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Özet

Primary immunodeficiencies (PID) are hereditary disorders of immune system that increase the susceptibility to either infectious or non-infectious complications related to the immune system. Most of the patients are diagnosed in childhood period. Therefore, family doctors and pediatricians are responsible for recognizing PID at an early stage. Especially live vaccines should not be applied to these patients until the diagnosis is confirmed. Some warning situations are recently defined for the early diagnosis of PID. Traditional treatments of immunodeficiency include specific and supportive treatments as well as support for the immune system and treatments of developing infections and complications. Currently, there are also curative treatments for PID available, such as allogeneic stem cell transplantation, thymus transplantation and gene therapy.