These congenital disorders are defects in the various pathways of immune system which may present in the first year of life or later in childhood.
The immune system is an organized array of various hematopoietic cells like B, T, NK cells and various cytokines which protect us from different microbial infections. The primary immune deficiencies (PID) may have various clinical manifestations like recurrent infections, failure to thrive and frequent hospitalization. In addition to infections, they may manifest with other clinical features like edema, cellulites, urticaria, rheumatological problems like systemic lupus erythematosus (SLE), arthritis etc.
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