IVIG - Transient hypogammaglobulinemia of infancy

Information for those receiving IVIG shared with from a client, currently waiting for the references from which she obtained this information....

Transient hypogammaglobulinemia of infancy

Although most IgG deficiencies are inherited defects, transient hypogammaglobulinemia appears to be an acquired defect resulting from a delayed onset of IgG synthesis.

Infants transplacentally obtain maternal IgG antibodies during the third trimester of gestation. Because the biologic half-life of IgG is approximately 21-28 days, the lowest levels of infant serum IgG are attained by postnatal months 2-4.

In some infants, this physiologic drop of serum IgG concentration is prolonged, sometimes for as long as 17-19 months in full-term infants and 36 months in premature infants. During this time, the infant is susceptible to recurrent bacterial infections, and the condition is referred to as transient hypogammaglobulinemia of infancy. Fortunately, this type of immunodeficiency often resolves spontaneously by age 4 years, and affected infants do not usually require IgG replacement.

Transient hypogammaglobulinemia of infancy is an immunodeficiency disorder in which antibody production by an infant is delayed.
At birth, the immune system is not fully developed. Most of the antibodies in infants are those produced by the mother and transferred via the placenta before birth. Antibodies from the mother protect infants against infection until infants start to produce their own antibodies, usually by age 6 months. Infants who have transient hypogammaglobulinemia of infancy do not start producing antibodies until later. As a result, antibody levels become low starting at age 3 to 6 months and return to normal at about age 12 to 36 months. This disorder is more common among premature infants, because they receive fewer antibodies from the mother. Although the disorder is present at birth, it is not hereditary.
Most infants with the disorder have some antibodies. Therefore, they do not have a problem with infections and need no treatment. However, some infants, particularly those born prematurely, develop infections frequently. Immune globulin can prevent and help treat infections. It is usually given for about 6 to 12 months. Antibiotics are given when needed. Lifespan is unaffected.

MANAGEMENT:

1. Supportive
in mild to moderate cases, moniter immunoglobulin levels every 3 to 4 months
important to rule out other permanent immunodeficiencies

2. Intravenous Immunoglobulin (IV IgG)
indicated in patients with severe recurrent infections
replacement for 12-36 months
discontinue when there is evidence of in vivo IgG synthesis

3. Prognosis
self limiting with recovery between 18-48 months of age
excellent prognosis with onset of normal IgG synthesis