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Vol 19, No 2 (2020)
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SOCIAL PEDIATRICS AND HEALTH CARE

96-106 3010
Abstract

The reasons and dynamics of child mortality in Russian Federation during the XXI century are presented. The data of official statistics from Rosstat, World Bank and World Data Atlas have been analyzed. The following medical statistical regularities have been revealed: reduction of child mortality rate in the structure of total number of deaths in Russian population and infant mortality rate in the structure of child mortality; reduction of regional varieties of infant mortality rates; continued transfer of live births to stillborn births in some regions; external factors are also very significant not only in adolescents but also in infants. Priority areas for further reduction of child mortality in Russian Federation are provided: regional approach in the implementation of measures for reduction of child mortality; preventive measures aimed on reducing of genetic burden in population; full supply on the management of children with rare diseases by the government; establishment of the system for medical and social rehabilitation of children; implementation of the system of effective medical and social care for children with high social risk.

REVIEW

107-115 1699
Abstract

Various aspects of muscle strength decrease and loss of motor skills in patients with cerebral palsy (CP) are considered in this review. Pathophysiological mechanisms caused by primary damage and reorganization of central nervous system (CNS), structural-functional changes of muscle tissue, biomechanical disbalance are described. Changes at patients with CP equate with sarcopenia (physiological process of muscle strength and function loss in elderly patients). It has been shown that patients with cerebral palsy have such phenomenon as «early aging» of musculo-skeletal system. Potential approaches for prevention of sarcopenia in children and adults with cerebral palsy are discussed. Understanding of the described mechanisms of primary and age-related changes in muscle tissue in early CNS injuries is necessary to plan the activities of daily living for patients, choose the correct rehabilitation tactics, minimize adverse therapeutic effects, and reasonable management of comorbid conditions.

116-122 18802
Abstract

There is crucial question on further therapeutic tactics for the management of such patients (within the severe epidemiological situation caused by the pandemic of new coronavirus SARS-CoV-2) due to widespread application of genetically engineered biologic drugs (GEBD)in management of immune mediated inflammatory diseases. This review sums up current data on the pathogenesis of COVID-19 with the development of acute respiratory distress syndrome associated with cytokine release syndrome (cytokine storm). The effects of GEBD on COVID-19 pathogenesis and their role in management of COVID-19 severe forms of are considered. This review presents the latest guidelines of international associations/consensus and observations of doctors of various specialties on the issue of interruption/continuation of GEBD therapy and with further consequence evaluation in case of interruption of biological therapy.

123-131 2308
Abstract

First cases of new coronaviral infection were reported in Wuhan, Hubei Province, China in December 2019. The infection had caused the death of more than 100,000 people worldwide by the beginning of April 2020. This review analysed the characteristics of the infection course in adults and children, as well as capabilities of diagnostics, treatment and prevention of COVID-19. It is possible to allocate groups of high risk of development of severe forms of disease (elderly people, people with latent tuberculosis infection, adult patients with severe comorbidity) according to the published data. Currently there is limited epidemiological data on the prevalence, morbidity and mortality of COVID-19 in the child population. However, it is already possible to conclude that 90% of cases in children population have mild, moderate and asymptomatic course of the disease. Treatment of patients with COVID-19 is limited due to the lack of means for etiotropic therapy and the possibility of using of only symptomatic therapy. There are no vaccines for COVID-19 prevention.

CLINICAL OBSERVATIONS

132-141 1156
Abstract

Mucopolysaccharidosis type I mild forms include Scheie syndrome and Hurler-Scheie syndrome that are characterized by slow progression, intact intelligence, and primarily effect on visual organ, musculoskeletal and cardiovascular systems. Early diagnostics, multidisciplinary approach to examination and monitoring, timely management are crucial in maintenance of patients' quality of life, preventing complications development and early disability. The article provides the overview of published data and description of 3 clinical cases with mild course of mucopolysaccharidosis type I.

EXCHANGE OF EXPERIENCE

142-145 1209
Abstract

Congenital cleft lip and palate is one of the most common congenital anomalies in children. It has significant medical and social influence on the self-realization of these patients in society. This review of various researches' results from different countries and territories of the Russian Federation (according to the MEDLINE and eLIBRARY.ru databases) provides information on prevalence rate of this pathology among children of our country and worldwide. The role of medical care organization remains unknown among all the presented results of the studies about the role of risk factors of congenital cleft lip and palate development (such as: inheritance, lifestyle, environment).

146-149 1445
Abstract

The article describes genetic factors associated with toe walking in children. Association of gait abnormality with variants in genes listed below is shown: PMP22 (peripheral myelin protein 22), EGR2 (early growth response protein 2), AIFM1 (apoptosis inducing factor mitochondria associated 1), MORC2 (member of the Microrchidia family CW-type zinc finger 2), DHTKD1 (dehydrogenase E1 and transketolase domain containing 1), GDAP1 (ganglioside induced differentiation associated protein 1), KIF1B (kinesin family member 1B), FGD4 (FYVE, RhoGEF and PH domain containing 4), SBF2 (SET binding factor 2), SH3TC2 (SH3 domain and tetratricopeptide repeats 2), NAGLU (N-acetyl-alpha-glucosaminidase), NEFL (neurofilament light) и PRX (periaxin). Genetic testing of patients with toe walking is crucial for accurate diagnostics of this pathology as well as clinical examination.

150-161 1170
Abstract

The localized scleroderma (morphea) is the clinical option of the juvenile scleroderma, the third in prevalence rheumatic condition in pediatrics. The article summarizes all the data on the classification, diagnostics, and differential diagnosis of juvenile localized scleroderma. The recent international guidelines on the localized scleroderma in pediatrics (the European consensus of pediatric rheumatologists, the German and Japanese national guidelines) are presented in the article.

162-167 889
Abstract

Meningococcal disease is the urgent problem of healthcare due to its widespread prevalence, high incidence, severe course and high risk of mortality. Vaccination of Russian Federation population with polyvalent meningococcal vaccine for serogroups A, C, W, Y has not been studied before within the framework of regional immunization programs. The article presents the results of epidemiological analysis of the situation on meningococcal disease in Tyumen region and Yamalo-Nenets autonomous district (YNAD) in 2014-2019. The obtained data were used to develop the immunization strategy against meningococcal disease within the framework of the regional immunization calendar in Tyumen region and regional immunization programs in YNAD. The clinical case of severe course of meningococcemia with complications is presented in the article. Clinical conditions and syndromes which are common for practice of therapists during the management of patients with generalized forms of meningococcal disease are described.

INFORMATION FROM THE UNION OF PEDIATRICIANS OF RUSSIA



ISSN 1682-5527 (Print)
ISSN 1682-5535 (Online)