Transient hypogammaglobulinemia of infancy is a form of
hypogammaglobulinemia appearing after birth, leading to a reduction in the level of
IgG, and also sometimes
IgA[1][2] and
IgM.[3]
It can result in increased infections, but it can also present without symptoms.[4]
It is unknown what specifically causes infantile transient hypogammaglobulinemia. The following are some of the hypothesized mechanisms: 1) defective
T cells that prevent
B cells from stimulating the proper synthesis of
antibodies; 2) maternal
IgG suppresses the production of
IgG; 3) low levels of vital
cytokines; and 4) genetic variations in families predisposed to
immunodeficiency.[9][10][11]
Mechanism
Normally, a newborn's
immunoglobulins come from the mother during pregnancy and wane after birth until 3–6 months of age, when the infant begins to start to produce their own
IgG. However, in transient hypogammaglobulinemia of infancy, the
IgG synthesis is delayed, and the
hypogammaglobulinemia is prolonged beyond age 6 months.[12]
Diagnosis
Transient hypogammaglobulinemia of infancy can be identified if the mean age-specific reference values for serum
IgG levels are decreased by more than two standard deviations.[13]
Epidemiology
Transient hypogammaglobulinemia of infancy is thought to affect between 0.061 and 1.1 out of every 1000 live births.[14][15]
^Kiliç SS, Tezcan I, Sanal O, Metin A, Ersoy F (2000). "Transient hypogammaglobulinemia of infancy: clinical and immunologic features of 40 new cases". Pediatr Int. 42 (6): 647–50.
doi:
10.1046/j.1442-200x.2000.01301.x.
PMID11192522.
S2CID25895833.
^Dorsey, Morna J.; Orange, Jordan S. (2006). "Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy". Annals of Allergy, Asthma & Immunology. 97 (5). Elsevier BV: 590–595.
doi:
10.1016/s1081-1206(10)61085-x.
ISSN1081-1206.
PMID17165264.
^Whelan, M. A.; Hwan, W. H.; Beausoleil, J.; Hauck, W. W.; Mcgeady, S. J. (2006). "Infants Presenting with Recurrent Infections and Low Immunoglobulins: Characteristics and Analysis of Normalization". Journal of Clinical Immunology. 26 (1). Springer Science and Business Media LLC: 7–11.
doi:
10.1007/s10875-006-8144-1.
ISSN0271-9142.
S2CID24144434.
^Tiller, Thomas L.; Buckley, Rebecca H. (1978). "Transient hypogammaglobulinemia of infancy: Review of the literature, clinical and immunologic features of 11 new cases, and long-term follow-up". The Journal of Pediatrics. 92 (3). Elsevier BV: 347–353.
doi:
10.1016/s0022-3476(78)80417-x.
ISSN0022-3476.
PMID632973.