Diagnostic criteria of hypogammaglobulinemia in infancy

Of all of the primary immunodeficiency diseases, those affecting B cell function are the most frequent. Selective absence of serum and secretory IgA is the most common defect, with rates ranging from 1/333 persons to 1/16.000 among different race. By contrast, it has been estimated that hypo/agammaglobulinemia occurs with a frequency of only 1/50.000 persons. Patients with antibody deficiency are usually recognized because they have recurrent infections with encapsulated bacteria or a history of failure to respond to antibiotic treatment, but some individuals, mainly those affected by IgA deficiency or transient hypogammaglobulinemia of infancy, may have few or no infections.