Interstitial lung diseases refer to a large group of diseases with a severe course and unfavorable prognosis, since the most forms of these diseases cause irreversible fibrosis of the lung tissue and severe respiratory failure. Lung transplantation remains the only way to save the patient’s.
The article highlights the current state of the problem, achievements and prospects in the study, diagnosis and approaches to the treatment of interstitial lung diseases in children. The authors emphasize that this direction is one of the most relevant in pediatric pulmonology and clinical medicine in general.

Despite the recent achievements in searching for the causes of monogenic human diseases, there is still a massive gap in understanding the molecular causes of phenotypic variability. At the moment, it is evident that the pathogenic genetic variant often acts together with the other genetic and non-genetic factors that can reduce or, on the contrary, aggravate the severity of the disease. Thus, to completely understand the disease, we shall consider the entire set of mechanisms leading to the resulting phenotype. This paper reviews the current state of the art in identifying genetic and non-genetic phenotype modifiers for rare monogenic cardiovascular diseases.

The article presents literature review of the diseases associated with mutations in the FLNA gene encoding filamin A, which is a cytoskeleton protein with polymorphic functions. The mutations of this gene lead to the damage of the central nervous system (periventricular nodular heterotopy), respiratory organs (emphysema, interstitial lung disease), heart (congenital heart defects and minor heart abnormalities); the listed diseases can be diagnosed at different ages. The information presented in this review can be useful for clinical geneticists, specialists in rare (orphan) diseases, pediatricians, neonatologists, neurologists, epileptologists, pulmonologists, surgeons, cardiologists for timely diagnosis and improvement of medical care for patients requiring a multidisciplinary approach.

This review demonstrates the role of the immune system in the development and progression of cardiac pathology caused by viral infection. The authors describe the role of lymphocytes (T- helper-17–Th17) in the viral persistence in myocardial tissue. They provide the information on increased level of interleukin-17A, the main Th17 cytokine in patients with dilated cardiomyopathy, which proves role of these cells in the progression of cardiac pathology. They discuss the role of T regulatory (Treg) lymphocytes in inflammatory heart disease.

The article provides current information on comorbid diseases in both adults and children with cholelithiasis. It describes their pathogenetic relationship with the formation of gallstones. The authors highlight the causal factors in the development of both gallstone disease and accompanying comorbid conditions. They demonstrate the generality of metabolic disorders in cholelithiasis and metabolic syndrome. The article describes the mechanisms of formation of both cholesterol and pigmented gallstones, which can be formed against the background of both somatic and infectious diseases. The article shows the role of drugs in the formation of gallstones. The authors substantiate the necessity of an individual approach and the development of personalized methods of prevention and treatment of cholelithiasis in children.

Objectives: To study the potential of the bioimpedance method for nutritional status assessment in children with cerebral palsy.
Material and methods. There were examined 89 children with cerebral palsy (average age: 10,24 years±3,6 years). Such anthropometric indicators as body height (cm), body weight (kg) and body mass index (BMI, kg/m²) were investigated. The scientists formed two comparison groups: Group 1: 40 children without malnutrition (z-score BMI over 1), Group 2: 49 children with malnutrition (z-score BMI is 1,1 and less). The body composition was evaluated by bioimpedancemetry in both groups of comparison.
Results. The scientist found significant differences in body composition in terms of fat mass between boys and girls with cerebral palsy, both with malnutrition and without nutritional deficiency. They determined that children with cerebral palsy without malnutrition demonstrate various changes in the parameters of the body composition comparable to those in patients with trophic deficiency, even before the anthropometry indicators change. According to the parameters of lean mass, active cell mass in the group of children without malnutrition, the results of 40% of boys and girls were below the normative values, which indicated an altered tissue composition of the body and existing deficiency of the protein component.
Conclusion. The scientists determined unidirectional changes in the body component composition in children with cerebral palsy and malnutrition and in children without trophic deficiency. The assessment of the body by bioimpedance measurement can serve as an effective tool for the diagnosis of nutritional disorders in children with cerebral palsy.

Objective. To determine the clinical and metabolic changes in children born from mothers with gestational diabetes mellitus and to predict perinatal injury of the central nervous system (CNS), taking into account the level of maternal hyperglycemia.
Material and methods. The period of early postnatal adaptation was analyzed in 258 full-term infants, who were divided into two groups, depending on the glucose level in the mother’s venous blood during pregnancy: Group 1: 5,1–5,6 mmol/L, Group 2: 5,7–7,0 mmol/L.
Results. Based on clinical, functional and laboratory markers (electrolyte balance and carbohydrate metabolism in the blood of a newborn) there was established a correlation between the severity of maternal hyperglycemia and the severity of neonatal disorders. In Group II infants born from mothers with more severe hyperglycemia are more likely to have a respiratory distress syndrome and ischemic-hypoxic injury of the central nervous system in combination with excess birth weight which significantly complicates postnatal adaptation.
Conclusion. The concentration of neuron-specific enolase of 4,9 ng/ml in the fetal amniotic fluid is an antenatal marker of perinatal damage to the central nervous system in a newborn.

40 adolescents aged 15–17 years with functional cardiovascular pathology of vegetative origin underwent a 24-hour blood pressure monitoring (using the oscillometric method BPLabVasotens, Peter Telegin LLC, Nizhny Novgorod) with an assessment of central blood pressure parameters and rigidity of the main arteries.
The scientists found significantly higher values of central systolic pressure during the day and night hours in the group of adolescents with arterial hypertension (n=13) compared to adolescents with normal blood pressure (n=27). They determined significantly higher values of the pulse wave velocity both during 24 hours and in the day and night hours in the group with arterial hypertension compared to the group of adolescents with normal blood pressure. No differences were found in the parameters of the propagation time of the reflected pulse wave and the augmentation index. The time of the reflected pulse wave propagation was significantly lower at nighttime compared to the daytime. In the group with arterial hypertension, the rate of pulse wave propagation in the aorta correlated only with the values of diastolic pressure over 24 hours and diastolic pressure in the daytime. In the adolescents with normal blood pressure, the pulse wave velocity correlated with systolic and pulse blood pressure. The augmentation index in the group of adolescents with arterial hypertension correlated with diastolic pressure, in contrast to the group of adolescents with normal blood pressure, where such a correlation was not detected.

The authors present their own results of scientific research on the clinical and paraclinical characteristics of various stages of chronic kidney disease in children. They determine pathogenetic markers for the diagnosis and prognosis of chronic kidney disease in childhood with the assessment of the set of endothelial dysfunction markers. The authors proved diagnostic and prognostic value of a stepwise increase in arterial hypertension, urinary excretion of prosclerotic, pro-inflammatory cytokines and growth factors with a simultaneous decrease in urinary excretion of anti-inflammatory cytokines, an increase in the level of microalbuminuria, disturbances of urodynamics and intrarenal hemodynamics.
The authors developed an algorithm for the diagnosis of chronic kidney disease in childhood, taking into account pathogenetic factors with an assessment of the complex of clinical and paraclinical, structural, functional parameters, hemodynamic changes in the kidneys in combination with the parameters of endothelial function and cytokine status.

Objective. To study predictors of bronchial asthma development in children of 3-4 years old with recurrent broncho-obstructive syndrome by 5-6 years of age.
Material and methods. The authors conducted a prospective cohort study in 2018-2020 with the participation of 61 children aged 3–4 years with recurrent broncho-obstructive syndrome. Inclusion criteria: age 3–4 years; ≥ 3 episodes of broncho-obstructive syndrome in the past 12 months; informed voluntary consent. At the first visit the scientists determined the levels of serum periostin, specific inhalation allergens IgE, total IgE and the number of eosinophils in the blood. In 24 months, at the second visit, bronchial asthma was diagnosed based on the GINA criteria (updated 2019). Binary logistic regression analysis was used to study the predictors of bronchial asthma development.
Results. In 24 months from the beginning of the study, bronchial asthma was diagnosed in 29 (47%) children with recurrent broncho-obstructive syndrome. The level of serum periostin and total IgE had no effect on the risk of developing bronchial asthma (p=0,194; p=0,269). According to binary logistic regression, the predictors of bronchial asthma development are burdened family allergic history (odds ratio – OR 3,81; 95% confidence interval – CI 1,14-12,74; p=0,015), allergic rhinitis (OR 4,94; 95% CI 1,37–7,80; p=0,0001), sensitization to the Dermatophagoides pteronyssinus (OR 1,80; 95% CI 1,36–1,13; p=0,045) and eosinophilia (OR 2,10 ; 95% CI 1,23-4,83; p=0,055).
Conclusion. A burdened family allergic history, the presence of allergic rhinitis, sensitization to the Dermatophagoides pteronyssinus, eosinophilia are the predictors of bronchial asthma development in children with recurrent broncho-obstructive syndrome by 5-6 years of age.

The article discusses diagnostic approaches to the patients of the first months of life with the signs of acute respiratory failure. It describes a clinical example of a two-month-old child with a cardiopulmonary insufficiency hospitalized in the infectious hospital; the insufficiency was regarded as a manifestation of acute infection of the lower respiratory tract. A detailed analysis of the situation revealed another reason – congenital heart disease. The authors discussed the issues of differential diagnosis.

Takotsubo syndrome (stress-induced cardiomyopathy) is well known in adult cardiology practice, and it also occurs in children. Takotsubo syndrome develops after emotional or physical stress. There is an acute transient disorder of the left ventricle contractility of the apex. The development of pain syndrome, electrocardiographic changes and troponins elevation imitate myocardial infarction. The key diagnostic point is intact coronary arteries and complete reversibility of local contractility disorders over the time.
The article describes the clinical case of takotsubo syndrome in an adolescent with type III spinal muscular atrophy after planned surgery (repeated posterior spondylodesis).Takotsubo syndrome with clinical manifestations of acute left ventricular failure and pulmonary edema developed in the early postoperative period with distinctive electrocardiographic changes (ST segment elevation, local impairment of left ventricular contractility according by echocardiography and ventriculography, and increased levels of troponin). Timely examination and correction of therapy confirm the clinical diagnosis. The regression of symptoms occurred within a few days.

Chickenpox is a common disease leading to a large number of clinical manifestations, ranging from mild spontaneously resolving forms to severe complicated cases requiring hospitalization and parenteral therapy. Despite the fact that this infection is benign in the majority of cases, it can lead to disseminated life-threatening processes in pregnant women and unimmunized newborns infected during the perinatal period, as well as it can cause intrauterine death and fetal abnormalities.
Currently, there are no unified therapeutic approaches in the management of pregnant women with chickenpox. The nature and severity of infection in children depends on the moment of infection (before or after birth, intrapartum), the immune status of the mother against the human herpesvirus type 3 (HHV-3), the gestational age of the fetus and the presence of concomitant conditions.

Currently, there is a lot of effort that is been done to optimize healthcare management for patients with hereditary angioedema, but there are still difficulties in the regions. Epidemiological data on hereditary angioedema indicate a low level of diagnosis. In several regions, patient pathway have been developed with a detailed description of the different steps on the patient healthcare journey. The patient pathway is a key component of high-quality medical care with optimal timeliness and financial costs. The article is devoted to the organization of hereditary angioedema patients’ pathway.

The study and monitoring of international indicators of the health status of premature infants is the current vector of scientific research in neonatology.
Objective. To assess the effectiveness of the nursing technologies of premature babies in the Republic of Belarus based on the analysis of long-term indicators of morbidity, mortality and survival of the infants with extremely low body weight.
Material and methods. Statistical reports data were used for a retrospective analysis of epidemiological indicators for the period of 2002–2018.
Results. The proportion of premature infants in the Republic of Belarus was stabilized at 4,2–4,5% increase in the absolute numbers, including those born weighing less than 1500 g. The ratio of the absolute number of alive and stillborn infants with extremely low body weight is many times higher than the initial values (p<0,05). The survival rate of infants with a body weight of 500–999g up to 1 year increased 2 times to the maximum level in 2018 (81,5%). There was an increase in the overall incidence of premature children in 2002–2010 (p<0,05) and a decrease by 2016 (p=0,001), including those born with a body weight of 500–999 g. Respiratory distress syndrome, intrauterine hypoxia and asphyxia during childbirth, an increase in the frequency of infections specific to the perinatal period (p <0,05) were registered more often. Among the children born with a body weight of 500–999 g, the incidence of congenital pneumonia for the period 2002–2009 increased to a maximum level of 263,4 ‰ (p <0,05) with a twofold decrease by 2018. The republican mortality rate of premature babies was stabilized at the level of 0,95 ‰ in the last 9 years, while the indicator was decreased 5 times among children weighing 500–999 g.
Conclusion. The assessment of the long-term dynamics of the main indicators of the health status of premature infants in the Republic of Belarus testified to the effectiveness of a complex of organizational and medical measures for their nursing.

Danon disease is a rare hereditary disease with predominant damage to the heart and skeletal muscles. Danon disease is referred to lysosomal storage disorders with severe, progressive course and it often leads to an early mortality. The main cause of Danon disease is the mutations in the LAMP2 gene in the Xq24–q25 chromosome region. Danon disease has X-linked dominant nature of inheritance; women have a milder phenotype with older heart damage. For the first time the disease was described in boys with cardiomyopathy, severe skeletal myopathy and intellectual deficiency. The article presents up-to-date review on Danon disease and case reports of the cardiology department of the National Medical Research Center for Children’s Health, where 5 male patients were observed from 2014 to 2020, and the average age at diagnosis – 13,2 years. During the lab examination three patients demonstrated a significant increase of the heart failure marker (NT-proBNP) – more than 5 thousand pg/ml, the intracellular enzymes was more than 2,5 norms in 4 patients. The Wolff–Parkinson–White phenomenon is recorded in all patients on the ECG. Echocardiography in one patient showed a rare combination of myocardial hypertrophy and non-compact left ventricular myocardium. 2 patients had a combination of hypertrophic and dilated phenotypes with a reduced ejection fraction on Echo and severe myocardial fibrosis on MRI of the heart, which clinically manifested with chronic heart failure refractory to drug therapy, which required heart transplantation.

Objective. To evaluate the features of the course of the early neonatal period of newborns born from young women under conditions of epidural analgesia.
Material and methods. The authors conducted a prospective, comparative, randomized, longitudinal, monocenter study. Patients of Group 1 (n=25) were anesthetized with a narcotic analgesic (2% Тrimeperedine 1,0 ml intramuscularly). Patients of Group 2 (n=30) received long-term epidural analgesia (EA) with 0,2% Ropivacaine (10,0 ml/hour). All patients delivered full-term infants. The course of labor, clinical and laboratory characteristics of newborns in the early neonatal period were evaluated.
Results. There were no significant differences in the duration of labor, volume of blood loss, and other characteristics between the groups. There were no clinical differences between the groups of newborns. We did not find a negative effect of epidural analgesia on the Apgar score at the 1st (p=0,166) and 5th (p=0,217) minutes of life and the neuropsychiatric status of the newborn (p=0,322). At the same time, in the group of long-term epidural analgesia, there was a tendency to a higher incidence of moderate and mild asphyxia (19,2% versus 9,5%; p=0,436). When comparing the acid-base state of umbilical cord arterial blood, significant differences were found in the following indicators: lower pH (p=0,042) and pO₂ level (p=0,007) and higher pCO₂ level (p=0,031) in arterial cord blood.
Conclusion. Epidural analgesia during labor in young women is accompanied by a lower level of pH and pO₂ and a higher level of pCO₂ in the arterial cord blood as compared to a Group of Тrimeperedine, which indicates a more pronounced shift in the acid-base state of the fetal blood. When analyzing neurological outcomes in newborns, there were no statistically significant differences. However, further follow-up is required for children born from young mothers who have received long-term epidural analgesia in labor.

Objective. To study microbiological peculiarities of intestinal microflora in children of early age with acute intestinal infections.
Material and methods. The scientists carried out the bacteriological examination of 338 children with acute intestinal infections, who were on inpatient and outpatient treatment. The age structure of patients on inpatient treatment from 0 to 6 month – 52 (29,1%), 7–12 month – 57 (31,8%), 1–3 years – 70 (39,1%). The distribution of children on outpatient treatment: from 0 to 6 month – 59 (37,0%), 7–12 month – 35 (22,0%), 1–3 years – 65 (41,0%).
Results. It was determined that absence of Escherichia coli (55,7%) and bifidobacterium (81,2%) prevailed in patients on inpatient treatment while in patients on outpatient treatment there was the reduction of these bacteria (67,3% and 36,5% accordingly). Among the representatives of opportunistic microflora, the leading place was occupied by Candida albicans, which was diagnosed in 50% of both inpatient and outpatient children. Only 12,8% cases there were diagnosed with Staphylococcus aureus, Proteus, one patient was diagnosed with Klebsiella, and 5 (9,6%) patients were diagnosed with Salmonellaenteritidis.

Objective. To assess the condition of newborns from women of the late reproductive period (LRP), depending on the parity of births.
Material and methods. The authors examined 130 pregnant women and their 130 newborns. Group I consisted of 60 primiparous women of late reproductive period and their 60 newborns, Group II included 40 multiparous women of the late reproductive period and their 40 newborns, Group III (control group) consisted of 30 healthy pregnant women aged from 18 to 25 years and their 30 newborns. The authors performed a retrospective analysis of the gestation course and perinatal outcomes. Ultrasound and neurosonography were used among the instrumental research methods.
Results. Women of the late reproductive period gave birth to children in a state of asphyxia twice more often, who develop neurological disorders 1,5 times more often and complex postnatal adaptation and disorders of the perinatal period are twice more likely. Among women of the late reproductive period, primiparous women gave birth to children with the most severe disorders.
Conclusion. The high incidence of neurological diseases in children born from women of the late reproductive period is associated with age-related changes in their germ cells, the implementation of assisted reproductive technologies, and the intake of hormonal drugs in early pregnancy. The data obtained should be taken into account by obstetricians in the course of pregnancy in women of late reproductive age; they should be included in the group of high risk of developing neurological disorders in children.