Background. The epidemiology of pulmonary hypertension (PH) in children in the Russian Federation has not been studied. Currently available data is limited to several foreign registries, mostly limited to the study of PH Group I cases. Objective.

The aim of the study is to analyze the prevalence of PH in the pediatric population of the Russian Federation.

Methods. Data on PH cases were collected from chief freelance pediatric cardiologists and pediatric cardiology specialists in 85 territorial entities of the country. Requests for data submission were sent January 2021, 2022, and 2023. The study included data from patients aged from 3 months to 17 years with established diagnosis of PH and available catheterization and/or echocardiography results. The PH diagnosis was verified based on mean pulmonary artery pressure (mPAP) values (> 20 mmHg for biventricular circulation and > 15 mmHg in univentricular hemodynamics) or, in their absence, systolic pulmonary artery pressure values (> 36 mmHg). PH clinical groups were created according to the diagnosis in medical records. Publicly available Russian Federal State Statistics Service data on pediatric population (total and by territorial entities) as of January 1, 2022 and 2023 were used to evaluate PH prevalence.

Results. The study included data from549 patients with PH from 60 territorial entities of Russia from February 2021 to February 2023. Mean age was 10.0 (4.8) years, with 56% being female. The diagnosis was verified via cardiac catheterization in 71.5% of patients. The overall prevalence of PH was 22.5 cases (95% CI 18.3–27.4) per 1 million pediatric population; PH Group I (pulmonary arterial hypertension; PAH) accounted for 11.8 cases, including idiopathic PAH — 4.18 and PAH associated with congenital heart disease (CHD) — 7.16 per 1 million pediatric population. CHD was associated with 66.4% of all PH cases (Groups I and V). The highest prevalence (7.8 per 1 million pediatric population) was observed for PH cases associated with complex CHD.

Conclusion. PH prevalence, its clinical groups and subgroups among pediatric population of Russian Federation is presented. The obtained data is crucial for evaluation of population's need for specialized medical care for this complex group of patients at both regional and federal levels.

Background. The study was conducted based on data from the Russian registry of patients with mucopolysaccharidosis (MPS) type II. There are no similar studies based on analysis of data from the national MPS registry in Russian Federation.

Objective. The aim of the study is to conduct a comprehensive analysis of MPS type II epidemiology and phenotypic features in Russian patients using data from the national registry, as well as to compare the obtained results with corresponding data from international registries.

Methods. The retrospective, cross-sectional observational study included data from 177 patients with confirmed diagnosis of MPS type II. Data on medical history, clinical, laboratory, and instrumental examination methods, as well as data on enzyme replacement therapy (ERT) over the period from 2008 to March 2026 was analyzed.

Results. The prevalence of MPS type II in Russian Federation was 0.1 per 100,000 population, and 0.54 per 100,000 live births (approximately 1 in 185,000 live births). The geographical distribution of patients was uneven. The median age of first symptoms was 1.0 (0; 2.0) years, the range — from the first months of life to 23 years, and the median age of established diagnosis was 3.0 (2.0; 6.0) years, the range — from 0.1 to 38.9 years. The major symptoms of MPS type II in children at the time of diagnosis included musculoskeletal disorders (99.1%), Hurler phenotype 96.4%, short neck was detected in 88.8%, hepatomegaly (based on physical examination findings) in 91.9% of patients, according to abdominal ultrasound data in 87.7% patients, umbilical and inguinal hernias in 82.1% of patients, cardiovascular damage — in 80.3%, chest deformity — 80.3%, contractures of the hand joints (74.5%), and ENT manifestations (62.4%). 151 (85.3%) patients were alive at the time of data collection. ERT was administered to 140 out of 160 (87.5%) patients with MPS type II. Frequent variants in the IDS gene typical for the Russian population were revealed: recombination IDS/ps IDS2 ex3/int7, variants p.Gly374=, p.Arg468Gln, p.Ala85Thr, p.Pro86Leu and p.Arg443Ter.

Conclusion. The prevalence of MPS type II in Russian Federation is comparable to data from international registries. The registry-based approach enables the dynamic analysis of the disease’s clinical signs, treatment efficacy evaluation, and planning of healthcare resources. Further expansion of clinical part of the registry, regular data updates, and integration with international registry platforms are crucial for in-depth research and for obtaining more comprehensive data on patients with orphan diseases.

Background. Modern clinical guidelines recommend surfactant administration when the oxygen requirement (FiO2) exceeds 0.3 during non-invasive respiratory support via continuous positive airway pressure (CPAP). However, this criterion is nonspecific and does not reflect the true extent of lung parenchymal involvement. Lung ultrasound enables a quantitative assessment of aeration decrease at the bedside without radiation exposure and prior to the onset of oxygen dependency.

The aim of the study was to evaluate the predictive characteristics of ultrasound assessment of lung parenchymal aeration in determining the need for surfactant administration in preterm infants with respiratory distress syndrome (RDS) on non-invasive respiratory support.

Methods. This prospective study included preterm infants with a gestational age (GA) of 26–34 weeks with RDS who received nasal CPAP (NCPAP) from birth with an FiO₂ ≤ 0.3. Lung parenchymal aeration was evaluated via ultrasound within the first 2 hours of life. Each scaned zone (anterior, lateral, and posterior on both sides) was assigned points from 0 to 3, where 0 — A-pattern (horizontal artifacts, normal aeration); 1 — B-pattern (fewer than 3 clearly defined B-lines); 2 — severe alveolar-interstitial syndrome (multiple confluent B-lines; possible subpleural consolidations); 3 — extensive lung parenchymal consolidation. Zone scores were summed up. The possible range for the total score was 0 to 18. The predicted outcome was surfactant administration within the first 3 hours of life.

Results. The study included 175 preterm infants; surfactant was administered to 95 (54%) of them. The median total lung ultrasound score was 10 (8; 12) in the group receiving surfactant and 4 (2; 6) in the group not receiving surfactant (p < 0.001). Differences in total scores between the groups were observed across all lung scanning zones (p < 0.001). The area under the ROC curve (AUC) for the total score was 0.911 (95% CI 0.865–0.951). At a threshold of ≥ 8 points, sensitivity was 82.1%, specificity was 96.2%, positive predictive value was 96.3%, and negative predictive value was 81.9%.

Conclusion. Ultrasound assessment of lung parenchymal aeration during the first 2 hours of life is an accurate, independent predictor of the need for initial surfactant administration in preterm infants with RDS on CPAP.

Background. Infant mortality is a major indicator of public health and healthcare system efficacy. Maternity and childcare services in Russian Federation underwent modernization in 2013–2015; however, still there are significant interregional differences. Objective.

The aim of the study is to examine the differences, stability, and dynamics of infant mortality in territorial entities of Russian Federation in 2013–2025.

Methods. Data from Federal State Statistics Service for 83 territorial entities was analyzed. The methods included percentile analysis, calculation of standardized ratios, evaluation of regional mobility (Mi indices), cluster analysis (k-means, k = 4), and Pearson correlation analysis.

Results. The national infant mortality rate declined from 8.2 to 3.6 per 1,000 live births (mean annual rate of decline is 6.8%). The most significant declines were observed in the Far Eastern Federal District (–8.9%) and the North Caucasus Federal District (–8.7%). The long-term mean rate remained highest in the North Caucasus Federal District (7.5 ± 2.4‰) and lowest in the Northwestern Federal District (4.6 ± 0.8‰). Variation coefficient decreased slightly from 0.32 to 0.28. The overall Mi index was 0.69 (8 group changes over 12 transitions). Only the Chukotka Autonomous Okrug (1.2%) turned out to be stable — the only steadily unfavorable region. Relatively consistently unfavorable were 3 regions: Jewish Autonomous Oblast, Republic of Tyva, Republic of Dagestan. Consistently favorable were 2 regions: Chuvash Republic, Saint Petersburg, and relatively favorable — 6 regions. Cluster analysis identified 4 dynamics types: cluster 1 (low baseline level, sustained decline) — 23 regions (27.1%); cluster 2 (high baseline level, rapid decline) — 16 regions (19.3%); cluster 3 (moderate baseline level, moderate decline) — 33 regions (39.8%); cluster 4 (high baseline level, high mobility) — 10 regions (12.0%). Statistically significant positive correlation was revealed between long-term mean infant mortality rate and its interannual variability (r = 0.72; p < 0.001; r² = 0.52).

Conclusion. The period from 2013 to 2025 is characterized by steady decrease in infant mortality in Russia while maintaining interregional differences and high mobility over the regions. The highlighted dynamics types can serve as the basis for differentiated approach to perinatal care management.

Background. Human papillomavirus (HPV) infection is a significant risk factor for the development of HPV-associated precancerous and malignant diseases. Recombinant adsorbed vaccine against HPV (types 6, 11, 16, and 18) has been developed in Russian Federation to prevent these conditions. Vaccine immunogenicity and safety have been studied in adults.

Objective. The aim of the study is to evaluate the safety and immunogenicity of Russian recombinant adsorbed quadrivalent vaccine against HPV (types 6, 11, 16, and 18) in children aged 9–17 years in comparison to reference quadrivalent vaccine. Study design. Multicenter, randomized, double-blind, parallel-group, phase III clinical trial with a 1 : 1 participant allocation ratio.

Methods. The study included healthy seronegative children aged 9–17 years. Study participants aged 9–13 years received either the investigational vaccine or the reference vaccine. Vaccine safety was assessed from April 2, 2024 to April 14, 2025, in all participants who received at least one vaccine dose (as-treated population). Any post-vaccination reactions and adverse events (AE) were recorded, their correlations with vaccination, severity, and duration were evaluated. The primary efficacy endpoint was the geometric mean titer ratio (GMT Ratio) of antibodies to HPV 1 month after full vaccination course (Per-Protocol (PP) population); additionally, the GMT Ratio was evaluated for every HPV type. Randomization was centralized and based on computer-generated list. Allocation concealment was ensured via the method of sequentially numbered, opaque, sealed envelopes.

Results. A total of 705 AEs were registered during the study period, 426 of which were considered related to vaccination. No severe vaccination-related AEs were reported. The investigational vaccine demonstrated comparable immunogenicity to the reference drug across all four HPV types in both age cohorts. The seroconversion rate in PP population ranged from 97% to 100%. Higher GMT values were observed in participants aged 9–13 years compared to those aged 14–17 years. GMT values were higher in the investigational drug group in both age cohorts for HPV type 18.

Conclusion. Favorable safety profile and adequate immunogenicity of the investigational vaccine were recorded compared to the reference vaccine in children aged 9–17 years. The obtained data suggest that the investigational vaccine is a promising drug for HPV-associated diseases prevention.

Background. Generalized forms of meningococcal disease caused by Neisseria meningitidis are characterized by rapid course and high mortality. The pathogen serogroup distribution varies between regions and changes over time, which determines the need for vaccines covering currently relevant serogroups A, C, W and Y. Licensed quadrivalent conjugate vaccines differ both in polysaccharide content and in carrier protein, which properties are considered among the factors shaping the T-dependent immune response.

Objective. The aim of the study is to evaluate immunogenicity, reactogenicity, and safety of meningococcal conjugate vaccine MenACWY-CRM197 compared with previously licensed MenACWY-DT vaccine in healthy people aged 3–55 years.

Methods. Multicenter, randomized, comparative, single-blind, parallel-group, phase III trial was conducted in 16 clinical centers among Russian Federation. All study participants were allocated 1:1 across three sequential age groups (I — 18–55 years, II — 11–17 years, III — 3–10 years). The transition between groups was performed after positive conclusion on vaccine safety according to interim safety data by independent data monitoring committee. Both vaccines were administered intramuscularly once at dosage of 0.5 ml. The primary endpoint was the proportion of participants achieving seroconversion based on serum bactericidal antibody (SBA) titers against meningococcal serogroups A, C, Y, and W at Visit 3 (Day 29 ± 1); non-inferiority was considered demonstrated if the lower bound of the 95% confidence interval (CI) for the difference in proportions exceeded –10%. Secondary endpoints (seroprotection at SBA titer > 1:4, geometric mean titers (GMT), reverse cumulative titer distribution curves) and safety outcomes were analyzed without adjustment for comparisons multiplicity; corresponding p-values are descriptive.

Results. A total of 240 participants were randomized (120 per group); the per-protocol population comprised 239 participants. Seroconversion by Visit 3 was achieved in at least 98.33% of participants for each of the four meningococcal serogroups in both groups; and it was achieved in all participants for serogroups C and W. The lower bounds of the 95% CI for the proportions difference for meningococcal serogroup A (0.83%; 95% CI –3.82…5.72) and serogroup Y (–0.007%; 95% CI –4.51…4.47) exceeded the non-inferiority margin confirming the study hypothesis for the primary endpoint. SBA titers increased significantly from Visit 1 to Visit 3 for all serogroups in both groups (p < 0.001). No differences in GMT between groups were revealed for meningococcal serogroups A, W, and Y; while the post-vaccination GMT in the investigational vaccine group was higher (306.7 vs 222.9; p = 0.017) for serogroup C. The studied vaccine demonstrated favorable safety profile. At least one adverse event (AE) was reported in 53.33% of participants in the investigational vaccine group and in 56.67% of the reference group (p = 0.604). No differences between groups were revealed in the distribution of AE by severity, causal relationship, or outcomes in the pooled population. The distribution of AE by severity differed (ratio of mild AE 86.46% vs 73.68% in the comparison group; p = 0.033) in the analysis by age stratum, among adolescents aged 11–17 years in the MenACWY-CRM197 vaccine group; in children aged 3–10 years, systemic reactions were reported in 20.0% and 40.0% of participants, respectively (p = 0.051). No serious AE related to vaccination and no fatal outcomes were reported.

Conclusion. In terms of seroconversion frequency against all four meningococcal serogroups, the MenACWY-CRM197 vaccine was non-inferior to the reference vaccine. Safety and reactogenicity profiles in the pooled population were comparable. The superior result of the investigational vaccine for secondary endpoints (GMT against meningococcal serogroup C) and the results of reactogenicity analysis by age stratum were obtained without controlling for type I error probability and require further study in specifically designed trials. The obtained data confirms the immunogenicity and acceptable tolerability of the MenACWY-CRM197 vaccine in individuals aged from 3 to 55 years. The study results allow us to consider using this vaccine for meningococcal infection prevention in children, adolescents, and adults as part of mass vaccination.

Trial registration: protocol GNG-DE-III-24, version 1.0, August 15, 2024; clinical trial authorization by the Ministry of Health of Russian Federation No.532, November 07, 2024.

Background. Non-celiac gluten sensitivity (NCGS) is a syndrome of intestinal and extraintestinal symptoms associated with wheat consumption in individuals without celiac disease or wheat allergy. This form of gluten intolerance is not well known and is rarely diagnosed in patients due to nonspecific symptoms, lack of biological marker, and diagnostic algorithm complexity. Altogether, it leads to inefficiency of standard therapy for digestive system, nervous system, and other organs diseases.

Case description. The girl had been experiencing recurrent toxic-allergic keratoconjunctivitis with progressive vision loss, as well as weakness, headaches, brain fog, irritability, anxiety, depression, mood swings, sleep disturbances, arthralgia, and myalgia from the age of 4.5. Less pronounced were flatulence, constipation, impaired hands and feet sensitivity, skin rash, lack of coordination, decreased physical development. Gluten intolerance was suspected at the age of 7.5, whereas celiac disease and gluten allergy were excluded. Gluten-free diet (GFD) test led to fast symptom relief up to 80–100%, parents categorically refused to perform gluten challenge. Psycho-neurological and gastrointestinal symptoms did not relapse and there were no signs of keratoconjunctivitis on further strict GFD for 3.5 years.

Conclusion. NCGS in children manifests with variety of intestinal and extraintestinal symptoms. Recurrent keratoconjunctivitis can be possible manifestation of NCGS. NCGS diagnosis requires celiac disease and gluten allergy exclusion, as well as conducting diagnostic tests with GFD and gluten challenge. Significant relief in symptoms on GFD and worsening upon challenge confirm the presence of NCGS. Performing gluten challenge may not be recommended in cases of recurrent keratoconjunctivitis associated with NCGS. These children require continuation of GFD for longer period of time.

Background. The annular pancreas (AP) is presented by the ring of pancreatic tissue surrounding the descending duodenum. The sensitivity of instrumental methods for AP diagnosis is limited. The applicability of endoscopic ultrasound (US) for AP diagnosis requires additional research. Case study. A 13-year-old boy. The child had repeated vomiting, abdominal pain, frequent bronchopulmonary diseases (including bilateral pneumonia), and body weight loss since the age of six (according to parents). He was repeatedly hospitalized. Upper mesenteric artery syndrome was suspected based on the computed tomography data. Several other imaging studies have been performed. The diagnosis of AP was suspected for the first time after endoscopic US. Finally, it was confirmed during the surgery — duodenal bypass surgery was performed. The child has shown significant improvement in general condition as well as increase in body weight (according to his mother) 1 year after the procedure. The child was followed-up for 3 years, there were no complaints mentioned previously.

Conclusion. Endoscopic US is a relatively new diagnostic tool in the pediatric endoscopists, arsenal. This method’s efficacy in detecting the changes in child's pancreas shape and ductal system was demonstrated.

Background. Relapsing polychondritis (RP) is a systemic immunoinflammatory disease characterized by recurrent episodes of inflammation of cartilage tissue and other proteoglycan-rich structures. There are various phenotypes of this disease: from isolated damage of the nose or auricles cartilages to systemic inflammation with laboratory changes imitating various rheumatic pathologies. RP is rare among children as pediatric population is characterized by more frequent involvement of respiratory tract with obstruction and life-threatening stenoses. Its diagnosis is significantly difficult then there are no signs of nasal or auricles chondritis, arthritis, or costochondritis.

Case study. This article describes a clinical case of RP in 14-year-old female patient. Disease onset occurred with subglottic laryngeal stenosis and progressive respiratory distress, required tracheostomy. The persistent dyspnea, decrease in body weight, and increase in laboratory activity were undulated. Later, auricles and rib-sternal joints chondritis was revealed. As a result, diagnosis of primary relapsing polychondritis was established. The combined treatment with glucocorticoids and genetically engineered biologic drug, golimumab, led to positive effect: decrease in laboratory activity, significant improvement in breathing, and increase in exercise tolerance. In the absence of any dynamics in subglottic laryngeal stenosis severity according to computed tomography and fibrolaryngoscopy, the patient requires regular monitoring and alertness regarding the possible need for subsequent surgical intervention.

Conclusion. Recurrent episodes of airway stenosis combined with systemic inflammation should raise concerns about relapsing polychondritis. Pain and swelling of auricles, nasal septum perforation, costochondritis, and arthritis are not presented in all patients. However, they can occur after the development of life-threatening breathing difficulties, can less bother the patient, and can remain undiagnosed during clinical examination. Clinical and instrumental diagnosis requires participation of multidisciplinary team of specialists.

The first 1000 days of life represent a critical window for metabolic programming. Adverse exposures during this period, acting through epigenetic mechanisms, can predispose individuals to obesity, type 2 diabetes, and cardiovascular disease across the lifespan. Identifying modifiable risk factors and implementing early interventions offers substantial potential for primary disease prevention. This review aims to consolidate evidence on modifiable risk factors operating during the periconceptional, antenatal, and early postnatal periods, and their impact on epigenetic programming and childhood obesity risk, underscoring the need for preventive strategies starting as early as the preconceptional period and continuing through the first two years of life.

Human papillomavirus (HPV) infection is highly contagious and is the most common sexually transmitted infection. It causes irreversible damage to human reproductive health, leading to benign and malignant neoplasms in anogenital and other areas. Cervical cancer continues to have the leading position in mortality among young women of reproductive age. The key tool in combating HPV infection is primary prevention, vaccination; furthermore, the implementation of the Global Strategy for HPV vaccination will prevent up to 60 million cases of cervical cancer and 45 million deaths over the next 100 years. The presented predictive and pharmacoeconomic model makes it possible to estimate the scale of lost demographic and financial benefits due to delayed inclusion of HPV vaccination in the National Immunisation Schedule, and mathematically proves the importance of immediate launch of overall vaccination to minimize the state’s long-term economic damage.