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.

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.

Currently, there is evidence that hepcidin is the main regulator of iron metabolism in human and pathogenesis key factor for anemia of inflammation. However, the role of hepcidin in multifactorial pathogenesis of anemia in pregnancy is not clear. We presented the results of the laboratory examinations of 78 pregnant women sera in hepcidin, ferritin, erythropoietin during pregnancy, and 116 sera of pregnant women with iron deficiency anemia (IDA) and anemia of mixed origin. The obtained data indicate a statistically significant decrease in the mean hepcidin concentration in pregnants versus non pregnant women. Mean hepcidin level in pregnant women with IDA was decreased, compared with anemia of mixed origin pregnants (phepcidin concentrations may be useful laboratory test for differential diagnostic of anemia during pregnancy and for determination of optimal therapeutic option between oral iron, parenteral iron or using erythropoiesis -stimulating agents (ESAs) in combination with iron products.

Currently, there is evidence that hepcidin is the main regulator of iron metabolism in human and pathogenesis key factor for anemia of inflammation. However, the role of hepcidin in multifactorial pathogenesis of anemia in pregnancy is not clear. We presented the results of the laboratory examinations of 78 pregnant women sera in hepcidin, ferritin, erythropoietin during pregnancy, and 116 sera of pregnant women with iron deficiency anemia (IDA) and anemia of mixed origin. The obtained data indicate a statistically significant decrease in the mean hepcidin concentration in pregnants versus non pregnant women. Mean hepcidin level in pregnant women with IDA was decreased, compared with anemia of mixed origin pregnants (p<0.0001). Evaluation of hepcidin concentrations may be useful laboratory test for differential diagnostic of anemia during pregnancy and for determination of optimal therapeutic option between oral iron, parenteral iron or using erythropoiesis -stimulating agents (ESAs) in combination with iron products.

The article is devoted to early anemia in premature newborns, which occupies the 1st place among the indications for blood transfusion in children with very low and extremely low birth weight. Ways of optimization of anemia treatment in premature newborns are shown based on the analysis of international experience and the results of in-house researches. The most important of these include limiting the number of blood transfusions, justified early application of recombinant human erythropoietin and reduction in the volume of blood samples for laboratory tests.