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Vol 78, No 5 (2023)
- Year: 2023
- Articles: 10
- URL: https://vestnikramn.spr-journal.ru/jour/issue/view/94
- DOI: https://doi.org/10.15690/vramn.785
PEDIATRICS: CURRENT ISSUES
Scientific Research in the Field of Children’s Health and Development
Abstract
The focus on the interests of children has always been inherent in the Russian State, but the most effective measures have been implemented already in this century: 4 years before WHO announced the Global Strategy for Mothers and Children in 2016, Russian President Vladimir Putin signed the Decree on the National Strategy for Children for 2012–2017, in 2017. The President announced the “Decade of Childhood”, and in 2020 The priority of childhood was enshrined in the Constitution of the Russian Federation. In the Russian Federation, the most child-oriented system of children’s healthcare has been built, in medical and scientific organizations of the pediatric profile of the Russian Federation, specialists with medical, biomedical, psychological, pedagogical, and social education are specially trained to work with children of different ages, and scientific research in the field of pediatrics is carried out by specialized researchers within the framework of special programs focused on the needs of children’s healthcare. A detailed analysis of the structure and content of the projects implemented in 2022 has been carried out. Scientific projects involving children compared to 2018, when the implementation of the “Decade of Childhood” program began, which indicates that scientific research in the field of children’s health and development is voluminous, but uncoordinated. Many topics are carried out simultaneously by several scientific and medical or educational organizations, but they are not coordinated in unified scientific and methodological approaches. At the same time, there are systemic problems, to solve which it is necessary to switch to network scientific research of population health; to strengthen the integration of research teams both in the context of interdepartmental cooperation (the Ministry of Education and Science of the Russian Federation and the Ministry of Health of the Russian Federation — Rospotrebnadzor — Federal Medical and Biological Agency), and joint scientific work with other ministries (the Ministry of Education of the Russian Federation, the Ministry of Sports of the Russian Federation, the Ministry of Culture of the Russian Federation, etc.); as well as to improve financial mechanisms for managing scientific activities.
384-399
Psychophysical and Electrophysiological Parameters of the Auditory Analyzer as Indicators of the Effectiveness of Cochlear Implantation in Children with Bilateral Deafness
Abstract
Background. The results of rehabilitation of patients with deafness after cochlear implantation (CI) depends on many factors related on both the individual characteristics of the patient and the technical parameters of auditory nerve stimulation — fitting of the CI system processor. Article presents the results of a study of the relationship between psychophysical (subjective) auditory sensations and electrophysiological (objective) responses of the structures of the auditory analyzer to electrical stimulation. Aims — to evaluate the relationship between the electrophysiological responses of the auditory analyzer and the psychophysical parameters of the thresholds of sound perception and speech intelligibility in patients with deafness after CI. Methods. The study involved 88 patients with IV bilateral sensorineural hearing loss, borderline with deafness, aged 6 to 17 years 11 months, among whom 54.5% (n = 48) of girls and 45.5% (n = 40) of boys. Of the patients included in the follow-up, 40% (n = 35) underwent unilateral CI; 60% (n = 53) of children underwent bilateral sequential CI. In our study, slightly more than half of the patients (51.1%) had experience using the CI system for more than 5 years. At the first stage of the study, we studied the relationship between sound perception thresholds (tonal audiometry in a free field) and registered ASSR (auditory steady state response) thresholds in patients with deafness receiving rehabilitation after CI surgery. The relationship between behavioral and electrophysiological (objective) thresholds was assessed by comparing the results of tonal threshold audiometry and the responses received during ASSR registration to acoustic stimulation in a free field. At the second stage, patients with indications for processor fitting (decreased speech intelligibility, uncomfortable sensations) underwent speech audiometry, evoked compound action potentials (ECAP) registration, according to the results of which the parameters of the CI processor were changed. Results. Research data showed the effectiveness of the approach of including the ECAP registration test, followed by the creation of a fitting card and correction of the level of auditory nerve stimulation under the control of speech audiometry in the protocol of fitting the CI processor. Conclusions. The results of the conducted studies allow us to recommend the use of the developed method of objective recording of sound perception thresholds in patients with CI as one of the indicators of the effectiveness of CI in the early period of auditory-speech rehabilitation in patients with undeveloped speech skills and when it is impossible to conduct objective tests accompanied by supra-threshold stimulation of the auditory nerve.
400-407
A Single-Stage Population-Based Study of the Relationship between Cognitive and Somatic Health Parameters in Children of Secondary School Age
Abstract
Background. One of the four important components of the formation of cognitive functions is somatic health. But to date, there are no population studies that consider the relationship with cognitive functions and school performance of a large range of somatic factors, which allows us to compare the strength of their hypothetical contribution to cognitive functioning with each other. This study is the second part of a population-based study, the first part of which is presented in the previous publication "A Single-Stage Population-Based Study of the Prevalence of Mild Cognitive Impairment in Children of Secondary School Age". Aims — to determine the main patterns in the relationship between cognitive-academic and somatic factors in a cohort of Russian children, 5th grade students at school. Methods. In Russian schoolchildren of the 5th grades of municipalities representing cities of all federal districts of the Russian Federation, the links with integrative cognitive success, the number of subtests performed at the level of mild cognitive impairment, the results of individual cognitive subtests, academic performance and the leading hand factor were analyzed — the following somatic factors: the presence of skin pathology, bronchial asthma, orthopedic, ophthalmological disorders, visual acuity, body mass index, parameters of the study of the function of external respiration, electrocardiography, ultrasound examination of the thyroid gland, laboratory blood tests. Results. The results of the survey of 1036 participants, 51% of them girls, were admitted to the analysis. It has been established that iron content is directly related to integrative cognitive success and school performance, the relationship is especially strong between subgroups of iron content above and below 26.4 mmol/l. Clinical levels of erythrocytes are more strongly associated with integrative cognitive success and individual cognitive functions than other factors: in erythropenia cognitive parameters are worse. The presence of thyroid cysts directly correlates with some of the worst parameters of cognitive activity. High body mass index and low hemoglobin are associated with poorer academic performance. Conclusions. The results of the study for the first time on a cohort of Russian schoolchildren showed a connection with cognitive activity and school performance of a number of somatic factors, including iron content, which requires further in-depth study.
408-430
Structural Parameters of the Brain and Bone Structures of the Head and Neck in Patients with Various Types of Mucopolysaccharidoses According to Magnetic Resonance Imaging of the Brain
Abstract
Background. Mucopolysaccharidoses are diseases from the group of lysosomal storage diseases that have a progressive course. CNS damage is one of the main factors in the development of severe, life-threatening complications. Aims — аssessment of structural changes in the brain and bones of the head and neck in patients with various types of mucopolysaccharidoses. Methods. The research included 136 children aged from 11 months to 17 years, 81 patients of which showed various types of mucopolysaccharidoses: MPS I — 15 people, MPS II — 37, MPS III A — 10, MPS IIIB — 4, MPS IIIC — 2, MPS IVA — 6, VI — 7 people. The control group included 56 children without neurological, psychiatric and severe somatic illnesses. Results. For mucopolysaccharidoses types I, II, III and VI, the most characteristic structural changes on the brain MRI were white matter lesions, mainly periventricular: expansion of the perivascular spaces (70%), atrophy of the cerebral hemispheres (42%), hippocampus, (31%), ventriculomegaly (6.2%), stenosis of the cervical spine (64%), hydrocephalus, expansion of the cerebrospinal fluid spaces of the posterior cranial fossa, arachnoid cysts. Conclusions. The results of the obtained data analysis made it possible to identify the macrostructural specifics of the brain disorders and cervical spine in various types of MPS, as well as their prognostic significance.
431-440
Biologic Drug Survival in Pediatric Psoriasis: A Retro-Prospective Observational Study
Abstract
Background. Irrational selection of biological therapy leads to its inefficiency and early cancellation, aggravating the course of psoriasis and worsening the quality of life, which has negative socio-economic consequences associated with the cost of initiating and providing the patient with a new biological agent. Currently there are no clearly defined strategies and unified protocols for therapeutic tactics regarding the choice and switching of biological drugs in children with psoriasis, which requires research aimed at studying the survival of biological therapy in order to draw up evidence-based recommendations with a high evidence base. Aims — study aims to determine survival rate and significant predictors of biological therapy discontinuation. Methods. A retro-prospective observational study was conducted, which included patients with psoriasis vulgaris, aged 4 to 18 years, who had previously received or needed biological therapy. The data of patients over an 8-year period (from 2015 to 2023) were analyzed. Follow-up was performed from initiation to discontinuation of biological therapy, in cases where outcomes were unknown data was censored. Analysis of therapy survival was carried out using the Kaplan–Meier method with curve construction, the assessment of intergroup differences was carried out using a log rank test. Identification of significant predictors of biological therapy discontinuation was carried out using the Cox multivariate regression method. Results. 430 cases of biological therapy were selected from patients with psoriasis vulgaris aged 5 to 18 years. The highest survival rate of biological therapy was obtained for ustekinumab — 54.9 months, the lowest values — for etanercept (26.7 months). The survival values of adalimumab — 33.9 months and secukinumab — 34.5 months did not statistically significantly differ from each other (p = 0.387). Predictors of early discontinuation of biological therapy were established: presence of a burdened family history (HR = 3.861, p = 0.006); delayed prescription of biological therapy — > 2 years from the date of diagnosis (HR = 1.447, p = 0.045); long-term (> 6 months) use of methotrexate (HR = 3.085, p < 0.001) or cyclosporine (HR = 4.538, p < 0.001) previous to the biological treatment; the presence of comorbidity (inflammatory bowel diseases (HR = 4.938, p = 0.001), metabolic syndrome (HR = 3.947, p < 0.001) or psoriatic arthritis (HR = 2.337, p < 0.001). Conclusions. Proved the inexpediency of long-term immunosuppressive treatment with non-biological drugs and the need for early prescription of biological therapy. In the presence of a burdened family history and disease duration > 2 years since the diagnosis of psoriasis, ustekinumab or secukinumab are recommended as first-line biological therapy in children, the use of which is also associated with a longer duration of treatment and a lower risk of developing adverse events in patient with metabolic syndrome. In active Crohn’s disease, adalimumab is most recommended, followed by a possible switch to ustekinumab. Secukinumab is the drug of choice for patients diagnosed with psoriatic arthritis.
441-454
Modern Possibilities for Predicting Systemic Manifestations of Food Allergy Based on the Results of a Prospective Cohort Study
Abstract
Background. Food allergies (FA) are a common problem in childhood. Food anaphylaxis (FAN) can be life threatening. In this regard, it is reasonable to study clinical and anamnestic data and factors that can influence FAN formation in order to develop mathematical models of prognosis aimed at reducing acute episodes of FA, including FAN. Aims — to establish clinical and pathogenetic factors contributing to the formation of systemic manifestations of FA in children and to develop a mathematical method for predicting severe systemic reactions to food. Methods. A prospective cohort open study included 76 children (5.12 ± 3.74 years) suffering from persistent FA with a history of FAN episodes. The comparison group included 134 children (7.18 ± 2.52 years old) who had atopic diseases, local manifestations of FA, but without systemic reactions to food. Illness and life history, presence and severity of concomitant diseases were studied, general clinical and immunological studies were performed (in blood serum and oral cavity). Results. Based on the data in 210 children, the odds ratio (OR) of systemic FA formation was calculated, where the risks were: caesarean section (OR 1.8; 95% CI [1.02; 3.01]; p < 0.05), immediate allergic reactions to food (OR 3.3; 95% CI [1.47; 7.39]; p < 0.05), maternal anemia during pregnancy (OR 3.5; 95% CI [1.83; 6.57]; p < 0.05), allergic diseases in siblings (OR 4.8; 95% CI [2.04; 11.18]; p < 0.05), presence of atopic dermatitis (AD) (OR 3.7; 95% CI [1.77; 7.68]; p < 0.05), allergy to cow’s milk proteins (CMP) (OR 7.8; 95% CI [4.31; 14.24]; p < 0.05), clinical need for introduction of amino acid formulae (OR 100.8; 95% CI [13.37; 760.67]; p < 0.05). A mathematical method was found for predicting FAN with a decision rule Y ≥ 0.35, with a forecast efficiency of up to 90% when using a linear regression equation: Y = 0.0518 + 0.2983×A1 + 0.2390 × A2 + 0.4793×A3 – 0.0037 × A4 + 0.0002 × A5, where A1 is CMP as the first allergic product (0 or 1), A2 is rapid onset of reaction (0 or 1), A3 is amino acid formulae use (0 or 1), A4 is age of wheezing (in months), A5 is eosinophils (cells/µl). Conclusions. Symptoms of AD and severe FA to CMP, requiring amino acid formulae introduction, should be considered as alarming anamnestic data for predicting possible FAN episodes, if the child has immediate type of FA, the mother’s pregnancy proceeded against the background of anemia, delivery was carried out operatively way and there are already children with atopic diseases in the family. The proposed mathematical method for predicting FAN makes it possible to determine probability of severe allergic reactions to food in children with an efficiency of up to 90%.
455-466
The Prevalence of Sensitization Profiles to Various Allergens in Children in the Moscow Metropolitan Area
Abstract
Background. In Russian scientific literature there is a limited amount of data on the prevalence of sensitization to various types of allergens in children. The determination of sensitization profiles is important for the formation of personalized measures for patients. Aim — to reveal the prevalence of sensitization profiles to allergen extracts and components in children with different variants of the atopic phenotype, taking into account regional characteristics. Methods. A cross-sectional study was conducted in 160 children from 0 months to 17 years 11 months with symptoms of allergic rhinitis, food allergy, atopic dermatitis, living in the Moscow metropolitan area. The sensitization to 9 extracts of allergens was analyzed in all children using the ImmunoCAP method, component resolved diagnostics was held with “Allergochip ISAC ImmunoCAP” in 143 patients. Results. sensitization to birch pollen was detected most often (68%), less often (52%) — to timothy grass, (48.1%) — to mugwort. Among food allergens, most often (55% each) children were sensitized to extracts of egg white, wheat flour, less often — cow’s milk (48%), Atlantic cod (15.6%). Analysis of molecular sensitization showed that most often patients were sensitized to Bet v 1 (64%), Aln g 1 (50.3%), Сor 1.0101 (48.3%), Fel d 1 (42.7%) cases. Sensitization to cat/dog allergen components was detected in 79.7% patients. Asymptomatic sensitization to cat allergens was observed in 35.8%, to dog — in 40% patients. More than half of the patients had sensitization to the PR-10 components. From 2 to 10% of patients had sensitization to LTP, most often to the Jug r 3 walnut component in 10.5% patients. Conclusion. The profiles of molecular sensitization in children with various variants of the atopic phenotype were clarified, the prevalence of sensitization to extracts and components of pollen, household, food and animal allergens, the incidence of true and asymptomatic sensitization were determined.
467-482
Innovative Technologies in Pediatric Neuro-Oncology
Abstract
At the present time pediatric neuro-oncology develops rapidly mostly due to the deep understanding of etiology and pathogenesis of the brain tumors in children, widespread introduction of molecular genetic technologies into diagnostic workflow and emergence of targeted therapeutic agents directing to the neoplastic cells. Many tumor entities undistinguishable at the level of histopathology were classified by the molecular techniques and now present as unique disorders. Clinical heterogeneity unraveled by molecular classification is a basis for modern risk stratification approaches. Variety of new tumor entities were discovered only because of implementation of advanced molecular diagnostics, which led to identification of the recurrent genetic aberration in neuroepithelial tumors with BCOR and PATZ1 genes alteration, intracranial mesenchymal tumors with FET-CREB rearrangements. The discovery of the targetable molecular drivers in gliomas allows the introduction of targeted therapies to the pediatric neuro-oncology with high results unreachable by other methods. In the current article we describe the experience of D. Rogachev National Medical Research Center in molecular diagnostics of pediatric brain tumors and targeted therapy in patients with different types of gliomas.
483-491
Primary Immunodeficiency with the PI3K Delta Activation: Clinical Features and Prospective Therapy
Abstract
Primary immunodeficiency (PID) constitute a polymorphic group of genetic life-threatening disorders. APDS (activated phosphoinositide 3-kinase δ) represents a rare PID caused by monoallelic gain of function defects in the PIK3CD gene, or monoallelic loss of function defects in the PIK3R1 and PTEN genes. Disease symptoms usually manifest early in life and include recurrent bacterial infections, non-malignant and malignant lymphoproliferation, persistent herpes virus infections and a whole spectrum of autoimmune manifestations. Immunological features include T and B lymphocytes defects. APDS treatment including immunoglobulin substitution and immunosuppression does not always lead to complete remission of the disease. Hematopoietic stem cell transplantation is a curative option leading to disease resolution in 75–80% of the patients. In 2023 FDA approved leniolisib — selective PI3Kδ inhibitor — for APDS treatment. In the clinical trial the preparation demonstrated safety and efficacy for the patients with APDS ages 12 and above. Approval of the drug created specifically to treat this rare variant of PID opens a new era of the targeted treatment for the patients with this orphan disease.
492-497
Efficacy of Propafenone for Treating Idiopathic Premature Ventricular Contractions in Children
Abstract
Background. Premature ventricular contractions (PVCs) are a common heart rhythm disorder in children, often following a benign course. However, in rare cases, antiarrhythmic therapy is required for managing this arrhythmia. Limited data is available regarding the effectiveness of such therapy specifically in children. Objective. To evaluate the efficacy and safety of propafenone administration in children with idiopathic PVCs. Methods. The study enrolled 33 children aged 11.1 ± 4.8 years (29 days — 17 years) with idiopathic PVCs. Propafenone dose was titrated from initial (5–7 mg/kg/day) to effective (no more than 15 mg/kg/day) dose under clinical and electrocardiographic control to monitor for proarrhythmias and other side effects. An increase in PQ interval duration and a widening of the QRS complex no more than 25% compared to baseline was considered acceptable. Results. The incidence of PVCs before propafenone administration was 36.3 ± 10.1 (22.0–60.6) thousands/day or 33.2 ± 8.4% (20.5–50.0). Complex forms of PVCs were observed in 25 (75.8%) children: 25 (75.8%) had frequent paired PVCs, 6 (18.2%) had polymorphic PVCs, 20 (60.6%) had short runs of ventricular tachycardia (VT), and 19 (27.6%) had a combination of various forms of PVCs. The starting dose of propafenone was 5.6 ± 2.2 mg/kg/day with subsequent increases to 10 ± 0.8 mg/kg/day. Propafenone was employed as the initial therapy in 23 (69.7%) children and as a second line therapy in 10 (30.3%) children, following the ineffectiveness or adverse effects of beta-adrenoblockers. The duration of propafenone therapy was 95.8 ± 62.8 days. Propafenone was effective in 25 (75.8%) children. The average reduction in the number of PVCs during therapy was 71.7 ± 32.6% (13.0–100.0) during therapy. One (3%) child developed proarrhythmia — sustained VT and 1 (3%) child had gastrointestinal side effects. Conclusion. Propafenone is an effective antiarrhythmic drug in children with idiopathic PVCs, including patients with poor response to beta-adrenoblockers. Arrhythmogenic and extracardiac side effects are possible during propafenone therapy, which require clinical and electrocardiographic observation in the process of selecting an effective dose of the drug.
498-504