MANAGEMENT OF PRIMARY IMMUNODEFICIENCY
Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. [Epub ahead of print September 11, 2015]. doi: 10.1016/j.jaci.2015.04.049.
This practice parameter is intended to provide practical guidance on the clinical recognition and diagnosis of primary immunodeficiency (PIDD), along with general principles on management of these disorders. Highlights include
• PIDD has a prevalence of 1:2,000 children.
• PIDD is subdivided into humoral or antibody deficiencies and combined immunodeficiency.
• Initial evaluation is guided by the clinical presentation, and screening tests are applied and followed by advanced tests, ensuring efficient and thorough evaluation of mechanisms of immune dysfunction that underlie the clinical presentation.
• Diagnosis and therapy should be guided overall or performed in consultation with persons and centers with knowledge and experience diagnosing and treating a broad range of immunodeficiencies.
COMMENTARY
Clinicians should be aware of PIDD in order to refer appropriate patients on to an allergist/immunologist for further evaluation. While many different types of PIDD exist, they generally present with recurrent or severe infections or infections by unusual organisms. For example, approximately a quarter of patients older than 2 with invasive pneumococcal disease have an identifiable PIDD. This is a group of disorders that, while rare, is helpful to be aware of and to refer on for further evaluation when indicated.
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