Wiskott-Aldrich syndrome refers to hereditary ailments that only boys suffer from. It appears already in early childhood and, as a rule, leads to death in five to ten years.
This disease was first described in 1937year German pediatrician Viscott. He observed it from three siblings, who suffered from persistent bloody diarrhea, eczema and constant ear inflammation, although the four sisters were absolutely healthy. Already in 1954, an American pediatrician named Aldrich established that the disease is inherited as an X-linked recessive trait.
The disease is transmitted to the boys, and the carriers of the mutating chromosome are women. The geographical factor is not affected by the prevalence of the problem.
Wiskott-Aldrich syndrome is associated with a decreasednumber of platelets. In the first years of life, the patient exhibits the following symptoms: poor blood clotting, uninterrupted eczema, bloody diarrhea. Subsequently, there is a primary immunodeficiency state. Because of a lack of T and B lymphocytes, a person is susceptible to all sorts of viral and bacterial diseases. Immunodeficiency conditions in children provoke a constant inflammation of the middle ear, pneumonia, sinusitis, dropsy and many other serious diseases. In patients with this disease, the risk of oncology is much higher. It should be noted that adults are more likely to develop malignant tumors.
У страдающих синдромом Вискота-Олдрича очень often only hemorrhagic syndrome is observed, which leads to incorrect diagnosis. In such cases, only after the DNA analysis, at which it is possible to identify the gene responsible for this syndrome, prescribe the correct treatment.
Unfortunately, modern science has not yet foundmedicines for this serious illness. It is known that platelets are destroyed in the spleen, therefore, in the patients who were diagnosed with the "Wiskott-Aldrich Syndrome", this organ was removed. And the patients began to feel much better. Continuous transfusion of immunoglobulin and the administration of appropriate antibiotics also improves the overall condition of the patient. At present, the introduction of healthy stem cells into the bone marrow is practiced. But while this method is carried out only experimentally. Also, couples who have had this syndrome in the family are advised to take all necessary tests before planning pregnancy.
