and to immune therapy. This is followed by transplantation of a bone brain. Then follows the healing of new tissue and recovery.
There is also a treatment with antibiotics and other drugs, used blood transfusion and treatment for eczema (ointment and creams).
Currently actively working on the development of gene therapy. It will allow you to enter in the body of the desired genes with the help of intermediaries and use them to replace affected cells.