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Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)

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Vol 68, No 6 (2023)
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LEAD ARTICLE

5-14 720
Abstract

Etiology of autoimmune gastritis, particularly in children, is still unknown. However, the role of Helicobacter pylori and Epstein–Barr virus in the development of autoimmune gastritis is being considered. The formation of autoimmune gastritis is based on an autoimmune reaction mediated by CD4+ T-lymphocytes and the formation of antibodies to gastric parietal cells, the target of which is gastric Н+/К+-АТPase, with subsequent destruction of parietal cells and the development of mucosal atrophy. Autoimmune gastritis is considered a precancerous condition. The clinical picture of autoimmune gastritis in children is not associated with any specific symptoms of the digestive organs. Abdominal pain is uncommon. Specific manifestations of a dyspeptic nature are rare. Often there is a syndrome of chronic nonspecific intoxication. Red blood counts in most children with autoimmune gastritis are within the age norm. Iron deficiency anemia occurs in 13.8% of patients. Vitamin B12 deficiency anemia does not occur in children. Autoantibodies to the parietal cells of the stomach are considered to be a serum marker and diagnostic criterion for autoimmune gastritis in children. Treatment of autoimmune gastritis is aimed at preventing iron and/or vitamin B12 deficiency. No specific methods of treatment have been developed so far. Conclusion. The incidence of autoimmune gastritis in children is underestimated. The role of Helicobacter pylori in autoimmune gastritis has not been confirmed. There is a close correlation of antibodies to gastric parietal cells with Epstein–Barr viral DNA. Due to adverse outcomes and the risk of malignancy, early diagnosis of the disease is important. Atrophic gastritis and intestinal metaplasia are precancerous conditions, although extremely rare in childhood, they should not be neglected.

LITERATURE REVIEWS

15-23 378
Abstract

Congenital anomalies of the kidney and urinary tract (CAKUT) include a wide range of structural anomalies that develop as a result of impaired morphogenesis of the kidneys and/or urinary tract. CAKUT accounts for about 40–50% of patients under 21 with chronic kidney disease worldwide. Most congenital anomalies of the urinary system are diagnosed in utero or during the first months of a child’s life, which determines further tactics for managing a child, depending on the severity of CAKUT and the degree of decrease in the filtration function of the kidneys. The article provides modern data on the causes of the development of anomalies of the urinary system, provides examples of the development of scientific areas that will be aimed at predicting the occurrence of malformations. In addition, early markers of the disease are considered. Thus, the problem of anomalies of the urinary system remains relevant to the present despite the significant contribution of genetics to understanding the pathogenesis and predicting the development of CAKUT.

ORIGINAL ARTICLES

24-30 475
Abstract

Data on the course of pregnancy complicated by SARS-CoV-2 infection, and the condition ofthe fetus and newborn are of great interest to neonatologists and pediatricians.

Purpose. The study aimed at evaluating the clinical and laboratory features of the early neonatal period and individual health indicators in infancy in full-term newborns from mothers with gestational diabetes mellitus and COVID-19 during pregnancy.

Materials and Methods. Two groups of full-term newborns whose mothers’ pregnancy was complicated by gestational diabetes mellitus were compared in matched pairs (each group included 80 patients). In the first group of newborns, COVID-19 was diagnosed in mothers during pregnancy, in the second group, COVID-19 was not observed in mothers. At the age of 9 months, an analysis of outpatient medical records was carried out and a questionnaire survey of mothers was conducted. The methods of collecting and processing information using the Microsoft Excel 2016 software package for Windows 7, IBM SPSS Statistics v22 were used. Results. COVID-19 infection during pregnancy is associated with a higher frequency of respiratory and neurological disorders in the newborn, and with problems of enteral feeding. In the peripheral blood, the indices fell within the limits of reference values, but there was a decrease in leukocytes, platelets and an increase in the fraction of segmented neutrophils. At the age of 9 months, the children of the main group had a larger body weight and head circumference, a higher incidence of chronic morbidity was noted, and the children were registered with a neurologist, cardiologist and endocrinologist.

Conclusions. Clinical and laboratory features of early neonatal adaptation of children from mothers with gestational diabetes mellitus and COVID-19 during pregnancy were revealed. A more severe course of diabetic fetopathy has not been proven in children from women who suffered COVID-19 during pregnancy. The higher incidence of chronic morbidity in infancy requires further study.

31-35 306
Abstract

The high incidence of respiratory viral infections in children of early, preschool, and primary school age is an urgent problem for practical health care.

Purpose. The purpose of the study was to investigate factors contributing to the formation of a group of children who are frequently ill at an early age, born as a result of assisted reproductive technologies.

Materials and Methods. The observation group consisted of 60 frequently ill children of the first three years of life born as a result of assisted reproductive technology application. The comparison group consisted of 22 children of similar age born using assisted reproductive technologies, not belonging to the group of frequently ill.

Results. Among the endogenous risk factors affecting the increased frequency of acute respiratory infections in children born with the help of assisted reproductive technologies, the most significant are the following: intrauterine hypotrophy (p=0.026), early initiation of formula feeding (p=0.003), allergic diseases (p=0.049). Exogenous risk factors for the formation of a group of frequently ill children born with the use of assisted reproductive technologies are the effect of antibiotic therapy on the child’s immune system, the lack of vaccination against hemophilic infection.

Conclusion. Frequently ill children require a comprehensive examination to identify background conditions, timely vaccination, and reasonable prescription of antibiotic therapy.

36-40 799
Abstract

In 10–20% of cases, congenital heart defects are caused by chromosomal abnormalities and gene mutation. Purpose. The purpose of the study: analysis of the genetic causes of congenital heart defects in children.

Materials and Methods. The analysis of the medical history and the results of Genetics and Molecular Research (GMR) in 15 children with congenital heart defects were carried out.

Results. Genome-wide DNA sequencing in patients with congenital heart defects revealed deletion 21q22.3 with duplication 4q31.1 ->qter and pathogenic variants in the genes PPP1CB, FN1, PHF6, CITIED2, ARID2, KMT2A, CIT, JAG1, MED13L, ELN, CHRNA3, KCNK9. Conclusion. In the vast majority of cases in the examined children, congenital heart defects were caused by pathogenic gene variants or chromosomal abnormalities. Congenital heart defects are an absolute indication for cytogenetic and molecular genetic examination.

41-49 505
Abstract

Currently, there is no unified system of anti-relapse treatment of pyelonephritis in children.

Purpose. To assess the feasibility of prescribing and effectiveness of various anti-relapse therapy regimens to prevent exacerbations of the disease in children with chronic secondary pyelonephritis.

Materials and Methods. The study involved 158 children with chronic secondary pyelonephritis aged 2 to 14 years, including 130 (82.3%) girls and 28 boys (17.7%). The children were divided into the following groups: Group A (n=32) — furazidine — 14 days, anti-relapse therapy after 12 months; Group B (n=34) — furazidine — 14 days, then — Canephron 1 month, anti–relapse therapy after 12 months; Group C (n=34) — Canephron 3 months, anti-relapse therapy after 12 months; Group D (n=30) — furazidine — 14 days, then — Canephron 1 month of anti–relapse therapy after 6 months; Group E (n=28) — furazidine — 14 days of anti-relapse therapy was not performed. Results. It was found that the recurrence rate of chronic secondary pyelonephritis for a 24-month follow-up in Group A was 28.1% (n=9), in Group B, 14.7% (n=5), in Group C, 20.5% (n=7), in Group D, 10% (n=3), in Group E, 42.9% (n=12). The timing of relapse of chronic secondary pyelonephritis was different, with the largest increase in the proportion of patients with exacerbations in the period from 6 to 12 months. The average recurrence rate per year in the catamnesis in all children (n=158) was 1.4 [1.2–1.6], during the prospective follow-up, a statistically significant (p=0.001) decrease in the recurrence rate to 0.36 [0.2–0.79] was noted. Conclusion. The data obtained confirm the feasibility of anti-relapse therapy in children with chronic secondary pyelonephritis.

50-60 274
Abstract

Purpose. The study aims at optimizing the diagnosis of acute kidney injury in children with poisoning of chemical etiology based on the use of early markers of kidney damage.

Materials and Methods. The study enrolled 120 patients aged 1–18 years with poisoning of chemical etiology. Routine methods of kidney function estimation were supplemented by an investigation of urinary concentration of acute kidney injury early markers: lipocalin-2, kidney injury molecule-1. All children were hospitalized in a toxicology unit on the 1st day after exposure to toxic substance. Twenty practically healthy children aged 1–18 years were examined as control group.

Results. Only one patient (0.8%) had acute kidney injury determined as increased serum creatinine at the time of admission, but at the same time 35 children (29.2%) had increased levels of one or more urinary markers. By the 3rd day 32 of those 35 children with increased urinary markers developed acute kidney injury stages 1 or 2, determined as significant rise of serum creatinine and decrease of renal blood flow according to Doppler ultrasound. On the contrary, no patient with initially normal level of urinary markers developed acute kidney injury. Thus, 32/120 patients hospitalized with poisoning of chemical etiology, developed acute kidney injury (26.7%).

Conclusion. Increased urinary excretion of lipocalin-2 and kidney injury molecule-1 at the first day of acute chemical poisoning can be estimated as predictive marker of acute kidney injury development.

61-66 335
Abstract

Purpose. The purpose of the publication was to conduct an epidemiological survey for a comprehensive study of the dental status in children with autism spectrum disorder to clarify the areas of medical and social work and the potential scope of dental intervention.

Materials and Methods. We examined 98 children with autism spectrum disorders aged 3 to 7 years, 69 of them with temporary and 29 with mixed occlusion. A dental examination of patients was carried out, the state of oral hygiene, carious defects of the teeth, and the state of periodontal tissues were assessed. Results. It is shown that the state of oral hygiene in children with autism spectrum disorder is unsatisfactory. The need for dental care in 85±5.6% of patients was diagnosed; there was a high prevalence (78.6%) of caries of primary teeth against the background of a significant intensity of caries pathology (the share of the “D” component in the overall structure of caries intensity is 81±3.11%); The prevalence of gingivitis was 61%, and the proportion of children with healthy parodontium was 7.14%.

Conclusion. The results of the study determine the need to provide children with autism spectrum disorders with qualified dental care and its significant volumes. A set of recommended therapeutic and preventive measures has been proposed, including strengthening oral hygiene with particular regard to the specific characteristics of the underlying disease (emphasis on the motivational factor, transferring manual actions to a game format, using gadgets), strengthening pathogenetic therapy with the daily use of remineralizing drugs, the use of special physical therapeutic techniques (programs) in combination with regular examinations at the dentist and an increase in the intensity of preventive measures.

67-74 263
Abstract

The results of a study of the dynamics of ultrasound parameters of blood flow in the eye and orbit in patients with type 1 diabetes mellitus in childhood and adolescence are presented. The study included data from 70 patients aged 5 to 18 years, with disease duration up to 5 years, from 5 to 10 years, and more than 10 years. Ultrasound indicators of linear blood flow velocity and peripheral resistance index were analyzed in the ophthalmic arteries, central arteries and veins of the retina, posterior short ciliary arteries, and superior ophthalmic veins at different periods of the duration of type 1 diabetes mellitus. The study revealed a decrease in blood flow velocity in the central retinal arteries and in the posterior short ciliary arteries with increasing duration of type 1 diabetes. The greatest decrease in blood flow velocity was recorded in the group with a duration of diabetes mellitus of more than 10 years. The data obtained can be used to assess and dynamically monitor early disorders of the blood supply to the eye and orbit in patients with type 1 diabetes mellitus in childhood and adolescence.

75-84 232
Abstract

The formation of taste preferences and diversity of a child’s diet, as well as the prevention of neophobia, are relevant and discussed issues in clinical practice and scientific literature. A group of authors from St. Petersburg and Kazan conducted a multicenter clinical study to study both food tolerance and taste perception of two complementary feeding products based on goat milk (vegetable and vegetable-grain). We studied eating behavior using a questionnaire and physical development in 92 healthy children aged 8 to 11 months. Statistical processing of the material was carried out using the IBM Stata 12 application package. The assessment of food tolerance showed good results for all products studied. The physical development of children in both regions met the standards both at the beginning of testing and at the end. At the same time, taste perception had regional characteristics, depended on the place of residence of the children and, probably, on the mother’s food preferences during pregnancy for a variety of vegetable and vegetable-grain components of dishes. It should be taken into account when expanding the infant’s diet.

CLINICAL CASES

85-93 361
Abstract

The use of new psychoactive substances (NPS) is increasing every year among various social groups around the world. The use of narcotic drugs during pregnancy, according to various authors, remains at a high level from 2.8% to 7% of pregnant women. The use of new psychoactive substances during pregnancy is characterized by multi-organ damage to the fetus, like other groups of narcotic drugs, however, no organ and tissue specificity for new psychoactive substances has been described. Experimental studies have demonstrated negative pathophysiological mechanisms triggered by synthetic cathinones in the form of increased proapoptotic activity, the formation of autophagolysosomes and reactive oxygen species, and the pro-inflammatory orientation of the immune system. A clinical observation describes a predominant lesion of the nervous system (malformations of the brain, damage to the respiratory center) and the musculoskeletal system (pronounced myotonic syndrome, congenital pathological fractures of the femur), which leads to multiorgan dysfunction, an uncontrolled inflammatory response, and, as a result, to the development of severe disability in such children and growth of the cohort of palliative pediatric patients. In the presented literature review, the authors focus on the pathophysiological mechanisms of action of NPS for a better, deeper and more holistic understanding of the pathological process occurring in the body, in order to form and improve the clinical thinking of medical specialists and cite their own clinical observation as an illustration of the consequences of using new psychoactive substances during pregnancy. The authors believe that this review of the literature with a description of a clinical case is valuable in terms of practical applicability, both for clinicians in various fields and for researchers.

94-98 454
Abstract

Purpose. The purpose of the study is to show the importance of a multidisciplinary approach in the early verification of the primary immunodeficiency — Nijmegen breakage syndrome. A girl was admitted with complaints of daily productive cough, persistent obstruction of nasal breathing, mucopurulent discharge, recurrent respiratory tract infections for three years. From the age of 2, she suffered from protracted recurrent respiratory infections, repeatedly received inpatient treatment with systemic antibiotic therapy with insufficient effect in the form of maintaining complaints. When examining according to CT scan of the chest — single atelectasis, ground glass areas. Immunodeficiency states were excluded at the place of residence. On examination: phenotypic features of the type of «bird-like» face — sloping forehead, middle part of the face protruding forward, beak-shaped nose, large ears. Physical development is low: weight and height indicators are less than the 3rd percentile. From the nasal passages mucopurulent discharge, productive cough. Auscultatory — moist rales in all lung fields. For other organs and systems — without features. On examination: deep T-cell lymphopenia, according to CT scan signs of pansinusitis, bilateral otitis media; post-inflammatory pneumofibrotic changes. According to the results ofspirometry — mixed lesions. According to the results of immunological and genetic additional examination, the immunodeficiency state — Nijmegen breakage syndrome was verified, constant anti-inflammatory, antibacterial and antifungal, immunoglobulin replacement therapy was initiated.

Conclusion. The presented clinical observation reflects the features of the course of the Nijmegen breakage syndrome. This example demonstrates the diagnostic complexity in verifying the diagnosis and shows the great importance of a multidisciplinary approach for the timely initiation of appropriate therapy, which, in turn, affects the severity of the disease and quality of life.

99-104 379
Abstract

Tuberous sclerosis complex is an autosomal dominant hereditary disease characterized by the formation of multiple hamartomas in various organs and tissues. Although tuberous sclerosis is considered to be a rare condition, it is among the most common genetic diseases. According to the literature, 16 cases of tuberous sclerosis associated with congenital lymphedema have been revealed from 1984 in scientific publications. Only four of these cases were described in male patients. Such combinations have not been yet described in the Russian studies. The article discusses different aspects of a rare clinical case presented by the combination of tuberous sclerosis with congenital lymphedema in a male patient aged 1 year and 6 months.

105-111 287
Abstract

The article presents an analysis of the pathogenesis of neurofibromatosis type 1, the mechanism of damage to the central nervous system. It analyzes the general clinical symptoms of neurofibromatosis type 1, its diagnostic criteria, describes the specifics of cognitive development in this disease with an emphasis on behavioral and autism spectrum disorders. The authors describe a clinical case of neurofibromatosis type 1 (a boy, 6 years and 10 months), in which, along with coffee-colored skin spots, subcutaneous fibromas, one of the first symptoms of the disease was also a congenital false joint of the bones of the left leg. Absence of speech development and autism spectrum disorders are key problems in the cognitive status of the child.

112-120 916
Abstract

ANCA-associated vasculitis is a rare disease in children characterized by multiple organ involvement. In recent years, there has been found a connection between systemic vasculitis, including ANCA-associated, and the new coronavirus infection. There are certain difficulties in the differential diagnosis of these conditions. In addition, patients with ANCA-associated vasculitis have a high incidence of venous thromboembolic complications, which creates additional difficulties in the diagnosis and treatment of this disease, especially in children. The article presents the current data review and a clinical case of ANCA-associated systemic vasculitis with damage to the kidneys, lungs, joints (microscopic polyangiitis) in a 17-year-old teenager after the coronavirus infection.

121-125 435
Abstract

Cor Triatriatum is utterly rare congenital heart disease, which has characteristic magnetic resonance and echocardiographic symptoms. Complexity of the diagnostics is an asymptomatic clinical flow, so timely diagnostics and therapy will help to defer possible complications and improve the quality of the life of these patients.

SHARING EXPERIENCES

126-131 314
Abstract

Article retracted

Millions of babies are admitted to neonatal intensive care units worldwide, with a significant proportion facing various indications. Given that sepsis is a leading cause of mortality and morbidity among infants globally, including in India, the importance of efficient antibiotic use cannot be overstated. As the global problem of drug resistance intensifies, prudent antibiotic usage becomes crucial in mitigating treatment failures and ensuring the survival of newborns. Aim: to evaluate the pattern of antimicrobial drug utilization in neonatal intensive care unit (NICU) to assess the criteria for drug selection in tertiary care hospital.

Materials and Methods. It was an observational prospective study conducted for six months in which 200 neonates admitted to neonatal intensive care unit at tertiary care Hospital, were included. Demographic details, duration of hospital stay, reason for admission (disease pattern), various drugs prescribed were noted. Results. Out of 200 neonates the number of male babies admitted to neonatal intensive care unit were 115 (57.5%) and female babies were 85 (42.5%). Many of the neonates were from age group of 1 to 5 (58.5%) days. Most of the neonates were from weight group of 2.5 to 3 (26.5%) kg. Majority of neonates hospital admission duration was 3 to 6 days is 98 (49%) neonates. Most common disorder requiring neonatal intensive care unit admission was Preterm with Respiratory Distress Syndrome (29%) followed by Neonatal Sepsis (19.5%) and Preterm (11%). Other distress reported include birth asphyxia (16 neonates or 8%), Neonatal convulsions (12 neonates or 6%), Late preterm (13 neonates or 6.5%), Hypernatremia (9 neonates or 4.5%), Meconium Aspiration Syndrome (3 neonates or 1.5%), Neonatal Hypoglycemia (5 neonates or 2.5%) and others (23 neonates or 11.5%). Among 423 antibiotics most prescribed antibiotic was Inj. Piperacillin + Tazobactam (35.4%) followed by Gentamycin (30.2%). Out of 423 drugs 152 are fixed dose combination and single drugs are 271 in this study in which piperacillin + tazobactam of fixed dose combination and gentamicin dose combination and single drugs are 271 in this study in which piperacillin + tazobactam of fixed dose combination and gentamicin of single drug had prescribed in more amount.

Conclusion. The findings of this study will contribute to the existing body of knowledge regarding the appropriate use of antibiotics in the NICU, thereby providing insights for healthcare professionals to make informed decisions regarding antibiotic therapy. Empirical antibiotics were administered to every newborn based on their individual conditions. The antibiotic utilization pattern within this study group was determined to be rational.

TO THE PRACTITIONER ASSISTANCE

132-136 226
Abstract

The article presents algorithm for early diagnosis and prevention of sick sinus syndrome in children considering the degree and stability of bradycardia that demonstrated management of patient during the progression of sick sinus syndrome.

137-143 357
Abstract

The paper gives the data available in the literature, diagnostic issues and current treatments a rare genetic syndrome in children — autoimmune polyglandular syndrome type 1. Emphasis is laid on major difficulties in the differential and true diagnosis of this disease. The author describes a clinical case. The article presents the importance of the interdisciplinary approach.

HISTORY OF PEDIATRICS

144-147 263
Abstract

The article is devoted to the life and scientific career of Margarita Vladimirovna Zhilinskaya, Doctor of Medical Sciences, Professor, head of the first department of computer diagnostics in pediatrics in Russia and the USSR created in 1970 on her initiative. For 5 years before that, she was the Chief Freelance Pediatrician of the Ministry of Health of the RSFSR. The new area of activity was a continuation of research on the application of mathematical methods in clinical work. The department actively developed and implemented methods for planning research, assessing the informativeness of signs, multiple connections of signs in the analysis of the pathogenesis of diseases, for which the method of correlation pleiades was developed. The main paradigm in as early as the beginning of the department’s activity was a comprehensive quantitative assessment of the conditions of the child’s organism, mathematical analysis of risk factors and the formation of risk groups for diseases in children on this basis. At the same time, computer programs were created for differential diagnosis and evaluation of treatment effectiveness. Employees of the department conducted comprehensive research in the field of developing computer systems for rapid and in-depth analysis of infant mortality. For the first time, a mathematical apparatus was implemented to quantify the contribution of qualitative characteristics to the outcome of the disease. Research conducted under the scientific guidance of M.V. Zhilinskaya in 1970–1979 laid the ground for the subsequent creation of an automated system for clinical examination of pediatric population and numerous systems for disease diagnosing.

INFORMATION



ISSN 1027-4065 (Print)
ISSN 2500-2228 (Online)