Pneumonia in children remains one of the most serious diseases. Despite intensive study, the diagnosis and treatment still present a variety of problems. The article is aimed to identify these problems and show the ways of their solution.

One of the criteria for the diagnosis of pneumonia is pulmonary infiltrate on the X-ray image. Community-acquired pneumonia is often caused by Pneumococci and mycoplasma; respiratory viruses, influenza usually serve as contributing factors for bacterial infection. The high level of hyperand underdiagnosis of community-acquired pneumonia is associated with its similarity with ARVI; the registration of general violations (signs of the severity of the disease) improves the quality of diagnosis. The clinical and radiological picture helps to distinguish pneumococcal pneumonia from pneumonia caused by mycoplasma. To improve the diagnosis of community-acquired pneumonia, it is necessary to study inflammation markers during the first examination of the patient, which is impossible in the conditions of home care. Due to the growth of resistance of pneumococci to macrolides a community-acquired pneumonia of pneumococcal (and unclear) etiology requires the use of amoxicillin in high doses and macrolides (in case of atypical pneumonia). Gentle therapy of destructive pneumonia, steroids for metapneumonic pleurisy allow avoiding invasive interventions and help to repair lung tissue.

Veltischev Researchand Clinical Institutefor Pediatricsofthe Pirogov Russian National Research Medical University, Moscow, Russia The article presents modern data on the most common monogenic connective tissue disease – Ehlers–Danlos syndrome. The authors describe two previous classifications of the syndrome: Berlin (1988) classification, which distinguishes 11 types of the disease, and Beyton (1998) classification, which includes 6 types of the syndrome and takes into account the results of molecular genetic studies. Particular attention is paid to a new classification, proposed by the International Consortium in 2017. This classification is based on the clinical and molecular genetic data and unites 13 types of Ehlers–Danlos syndrome, divided in 7 groups (A–G), depending on the main molecular defect. This defect determines the violation of various collagen structures (primary, spatial, cross-linking) and others constituents of the connective tissue (myomatrix, glycosaminoglycans, complement component, etc.). The classification provides general clinical symptoms for all types of the disease and comprehensive information on the specific signs of each of the 13 types of the syndrome.

The authors discuss approaches to the pathogenetic therapy of the syndrome, the possibilities of symptomatic treatment, including both medications of different spectrum of action, and physiotherapeutic measures, exercise therapy. The complex of the listed therapeutic measures is aimed at stabilizing the main pathological process, preventing complications, improving the patient’s quality of life and social adaptation. The authors emphasize that correct patient management, targeted medical supervision and medical genetic counseling requires molecular genetic verification of the diagnosis.

.Both changes in diet and pathological conditions caused by an infectious agent, allergic or autoimmune inflammatory process, affect the biological rhythms of the digestive tract, which negatively affects the intestinal microbiota and increases the permeability of the intestinal mucosa. The altered microbiota potentiates inflammation and causes a “vicious circle”. The zonulin protein is the agent that modulates the density of intercellular connections. The review presents data on the biological role of zonulin, correction of its synthesis violation with the help of functional products for baby food.

Sclerosing cholangitis is one of the most common hepatologic extraintestinal manifestations of inflammatory bowel disease. The article discusses the phenotype of the combination of sclerosing cholangitis and inflammatory bowel disease. The authors present their theories of the etiopathogenesis of sclerosing cholangitis in patients with inflammatory bowel disease, as well as some features of the phenotype of both mixed and monogenic forms of diseases.

Sclerosing cholangitis in combination with inflammatory bowel disease is commonly associated with pancolitis, but the endoscopically visualized activity of inflammatory bowel diseases is significantly lower and clinical symptoms are less pronounced. The authors have established that the patients with the combination of sclerosing cholangitis and inflammatory bowel disease are at the increased risk of developing malignant neoplasms. The formation mechanisms of a combination of inflammatory bowel disease and sclerosing cholangitis remain poorly understood, although this pathology is influenced by lymphocytic cross-reactivity, aberrant recognition of microbiotic epitopes and intestinal microbiota imbalance. New biological agents aimed at correcting the interaction between the immune system and target organs may provide new ways of treatment for sclerosing cholangitis associated with inflammatory bowel disease.

For the first time in 1946 E.L. Potter (1901–1993) described the characteristic appearance of stillborns and deceased newborns with bilateral renal agenesis. Due to the further observations Potter distinguished the syndrome (Q60.6) – a set of characteristic external signs that are formed due to the extreme degree of oligohydramnios and intrauterine compression of the fetus. Classical Potter syndrome is diagnosed by the disfunction of both kidneys in the fetus (for example, bilateral agenesis), which leads to death. The term «Potter sequence» or oligohydramnios sequence with diverse causes has received the wide clinical use. The term «renal oligohydramnios» (ROH) is used to describe oligohydramnios resulting from a decrease or absence of fetal kidney function. The authors state that renal oligohydramnios and Potter sequence often develop in the fetus with cystic kidney disease with the formation of cysts in the parenchyma of both kidneys (autosomal recessive polycystic kidney disease, autosomal dominant polycystic kidney disease, glomerulocystic kidney disease associated with HNF1ß/TCF2 gene mutations, renal-coloboma syndrome, cystic renal hypoplasia, cystic renal dysplasia with mutations of the CEP55 gene).

Intrauterine intravascular blood transfusion is an effective and relatively safe treatment method for severe fetal anemia. This technique have been used for more than 30 years already, however, there are no systematic works devoted to the features of the cardiovascular system in fetuses and newborns after intrauterine intravascular blood transfusion. The authors have decided to study this problem and have conducted a prospective trial of fetuses and children (n=34) with hemolytic anemia who underwent intrauterine blood transfusion. To assess the condition of the cardiovascular system, the scientists have carried out an ultrasound examination of the heart, which results have revealed pathological changes: dilatation of the cardiac cavities, myocardial hypertrophy, hydropericardium, cardiomegaly still present in the postnatal period. The scientists have used fetal and neonatal therapy for the patients with these pathologies. In the case of late correction of severe fetal anemia, there was a progressive deterioration in the condition of the fetus and remodeling of the cardiovascular system, which indicates the need for timely diagnosis. Based on the results of the study, the scientists have developed the protocols for ultrasound diagnostics of fetuses and newborns with moderate and severe anemia.

The article presents the literature data and the results of the authors’ observations of preterm children in the postnatal period, born with extremely low and very low body weight, with the definition of the leading psycho-neurological disorders, which cause disabling conditions in this group of patients. The authors highlight the difficulties of predicting the neuropsychological development of deeply premature and extremely premature children. The scientists note the importance of a comprehensive assessment of their health status. They justify the necessity of improving the system of medical support for such children at all stages of nursing, rehabilitation and the introduction of a personalized approach to clinical practice with the development of differentiated programs of medical supervision and habilitation.

Objective: to study the clinical features of TORCH syndrome in newborns with congenital cytomegalovirus infection depending on body weight at birth

Characteristics of children and research methods. The study included 70 patients (43 premature, 27 full-term) with with congenital acute cytomegalovirus infection, manifested form. Patient characteristics: Group 1: 21 premature children with extremely and very low body weight at birth; Group 2: 22 premature children with very low body weight; Group 3: 27 full-term children with body weight more than 2,500 g. The congenital cytomegalovirus infection was diagnosed on the presence of TORCH syndrome and etiological verification in the first 3 weeks of life.

Results. The authors found that children with extremely and very low body weight significantly more often (p<0.05) had hydrocephalus and interstitial pneumonia than children with low body weight. The full-term patients weighing more than 2,500 g suffered from hemolytic anemia, brain calcifications and congenital heart defects (p<0.05) statistically significantly more often than preterm patients. Reduced duration of pregnancy in preterm labor contributes to the rare occurrence of hemolytic anemia and calcifications in the brain of premature babies. Periventricular localization of calcifications is characteristic of full-term infants. It is necessary to exclude congenital cytomegalovirus infection in newborns with congenital heart defects and other clinical manifestations of TORCH syndrome.

Conclusion. The results of the study can be used to diagnose cytomegalovirus infection in children, depending on the birth weight.

The article evaluates the effectiveness of thiamphenicol glycinate acetylcysteinate for inhalation versus systemic antibiotic for oral administration in complex treatment of uncomplicated rhinosinusitis in the outpatient conditions.

Characteristics of children and research methods. The prospective, single-center, randomized, pilot, open-label comparative study included 61 patients from 3 to 9 years old with acute rhinosinusitis meeting the inclusion criteria. Group 1: children were prescribed thiamphenicol glycinate acetylcysteinate (0.125 mg per inhalation twice a day), Group 2: patients received amoxicillin clavulanate at an age-specific dosage of 40-45 mg/kg orally 2 or 3 times a day. At each visit (Day 0 and Day 7–8) the scientists assessed such symptoms as nasal breathing and rhinoscopy findings (edema, hyperemia of the mucous membrane and nasal discharge) on a 10-point visual analogue scale. At the repeated examination (Day 7-8), they assessed the effectiveness of the treatment (recovery/improvement/cure), the duration of treatment (in days) and the number of drugs taken simultaneously, as well as the tolerability of treatment by the presence/absence of undesirable or side effects. The Python language (Python 3.8.) was used for statistical processing of the obtained data. They used the built-in functions from the Scipy module for calculation.

Results. At the time of inclusion in the study (Day 0), the condition of the patients in both groups was comparable (p>0.05). When comparing condition of the patients on Day 0 and Day 7-8 there was a therapeutic effect in both groups for all studied symptoms (p<0.01) and for the duration of the treatment course (p=0.2302). Thiamphenicol glyceinate acetylcysteinate reduced the need for other drugs in the complex treatment of sinusitis (p<0.001). In most cases the result of treatment was assessed as recovery. None of the observed patients had undesirable side effects.

Conclusion. Thiamphenicol glycinate acetylcysteinate (Fluimucil-antibiotic IT) in the form of inhalation is as effective as amoxicillin clavulanate for the treatment of children with acute uncomplicated rhinosinusitis. A decrease in the need to take additional drugs (p<0.001) can be considered as the advantage of thiamphenicol glycinate acetylcysteinate in complex treatment for uncomplicated rhinosinusitis.

The study of adolescent obesity and pathogenic factors of its development is getting more and more important as the disease aggravates in adulthood. The serious progress in the study of the pathogenesis of this process can be achieved by analyzing the molecular mechanisms of the obesity development in childhood and adolescence. An individual approach to the diagnosis and treatment of obesity includes various factors, including the patient’s nationality. In recent decades there is an increase in the incidence of obesity among the representatives of the Mongoloid race, which is not characteristic of this racial group. Thus, the aim of the study was to analyze the state of lipid metabolism and the level of antioxidant components in the Buryat girls with exogenous constitutional obesity of the 1st degree. The authors examined 44 girls with exogenous constitutional obesity of the 1st degree and 48 practically healthy adolescent girls (comparison group) of the same age. All the girls belonged to the Buryat ethnic group. They used spectrophotometric and fluorometric research methods. The study revealed that exogenous constitutional obesity in adolescent girls is accompanied by the development of dyslipidemia (increased concentrations of total cholesterol, triacylglycerols, very low density lipoprotein cholesterol, an increase in the atherogenic coefficient, a decreased level of high density lipoprotein cholesterol), as well as a deficiency of antioxidant defense components (decreased levels α-tocopherol, retinol, activity of superoxide dismutase and glutathione-S-transferase). These results expand the understanding of the pathophysiological mechanisms of adolescent obesity and develop individual approaches to the treatment of this condition.

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Objective. To study the opinion of parents (legal representatives) on the problems of medical care for children with oncological diseases.

Methods. The study was based on the Questionnaire for parents (legal representatives) on medical care children with cancer. The questionnaire consisted of 27 questions, the respondents were asked to choose one or several answers or to enter their own option.

Results. This medical and social study demonstrated that 81.1±1.9% of respondents are not satisfied with the attitude of doctors and nurses towards them and their children, 15.9±1.8% of respondents are partially satisfied and only 3±0.8% of respondents are fully satisfied. The reasons for dissatisfaction were as follows: rude communication (35.8±2.4%), inadequate attention of medical personnel (21.3±2.0%) and lack of interest in the treatment success (19.7±2.0%). The combination of these options was noted by 23.2±2.0% of the respondents. 63.7±2.4% of respondents would prefer to treat their children at a medical organization of federal subordination, 33.9±2.4% – at medical organization outside the territory of the Russian Federation, of which 57.4±2.4% would like to receive a patient-oriented service and simplified routing between medical organizations during treatment. 12.7±1.6% mistrust in the qualifications of medical personnel in the Russian Federation, 11.8±1.6% of respondents indicated the lack of comfortable conditions for examination and treatment, 5.3±1.1% of respondents indicated the lack of necessary diagnostic and treatment methods and 12.8 1.6% of respondents indicated a combination of the above options.

Conclusion. The results obtained indicate the need to implement patient-oriented approaches and to improve the routing of children with cancer.

The article describes a clinical case of a teenage girl with systemic lupus erythematosus with neuropsychiatric manifestations. The overview part describes modern approaches to the diagnosis and treatment of children with systemic lupus erythematosus with central nervous system lesions. The authors describe actual Russian and international clinical recommendations, they pay particular attention to the imperfection and inconsistency of classifications, differences in terminological systems, and the features of the rheumatologists’ and psychiatrists’ clinical practice. They provide the recommendations on the correct diagnosis wording in children with neuropsychiatric symptoms as a part of autoimmune disease. The article substantiates the necessity of an interdisciplinary approach and the cooperation of pediatric rheumatologists, psychiatrists, neurologists, clinical psychologists.

Donnai–Barrow syndrome is a multi-system disorder characterized by a variable combination of congenital anomalies, progressive myopia, sensorineural hearing loss, intellectual disability and renal disease. The article describes clinical cases of children with different phenotypes of the syndrome, including different renal disorders. One patient had isolated low-molecular-weight proteinuria, another patient suffered from proteinuria, hypercalciuria, nephrocalcinosis. Disruption of megaline-mediated endocytosis, retrograde endosomal transport of ligands, mitochondrial dysfunction, stress of the endoplasmic reticulum can lead to a different spectrum and various degrees of severity of tubular dysfunction in Donnai-Barrow syndrome. A variety of clinical manifestations of the disease can lead to a low diagnosis of Donnai-Barrow syndrome and inadequate patient management.

The article presents a rare case of type II mucopolysaccharidosis (MPs) in children. From the age of one and a half, the child was observed by an ENT doctor for 2–3 degree adenoids. At the age of 2, an adenotomy was performed. In the future, a relapse of adenoid hyperplasia. 1 year of age according to the ultrasound revealed hepatosplenomegaly. At the age of three years, type 2 mucopolysaccharidosis was suspected, which was confirmed on the basis of increased levels of heparan sulfate and dermatansulfate in the urine, molecular genetic diagnostics (mutation in exon 3 of IDS Lis135Glu in the hemizygous state. Appointed replacement therapy: Elaprase. Clinical polymorphism, different severity of symptoms combined with rare occurrence cause certain difficulties in early identification of MPs-II. Timely diagnosis is extremely important for referral of such children to specialists of an interdisciplinary center who have experience in specific treatment that is most effective in the early stage of the disease.

The physical development of newborns and infants is the most important parameter in assessing their health. However, the standards of anthropometric parameters, and most importantly their growth in premature babies, especially those born with low and extremely low body weight, are still controversial.

Objective: to apply the methodology for measuring and assessing the main anthropometric indicators of newborns using the international standards of postnatal growth of premature babies INTERGROWTH-21st to resolve the issue of the feasibility of its adaptation and widespread introduction into neonatological and pediatric practice in the Russian Federation. 4876 children were born at Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology (participant of the international project for the implementation of INTERGROWTH-21st standards in neonatological and pediatric practice) from 15.05.2018 to 26.11.2018. 439 (9%) of them were born premature, of which 376 children (89,3%) with gestational age confirmed by ultrasound were included in the study. There was a detailed file for each child to assess the environment and health of the mother, the course of pregnancy, childbirth, the state of health of the child from birth to discharge from the hospital. 2.1% of children classified by INTERGROWTH-21st as children with intrauterine growth retardation, according to Fenton charts, had normal intrauterine growth. When comparing the postnatal growth at discharge from hospital on the Fenton scale, more children were classified as children with impaired postnatal growth; the discrepancy between INTERGROWTH-21st and Fenton diagrams was 8%. At discharge, 52% of premature babies were exclusively breastfed, 28.8% of premature babies were at mixed feeding.

Conclusion. On the one hand, the use of INTERGROWTH-21st scales helps to correct nutrition in case of delay in physical development, and, on the other hand, it reduces the risk of metabolic disorders associated with an aggressive desire to achieve rapid growth of a premature baby.

Congenital malformations of the bronchopulmonary system in combination with tuberculosis are extremely rare in practice, but they usually require surgical treatment. This combination often causes diagnostic difficulties.

Characteristics of children and research methods. We have analyzed the experience of surgical treatment of a combination of various congenital lung defects and tuberculosis in 20 children. 9 (45%) children had lung sequestration, 4 (20%) children had lung hypoplasia, 5 (25%) children had congenital bronchiectasis, 1 (5%) child had arteriovenous malformation, and 1 (5%) child had a congenital lung cyst. There were 3 segmental resections, 15 lobeand bilobectomies, and 3 pneumonectomies. All surgeries were performed using minimally invasive approaches (VATS).

Results. 11 (55%) patients had diagnostic difficulties of concomitant pathology. Only in 5 (25%) cases they found mycobacteria in sputum before surgery. Postoperative study of material from tuberculous lesions helped to establish multidrug resistance of mycobacterium tuberculosis and to change the chemotherapy regimen in 3 (15%) patients. There were no postoperative complications or deaths. All surgeries were effective. Long-term results were studied in 16 (80%) of 20 patients over a period from 1 to 10 years. No cases of reactivation of tuberculosis or disability.

Conclusion. Resection surgery for combination of tuberculosis and congenital lung defects allowed to clarify the diagnosis and to achieve a direct effect in all patients by clarifying the drug resistance of mycobacterium tuberculosis and to optimize postoperative chemotherapy.

The article presents the main results of a randomized clinical trial of the efficacy and safety of Panavir®, rectal suppositories 100 μg, in children with influenza and other acute respiratory viral infections (ARVI).

Characteristics of children and research methods. The trial included 40 children from 12 to 17 years old, the children were randomized into 2 groups: 20 children were treated with Panavir®, 1 rectal suppository per day for 7days, 20 children received placebo according to Panavir® regimen on the background of standard symptomatic therapy. The scientists monitored the efficacy criteria: the primary criteria: recovery time and the number of patients with successful treatment (normalization of axillary body temperature no later than 48 hours of therapy); the main secondary criteria: the timing of the normalization of body temperature, the disappearance of intoxication and catarrhal symptoms and satisfaction with the results of treatment by the parents /adoptive parents of patients on the IMPSS scale.

Results. In the Group of Panavir®, the authors registered shorter mean recovery periods (4.5 days versus 7.4 days in the Group of Placebo), normalization of body temperature (39.1 hours versus 76.1 hours) and regression of intoxication manifestations (2.9 days versus 4.3 days) and catarrhal (3.4 days versus 5.6 days) syndromes (with statistical significance of intergroup differences). The treatment success was achieved in 85% of cases in the Panavir® Group versus 30% of cases in the Placebo Group; according to this criterion Panavir® exceeded placebo by at least 24.7%. At the end of the therapy course, 80% of the parents /adoptive parents were satisfied with the treatment results in the Panavir® Group versus 25% of cases in the Placebo Group. The authors determined a comparable incidence of adverse events in the Panavir® and Placebo Groups. There were no adverse events associated with the administration of Panavir®.

Conclusion. This study established the efficacy and safety of Panavir® in the treatment of children of 12-17 years old with influenza and other acute respiratory viral infections. This drug with a combined antiviral and immunomodulatory effect is promising for pediatric practice.

The article discusses the issues of the protective mechanisms of the mucous membranes of the upper respiratory tract: mucociliary clearance, production of protective proteins and the mucosal immunity system. The data on the significance and relationship of lymphoid structures associated with mucous membranes of various anatomical localization are presented. The mechanisms of action of topical bacterial lysates are considered, their effectiveness in the treatment and prevention of respiratory diseases and their complications is described.