Immunodeficiencies are no longer considered rare conditions. Although susceptibility to infections has become well-recognized as a sign of most primary immunodeficiencies, some children will present with noninfectious manifestations that remain underappreciated and warrant evaluation by an immunologic specialist. Providers must also consider common secondary causes of immunodeficiency in children.

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Immunodeficiency disorders represent defects in the immune system that result in weakened or dysregulated immune defense. These deficiencies may occur as primary diseases or secondary conditions. This article reviews primary and secondary immunodeficiencies, with particular emphasis on the evaluation and management of children with primary immunodeficiencies (PIDs).

PIDs constitute inherent defects in immunity, most of which arise from inborn deviations in the genetic code. More than 300 PIDs have been identified. These conditions have been placed into 9 categories corresponding with their clinical and immunologic phenotypes by an international group of experts...

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