According to the data from Russian primary immunodeficiencies (PID) registry 71% of registered patients were treated with immunoglobulins (IG). Regular immunoglobulin substitutions were reported in 90% of patients with primary antibody deficiencies (PAD), 86% - with syndromic PID and 91% of patients with combined T and B cell defects. The study was supported by Academic Council of Dmitry Rogachev National Medical Center of Pediatric Hematology, Oncology and Immunology and approved by Local Ethical Committee within the Russian PID registry. Regular IG substitution was analyzed in the representative cohort of 235 PID patients from 12 Russian regions. Of these 121 were children, 114 – adults. In 78% cases IG treatment has been started during the first 10 years of life. 80% patients were treated with highly safe products (Octagam 5% and 10%, Privigen, IG VENA, Gamunex) reaching therapeutic median pre-infusion level of serum IgG of 7 g/l. Significantly lower levels of pre-infusion serum IgG were observed in patients treated with Gabreglobin-IgG. Irregular treatment was observed in 61% of patients mainly due to the poor drug supply (lack of medication in the health care centers). Infections were reported in 90% percent of patients with irregular treatment. Unscheduled hospitalizations were two times more frequent in the group of patients with irregular IVIG treatment. Additionally, we assessed quality of life of patients with regular IVIG treatment, which significantly improved in comparison with the pretreatment period and became comparable to the group of healthy controls.

Nowadays sepsis is grave complication of infection end the cause of death reanimation. In this survey, we would like to emphasize the importance of the control over the activation over the compliment system. It has been proved of animal model a complement one of the key role in the development of hyperactive immunity response, later resulting in violation of immunity homeostasis. Mice which had C3–/–, C4–/– deficit, aft receiving a LPS dose intraperitoneallis showed a better survival to compare with the control group of animals. There exist clinical data which confirm active participation of the compliment in the chain of the septic process. The research showed the patient affected by sepsis, had protein C3 and C4 concentration correlating which mortality at the time of diagnosis. The is chemoattractants, protein C3a and C5a, turn tube the result of complements pathway activation. The chemoattractants, provoke the extraction a big number of cytokines. Vessels permeability increase and DIC-syndrome activation wis it, multiple organ dysfunction develops. Ishemiareperfusion launch triggers aseptic inflammation, which appears decentralization and DIC-syndrome. C1-INH controls the work of classical way complement and Hemostasis System. Researchers the deficit in C1-INH animals and patients affected bay sepsis, which is proved in laboratory and clinics. The remedy C1-INH (Berinert, CSL Behring) appeared over 25 years ago and was used and therapy hereditary angioedema. For the lasted years we accumulated a considerable quantity of fasts of C1-INH use which after pathologies: heart attack, Ischemia-reperfusion injury, trauma provoked by cardiopulmonary bypass. The use of C1-INH on animal models septic in clinical research their efficacy and safety.

THE AIM. To reveal the informative indicators for the prediction of progression of reflux nephropathy in children with vesicoureteral reflux based on the study of the urinary excretion of markers of sclerosis (transforming growth factor-β 1 ), angiotensin II, procalcitonin, β 2 -microglobulin) and collagenopathy (peptidoglycans and free hydroxyproline). PATIENTS AND METHODS: the study group included 71 (52 girls) the patient at the age of 5.69±0.44 years with vesicoureteral reflux (VUR). Based on the results of DMSA-scintigraphy, all children were divided into 2 groups: the control group 9 children with VUR without foci of sclerosis and a study group included 62 patients with different degrees of reflux nephropathy (RN). The control group consisted of 20 healthy children at the age of 6.24 ± 0.31 years. In all children investigated the urinary excretion of AngII, TGF-β 1 , PCT, β 2 -MG, PSH and FH and counted it to the level of creatinine in the urine. RESULTS. Urinary levels of AngII, TGF-β 1 , PCT, β 2 -MG, PSH, and FH was significantly higher in groups of children with RN when compared with the control group. The highest level AngII, TGF-β 1 , PCT was defined in the main group (p<0.05). Patients with a severe degree of RN, the ><0.05). Patients with a severe degree of RN, thelevel of AngII, TGF-β 1 , PCT in urine was higher when compared with the control group and the group of children without foci of sclerosis (p<0.05). CONCLUSION. Installed direct correlation between the level of excretion AngII, TGF-β 1 , PCT and the severity of RN in patients with VUR indicates the feasibility and prospects of their inclusion as diagnostic markers predict the severity of reflux nephropathy.