oa Current Allergy & Clinical Immunology - Primary Immunodeficiency - PID123

Volume 32 Number 2
  • ISSN : 1609-3607



For the past couple of weeks, Dr Goodheart has been seeing a 15-month-old boy, Nathan, who had a history of recurrent infections requiring longer than usual courses of intravenous antibiotics and also two intensive care unit admissions. Based on previous laboratory results, Nathan had low to absent IgG and IgA but normal to raised IgM antibody levels. B and T lymphocyte counts were normal. Dr Goodheart’s suspicion of a possible X-Linked Hyper IgM Syndrome (due to defects in CD40Ligand-CD40 interaction)1,2 as underlying immune defect increased when she found out that two of Nathan’s uncles had died in early infancy due to severe infections.

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