Background. The emergence of new functional capabilities of statistical accounting made it possible to conduct a comparative analysis of the morbidity of allergic pathologies according to the registers of allergists and pediatricians from the Unified Medical Information and Analytical System (UMIAS) of Moscow with data from the Form of Federal Statistical Observation No. 12 (FSO No. 12). The aim of the study is to investigate the potential of using UMIAS for assessing/monitoring the morbidity of allergic diseases, including bronchial asthma in children, using the example of several outpatient clinics (OPCs) in Moscow. Methods. A study of combined design has been carried out. The data of children of several OPCs in Moscow were analyzed — data from UMIAS (observation registers of pediatricians and allergist-immunologists) and from the reporting forms of the FSO No. 12. Results. For a comparative analysis of statistical data from UMIAS and FSO No. 12, we studied the information of 60,851 children under 18 years of age. It was revealed that out of 60,851 children: allergic rhinitis according to FSO No. 12 and UMIAS was established in 1001 and 1059 patients; atopic dermatitis — in 142 and 345; urticaria — in 363 and 33; angioedema — in 4 and 16, respectively; food allergy — in 233 children according (to FSO No. 12) and in none of the children (according to UMIAS). Out of 60,851 children, 619 children were diagnosed with bronchial asthma according to the annual report (FSO No. 12) and 537 according to the pediatrician’s observation registers (UMIAS). At the same time, it was found that the diagnosis of bronchial asthma is not available as a separate nosology in the registry of an allergist-immunologist, and information about children with bronchial asthma is available to this specialist only when analyzing the uploaded information about children with other allergic diseases. Conclusion. A adequate sample ensured a high representativeness of the results obtained. The differences in the incidence rates of allergic diseases revealed by a comparative analysis of data from various sources — UMIAS and FSO No. 12 — indicate the need to improve both the system of statistical registration of incidence and the development of modern algorithms for early diagnosis and dynamic monitoring of children with allergies.

The aim of the study is assess the reliability, internal stability and effectiveness of adaptation of individual questions of the QAA-25 questionnaire to assess the potential adherence to treatment of persons aged 12–14 years, in comparison with a similar QAA-25 questionnaire for persons aged 15–17 years. Material and Methods. In a descriptive observational study, including 314 students of general schools in Omsk the level of treatment adherence was determined using the QAA-25 questionnaire, using traditional and alternative formulations of individual questions. Results. For adolescents aged 12–14 years a good diagnostic value was shown higher individual testing quality (absence of blank answers to questions) of the tested version of the questionnaire compared to the alternative one. 96.8% of respondents indicated that the wording of the specialized questionnaire was “more accurate” and “more correct”. The tested version of the questionnaire demonstrated good reliability (Cronbach’s alpha total 0.901, based on standardized items αst 0.914) with high internal consistency (consistent elimination of scale items maintains questionnaire validity in the 0.886–0.909 range), with near perfect expert agreement (Cohen’s kappa 0.908). Conclusion. The questionnaire is characterized by good validity and has a high internal stability, and the content and wording of all questions of the questionnaire are adequately perceived by the audience for which the questionnaire is designed. This makes it advisable to use it to assess the potential adherence to treatment of adolescents aged 12–14 years.

Duchenne muscular dystrophy is one of the most common forms of childhood muscular dystrophies. Its incidence is 1 in 3.5–6 thousand newborn boys according to various sources. The disease is caused by the mutation in the DMD gene coding the dystrophin protein, it leads to the dystrophin absence or malfunction. The disease is characterized by proximal muscle weakness and gastrocnemius muscles pseudohypertrophy. In average, patients lose the ability to walk by themselves by the age of 11 and become nonambulatory. The authors have present modern epidemiological data and etiopathogenesis features of Duchenne muscular dystrophy, and have described clinical signs of different disease stages. The algorithm and key points of differential diagnosis are indicated. Special attention was given to the patients’ management: pathogenetic treatment and rehabilitation of pediatric patients.

The Union of Pediatricians of Russia together with the Russian Association of Allergologists and Clinical Immunologists and the Russian Society of Dermatovenerologists and Cosmetologists have developed new clinical guidelines for the urticaria in adults and children. Urticaria is a common disease; its various clinical variants are diagnosed in 15–25% of people in the global population, and a quarter of all cases belongs to chronic urticaria. The prevalence of acute urticaria is 20%, and 2.1–6.7% in child population, whereas acute urticaria is more common in children than in adults. The prevalence of chronic urticaria in adults in the general population is 0.7 and 1.4%, and 1.1% in children under 15 years of age, according to the systematic review and meta-analysis, respectively. This article covers features of epidemiology, etiology, and pathogenesis of the disease with particular focus on differential diagnostic search. Guidelines on treatment and step-by-step therapy scheme (both based on principles of evidencebased medicine) for pediatric patients were presented. Clarification on the analysis of the therapy efficacy and the degree of disease activity was given.

This article presents practical data, topical for pediatricians, on the child’s body provision with the essential trace element — iron; and on iron deficiency conditions development and staging in children. Clinical and laboratory criteria for the identification of such conditions are defined; data on their prevalence in tender-age infants is outlined. The results of modern studies showing the correlations between iron deficiency and delayed developmental conditions in children (including cognitive ones) are presented. Alimental factors (associated with body provision with iron) and nutritional strategies (associated with supplemental feeding timely administration, adequacy, and diversity) are described in detail. They are focused on effective and safe prevention of latent iron deficiency.

The scientific review of the literature provides information on current clinical observations of the use of proton pump inhibitors in large randomized trials of Russian and foreign scientists, issues of their classification, pharmacokinetics, pharmacodynamics, pharmacogenetics, efficacy and safety of prescribing in pediatric practice, due to the growth of acid-dependent conditions in children and the need for further systematic research with the development of approaches to personalization of prescribing drugs for each age group.

Glycogen storage disease type Ib (GSD Ib) — is a disease from the group of hereditary metabolic diseases caused by insufficiency of the glucose-6-phosphate transporter (G6PT, SLC37A4), which leads to a violation of both glycogenolysis and gluconeogenesis and, as a consequence, to excessive accumulation of glycogen and fat in the liver, kidneys and intestinal mucosa. The main clinical manifestations and laboratory data include growth retardation, hepatomegaly, hypoglycemia, lactic acidosis, hyperuricemia and hyperlipidemia. Complications of this disease are hepatocellular adenoma with a possible risk of malignancy, nephropathy and osteoporosis. A specific sign of GSD Ib is neutropenia with impaired neutrophil function, which creates prerequisites for recurrent infections and the development of inflammatory bowel disease. Until the present, enzyme replacement therapy of GSD Ib has not been developed, therefore, the main methods of treatment are a specialized diet with the addition of raw corn starch (for relief of hypoglycemia) and the use of granulocyte colony stimulating factor (for relief of neutropenia). However, the recent establishment of the role of 1,5-anhydroglucitol in the pathogenesis of neutrophil dysfunction in GSD Ib has led to a reprofiling of indications for the use of empagliflozin, a type 2 renal sodium—glucose cotransporter inhibitor (SGLT2). In the modern literature, it is reported about a minor, but very successful experience of its use in patients with GSD Ib (outside the framework of official indications for use) and a beneficial effect on neutrophil dysfunction and its clinical consequences. Oddly enough, this hypoglycemic drug improved not only metabolic, but also glycemic control in patients with GSD Ib, despite the fact that the pathology is based on chronic hypoglycemia. More and more evidence points to the role of empagliflozin in the regulation of cellular homeostasis (for example, fatty acid metabolism, glucose, cholesterol, apoptosis and cell proliferation, in particular in the liver) by influencing the activity of sirtuin 1 (SIRT1), AMP-activated protein kinase (AMPK) and signal molecules such as -serine/threonine protein kinase (Akt) and a mechanical target of rapamycin (mTOR), which leads to an improvement in the structure and function of mitochondria, stimulation of autophagy, reducing oxidative stress and suppressing inflammation. Modulation of these pathways shifts oxidative metabolism from carbohydrates to lipids and leads to a key decrease in insulin levels, resistance to it, glucose and lipotoxicity. This review presents current data on the pathogenesis of neutropenia and the possibility of using empagliflozin for its relief in patients with GSD Ib.

The study data of the last two decades on primary and secondary immunodeficiency in congenital heart defects (CHD) as a cause of frequent infectious complications before and after cardiac surgery are presented. Based on screenings of various levels, data are provided on the greater severity of immunological disorders in critical and cyanotic CHD in conotruncal defects compared with those in septal defects and stenotic defects. Violations were more often related to T-cell function and immunoglobulin deficiency (especially the IgG and IgG4 subgroups). Various types of primary immunodeficiency were found in 13 genetic syndromes in combination with CHD. The review discusses the possibility of using the technique of quantitative determination of DNA TREC and KREC — by-products of maturation of T- and B-cell receptors, which allows us to judge the defects of the T- and B-cell links of the immune system to predict infectious complications in children with CHD. The data of our own study of 200 infants with CHD (in 5% of cases with syndromic forms of CHD) are presented, where a decrease in TREC was found in 23.5% of cases, including all infants with syndromic forms, more often with cyanotic and conotruncal CHD and in children admitted in critical conditions. In children with reduced TREC values, infectious complications in the postoperative period were observed significantly more often than in children with normal indicators (36 and 3.6%, respectively). The analysis of publications confirmed the importance of TREC and KREC screening for targeted preoperative preparation in order to reduce postoperative complications and reduce the risk of mortality in CHD.

Cystic fibrosis is a severe hereditary disease with polysystemic manifestations and progressive course. Malnutrition in cystic fibrosis occurs as a result of exocrine insufficiency of the pancreas, an increase in energy losses in chronic inflammation in the bronchopulmonary system, manifested by increased stress on the respiratory system. The presented literature review highlights the modern principles of prevention and correction of malnutrition in children with cystic fibrosis, identifies the most promising methods for further development that correct nutritional status disorders. The review has shown that an active approach to nutrition at any age, the use of aggressive methods of nutritional support against the background of enzyme replacement therapy, timely and adequate therapy of respiratory tract pathology lead to an improvement in the indicators of nutritional status in cystic fibrosis. The most promising is the further development of targeted therapy, which allows, as a result of exposure to the etiopathogenetic mechanisms of the disease, to reduce the frequency and severity of bronchopulmonary exacerbations, partially restore the exocrine function of the pancreas, which is manifested in patients with cystic fibrosis by an increase in body weight and mass-growth index.