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Primary ciliary dyskinesia (PCD) is a rare genetic disease belonging to the group of ciliopathies. The disease develops because a defect in the ultrastructure of the epithelial cilia in the respiratory tract and similar structures (sperm flagella, villi of the fallopian tubes, ventricular ependyma, etc.) disturbs their motor function. Currently, various clinical and genetic variants of the disease are distinguished, increasing the effectiveness of dynamic examination and treatment.Aim. In this article, we describe a patient with a rare variant of PCD that we identified in combination with a mutation in the ENG gene responsible for the development of hereditary hemorrhagic telangiectasia type 1 (HHT-1). HHT-1 is a rare hereditary disease that manifests as various vascular dysplasias, including arteriovenous malformations (AVM) in the lungs, which can significantly worsen the course of the disease and be a predictor of an unfavorable outcome.Conclusion. The presented case demonstrates a combination of two rare genetic diseases in a child. The uniqueness of the case also lies in the fact that the identified rare mutation in the DRC1 gene responsible for the development of PCD is not associated with a loss of motility of the cilia of the ciliated epithelium, which makes the testing and the correct diagnosis even more difficult.

Recurrent episodes of bronchial obstruction in preschool children are one of the important risk factors for the development of bronchial asthma.Purpose. The study aimed at investigating the clinical and immunological characteristics of children with acute bronchitis and to determine the clinical effectiveness of course anti-inflammatory therapy.Material and methods. The study was a single-center prospective cohort, including 1–5-year-old patients with acute bronchitis (main group, n = 109): a group with acute simple bronchitis (n = 34) and acute obstructive bronchitis (n = 75). Reference group: children of health group 2 (n = 29). Clinical and anamnestic and laboratory methods were used. Patients with a positive API (Asthma Predictive Index) were administered montelukast at a dose of 4 mg orally once a day for 3 months (control points at 1 and 3 months), reference patients received symptomatic therapy for respiratory infection. The end point of the study is the absence of bronchial obstruction. The effectiveness of the use of Montelukast was evaluated by calculating the clinical index, efficiency coefficient. Results. A statistically significant predominance of intoxication symptoms was found in patients with acute simple bronchitis (p = 0.02), signs of respiratory failure (p < 0.001), and peripheral eosinophilia — in children with acute obstructive bronchitis (p = 0.02). In etiology, rhinovirus significantly prevailed in patients with bronchial obstruction, and RS virus prevailed in patients with acute simple bronchitis (p = 0.02). The cytokine profile of patients with acute obstructive bronchitis is characterized by a decrease in the levels of macrophage inflammatory proteins (p = 0.01) and IL-8 (p = 0.048). A prolonged course of montelukast in children with a positive API significantly reduces the severity of subsequent acute respiratory infections, reduces the frequency of bronchial obstruction and the risk of bronchial asthma (p < 0.001).Conclusion. Evaluation of the results of montelukast therapy demonstrated clinical efficacy in patients with a positive API index.

Aim. Selection and combination of immunodiagnostic tests to detect active tuberculosis (TB) in children with concomitant bronchopulmonary pathologies.Materials and methods. A prospective study of children and adolescents (n = 236) was conducted, which included cases with localized pulmonary tuberculosis; pulmonary tuberculosis associated with allergic or infectious and inflammatory forms of chronic nonspecific lung diseases (CNSLD); chronic nonspecific lung diseases under a significant absence of active TB but the presence of Mycobacterium tuberculosis. All patients underwent immunodiagnostic tests by Mantoux test, recombinant tuberculosis allergen (RTA) test, and QuantiFERON – GIT.Results. Mantoux and RTA tests were found to exhibit high sensitivity in children both with tuberculosis associated with concomitant pathologies and without such pathologies. Differences were observed in the results obtained by the Mantoux test in children with tuberculosis-associated bronchopulmonary pathology and those with localized tuberculosis. The Mantoux test may be positive as a result of many factors, other than active TB infection. Thus, children with allergic and infectious-inflammatory pathologies of the lungs exhibit an altered sensitivity to the Mantoux test. Such cases require in vitro diagnostics with the Quanti- FERON test, whose sensitivity is high despite the presence of CNSLD.Conclusions. Children with infectious-inflammatory CNSLD should undergo TB examination in stages based on in vivo and in vitro tests, when necessary. Children with allergic CNSLD should undergo in vitro tests at the first stage.

Heterotaxy syndrome is a congenital malformation in which the internal organs of the chest and abdominal cavity have an abnormal location. People suffering from this syndrome have multiple complex defects in the heart, blood vessels, spleen, liver, lungs and other organs. Heterotaxy is a rare pathology that requires a multidisciplinary approach to diagnosis. This article demonstrates a rare case of heterotaxy observed in the pulmonology clinic of the Veltischev Institute.

The problem of the combination of tuberculosis and chronic nonspecific diseases of the bronchopulmonary system in children and adolescents has not been sufficiently studied to date and remains relevant.Purpose. The study aims at determination of predictors of tuberculosis infection to identify groups at high risk of developing tuberculosis among children with chronic nonspecific pathology.Materials and research methods. A prospective study of children and adolescents (n = 158) with isolated respiratory tuberculosis and its combination with various forms of chronic obstructive pulmonary disease (lung defects, chronic bronchitis, bronchial asthma) was conducted. These two groups were compared in respect of the medical history and physical examination data, as well as computed tomography findings.Results. The predictors of the development of tuberculosis included cases of pneumonia at an early age, acute respiratory infections 3 or more times a year, and the early age of children infected with Mycobacterium tuberculosis.Conclusion. These conditions increase the risk of developing active tuberculosis in children and cause its more severe course. In addition, chronic nonspecific lung diseases associated with tuberculosis often complicate the course of a specific process and are often characterized by recurrent attacks.

Primary ciliary dyskinesia (PCD) is a rare hereditary disease from the group of ciliopathies, which is based on a defect in the cilia ultrastructure of the respiratory epithelium and similar structures (sperm flagella, villi of the fallopian tubes, ventricular ependyma, etc.), leading to motor function impairment. The prevalence of the disease varies significantly around the world and is not known reliably in the Russian Federation.The aim of the review was to analyze literature data on modern approaches to the diagnosis and treatment of PCD.Methodology. The data of 90 articles and the opinions of experts providing care to patients with PCD were used.Results. The classic manifestations of PCD depend on age. The leading manifestations of the disease in patients with PCD are recurrent inflammatory diseases of the upper and lower respiratory tract (bronchitis, pneumonia), with the formation of bronchiectasis, damage to the ENT organs (chronic rhinitis, rhinosinusitis, nasal polyposis, repeated otitis media, progressive hearing loss). Currently, there is no single method which could serve as a “gold” standard for diagnosing PCD. The diagnosis of PCD is based on the characteristic clinical picture in combination with the results of special tests (nitric oxide in exhaled air, DNA diagnostics, high-speed video microscopy, transmission electron microscopy). The genetic diagnostics has not been developed sufficiently in the global practice yet and is unavailable in our country. The approach to the treatment of a patient with PCD should be multidisciplinary due to multiple organ lesions. According to the European consensus, the goal of PCD therapy is to restore or maintain normal lung function. There have been no randomized trials of treatment for PCD, and therefore all treatment recommendations are based on very low-level evidence or extrapolated from cystic fibrosis guidelines. Recommendations on mucolytic, antibacterial and anti-inflammatory therapy of PCD are given with consideration for the international and domestic experience.Conclusion. The development of a new version of clinical guidelines containing up-to-date relevant information will improve the diagnosis and treatment of PCD in the Russian Federation.

Objective. To determine the trends in the structure and assess medical, social and clinical aspects of the consumption of tobacco and nicotine-containing products among adolescents aged 1417. Material and methods. During the study, 352 adolescents aged 14 to 18 years studying in urban (n = 202) and rural (n = 150) schools of Yaroslavl region were surveyed in 20202021. Statistical processing of the data was carried out using computer programs "Statistica" v.10.0. Results. The survey of teenagers revealed a fairly high prevalence not only of active tobacco smoking (tried 37 %, actively smoked 30 % of adolescents), but also the use of "new" nicotine-containing products (vape, hookah, electronic cigarettes, snus, snuff) (tried 38 %, actively used 7 % of teenagers). The structure of active tobacco use and NRTI use was found to differ by the place of residence. Active tobacco use accounted for 46.7 % of rural adolescents and 19.8 % of urban residents (p 0,01). Conclusions. In modern adolescents, along with tobacco smoking, a negative trend has been detected to increase the use of nicotine-containing products, especially hookah. This substantiates the need to improve educational anti-nicotine programs, to introduce a system of regular regional monitoring of the prevalence of traditional and new nicotine-containing products use among children and adolescents in order to make quick and effective decisions.

The article discusses the role of the central nervous system, including mental disorders, in bronchial asthma in children. This problem has been of interest to practicing pediatricians and researchers for a long time, but so far it has not had unambiguous solutions. The authors have a diverse approach to its assessment, leaning towards the modern concept of ‘psychopathological’ or ‘psychological’ diathesis put forward by S.Yu. Tsyrkin. On specific clinical examples, the authors clearly demonstrated the influence of mental disorders on the course of bronchial asthma in children, which, of course, must be taken into account in practical work by pediatric pulmonologists.

Based on the analysis of foreign and domestic literature, the authors present the approaches to determining the phenotypes of bronchial obstructive syndrome in preschool children. They outline the data on the main foreign studies devoted to the peculiarities of the bronchial obstruction syndrome course depending on age and clinical outcome. They discuss the risk factors leading to the recurrent course of bronchial obstruction, tools for predicting bronchial asthma, and the choice of phenotype-dependent therapy strategy.

Respiratory diseases persistently lead in the structure of general morbidity in both children and adolescents. Acute respiratory infections are the most common among them. Interest of pediatricians to phytotherapy is not accidental and is due to the fact that it has a mild therapeutic effect, enhances the effectiveness of complex therapy, has few side effects. Of particular interest are herbal remedies containing ivy extract (Hedera helix), the active ingredients are mainly found in ivy leaves, they are biologically active substances: Triterpene saponins, glycosides, also contain carbohydrates, essential oils, steroids, phenolcarboxylic acids, coumarins, flavonoids, carotenoids, tocopherols (vitamin E), B and C vitamins, tannins, phytoncides. Ivy preparations are prescribed as an expectorant in the treatment of infectious and inflammatory diseases of the upper and lower respiratory tract due to their proven multidirectional effects: secretolytic, mucolytic, bronchospasmolytic. They also have anti-inflammatory, reparative, antioxidant effects.This article presents a clinical example of an officinal ivy-based preparation used in the treatment of a 3.5 year old patient diagnosed with acute respiratory viral infection. Temperature increased to 38 °C, runny nose; on the second day of illness the temperature remained subfebrile and a dry cough appeared. Nasal vasoconstrictor drops and oral acetylcysteine were prescribed, but there was no relief. On the third day of the illness, against the background of subfebrile temperature, the cough persisted, single dry rales were heard in the lungs, wheezing appeared during physical exertion. Acetylcysteine was withdrawn and replaced by ivy-based drops (16 drops 3 times a day orally after meals in a small amount of water). After 2 days the cough was moist, the sputum was easily expectorated, the wheezing in the lungs was almost gone, there was no more wheezing and the temperature was normal. By the sixth day of treatment the cough was gone and the girl had almost recovered. Nevertheless, Gedelix was continued for another 4 days. Prospective results and detailed study of the effects of herbal preparations on the basis of ivy extracts served as the basis for their recommendation as a complex therapy of cough in children.

The respiratory diseases are consistently ranked first in the morbidity pattern among children and adolescents in the Russian Federation. Registry data show that 10-12% of children with pulmonary hypertension have PH-associated chronic lung pathology. Pulmonary hypertension as a life-threatening complication of such chronic lung diseases as hypersensitive pneumonitis, bronchiectasis, bronchial asthma, etc. aggravates the severity of their clinical course and has a great impact on the outcome of the disease. The article summarizes modern data on pulmonary hypertension in children of various ages associated with chronic bronchopulmonary pathology. The issues of classification, pathogenetic mechanisms, criteria and modern methods of diagnosis and treatment, as well as prognosis are considered. The authors come to the conclusion that despite the in-depth study of the pathogenetic aspects of the development of pulmonary hypertension in chronic bronchopulmonary pathology in children, which has recently made it possible to shed light on the understanding of many of its aspects, it should be admitted that the problem as a whole is far from being resolved. Early detection and treatment of pulmonary hemodynamic pathology is extremely important to prevent the formation of cor pulmonale. Pulmonary hypertension, which complicates the chronic lung diseases, is still incurable and is associated with high mortality. Now, young patients with pulmonary hypertension are still often examined and treated using guidelines for adult patients. However, differences in etiology, clinical presentations and diagnostic capabilities require a special approach to studying this problem in children, which raises the question of the need to develop separate clinical guidelines for pediatric practice.

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.

The results of the clinical approbation of Patient-specific therapy of bronchial asthma in children using the course method of applicationof the monoclonal antibodies (anti-IgE-therapy) by the Ministry of Health are summarized in order to assess the effect of this typeof treatment on the disease exacerbation rate, including those associated with the seasonal acute respiratory infections. The conclusion was made regarding the high clinical efficiency in relation to all parameters studied and safety of this type of the treatment. Itwas determined that the monitoring of the level of specific IgE receptors on basophils using the cytoflowmetry method, which is concomitant to anti-IgE therapy, makes it possible to predict the efficiency and individual duration of anti-IgE therapy for uncontrolledatopic bronchial asthma in children.

The Article considers the argumentative issues of the diagnosis of the virus-induced bronchial asthma and recurrent obstructive bronchitis in the children of infant and preschool age, the commonality of their pathogenesis, the feasibility and efficiency of their therapy using the monteleukast – the antileukotriene receptor inhibitor. Along with the anti-inflammatory properties of the monteleukast, the potential antiviral activity discovered in it was considered, in particular, against the SARS-CoV-2 virus, which causes COVID-19.

Objective: The objective is to assess the clinical efficacy of the inclusion of release-active medication of affinity purified antibodies to protein S100 in complex rehabilitation of infants with old acute obstructive bronchitis affected by perinatal pathology of central nervous system of hypoxic genesis.Methods: 210 infants aged from 2–36 months, who suffered events of acute obstructive bronchitis, were examined. Along with clinical methods of examination, a computer bronchophonography and assessment of heart rate variability were performed.Results: Additional inclusion of release-active medication ‘Tenoten for children’ in the complex of rehabilitation of these patients had a positive effect on autonomic balance and autonomic component of bronchial obstruction. At the same time, the short course of Tenoten (1 month) did not prevent recurrent events of bronchial obstruction.Conclusions: Probably, patients with recurrent events of acute bronchial obstruction in combination with pronounced functional disorders of vegetative state require longer courses of release-active medication which normalized the activity of the central nervous system.

Primary ciliary dyskinesia is a rare genetically determined pathology leading to the development of chronic inflammatory lesions of the respiratory system in children, impaired fertile function in older patients. The disease is characterized by an autosomal recessive mode of inheritance with marked genetic heterogeneity. The article describes clinical observation of a patient – carrier of a rare mutation and describes the features of this case.Conflict of interest: The authors of this article confirmed the lack of conflict of interest and financial support, which should be reported.

Тhe article provides an overview of modern methods of assessing the respiratory function in children taking into account their age peculiarities. The emphasis is made on high-precision ultrasonic spirometry, evaluation of static pulmonary volumes and determination of the ventilation inhomogeneity index by means of nitrogen washout with oxygen compared to the traditionally used methods. Authors’ own results of approbation of these test methods are presented and their comparative assessment is provided.

The definition of severe asthma is based on the criteria for clinical control, the treatment received, as well as the response to therapy, the assessment of future risk. In severe bronchial asthma, control can be achieved only at the highest possible level of therapy, namely, in treatment corresponding to the 4th or 5th stage. The article highlights the features of the clinical phenotype of severe bronchial asthma in children. Purpose: to identify the predictors of severe phenotype in children, the analysis of clinical and anamnestic features, to study the dynamics of disease control. Materials and methods: a group of patients with different degrees of severity of bronchial asthma aged 3 to 12 years, both sexes, was studied. The clinical aspects of the disease, the dynamics of control over bronchial asthma have been studied. Functional tests were carried out: examination of the function of external respiration, pyclofometry. Data of a specific allergic diagnosis (skin tests with non-bacterial allergens, detection of specific IgE antibodies), self-monitoring tests were studied. Statistical methods used a nonparametric method, a c2 distribution, a Pearson test, using conjugacy tables. Results and discussion: the family female phenotype is a predictor of severe bronchial asthma in patients in this group. The severity of the disease on the background of therapy for five years in a group of patients was revised only in a third of children. With this phenotype, partial control over the disease was achieved. The ineffectiveness of control is associated with the presence of a comorbid background: the pathology of the nervous system, the gastrointestinal tract, endocrine disorders. The severe phenotype of bronchial asthma, independently of age debut, was significantly less frequent than in the case of moderate disease.