Primary immunodeficiency disorders in infancy

Certain primary immunodeficiency disorders may become evident during the first year of life. These disorders are characterized by recurrent infections. Prompt diagnosis is necessary, for infants may suffer significant morbidity and mortality. A basic knowledge of host defense mechanisms, including the natural and humoral systems, is essential to the understanding of immunodeficiency disorders. This article discusses the clinical presentation, evaluation, and treatment strategies available to infants with primary immunodeficiency disorders. Five specific disorders are discussed in detail, and specific treatments are reviewed.