Diagnostic approach of hypogammaglobulinemia in infancy

Pediatr Allergy Immunol. 2020 Feb:31 Suppl 24:11-12. doi: 10.1111/pai.13166.

Abstract

Primary B-cell immunodeficiency is the most frequent immune defect in infancy. Selective absence of serum and secretory immunoglobulin IgA is the most common, with rates ranging from 1/333 persons to 1/16 000, among different races. By contrast, it has been estimated that hypo/agammaglobulinemia occurs with a frequency of 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 adequately to antibiotic treatment. However, some individuals, mainly those affected by IgA deficiency (SIgAD) or transient hypogammaglobulinemia of infancy , may have few or no infections.

Keywords: B cell; immunodeficiency; immunoglobulin; infancy.

MeSH terms

  • Agammaglobulinemia / diagnosis*
  • Agammaglobulinemia / genetics
  • B-Lymphocytes / immunology*
  • CD40 Ligand / genetics
  • Humans
  • Hyper-IgM Immunodeficiency Syndrome, Type 1 / diagnosis*
  • Hyper-IgM Immunodeficiency Syndrome, Type 1 / genetics
  • IgA Deficiency / diagnosis*
  • IgA Deficiency / genetics
  • Immunoglobulin A / genetics*
  • Infant
  • Infant, Newborn
  • Infections / diagnosis*
  • Infections / genetics
  • Lymphocyte Activation
  • Recurrence

Substances

  • Immunoglobulin A
  • CD40 Ligand