Immunoglobulin deficiencies: the B-lymphocyte side of DiGeorge Syndrome

J Pediatr. 2012 Nov;161(5):950-3. doi: 10.1016/j.jpeds.2012.06.018. Epub 2012 Jul 17.

Abstract

DiGeorge syndrome is associated with a T-lymphocyte immunodeficiency. The prevalence of hypogammaglobulinemia has not been reported. We found that 3% of patients with DiGeorge syndrome were receiving immunoglobulin replacement therapy and 6% of patients over the age of 3 years had hypogammaglobulinemia. We conclude that DiGeorge syndrome is associated with significant humoral immune deficiency.

MeSH terms

  • Adolescent
  • Adult
  • B-Lymphocytes / cytology*
  • Child
  • Child, Preschool
  • Cohort Studies
  • DiGeorge Syndrome / diagnosis*
  • DiGeorge Syndrome / genetics
  • Europe
  • Humans
  • IgA Deficiency / blood
  • IgA Deficiency / diagnosis*
  • Immunoglobulin A / metabolism
  • Immunoglobulin G / metabolism
  • Immunoglobulin M / metabolism
  • Immunoglobulins / metabolism
  • Infant
  • Infant, Newborn
  • Middle Aged
  • Prevalence
  • Registries
  • T-Lymphocytes / cytology
  • United States

Substances

  • Immunoglobulin A
  • Immunoglobulin G
  • Immunoglobulin M
  • Immunoglobulins