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Bulletin of Siberian Medicine

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Vol 17, No 2 (2018)
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https://doi.org/10.20538/1682-0363-2018-17-2

ORIGINAL PAPERS

6-12 1051
Abstract

The purpose of this study is to reveal the peculiarities of the state, polymorphism of hemostatic genes in newborns with thrombotic and related ischemic events.

Materials and methods. The analysis of 40 fixed cards for newborns with thrombosis and isschemic events who were in the offices of GBUZ NSO “Novosibirsk State regional clinical hospital”. A study of indicators of blood coagulation, defined by the carriage of thrombohemorrhagic gene polymorphic variants was conducted. The control group included 30 healthy newborns.

Results. The structure of thrombotic complications was analyzed, and a number of ischemic complications were revealed in infants. A statistical analysis was carried out, which allowed the presence of features of hemostatic system in thrombotic and related ischemic events in newborns to be established. The most significant gene polymorphisms found with these complications in the neonatal period of life are revealed.

Conclusion. The hemostatic system in children of the considered group is characterized by a high Level of d-dimers, in the neonatal period, Leiden mutation is the most important, thrombotic and ischemic events are more common in children with a combination of several polymorphisms of the genes of the hemostasis system. 

13-20 2787
Abstract

Aim. Polycystic ovary syndrome is a common cause of infertility. In addition, polycystic ovary syndrome is often associated with metabolic complications. In most women hyperandrogenic manifestations occur during puberty. Early detection of polycystic ovary syndrome and associated metabolic problems can solve the problem of puberty and prevent infertility, metabolic syndrome, and diabetes mellitus type 2 in women of reproductive age. One of the objectives of the study was to evaluate the effectiveness of the simultaneous use of a combined oral preparation (ethinyl estradiol + drospirenone) and metformin in adolescent girls with polycystic ovary syndrome.

Materials and methods. 113 adolescent girls with hyperandrogenism from 14 to 19 years were included. 32 of these girls aged 14–18 years with polycystic ovary syndrome were treated with medication ethinyl estradiol + drospirenone. Of these 32 patients, 20 girls with insulin resistance were obtained simultaneously with metformin. The following methods were used: examination, evaluation of hair (Ferriman-Gallwey score), pelviс ultrasound, determination of hormone status (LH, FSH, PRL, 17-OHP, E2 , TSH, DHEA-s, SHBG, T), glucose, insulin, and glucose tolerance test. The diagnostic technique used the Sultan C. criteria of polycystic ovary syndrome (2004).

Results.A reduction in the frequency characteristics after treatment was shown: of laboratory hyperandrogenism 90.6% , dermopathy 65.6% , insulin resistance 43.8%, hyperinsulinemia 18.8%, ultrasonic signs of PCOS 34.4%.

Conclusions. The study results confirm the efficacy of the treatment of polycystic ovary syndrome with insulin resistance during puberty by Low-dose combined oral preparation (0.03 mg ethinyl estradiol and 3 mg drospirenone) in combination with metformin. 

21-30 1278
Abstract

The phenomenon of eating behavior in the development of obesity in the pediatric age group remains poorly understood.

The purpose of the study. To determine the peculiarities of eating behavior of children of various age groups depending on body mass.

Material and methods. A comparative prospective study of 905 children: 342 children 1–7 years (4.7 ± 1.29), 249 children aged 8–12 (11.08 ± 0.81) and 314 adolescents 13–18 (14.25 ± 1,34). A physical survey was conducted according to standard methods with calculation of body mass index. For the study of eating behavior of preschool children, a survey of the parents was conducted using the scales of CEBQ (Child Eating Behaviour Questionnaire; J. Wardle, 2001; by the license author), an assessment of eating behavior of children 8–12 years was conducted through the analysis of the questionnaire FEV - II, and an assessment of adolescents was done with the use of the Dutch questionnaire DEBQ (Dutch Eating Behavior Questionnaire). Statistical analysis: STATISTICA (version 8), Microsoft Excel 7,0.

Results. Assessment of the frequency of occurrence of eating disorders in children and adolescents showed that regardless of age and body mass, all the children had eating disorders. The development of pathological disorders begins at 5-years of age and acquires a clear shape at 7 years. All children with normal body weight was dominated by external type of eating behavior, in subgroups of patients with overweight / obesity was the prevalent restrictive type. In patients with obesity, a third had combined forms of food behavior with the prevalence of the combination in children 8–12 years of restrictive and external types, in children 13–18 years of restrictive and emotional types. The frequency of occurrence of these combinations was increased together with the increase in children of body weight. 

31-40 969
Abstract

The aim of the work is to study the state of health and peculiarities of metabolic reactions in adolescents, representatives of the small Siberian ethnos - tofalars and caucasians.

Materials and methods. Clinico-biochemical examination of children and adolescents, representatives of a small ethnic group – Тofalars and Caucasians, permanently residing in the territory of Tofalaria in the Irkutsk region. The metabolic status of adolescents was assessed using spectrophotometric (total cholesterol, diene conjugates), fluorometric (superoxide dismutase activity, α-tocopherol and retinol levels), immunoenzymes (determination of thyroid-stimulating hormone concentrations, free fractions of triiodothyronine and thyroxine, cortisol).

Results. An increased incidence of diseases of the musculoskeletal system and connective tissue, as well as diseases of the endocrine system and eating disorders – among Тofalars; diseases of the genitourinary system – among Сaucasians. For practically healthy Тofalar teenagers, in contrast to Сaucasians of the newcomer population, adaptive metabolism reactions are characteristic, which is expressed by a reduced level of total cholesterol and increased values of antioxidant factors (the level of fat-soluble vitamins – α-tocopherol and retinol), and adaptive changes in the system of neuroendocrine regulation.

Сonclusion. An assessment of the health status of children and adolescents in Tofalaria has shown the need for more intensive monitoring of the incidence rate in the specified region, with the obligatory consideration of the ethnic factor for the development of regional ethno-specific preventive measures. 

41-48 1216
Abstract

Development of the undifferentiated connective tissue dysplasia (UCTD) is influenced by various adverse factors to the fetus during intrauterine growth. There is not a clear linkage to the contribution of the separate factors to the development of the UCTD.

Aims of the study are to determine the risk factors associated with the development of the UCTD of the adolescents and to appreciate the predictive value of the examined factors related to UCTD to form the dispensary groups of the children with UCTD.

Materials and methods. During the first stage of the study there was an examination of 110 adolescents at the age of 10–14 years old, the UCTD presence was established according to the scale proposed by T.I. Kadurina and V.N. Gorbunova (2009). During the second stage the data of the prenatal casework and maternity hospital records attached to the out-patient medical records were examined to detect the specialties of the antenatal life course. During the third stage 2 groups were formed: with the presence of UCTD (n = 81) and without UCTD (n = 29). The mother groups were formed accordingly with the adolescents groups.

Results. The pregnancy of the mothers of the adolescents with UCTD proceeded against the anemia, threatening miscarriage, chronic fetal hypoxia, chronic fetoplacental insufficiency, toxicosis, gestosis, and concomitant chronic conditions (thyroid, kidneys, digestive apparatus, aspiration pathology) more common than the pregnancy of the mothers of the adolescents without UCTD. The binary logistic regression method allowed the predictors of the UCTD’s formation to be determined, there were: toxicosis during the gestation course (OR = 10.9; CI 95% 2.94–40.49), anemia of pregnancy (OR = 8.6; CI 95%: 2.42–30.81), gestosis (OR = 6.53; CI 95%: 1.27–33.71), chronic fetal hypoxia (OR = 4.4; CI 95%: 1.09–17.83) and pre-existing chronic conditions of the mothers (OR = 3.6; CI 95%: 0.86–15.48). Different factor combination enhances the likelihood of the delivery of a child with UCTD more than 6.5-fold: chronic conditions of the mothers and chronic fetal hypoxia (OR = 6.8; CI 95%: 1.95–48.57); anemia of pregnancy and chronic fetal hypoxia (OR = 7.2; CI 95%: 1.01–50.99), and toxicosis (OR = 10.4; CI 95%: 1.48–72.82).

Conclusion. The risk factors associated to the development of UCTD in adolescents are: toxicosis during the gestation course, anemia of pregnancy, gestosis, chronic fetal hypoxia and pre-existing chronic conditions of the mothers. The use of the equation of the binary logistic regression allows the prediction of the possibility of the development of UCTD in adolescents at the level of 80.9% and to form the dispensary groups of children in order to prevent the development of dysplastic-dependent pathology

49-59 924
Abstract

Purpose of the study. To assess the influence of main clinical risk factors on the effectiveness of the in vitro fertilization (IVF) program.

Materials and methods. The study included 3,969 IVF programs conducted in the “Medica” clinic of the “Mother and Child” group of companies in Novokuznetsk for 2010–2015. The inclusion criterion was the availability of information on the results of the procedure conducted.

Results. The structure of the considered risk factors was identified, their key features were identified: the majority of women who underwent IVF procedure were under the age of 34, inclusive, more than half of all procedures were performed at the first attempt. The main causes of female infertility were: tubal factor, including those associated with chronic endometritis, polycystic ovary syndrome, external genital endometriosis and adenomyosis. In a number of cases, as a concomitant gynecological disease, uterine fibroids have been extirpated, which is not the main cause of infertility, but the presence of which can influence the effectiveness of IVF programs. Statistically significantly more often, fibroids were found in adenomyosis and tubal factors associated with chronic endometritis. A quantitative assessment of the degree of influence of the considered risk factors on the effectiveness of IVF programs was considered, and the influence of both individual factors and their combination was considered.

Conclusion. The main factors statistically significantly increasing the risk of a negative outcome of the program are: the age of a woman over 30; repeated attempt at the age of up to 30 years inclusive (in comparison with the first attempt); tubular factor associated with chronic endometritis; presence of uterine fibroids in the tubular factor associated with chronic endometritis; The presence of fibroids in the tubular factor, complicated by adenomyosis. 

60-70 974
Abstract

Aim. The paper presents prevalence and disturbances of cardiac rhythm and conduction in children with a single ventricle (SV) at different stages of hemodynamic defect correction. It also analyzes risk factors of arrhythmia.

Materials and methods. A total of 70 patients with SV was performed all stages of hemodynamic correction of defect. To assess disorders of rhythm and conduction, echocardiography, pulseoxymetry, cardiac catheterization, pulmonary angiography, ECG, and Holter monitoring were performed before and after surgery on all children at every stage of hemodynamic correction.

Results. Absence of heart rhythm disturbances (HRD) at 1st stage of hemodynamic correction was 98.1%, at 2nd – 80%, at 3rd – 48.6% and at 4th – 41.5%. Sinus node dysfunction is the most often HRD from the second stage of hemodynamic correction. Statistical analysis showed significant decrease of saturation ((73 ± 12) и (81 ± 4)% correspondingly, p < 0.05) and increase of average pressure in the pulmonary artery after TCPC ((9.6 ± 4.2) and (11.5 ± 5.8)% correspondingly, p < 0.05) in children with heart rhythm disturbances in comparison to children without arrhythmias. The average age of children with arrhythmias after TCPC and after fenestration closure, correspondingly, was significantly higher than in the group of children without arrhythmias ((72 ± 43) mon and (52 ± 27) mon correspondingly, p < 0.05). Studies of arrhythmias in children with SV require follow-up as they significantly influence on the disease and life quality. 

71-79 2965
Abstract

The purpose of this study is to evaluate the ability of L-tryptophan used in complex therapy of delayed psycho-speech development in children to influence the dynamics of cognitive performance.

Materials and methods. The study included 80 children aged 3–7 years with delayed psycho-speech development (DPSD). The main group consisted of 37 children (25 male and 12 female), a control group – 43 patients (30 male and 13 female). Both groups of children were comparable in age, sex, degree of speech and cognitive impairment, and IQ level. Patients in the main group have taken standard therapy with L-tryptophan (25–50 mg per day depending on the age) supplement for 14 days. Control patients have taken standard therapy only. Psychodiagnostic tests at the entrance and exit of patients from this study was carried out for evaluation the dynamics of cognitive functions. All differences were considered significant at p < 0.05.

Results. The study suggests that oral L-tryptophan intake selectively affects the dynamics of cognitive performance in children with DPSD. So, we studied pre- and post-treatment cognitive performance in both group patients and noted a significant improvement in the visual memory (p < 0.001) and an increase in thinking productivity (p < 0.001) in the tryptophan group versus similar indicators in the control group. Intergroup differences (p < 0.001 and p = 0.026, respectively, for the main and control groups) also found.

Conclusion. Two-week L-tryptophan intake in complex therapy of DPSD in children can significantly improve the cognitive activity, and greatly increase both the effectiveness and sustainability of treatment outcomes, which will significantly reduce the time and frequency of hospitalization and financial costs for the rehabilitation of this patients. 

80-92 1407
Abstract

Aim. To characterize the physical development (PD) and eating behavior (EB) of children and adolescents born prematurely.

Materials and methods. The study involved 123 people born in the period 1997-2008, 58 children born prematurely, age (12 ± 1.3) years, gestation period (33.07 ± 1.9) weeks (main group). The control group consisted of 65 full-term peers, age (12.7 ± 2.2) years, gestational age (39.5 ± 0.8) weeks. Participants were evaluated by PD (height, weight, body mass index (BMI), waist circumference (WC), throat circumference (TC)) under the WHO AnthroPlus program, and EB using the Dutch DEBQ questionnaire.

Results. Growth and Z-score growth of boys and girls of the main group is lower than that of their fullterm peers, p = 0.04; 0.004 (p < 0.0001 for Z-score growth). Z-score BMI in boys and girls of the main group is higher than in full-term, p = 0.04; 0.01. WC/TC the girls of the main group is higher than in fullterm peers, p < 0.0001. In premature boys, the restrictive type is 13.3%, emotional and external – 50%, the combination of emotional and external – 33.3%. In the control restrictive – 5,7%, emotional – 28,6%, external – 32,3%, the combination of emotional and external – 10,8%, the combination of all three types – 5,7%. In the group of premature girls restrictive –7.1% against 36.7% in the control, (p = 0.007), emotsiogenic – 10.7% against 36.6% of the control (p = 0.02), external – 10.3% against 60% of the control (p = 0.003). In the control group the combination of external and emotiogenic – 16.7% (in the primary group is 0, p = 0.02). Preterm boys emotiogenic and external and their combination more often than girls (p = 0.001; 0.02; 0.001).

Conclusion. Children and adolescents born prematurely are below full-term peers and have a high z-score of BMI. Boys realize violations of external and emotional types, and their combination. 

93-99 942
Abstract

Aim. To study the relationship between obesity of the mother, the body weight of the newborn and the formation of metabolic disturbances in subsequent periods of life.

Materials and methods. A prospective study was conducted in which 1,000 women of reproductive age and their newborns were included by the random number method. The mean age is 29.5 [25.0; 33.5] years. The examination included the collection of anamnesis, measurement of body height and body weight, blood pressure, waist circumference and body mass index determination. Micro- and macrosomia in term infants was diagnosed with a birth weight less than 15 and more than 97 percentiles (WHO, 2005). The laboratory study included the determination of glucose, cholesterol, triglycerides, high and low density lipoproteins, insulin and C-peptides of basal and stimulated levels, leptin, adiponectin, determination of insulin resistance indices (HOMA-IR) and pancreatic β-cell function (HOMA-β). The statistical analysis of the material was carried out using the Statistica 10 software package, SPSS 13.0. Statistically significant differences were taken into account at a level of p < 0.05.

Results. It was found that when pregnancy occurs, 41.0% of women are overweight or obese. In the study, the pathological mass of the body in the neonatal period was detected in every fourth newborn (24.8%), including microsomia (11.3%) and macrosomia (13.5%). As a result, differences in the body weight of newborns were found, depending on the maternal pregestational BMI. For example, women with obesity of the first degree were more likely to have children with macrosomia in 33.3% of cases than women with normal body weight, whose incidence of children with macrosomia was detected in 12.0% of cases (OR 6.8; 95% CI 2.66–17.56; p < 0.001). In women in the reproductive period, macrosomia at birth was more characteristic for people with a metabolic syndrome, and was associated with hormone-metabolic changes.

Conclusion. Тhe results demonstrate the effect of pre-gestational body weight in women with obesity on the body weight of offspring at birth. Both macrosomia and microsomia at birth can be risk factors for the development of the metabolic syndrome in the long term, which confirms the importance of preventing overweight and obesity in women of childbearing age in the pre-gestational period. 

REVIEW AND LECTURES

100-113 1407
Abstract

Objective: to systematically review foreign literature and interpret results of the review. Tasks: to review foreign papers where factors are considered which provoke development of depression: hormonal, psychological, social as well as neuromediators, hormonal and immune disturbances in depressive disorders in women with physiological climacteric and climacteric syndrome; issues of differential diagnostics of depressions, psychopharmaco- and psychotherapy with subsequent evaluation of clinical efficiency; personality profile of pateints with affective psychopathology.

Methods of search: by keywords in Web of Science Core Collection database across foreign journals (2012– 2016). Criteria of inclusion of papers in the review are determined by themes of studies: 1) women of climacteric age; 2) presence of depression or depressive symptoms; 3) presence of climacteric disturbances. In the abstract-bibliographic and scientometric database Web of Science Core Collection 70 bibliographic sources are selected across foreign journals between 2012 and 2016, including journals with high Impact Factor. Studies included in the review are performed at the university clinics, specialized centers. Clinical and sociodemographic characteristics of female patients meet the criterion of compatibility. Most discussed papers are devoted to study of clinical and social-psychological factors of development of climacteric depression. In a number of papers the efficiency of antidepressant therapy, alternative methods and supplementary therapy in women with depressive disorders, climacteric disturbances and co-occurring physical diseases is shown. Most works are performed with involvement of questionnaires (sociodemographic data, anamnesis) and international clinical scales. The main results of the discussed papers are outlined in thematic rubrics.

Conclusion. The European and American papers are used in this review more frequently; studies from Asian countries are used more seldom. Reviewed foreign publications reflect worldwide trend to increase of climacteric depression (CD) in the female population with presence in the anamnesis of adolescent (psychoendocrine alteration) and postpartum depression, premenstrual syndrome. Low timely diagnostics of depressions, high incidence rate of somaticized CD are noted. In the structure of climacteric syndrome the psychoemotional disturbances predominate above neurovegetative and metabolic-endocrine or are combined with vegetative dysfunction. The participation in formation of CD (with predominance of mild/moderate severity) of neurohormonal, genetic, biochemical, social-environmental, psychological factors is shown. In the reviewed sources low mood, loss of previous priorities, decrease of productivity and concentration of attention, position of being unprotected, dependence, lack of confidence, self-humiliation, repentance, unbelief in future, insomnias, hypo-/hyperrexia with change of body mass are described in CD but there are no publications on suicidal ideation. It is indicated that CD can flow with hysteric- and nosophobic, somatohypochondriac and asthenohypochondriac component. The authors consider that somatization as an experience of climacteric stress leads to somaticized CD with accent on physical symptoms and repression of depression and anxiety although an association of specific somatic nosologies with symptoms of CD is not described. The association of CD with social-environmental factors (gender, education, profession, social position, financial wealth) is discussed, achievement of the woman is considered as a actor of reduction of CD risk. The authors are highly interested in search for genetic markers (heredity, suicides in relatives), impairment of neuromediator exchange (neurotransmitters serotonin, dopamine, adrenaline, and noradrenaline), neuromorphologic alterations in brain sensorimotor cortex (motor function, attention, perception, memory, and emotional-motivational response), hormonal disturbances (neuroendocrine and metabolic) and psychoneuroimmunological patterns of association with CD. Psychopharmacotherapy in CD is constructed with account for depressive symptoms (antidepressants of activating/sedative action in long-term maintenance regime), background and co-occurring diseases (adequate and pathogenetic and immunotherapy) with involvement in case of absence of contraindications of substitutive hormonotherapy (estrogen, progesterone). Beyond conventional schemes of the therapy the alternative therapy of CD (acupuncture, yoga, phytoestrogen collections, and food additives) is discussed. For heightening the efficiency and safety of the therapy of CD the training in detection of CD signs both for female patients and nurses, psychologists, social workers is proposed. 

114-120 1651
Abstract
Atopic march is a variant flowing of atopia that begins in early childhood as atopic dermatitis, then developinto other allergic diseases (bronchial asthma, allergic rhinitis) at an older age. The state of the epidermal barrier and sensitization to inhaled allergens are considered as predictors for the development of atopic march. Data on the importance of these factors in the development of atopic march and information about possible approaches to prevention are presented in this article.
121-132 1417
Abstract
The steady rise of obesity in children and adolescents emphasizes the need for new, integrated approaches to its diagnosis and therapy. When diagnosing obesity and choosing methods for its correction, it is fundamentally important to use reliable methods of estimating the amount of adipose tissue. Using the body mass index is not always sufficient, since it does not provide complete information on quantitative content in the body weight of the patient’s body. For these purposes in clinical medicine use of bioimpedance analysis to assess the indicators, which characterize the basal metabolism, active cell mass, fat and basirova mass and total water content in the body. However, the holding of bioimpedance body composition analysis is currently limited mainly to dietetics and sports medicine, and adult patients. Quite interesting is the use of the bioimpedance method in the pediatric age group for accurate evaluation of body composition of children of various ages and body weight that will allow for dynamic control of all types of metabolism to evaluate the effectiveness of the observation and treatment of patients with overweight and obesity.
133-142 1023
Abstract
A review of the literature devoted to the analysis of conditions and development of the female reproductive gland. From the moment of the formation of urogenital scallops to the formation of a full gonad, at the different stages of ontogeny, a huge number of genes, factors and proteins are expressed, since in them the primary sex cells and in their surroundings render folliculogenesis and determine the reproductive health of a woman. Aberrant production of these factors can be the cause of ovarian dysfunction and disorders. Different points of view on the emergence of the problem of migration and colonization of germ cells in female gonads, as well as directions for further fundamental and practical research. The development and differentiation of the follicle is a succession of events that are tightly regulated by endocrine factors, intraocular regulators and intercellular interactions. The initial migration of germ cells and their multiplication within the urogenital scallop require regulation using integrated signals such as growth factors, transcription and differentiation, secreted oocytes, transforming growth factors, growth factor and differentiation-9, bone morphogenetic proteins, stem cell factor, basic growth factor of fibroblasts, tumor factor of Williams transcription, gene steroidogenesis, antimulylerovy hormone, meiosis-regulating genes and many others, as well as a contact these interactions of germ cells with extracellular matrix proteins and cellular substrates attract the developing gonads. Recent studies indicate the possibility of obtaining human gametes from the culture of stem cells, a distinct molecular genetic mechanism of the origin, migration and colonization of the sex cells. Consequently, the understanding that all the subtleties and molecular mechanisms at each stage of the bookmarking and development of the ovaries, germ cells and their environment, the death of gametes, can help in finding regulators and preventing pathological follicular depletion.
143-155 1059
Abstract
The severity of the disease is the main factor for further treatment. Bronchoobstructive syndrome of children is widespread. Early detection of the disease severity and correct treatment eliminate the clinical manifestation of the syndrome and improve the quality of patients life. Criteria of severity, as a rule, serve a certain set of clinical and paraclinical parameters used for different nosological forms. In particular, the decrease in parameters of the function of external respiration is the “gold standard” for assessing the severity. Lung function tests for preschoolers are limited. Clinical respiratory scales may be useful in the evaluation of preschooler. Alternative assessment respiratory function is sometimes used as clinical scale. Numerous studies devoted to the objective assessment of bronchial obstruction syndrome severity. A scientific literature review is dedicated to the analysis to the assessment of the bronchoobstructive syndrome severity degree and the effectiveness of therapy at preschool-aged children.
156-166 1024
Abstract
Hen’s egg allergy is one of the most common problems in pediatrics. This pathology is associated with severe clinical manifestations, often with a risk of anaphylactic reactions. This review presents current data on the epidemiology and clinical presentation, as well as aspects of the natural history and management of hen’s egg allergy. The paper will provide data on allergenic proteins of hen’s egg, including data on structure, physicochemical properties, IgE-binding capacity, and cross-reactivity. Finally, practical information about egg allergy and immunization will be presented.
167-174 910
Abstract
Relapsing polychondritis (RPC) is an autoimmune disease characterized by the inflammation of cartilaginous tissues and other proteoglycan rich tissues. A concomitant disease, particularly myelodysplasia or systemic autoimmune disease can be detected in one-third of the patients with RPC. Unlike adults in children, RPC is less often associated with other autoimmune diseases. The diagnosis of RPC is established using the criteria of Mc Adam (1976) or Damiani (1979). The basis of the pathogenesis of RPC is an autoimmune reaction, which is initially directed against cartilage and then spreads to non-cartilaginous tissues. One of the elements in the pathogenesis of RPC is the mechanical trauma of cartilage, resulting in the release of pro-inflammatory cytokines (tumor necrosis factor alpha, interferon-γ, interleukin-8, and macrophage inflammatory protein 1) and local inflammation followed by the formation of autoantibodies in a patient with a genetic predisposition. In the treatment of RPC, steroids, non-steroidal anti-inflammatory drugs, colchicine are used and, if they are ineffective, immunosuppressants are prescribed. The most effective anti-cytokine drugs used in the treatment of RPC are tumor necrosis factor-alpha (TNF-α) inhibitors, IL-1 receptor antagonists, an inhibitor of the costimulatory pathway of T-lymphocyte activation, monoclonal antibodies against the IL-6 receptor. Given the fact that management of these patients is very complex, the aim of the study is to review available data on pathogenetic mechanisms of biological agents in managing of relapsing polychondritis.

CLINICAL CASES

175-180 1025
Abstract

The most common surgical pathology of childhood, which is necessary for doctors of all specialties, is the inguinal hernia. In the treatment of this pathology, both open procedure and laparoscopic surgery are used. Infringement of a hernia, manifested by sudden or gradual compression of its contents in the gates, is a dangerous complication.

Violation of the formation of sex - a condition associated with the clinical and biochemical manifestation of the discrepancy between the genetic, gonadal and phenotypic sex of the child. Traditionally, the term “hermaphroditism” is used to describe this state. A distinction is recognized between true hermaphroditism (gonadal) and false hermaphroditism (the presence of signs opposite to the gonadal sex of the subject). True hermaphroditism is a rare disease. False female hermaphroditism is attributed to conditions in which the female karyotype and gonads that are characteristic of the female sex are determined. But the external genitalia have a bisexual structure. The most common cause of false female hermaphroditism is congenital dysfunction of the adrenal cortex.

According to several authors, a frequent cause of amblysexuality of the genitals in newborns is mixed gonadal dysgenesis – a condition in which phenotypic men or women have an egg on one side, and a gonadal cord located either in a wide bundle or on the pelvic wall and consisting from the stroma of the ovary on the other. At birth, the external genitalia are usually the penis, the uro-genital sinus and the labia genitalia, which are intertwined in the scrotum.

Evaluation of the patient’s local status with violation of the formation of sex in the occurrence of surgical pathology is not an easy task, since the appearance of the external genitalia in the XX and XY subjects is the same. In 2015, in connection with the injured inguinal hernia in the department of pediatric surgery No. 1, the State Children’s Clinical Hospital “OKB No. 2” in Tyumen received treatment for two children with a violation of the formation of sex. Difficulties in interpreting intraoperative data prompted us to describe these cases. 



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ISSN 1682-0363 (Print)
ISSN 1819-3684 (Online)