This article is devoted to the issue of nursing and further monitoring of children born with very low and extremely low body weight. The article presents the data of international statistics and seven-year experience of the Rehabilitation Center for children born with very low and extremely low body weight, based on a large multidisciplinary children’s hospital. The authors pay particular attention to improvement of medical care of children born with very low and extremely low body weight.

Alpha-1-antitrypsin deficiency is an autosomal recessive disease characterized by both liver damage and lung disease in children and adults because of a decrease in the serum protein content due to the mutations in the PI (proteinase inhibitor) gene. The majority of liver diseases are associated with a homozygous mutation of the Z allele. There are many variations of clinical manifestations of the liver disease in children with the PI*ZZ genotype.  In the neonatal period, liver disease is usually cholestatic; and it is accompanied by a prolonged cholestatic jaundice, skin itching, which can be determined only later (after 6 months), decreased appetite and bad weight gain, hepato- and splenomegaly. The article describes the pathophysiology of liver damage in children with alpha-1-antitrypsin deficiency. The authors provide their recommendations for the management of children with suspected and confirmed alpha-1-antitrypsin deficiency.

The article presents a modern view of obesity as a chronic inflammation of adipose tissue. Obesity is accompanied by metabolic changes in lipid, protein, carbohydrate, mineral metabolism and disorders in the hormonal function of adipose tissue as an endo- and paracrine organ. At the moment, there are searched the biochemical markers of metabolic disorders of obesity. The obesity-related factors (hyperglycemia, increased lipid levels, insulin resistance, chronic inflammation, hyperleptinemia, endothelial dysfunction, impaired respiratory function of mitochondria, minerals and microelements deficiency) form and increase oxidative stress making it an integral component of the pathogenesis of obesity and possible complications. Given the important role of Q10 coenzyme in antioxidant tissue protection, the authors discuss the relationship of obesity and metabolic disorders to the endogenous levels of Q10 coenzyme and its possible use for pharmacological correction.

Enuresis is a considerable medical and socio-psychological problem. The pathophysiology of enuresis remains unknown; none of the existing theories can explain its nature in full. The article reviews the studies devoted to the role of obstructive sleep disturbances in the occurrence of enuresis. The mentioned sleep disturbances in children are associated with other related disorders: pulmonary hypertension, developmental delay, sleepwalking, attention deficit and hyperactivity disorder, obstructive sleep apnea syndrome and enuresis. Numerous studies confirm the correlation of the mentioned syndromes. The obstruction of the upper respiratory tract during sleep is accompanied by an increase in the efforts of the respiratory muscles and negative intrathoracic pressure, which is associated with high atrial natriuretic peptide and low antidiuretic hormone levels. In most cases the surgical removal of respiratory tract obstruction (adenotonsillectomy) leads to significant improvement or complete resolution of enuresis. This category of patients requires a comprehensive interdisciplinary approach to the examination and treatment by the pediatricians, psycho-neurologists and ENT specialists.

Based on the analysis of foreign and domestic literature, the article presents the features of infectious mononucleosis caused by the main pathogens from the Herpesviridae family, course of the disease at various phases of the infectious process. The article identifies clinical and laboratory manifestations characteristic of each pathogen. The authors discuss the issues related to the lack of the unified terminology for describing chronic herpes virus infection. The article discusses the causes of persistent herpes virus infections, risk factors for the adverse course and outcome of herpes virus infections.

Purpose: To integrate clinical databases based on the information and analytical systems of medical organizations (MO) and to evaluate outcomes and health of children born by assisted reproductive technologies (ART).

Research methods: To form database by integrating information and analytical systems of the medical organizations. To search and unify the data by means of freeware relational database management system (DBMS) – MySQL.

Results. A prototype application for the management and support of a unified clinical database to analyze and evaluate the outcomes of ART. To compare information from individual databases of MO one need to match fields in the form of unique personal records, field validation, processing of missing data. Significant (p <0.001) differences in gestational age and anthropometric data in the databases of medical organizations (76% of full-term children from the Automated System “Regional obstetric monitoring” (AS ROM) database compared with 18% according to the IS “Register of Children requiring Early Care”) were determined by the introduction of the conception of ART, along with the presence of perinatal risk factors in children. The created software can be used to create registers of medical organizations, as well as to support clinical decision-making in forecasting, modeling outcomes and children’s health after ART, developing personalized treatment and rehabilitation programs.

Conclusion. It is necessary to develop and implement information systems, to create ART registers on the basis of regional and national registers, to analyze the outcomes of the use of reproduction methods. 

Purpose. To identify the effects of gestational iron deficiency anemia after treatment with iron-containing drugs  during the perinatal period on the health status of full-term children during their 1st year of life.

Patients and methods.The authors carried out a prospective observational cohort study of full-term infants of I & II health group from their birth to twelve months. The course of pregnancy and labor was analyzed retrospectively. Hemoglobin, serum ferritin and C-reactive protein were measured in the six and twelve months old children and their mothers in 6 months after giving birth. We examined 140 couples of mother-child with gestational anemia (Group 1) and 166 couples without anemia during pregnancy (Group 2) in 6 months after giving birth, and in 12 months – 103 and 131 couples respectively. All women with iron deficiency anemia (Group 1) received therapy during pregnancy.

Results. Gestational anemia is associated with threatened miscarriage (Odds Ratio (OR) 3.496; 95% CI 2.013–6.072), chronic placental insufficiency (OR 1.907; 95% CI 1.025–3.548), delivery by cesarean section (OR 2.729; 95% CI 1.651–4.502), increased infectious morbidity in pregnant women (OR 1.079; 95% CI 1.025–3.548) and insufficient lactation (OR 1.990; 95% CI 1.209– 3.277). Treated anemia during pregnancy is not associated with low birth weight and low weight of 6 and 12 months old children. 8.6% of 6 months old children suffered from iron deficiency anemia, which is two times lower than in the group without anemia (p=0.007). The frequency of iron deficiency anemia did not differ in 12 months old children (р=0.543).

Conclusion: Iron supplementation reduces the negative impact of anemia on child’s health, including anthropometric indicators, iron stores, and risk of infectious diseases. The indirect effect is maintained through the adverse course of the perinatal period and the low duration of lactation. 

Purpose. To determine the anamnestic predictors of severe bronchial asthma in children. Material and methods. We analyzed risk factors of 335 patients with bronchial asthma. We statistically selected 287 patients and developed a prognostic model. Results. The predictors of severe bronchial asthma are combination of factors such as high incidence of ARVI in the first 3 years of life (odds ratio – OR 4.5; p<0.0001), smoking parents (OR 3.9; p<0.01), female (OR 2.06; p=0.041), patient age (OR 1.44; p<0.0001) and the early disease debut (OR 0.58; p<0.0001). Conclusion. The results obtained should be used to predict the severe course of bronchial asthma based on the analysis of anamnestic data complex available in practical healthcare, to organize dispensary observation of sick children.

Purpose. To study the prevalence of the digestive system pathology in children with systemic connective tissue diseases.

Characteristics of children and research methods. We carried out a clinical and instrumental examination of 108 children with systemic connective tissue diseases hospitalized in the rheumatology department of the 4th City Children’s Clinical Hospital in Minsk from 2008 to 2015. 60 patients suffered from juvenile idiopathic arthritis (mean age 12.3 [9.4; 15.6] years), 23 children suffered from juvenile scleroderma (mean age 11.8 [9.7; 14.9] years) and 25 children suffered from systemic lupus erythematosus (mean age 13.1 [12.2; 16.3] years). All patients received long-term immunosuppressive and anti-inflammatory therapy.

Results. 75.9% patients had gastroenterological complaints, such as abdominal pains, nausea, heartburn. 69.4% of patients had endoscopic changes in the esophagus, stomach, and/or duodenum. According to the results of the morphological study, 43.5% of patients with systemic connective tissue diseases had mild inflammatory process, 29.6% of patients had average inflammatory process, and 3.7% of patients had severe inflammatory process. Among the children examined, 33 (55%) patients with juvenile idiopathic arthritis, 12 (48%) children with systemic lupus erythematosus and 11 (47.8%) patients with juvenile scleroderma were infected with Helicobacter pylori. Pathological changes of the hepatobiliary system and pancreas were diagnosed in 83.3% of children with systemic connective tissue diseases.

Conclusion. The revealed changes indicate a probable connection between the pathology of the digestive organs in children with systemic connective tissue diseases and substantiate the gastroenterological examination of this category of patients. 

Nephropathy is a common associated pathology with hereditary connective tissue dysplasia

Purpose. To determine clinical and laboratory signs of renal pathology in the conditions of persisting hypoxic syndrome and anatomic abnormalities of the urinary system with hereditary connective tissue dysplasia syndromes in children

Characteristics of children and research methods. We examined 36 children with Ehlers–Danlos syndrome and 10 children with Marfan syndrome to reveal signs of metabolic disorders in the blood and urine.

Results. All children revealed abnormalities of the urinary system. In addition, children with Ehlers–Danlos syndrome demonstrated an increase in certain signs of dysmetabolic nephropathy when growing older: an increased content of parathyroid hormone in the blood, which inactivation and elimination is normally provided by the kidneys. Hypermicroproteinuria with a high content of microelements in proteins, increased excretion of medium molecules, lipid hydroperoxides, glycosaminoglycans, a decrease in antioxidant defense and crystal formation inhibitors are the characteristic signs of dysmetabolic nephropathy.

Conclusion. Children with hereditary connective tissue dysplasia syndromes have a risk of developing nephropathy with signs characteristic of dysmetabolic nephropathy, requiring dynamic monitoring by a nephrologist. 

Purpose. To examine the indicators of iron deficiency, the levels of hemoglobin, erythropoietin, hypoxia-induced factor 1-alpha (HIF-1α) in the blood of children with anemia and chronic kidney disease C1-5 prior to the dialysis and on its background, receiving and not receiving iron preparations and erythropoietin-stimulating drugs to establish the role of HIF-1α in the regulation of erythropoietin synthesis and erythropoiesis.

Results. The patients (n=80) with anemia and chronic kidney disease were divided into 3 groups: Group 1: 32 patients with chronic kidney disease C1-5 prior to the dialysis, not receiving therapy; Group 2: 18 patients with chronic kidney disease C2-5 prior to the dialysis, receiving iron-containing preparations and erythropoietin-stimulating drugs; Group 3: 30 patients with chronic kidney disease C3-5 on dialysis, receiving iron preparations and erythropoietin-stimulating drugs. Group 1: we found the increased levels of erythropoietin (28.65 ± 3.66 MIU/ml) and HIF-1α (0.089 ± 0.011 ng/ml; p=0.014 and p=0.005, respectively); Group 2: 63.01 ± 14.84 mIU/ml and 0.138 ± 0.025 ng/ml; p=0.0088 and p=0.005, respectively). Group 3: we found the increased level of HIF-1α (0.098 ± 0.01 ng/ml; p=0.005).

Conclusion. An increase in concentration of HIF-1α in children with anemia and chronic kidney disease C1-5 prior and on dialysis receiving and not receiving therapy with iron-containing drugs and erythropoietin-stimulating agents confirms the role of HIF-1α in the regulation of erythropoietin and erythropoiesis synthesis in anemia. 

In the first quarter of 2019, we registered 872 cases of measles in Russia, indicating a tense epidemic situation of this disease.

Purpose. To determine clinical and epidemiological features of measles in the Astrakhan region (AR) from January 2013 to April 2019.

Material and methods. We analyzed 663 cases of measles confirmed by enzyme immunoassay (293 children + 370 adults) in the AR from January 2013 to April 2019.

Results. We found that in the age structure of measles incidence in AO prevail patients aged from 18 to 29 years old, unvaccinated or lacking vaccination information, unvaccinated children of 1-2 years old and children under 1 year old, not subject to measles vaccination. The main reasons for the lack of vaccination in children with measles under 17 years are medical withdrawal; and among adults – insufficient vaccination of people under 35 years and the absence of vaccination in people over 35 years. The main reasons for the incidence of measles among vaccinated children and adults is the violations of vaccination, inaccuracy of official measles vaccination data, which is confirmed by serological monitoring, indicating a decrease in the seropositive individuals among vaccinated persons. The presence of measles foci and the spread of infection can be explained by nosocomial outbreaks, spread of measles in the organized groups as a result of untimely identification and isolation of patients. Measles in children and adults of different age groups, both vaccinated and unvaccinated, occurred typically with a predominance of moderate form. The Filatov – Koplik symptom, despite its detection in only 54% of patients, remains the main sign for the diagnosis of measles in the catarrhal period of the disease, and pigmentation is a sign of the past illness. 

Malignant neoplasms prevail in the structure of mortality in children all over the world, while most patients are diagnosed at the common stages; the second place is taken by external causes. It determines a particular relevance of developing new approaches to strengthen the role of district pediatricians in the early diagnosis of malignant neoplasms in children.

Material and methods. The Algorithm for determining the appropriateness of referring a patient to a pediatric oncologist was introduced in the medical organizations of the Arkhangelsk region in 2016. To evalute the effectiveness of the Algorithm, we compared two groups of patients with histologically verified solid malignant neoplasms: Group 1: 49 patients who received medical care in the Arkhangelsk region in 2011–2015 (prior to the experiment); Group 2: 51 patients who received medical care in the Department of Pediatric Oncology of the Arkhangelsk region in 2016–2018.

Results. The average survival time was significantly increased in Group 2016–2018 (30.3 ± 1.57 months) as compared with the survival time of patients receiving treatment in 2011–2015 (25.04 ± 2.05 months) (p=0.045). The following time parameters were reduced: from the moment of contacting the pediatric oncologist to the verification of the diagnosis – from 9.0 to 7.0 days; from verification of the diagnosis to the start of specialized treatment – from 12.0 to 8.0 days; from the moment of contacting the local pediatrician to the referral to the pediatric oncologist – from 11.0 to 2.0 days; from the moment of contacting the local pediatrician to the start of specialized treatment – from 23.0 to 9.0 days.

Conclusion: The organizational experiment confirmed the effectiveness of the Algorithm and the expediency of its implementation in the medical organizations. 

This article discusses the common nutritional problems of children in their first years of life, leading to nutritional deficiency in infants. A high frequency of micronutrient and vitamin deficiencies in nutrition is observed in both nursing mothers and their children; it can be explained by a lack of awareness of parents about the rules of feeding, the introduction of complementary foods and the fear of introducing solid food to children, especially if they have typical functional gastrointestinal disorders. This problem is solved by the fulfillment of the available clinical feeding recommendations, the rules for the timely and safe introduction of complementary foods using high-quality industrial products to ensure the physiological needs of the child in food ingredients while observing the psychological comfort of the children and their parents.

In 2016, a multicenter double-blind placebo-controlled randomized clinical trial in parallel groups was completed in the Russian Federation to evaluate the efficacy of the liquid dosage form of Anaferon for children in the treatment of acute respiratory viral infections in children. The design and main results of the study are presented in the largest foreign database ClinicalTrials. gov of the National Library of Medicine of the USA. In November 2018, the collection of reports of the XIII Russian Scientific  and Practical Conference with international participation “Health is the basis of human potential: problems and solutions” published the article of the Russian chemist N.N. Khromov-Borisov, where the author questions the reliability of the study of the liquid form of Anaferon for children. The presented article analyzes the legitimacy of the author’s claims. From the point of view of statistics, the analysis of N.N. Khromov-Borisov cannot be considered objective and correct, since it contains gross methodological errors.

The thrombotic complications develop in 3,6–13% of children with congenital heart defects in the postoperative period, which makes the diagnosis and prevention of thrombosis relevant. Permanent risk factors include the carriage of mutations/polymorphic variants of genes, leading to an imbalance in the procoagulant and anticoagulant hemostatic systems. In some cases, children after cardiac surgery are prescribed anticoagulants, warfarin is the most commonly used vitamin K antagonist, which can cause warfarin-associated skin necrosis as a result of vascular microthrombosis of the microvasculature. We provide our own observation – a case of thrombosis and warfarin-associated skin necrosis in a child after a multi-stage correction of congenital heart defect on the background of indirect anticoagulant therapy.

The article provides literature data and description of the clinical case of CHARGE syndrome with an autosomal – dominant type of inheritance in a child of 3 years and 7 months. The name of the syndrome is formed by the first letters of the characteristic congenital malformations: “C” (coloboma) – optic disc coloboma, “H” (heart defects) – cardiovascular abnormalities, “A” (atresia of choanae) – atresia or stenosis of choan, “R” (retardation of growth and development) – growth deficit, developmental delay, “G” (genital anomalies) – genital anomalies, “E” (ear abnormalities and sensorineural hearing loss) – hearing organ abnormalities. A feature of the clinical case is the early diagnosis of CHARGE syndrome, manifested by bilateral optic disc coloboma; unilateral paresis of the facial nerve; congenital heart disease (open aortic duct); retardation of growth, psychomotor and psycho-speech development; cryptorchidism; bilateral anomaly of the outer ear (short, wide, absence of the lobe and external curl), sensorineural hearing loss; bilateral renal hypopalasia, chronic kidney disease.

Annually more than 15 million children are born prematurely in the world. The clinical case illustrates the importance of an integrated approach for follow-up observation of children from 0 to 36 months old, born with very low and extremely low body weight. We studied the effectiveness of the Rehabilitation Center for children born with very low and extremely low body weight on the basis of a large multidisciplinary Bashlyaeva children’s hospital in Moscow 2012–2018. This center is necessary for a comprehensive assessment of the health status of children born with very low and extremely low body weight, as well as for the dynamic and timely diagnostics and the application of optimal treatment of revealed pathological changes. The development of differentiated programs for medical examination and rehabilitation of this group of children should include an optimal protocol of dynamic monitoring. It will reduce both the rate of incidence and disability, and improve the quality of life and social adaptation of children.

Adenotomy is one of the most common surgeries on the part of ENT organs in childhood, it is usually performed under endotracheal anesthesia.

Purpose. To evaluate the effectiveness of the spray containing sea water and brown algae extract in the early postoperative period in children after adenotomy.

Characteristics of the children and research methods. 24 patients aged from 4 to 10 years (12 boys and 12 girls) were examined and treated in the Otorhinolaryngology Department of the Perm Regional Children’s Clinical Hospital. All patients underwent adenotomy due to hypertrophy of the pharyngeal tonsil of the II–III degree, they received antibiotics for 5 days after surgery. The patients were divided into 2 groups: Group 1 (study group, n=12) used, Aqualor Protect spray for irrigation therapy of the nasal cavity and nasopharynx; Group 2 (comparison group, n=12) used a spray with isotonic sodium chloride solution for irrigation therapy. 2 doses of saline solution were sprayed into each nostril 4 times for 4 days.

Results. According to the medical examination, the study group demonstrated more pronounced improvement of the following indicators (visual analogue scale scores): overall health (p<0.05), swelling of the nasal cavity (p<0.05), hyperemia of the mucous membrane of the nasal cavity (p<0.05), improved nasal breathing (p<0.034), the presence of mucous-hemorrhagic crusts in the nasal cavity (p<0.0001), general feelings (p<0.02).

Conclusion. The spray with seawater and brown algae extract is more effective than nasal spray with isotonic solution in the early postoperative period in children after adenotomy. 

Objective: to present data and analyze current data on diagnostics of acute skin failure in children. The term “acute skin failure” is rarely used in the literature. Among the works published in Russian, we found only one study of our colleagues from the Republic of Belarus, where we met the definition and description of this term. When searching the Pubmed database, we found 58 scientific works, only 7 of them were devoted to children. When determining the presence or absence of multiple organ failure syndrome, none of the scales used for adults and children included skin disorders. The majority of scales described changes in the respiratory system, kidneys, liver, cardiovascular system, neurological status and blood system. However, acute skin failure is a serious problem similar to insufficiency of internal organs. The clinical case demonstrates the possibility of effective intensive care in a child with acute skin failure. We believe that the diagnosis of acute skin failure as a primary or secondary component of multiple organ failure syndrome in children in the intensive care unit is an important step towards the correct and adequate treatment of such patients.

The article presents the main milestones of the life and work of the academician G.N. Speransky, the scientific priorities of the scientist, and the scientific activities of some of his outstanding students. Since 1931, G.N. Speransky, the head of the Department of Pediatrics of the Central Institute for Advanced Medical Studies, is the author of more than 200 scientific papers on the development of the problems of physiology and pathology of early childhood, including the neonatal period. He scientifically substantiated methods of feeding, caring and raising children. G.N. Speransky, together with co-authors, was awarded the Lenin Prize for a series of works on the physiology and pathology of young children. A significant number of his works are devoted to neonatal sepsis, respiratory diseases, and acute gastrointestinal diseases. In 1922, G.N. Speransky organized journal “Pediatrics”, he was the editor for 47 years. G.N. Speransky was also the editor of the Pediatrics section of the 1st and 2nd editions of the Big Medical Encyclopedia, chairman of the All-Union Society of Pediatricians.