Primary immunodeficiencies

Classification of primary immunodeficiencies and evaluation of immunity.

Based on the currently available information on the mechanism of primary immunodeficiencies, international classification divides the disease into main groups:

  • with a primary lesion of humoral immunity;
  • combined immunodeficiency (loss of cellular and humoral immunity);
  • defects of phagocytosis system;
  • defects in the complement system;
  • other clearly defined CID.

can view the complete classification in the specialized literature, but it is important to have the Repose of the typical manifestations of immunodeficiency major groups, the general laws of their diagnosis and treatment.

immunity rank

important role in identifying the correct assessment of PIDS plays the patient's history of the disease, and family history.Thus, the presence of frequent but non-severe respiratory viral infections occurring in children of preschool and primary school age, as a rule, is not a sufficient basis for the assumption of diagnosis PIDS

.However, frequent infections in adults, a history of a patient of any age recurrent pneumonia, as well as serious infectious processes, such as sepsis, purulent meningitis, osteomyelitis, purulent skin infections, require laboratory immunoassay.Have a family history of deaths of children with clinical infectious disease, and the presence of patients with a diagnosis of primary CID is also a risk factor for the presence of PIDS have examined patients.

As mentioned above, most of the PIDS will debut in childhood.However, for many primary immunodeficiencies Diseases characterized by "erased form," first manifested in adolescence and even adulthood.In addition, for such diseases as common variable immunodeficiency, is characterized by symptoms first appeared after 20 years of life.In this connection, knowledge of primary immunodeficiencies is necessary not only pediatricians but also therapists, and physicians of other specialties.

specialized immunological examination carried out in specially equipped laboratories.However, simple laboratory techniques often allow IDS to suspect at an early stage.For persistent lymphopenia (reduction in the number of lymphocytes less than 1500 / mm), especially in young children is usually a sign of IDS with the defeat of cellular immunity.A significant reduction in the gamma fraction of the total protein electrophoregram may indicate violations of immunoglobulin synthesis.

Laboratory methods for detection of immunodeficiency disorders include an evaluation of the humoral and cellular immunity, the complement system, an analysis of other effector mechanisms, including phagocytosis and the inflammatory response.Screening study, among the total blood analysis must begin by examining the concentration of immunoglobulins IgM, IgG, IgA, followed by quantitation of the major cell populations of lymphocytes: T cells, B cells, natural killer cells.

Serum immunoglobulins, as well as the ratio of lymphocyte subpopulations depends on the age and clinical condition of the patient, so it is necessary to take into account age norms in the evaluation of research.If you suspect a defect in the complement system total hemolytic complement activity and the individual components of the complement of both classical and alternative pathway activation can be measured by immunochemical and functional methods.Evaluation of the immune response may include determining the humoral response to specific vaccinal antigens such as diphtheria and tetanus toxins, killed Polio antigens and the like. D.

Qualitative assessment of cellular immunity is usually carried out in vitro, by determining the lymphocyte response to mitogens, and specificantigens.Evaluation of phagocytosis is performed by measuring the degree of dye reduction of nitro-blue tetrazolium after specific stimulation of blood cells.In addition, phagocytosis activity is proportional to the number of dead microorganisms or depleted oxygen radicals amount, which is determined via chemiluminescence.Additionally it is possible in vitro evaluation of chemotaxis, chemokinesis, and the production and secretion of certain inflammatory cytokines.

For many primary immunodeficiencies described chromosomal localization of the defective gene that creates the basis for identifying the genetic carrier and prenatal diagnosis of PIDS.Currently, prenatal diagnosis carried out on samples of fetal blood, amniotic cells or chorionic villus sampling.

Magazine "Difficult patient» №2-2007.

article is provided by a charitable foundation to help children with impaired immunity "Sunflower".

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