Medical Encyclopedia

Agammaglobulinemia

What is agammaglobulinemia?

Approximately 40% of the proteins are globulins. Gamma globulin is particularly important for the immune system of the human body; it is produced by the lymphatic system. It contains antibodies that serve a protective function, eliminating foreign substances and cells. Scientists have identified five subgroups gammaglobulin. Immune reactions are possible only when the plasma contains sufficient gammaglobulin. Reduction of these proteins leads to a weakening of the functions of the defense system of the body and the development of the disease, which is called the lack of antibody syndrome or hypogammaglobulinemia. If the body does not produce gamma globulin, then there is an agammaglobulinemia. The disease belongs to the immunodeficiency.

Symptoms of agammaglobulinemia

  • Low resistance to infectious diseases.
  • The frequent incidence of middle ear infections, sinusitis, pneumonia.

Causes of agammaglobulinemia

Agammaglobulinemia appears due to a sharp reduction of gammaglobulin or abnormal enlargement of the lymph vessels. A great loss of blood can also be a cause of the disease. Congenital agammaglobulinemia is less common. There are many forms of agammaglobulinemia. At the end of pregnancy, the fetus begins to receive immunoglobulins from the mother through the placenta that provide passive immunity of the baby after the birth. They persist for about 3-4 months. After that, the baby starts to develop its own immunoglobulins: some are formed in the first year of life, others - as a teenager. In some children this process is slow, and they are sick of inflammatory and infectious diseases more often than other children. The first symptoms of transient hypogammaglobulinemia appear at 3-6 months of age and are observed up to 6-18 months. However, almost 100% of patients recover completely. If the lymphatic system is weakened or underdeveloped, then the globulins are not formed, and the child dies in the first years of life.

Treatment of agammaglobulinemia

Agammaglobulinemia

Today, the lack immunoglobulins are most often replaced by other, which are isolated from human plasma. Thus, those substances, that the body is not enough, you can enter it. In some forms of congenital or acquired hypo-or agammaglobulinemia treatment is to stimulate the production of immunoglobulins in the patient. In severe cases, children for some time have to live in a sterile environment in the special wards. Infectious diseases, which is caused by the weakening of immunity, are treated with antibiotics and other medications.

If the child is very weak and often sick infectious diseases, it can be assumed that he has a lack of antibody syndrome. However, the disease is extremely rare, so the causes of immunodeficiency may be different.

Diagnosis is based on careful analysis of the patient's blood. Depending on the severity of the disease, the child is treated as an outpatient or inpatient. Treatment is carried out to the full restoration of the immune system. Adults with agammaglobulinemia are treated for a similar scheme.

Course of the disease

If untreated, patients die because the body can not fight viruses. Very often patients suffer from a viral inflammation of the middle ear, sinusit, bronchial tubes, lungs and meninges. Viruses are often caused by infections of the digestive tract. Sometimes there is a so-called selective immunological globulinemia, which is characterized by deficiency of one of immunoglobulins such as immunoglobulin A. In this case, the child is subject to frequent upper respiratory infections and other illnesses.

People who suffer from a lack of antibody syndrome, vaccinations are contraindicated against polio, measles, mumps, and rubella as vaccine can cause the very disease that should prevent.

Other articles:

Anemia

Hyperemia