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Transient Hypogammaglobulinemia of Infancy

by James Fernandez, MD, PhD

Transient hypogammaglobulinemia of infancy is a temporary decrease in serum IgG and sometimes IgA and other Ig isotypes to levels below age-appropriate normal values.

In transient hypogammaglobulinemia of infancy, IgG levels continue to be low after the physiologic fall in maternal IgG at about age 3 to 6 mo. The cause and inheritance patterns are unknown. The condition rarely leads to significant infections and is not thought to be a true immunodeficiency. The condition is usually asymptomatic. However, a few patients develop sinopulmonary or GI infections, candidiasis, and/or meningitis.

Diagnosis is based on low serum Ig levels (at least 2 standard deviations below the mean for age) and tests showing that antibody production in response to vaccine antigens (eg, tetanus, diphtheria) is normal. Thus, this condition can be distinguished from permanent forms of hypogammaglobulinemia, in which specific antibodies to vaccine antigens are not produced.

Patient with recurrent infections can be temporarily treated with prophylactic antibiotics. IV immunoglobulin is usually unnecessary. This condition may persist for months to a few years but usually resolves.

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