I.P.Pavlov Russian Medical Biological Herald最新文献

{"title":"Consanguineous marriage as socio-medical problem","authors":"O. B. Bobdjonova, F. M. Abdurakhmanova","doi":"10.23888/pavlovj2020282249-258","DOIUrl":"https://doi.org/10.23888/pavlovj2020282249-258","url":null,"abstract":"Despite the availability of a large amount of scientific data on the negative impact of consanguineous marriages on the health of children born in such families, the significance of this problem is not decreasing in a number of countries. Aim . Analysis of the modern scientific base on the problem of consanguineous marriages, assessment of their impact on the development of a particular pathology in children. The article reflects the position of modern clinical recommendations, the results of recent studies, and some controversial and unresolved issues. Despite the emerging trend of declining consanguineous marriages in recent decades, this problem involves at least 20% of the population and dozens of countries around the world. The prevalence of consanguineous marriages in the world varies significantly, ranging from 0.1-0.4% in the United States and European countries to more than 50% in North Africa, the Middle East and West Asia. The most common form of consanguineous marriage is a marriage between first cousins. In general, rural residents who have a lower level of education are more likely to enter into consanguineous marriages. Conclusion . Despite a number of positive social and economic aspects of consanguineous marriages, from the point of view of genetics and medicine, it is necessary to take into account the negative impact of consanguineous marriages, which consists in an increased genetic risk to offspring and a high incidence of congenital pathology in newborns. An assessment of the socio-demographic aspects of consanguineous marriages shows that their greater prevalence is often due to poor economic conditions, lack of education, and low social security.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"78 1","pages":"249-258"},"PeriodicalIF":0.0,"publicationDate":"2020-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78107108","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Methods for improving the results of using a synthetic prosthesis in the popliteal-tibial segment in critical limb ischemia","authors":"A. V. Matyushkin, A. A. Lobachev","doi":"10.23888/pavlovj2020282200-212","DOIUrl":"https://doi.org/10.23888/pavlovj2020282200-212","url":null,"abstract":"Aim . Analysis of immediate and long-term results of using a synthetic prosthesis for femoral-distal-popliteal and tibial bypass grafting in patients with critical lower limb ischemia. Materials and Methods . Patients with symptoms of chronic arterial ischemia of the lower extremities in the stage of critical ischemia (n = 109) were operated at the university surgical clinic on the base of D.D. Pletnev City Clinical Hospital, Moscow, in the period from 2009 to 2017. All the patients underwent the operation of femoral-distal popliteal or femoral-tibial bypass grafting using a synthetic explant (PTFE). Shunting with a synthetic prosthesis was performed in 33 (30.3%) patients, and in 76 (69.7%) cases a combination of an explant with a section of autovein was used in the form of a combined shunt in 44 patients (40.4%) (synthetic prosthesis + autovein) or a patch- or cuff plasty of distal anastomosis in 32 patients (29.3 %). In 14 (12.8%) patients of both groups, the intervention was supplemented by intraoperative balloon angioplasty of the lower leg arteries. Long-term results were evaluated within the period up to 5 years after surgery. Kaplan-Meyer survival analysis was used for the calculation of results. Results . The cumulative 5-year patency of the synthetic prosthesis was 42.9%. However, use of the prosthesis in combination with the autovein section in the form of a combined shunt or plastic surgery of the distal anastomosis significantly improved the values of cumulative patency (54.5%) and limb retention (83.6%). Conclusions . The results obtained make it possible to recommend the use of a synthetic prosthesis in combination with an autovenous plastic surgery of a distal anastomosis or by combining a prosthesis with a small section of an autovein in the absence of a complete autovein. Regular monitoring of such shunts in the long-term period and timely follow-up interventions are necessary for improving long-term results.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"75 1","pages":"200-212"},"PeriodicalIF":0.0,"publicationDate":"2020-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83220571","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Methods of pharmacological correction of intrauterine growth restriction syndrome","authors":"O. I. Klycheva, A. B. Khuraseva","doi":"10.23888/pavlovj2020282213-222","DOIUrl":"https://doi.org/10.23888/pavlovj2020282213-222","url":null,"abstract":"Aim . Comparative analysis of the effectiveness of pharmacological correction of intrauterine growth restriction syndrome (IGRS) by monotherapy with diosmin and dipiridamol. Materials and Methods. Retrospective and prospective examination of 80 pregnant women with singleton pregnancy with gestational age from 28 to 36 weeks with confirmed diagnosis of IGRS of 1 or 2 degree asymmetric form was conducted. 75.0% Of pregnant women that participated in the study, were of the average reproductive age (23-29 years of age). The share of young first-time-mother in I group was 10.0%, in II group – 15.0%, the share of age first-time-mothers was 17.5 and 10.0%, respectively. Extended history taking and history analysis, general clinical and obstetric-gynecological examination were conducted, laboratory and ultrasound methods were used. Newborns were evaluated on Apgar scale at birth and in 5 minutes. In the early neonatal period, inborn and transient pathological syndromes were evaluated. Results . In patients receiving diosmin (n=40), reduction  of the resistance index of the right and left uterine arteries  to 0.440±0.004 and 0.460±0.004, respectively,  and of the umbilical artery to 0.56±0.02 was achieved, that is lower than in the group of patients taking dipiridamol (n=40). A positive influence of diosmin on the intrauterine condition of the fetus was found that was manifested by its increased compensatory capacities for adaptation  to chronic hypoxia in reliably higher percent of cases as compared to dipiridamol. After pharmacological correction, a tendency to normalization of the main parameters of the system of hemostasis was found in higher percent in women taking diosmin. This, in turn, produced a favorable influence on the volume of blood loss in physiological deliveries. Thus, in I group the average amount of blood loss was 180±15 ml, while in II group it was 265±15 ml (р<0.05). However, in operative delivery no such differences were obtained. In I group immediately after deliveries 10.0% of newborns were transferred to the resuscitation and intensive care unit, in II group transfer to resuscitation department was required in 37.5% of infants (<0.05). All the rest of children immediately after birth in the satisfactory condition were placed to one ward with mother, and they did not require resuscitation measures.  Conclusion . Increase in the compensatory capacities of the fetus was shown in the conditions of chronic intrauterine hypoxia in a reliably higher percent of cases after pharmacological correction with diosmin. This, in turn, produced a favorable influence on perinatal outcomes, birth of children with a higher parameters of mass and height and health index.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"14 1","pages":"213-222"},"PeriodicalIF":0.0,"publicationDate":"2020-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88147277","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A study of influence of progesterone on activity of Glycoprotein-P in vitro","authors":"P. Erokhina, Y. Abalenikhina, A. Shchulkin, I. V. Chernykh, N. M. Popova, A. A. Slepnev, E. Yakusheva","doi":"10.23888/pavlovj2020282135-142","DOIUrl":"https://doi.org/10.23888/pavlovj2020282135-142","url":null,"abstract":"Background . Glycoprotein-P (Pgp, АВСВ1) is a transporter protein participating in pharmacokinetics of medical drugs, and also in development of resistance of tumor cells to chemotherapy. Aim . To study the influence of progesterone on the activity of Pgp in vitro on a cell model of human small intestinal epithelium. Materials and Methods . The work was conducted on Caco-2 cells. The activity of Pgp was eva-luated by transport of fexofenadine in a special transwell-system. Concentration of fexofenadine was analyzed by HPLC method. The amount of Pgp was determined by EIA method. Four series of experiments were conducted: control – cells preincubated with clean transport medium without addition of any substances; influence of rifampicin on the activity and synthesis of Pgp in the concentration 10 µmol/l in preincubation for 3 days (induction control); influence of progesterone on the activity of Pgp in concentrations 1, 10 and 100 µmol/l in preincubation for 30 min; influence of progesterone on the activity and synthesis of Pgp in concentrations 1, 10 and 100 µmol/l in preincubation for 3 days. Results . Progesterone in the concentrations 1 and 10 µM in incubation with cells within 30 minutes did not show any reliable influence on the activity of Pgp, however, in concentration 100 µM it reduced the activity of the transporter protein. In incubation of Caco-2 cells with progesterone in concentrations 1, 10 and 100 µM within 3 days the activity of Pgp remained unchanged. Progesterone in concentration 100 µM in incubation within 3 days significantly increased synthesis of Pgp in enterocytes by 114.3% as compared to control, and in other used concentrations (1 and 10 µM) it produced no reliable effect. Conclusion . In in vitro experiments on Caco-2 cells progesterone in concentration 100 µM produces a direct inhibiting effect on the activity of Pgp; however, in incubation within 3 days it increases synthesis of the transporter protein, which cancels out its inhibitory activity.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"13 1","pages":"135-142"},"PeriodicalIF":0.0,"publicationDate":"2020-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73651355","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The influence of in utero exposure to formaldehyde on newborn rat thymus structure","authors":"I. Vash","doi":"10.23888/pavlovj2020282143-152","DOIUrl":"https://doi.org/10.23888/pavlovj2020282143-152","url":null,"abstract":"Aim. To study the structure of the thymus of newborn rats born from female rats exposed to inhalation of formaldehyde during gestation. Materials and Methods. The work was carried out on 72 white outbred rats of the first day of postnatal development. The first group consisted of newborn rats (n=37) – the offspring of six rats that were exposed to formaldehyde (FA) in concentration of 2.766 mg/m 3 during the entire period of pregnancy. FA exposure was given once a day for 60 minutes in a 1 m 3 exposure chamber. The second group included control animals (n=35), which were kept in conditions similar to those of the experimental groups with the exclusion of the effect of FA. In the newborn rats, the body mass and the absolute and relative mass of the thymus were determined. The structure of thymus was studied in a light optical microscope. The number of cells in the cortex and medulla of the thymus was counted over an area of 2500 μm 2 . Results. The body mass and absolute mass of thymus of the newborn rats of the first group showed a statistically significant reduction against control values. The differences between the values of relative mass of thymus in the compared groups were insignificant. The number of cells in the cortex and medulla of the thymus under the influence of FA did not show any significant changes. Conclusion. The effect of inhalation of FA on the organism of pregnant rats during the entire gestation period consists in a decrease in the body mass and in the absolute mass of the thymus of newborn rats. With this, the relative mass of the thymus did not undergo significant changes. The thymus structure in the light microscope did not show any significant changes either.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"1 1","pages":"143-152"},"PeriodicalIF":0.0,"publicationDate":"2020-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73761483","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Experience of application of mechanochemical scleroobliteration in treatment for recurrence of lower extremity varicose vein disease","authors":"A. Gaibov, Okildzhon Ne’matzoda, Shakhnoza M. Burieva, E. Kalmykov","doi":"10.23888/pavlovj202028157-66","DOIUrl":"https://doi.org/10.23888/pavlovj202028157-66","url":null,"abstract":"Aim. Evaluation of the effectiveness of mechanochemical scleroobliteration in treatment for recurrent lower extremity varicose veins (VVD). Materials and Methods . A retrospective analysis of the results of examination and treatment of 19 patients (17 women and 2 men, average age 36.3±4.5 years) with recurrences of VVD, in whom mechanochemical sclerotherapy as the main method of treatment was used. In all cases, mechanochemical ablation of the superficial venous trunks was performed using Phlebogriph catheter. As a hardener, 3% sodium tetradecyl sulfate solution (fibro-vein) was used in the volume not more than 10 ml per procedure. Results . According to the CEAP classification, 15 patients had C2 and 4 patients had C3 class. Recurrence of VVD in one lower extremity was diagnosed in 17 (89.5%) patients, and bilateral – in 2 (10.5%). In 6 (31.6%) cases, recurrence occurred in 5 or more years after the first operation, in 8 (42.1%) patients – after 3-5 years, in 5 (26.3%) – after 1-3 years. The diameter of varicose veins before treatment according to color duplex scanning (CDR) was 7.9±0.8 mm. The duration of the detected saphenofemoral reflux (n=10) with the trunk of the great saphenous vein (GSV) left on the hip was 5.7±1.4 s, with the length 31.2±31.4 mm. With the preserved зtrunk of the GSV, crossectomy was performed in combination with mechanochemical scleroobliteration. Crossectomy was also performed in two patients with repeated dilation of the trunk of the small saphenous vein (SSV) followed by mechanochemical scleroobliteration. In two cases, insufficient shin perforants were ligated from mini-incisions, and in two more cases, foam scleroobliteration of them was performed. Within 3 weeks after the procedure, a good result was recorded in 94.7% of cases in the form of complete occlusion of sclerotized veins with the absence of reflux in them. Only in one observation incomplete occlusion of the sclerotized vein on the hip was noted, which required a repeated procedure. Long-term results were studied in 19 patients, in 94.7% of who complete obliteration of sclerotized veins and improvement of the clinical course of chronic venous disease were recorded. Conclusion . Mechanochemical scleroobliteration has proven to be an effective method of treatment for lower extremity VVD being a minimally invasive procedure.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"8 1","pages":"57-66"},"PeriodicalIF":0.0,"publicationDate":"2020-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81358071","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Succinate and succinate dehydrogenase of mononuclear blood leukocytes as markers of adaptation of mitochondria to hypoxia in patients with exacerbation of chronic obstructive pulmonary disease","authors":"E. Belskikh, O. M. Uryasiev, V. Zvyagina, S. V. Faletrova","doi":"10.23888/pavlovj202028113-20","DOIUrl":"https://doi.org/10.23888/pavlovj202028113-20","url":null,"abstract":"Aim . To study the concentration of succinate and the activity of succinate dehydrogenase (SDH) of mononuclear blood leukocytes as markers of rapid adaptation of mitochondria to hypoxia in patients with exacerbation of chronic obstructive pulmonary disease (COPD). Materials and Methods . The study involved 58 patients with COPD and 13 conventionally healthy volunteers of 40-75 years of age. In accordance with GOLD 2018 principles of complex assessment, the patients were divided to groups B (n=18), C (n=20), D (n=20) comparable in age, FEV 1 and in pack-of-cigarettes/year index. Patients of D group were characterized by more pronounced hypoxemia. Activity of SDH and concentration of succinate were determined in mononuclear leukocytes isolated from blood. Results. Patients with exacerbation of COPD divided to groups on the basis of the frequency of exacerbations and evidence of symptoms, were characterized by different severity of disorders of mitochondrial functions of mononuclear leukocytes. Patients of C group had the highest succinate concentration (428 [357;545] nmol/10 6 cells in I ml of suspension) and SDH activity (64[56;73] nmol of succinate/min * 10 6 cells of 1 ml of suspension) in mononuclear leukocytes as compared to groups B (1.43-times reduction of succinate, p<0.002; 1.88-times reduction of SDH, p=0.0015) and D (2.06-times reduction of succinate, p<0.0001; 4.26-times reduction of SDH, p<0.0001). Patients of D group demonstrated the most pronounced reduction of markers of adaptation to hypoxia. Conclusions. A small amount of symptoms in exacerbation of COPD is associated with the highest parameters of the mechanism of rapid adaptation of mitochondria of mononuclear leukocytes to hypoxia. Existence of evident symptoms and frequent exacerbations in patents is associated with a severe frustration of mechanisms of adaptation of mitochondria to hypoxia.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"1 1","pages":"13-20"},"PeriodicalIF":0.0,"publicationDate":"2020-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73072036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A study of the main parameters of immunogenicity of Ultrix vaccine","authors":"O. Evdokimova, S. V. Afanasiev, O. Antonova, V. I. Konopleva, Ilya S. Gorelov, Alla P. Kruglova, V. Biryukov","doi":"10.23888/pavlovj202028121-29","DOIUrl":"https://doi.org/10.23888/pavlovj202028121-29","url":null,"abstract":"Aim . Clinical trial of Ultrix vaccine of OOO FORT manufacture containing different serotypes of influenza virus: H1N1 A/California/7/2009 (H1N1) pdm 09, H3N2 A/HongKong/ 4801/2014, NYMCX-263B(15/184) and B/Brisbane/60/2008 NYMCBX-35 (15/300) Victo-rialineage strains (epidemiological season of 2016) and H1N1 A/Michigan/45/2015 NYMCX-275 (16/248), H3N2 A/HongKong/4801/2014, NYMCX-263B(15/184) иB/Brisbane/60/2008 NYMCBX-35 (15/300) Victorialineage strains (epidemiological season of 2017). Materials and Methods. A study of the basic parameters of immunogenicity included determination of the geometric mean value of the antibody titer, of seroprotection and seroconversion and of relative number of individuals with 4-fold increase in the antibody titer after vaccination. Immunogenicity was determined by a micromethod in hemagglutination inhibition reaction. Sera were tested with diagnosticums obtained from serotypes of influenza virus identical to vaccinal strains. Results. The level of seroprotection with Ultrix made 91.7-95.8% (2016) and 93.8-97.9% (2017). The maximal level of seroprotection was achieved in 6 months after vaccination with Ultrix containing H1N1A/California serotype. 2.55-4.36-Fold increase in the geometric mean value of anti-HA to all vaccinal strains was found in vaccination in 2016 and 2017, and 4-fold increase in antibody titer in more than 70% of volunteers on the 21st day after the first immunization in 2016. Conclusion. The obtained results of clinical trials of Ultrix vaccine with different antigenic composition confirms the correspondence of the immunogenicity parameters of the drug to the requirements of the Committee for Proprietary Medical Products (CPMPEMEA, CPMP/ EWP/1045/01) and of State Pharmacopoeia of RF of XIII edition (SP SP XIII).","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"19 1","pages":"21-29"},"PeriodicalIF":0.0,"publicationDate":"2020-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74867716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Physical development of primary school-age orphan boys with mental retardation","authors":"E. S. Ovcharenko, V. V. Fefelova, E. Kasparov, T. P. Koloskova, O. Smirnova, I. Ignatova","doi":"10.23888/pavlovj202028130-36","DOIUrl":"https://doi.org/10.23888/pavlovj202028130-36","url":null,"abstract":"Mental retardation is considered one of the most common pathologies of children's mental development. Parameters of physical development are actively used as informative markers of health status, social and hygienic well-being both in norm and in various pathologies. At the same time, not enough attention is given to orphans with intellectual disabilities in this context. Aim. To study the parameters of physical development in primary school-age boys with mental retardation brought up in a specialized orphanage. Materials and Methods . 34 Boys (7-11 years old) were examined. Of these, 20 children with a diagnosis of moderate mental retardation (F71) were brought up in a specialized children's home for mentally retarded children (without parental care). The control group included 14 intellectually healthy boys of the same age who were brought up in an orphanage of a physiological type. Anthropometric parameters (body length, body weight, chest and head circumference, cross-section diameter of the chest), ‘sthenia’ index, level of physical development, and Quetelet II index were studied. Results. The data obtained indicate a sharp decrease in parameters that characterize physical development of orphan boys with mental retardation in comparison with intellectually healthy orphan boys. Since children were in the same social and hygienic conditions, the identified features may be due to the combined influence of the presence of deviations in the intellectual deve-lopment of children and upbringing in a residential home. Conclusion . The results obtained determine the need for closer medical and hygienic support for orphans with mental retardation.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"22 1","pages":"30-36"},"PeriodicalIF":0.0,"publicationDate":"2020-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82472359","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiomyopathy in children – clinical, genetic and morphological aspects","authors":"O. Saryeva, L. Kulida, E. Protsenko, M. V. Malysheva","doi":"10.23888/pavlovj202028199-110","DOIUrl":"https://doi.org/10.23888/pavlovj202028199-110","url":null,"abstract":"Cardiomyopathy is one of serious and complex problems of pediatric cardiology. Many of them are the cause of sudden death and are familial in character. Disappointing statistics increases the relevance of the problem of cardiomyopathy and dictates the need for in-depth study of the etiology and pathogenesis, structural bases and experience in clinical and morphological diagnosis of this pathology in children. Of particular importance from a practical point of view is the development of prognostic factors in primary and secondary cardiomyopathies. This literature review provides information on the etiology, pathogenesis, clinical manifestations, pathomorphological changes and outcomes of such cardiomyopathies as hypertrophic, dilated cardiomyopathies, non-compact left ventricular myocardium and histiocytoid cardiomyopathy. Peculiarities of restructure of the myocardium in the analyzed cardiomyopathies and their relationship with systolic and diastolic myocardial dysfunction are shown. Molecular genetic aspects of diagnosis of etiology and pathogenesis of this pathology in children are given in detail. The necessity of systematic pathomorphological study of the heart with full analysis of contractile, conducting microcirculatory and neuroautonomic structures in considered variants of cardiovascular pathology is emphasized. These data will help outline future research priorities for this group of diseases to provide earlier diagnosis, improve clinical outcomes and the quality of life of these children and their families.","PeriodicalId":13184,"journal":{"name":"I.P.Pavlov Russian Medical Biological Herald","volume":"227 1","pages":"99-110"},"PeriodicalIF":0.0,"publicationDate":"2020-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85013917","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}