Vol 9, No 3 (2018)

Articles
Congenital disorders of glycosylation
Ivanov D.O., Novikova V.P., Pokhlebkina A.A.
Abstract

Congenital disorders of glycosylation (CDG) is a genetically heterogeneous and clinically polymorphic group of diseases caused by defects in various enzymes, the synthesis and processing of N-linked glycans or oligosaccharides into glycoproteins. Approximately half of all proteins expressed in cells are glycosylated to achieve their full functionality. Basically there are 2 variants of glycosylation: N-glycosylation and O-glycosylation. N-glycans are bound to the amide group of aspartine, whereas O-glycans are bonded to the hydroxyl group of serine or threonine. Synthesis of N-glycans occurs in 3 stages: the formation of nucleotide-linked sugars, assembly (in the cytosol and endoplasmic reticulum) and treatment (in the Golgi apparatus). Synthesis of O-glycans occurs mainly in the Golgi apparatus. The most frequently identified types of CDG are associated with a defect in the N-glycosylation pathway. CDGs are typically multisystem disorders with varying clinical manifestations such as hepatomegaly, cholestasis, liver failure, developmental delay, hypotonia, convulsions, facial dysmorphism and gastrointestinal disorders. Also histological findings showed liver fibrosis, malformation of the ducts, cirrhosis, and steatosis. CDGs typically present in the first months of life, and about 20% of patients do not survive to 5 years. The first line of CDG screening is based on the analysis of N-glycosylation of transf ferin. Exome sequencing or targeted gene panel is used for diagnosis. Several CDG subtypes are amenable to teraphy with mannose and galactose.

Pediatrician (St. Petersburg). 2018;9(3):5-15
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Apnea within the first year of life in premature infants with bronchopulmonary displasia and pulmonary hypertension
Solomakha A.Y., Petrova N.A., Ivanov D.O., Sviryaev Y.V.
Abstract

Infants with severe and moderate bronchopulmonary dysplasia (BPD) are characterized by long-term persistence of apnea of prematurity and often have a pulmonary hypertension (PH). Respiratory pauses, accompanied by intermittent hypoxia, do not clinically manifest themselves, therefore cardiorespiratory monitoring (CRM) is required. We hypothesized that the persistent of apnea, as the cause of hypoxemia episodes, may be associated with the persistence of PH in infants with BPD.

The aim of the study was to evaluate the dynamics of cardiorespiratory parameters and to study the relationship between obstructive apnea and PH during the first year of life of premature infants with BPD + PH.

Materials and methods. CRM was performed in 58 infants were born at 26 0/7-31 0/7 weeks gestation and with birth weight less than 1500 grams, before discharge from the hospital (35-44 weeks of post menstrual age). 14 infants did not have BPD (group without BPD). 44 infants had BPD and 17 of them had a complication of this disease PH (BLD + PH group). Other infants with BPD did not have PH (BLD-PH group). Eight infants with BPD also underwent a study at home (aged 9 to 10 months of life).

Results. Preterm infants with BPD + PH were more significant decrease in the average SpO2, higher desaturation index and more a number of desaturation episodes of <10% compared to infants with BPD-PH and without BPD. There was no difference in the apnea/hypopnea index and frequency of occurrence of different types of apnea between groups. There was no difference in cardiorespiratory performance in infants with BPD+PH compared to infants with BPD-PH in 9-10 months of life.

Conclusions. There was a positive dynamics of cardiorespiratory parameters in infants with BPD+PH in 9-10 months after discharge from the hospital. The number of infants with an index of OA> 1/hour is higher in the group BPD+LH.

Pediatrician (St. Petersburg). 2018;9(3):16-23
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Maternal obesity and child health at different ages
Petrenko Y.V., Novikova V.P., Polunina A.V.
Abstract

In recent years, the number of obese women of childbearing age has increased significantly. The aim of this study was to reveal the influence of maternal obesity during pregnancy on the longterm health of the offspring.

This study was performed in the outpatient clinic in St. Petersburg with 76 adolescents with chronic diseases aged from 6 to 17 years. The mean age of the examined was 12.67 ± 3.19 years; the ratio of girls to boys was 6 : 7. Children were divided into 2 groups: the main group included 26 adolescents whose mothers were obese before and during pregnancy. 50 teen agers from mothers with normal BMI during pregnancy presented comparison group. The main group and the comparison group did not differ in age and sex. The children were examined by a pediatrician. Data on the transferred diseases are obtained from an outpatient card. It has been established that maternal obesity may be considered a risk factor for miscarriage, having low birth weight babies or babies weighing more than 4 kg, as well as a risk factor for rickets, parathrophies and functional constipation in the first year of life. In adolescentes, obesity, euthyroid goiter, hypothalamic syndrome and other endocrinopathies are typical for children born from obese mothers. Several medical conditions related to obesity such as chronic pancreatitis, hiatal hernia, iron deficiency anemia and orthopaedic foot and ankle pathology are commonly seen in children of obese mothers. Maternal obesity is associated with diseases of children not only in the period of newborn, but also in adolescence.

Pediatrician (St. Petersburg). 2018;9(3):24-27
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Increasing of assessment effectiveness of neurological evaluation in detection traumatic intracranial injures in children with clinical criteria of mild traumatic brain injury
Povzun A.A., Shchugareva L.M., Iova A.S.
Abstract

Objective. To increase the effectiveness of neurological evaluation in detecting traumatic intracranial injures in children under 18 years old with clinical criteria of mild traumatic brain injury.

Materials and methods. Clinical and clinical-sonographic evaluations of 256 patients aged 0-18 years with clinical criteria of mild traumatic brain injury were performed. Depending of detected clinical and neurological risk factors and the results of the primary clinical and sonographic examination, children were divided into two groups: group I (high or medium risk of traumatic intracranial injury) – 174 (67.9%), group II (low/no risk of traumatic intracranial injury) – 82 (32.1%). Verification of important traumatic and non-traumatic intracranial changes revealed by primary sonographic exam was confirmed by using computerized tomography.

Results and conclusion. Identification of traumatic intracranial injures by results of primary clinical examination is most effective when two or more higher or medium risk factors were detected. Application of primary clinical and sonographic examination increases diagnostic efficiency of neurological evaluation to 10.1% and the possibility of detecting traumatic intracranial injures to 57.1%. In 7.0% of cases emergency computed tomography were determined, dynamic observation was performed in 32.1% of cases. Significant non-traumatic brain diseases (cysts, hydrocephalus, congenital malformations) were diagnosed in 3.6% of cases by results primary clinical and sonographic examination.

Pediatrician (St. Petersburg). 2018;9(3):28-33
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Children juries in Dagestan
Daudova R.D., Bogatyreva E.M.
Abstract

The aim of this work is to study the peculiarities of children’s injuries in Dagestan.

Methods. We analyzed the data of the State institution of the Republic of Dagestan Republican Medical Information and Analytical Center and the data of statistical reports of the Department of traumatology of the Republican Children’s clinical hospital named after N.M. Kuraev of the Makhachkala city. Various aspects of child’s injuries had been studied within the 2012-2015 years, taking into account the cause, age, sex, type of injury.

Results. The injury frequency takes up the 3rd place in the structure of Dagestan population’ mortality. The numbers of injuries increase as the age of children increase. The greatest number of injuries are among teenage age children that makes 47.7% from the total number of children who applied to the Department of traumatology. Street injuries are prevalent in summer, and it is 67% of all types of injuries at this time of year. The pattern of domestic injuries in Dagestan is not evenly distributed across the time of year. Most injuries occur in winter – 31% and in spring – 28%. Boys are twice as likely to be involved in accidents (68%) as girls (32%). The data obtained allow us to work out recommendations for the prevention of child’s injuries in Dagestan.

Conclusions. The predominant types of child’s injuries in Dagestan are street and household. Bruises and fractures, as well as wounds sharp, piercing, cutting objects are in the first place among children’s injuries.

Pediatrician (St. Petersburg). 2018;9(3):34-40
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Clinico-laboratory efficacy of the drug Viferon® in viral lesions of the gastroin-testinal tract in children
Timchenko V.N., Subbotina M.D., Kaplina T.A., Bulina O.V., Sukhovetskaya V.F., Hakizimana J., Shakmaeva M.A.
Abstract

In recent years, viral lesions of the gastrointestinal tract take the leading place in the etiological structure of acute intestinal infections. The study of the clinical course and treatment of viral diarrhea in children is an urgent task in modern conditions. We analyzed the disease of 2568 children aged 1 month to 17 years who were hospitalized in a Hospital No 3 in St. Petersburg in the period of 2016-2017. All patients underwent a standard laboratory examination. The etiological interpretation was carried out with the help of polymerase chain reaction, bacteriological and serological methods of investigation. Of the 2568 children in 1502 (58.5%) patients, the diagnosis was confirmed. The share of bacterial diarrhea was 31.8%, viral lesions of the gastrointestinal tract – 68.2%. Among the decoded viral lesions of the gastrointestinal tract, the share of rotavirus infection was 52.8%, norovirus infection – 24.8%, mixed infection – 19.6%. A high proportion of viral lesions of the gastrointestinal tract was established in children under 3 years of age (71.7%). The main reason for hospitalization of children under 5 years of age is rotavirus infection, from 5 to 12 years – norovirus infection. Almost always (87.6%), viral diarrhea proceeded in a moderate form. In infants, mixed viral infection was recorded in severe form. The use of Viferon® in the form of rectal suppositories to supplement the basic therapy of viral intestinal infections significantly reduces the duration of intoxication syndrome, fever, diarrhea, catarrhal syndrome, the duration elimination of viruses, and the length of stay in the hospital.

Pediatrician (St. Petersburg). 2018;9(3):41-48
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VEGF dynamical changes in laboratory rodents with transplanted experimental tumors of various histological types
Trashkov A.P., Verlov N.A., Artemenko M.R., Pechatnikova V.A., Zelenenko M.A., Pakhomova M.A., Vasiliev A.G.
Abstract

Vascular endothelial growth factor (VEGF) is one of the most important cytokines in charge of proliferation, migration and differentiation of endothelial cells in physiological and pathological processes. Because of this they are involved in pathogenesis of neoplasmatic process namely mechanisms of neoangiogenesis – development of blood-vessels’ network in the tumor as well as adjoining intact tissues. Almost all contemporary antiangiogenic medicines are targeted at either VEGF or its receptors. However, there are practically no conventional models of oncologic pathology nowadays described in detail and recommended for preclinical studies. The present study focuses at changes of VEGF concentrations at va rious stages of disease using experimental tumors of different histological types, intensity of neoplasmatic growth and localization. Development of experimental transplantable tumors of various histological types and locations has been demonstrated to be usually accompanied by increased VEGF blood serum concentration in experimental animals; the dynamic of this increase depending upon the intensity of the tumor growth. A statistically valid decrease of VEGF level in comparison with the previous control point of the study has been demonstrated in BALB/c male mice with subcutaneously transplanted colonic adenocarcinoma on the background of active development of the tumor at the 45th day of the study. Pliss’ Lymphoma, and Lymphocytal Leukemia Р-388 models have been demonstrated to be optimal for the assessment of medicines’ aimed at VEGF elimination pharmacological activity.

Pediatrician (St. Petersburg). 2018;9(3):49-56
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Correction of pain-related and affective behavior by combination of fluoxetine and buspirone in prenatally stressed rats
Butkevich I.P., Mikhaylenko V.A., Shimarayeva T.N., Kochubeev A.V.
Abstract

The inhibitor of serotonin (5-HT) reuptake fluoxetine does not cause any improvement in every patient suffering from depression; moreover, fluoxetine during pregnancy disrupts the normal development of the fetus. Clinical observations in adult patients suggest that treatment of depression with a combination of fluoxetine and 5-HT1A receptor agonists has a higher therapeutic effect than the effect of a single fluoxetine. In the present study, the effect of chronic management of the combination of fluoxetine and a 5-HT1A receptor agonist anxiolytic buspirone was examined in order to correct the adverse effects of prenatal stress on pain and affective behavior in the female offspring during the prepubertal period of development. It was found that the immobilization stress of rat dams in the last third of pregnancy (the stress of rat dams during pregnancy is used as a model of human depression) caused in the offspring the increased functional activity of the pain system, an increase in the level of depressive-like behavior. Chronic administration to pregnant dams of fluoxetine, buspirone, or their combination has improved the characteristics under study in the female offspring. The results indicate that the chronic administration of a combination of fluoxetine and buspirone during the gestation of rat dams neutralized the negative effect of prenatal stress in female offspring. More effective action of the combination of the drugs compared with the action of fluoxetine was revealed in the pain sensitivity to thermal stimulus and in the first acute phase of pain behavior in the formalin test. Undesirable deviations in the investigated types of behavior in female rats subjected to pharmacological effects of each of the drugs during the prenatal period were not revealed.

Pediatrician (St. Petersburg). 2018;9(3):57-63
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Functional state of the cardiovascular system in adolescents and young men of military age with bradyarrhythmia
Klubkova I.A., Avdeeva M.V., Shcheglova L.V., Kiryanova D.R., Erina M.Y.
Abstract

The article presents the results of clinical and instrumental examination of adolescents and young men of conscript age with sinus bradycardia. The study involved 2067 people (mean age19,7 ± 2,7 years), subject to conscription (from 17 to 27 years) and potential conscripts (16-year-olds). According to the results of the screening express of ECG diagnostics, all participants of the study were divided into groups – with normocardia (n = 1266), with tachycardia (n = 171) and with bradycardia (n = 630). The article shows that in patients with sinus bradycardia, the size of the left atrium (р < 0,05), pulmonary artery trunk (р < 0,05), right atrial (р < 0,05) and right ventricle (р < 0,05) more than in individuals with normosystole and tachysystole types of heart rhythm. In patients with bradysystole type of heart rhythm, lower values of the ejection fraction (р < 0,05) were recorded in comparison with other types of rhythm at higher shock volume of blood (р < 0,05), the end diastolic volume of the left ventricle (р < 0,05) and the end diastolic size of the left ventricle (р < 0,05). Patients with bradysystole type of heart rhythm were characterized by higher rates of inotropic (р < 0,01), chronotropic reserve (р < 0,05), duration of the recovery period (р < 0,01) with lower coronary reserve indices (р < 0,01) and maximum oxygen consumption (р < 0,05) compared with normosystole and tachysystole types of rhythm. In 63,7% of patients with sinus bradycardia, the Holter monitoring determined a decrease in the mean level of heart rate below the reference values. Sinus bradycardia is associated with rhythm and conduction disorders, as well as chronobiorythmological desynchronizes.

Pediatrician (St. Petersburg). 2018;9(3):64-71
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Self-assessment of health and readiness for military service of senior pupils of Yakutsk
Iurev V.K., Zhirkov P.G.
Abstract

At present, the state of health of young men deserves special attention on the part of the state, since they are not only the economic, social, demographic, but also the defense potential of the country. The evaluation of the health of young people of their own health in terms of the ability to perform certain social functions and roles, that is, self-evaluation of health, is important in assessing health. In order to study the opinion of the young men about their health and their compliance with the requirements of military service, an anonymous survey of 325 senior pupils of four Yakutsk general education schools was conducted. It was found that many young men of Yakutsk low estimate their readiness for military service. An analysis of the self-assessment of the degree of physical training made it possible to establish that more than a third of high school students believe that they have poor or insufficient physical training, more than a quarter believe that they are not physically ready for military service. At the same time, less than a third seek to improve their physical training through regular physical training and sports, the remaining boys regularly do not engage in physical culture and sports, explaining this subjective reasons. Most know that there are such diseases in which one does not join the army, and one in four believes that he has such a disease. Of the 100 senior pupils, 8,2 ± 1,5 assess their health as bad, 11,2 ± 1,8 consider that for health reasons they are not fit for military service, at the same time 42,4 ± 2,8 believe that to military service they are only limitedly fit or temporarily unfit. Statistically significant differences in the self-assessment of health and the degree of physical fitness between the Yakuts and the Russians were not established.

Pediatrician (St. Petersburg). 2018;9(3):72-76
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The history of development of hiatal hernias’ surgery
Akhmatov A.M., Tarbaev I.S., Vasilevsky D.I.
Abstract

The article presents the main stages of formation of modern ideas about hiatal hernias as one of the most frequent variants of visceral anatomy’s impairment. The history of development of hiatal hearnias’ surgery is presented from the moment of its birth to the present time. According to modern ideas hiatal hernias are considered to be a chronic recurrent disease when abdominal part of esophagus, part of stomach or other internal is dislocated into a mediastenum and hernia ring is presented by esophageal hiatus. During the long time hiatal hernias were considered to be just the anatomical phenomenon. It’s known that Angello Soresi was the first American surgeon who performed an operation on hiatal hernia in 1919. A lot of original techniques of hiatal hernias’ surgery were devised in 1950. Operations developed by American surgeon – Ronald Belsey and European clinicians – Philip Allison and others became mostly widespread. During the next 10 years (1960-1970) techniques with obligatory antireflux component were offered. Most effective and popular operations which are used nowadays are ones that were developed by Rudolph Nissen, Andre Toupet, Lucius Hill and others. New stage of hiatal hernias` surgery started at 90’s of the XX century – the century of laparoscopic technologies’ wide application into clinical practice. Bernard Dallemagen performed such an operation for the first time in 1991. V.A. Kubyshkin, V.D. Fedorov and many others became the ideologists and pioneers of laparoscopic surgery of haital hernias in Russia. Application of new treatment methods and standardization of most surgical techniques permitted to increase efficiency and safety of hiatal hernias’ surgery.

Pediatrician (St. Petersburg). 2018;9(3):77-80
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The model of medical and biological support center for sports-gifted children and adolescents
Kalinin A.V., Butko D.Y., Danilenko L.A., Artamonova M.V., Teryokhin V.S.
Abstract

One of the main problems of modern sport is propensity for various kinds of sport activity and individualization of the training process. To solve this problem effectively, the Center of testing, selection and support for sports-gifted children and adolescents has been created on the basis of Lesgaft National State University of Physical Education, Sport and Health. The structure of the Center includes the medical and biological department, the genetic department, the department of psychological evaluation and support, the sports and pedagogical department. In each department the individual quotients, based on the collected and analyzed information about the individual's makings and abilities, are compared with the standard requirements in a particular kind of sport. After that the opinion about the general and special sports talent of person is made. Coordination interaction between departments is provided by the Information and Organizational Department of the Center, which conducts final sports consultancy and support on the basis of the complex opinion of the individual profile of the child's sports talent. Individual profile of sports talent allows to determine the degree of development and retardation of main physical qualities and abilities, to define the directions of sport specialization, to develop a training program, to make amendments. Innovative programs of individualizing training process, which are provided in this center, are based on long-term practical experience in sports pedagogic, sports genetics, sports psychology and sports medicine. The center of testing, selection and support to sports-gifted children and adolescents is an innovative institution that has no analogues.

Pediatrician (St. Petersburg). 2018;9(3):81-84
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Principles autopsy in cases of perinatal death
Nasyrov R.A., Melnikova V.F., Krasnogorskaia O.L., Popova E.D., Narkevich T.A., Sidorova N.A., Paneiakh M.B., Fedotova E.P.
Abstract

Achievements in modern medicine, allowed since 2012, significantly reduce perinatal mortality in the Russian Federation. Despite the impressive rate of decline in perinatal mortality, there are still quite a few deaths that could be prevented. Causes and mechanisms of death in the perinatal period, starting from the 22nd week of intrauterine fetal development, on the 7th day after birth, are significantly different from the tanatogenesis in a patient living more than 7 days. Our work presents perinatal mortality rates in the Russian Federation for the period from 2010 to 2016 and formulates recommendations for the consistent implementation of all stages of an autopsy of the deceased in the perinatal period in full. Issues of approaches to pathoanatomical research, principles of diagnosis formulation and correct registration of medical documentation in stillbirth and in cases of death in the early neonatal period were discussed. The main criteria that allow differential diagnostics between antenatal intrapartum fetal death and the death of a child in the early neonatal period are considered. In addition, we have developed a classification of pathoanatomical diagnosis, taking into account the characteristics of the perinatal period and the interaction in the “mother-placenta-fetus” system. The authors note that in the case of a fatal outcome in the perinatal period, the final pathoanatomical diagnosis, in addition to the generally accepted headings, should take into account the mother's condition (pathology of pregnancy, childbirth), as well as the pathology of the afterbirth.

Pediatrician (St. Petersburg). 2018;9(3):85-105
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Laparoscopic sleeve gastrectomy in the Prader-Willi-Labhart syndrome (case report)
Anisimova K.A., Vasilevsky D.I., Davletbaeva L.I., Semenov D.Y., Lapshin A.S., Balandov S.G., Fadeeva I.I.
Abstract

The article presents clinical case surgical treatment of obesity in the patient with Prader-Willy-Labhart syndrome (PWS). The patient had characteristic phenotypic features of the disease: low growth, narrow temporal part of the skull, dolichocephaly, acromicria. There was a delay in psychomotor development, hypogonadism. Excess body weight was noted from three years old with a gradual progression of obesity. Now patient is 23 and her weight is 159 kg with an increase of 150 cm (BMI 70.7 kg/m2). The co-morbidities were represented by arterial hypertension, type 2 diabetes and bronchial asthma. The indication for surgical treatment was the morbid (III degree) obesity by the Prader-Willy-Labhart syndrome, complicated by the development of diseases associated with overweight and non-medicated therapy. After a long (three-week) preoperative preparation aimed at correcting the manifestations of bronchial asthma and obesity-related diseases, laparoscopic sleeve gastrectomy was performed. The choice of the procedure of operation was determined by its pathogenetic reasonability (resection the most part of the stomach, which is producing ghrelin), and severe comorbid background (which is limiting the time of surgery) and severe hyperphagia (which is requiring a procedure with a predominantly restrictive mechanism of action). After operation period flowed without complications. The patient noted decrease of the appetite and the relief keeping nutrition's regime. A year after laparoscopic sleeve gastrectomy the patient achieved reduction of 59 kg body mass and remission of co-morbidities. A positive effect in metabolic profile allows us to consider laparoscopic sleeve gastrectomy as an effective method for treating patients with Prader-Willy-Labhart syndrome.

Pediatrician (St. Petersburg). 2018;9(3):106-110
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“Unusual” course of lymphadenopathy in patients with Kawasaki disease: clinical case and literature data
Kaneva M.A., Borovleva K.V., Peredereeva V.S., Fedotova E.P., Melnikova L.N., Filippov D.V., Yakovleva T.V., Kostik M.M.
Abstract

Kawasaki disease is an acute febrile illness of childhood, one of its characteristic features is lymphadenopathy. Most often it manifests unilateral painful more than 1.5 sm in diameter enlargement of single or several cervical lymph nodes. In some cases increase in size can be observed in other lymph nodes groups: axillary, inguinal, paratracheal, para-aortic, retroperitoneal, mesenteric. In that case the dimension is smaller than 1.5 sm in diameter. Usually it occurs at the same time as fever and fades away after inflammation is terminated. Most typical morphological features are non-purulent necrotic changes with subcapsular necrotic lesions and non-specific changes: presence of enlargement of paracortical zone and expansion of sinus. The article presents a clinical case of Kawasaki disease diagnosis accompanied by severe lymphadenopathy, persistence of fever and progression of lymphadenopathy, appearance of supraclavicular lymph node after the introduction of intravenous immunoglobulin. This reflects the non-smooth course of the disease and required differential diagnosis, primarily with lymphoproliferative disease. A review of “unusual” cases of lymphadenopathy in patients with Kawasaki disease described in the literature is presented. The importance of carrying out a histological examination of the lymph node in a nonsmooth course of the disease is underlined. Histological variants of lymph node involvement in patients with Kawasaki disease are described.

Pediatrician (St. Petersburg). 2018;9(3):111-117
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Surgical treatment of advanced congenital kyphoscoliosis: A case report
Kosulin A.V., Elyakin D.V., Dmitrieva N.N., Abzalieva A.D., Blazhenko A.A., Volchenko L.V.
Abstract

Clinical case of surgical treatment of advanced congenital kyphoscoliosis in 12 years old child is reported. Radiographs and CT at presentation failed to reveal the entire anomaly. By analysis of priorly made radiographs hemivertebra L1 was recognized. At genitourinary assessment L-shaped kidney was detected. A full-size 3D-printed model was used for surgical planning. Vertebral column resection as treatment option was discussed, but due to high neurologic complication rate this approach was refused. After preoperative 35 days halo-femoral traction anterior instrumented fusion was performed. Right ureter was stented for intraoperative urologic injury prevention. Scoliosis correction of 32% and kyphosis of 14% was achieved. 5 months later definitive posterior uninstrumented fusion was performed. Spinal deformities caused by vertebral malformations progress dramatically unless they are treated early. An advanced case is a clinical challenge because of technical difficulties and poor result. Vertebral column resection is a treatment option for severe kyphoscoliosis, but it’s indications should be strictly individualized. Preoperative halo-femoral traction is the most safe correction method in spinal deformity due to prolonged and gradual action. 3d-printed models are practical for surgical planning. The model may be used intraoperatively as a 3D-reference. In congenital scoliosis genitourinary system should be assessed meticulously, and urologist advice in relevant case is necessary.

Pediatrician (St. Petersburg). 2018;9(3):118-123
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Psychological features of children and adolescents in their perception of the disease at different stages of its course
Alexandrova O.V., Tkachenko A.E., Kushnareva I.V.
Abstract

The article is devoted to the analysis of the problem of a difficult life situation associated with a disease that threatens the child's life. The specifics of this situation are determined by the fact that, unlike other life catastrophes, it primarily affects the scope of individual personal and family values, as well as the relationship with the child, as an object of high-value affection, and refers to the turning points of the life. Factors that exhaust this situation are under review in the article. The parameters influencing the formation of an internal aspect of a child’s disease are studied. The problem of the correlation of age and psychological characteristics of children and teenagers is analyzed through the prism of their perception of their own illness and their readiness to cooperate with medical personnel and inner circle at each of the stages of the medical process, starting from the moment of diagnosis. The peculiarities of interaction with children and teenagers under conditions of hospitalization are considered: from a newborn to the age of 2 years, from 2 to 7 years, from 7 to 12 years, from 12 years and older. It is noted that the children’s attitude to the disease, as well as to other important components of their life, also depends on the emotional assessment of significant people. This attitude is largely unconscious and does not include the logical analysis which is inherent in an adult. A special role is given to the problem of informing children about the changes taking place with them in connection with the disease, both at the current time and in the distant future. It is discussed the need to develop such a form of support which can give the child more opportunities to accept the current situation that separates him from his past way of life.

Pediatrician (St. Petersburg). 2018;9(3):124-127
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Philosophical and anthropological aspect of A.A. Ukhtomsky's teaching about the dominant
Tkachenko A.S., Ivanov D.O., Berezkina E.N.
Abstract

Development context of scientific thought in the early 20 century let us see the origins of many ideas, that became fundamental in Russian philosophical anthropology. Ukhtomsky’s theory about dominant – the one of the most important in the psycho-physiological development of the 20th century. His ideas useful not only for theoretical physiology, anthropology, psychology, but also in practice, for labor, science and creative activity optimization. Dominant principle – is not only the main way of brain centers cooperation, but also all nervous system and body as a whole. It gives an opportunity to learn the contradictory and hard structure of human behavior, of a moral and creative person components, describes way of human attitude to the external environment as an active, not passive observer. Dominant connects rather elements in a single unit, moving in one direction, and present not only in physiology, but also in psychic experience, literature and art. In the philosophical-ethical attitude A.A. Ukhtomsky was developing the conception of “dominant on the other face”. It addresses on original person, see Interlocutor in his identity and uniqueness. The concept of morality of A.A. Ukhtomsky is based on the definitions of “honored interlocutor” and “double”. Deserve the Interlocutor – means to see in him something bigger, than own reflection. In the science work of A.A. Ukhtomsky we see systematic approach to the study of physiological discoveries and solving real-life problems. In an effort to understand, what a person is, how his mental development takes place, he doesn’t only makes actual discoveries, but also try to give them general scientific significance, to enter them into the general scientific system of knowledge. The systematic scientific approach is particularly relevant at the present time, when the amount of various information is growing exponentially, and the general concept of scientific knowledge, which allows to combine work in various fields of science, doesn’t exist. In our opinion, its base can give us the A.A. Ukhtomsky’s dominant principle.

Pediatrician (St. Petersburg). 2018;9(3):128-136
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