The article presents materials and results of the VII All-Russian Congress with international participation of the Consortium 5P Children`s Medicine. The importance of this event for the development of Russian pediatric medicine and its significance in the world of medical care for the children are considered in the framework of extrapolation of the best pediatric practices, highlighting new achievements in fundamental and applied pediatrics.

Russian-language studies of healthcare worker burnout are predominantly single cross-sectional surveys with 100–300 participants; no regional-scale data on medical institutions providing pediatric care have been published.

Aim. To estimate the prevalence and structural characteristics of occupational burnout among healthcare workers in medical institutions providing pediatric care in the Moscow Region and describe its associations with demographic and occupational characteristics.

Materials and methods. Two independent waves were conducted (September 2024, N = 2047, March 2025, N = 931; total 2978 observations from medical institutions of the Moscow Region providing pediatric care) using the MBI-HSS in the Russian adaptation by N.E. Vodopyanova and E.S. Starchenkova. Mann – Whitney U, Kruskal – Wallis H, Pearson χ² tests and binary logistic regression were used.

Results. High-degree burnout prevalence was 35.4% in September 2024 and 40.1% in March 2025 (χ²(2) = 6.62; p = 0.037). The between-wave increase persisted after adjustment for demographic and occupational covariates (OR = 1.26; 95% CI 1.07–1.48; p = 0.005). Differences between physicians and nursing staff did not reach statistical significance; non-medical personnel showed a protective profile (OR = 0.63; 95% CI 0.40–0.99; p = 0.047). An age gradient emerged only in wave 2: 50.8% of specialists aged 30–39 vs 29.0% of those aged 60+ showed high-degree burnout.

Conclusion. The study provides regional-scale burnout prevalence estimates in pediatric healthcare of the Moscow Region using a design rarely applied in Russian-language burnout research. The 35–40% baseline establishes a reference point for evaluating preventive programs.

Microbial inflammation caused by P. aeruginosa is associated with low lung function, inadequate nutritional status, severe disease course, the presence of respiratory complications and early mortality. The window of opportunity for eradication is the early detection of colonization of P. aeruginosa in the respiratory tract. The highest chance of completely eradicating P. aeruginosa exists if treatment is started in the first month after its detection.

Objective. To evaluate the strategies of inhaled antibiotic therapy in children with cystic fibrosis when P. aeruginosa is initially detected in the sputum.

Materials. The study included pediatric patients with CF who were observed in the Department of Cystic Fibrosis of the Moscow Regional Research Institute of Childhood of the Ministry of Health of the Moscow Region. The number of patients with the first isolation of P. aeruginosa among patients in the Moscow Region during the 4-year follow-up period was 37 children (19 males / 18 females). The median age of the children was 4.87 (3.33; 9.9) years. 89% of the patients had a ‘’severe genotype’’. The median age of diagnosis was Me (Q1; Q3) 0.2 (0.1; 0.2) years. All children underwent microbiological monitoring on a regular basis, once a quarter, before inclusion in the study.

Results. Patients on inhaled tobramycin or colistimethate sodium in combination with ciprofloxacin had a 100% positive result in both cases, with all children achieving eradication during follow-up for 2 years or more. A high percentage of eradication was achieved in patients receiving inhaled tobramycin therapy, with 93.3% achieving persistent eradication. Lower rates were observed in patients receiving only sodium colistimethate inhalation therapy, with 75% achieving eradication, and in patients alternating between inhaled tobramycin and sodium colistimethate, with 66.6% achieving eradication. However, a statistical analysis of the results of the effectiveness of the antibacterial therapy schemes did not show any advantage of any of the schemes (p = 0.94). Patients with primary P. aeruginosa isolation showed better external respiratory function results one year after eradication therapy compared to patients with chronic P. aeruginosa – Me (Q1; Q3) FEV1 (p1-2 = 0.004), FVC (p1-2 = 0.039). Children who underwent eradication therapy had a statistical difference in terms of nutritional status according to the BMI percentile – Me (Q1; Q3) per BMI kg/m² compared to the group with chronic P. aeruginosa (p1-2 = 0.016).

Conclusion. Early eradication therapy at the first P. aeruginosa isolation helps to prevent chronic infection, avoid lung function decline, structural damage to the lungs, and delay chronic colonization. Early start of eradication therapy depends on constant regular microbiological monitoring, which requires a modern organization of dynamic monitoring of patients with cystic fibrosis.

Merosin-deficient muscular dystrophy is a neuromuscular disorder with a heterogeneous phenotype caused by deficiency of the extracellular matrix protein laminin-211. Merosinopathy has traditionally been considered primarily a skeletal muscle disease, manifesting with muscular hypotonia, delayed motor development, progressive muscle weakness, contractures, scoliosis, respiratory and nutritional complications. However, accumulating evidence indicates that in merosin-deficient muscular dystrophy the pathological process is not limited to muscle tissue. Involvement of the central nervous system is of particular importance, as it creates pathogenetic prerequisites for impairments in mental development. In the present study, a comprehensive assessment of motor, adaptive, cognitive, communicative, and socioemotional domains was performed using the DP-3 questionnaire in patients with congenital and limbgirdle forms of merosin-deficient muscular dystrophy. The most pronounced decrease in scores was observed in the motor and adaptive domains in patients with the congenital form of the disease; at the same time, some patients demonstrated impairments in the cognitive, communicative, and socio-emotional domains, whereas in individual cases cognitive scores were within or above the average range. These findings highlight the heterogeneity of the cognitive-behavioral profile of the disease and emphasize the need for individualized comprehensive assessment of mental development in patients with merosinopathy.

Introduction. Heterotopy of the gastric mucosa in the rectum is an extremely rare congenital anomaly. Fewer than 30 such cases in children have been reported in the global literature. The clinical presentation is nonspecific and most commonly includes rectal bleeding, pain, or discomfort during defecation, which complicates timely diagnosis.

Objective. To present a clinical case of a rare localization of gastric mucosa heterotopy in the rectum in an 8‑year‑old child and to discuss the optimal diagnostic and treatment strategy.

Materials and methods. An 8‑year‑old boy was hospitalized with complaints of recurrent rectal bleeding since the age of 3 months and rectal mucosal prolapse since the age of 7 years. Physical examination, colonoscopy with biopsy, and histological analysis of the biopsy specimens were performed to confirm the diagnosis.

Results. Colonoscopy revealed a polypoid mass on a broad base in the anal canal, up to 2,0 cm in diameter. Histological examination confirmed heterotopy of gastric mucosa cells. Transanal mucosal‑submucosal excision of the lesion was performed. The postoperative period was uneventful. Histological analysis of the resected specimen confirmed the diagnosis.

Conclusion. Despite its rarity, gastric mucosa heterotopy in the rectum should be included in the differential diagnosis of rectal bleeding in children. Surgical excision is the treatment of choice: it allows for complete removal of the pathological focus.

Autosomal recessive axonal peripheral neuropathy with neuromyotonia (ARAN-NM) is a rare form of hereditary motor-sensory neuropathy belonging to the spectrum of Charcot Marie–Tooth disease (CMT). The condition is associated with biallelic loss of function mutations in the HINT1 gene. Clinically, ARAN NM is characterized by onset in the first or second decade of life, progressive distal weakness and atrophy of the lower limb muscles, gait impairment, and foot deformities. Approximately 80% of patients exhibit neuromyotonia – delayed muscle relaxation, stiffness, cramps, and fasciculations that worsen with cold exposure. The main instrumental diagnostic methods are stimulation electroneuromyography (ENMG) and needle electromyography (EMG), which reveal an axonal pattern of damage and neuromyotonic discharges. Currently, no disease modifying therapy is available. The first line drug for relieving neuromyotonia is carbamazepine. Non pharmacological rehabilitation, including therapeutic exercise, physiotherapy, and orthopaedic correction of foot deformities, plays a significant role in the comprehensive medical management of children with ARAN NM.

Introduction. Type 3 Hermansky-Pudlak syndrome (GFS) is a rare autosomal recessive disease characterized mainly by oculocutaneous albinism and platelet quality defects, other clinical manifestations characteristic of other types of GFS (interstitial pulmonary fibrosis and granulomatous colitis in types 1, 2, 4 and immunodeficiency in types 2 and 10) are absent. Usually, hemorrhagic manifestations in type 3 GBS are limited to the skin-mucosal syndrome, whereas the development of severe gastrointestinal bleeding, according to the literature, is not typical and requires a more in-depth analysis.

Goal. To present a clinical case of a child with type 3 GST and recurrent GCC, demonstrate the difficulties of differential diagnosis, and justify the need for a multidisciplinary approach.

Materials and methods. A retrospective analysis of the medical documentation of an 8-year-old patient with genetically confirmed type 3 GST, including data from endoscopic, radiation, and hemostasis methods, was performed.

Results. A patient with a previously known platelet defect developed two episodes of severe GCC with a drop in hemoglobin to 77 g/L, requiring blood transfusions. The patient was comprehensively examined in the field of gastroenterology and hematology. Capsule endoscopy revealed angiodysplasia of the ileum, but it was impossible to determine them as unambiguous causes of bleeding at the time of examination. According to the results of the examination, no unambiguous cause of bleeding was identified, and therefore three hypotheses were formulated explaining the clinical course of the disease: 1- a combination of mutations in the HPS3 gene leads to a more severe course of hemorrhagic syndrome; 2- failure to follow doctors’ recommendations, eating disorders and regimen leads to a more severe course of hemorrhagic syndrome; 3- primary defects platelets worsen the course of intestinal structural defects.

Conclusion. Despite the mild hematological manifestations of SGP3 described in the literature, caution should be exercised regarding the possible more severe course of the disease, including bleeding from the gastrointestinal tract. It is necessary to actively search for and exclude structural vascular anomalies and inflammatory changes in the gastrointestinal tract, and differential diagnosis requires interdisciplinary interaction and a re-revision of instrumental data. A more active accumulation of data will help to compare the clinical course with different genetic variants and identify the variants associated with the most severe course of the disease.

Glucose homeostasis disorders – hypoglycemia and hyperglycemia are among the most common metabolic disturbances in the neonatal period and are independently associated with adverse neurodevelopmental outcomes. The growing use of continuous glucose monitoring (CGM) is reshaping dysglycemia detection, yet its place in neonatology remains incompletely defined.

Aim. To summarise current evidence on the pathophysiology of neonatal glucose disorders, diagnostic thresholds, intermittent and continuous glucose monitoring methods, and the impact of feeding strategies and clinical management on short- and long-term outcomes.

Materials and methods. PubMed/MEDLINE, Scopus, Cochrane Library and eLIBRARY were searched for publications from 2000 to 2025 using the keywords: neonatal hypoglycemia, neonatal hyperglycemia, continuous glucose monitoring, preterm, feeding, neurodevelopment. Clinical practice guidelines (AAP, PES, ESPGHAN, draft Russian national guidelines 2024), systematic reviews, meta-analyses, randomised and cohort studies and expert consensuses were included.

Results. Glucose thresholds for hypoglycemia vary across guidance documents (2.2–2.6 mmol/L per AAP, ≥ 2.8 mmol/L per PES during the first 48 hours of life) and should be selected according to clinical context and postnatal age. CGM increases the detection of hypoglycemic events 2- to 8-fold – most of them asymptomatic and nocturnal – shortens the cumulative duration of dysglycemia, and when combined with decision-support algorithms reduces glycemic variability in extremely preterm infants. Timely breastfeeding and supervised dextrose gel are comparable to intravenous glucose infusion for managing asymptomatic hypoglycemia, while reducing mother–infant separation. Limitations remain related to CGM accuracy at low glucose concentrations and the paucity of randomised data on long-term neurocognitive outcomes.

Conclusion. Implementation of CGM in neonatal units is justified in high-risk groups, provided that devices are validated, response algorithms are standardised and CGM data are integrated into clinical decision-making. Future research should define target glycemic ranges, evaluate long-term outcomes and assess cost-effectiveness.