A5031142755- Cystic Fibrosis Research Advances
- Pediatric health and respiratory diseases
- Neonatal Respiratory Health Research
- Human Health and Disease
- Respiratory viral infections research
- Respiratory and Cough-Related Research
- Child Nutrition and Feeding Issues
- Inhalation and Respiratory Drug Delivery
- Tracheal and airway disorders
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Asthma and respiratory diseases
- Adolescent and Pediatric Healthcare
- Healthcare Systems and Public Health
- Digestive system and related health
- Child and Adolescent Health
- Bacterial biofilms and quorum sensing
- Child Nutrition and Water Access
- Pneumonia and Respiratory Infections
- Congenital Diaphragmatic Hernia Studies
- Immunodeficiency and Autoimmune Disorders
- Long-Term Effects of COVID-19
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Immune responses and vaccinations
- Gut microbiota and health
- COVID-19 and Mental Health
Scientific Center of Children's Health
2015-2025
Ministry of Health of the Russian Federation
2018-2025
Russian Children's Clinical Hospital
2018-2025
Sechenov University
2014-2025
Center for Children
2012-2025
City Clinical Hospital
2024
Liverpool Heart and Chest Hospital
2021
University of Liverpool
2021
Imperial College London
2021
Pirogov Russian National Research Medical University
2019
Primary ciliary dyskinesia (PCD) is a rare hereditary disease from the group of ciliopathies, which based on defect in cilia ultrastructure respiratory epithelium and similar structures (sperm flagella, villi fallopian tubes, ventricular ependyma, etc.), leading to motor function impairment. The prevalence varies significantly around world not known reliably Russian Federation. aim review was analyze literature data modern approaches diagnosis treatment PCD. Methodology. 90 articles opinions...
The aim of the review is to analyse Guidelines in updated version ESPEN-ESPGHAN-ECFS 2024 on infant, child and adult nutrition cystic fibrosis (CF). previous Guideline was published 2016. An expert working group physicians, dietitians, patient community representatives nutritional recommendations, including methods for assessment management at all ages. Chapters pregnancy, liver disease, CF-related diabetes, bone mineral supplements, probiotics have been expanded. There are new chapters use...
T.E. Borovik, O.I. Simonova, S.V. Voronin, T.V. Bushuyeva, Ina Sokolov, A.A. Pashkevich, A.E. Lavrova, N.A. Ilyenkova, D.F. Sergiyenko, Agafonova, N.Yu. Filina, I.P. Karimova. "Recent developments in the nutrition of children with cystic fibrosis," Resolution by Expert Council dated December 12, 2024, Moscow, Russia. Pediatria n.a. G.N. Speransky. 2025; 104 (1): 151-154. DOI: 10.24110/0031-403X-2025-104-1-151-154.
Introduction. The microbial biodiversity of the respiratory tract in children with cystic fibrosis (CF) various subjects North Caucasus Federal District (NCFD) has not yet been sufficiently described. In NCFD CF were previously shown to have specific clinical manifestations disease and changes frequencies pathogenic variants CFTR gene. this regard, topic work is relevant. aim study. To detect microbiota composition NCFD. Materials methods. There examined one hundred 48 children, who...
The aim of the experts who provide health care for patients with cystic fibrosis (CF) has been to develop clinical guidelines on children and adults. Methods. 2020 Clinical Guidelines were based European consensus documents national “Cystic fibrosis: definition, diagnostic criteria, therapy” (2016, 2019). When creating guidelines, guided by Article 2 Federal Law No.323-FL “On basics in Russian Federation” as November 21st, 2011, regulatory legal governing development approval...
Cystic fibrosis (CF; OMIM #219700) is a common autosomal recessive disease caused by pathogenic variants (henceforward mutations) in the cystic transmembrane conductance regulator gene (CFTR). The spectrum and frequencies of CFTR mutations vary among different populations. Characterization specific distribution can be used to optimize genetic counseling, foster reproductive choices, facilitate introduction mutation-specific therapies. Chechens are distinct Caucasian ethnic group Nakh peoples...
Chronic endobronchial inflammation and bacterial infection are the main causes of morbidity mortality in cystic fibrosis (CF), an autosomal recessive genetic disorder associated with improper function chloride channels. Inflammation CF lung is greatly amplified after Pseudomonas aeruginosa infection. In this study relationship between P. status inflammatory markers has been investigated. Seventeen children acute exacerbation were examined. patients without characterized by elevated activity...
научный биометрический журнал, который знакомит читателя с клиническими рекомендациями и особенностями применения лекарственных вакцинных средств у детей, предоставляет исчерпывающую информацию о воздействии на плод, проводимых в
The main causative agents of lung infection in patients with cystic fibrosis (CF) are P. aeruginosa, S. aureus and H. influenzae. In the last decade, gram-negative nonfermentative microorganisms (NFMO) - Вurkholderia cepacia complex (Bcc), Stenotrophomonas maltophilia, Achromobacter xylosoxidans non-tuberculous mycobacteria, fungi genus Aspergillus have acquired clinical significance. It is found that chronic 2/3 cases caused by association microorganisms. Among hospitalized patients,...
Introduction. Currently, there are still problems in the rehabilitation of children with chronic diseases and disabilities, as well establishment disability itself, which is primarily due to lack common criteria for describing health, functioning child’s body, their disorders at different ages assessing effectiveness measures. Purpose. To develop disturbance suffered from various disabling terms quality life. Materials methods. By means developed Russian-language version standardized...
Objective. To assess the efficacy of nocturnal hyperalimentation via a low-profile gastrostomy tube for in improvement nutritional status and lung function children with severe cystic fibrosis (CF). Patients methods. We used nutrient solution installed under endoscopic control to improve 16 CF protein-calorie malnutrition aged between 5.5 17.5 years. The mean duration was 1.3 years (range: 7 months–54 months). evaluated using WHOAntho Plus software; pulmonary by assessing forced expiratory...
The 15-years practice of Pulmozyme (dornase alfa) usage showed its high efficacy and safety in cystic fibrosis treatment children various age, including infants. the drug is associate with clinical effects: strong mucolytic, anti-inflammatory antibacterial effects. appropriateness this into complex basic therapy order to prevent treat chronic bronchitis polypous pansinusitis as soon disease diagnosed was confirmed studies. Regular use results relapse rate decrease, remission periods...
The effect of pharmacones clathration with glycosides results in significant decrease drug agent dosage. We investigated the clathrates hypotensive Nifedipine glycyrrhizic acid, which was prepared molecular proportion 1:4 respectively. Present study carried out on calcium channels neurons Lymnaea stagnalis mollusc. Keywords: Pharmacones clathration, Nifedipine, Glycyrrhizic Calcium
Background. Cystic fibrosis is a hereditary disease that occurs as result of mutations in the regulator gene chloride ion transmembrane transport (CFTR). Finding CFTR necessary for identification clinical features cystic fibrosis. Objective. Our aim was to identify genotype-phenotype correlations between first class pathogenicity and manifestations based on studying prevalence structure mutations. Methods. The study included children under 18 years with admitted hospital 2013 2017. Biallelic...
Since the beginning of COVID-19 epidemic, European cystic fibrosis society (ECFS) has decided to launch a special ECFS-COVID-19 program collect information on characteristics in patients with (CF). The results should help timely and efficiently provide CF necessary care. Initially, it was assumed that would be severe patients. aim . Тo assess prevalence (CF) Russian Federation (RF). Methods 6 cases (4 children 2 adults) were analyzed. Results There are 405,843 infected SARS-CoV-2 Russia,...
научный биометрический журнал, который знакомит читателя с клиническими рекомендациями и особенностями применения лекарственных вакцинных средств у детей, предоставляет исчерпывающую информацию о воздействии на плод, проводимых в
The authors show the data on current microbiological pattern in children with acute and chronic respiratory disorders dynamics of microflora susceptibility to most frequently used antibiotics over recent years. principles antibiotic choice control for their efficiency, peculiarities usage common side effects are given. New aspects combined antibacterial agents - ecoantibiotics – discussed. Including Lactulose Anhydro into composition allows achieve high efficacy does not cause...
Bronchial obstruction is a widespread pathological condition in children. The development of this syndrome bronchial asthma caused by an allergic inflammation the mucous membranes respiratory tract with participation leukotrienes. It found that blocking leukotriene receptors using montelukast drugs has therapeutic effect, including children at age 2 and above. are well tolerated children, can prevent post-exertional bronchospasm some clinical situations be used as alternative to glucocorticoids.
The author analyzed the most common questions of mucolytic agents selection asked by clinicians. various clinical effects N-acetyl cysteine and indications for different forms this drug, especially in infants, are discussed article. Indications contraindications acetyl usage treatment dry productive cough combination with other medicines first 24 hours acute respiratory tract infections, accompanied adherent bronchial discharge, listed. causes pulmonary «swamping» measures prevention condition shown.
The article discusses the use of N-acetylcysteine in pediatric practice and examines various clinical effects acetylcysteine, including acute respiratory infections children, peculiarities its application. causes for phenomenon «lung bogging» course treatment with mucoactive means are analyzed separately measures prevention proposed. contains results multicenter studies data systematic reviews that show high therapeutic efficacy safety acetylcysteine treating both chronic upper lower tracts....