The Clinical Presentation of the Primary Immunodeficiency Diseases a Primer for Physicians
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چکیده
The primary immunodeficiency diseases are a heterogeneous group of over 50 disorders which affect the cells, tissues and proteins of the immune system. These disorders were originally felt to be rare, to occur only in infants and young children and to be associated with severe clinical symptoms. However, as clinical experience with the primary immunodeficiency diseases has grown, it has become clear that they are much more common than originally appreciated, that they can be present in older children, adolescents, and adults, and that they can be associated in some patients with relatively mild clinical disease. The early detection of patients with primary immunodeficiency diseases is critically important. Effective therapy is available for virtually all of the different disorders, but is most beneficial when instituted before there has been damage to target organs (e.g. the lung) by infection or autoimmune disease. Similarly, early recognition of primary immunodeficiency may lead to a precise genetic diagnosis which in turn may be important to the family in planning their future reproductive options. This Primer has been written in an effort to alert primary care physicians to the signs and symptoms of primary immunodeficiency diseases in the hope that these patients will be diagnosed earlier in the course of their illness, allowing for more effective therapy and improved prognosis.
منابع مشابه
Primary immunodeficiency diseases (PIDs) is a diverse group of diseases, characterized by a defect in the immune system. These patients are susceptible to recurrent respiratory infections, gastrointestinal problems, autoimmune diseases, and malignancies. In most cases, patients with primary immunodeficiency disorders have genetic defects and are monogenic disorders that follow a simple Mendelia...
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