Transient hypogammaglobulinemia of infancy

[Becky]

In transient hypogammaglobulinemia of infancy, production of normal amounts of antibodies (immunoglobulins) in infants is delayed.
(See also Overview of Immunodeficiency Disorders.)
At birth, the immune system is not fully developed. Most of the immunoglobulins in infants are those produced by the mother and transferred via the placenta before birth. Immunoglobulins from the mother protect infants against infection until infants start to produce their own, usually by age 6 months. About the same time, levels of immunoglobulins from the mother start to decrease.
In infants with transient hypogammaglobulinemia of infancy, production of normal amounts of immunoglobulins is delayed. As a result, immunoglobulin levels become low starting at age 3 to 6 months and return to normal at about age 12 to 36 months.
Transient hypogammaglobulinemia of infancy rarely leads to serious infections and is not thought to be a true immunodeficiency. However, a few infants develop frequent sinus, lung, or digestive tract infections, candidiasis (a fungal infection), and/or meningitis.
This condition is more common among premature infants because they receive fewer immunoglobulins from the mother.
Blood tests are done to measure levels of immunoglobulins and to evaluate immunoglobulin production in response to vaccines. Usually, infants with the disorder produce normal amounts of antibodies in response to the vaccines they are given and to infectious organisms they are exposed to. However, if infants, particularly those born prematurely, have frequent infections, they may be given antibiotics to prevent more infections from developing.
This disorder may last for months to a few years but usually resolves without treatment.

[Michiko]

Transient hypogammaglobulinemia of infancy is a temporary decrease in serum IgG and sometimes IgA and other immunoglobulin (Ig) isotypes to levels below age-appropriate normal values.
(See also Overview of Immunodeficiency Disorders and Approach to the Patient With an Immunodeficiency Disorder.)
Transient hypogammaglobulinemia of infancy is a primary immunodeficiency disorder that involves humoral immunity deficiencies. In this disorder, IgG levels continue to be low after the physiologic fall in maternal IgG at about age 3 to 6 months. 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 gastrointestinal infections, candidiasis, and/or meningitis.
Diagnosis of transient hypogammaglobulinemia 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.
Patients with recurrent infections can be temporarily treated with prophylactic antibiotics. Immune globulin is usually unnecessary.
Transient hypogammaglobulinemia of infancy may persist for months to a few years but usually resolves.