There are only limited data coming from isolated case reports regarding the real-world use of emicizumab for the treatment of children with hemophilia A and inhibitors (HAI) in Russia. The aim of the study was to evaluate the efficacy and safety of emicizumab prophylaxis in children with severe HAI. Ethical approval was not required since the study only involved the use of anonymized and generalized retrospective data obtained during routine clinical practice. We retrospectively analyzed medical records of children with HAI who had been treated with emicizumab at 11 institutions located in Russia, taking into consideration such parameters as annualized bleeding rates (ABR), annualized spontaneous bleeding rates (ASBR), annualized joint bleeding rates (AJBR) and annualized bleeding rates for bleeds requiring additional therapy (ABRRT), as well as the presence and severity of adverse events during the treatment. The median age of patients at the time of initiation of emicizumab prophylaxis was 65 (11–170) months. Before the treatment, ABR was 19.9 (95% confidence interval (CI), 15.4–26.1), ASBR – 13.6 (95% CI, 10.6–17.8), AJBR – 6.6 (95% CI, 4.7–9.7), ABRRT – 16.6 (95% CI, 12.4–22.7). After the initiation of the treatment, bleeding rates changed dramatically: ABR decreased by 98.6% (95% CI, 96.7–99.4), AJBR – by 99.4% (95% CI, 95.3–99.9), ABRRT – by 98.8% (95% CI, 96.8–99.6); and there were no signs of spontaneous bleeding during 10 (1–32) months of treatment. No adverse events leading to the interruption or discontinuation of the treatment with emicizumab were reported. The use of emicizumab in children with HAI in the real-world clinical setting results in a significant (> 98%) and safe reduction in bleeding episodes without any signs of spontaneous bleeding.

Цель обзора. Показать, что анемия пожилых не является неизбежным следствием старения. Несмотря на высокую распространенность анемии у пожилых пациентов, этому явлению не уделяется необходимого внимания.Основные положения. Анемия пожилых представляет значительную проблему для здравоохранения. Это распространенное состояние у пожилых людей, которое ухудшает течение практически всех заболеваний, а также увеличивает частоту смерти от всех причин. Диагностика причин анемий в пожилом возрасте бывает затруднена из-за полиэтиологичности анемии в пожилом возрасте. Наиболее частыми этиологическими факторами анемий у пожилых являются абсолютный дефицит железа, хроническое воспаление, хроническая болезнь почек, дефицит витамина В12. Большуюгруппу составляют анемии неясной этиологии.Заключение. Анемию пожилых следует рассматривать именно как заболевание и лечить, а не списывать ее как «неизбежное следствие старения». Диагностические алгоритмы для пациентов молодого возраста недостаточно надежны для пожилых пациентов. Purpose. To show that anemia is harmful to elderly patients and is not an inevitable consequence of aging. Despite the high prevalence of anemia in elderly patients, this phenomenon is not given the necessary attention by clinicians.Background. Anemia in elderly is a significant public health problem. It is a common condition in the elderly that worsens almost all diseases and increases the incidence of all-cause death. Diagnosis of the causes of anemia in the elderly is difficult because of polyetiology of the phenomenon. The most common etiological factor of anemia in the elderly is an absolute iron deficiency, chronic inflammation, chronic kidney disease,vitamin B12 deficiency. A large group is anemia of unknown etiology.Conclusion. Anemia in elderly should be considered as a disease to be treated, as opposedto being accepted as an "inevitable consequence of aging". Today’s diagnostic and treated algorithms for young and adult patients are not sufficiently reliable for elderly ones.

Железодефицитная анемия — полиэтиологичное заболевание, развитие которого связано с дефицитом железа в организме из-за нарушения поступления, усвоения или повышенных потерь данного микроэлемента, характеризующееся микроцитозом и гипохромной анемией. В практическом руководстве подробно описывается этиология, лабораторная и инструментальная диагностика, тактика ведения пациентов в различных клинических ситуациях. Практическое руководство предназначено для специалистов практического здравоохранения, оказывающих первичную медико-санитарную помощь, в том числе врачей-терапевтов, врачей общей практики (семейной медицины), кардиологов, гастроэнтерологов и других специалистов, оказывающих помощь пациентам с железодефицитной анемией.

Cat scratch disease is caused by the gram-negative intracellular bacterium Bartonella henselae (B. henselae). Human infection occurs mainly through cat scratches and bites. In typical cases, the clinical course is presented by a primary affect on the skin and regional lymphadenitis. The atypical presentation of infection can occur with fever, abdominal, ocular and neurological manifestations. A special feature of the abdominal form is the presence of solitary or multiple hypo/anechoic foci of different size and shape in the liver and spleen. B. henselae infection is one of the most common mimics of malignant lymphoma, when the spleen is involved in combination with B symptoms (weight loss, night sweats and prolonged fever). A history of recent contact with cats and diagnostic IgG titers to B. henselae indicate infection. There is no consensus on the choice of antimicrobial drug and the duration of therapy for systemic forms. The article presents its own experience in the diagnosis and treatment of such patients. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.

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.

Vitamin B12 (cobalamin) deficiency in infants exclusively breastfed is usually the result of its deficiency in the mothers (vegetarians or in the presence of unrecognized pernicious anemia or malabsorption syndrome). Cobalamin is crucial for the normal development of the nervous system, the production of erythrocytes and the synthesis of DNA. The classic manifestation of its deficiency is megaloblastic anemia and, in advanced cases, pancytopenia. However, infants often present neurological symptoms (fatigue, failure to thrive, regress of developmental skills, lethargy, motor disorders), as a consequence of inadequate myelination of the brain. Cobalamin treatment is effective in the most children, however, the neurological deficit varying degrees may persist. The long-term prognosis depends on overall duration of vitamin B12 deficiency and severity of symptoms. The article presents our own interesting clinical observations. Parents gave their permission for using personal data for clinical research and publications.

AIM To assess intrinsic coagulation pathway factors activity and hemostatic markers of endothelial dysfunction in patients with peripheral artery disease (PAD) before and after lower extremity bypass surgery. MATERIAL AND METHODS 80 patients with PAD Fontaine grade IIB-III were enrolled. 40 patients underwent open aorto-femoral-popliteal repair (group A) and 40 patients - medication (group B). Before and in 3 months after surgery peripheral venous blood samples were collected to assess the activity of factors VIII, IX, XI, von Willebrand factor (VWF), protein C (PrC), and metabolites of nitric oxide II (NO). RESULTS Increased preoperative activity of VIII, IX, XI, and VWF factors compared with normal values was observed in group A. After 3 months there was an additional increase of VIII and VWF factors' activity. Activity of IX and XI factors remained increased on background of reduced level of NO metabolites. In group B increased activity of IX, XI, and VWF factors was revealed while levels of NO metabolites and PrC were normal. CONCLUSION Thus, PAD is followed by prothrombotic state especially in patients after surgery. Surgical procedures are associated with more severe hypercoagulation.

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.

The review analyzes the major foreign publications on the iron exchange in mycobacteria for the recent years. The issues of iron absorption by tuberculous mycobacteria, its preservation and regulation of their metabolism have been reviewed. Deeper and more detail description of the above process will provide new opportunities for therapeutic interventions into it and assist in development of new tuberculosis treatment techniques.

Iron-deficiency anaemia (IDA) has been studied well enough. But in clinical practice there are a number of problems related to diagnosis, treatment and prevention of iron deficiency, resulting in a high incidence of IDA and iron deficiency in Russia. One of the problems remains a choice of a medicinal formulation for oral IDA therapy, namely, a comparative efficacy and safety of ferrous (+2) and ferric (+3) iron preparations. Ferrous iron salts have long and often been used in clinical practice. They have a higher bioavailability. But according to literature data, the possibility of development of adverse events in using ferrous iron salt formulations is often higher than when using ferric iron preparations. Analysis of literature data is demonstrable of an equal efficacy of there two groups of iron drugs in patients with IDA.

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.

Iron-deficiency anaemia (IDA) has been studied well enough. But in clinical practice there are a number of problems related to diagnosis, treatment and prevention of iron deficiency, resulting in a high incidence of IDA and iron deficiency in Russia. One of the problems remains a choice of a medicinal formulation for oral IDA therapy, namely, a comparative efficacy and safety of ferrous (+2) and ferric (+3) iron preparations. Ferrous iron salts have long and often been used in clinical practice. They have a higher bioavailability. But according to literature data, the possibility of development of adverse events in using ferrous iron salt formulations is often higher than when using ferric iron preparations. Analysis of literature data is demonstrable of an equal efficacy of there two groups of iron drugs in patients with IDA.

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.

The study included 28 patients with pulmonary tuberculosis and a possible postoperative hemoglobin level of less than 120 g/l. For the prevention of postoperative anemia it is used recombinant human erythropoietin alpha and iron (III) hydroxide sucrose complex. Hemoglobin levels were monitored for 4 weeks after surgery and it is estimated the need for blood transfusions. Use of recombinant human erythropoietin and iron sucrose in patients with pulmonary tuberculosis increases basic hematological parameters as compared with the control group, and reduces the risk of postoperative anemia.