TRANSIENT HYPOGAMMAGLOBULINEMIA OF INFANCY
 
PEDBASE.org - The Pediatric Database - Detailed information of TRANSIENT HYPOGAMMAGLOBULINEMIA OF INFANCY

 

TRANSIENT HYPOGAMMAGLOBULINEMIA OF INFANCY

 

DEFINITION:

A disorder characterized by an abnormally prolonged delay in the onset of immunoglobulin synthesis by an infant resulting in recurrent viral and pyogenic infections.

EPIDEMIOLOGY:

  • incidence: rare
  • age of onset:
    • after 6 months of age
  • risk factors:
    • familial in some cases (relatives with immunodeficiencies parti-cularly Severe Combined Immunodeficiency Disease)
    • M = F

PATHOGENESIS:

1. Background

  • the normal full-term infant acquires a full complement of IgG by active transport of IgG across the placenta during the last trimester of pregnancy
  • as maternally-derived IgG is slowly metabolized (half life between 25-30 days), a physiologic nadir of IgG occurs between the 3rd and 6th months of age
  • the fetus is capable of forming antibodies in utero after 20 weeks gestation but significant synthesis of IgG by the infant does not usually begin until 2-3 months of age

2. Etiology

  • etiology is unknown
  • hypothesized that a defect in the number and/or function of T helper cells may interfere with the terminal differentiation of B cells into antibody-producing plasma cells

CLINICAL FEATURES:

1. Immunodeficiency Manifestations

1. Respiratory

  • recurrent otitis media and bronchitis most common
  • chronic cough

2. Others

  • rarely infections of the skin or meninges

INVESTIGATIONS:

1. Serum

1. Humoral-Mediated

  • normal number of circulating B and T cells
  • reduced immunoglobulin levels especially IgG

2. Lymph Node Biopsy

  • very small or no germinal centres
  • marked reduction in the number of plasma cells

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

 

 

Pediatric Database - TRANSIENT HYPOGAMMAGLOBULINEMIA OF INFANCY

 

Pediatric Organization - Pedbase [at] Gmail.com

 
1994 -2007 Pedbase.org 

Powered by Database of Pediatrics- TRANSIENT HYPOGAMMAGLOBULINEMIA OF INFANCY