Dmitry Yurievich Ovsyannikov

Introduction: Vascular compression of the tracheobronchial tree (TBT) and esophagus occurs in congenital anomalies of the aortic arch, its branches, and the pulmonary arteries.Although the vast majority of patients with this condition are symptomatic, vascular compression is often not diagnosed promptly. The clinical presentation results from compression of the trachea and/or esophagus; therefore, the predominant manifestations are dyspnea (stridor, cough, and shortness of breath) and/or dysphagia (choking, difficulty swallowing, and regurgitation). Diagnostic approaches and methods of surgical treatment remain clinically relevant. The timing of and indications for surgical correction remain controversial, and outcome data are limited to a small number of publications. Objective: To evaluate the effect of surgical treatment of vascular rings on symptom resolution during the early postoperative period in infants and young children; to define indications for surgical treatment; to report the frequency of aortopexy used to preventresidual symptoms according to vascular ring anatomy; and to assess the incidence of chylothorax and its association with the anatomical type of vascular ring. Methods: This retrospective, nonrandomized cohort study included 58 pediatric patients who underwent surgery for an isolated congenital vascular ring causing compression ofthe TBT and esophagus at a single center between January 2018 and July 2024. Patients were divided into four groups according to vascular ring anatomy: double aortic arch (n = 16; 27.6%), right aortic arch with a left ligamentum arteriosum (n = 34; 58.6%), left aortic arch with a right ligamentum arteriosum (n = 4; 6.9%), and pulmonary artery sling (n = 4; 6.9%). All patients underwent contrast-enhanced multidetector computed tomography for diagnostic confirmation and surgical planning. Early symptom resolution,technical aspects of surgical correction, postoperative length of stay, and postoperative complications were assessed. Results: The median age at surgery was 7 months (Q1–Q3, 4–25 months). Preoperative symptoms (dyspnea and/or dysphagia) were present in 84.5% of patients. There were no statistically significant between-group differences in anthropometric characteristics, age, or symptom status (p > 0.05). Complete symptom resolution by hospital discharge was achieved in 83.7% of symptomatic patients. Length of hospital stay differed significantly among the groups (p = 0.028), with the highest median in the pulmonary artery sling group (18 hospital days), owing to the need for cardiopulmonary bypass and a more severe baseline condition. Chylothorax was the most common complication, occurring in 13 patients (22.4%) and most frequently in those with a double aortic arch (43.8%). Aortopexy was performed most frequently in patients with a right aortic arch and left ligamentum arteriosum(70.6%; p = 0.001). There were no deaths. Conclusion: Surgical correction of vascular rings in infants and young children was highly effective in the early postoperative period, with complete symptom resolution in 83.7% of symptomatic patients and no mortality. These findings support the safety of surgery when combined with early diagnosis and a multidisciplinary approach. In our opinion, for a vascular ring formed by a right aortic arch and left ligamentum arteriosum, adding aortopexy to division of the ligamentum arteriosum may visibly eliminate residual esophageal compression.The long-term clinical benefit of this approach requires further evaluation. The principal clinical concern is the substantial incidence of postoperative chylothorax (22.4%), underscoring the need to improve surgical techniques and management protocols and to identify the underlying causes. Received 17 February 2026. Revised 20 May 2026. Accepted 23 June 2026. Informed consentThe patient’s official representative has provided informed consent for the use of the recordings for medical purposes. FundingThe study did not have sponsorship. Conflict of interestThe authors declare no conflict of interest.Ethics approvalThis retrospective study was approved by the local ethics committee of Morozov Children’s City Clinical Hospital (Protocol No. 7 dated December 10, 2025). The study was conducted in accordance with the ethical principles of the World Medical AssociationDeclaration of Helsinki, as amended in October 2024. Consent was obtained from the patients’ legal representatives by telephone. Patient data wereanonymized before analysis. Contribution of the authorsData collection and analysis: A.V. Bedin, Yu.N. ShamrinStatistical analysis: A.V. BedinDrafting the article: A.V. Bedin, Yu.N. Shamrin, A.Yu. Gotskina, E.S. KorochkinaCritical revision of the article: M.M. Kurako, M.A. Abramyan, K.A. KhasanovaFinal approval of the version to be published: all authors

Background. Idiopathic pulmonary hemosiderosis (IPH) is a rare interstitial lung disease associated with recurrent diffuse alveolar hemorrhage, anemia, and the risk of pulmonary fibrosis. Aim. To describe the clinical and laboratory, computed tomographic, diagnostic, therapeutic characteristics and outcomes of children with IPH. Materials and methods. This ambispective descriptive multicenter study included 11 patients with disease onset before 18 years of age and pulmonary hemorrhage/hemosiderosis documented by bronchoscopy with bronchoalveolar lavage, cytological and/or morphological examination, after exclusion of alternative causes. Clinical history, laboratory and instrumental findings, treatment, and outcomes were evaluated. Results. The cohort included 5 boys and 6 girls. The median age at IPH onset was 6.0 [3.7; 8.6] years, the median age at diagnosis was 8.0 [4.9; 9.7] years, the median diagnostic delay was 4.9 [1.8; 22.8] months, and the median minimum hemoglobin level was 52 [38.5; 98.5] g/L (data available for 10 patients). Anemia was documented in all patients, and hemoptysis in 6/11 (55%). Signs of diffuse lung involvement on chest CT were present in all patients; ground-glass opacities and consolidation were the most common findings. Six patients required ICU care, 3 required mechanical ventilation, and 8 received red blood cell transfusions. All patients received systemic glucocorticoids; azathioprine was used in 5, rituximab in 4, and cyclophosphamide in 1 patient. Three patients died. Conclusion. IPH debuts anemia in children. The absence of hemoptysis does not exclude diffuse alveolar hemorrhage. Early computed tomography of the chest organs, bronchoscopy with bronchoalveolar lavage, the search for siderophages and the exclusion of secondary causes are the key stages of diagnosis. In severe and recurrent cases, multidisciplinary management and steroid-sparing immunosuppressive therapy are required.

Atelectasis, characterized by partial or complete collapse of the lung or its lobe/segment, is a common and serious clinical problem in children. No proven treatments for atelectasis exist. Dornase alfa (recombinant human deoxyribonuclease) reduces the viscosity of purulent secretions in the bronchi and is effective in patients with cystic fibrosis. Data on the use of dornase alfa in other diseases are limited. This article presents clinical observations of two girls – a 2-month-old with lung hypoplasia due to diaphragmatic hernia and a 7-year-old with status asthmaticus and acute respiratory distress syndrome, hospitalized in intensive care units and requiring extracorporeal membrane oxygenation therapy, who showed positive dynamics in the form of atelectasis resolution during dornase alfa therapy. The presented clinical observations, as well as the analysis of cases described in the literature on the use of dornase alfa in children with atelectasis, demonstrate the efficacy and safety of the drug in children of different ages, including those on extracorporeal membrane oxygenation.

N.N. Volodin, D.Yu. Ovsyannikov. Physician, Scientist, Educator, Healthcare Organizer – Natalia Sergeevna Kislyak. Pediatria n.a. G.N. Speransky. 2026; 105 (4): 44–46. DOI: 10.24110/0031-403X-2026-105-4-44-46.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular neoplasm of intermediate malignancy characterized by a highly variable clinical course and significant diagnostic challenges. In pediatric practice, the incidence of this condition is extremely low, which often leads to diagnostic errors, particularly in cases with atypical molecular variants. Objective: To demonstrate the clinical, instrumental, and morphological features of pulmonary epithelioid hemangioendothelioma in children and to highlight the challenges of differential diagnosis. Materials and Methods: We present a clinical case of a 14-year-old adolescent with bilateral diffuse lung involvement who underwent a comprehensive diagnostic evaluation, including chest computed tomography, bronchoscopy with biopsy, histological and immunohistochemical analysis, as well as lung function testing. Results: Multiple bilateral pulmonary nodules were identified, while no significant functional impairment was observed. The initial histological findings were consistent with organizing pneumonia. The differential diagnosis included tuberculosis, sarcoidosis, pulmonary alveolar proteinosis, and IgG4-related lung disease. The final diagnosis was established after expert histopathological review, revealing features of PEH with positive expression of endothelial markers (CD31, CD34, ERG) and absence of CAMTA1 and TFE3 expression. This pattern does not correspond to the typical molecular variants observed in the majority of cases and underscores the molecular heterogeneity of the tumor. Conclusion: This case demonstrates that PEH may mimic inflammatory lung diseases and be associated with negative results for key molecular markers (CAMTA1, TFE3). Accurate diagnosis requires a comprehensive approach, including detailed histological and immunohistochemical evaluation. Awareness of this entity in the differential diagnosis of diffuse lung diseases in children may reduce the risk of diagnostic errors and highlights the importance of a multidisciplinary approach involving pediatricians, pulmonologists, oncologists, and pathologists.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease associated with recurrent diffuse alveolar hemorrhage and progressive accumulation of hemosiderin in the lung parenchyma. The classical clinical presentation is characterized by a triad: hemoptysis, iron-deficiency anemia, and diffuse pulmonary infiltrates. The incidence of IPH is estimated at 0.24—1.23 per 1 million children per year, and the disease course may be severe and accompanied by considerable mortality, underscoring the importance of timely diagnosis. This article systematizes current data on epidemiology and possible mechanisms of disease development, summarizes diagnostic approaches to the identification of diffuse alveolar hemorrhage, and reviews key methods for diagnostic confirmation, including bronchoscopy with bronchoalveolar lavage and morphological examination of lung tissue. Particular attention is given to differential diagnosis and contemporary therapeutic strategies based on systemic glucocorticosteroids and immunosuppressive therapy, which may reduce the frequency of relapses and improve disease outcomes.

Undiagnosed comorbid conditions are a common cause of difficulty in achieving control of bronchial asthma (BA). One of the comorbid conditions in asthma is bronchiectasis (BE). Objective . To characterize clinical, laboratory, and radiological features of lung damage in children hospitalized for asthma and BE. Materials and methods. Patients with asthma and BA were observed at the Morozovskaya Children’s City Clinical Hospital from January 2023 to July 2025. The criteria for inclusion in the study were pediatric age, confirmed asthma, and the presence of BE unrelated to cystic fibrosis. The following diagnostic tools were used: analysis of clinical history, allergology and immunology tests, radiography, thoracic computed tomography, fiberoptic bronchoscopy with microbiological examination of bronchoalveolar lavage fluid, pulmonary function test, as well as the study of ciliary motility, and genetic tests. Results . In the period from January 1, 2023 to July 1, 2025 19 children with asthma and BE admitted to the Department of Pulmonology at the Morozovskaya Children’s City Clinical Hospital accounted for 1,4% of all children with asthma hospitalized during this period (19/1392). There were 10 (53%) male patients with asthma and BE; at the moment of admission, the median age of patients was 9.0 [7.0; 11.0] years; the median age at the onset of asthma was 1,5 [0.54; 2.0] years, asthma duration was 8,0 [5.5; 10.0] years, while the median length diagnostic delay was 5,5 [2.75; 8.5] years. Upon admission, 17 (89%) children reported a decrease in exercise tolerance, 15 (79%) — shortness of breath, 10 (53%) — dry cough; 6 (32%) patients had dry wheezing, 3 (16%) — wheezing. The levels of eosinophils and monocytes were respectively 0,22 [0.07; 0.34] and 0,42 [0.33; 0.46] cl/ml; median forced expiratory volume in 1 sec. was 80,0 [47.0; 89.0]%. A positive family history of allergy was present in 12 (63%) patients, concomitant allergic rhinitis in 10 (53%), and atopic dermatitis in 6 (32%). Primary ciliary dyskinesia was confirmed in 2 (11%) patients, while atopic asthma prevailed in 15 (79%), and non-atopic asthma in 4 (21%). At the time of enrollment, 13 (68%) patients had moderate asthma, 3 (16%) mild asthma, and 3 (16%) severe asthma; 6 (32%) patients had partially controlled asthma, and 4 (21%) uncontrolled asthma; 10 (53%) patients were hospitalized for asthma exacerbation. Chest CT scans revealed BE in the middle lobe of the right and/or lingual segments of the left lung in 13 (69%) patients, while 14 (74%) patients had traction BE. The majority of patients (13/16) had purulent endobronchitis. The most common pathogen detected in the culture of bronchoalveolar lavage fluid was H. influenzae. Conclusion . A single-center study was conducted to characterize the clinical, laboratory and radiological outcomes in pediatric patients hospitalized with asthma and BA.

BACKGROUND: Bronchial asthma and allergic rhinitis often concomitant to form a single clinical and pathogenetic syndrome based on the activation of T2 inflammation. Important biomarkers of this type of inflammation are eosinophils and total immunoglobulin E. Prolonged course of allergic rhinitis in patients with asthma can lead to the development of chronic rhinosinusitis with polypoid-hyperplastic changes in the sinonasal mucosa. The effect of polypoid-hyperplastic changes on the severity of clinical manifestations and the level of systemic biomarkers of T2 inflammation in patients with a combination of asthma and allergic rhinitis in the pediatric population cannot be considered established. AIM: Сomparаtive the clinical manifestations (assessed by TNSS and SNOT-22 scales) and the content of systemic biomarkers of T2 inflammation between groups with the presence and absence of polypoid-hyperplastic changes in children and adolescents with combined course of bronchial asthma and allergic rhinitis. METHODS: The single-stage, single-center observational study included patients with asthma and AR aged 6–17 years. All patients underwent clinical and laboratory examinations, rhino endoscopy and computed tomography of the nose and paranasal sinuses according to indications. RESULTS: Totally, 268 patients were examined: 203 (75.75 %) boys and 65 (24.25 %) girls. Polypoid-hyperplastic changes in the sinonasal mucosa were detected in 31.84 % of patients. Clinical symptoms (assessed using the TNSS and SNOT-22 scales) were more pronounced in children with polypoid-hyperplastic changes compared to those without polypoid-hyperplastic changes (p 0.001), but there was no significant difference between boys and girls in terms of these symptoms. At the same time, TNSS scores were statistically significantly higher for girls than for boys (p = 0.045), and SNOT22 scores were significantly higher in both groups (p 0.05). Higher levels of eosinophils and total immunoglobulin E were also recorded by children with polypoid-hyperplastic changes (p 0.001 and p = 0,001respectively), indicating a significant role for systemic T2 inflammation in this. CONCLUSION: The presence of polypoid-hyperplastic changes in the sinonasal mucosa by children and adolescents with bronchial asthma and allergic rhinitis correlates with increased clinical symptoms and increased levels of eosinophils in blood and total serum immunoglobulin E, especially in girls. The results obtained indicate the potential involvement of systemic T2-inflammation in the processes of pathological remodeling and the formation of structural disorders of polypoid-hyperplastic changes in the sinonasal mucosa.

Post-infectious bronchiolitis obliterans (PIBO) is a chronic obstructive lung disease that develops in children following lower respiratory tract infections (LRTI). The pathogenesis is based on impaired reparative processes in the bronchiolar epithelium, productive-sclerotic inflammation, and the formation of peribronchiolar fibrosis, accompanied by chronic neutrophilic-lymphocytic inflammation and cytokine imbalance. The clinical presentation of PIBO is characterized by chronic cough, expiratory dyspnea, hypoxemia, and prolonged bronchial obstruction. The diagnosis is established based on a history of severe LRTI, persistent clinical signs of airway obstruction, and characteristic findings on chest computed tomography, including mosaic attenuation and air-trapping phenomena. Despite the use of various pharmacological strategies, including systemic and inhaled corticosteroids, macrolides, and leukotriene receptor antagonists, a standardized therapy protocol for PIBO has not yet been established. The disease course is variable: most patients demonstrate a reduction in respiratory symptoms and improvement in functional parameters with age, whereas others may experience slow disease progression. Computed tomography features of small airway involvement may persist or vary over time.

Background. Congenital alveolar dysplasia (CAD) and alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) are rare, fatal, genetically determined interstitial lung diseases in infants, associated with pulmonary hypertension. Aim. Clinical, instrumental, and pathomorphological characteristics of patients with CAD and ACD/MPV. Materials and methods. Fifteen patients with CAD (9) and ACD/MPV (6) were included in a multicenter study. The following examination methods were used: echocardiography, chest X-ray, and chest computed tomography, angiopulmonography, catheterization of the right heart, whole-exome sequencing, and histological autopsy. Results. CAD and ACD/MPV were diagnosed in term infants (13/15) and manifested in the first 3 days of life with high pulmonary hypertension resistant to therapy. Deaths were recorded in the first 3 months. Conclusion. CAD and ACD/MPV have similar clinical presentations. Diagnosis can be verified through histological examination of the lungs and whole-exome sequencing.