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

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Vol 64, No 4 (2019)
View or download the full issue PDF (Russian)
https://doi.org/10.21508/1027-4065-2019-64-4

LEAD ARTICLE

6-10 1298
Abstract
The article discusses the current state of the medical care for fetuses, newborns and children with cardiac rhythm and conduction disturbances. The authors discuss the features of prenatal diagnostics of arrhythmias and management of pregnancy and childbirth of women with this pathology of the fetus. They analyze the diagnostics and treatment issues of arrhythmias in children. The article substantiates the introduction of the perinatal cardiology concept and formulates the main issues of this stage of development.

REVIEWS

11-18 1146
Abstract
Genetic aspects regulate the intensity and rate of aging (no toxic effects considered), their negative role depends on the pathogenicity of the mutation. The light variant of the genetic “defect” has no clinical signs which feature a certain known genetic syndrome, but it has the biochemical, immunological, vascular and other abnormalities leading to pathological aging. In the most severe case, e.g. progeria, pathological aging is the main phenotypic symptom that manifests already in childhood. The subject of the pathological aging research covers the whole range of intermediate states. The review focuses on aging in individuals without validated signs of disease: coronary heart disease, hypertension, diabetes or fasting hyperglycemia, hyperlipidemia, and others. The authors present the main searching directions of aging biomarkers (size and speed of telomere shortening, breaks in their terminal loops; expression of inflammatory proteins, synaptic interactions proteins and neurotrophic processes; mitochondrial biogenesis; endothelial dysfunction; DNA methylation activity).
19-25 1082
Abstract
The authors present a review of the literature devoted to the problem of programming the formation of the cardiovascular system structure and function in children born from mothers with preeclampsia. These children are at high risk of developing cardiovascular diseases. Pre-eclampsia is caused by the endothelium dysfunction, deregulation of the immune and inflammatory factors during pregnancy. Experimental studies identify these factors as key epigenetic factors programming the condition of the cardiovascular system of the offspring. The modern concept of intrauterine programming, describing this phenomenon, focuses on three main areas of research: experimental models simulating the intrauterine environment with preeclampsia; research of the pathological phenotype formation under the influence of these factors; epigenetic studies of the influence of preeclampsia on the cardiovascular system functioning. The article discusses the perspectives of epigenetic programming prevention.

ORIGINAL ARTICLES

26-34 1064
Abstract

The article presents the results of intravenous transplantation of allogeneic multipotent mesenchymal stromal cells, derived from a human umbilical cord, to a child with Crigler–Najjarsyndrome type I during the first 2 years of life. The therapy is aimed at reduction of the duration of phototherapy while maintaining a safe level of serum bilirubin.

In this study, a five-day-old child with the bilirubin level of 340 µmol/l was treated with phototherapy for 16–18 hours daily in the neonatal period. Then, phototherapy was reduced to 14–16 hours. The level of bilirubin varied from 329 to 407 μmol/l. At the age of 2 months, it was decided to use multipotent mesenchymal stromal cells with a significant decrease in the duration of phototherapy up to 2 hours a day. During the observation period (2 years at the time of writing this article) the child received 6 injections of multipotent mesenchymal stromal cells. A positive effect developed within 4–7 days after administration and persisted for 2–3 months. There were no side effects or complications during and after transplantation.

Thus, intravenous transplantation of multipotent mesenchymal stromal cells is an effective treatment of Crigler–Najjar syndrome type I; it reducesthe need for phototherapy,significantly improvesthe quality of life of the patients and prolongstheir life with native liver. 

35-37 992
Abstract
The purpose of this work was to determine the features of the state of newborns of underage mothers. The children of minor pregnant women were examined (n=483), who were observed and gave birth to the Maternity Hospital No. 10 in St. Petersburg in 2004–2014. [1 group – 13–15 years old (n=49); 2 group – 16–17 years old (n=434)]. The comparison group consisted of 110 children of women of medium reproductive age. Significant differences in the anthropometric characteristics of newborns between groups were not found. It was shown that in children of minors of both age groups the hypotrophy of the newborn was significantly more frequently observed, as well as inflammatory diseases specific to the perinatal period (neonatal infection of the skin, conjunctivitis). Minors 13–15 years old were significantly more likely than 16–17 year olds and women of medium reproductive age to have asphyxia during labor and cephalhematoma.
38-44 1460
Abstract
Psychological features of mothers who have given birth to premature babies present one of the most complex and insufficiently explored areas of modern perinatal psychology. The progressive increase of the children born before the term as well as the insufficient development of psychological care programs for mothers at the women's consultation and nursing departments of newborns dictate the urgency and necessity of this problem. Using psychological techniques, the authors comprehensively assessed the personality characteristics, motives for the pregnancy preservation, personal and situational anxiety of mothers of premature babies at Perinatal Center №.1. The assessment revealed that the mothers with premature low-birth-weight babies experienced a sharp increase in situational anxiety compared to personal anxiety, while the mothers of full-term babies with a normal weight tended to decrease the level ofsituational anxiety. In addition, the mothers of premature babiessufferfrom expressed and severe depression more often than the mothers of full-term babies(p<0.05).
45-51 1128
Abstract
We studied the concentration of carnitine and its fractions in peripheral blood in 22 premature infants with fan-associated pneumonia and in 20 conditionally healthy premature infants by tandem mass spectrometry. The birth body weight of children was 2,086.32 ± 117.13 and 2,140.9 ± 74.4 g, gestational age was 33.7 ± 0.41 and 34.16 ± 0.51 weeks, respectively. The concentration of  total carnitine was at the lower limit or decreased in 10 (45%) children in the acute period of the disease. 4 newborns with fan-associated pneumonia demonstrated persistent carnitine deficiency: the content of free carnitine was very low: 7.47 – 8, 37 μmol/l (7.97 ± 0.197 μmol/l), the concentration of total carnitine was also reduced (21.55 – 22.01 μmol/l, 21.7 ± 0.366 μmol/l). The fractions of acylcarnitines varied widely throughout the disease. One child had high rates of C18OH (0.282 μmol/l; norm 0–0.110 μmol/l) and C18:1OH (0.282 μmol/l; norm 0–0.180 μmol/l) during the entire neonatal period. It could be associated with mitochondrial trifunctional protein deficiency. The study of total carnitine and its fractions in premature infants with fan-associated pneumonia allowed us to identify violations of its metabolism, both secondary and hereditary at early stage.
52-59 1612
Abstract

We studied the autopsy material obtained from 7 children who died in the neonatal period in order to evaluate the composition of lymphocytes of the intestinal mucosa against the background of morphological changes in the tissues of the gastrointestinal tract in newborns with sepsis. The main group consisted of 4 children with neonatal sepsis, the control group – of 3 newborns who died from other causes. The research material included the specimen of the small and large intestine.

Results. Small intestine: it was found that there were less CD4 + lymphocytes in the small intestinal mucosa in the group of children who died from neonatal sepsis in 75% of cases than in the control group, but this difference was not statistically significant (p=0.1). There were no differences in the number of CD8 + and CD20 + cells in the studied groups. Large intestine: the number of CD4 + lymphocytes of the mucous membrane of the colon was greater in the main group of children than in the control group (p=0.03). An increase in the number of CD4 + cells was registered in 3 of 4 cases of neonatal sepsis. The number of CD8+ and CD20+ lymphocytes in the studied groups was the same (р>0.05).

Conclusion. The increase in T-lymphocytes CD4+ in the mucous membrane of the large intestine is probably connected with the antigenic stimulation of opportunistic intestinal bacteria. We found no morphological signs of the suppression of the cells of adaptive immunity associated with the intestinal mucosa. 

60-64 1872
Abstract

Objective. To analyze the influence of clinical and biochemical markers on the diagnostic algorithm for hypoxic syndrome in children with genetic diseases of connective tissue.

Characteristics of children and methods. There were examined 60 children were examined: 50 children with Ehlers–Danlossyndrome and 10 children with Marfan syndrome.

Results. Constant fatigue, fast fatigue, low tolerance to physical exercises, pale skin, muscle hypotonia and headache are the most frequent symptoms of a hypoxic state in children with connective tissue dysfunction. At the biochemical level, hypoxic syndrome was characterized by signs of oxidative stress. The authors noted an decrease in pO2 , an increase in pCO2 in the circulating blood, an increase in the activity of anaerobic glycolysis with accumulation of lactate and pyruvate, a decrease in the synthesis of macroergs (ATP, ADP, AMP) and the activity of the mitochondrial respiratory enzymes, the emersion of mitochondrial insufficiency in the form of ripped red fibers (RRF) in myocytes , conglomerates of lipids, calcium, glycogen. There was an increased content of peroxidation products in the blood, urine and saliva.

Conclusion. Periodic use of antihypoxants, drugs with detoxifying and immunomodulatory effect is justified for the treatment of children. 

65-69 3138
Abstract

Objective. To evaluate the effectiveness of the natural terpenoids complex in preschool children with recurrent acute respiratory infections (ARI).

Material and methods. There were examined 7129 children of 3-7 years old, they were divided into 3 groups. In main group (n = 2400) there was endonasally (on the mucous membrane of the nasal cavity with cotton buds) used a complex of natural terpenoids 2 times a day (morning and evening) 5 days a week for 1 - 3 months. Prevention and treatment of children from the comparison group (n = 2,379) was carried out with dioxotetrahydroxy tetrahydronaphthalene. The control group consisted of children (n = 2,350), who received no therapy. Upon completion of the course of therapy, children were examined in dynamics.

Results. The authors noted positive clinical effect of the natural terpenoid complex, as evidenced by a 1.6 times decrease in acute respiratory infections during the year and the duration of the acute episode of the disease by 2.5 days, as well as a positive effect on the elimination of Epstein Barr virusin frequently ill children when using drug for 65.51 ± 2.98 days. The authors observed a favorable clinical effect against the decrease in neutrophils and eosinophils in the rhinocytogram, as well as mixed flora and cocci, indicating a decrease in mucosal neutrophilic inflammation and prevention of bacterial complications. 

70-75 1030
Abstract
В статье представлен анализ клинико-диагностических особенностей коклюша у детей старшего возраста. Удельный вес данной возрастной группы среди госпитализированных составлял 11,3±2,7–13,3±2,7% с преобладанием привитых против этой инфекции больных. У 93,6±3,6% пациентов заболевание протекало в среднетяжелой форме с типичным симптомокомплексом. Для верификации клинического диагноза был использован метод полимеразной цепной реакции (ПЦР) и серологический метод – иммуноферментный анализ (ИФА). Эффективность метода ПЦР составляла 87,8%. Особая диагностическая ценность метода ИФА определялась возможностью подтвердить диагноз коклюша у привитых детей и в поздние сроки болезни, что ранее представляло большие трудности. Основным серологическим критерием служило выявление ассоциации высокого уровня продукции иммуноглобулинов IgG и IgA.
76-82 956
Abstract
The aim of the study was to increase the level of diagnosis and treatment of chronic recurrent aphthous stomatitis in children with rheumatic diseases. We carried out a dental examination and treatment of 316 children with rheumatic diseases, 67 of them had chronic recurrent aphthous stomatitis. In the course of the study the authors determined and analyzed the etiological, pathogenetic and clinical features of the course of chronic recurrent aphthous stomatitis in children with rheumatic diseases. The study describes the examples of aphthousstomatitis of varying severity. Chronic recurrent aphthousstomatitisis an integralsymptom of a somatic disease, itstreatment requires the interaction of many specialists: dentist, pediatrician, rheumatologist, gastroenterologist, otolaryngologist, etc.

CLINICAL CASES

83-87 8451
Abstract
Hamman–Rich syndrome (idiopathic fibrosing alveolitis) is a lung disease of unknown nature, characterized by non-infectious inflammation of the interstitium, alveoli and terminal bronchioles with an outcome in progressive pulmonary fibrosis. Idiopathic fibrosing alveolitis in young children, despite the timely verification of the diagnosis and therapy, is characterized by an unfavorable prognosis and rapidly progressive course. The article describes the clinical case of Hamman–Rich syndrome in a child of 8 months, its main clinical manifestations and diagnostic features.
88-91 877
Abstract
Diabetes mellitus type 1 is one of the most common chronic diseases among children. It is a pathology of carbohydrate, fat and protein metabolism caused by insulin deficiency in the target tissue. Changes in the lipid profile can be both secondary and primary. The article describes a clinical case of a combination of diabetes mellitus type 1 and family hypercholesterolemia in a child. It presents the clinical and laboratory results of the examination of the child and his parents. Familial hypercholesterolemia is confirmed by mutation of the low density lipoprotein receptor gene (LDLR) in the heterozygous state. In addition, the proband has a polymorphism of the SLCO1B1 gene, which should be considered when prescribing therapy. The demonstrated clinical case shows that familial hypercholesterolemia can be an independent disease, aggravating the course of diabetes mellitus and requiring correction of the therapy.

SHARING EXPERIENCES

92-98 841
Abstract

The purpose of the study is to analyze the history and structure of the pathology of premature infants with extremely low body weight and very low body weight under dynamic follow up in the monitoring department for children of the perinatal risk group. Using the method of continuous sampling we analyzed observation maps of 95 premature infants with extremely low body weight and very low body weight of gestation age from 24 to 36 weeks.

All newborns have a low Apgar score at birth and at the 5th minute, and Apgar score was lower in  children with extremely low body weight as compared with children with very low body weight. All children had congenital anomalies of the organs and systems, marked signs of immaturity, with prevailing changes in  the cardiovascular system, passability of  the renal pelvis, and congenital deformity of thigh.

Among the conditions that occurred in the perinatal period, the authors diagnosed intraventricular hemorrhage in 50.5% of patients, 89.6% of them had hemorrhage of grade II – III, all children had respiratory distress syndrome. Bronchopulmonary dysplasia was diagnosed in 60% of premature infants, its moderate and severe forms in 1/3 of the patients. All children had anemia, and 13.7% of children had a neonatal syndrome from mothers with diabetes mellitus. 1/3 of premature infants had retinopathy; its frequency did not depend on the body weight at birth. All children had a combination of pathologies. Bronchopulmonary dysplasia was combined with respiratory distress syndrome and anemia in 100% of children. The combination of bronchopulmonary dysplasia with intraventricular hemorrhage was more often recorded in patients with extremely low body weight than in patients with very low body weight. 

99-104 1034
Abstract
Organizing an individual bank of breast milk helps to preserve an alternative possibility to continue breast-feeding of a newborn baby with pumped and (or) frozen mother’s milk. Pumped milk (milk defrosted not later than 3–6 months) is microbiologically safe food for a baby. Frozen pumped milk is much better than mixtures for babies. Sanitary Regulations and Standards 2.1.3.2630.-10 clause 3.5 are to be updated to reflect the technology of breast-feeding preservation forsick babies who do not have breast-feeding since the first days of life.
105-113 2034
Abstract

Acute respiratory infections (ARI) are major causes of morbidity in children. Symptomatic treatment is insufficiently effective and requires additional drugs with etiotropic action. This multi-center double blind placebo-controlled randomized clinical study with superiority model design considers a liquid formulation of Anaferon for children.

Methods. The study included 142 children from 1 month to 3 years 11 month 29 days old with ARI of the upper respiratory tract. They were randomized into Anaferon for children and Placebo groups (71 in each group) via interactive voice randomization system. The treatment period was 5 days, observation period – 14 days. The average duration of the symptoms and the severity of the respiratory disease were used as primary efficacy endpoints.

Results. All 142 patients were randomized and included into ITT (Intention to treat) analysis. 140 patients (70 patients in each group) were included into PP-analysis. The patients in the Anaferon for children group had better results with average duration of ARI as compared to the Placebo group (87.7±31.5h vs. 103.3±19.4h; p =0.007). The authors marked efficacy of Anaferon for children in such parameters as disease severity (ITT-analysis: p=0.0004), total severity of disease symptoms (ITT-analysis: ANOVA: “Group” factor p=0.0004; “Day” factor p<0.0001), and percentage of recovered patients (Log-rank test, p=0.035). There were no discrepancies between the groups in concomitant therapy (ITT-analysis: ANOVA “Group-Day” factor p=0.88), bacterial complications frequency (p=1.0), adverse effects (Fisher criteria: p=0.5321). The authors registered 14 cases of adverse events (10 cases in Anaferon group and 4 cases in Placebo group) in 11 patients. None of the adverse events had a certain or probable relation to the studied drug.

Conclusion. Anaferon for children proved their efficiency and safety in young children with ARI. 

TO THE PRACTITIONER ASSISTANCE

114-127 1332
Abstract
The article analyzes clinical guidelines oftheAmericanAcademy ofPediatrics dd 2017 “ClinicalPractice Guideline forScreening andManagement of High Blood Pressure in Children and Adolescents”. This document contains new values of blood pressure in children, replaces the term “prehypertension” with the term “elevated blood pressure”, provides a simplified classification of arterial hypertension in adolescents over 13 years and revisesthe guidelinesfor daily blood pressure monitoring and echocardiography. The documentspecifiesthe criteria for diagnosing increased body weight of the left ventricular myocardium, changesthe target blood pressure levels. This new guidelines helps to optimize the diagnostics and treatment of hypertension in children. At the same time there is need for further comprehensive analysis of clinicalrecommendations and assessment of their practicalsignificance for pediatrics.
128-132 2261
Abstract
The article summarizes the modern data on the role of leukotrienes in the pathogenesis of allergic diseases of the respiratory tract, in particular, bronchial asthma in children. It determines the role of anti-leukotriene drugs in the treatment of these diseases, the perspectives and key indications for the use of Montelukast in children with bronchial asthma and allergic rhinitis. The article pays attention to highly effective generics, in particular Singlon®.
133-140 949
Abstract
Dairy products are the most popular and important in their nutritional value products in the diet of adults and children. There are about 400 known types of these products in the world. Traditional dairy products are widely used both in daily diet and complex treatment of various infectious and non-infectious diseases. The probiotic and prebiotic components in their composition makes these products functional, since their regular use has a positive effect on the digestive processes, immune system, and the indicators of the physical development of young children. The surveys demonstrated the effectiveness of functional dairy products both in healthy children for the prevention of diseases and in children with various pathologies in the complex therapy.

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