What is a primary immune deficiency?

Imagine that you cannot hug and kiss your child, having not washed your hands for three minutes and not wearing a special suit. Imagine that he firstly saw the world through the window of the hospital ward. Imagine that your child lives for six months not at home but in hospital ...
Primary immune deficiency – a terrible diagnosis, indicating that the child was born without natural protection.
He is completely unable to fight infections, and what is the outside world for us, for him means a source of danger every minute.
Primary immune deficiency is a congenital immune system disorders that develop as a result of genetic disorders. The disease leads to chronic infection and inflammation of the organs and tissues. Without treatment, children with primary immune deficiency die from infectious complications.
In recent years it has become apparent that the primary immune deficiencies are more common than previously thought. The incidence of primary immunodeficiency in the world is an average of 1:10 000 (total for all forms). According to the Russian Children's Clinical Hospital, Moscow (2009) the frequency of occurrence in Russia - 1:200 000. In Russia 34 forms of primary immunodeficiencies (RCCH, Moscow) are registered.
The primary symptoms of congenital immunodeficiency may not be detected, because it has no unique features. It can manifest itself as a "common disease", such as nose, ears or lungs. There may be gastrointestinal problems or inflammation of the joints. Parents and physicians often do not realize that these problems are created by genetic defects of the immune system. Infections become chronic, creating complications, the patient is "not responding" to the ordinary course of antibiotics. Severe forms of primary immunodeficiency usually occur immediately after birth, or shortly after it.
If a doctor suspected primary immune deficiency, in order to accurately identify the type of pathology a series of complex immunological and genetic analysis are conducted - it helps to determine violations of the immune defense (cellular or humoral) as well as determine the type of mutation that caused the disease.
It is important to understand that the primary immunodeficiency - it's not AIDS (acquired immune deficiency). Although both types of immune deficiencies affect the immune system, primary immune deficiency is a genetic, as it was "embedded" in the body of a person's genes. This is similar to the color of your eyes: you cannot change it. Acquired immunodeficiency (AIDS) is an infectious disease and is caused by the virus.
Treatment of primary immune deficiencies is a difficult task. For the purpose of complex treatment it is necessary to establish the accurate diagnosis with the definition of impaired level of immune protection. In large urban hospitals patients are examined at the recommended clinical criteria, an immunological screening is carried out with subsequent refinement of the diagnosis in the Department of Clinical Immunology, Russian Children's Clinical hospital in Moscow.
With a lack of immunoglobulin a lifetime replacement therapy is conducted with sera containing antibodies or normal donor plasma. In case of infectious complications doctors prescribe antibiotics, antiviral or antifungal medications.
Full reconstruction of children’s immunity with primary immunodeficiency occurs after bone marrow transplantation. The successful operation means that the child's own immune system is formed.