Document Type: Review
Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Primary antibody deficiencies (PADs) are frequent primary immunodeficiencies in humans, characterized by hypogammaglobulinemia, defects in production of specific antibodies, and recurrent infections. Information about PADs is quickly developing, leading to improved diagnoses and efficient disease management. This study is a review of the pathogenesis, diagnosis, clinical manifestations, and management of PAD disorders such as agammaglobulinemia, common variable immunodeficiency, monogenic defects associated with hypogammaglobulinemia, class switch recombination deficiencies, selective IgA deficiency, subclass immunoglobulin isotypes deficiencies, specific antibody deficiency, and transient hypogammaglobulinemia.