What is primary immunodeficiency?
primary diseases of immunodeficiency are diseases in which part of the immune system is dysfunctional or completely absent. To be considered primary, immune disorder must be the basic cause of the disease itself. This means, for example, that immune dysfunction cannot be caused by drugs or other disorders. As a result, most primary immune disorders are genetic disorders that are diagnosed in very young children. However, there are several types of primary immune disorders and the symptoms may vary somewhat. The primary immune disorders varies in terms of part of the immune system, which is inoperative, and this affects which type of symptoms occurs.
Approximately half of the primary immunodeficiency disorders is caused by a lack of functional antibodies. One example is x-bound agammaglobulinemia, also known asxla. This recessive X -related disorder affects male children, while women with a defective gene are carriers. Children born with XLA are protectedOn the first few months of life antibodies provided by their mother in the uterus and breast milk. Once this protection has been scattered, they begin to suffer from recurring bacterial infections, and there is also a risk of secondary complications such as meningitis.
On the other hand, chronic granulomatous disease is a type of primary disease of immunodeficiency caused by the inability of phagocytes to kill organisms after ingestion. Children with this disorder suffer from recurring infections of very specific types. In particular, they are vulnerable to pneumonia, skin abscesses and skin infections and bone marrow and blood infections.
Another type of primary immunodeficiency affects the cascade of the supplement, the molecular chain reaction is triggered by bacterial infection and results in the formation of complementary proteins. These molecules in conjunction with antibodies help kill invasive bacteria. The complement cascade itself is a relatively small part of the immunAs a result, many people are not diagnosed with shortcomings of supplements until they reach maturity. People with this type of immunodeficiency retain fully functional t cells and B cells and are protected from most infections. In some cases, they may be susceptible to certain types of diseases, including bloodshed infections.
There are eight different classes of primary immune disorders and more than 120 different conditions that fall into one of these categories. Each class of disorder tends to require different treatment to engage different parts of the immune system. For example, XLA is successfully treated with regular doses of protective antibodies to increase passive immunity to infection. Chronic -grannulomatous disease is often treated with prophylactic antibiotics, while some types of lack of supplements do not require any treatment.
very serious immune disorders can be treated by transplantation of stem cells. This treatment is usually reserved for diseases such as a serious combined immunodeficiencies. People with this disorder have no functional cells or B cells and are susceptible to virtually all types of infection. Other types of primary immunodeficiency can be treated in this way, including XLA, but the treatment of stem cells is often not performed in people with XLA unless other treatment options disappoint.