Background. Reliever medication is necessary for every patient with bronchial asthma (BA) during exacerbation. Active stereoisomeric preparations are safer than racemic mixtures, conserving comparable efficacy. Pressurized metered dose inhaler (pMDI) levosalbutamol is registered for usage in Russia from age 4 years old, but pediatric population studies are insufficient.

The aim of the study is to evaluate levosalbutamol pMDI 45 mcg/dose efficacy and safety in treating BA exacerbations in pediatric patients in real clinical practice.

Materials and methods. 62 patients from 6 to 17 years old (mean age 9.8 ± 1.3 years) on maintenance treatment of low and medium dose inhalation glucocorticosteroids monotherapy were included during BA exacerbation. The study observation period lasted 7 ± 2 days. Levosalbutamol pMDI 45 mcg/dose was prescribed daily before maintenance inhalations and as needed, but no more than 8 doses per day. Asthma symptoms were evaluated by point-scale, spirometry with bronchodilator test was performed on inclusion visit and week after. Patients daily recorded complaitns and adverse events.

Results. All included patients had finished the study as per protocol. When conducting a bronchodilator test with levosalbutamol MDI at a dose of 90–180 mcg 15 minutes after inhalation in children under 12 years of age, the average score for the severity of asthma exacerbation symptoms decreased from 4.6 ± 1.3 to 1.5 ± 0.9 points (p = 0.0032), and the forced expiratory volume in the first second increased by 15.7 ± 2.8%. Any adverse events (AE) during 7 days were registered in 5 (8.1%) patients; cough was most prevalent (6.5%). There were no cases of preterm discontinuation due to AE or serious AE. At visit 2 no patient was registered with asthma exacerbation. There were no cases of tachyphylaxis in terms of bronchodilation due to levosalbutamol.

Conclusion. According to our data, levosalbutamol pMDI 45 mcg/dose administration has clinically significant bronchodilation effect and favorable safety profile in 6–17 years old children.

Background. Recently, the evidence base for modern methods of assessing liver fibrosis (LF) using serological biomarkers and calculated indices has been significantly expanded. The most promising markers include collagens of types I and III (COL1, COL3), hyaluronic acid (HA), growth differentiation factor-15 (GDF-15), monocyte chemotactic protein-1 (MCP-1), extracellular matrix protein-1 (ECM1), as well as APRI indices (Aspartate aminotransferase-to-platelet ratio index) and FIB-4 (Fibrosis-4 index). However, now the number of publications with the results of using this diagnostic complex in pediatric practice remains limited. Objective.

The aim of the study is to evaluate the diagnostic value of direct serological biomarkers (COL1, COL3, HA, GDF-15, MCP-1, ECM1) in combination with the APRI and FIB-4 indices for minimally invasive stratification of LF stages in children.

Methods. The study included 108 patients aged 2 years 4 months to 17 years 11 months (median age — 9.9 [6.9; 15.9] years) with chronic liver diseases of various etiologies, 51 of them with cryptogenic LF, 19 with glycogen storage diseases, 14 with autoimmune hepatitis, 14 with primary sclerosing cholangitis, 6 with Wilson disease, 2 with progressive familial intrahepatic cholestasis, 1 with glycogenic hepatopathy in the framework of Mauriac’s syndrome, 1 with Caroli disease. To determine the stage of LF, all children underwent two-dimensional shear-wave elasticity imaging (DSWE), evaluation of serum concentrations of direct LF biomarkers in vitro: GDF-15 (pg/mL); COL1 (ng/ml); COL3 (ng/ml); ECM1 (pg/mL) by Sandwich ELISA (enzyme-linked immunosorbent assay), and HA (ng/ml) by the method of general ELISA. Additionally, the APRI and FIB-4 indices were calculated.

Results. Concentrations of HA, GDF-15, and ECM1 in blood serum increased statistically significantly as LF progressed (p < 0.001 for all indicators). Data on statistically significant differences in COL1 (p < 0.001) and COL3 (p = 0.001) concentrations were obtained when distinguishing between early changes in hepatic parenchyma (stages F0–F1). When determining the threshold values of HA depending on the stage of LF, high sensitivity (> 90%) and specificity (> 94.6%) were found in differentiating pronounced structural changes in the liver from the early stages of fibrosis. When differentiating the F4 stage from F0, 100% sensitivity and 100% specificity were achieved at the GDF-15 level, 81.8% sensitivity and 90.6% specificity at the ECM1 level. The best indicators of sensitivity (70.3%) and specificity (69.4%) for COL3 were noted when distinguishing stages F0 from F1. The calculated FIB-4 and APRI indices demonstrated a significant increase in the median values in patients at late stages of LF: F4 from F0–F3 (p < 0.05) and F4 from F0–F2 (p < 0.011), respectively.

Conclusion. The serum concentrations of HA, GDF-15, ECM1, as well as the values of the calculated APRI and FIB-4 indices demonstrate unidirectional dynamics, increasing as the degree of LF in children increases. On the contrary, the concentrations of COL1 and COL3 decrease in this case at the stage of minimal fibrosis formation and remain low as the disease progresses compared with the F0 stage. The presented instrumental and laboratory complex can be considered as a promising minimally invasive diagnostic tool for stratification of LF stages in children.

Background. Hypophosphatasia (HPP) is a hereditary disorder belonging to the group of mineral metabolism disturbances, caused by deficiency of tissue-nonspecific alkaline phosphatase due to pathogenic and likely pathogenic variants in the ALPL gene. It is characterized by a wide spectrum of clinical manifestations, ranging from severe perinatal phenotypes to milder forms presenting in childhood and adulthood. Early loss of primary teeth, and later of permanent teeth, is often the first symptom of HPP but remains underestimated. HPP is not always recognized by physicians, especially in mild cases. The importance of radiological diagnostics in identifying all forms of HPP should be emphasized. Currently, with the availability of effective pathogenetic therapy, early diagnosis is of particular importance, as it allows the disease to be identified before life-threatening conditions develop. Case series. This article presents a case series of both severe HPP with manifestation at birth and milder forms involving dental and skeletal system involvement. Enzyme replacement therapy stabilized the condition of a patient with the severe perinatal form, as well as two patients with the childhood form of HPP, in whom early loss of primary teeth was the first clinical sign of the disease. Of particular interest is the description of a familial case involving the patient’s mother, who had clinical manifestations of HPP in early childhood, but the diagnosis was only established during the evaluation of her son.

Conclusion. Effective pathogenetic therapy has been developed for HPP, which improves the health status of patients. Early diagnosis and timely initiation of treatment will help preserve health and, in severe cases, save patients’ lives.

This article presents an analysis of current clinical guidelines for the management of patients with eosinophilic esophagitis (EoE) — a chronic immune-mediated disease of the esophagus characterized by eosinophilic infiltration of the mucosa (≥ 15 eos/hpf) and clinical manifestations of esophageal dysfunction. The review highlights key changes in diagnostic approaches and discusses contemporary treatment strategies, including dietary therapy (starting with the least restrictive diets), pharmacological treatment (proton pump inhibitors, topical steroids, biologic therapy), and endoscopic dilation for strictures. The authors emphasize the need for comprehensive monitoring (clinical, endoscopic, and histological) to assess treatment response and for long-term maintenance therapy given the chronic nature of the disease. Special attention is paid to pediatric aspects of patient management.

This article summarizes current epidemiological, etiological, and clinical aspects of food allergy in children. Evidence-based approaches to diagnosis (including differential diagnosis), treatment, and prevention are presented. Practical management algorithms for patients are outlined, developed on the basis of current scientific literature and recent clinical data on food allergy.

Allergic rhinitis is one of the most common chronic diseases of childhood. Early manifestation of symptoms, late diagnosis, and irrational (or insufficiently effective) pharmacotherapy increase the risk of polysensitization and contribute to the development of other diseases of the respiratory system and ENT organs. The pathogenetic and clinical heterogeneity of allergic rhinitis, as well as difficulties in achieving compliance, are the main reasons for the high variability in treatment response. The results of recent studies focusing on phenotypic differences in patients with allergic rhinitis open up prospects for the development of new effective personalized algorithms for medical care. A review of current research on the problem of heterogeneity (genetic, microbiome-related, and multimorbid) of pediatric allergic rhinitis and its associated variable efficacy of pharmacotherapy was conducted using data from the scientific electronic libraries eLibrary and PubMed. Current research findings may serve as a basis for the development of phenotype-oriented approaches to the treatment of allergic rhinitis in children — both by improving the efficacy of traditional drugs and their combinations (antihistamines, intranasal corticosteroids, antileukotriene agents) and through potential targeting of novel pharmacotherapeutic targets.

The keen researcher’s interest nowadays is focused on the study of potential celiac disease. It is characterized by increased levels of specific autoantibodies to tissue transglutaminase and endomysium and by HLA-DQ2/DQ8 alleles in the genotype with no or minimal changes in small intestine mucous membrane. Patients with potential celiac disease may have various intestinal and extraintestinal symptoms, however the disease can be asymptomatic. Disease pathogenetic mechanisms are not fully understood, and villous atrophy (typical for celiac disease) risks remain unknown. There is no management strategy for such patients. Gluten-free diet efficacy in patients with potential celiac disease, who do not have any clinical symptoms, is also not conclusively established.

Not only genetic predisposition, but also environmental factors lead to celiac disease development in a patient. The question of whether intestinal dysbiosis is disease cause or consequence remains opened. This review presents current achievements in studying correlations between intestinal microbiota state and celiac disease development, as well as evaluates methods for microbial imbalance correction in affecting disease pathogenetic mechanisms. Based on current studies analysis, it can be concluded that any microbiota changes in patients with celiac disease should be considered not only because of the disease, but also as part of complex of interacting causal factors, including dietary and psychosocial aspects. Potential probiotics strengths are associated with restoration of intestinal microflora composition and immune response modulation. Altogether, it increases resistance to pathogens and may reduce gastrointestinal symptoms severity in patients on gluten-free diet.

Cerebral palsy (CP) is one of the leading causes of neurological disability in childhood. Owing to modern medical advances, survival and life expectancy of patients with CP are increasing, and consequently, the number of adolescents and adults with CP is also growing. However, data on the development of neurological and associated symptoms characteristic of CP as patients age remain insufficient in the domestic literature, which limits the awareness of specialists regarding the age-related progression of the disease and reduces the quality of care provided to this patient population. This review analyzes and presents current data on the dynamics of the major neurological and associated symptoms in adolescents (10–17 years) and adults (18 years and older) with CP, as well as approaches to the identification and prevention of typical complications, for a broad range of specialists working with these patients. This first part of the review addresses general factors influencing the age-related changes in the condition of patients with CP, as well as motor functions, dysphagia, and sialorrhea in aging patients with CP.

Background. Recently, a significant increase in the use of Ancillary Assisted Reproductive Technologies (ART) is noted. Meanwhile, the electrophysiological state of the myocardium, including the risk of cardiac rhythm disorders (CRD), in newborns after in vitro fertilization (IVF) remains poorly understood. No comprehensive assessments of the parameters of a standard electrocardiogram (ECG) is available, as well as approaches to their treatment in the early neonatal period in this category of patients.

Objective. The aim of the study is to research the electrocardiographic effects of myocardial depolarization disorders in newborns conceived with IVF and to evaluate the effectiveness of Cytoflavin in treatment of the detected disorders in premature infants with cerebral ischemia (CI).

Methods. The study design included 2 stages. At the first stage, a single-center, nonconcurrent, cross-sectional comparative cohort study was conducted, which included 580 newborns divided into 2 groups: the main group (children after IVF, n = 206) and the comparison group (children from spontaneous pregnancy, n = 374). The groups were stratified by fetal age (35–37 and 38-40 weeks), and the presence of CI, grade I–II. On the 3rd day of life, all children underwent a standard ECG with an analysis of rhythm parameters, conduction, and markers of depolarization disorders. At the second stage, an open comparative randomized study of the efficacy of Cytoflavin (2 ml/kg/day) was conducted in 60 premature infants with CI. The dynamics of rhythm disturbances was assessed on the 5th day after therapy.

Results. Atrial rhythm (8.3% vs. 2.5%, p = 0.003) and supraventricular extrasystole (15.5% vs. 7.4%, p = 0.004) were more frequently reported in newborns conceived by IVF. Specific changes in depolarization parameters have been identified: a decrease in the amplitude and duration of the P wave, a shortening of the PQ interval, and an elongation of the QRS complex. The most pronounced violations of intraventricular conduction were observed in premature infants with СI of the main group. Multifactorial analysis confirmed that the method of conception (IVF) is an independent risk factor for depolarization disorders (OR = 1.58; 95% CI 1.05–2.38; p = 0.028) along with the presence of CI (OR = 2.41; 95% CI 1.60–3.64; p < 0.001). The administration of Cytoflavin at the basic therapy was accompanied by a statistically significant decrease in the frequency of sinus bradycardia and supraventricular extrasystole by day 5 of treatment (p < 0.05) both in the group of children after IVF and in children from spontaneous pregnancy.

Conclusion. Newborns conceived using IVF form an increased risk group for the development of myocardial depolarization disorders on the 3rd day of life. The greatest risk is observed when the IVF factor is combined with prematurity and CI. The use of Cytoflavin in premature infants with CI demonstrates a positive effect in correcting the identified CRD, which justifies the possibility of its use in this category of patients.

Background. Pearson syndrome (PS) is an exceptionally rare mitochondrial disorder of childhood, characterized by marked clinical polymorphism, which often leads to diagnostic errors and delayed correct diagnosis. The presented clinical case is intended to draw clinicians’ attention to the early hematological manifestations of the syndrome and to highlight the complexity of differential diagnosis in neonates and infants. Case Report. From the first day of life, the child was found to have hyporegenerative normocytic anemia (hemoglobin 90 g/L on day 2 of life), which required red blood cell transfusions. Subsequently, the development of pancytopenia (leukocytes down to 2 × 10⁹/L, platelets down to 93 × 10⁹/L) and episodes of severe hyperlactatemia — up to 11.2 mmol/L — were noted. Morphological examination of the bone marrow revealed vacuolization of cellular elements and moderate hypocellularity without narrowing of the erythroid lineage, which led to suspicion of PS. Molecular genetic testing confirmed the presence of a large deletion of mitochondrial DNA (~5000 bp) in a homoplasmic state, thus verifying PS. Symptomatic therapy was administered, including blood component transfusions, high-dose intravenous immunoglobulin, and antibacterial treatment, which resulted in temporary stabilization of blood counts.

Conclusion. The presented case report underscores the importance of including mitochondrial disorders early in the diagnostic workup of hyporegenerative normochromic normocytic anemia with elevated lactate levels in infants, demonstrates the need for a multidisciplinary approach, and highlights the necessity of timely genetic testing.

Background. Congenital disorder of glycosylation type Ia (Jaeken syndrome; congenital disorder of glycosylation, type Ia; CDG-Ia) is a hereditary progressive disorder with a pronounced neurodegenerative component, caused by pathogenic variants in the PMM2 gene (encoding the enzyme phosphomannomutase-2). CDG-Ia accounts for the majority of registered patients with congenital disorders of glycosylation (62% in 2018). At least 1,000 patients with CDG caused by PMM2 gene mutations are known, but due to diagnostic difficulties, the true number of individuals with this condition is undoubtedly much higher.

Case Report. We present a case report of CDG-Ia in a child with progressive ataxia, nystagmus, and delayed psycho-speech and motor development. The patient was a female infant, born from the third pregnancy, which was complicated by acute enteritis at 15 weeks of gestation and an acute respiratory viral infection at 20 weeks of gestation. Delivery was at term (42 weeks) and was the second childbirth. The child was born into a family with no history of hereditary disorders; the parents were 37 and 38 years old at the time of the girl’s birth. The disease manifested from birth with reduced sucking reflex, breast refusal, and slow weight gain. At 1.5 months of age, horizontal nystagmus was added to these symptoms. The diagnosis was established based on molecular genetic testing of the proband using next-generation sequencing to rule out hereditary ataxias, as well as transferrin isoelectric focusing (revealing an abnormal transferrin spectrum: abnormal diand asialotransferrins — isoforms S2 and S0), and magnetic resonance imaging of the brain (showing progressive cerebellar atrophy at 11 months of age).

Conclusion. We describe a case report of CDG-Ia in a child from a family in which both parents are carriers of mutant alleles and have a healthy child. Despite comprehensive patient management, progressive disease course was observed; the girl has severe psychomotor retardation and clinical signs of multisystem involvement of internal organs. Arresting disease progression is not feasible due to the underlying genetic defect in the synthesis of mannose and glycoproteins, which are essential components of most metabolic pathways in the body.

Background. Klippel – Trenone syndrome (KTS) is a rare genetic disease that occurs with a frequency of 1 per 100,000 people and is characterized by a typical triad of symptoms: malformation of the capillary network (like a “wine stain”), hypertrophy of soft tissues and bones, and venous malformations of the extremities. Adenocarcinoma is a malignant formation of the glandular epithelium. This disease is extremely rare in children (about 1 case per 1 million people under the age of 20 years annually) due to its large regenerative potential and the absence of comorbid conditions that accumulate with age. In the article, we presented a case demonstrating a combination of these rare and severe diseases in a child.

Case Report. We present a case report of the manifestation of adenocarcinoma of the transverse colon with the development of an acute intestinal obstruction clinic in a child with KTS.

Conclusion. This case report demonstrates the combination of KTS and moderately malignant transverse colon adenocarcinoma in a child. This case is unique and valuable for clinical practice, emphasizing the need for thorough examination of patients with KTS due to the risk of cancer due to mutations in the PIK3CA oncogene. The combination of KTS with aggressive carcinoma leads to the development of complex comorbid pathology. The mutual aggravation of these diseases critically limits the possibilities of therapeutic correction and, as a result, dramatically reduces the expected survival rate of such patients.

On March 14, 2026, the Council of Experts was held to address the issue of functional gastrointestinal disorders (FGIDs) in children, including those following acute intestinal infections (AIIs). It was noted that AIIs remain a significant medical and social problem in pediatrics, carrying risks of dehydration, toxic shock, organ damage, and the development of long-term post-infectious complications that impair quality of life. The experts confirmed the key role of baseline therapy for AIIs (oral/parenteral rehydration and diet therapy) and discussed the addition of the probiotic strain Lactobacillus rhamnosus GG (LGG) to standard protocols as an evidence-based approach to reducing the duration and severity of diarrhea and restoring gut microbiota, that may decrease the risk of subsequent FGIDs. In cases of already established FGIDs in children with a predominance of pain and spastic syndromes in the absence of organic pathology, the Council recommended thorough diagnostic evaluation and the use of trimebutine, a universal gastrointestinal motility regulator, including its novel pediatric gel formulation (Dinobutin), which provides analgesic, antispasmodic, and motility-normalizing effects and is potentially associated with higher adherence in children. It was noted that the combined use of LGG and trimebutine (for example, Normobact L and Dinobutin) may yield a more sustained clinical effect in children with FGIDs. The meeting concluded with a proposed management algorithm for patients with AIIs and post-infectious FGIDs, which could significantly improve patient outcomes and quality of life.