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

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Vol 65, No 5 (2020)
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https://doi.org/10.21508/1027-4065-2020-65-5

LEAD ARTICLE

7-11 1556
Abstract

Congenital malformations are the main cause of infant and child morbidity and mortality worldwide. Most congenital malformations are represented by non-syndromic forms, their etiology has not yet been studied. However, the latest genetic technologies have opened up new possibilities in the study of congenital malformations. The article presents latest achievements in the genetics of non-syndromic forms of congenital malformations. Genome-wide association study (GWAS) is an effective method for identifying gene variants associated with a predisposition to the congenital malformations; this method helps to identify correlations between single nucleotide polymorphism (SNP) and certain malformations. Numerous studies demonstrate that in addition to SNP the copy number variations (CNV) play an important role in the etiology of some birth defects (for example, congenital heart defects). It has been established that 5—10% of isolated congenital heart defects can be associated with rare CNV. Next-generation sequencing (NGS) is expected to play important role in the identification of birth defect etiology. The authors have obtained the first data on the genes involved in the development of malformations such as congenital heart defects, neural tube defects, facial clefts.

REVIEWS

12-22 2009
Abstract

Stress is the response of the organism to various external and internal events. All response reactions change from metabolic processes to metabolic stresses in minutes or even seconds. The scientists most often discuss oxidative, nitrosative and carbonyl stresses which are characterized by the accumulation of free radicals and other reactive oxygen species, as well as active carbonyl compounds, in the cells and extracellular fluid. These active (signal) molecules are powerful nonspecific modifiers of the structure and function of proteins, lipids, carbohydrates, and they interfere with bioenergetics. Small doses of active signal molecules are necessary for adaptive reactions of the body, they inhibit metabolic disorders, especially protein disorders, but their excessive accumulation causes pathological processes with pronounced modification of proteins and cardiovascular, neurodegenerative, autoimmune, connective tissue diseases and cancer. The authors discuss possible protection and prevention measures of metabolic stress.

23-30 878
Abstract

Regular physical activity improves health, prevents non-communicable diseases and improves the quality of life. Physical activity for patients with diabetes is one of the factors which complement the treatment of the disease, but the patient should be aware of individual load reactions, the need for constant blood glucose control, compliance with insulin administration and nutrition regulations. The article contains specific recommendations for children and adolescents.

31-36 963
Abstract

Worldwide there is a tendency of increasing acute systemic allergic reactions, especially those associated with food allergies. Numerous studies have revealed a relationship between the prevalence of anaphylaxis in the population and the geographical location (latitude) of the patient. This fact produced the hypothesis of an association between the anaphylaxis frequency reduction due to the higher contents of vitamin D. At the moment the role of vitamin D has not been fully studied in the development of allergic diseases. The effectiveness of additional vitamin D in the prevention and treatment of atopic dermatitis and bronchial asthma remains debatable. The article analyzes the data of possible association between vitamin D and anaphylaxis.

ORIGINAL ARTICLES

37-41 1485
Abstract

Objective. To assess the discriminatory ability of the PELOD 2 and pSOFA scales in the conditions of multidisciplinary neonatal intensive care unit.

Material and methods. It is a prospective, observational, single-center study. The end points are to assess the severity of the condition on the PELOD 2 and pSOFA scales; 28-day survival. The study includes 121 patients.

Results. While studying the discriminating power of the scales we have found that all scales have good model quality. At the same time, the pSOFA evaluation system has a statistically more significant (p<0.05) area under the ROC curve (0.903±0.09). Our analysis has not found significant differences in the characteristics of surrogate endpoints between the compared scales in the conditions of comparable points of separation of death risks (p<0.05).

Conclusion. The high discrimination ability of the pSOFA scale allows us to recommend it for clinical use in neonatal intensive care units.

42-46 1337
Abstract

The study aims to establish the role of polymorphism of genes encoding antioxidant enzymes in the development of CNS pathology in extremely premature newborns. In 72 examined newborns we determined the total antioxidant ability and allelic polymorphism of the sod2 and GCLC genes. During the work carried out, we were able to identify that the existence of the C/T genotype of С60Т polymorphism of the sod 2 gene in extremely premature newborns is a risk factor for the development of ischemic CNS injury. The findings broaden the understanding of the role of the antioxidant mechanism in protecting the CNS of extremely premature newborns and are the subject of further studies.

47-53 929
Abstract

Objective. To study the polymorphism of HLA system genes of II class (DRB1, DQA1, DQB1) in extremely preterm infants with birth weight less than l500g with congenital pneumonia, and to determine the risk factors for the formation of this disease.

Material and methods. The researchers carried out a comprehensive study of 103 newborns. Group I: children with clinical and laboratory signs of congenital pneumonia («=61), and Group II: children with respiratory distress syndrome and without congenital pneumonia («=42). Genomic DNA was isolated from lymphocytes of venous blood and buccal scraping epithelial cells. The HLA class II genes were analyzed using a classical polymerase chain reaction.

Results. The group of children with congenital pneumonia revealed an increase in the frequency of alleles DRB1*04 and DRB1*15, together with DQB1 0302/0602 genotype, thus, these genes may have the predisposing effect to the development of this disease and may serve a molecular genetic predictor of this disease. The genotype of preterm newborns with congenital pneumonia included the DRB1*13, DQA1*0103, DQB1*0501, DRB1*13/13; DQA1*0101/0103; DQB1*0501/0602; GSTM1 +/+ GSTT1 +/+ DRB1*13 DQA1*0501 DQB1*0301, alleles less frequently, those alleles had a protective effect against the development of the lung tissue inflammation. We found no statistically significant differences in the frequency of low-functional alleles of the glutatitone-S-trans-ferase family genes (GSTM1 and GStTi).

Conclusion. The results of the study can be used to personalize the treatment and diagnostic process for preterm infants.

54-57 847
Abstract

The article presents the results of clinical, molecular genetic data on the role of polymorphism of renin-angiotensin system genes ADD1, AGT, AGTR1, AGTR2, CYP11B2, GNB3, NOS3 in children with essential hypertension, taking into account the results of 24-hour blood pressure monitoring. The main Group consisted of 16 children (13 boys and 3 girls) aged from 13 to 17 years with essential arterial hypertension. The results of the study indicate a significant contribution of genetic factors to the development and progression of arterial hypertension in children.

CLINICAL CASES

58-65 1324
Abstract

In most cases children have a light form of new coronavirus infection COVID-19 as compared to adult patients; however, some children may develop severe forms of the disease. Children account for 1—11% of the confirmed patients, less than 1% of the patients have severe and extremely severe forms. The article describes a clinical case of a teenager with a severe course of COVID-19, characterized by the mismatch of physical data and a large degree of lung tissue damage, the development of hemorrhagic syndrome, low effectiveness of antibacterial and antiviral therapy, requiring Tocilizumab.

66-72 866
Abstract

To exchange experience, the article describes an interesting clinical case of coronavirus pneumonia in a pediatric patient with diabetes mellitus. This case deserves the attention of pediatricians and anesthetists. A teenager with diabetes mellitus had the clinical and laboratory signs of coronavirus pneumonia caused by COVID-19. The Protocol for the diagnosis, management and treatment of pediatric patients with COVID-19 written by a group of authors from Slovakia, approved by the Slovak Pediatric Society played an important role in the diagnosis and treatment of this patient. The Protocol describes an important role of computed tomography of the lungs in the timely diagnosis of coronavirus disease, since it is not always possible to diagnose viral pneumonia via X-rays. The authors describe the successful experience of intelligent ventilation mode of Adaptive Support Ventilation (ASV) — from intubation to extu-bation. For viral pneumonia, we recommend using the ASV mode to achieve protective ventilation and quickly wean the patient from the ventilator, to avoid complications.

73-82 980
Abstract

Galactosemia is a hereditary disease characterized by impaired galactose metabolism. A newborn fed by breast milk or an adapted infant formula quickly develop clinical manifestations of the disease associated with accumulation of galactose and its toxic metabolites in the body. Neonatal screening is currently used for early diagnosis of galactosemia and prevention of life-threatening complications. However, this disease may have a crisis course with rapid progression of the symptoms, thus requiring a multidisciplinary approach in the diagnosis and therapy, as well as doctors’ alertness while expecting for screening results in preterm babies. This article presents up-to-date literature data and clinical observation of the preterm newborn with a severe course of the classical galactosemia.

83-86 2757
Abstract

Holt—Oram syndrome is a rare genetic disease characterized by an abnormality of the upper limb, congenital heart disease and / or conduction abnormalities. The disease is caused by the mutations in the Tbox5 gene (allocation 12q24.21), inherited in an autosomal dominant manner. Heart septal defects and isolated thenar hypoplasia are typical congenital malformations. The article describes a clinical case of a 7-month-old girl with a family history of Holt—Oram syndrome: the absence of the first metacarpal bone of the left hand and multiple heart defects (atrial septal defect, multiple defects of the ventricular septum of the Swiss cheese type, aortic valve stenosis). The authors present a detailed clinical diagnosis of Holt—Oram syndrome, as well as genetic analysis and genetic testing of the child and immediate relatives.

87-92 875
Abstract

According to different authors, the osteoarticular syndrome is observed in every third patient with acute leukemia. The polymorphism of the clinical manifestations presents a diagnostic difficulty, while the abnormal x-ray findings are not traditional characteristics of patients with leukemia. In some cases, bone manifestations may be the first and only symptom of acute lymphoblastic leukemia, complicating the diagnosis and postponing the correct diagnosis.

The article describes 3 clinical cases of patients with a lesion of the osteo-articular system in the onset of acute leukemia. Despite the current complex laboratory and instrumental research methods, a particular attention should be paid to the difficulties of differential diagnosis between acute leukemia and other lesions of the osteo-articular system.

SHARING EXPERIENCES

93-99 4599
Abstract

The article describes a treatment protocol for pediatric patients with COVID-19 (SARS-CoV-2 virus) in Slovakia according to the recommendations of the Slovakian Pediatric Society. The aim of the article is to demonstrate possible treatment ways of pediatric patients with confirmed and probable COVID-19. This protocol is of exploratory nature, since it may be changed due to the large amount of incoming information.

TO THE PRACTITIONER ASSISTANCE

100-111 4708
Abstract

A group of experts, leading specialists in the field of pediatric gastroenterology have prepared clinical recommendations for the diagnosis and treatment of functional digestive disorders in children; they have summarized the international recommendations and domestic experience, suggesting the tactics of in everyday pediatrician’s practice. The second part of the guidelines discusses functional disorders with abdominal pain: functional nausea and vomiting, functional dyspepsia, irritable bowel syndrome, functional abdominal pain. The authors have clarified the definitions, classification and criteria for the diagnosis of functional dyspepsia and irritable bowel syndrome taking into account actual concepts. They also have defined current therapeutic principles. The diagnosis of functional nausea and vomiting, as well as functional abdominal pain, is rarely found in our pediatric practice, and therefore the detailed information provided on these diseases is of particular importance for a practitioner.

112-115 1882
Abstract

The myocardial infarction is considered to be an adult disease. Therefore, many pediatricians pay little attention to this problem. The electrocardiographic changes in children characteristic of myocardial infarction can be the signs of other pathology. The article describes four clinical cases of various nosologies accompanied by ST-segment elevation: a myocardial bridge, Kawasaki syndrome, abnormal discharge of the left coronary artery, postoperative complication after surgical treatment of congenital heart diseases. The authors discuss the difficulties of differential diagnostic search of those nosologies.

116-125 1351
Abstract

Today, the microbiota is recognized as a new «extracorporeal body» maintaining homeostasis of our body. The value of some microorganisms has already been determined, but the role of most of them is not yet clear. The researchers believe that 81- 99% of all microorganisms in healthy adults have been already identified. The mechanism of interaction of a consortium of microorganisms with each other and with the human body is not fully clarified yet. The species of the microbiota in newborns and children are not completely identified. In modern realities, the clinical significance of normal microflora is not fully understood. There have been published many reports on the direct role of the microbiota as a trigger mechanism for various diseases (atherosclerosis, obesity, type 2 diabetes mellitus, inflammatory bowel disease, irritable bowel syndrome, chronic obstructive pulmonary disease, atopic diseases, depression, autism etc.) and carcinogenesis. The study of communicative bacterial connections can be used for the development of new drugs and new strategic approaches to treatment.

HISTORY OF PEDIATRICS

126-131 977
Abstract

The article is devoted to the outstanding pediatrician — Professor Yu.E. Veltischev, the head of Moscow Research Institute of Pediatrics and Pediatric Surgery (now — Scientific Research Clinical Institute of Pediatrics, bearing his name) for many years. All achievements of Yuri Evgenievich were aimed at the future: genetics, immunology, preventive medicine, age physiology, molecular and cellular biology, homeostasis and enzymology, environmental pediatrics, telemedicine etc. Yu.E. Veltischev paid great attention to the anatomical and physiological characteristics of children, he emphasized that the pediatrics should be aimed not only at a sick child, but, above all, at a healthy child, its growth and development, biological and social adaptation, and the state of immunity. Yuri Evgenievich paid great attention to the issues of ethics, deontology and bioethics in pediatrics, pointing out that during the outstanding achievements of biotechnology, the preservation of the moral and ethical foundations of society, the protection of human values, primarily culture, science, education remains the issue of the day.

TO THE XVI RUSSIAN CONGRESS «PEDIATRICS AND PEDIATRIC SURGERY IN THE PRIVOLZHSKY FEDERAL DISTRICT». LEADING ARTICLE

135-138 681
Abstract

The article presents the current state of the domestic children’s neuro-oncological system; also it highlights the main development trends over the past decade. The authors summarize, analyze and compare data on the prevalence of malignant neoplasms of the central nervous system in children in the Republic of Tatarstan with the data of large-scale multicenter studies. The article presents the age-sex, histological and molecular structure of the central nervous system tumors through the prism of the new World Health Organization classification dd 2016, which determines the trends in the development of future systems for recording, diagnosis, treatment and rehabilitation of children with brain and spinal tumors.

TO THE XVI RUSSIAN CONGRESS «PEDIATRICS AND PEDIATRIC SURGERY IN THE PRIVOLZHSKY FEDERAL DISTRICT». LITERATURE REVIEW

139-144 1576
Abstract

Familial hypercholesterolemia Is the most common genetic disease in the world. It is characterized by an increase in the level of total cholesterol and low-density lipoproteins since childhood. It is desired to diagnose and start treatment in childhood prior to development of complications, such as aortic stenosis, atherosclerotic changes in the artery walls. Statins are recommended as the first-line medications. However, due to limited experience, the clinicians have different opinions regarding the age of initiation of hypolipidemic therapy. The article is aimed to critically examine the data on the use of statins in children with familial hypercholesterolemia, their effectiveness and safety available in the world literature.

145-151 1842
Abstract

The article reviews the role of nutrition in the development of inflammatory bowel diseases and modern methods of diet therapy of the patients with ulcerative colitis and Crohn’s disease.

Nutrition of the patients with inflammatory bowel disease, ulcerative colitis and Crohn’s disease is considered in two aspects — as a possible trigger and as a method of therapy. On the one hand, a diet rich in fruits, vegetables with a ratio not more than 5:1 m-6:m-3 of fatty acids and a 6—12 months breastfeeding are associated with a reduced risk of developing IBD. On the other hand, a high content of trans fats, sugar, higher grade flour, as well as some dietary supplements and vitamin D deficiency are considered as predisposing factors for the development of inflammatory bowel disease. The effectiveness of complete enteral nutrition of the patients with Crohn’s disease is a weighty argument in favor of the interconnection of nutrition and inflammatory bowel disease.

152-157 876
Abstract

The article reviews the influence of idiopathic (genetic) generalized forms of epilepsy on higher mental functions. It is a common view that those forms have the least impact on the maturation of mental functions in children and adolescents and on the neuropsychological status of adult patients. The results of the higher mental functions dynamics assessment in patients with idiopathic generalized epilepsies are heterogeneous for a number of reasons that are analyzed in the article (the most important reasons are the lack of a unified methodology for cognitive functions evaluation, the lack of separation of the effects of epilepsy and antiepileptic drugs, the lack of differentiation of genetic generalized forms of epilepsy when processing the results). According to the authors, the following mental functions decrease to a greater extent: memory, executive functions, visual-spatial gnosis and speech.

TO THE XVI RUSSIAN CONGRESS «PEDIATRICS AND PEDIATRIC SURGERY IN THE PRIVOLZHSKY FEDERAL DISTRICT». ORIGINAL ARTICLES

158-163 1264
Abstract

Currently, there is a trend towards the increase in invasive forms of infection (liver abscess, meningitis, endophthalmitis, etc.) caused by “hypervirulent” strains of Klebsiella pneumoniae (hvKP) in adults. We have studied the frequency of hvKP release in newborns and infants with various forms of community-acquired and nosocomial klebsiella infection. Strains of Klebsiella possessing the rmp A gene, which is associated with hypermucoid phenotype, were considered “hypervirulent. Klebsiella strains with the “hypermucoid phenotype”-associated rmpA gene were considered “hypervirulent”. The gene was detected by polymerase chain reaction. The authors studied a total of 85 strains. The rmpA gene was found in 20 (23%) Kl. pneumoniae strains, including 14 (33.3%) intestinal strains, 3 (25%) urine strains, 3 (15.7%) pharyngeal mucosa strains. The Klebsiella strains isolated from blood (n=5), endotracheal tube (n=6), cerebrospinal fluid (n=1) did not contain the gene. hvKP were significantly more often isolated in children with community-acquired infection (32%) than in children with nosocomial infection (11.4%; OR 3.647; 95% CI 1.100—12.096). Out of 20 children infected with hvKP, 12 (60%) patients were asymptomatic carriers, 3 (15%) children had symptoms of a urinary tract infection, and 5 (25%) children had symptoms of an intestinal infection. Infected children have both a potential risk of endogenous dissemination of the pathogen, and they are an epidemiologically dangerous to others.

164-168 761
Abstract

Objective. To evaluate the severity of spontaneous netosis in peripheral blood in neonatal children with sepsis.

Characteristics of children and research methods. The researchers have studied the severity of spontaneous netosis in 12 neonates with sepsis who were hospitalized in the neonatal intensive care unit of the City Children’s Hospital No.1 in Kazan. The diagnose was based on a systemic inflammatory response syndrome, an increase in the concentration of C-reactive protein in the blood of more than 1 mg/dL, the presence of one or more foci of infection, the development of organ dysfunction, and the presence of a microorganism in venous blood.

Results. It was found that the initial period of neonatal sepsis proceeds against the background of activation of netosis, which was manifested by neutrophil extracellular traps in the peripheral blood. Their quantity and percentage was significantly higher compared to the indicators of the control group (p=0.02 and p=0.0007 accordingly). The highest levels of neutrophils in the state of netosis (67% and 70%) were found in 2 neonates with septic shock and leukopenia (2.7·109/L and 2.8·109/L, respectively). Only one neonate with septic shock developed absolute neutropenia (less than 2•109/L) and leukopenia (less than 5·109 / L). Thrombocytopenia was noted in 5 of 12 neonates with sepsis, which was less than 100•109/L in every fourth child. At the same time, there were no clinical cases of hemorrhagic syndrome and blood clotting disorders.

Conclusion. The formation of neutrophilic extracellular traps is one of the most important mechanisms of anti-infective protection aimed at arresting the inflammatory process and clearing the internal media of the body.

169-175 849
Abstract

Objective. To study the state of endothelial function in children with familial hypercholesterolemia by evaluating the flow-dependent vasodilation of the brachial artery and serum nitric oxide and endothelin-1 levels.

Children characteristics and research methods. The study included total 62 children: 30 children (mean age 10,3 years ± 2,8) with a heterozygous form of familial hypercholesterolemia, not taking lipid-lowering therapy (main group), and 32 healthy children (mean age 10,3 years ± 2,8) in the control group. The content of endothelin-1 and nitric oxide in blood serum were determined by enzyme immunoassay. Flow-dependent vasodilation of brachial artery was measured using Philips Affinity 70 (Release 3,0.3, Philips Healthcare, USA).

Results. The analysis of endothelin-1 level did not reveal statistically significant differences between healthy children and patients with familial hypercholesterolemia (0,213 [0,012; 0,368] and 0,227 [0,15; 0,315] pmol/l accordingly; p=0,062). The level of nitrogen oxide in healthy children was 33,6 [20—51] pmol/l, while patients with familial hypercholesterolemia had statistically significantly higher level of nitrogen oxide — 103,7 [48—152] pmol/l, p=0,001). Flow-dependent brachial artery vasodilation in healthy children was 9,82 [7,8; 11,2]% and in patients with familial hypercholesterolemia this indicator was decreased — 9,46 [6,5; 10,8]% (p=0,742).

Conclusion. A study of endothelial function in children with familial hypercholesterolemia aged 7—11 years revealed an increase in the concentration of nitrogen oxide relative to healthy patients, probably of a compensatory nature. The flow-dependent vasodilation of the brachial artery and the level of endothelin-1 did not significantly differ from the control group. Due to possible cumulative effect of low-density proteins, it is necessary to monitor endothelial function in children with familial hypercholesterolemia in dynamics.

176-180 822
Abstract

Along with the increasing prevalence of type 1 diabetes among children and adolescents, there is a growth of rare variants of the disease — type 2 diabetes and monogenic forms of diabetes.

Objective. To study the characteristics of clinical course of diabetes mellitus in patients under 18 years, depending on the type of disease. Children characteristics and research methods. The researchers compared age, gender, and clinical and laboratory characteristics in the debut of the pathology in 43 patients with an atypical manifestation of type 1 diabetes mellitus.

Results. Despite the non-standard clinical manifestations of the disease, 62.8% of the patients in this cohort account for children with type 1 diabetes mellitus, 23.3% — for patients with MODY diabetes (23.3%), and 13.9% — for type 2 diabetes mellitus. There were no clinical and laboratory markers (due to insufficient number of patients), such as: burdened heredity, patient weight, level of residual secretion of insulin, glycemia, glycated hemoglobin at the moment of manifestation, which could be used as a guideline for the differential diagnosis. This fact justifies the usage of the entire range of laboratory and molecular genetic studies to verily the diagnosis.

181-186 746
Abstract

Toxic epidermal necrolysis (TEN) is a severe drug allergy. The objective was to analyze the clinical experience of 17 children of different age with toxic epidermal necrolysis. The authors found that medications against infection diseases were the most common reason for the development of the disease. In some cases we observed the delayed diagnosis, therapy without consideration of causative agents, and wrong doses of corticosteroids. The majority of cases require combined use of corticosteroids and intravenous immunoglobulins, however bacterial complications are possible in some cases.

187-192 673
Abstract

Objective. To establish the characteristics of the metabolism of macro- and microelements in children and adolescents involved in sports based on biological substrates.

Children characteristics and research methods. The study involved 130 children and adolescents from 12 to 17 years 11 months old. There were 102 children with an intense level of physical activity among them. The researchers used the traditional complex of clinical, laboratory and instrumental studies. The content of calcium, potassium, magnesium, iron, zinc, chromium and selenium in the saliva and hair of children and adolescents was studied by atomic emission spectrometry with inductively coupled plasma and mass spectrometry with inductively coupled plasma.

Results. The results of macro- and microelements in the saliva and hair of children and adolescents indicate significant changes in their values depending on the intensity of physical activity, as well as kind of sport. Potassium and selenium demonstrated the greatest changes. The results obtained open up new prospects for the formation of the health of children and adolescents involved in sports.

TO THE XVI RUSSIAN CONGRESS «PEDIATRICS AND PEDIATRIC SURGERY IN THE PRIVOLZHSKY FEDERAL DISTRICT». CLINICAL CASES

193-197 913
Abstract

The article presents the main issues of COVID-19 in children, concerning the epidemiology, clinical course and risk groups. Despite the fact that COVID-19 affects children less often and it usually has a mild or asymptomatic course, there are severe and extremely severe courses especially in children and adolescents with certain risk factors (such as immunodeficiency, oncological diseases, obesity or other chronic diseases). The authors describe a clinical case of the severe course of COVID-19 in a 14-year-old teenager.

198-203 1765
Abstract

Currently, the researchers from various countries have demonstrated that COVID-19 in children is diagnosed less frequently, and the course and severity of the pathological process is less pronounced than in adults. Children with COVID-19 often have asymptomatic and mild forms. However, the risk of developing severe course of COVID-19 is typical for children from risk groups. Newborns and young children, congenital malformations and concomitant diseases, primarily accompanied by immunodeficiency states of various etiologies (oncological and autoimmune diseases, diabetes mellitus) are the risk factors for the development of severe forms of coronavirus infection. The article presents two clinical cases of COVID-19 in children with acute lymphoblastic leukemia. It is shown that children with concomitant oncohematological pathology often have both mild and severe forms of coronavirus infection.

204-208 767
Abstract

Congenital leukemia is a rare disease in the neonatal period. This pathology has common clinical symptoms with a number of other common diseases of the neonatal period, such as sepsis, cytomegaly, congenital hepatitis, erythroblastosis, etc. In this regard, the description of each specific clinical observation may be of interest to practitioners. In the presented clinical case, the specific symptoms of congenital lymphoblastic leukemia in a newborn child, their rapid development and aggressive nature of the course, which resulted in death, were considered.

209-214 719
Abstract

Cerebral ischemia is the most common pathology of the central nervous system in newborns, and its neuropsychological consequences cause child disability. The global cerebral ischemia in a newborn may be caused by a violation of fetoplacental blood flow in pregnant woman with antiphospholipid syndrome. The article considers the role of antiphospholipid antibodies in the pathogenesis of hypoxice-mia-ischemic brain damage of the fetus and newborn child. The authors present a clinical case of a patient with spastic cerebral palsy and delayed psychomotor development born from a mother with antiphospholipid syndrome.

215-218 853
Abstract

Objective. To describe a case of occult bacteremia caused by Streptococcus pneumoniae.

Results. A 1 year and 1 month old child was diagnosed with pneumococcal infection without a focus of infection. After examination of the child, the doctors excluded foci of infection characteristics of pneumococcal infection, and the discharge of St. pneumoniae from the blood enabled to diagnose occult bacteremia. Despite bacteremia and signs of systemic inflammatory response, the absence of severe clinical manifestations and signs of multiple organ failure gave grounds to exclude sepsis.

Conclusion. This case demonstrates that bacteriological blood test in children with fever and without a focus of infection with pronounced inflammatory changes in the blood has important diagnostic value and helps to prescribe effective specific antibacterial therapy.

219-222 671
Abstract

The article describes a clinical case of monozygotic twins with hypertrophy of the organs of the lymphopharyngeal ring. The examination revealed IgG to the Epstein-Barr virus capsid antigen in both patients. One twin had Epstein-Barr virus DNA in the sample from the oropharynx. It was this child who had complaints typical of patients with adenoid vegetation, and he required surgical treatment (endoscopic shaver adenotomy and radio wave bilateral tonsillotomy). At the time of the initial examination and in the absence of complaints the second twin revealed no DNA of Epstein-Barr virus in the oropharynx. This patient was diagnosed with adenoids of 1-2 degree. Due to the complaints of the second twin that appeared during the following year of observation, with previously negative results on active Epstein-Barr virus markers served as the reason for a repeated specific laboratory examination. Epstein-Barr virus DNA was found in a sample from this child’s oropharynx. This case using the twin control method by partner enabled us to demonstrate the significance of Epstein-Barr virus in the pathogenesis and clinic manifestations of chronic diseases of the lympho-pharyngeal ring organs in children.

223-226 748
Abstract

Objective. To demonstrate the possible outcome and complications of testicular torsion, the importance of timely and correct choice of treatment tactics, as well as the functional long-term results of treatment.

The article describes a clinical case of complicated testicular torsion in a 14-year-old teenager with postoperative complications such as suppuration, postoperative wound failure, and partial testicular eventration, as well as treatment tactics and postoperative course of the patient. As a result of the treatment, the patient was discharged from the hospital in satisfactory condition.

Conclusion. Testicular torsion is urgent disease that requires emergency surgical treatment. Acute circulatory disorders in this pathology cause significant morphological changes in the testis, resulting to the development of destructive complications, loss of organ function, and in the long term — reproductive disfunction. Even a questionable diagnosis of testicular torsion requires urgent surgery — scrototomy, revision of the testicle.

TO THE XVI RUSSIAN CONGRESS «PEDIATRICS AND PEDIATRIC SURGERY IN THE PRIVOLZHSKY FEDERAL DISTRICT». EXPERIENCE EXCHANGE

227-231 590
Abstract

Objective: to study long-term neuropsychiatric outcomes in deeply premature infants with neonatal complications

Material and methods. The authors analyzed long-term neuropsychiatric outcomes during a three-year follow-up of 97 children born with a weight less than a 1000g in the department of catamnesis of premature children.

Results. The authors found the following predictors of the development of chronic pathology in a premature children: extremely low body weight at birth, long-term (more than 30 days after birth) use of additional oxygen, intraventricular hemorrhages of 3—4 degree and periventricular leukomalacia, the formation of broncho-pulmonary dysplasia in combination with low rates of physical (less than 10 centiles) and psychomotor development by 12 months of corrected age. The most significant factors that have a protective effect on the brain include: breastfeeding, protective regimen, developmental techniques, minimization of post-neonatal infection, prevention of broncho-pulmonary dysplasia.

Conclusion. The study of the health of deeply premature children during their first three and even more years of life is necessary to develop measures to improve antenatal, obstetric and neonatal care in order to increase survival and reduce disability among premature babies.

232-238 727
Abstract

The problem of treating patients with lymphangioma remains open to doctors of various surgical specialties due to the specific location, age of clinical manifestation and the lack of a single algorithm for the treatment of these patients.

The objective is to develop an optimal minimally invasive method for treating children with lymphangiomas. This article presents the results of surgical treatment of 7 children with lymphangioma according to two methods developed in our clinic using video endoscopic equipment. The patients with various types of lymphangioma and topographic localizations require an individual approach in choosing the optimal treatment method, taking into account all possible risks of relapse. The developed treatment methods using video-endoscopic equipment are applicable for minimally invasive surgical treatment methods in children (including newborns) and can be used to treat lymphangiomas of both superficial localization and multichamber diffuse lymphangiomas. 7 children were treated with the proposed methods. A positive result was achieved both in the case of radical simultaneous removal of lymphangioma under video control, and in the case of treatment method supplemented by sclerotherapy. We consider the proposed treatment methods acceptable due to their positive clinical result and good cosmetic effect.

TO THE XVI RUSSIAN CONGRESS «PEDIATRICS AND PEDIATRIC SURGERY IN THE PRIVOLZHSKY FEDERAL DISTRICT». FOR THE PRACTITIONER

239-244 649
Abstract

Complex congenital heart defects in newborns require the development of therapeutic tactics in a short time. It is often impossible for the patient to visit a cardiologist or cardiac surgeon due to the remoteness.

Objective: to describe the principles of the algorithm for remote diagnostics and treatment tactics of newborns with complex congenital heart defects.

The article presents the features of the course of heart disease in newborns, taking into account the specificity of hemodynamics. The authors describe the diagnostic principles using remote consultation of the patient. The authors developed a scale to choose therapeutic tactics by the specific group of the identified heart disease.

Conclusions: 1. Remote consultation on the diagnosis and treatment tactics of patients with congenital heart defects is highly relevant for regions with a low population density; 2. Remote contactless consultation is particularly important during periods of emergency and epidemics.

TO THE XVI RUSSIAN CONGRESS «PEDIATRICS AND PEDIATRIC SURGERY IN THE PRIVOLZHSKY FEDERAL DISTRICT». HISTORY OF PEDIATRICS

245-255 653
Abstract

2020 is the 100 anniversary of the of post-graduate education of pediatricians at the Kazan State Medical Academy. The article gives a brief essay on the history of the Department of Pediatrics, and further the Department of Pediatrics and Neonatology from the moment of its foundation to the present day.



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