Immunodeficiency in Children

What is Immunodeficiency in Children?

Immunodeficiency in children is a condition that develops as a result of damage to one or more parts of the immune system. Conditions are manifested in recurrent and severe infections and, possibly, in an increased frequency of autoimmune manifestations and tumor formations.

With some immunodeficiencies, the child may have an allergic manifestation. That is, the conditions under consideration include not only sensitivity to infections, but also non-infectious pathology.

Types of immunodeficiency:

  • primary
  • >secondary

Secondary are the result of another disease or external exposure. Primary immunodeficiencies in children are less common, they are genetically determined diseases, which is caused by a violation of some or one of the immune defense mechanisms.

According to the 2006 classification, primary immunodeficiencies are divided into the following groups:

  • predominantly humoral
  • combined with damage to T and B lymphocytes
  • state of immune dysregulation
  • clearly defined immunodeficiency states
  • defects of innate immunity
  • phagocytosis defects
  • complement system defects
  • autoinflammatory diseases

B-cell deficiency agammaglobulinemia is a form of total immunoglobulin deficiency, which is second most common after total variable immune deficiency. The disease begins due to a defect in the B-cell tyrosine kinase gene. Boys become ill with this form of immunodeficiency, and the disease manifests up to 12 months of the child’s life.

Causes of Immunodeficiency in Children

Primary immunodeficiencies are caused by gene defects that affect the immune system. Because the child is born already sick. A gene defect can appear immediately after conception, in such cases, parents do not have immunodeficiency.

Causes of secondary immunodeficiencies in children:

  • congenital and metabolic diseases (uremia, Down syndrome, etc.)
  • prematurity
  • infections (EBV, HIV, etc.)
  • immunosuppressive agents (cytostatics, irradiation, etc.)
  • surgical interventions and injuries
  • hematologic diseases (e.g. histiocytosis)

Pathogenesis during Immunodeficiency in Children

With secondary immunodeficiencies in children, the B-link (humoral), E-cell immunity is violated. There may also be violations in the system of non-specific defense factors. In other cases, defects are combined, leading to immunodeficiency.