Kazmirchuk V. Dysfunctions of immune system in children (identification, verification, immunorehabilitation).

The dissertation is devoted to identification as well as verification of immunodeficiency diseases (IDD) which are different by the genesis (hereditary, congenital, acquired). The dysfunctions have been identified by means of the developed clinico-amnestic criteria and pedigree data. Large data bulk (more than 7000 patients) was used to study the immune status in children with clinical signs of disorders in immune system. It was established that the immunodeficient diseases in children with clinical signs of the dysfunctions in immune system occur in 38.2% of cases, whereas 61.8% of patients had the secondary immunodeficiency. Clinico-immunologic observation upon 1625 children with IDD allowed us to verify a genesis of the disease: in 69% of cases it was acquire IDD, in 25% of cases - congenital IDD and in 6% of cases - hereditary IDD. It was established that the diagnostic value for IDD verification has valid decrease in the level of expression of CD54 (ICAM-1) molecules - to 2,08±0,51 when N =14-30.Positive correlation (r=+0,35) between the level of expression by CD4 and CD95 molecules on the surface of lymphocytes has been fixed. The levels of IL-1b (an average level 992 pkg/ml with variation range from 620,8 to 2072,0) or FTN-a (an average level 1189 pkg/ml with variation range from 560,1 to 1771,0) were determined to be 10-20 times higher in children with long-term amotivational subfebrile condition. In patients, having sluggish running of infectious diseases or latent running of sepsis, simultaneous increase of both IL-1b and FTNa can be observed. A heavy share of IL-12 (42,9%) and gINF (91,4%) deficiencies is worthy of notice in children with clinical signs of IDD. Along with that a relatively heavy share of extra high level IL-1b (16,3%) and IL-1 (17,8%0) was observed. Analyzing the clinical efficacy of polyoxidony in 36 children with IDD, we have determined that under the influence of this medicine phagocytic activity of neutrophils showed a valid rise (from 45,3±1,56 to 63,1±3,4%), as well as thelevel of CD3+ (from 49,63±4,4 to 57,2±1,9%), CD4+ (from 33,14±3,4 to 39,4±5,4%) and CD16+- lymphocytes (from 10,7±1,1 to 19,0±1,6%); while a valid lowering was noticed in a number of CD8+-lymphocytes (27,89±1,2 to 14,7±2,6%). 40 children with recurrent bronchopulmonary pathology, caused by an immune deficiency, have been treated with "Ukrain" (the medicine produced by Novitsky-farm company) to investigate its clinical effectiveness. Valid increase of CD3+-lymphocytes level under the influence of the therapy was observed in 14 children (35%), CD4+ - in 17 children (42,5%). In children treated with "Ukrain" we also observed a valid rise of IgG level which was reduced in 20 children (50%) before treatment. Other antibody contents were not changed reliably. Moreover, "Ukrain" contributed to the increase of both FVVL (from 66,1±2,8 to 70,5±2,8) and IVV 25 (from 53,6±7 to 80±6,9). It confirms the fact, that this immunomodulator improved the external respiration parameters in children with recurrent bronchopulmonarypathology.We have developed the principles of selective clinico-immunologic monitoring of children with IDD taking into consideration aetiology, clinical form of the disease as well as the nature of immune defects what allowed to lower a number of recurrences from 9 to 3-4 per year.