Formative experience of establishment and development of legal support for maternity and childhood in USSR is considered over time and systematized. The role of measures taken by the socio-legal cabinet, as well as social patronage as the method of dynamic preventive identification of families with children who are in difficult circumstances. The author's vision on the accumulated experience significance in solving modern problems of protecting the child health and rights is presented.

Neuropsychological tests can be used to diagnose post-surgery cognitive dysfunction in children. These tests are characterized by attractiveness, accessibility and short duration. Burdon Attention Test, Raven's matrices, various modifications of words memory tests meet all these requirements. Comprehensive assessment of cognitive functions (including MMSE scale and batteries of computer tests) seems to be optimal.

Systemic onset juvenile arthritis is the complex autoinflammatory disease with multigenic type of inheritance. It is characterized by significant systemic manifestations and unfavourable adverse events, among which macrophage activation syndrome and pulmonary involvement are playing crucial role. This article presents data on the frequency and features of the disease course, possible development predictors and clinical-radiological picture of pulmonary involvement in systemic onset juvenile arthritis. Possible factors (including hereditary) affecting the process of pulmonary involvement development and the role of genetically engineered biologic therapy are considered.

Background. The transition of adolescents with chronic diseases from pediatric to adult health service is often accompanied by a decrease in adherence to the recommendations, non-regular scheduled medical consultations, disease course monitoring violation. All together it can lead to exacerbation and progression of the chronic disease and a decrease in quality of life. Programs for patients with nephrological diseases that optimize the process of their transition to adult service can improve the course of chronic kidney disease (according to international experts). Objective. The aim of the study was to systematize data from the published literature on the transition of adolescents with chronic kidney disease to adult health service; identify gaps in scientific knowledge; and determine areas for future research. Methods. Scoping review was performed by searching for articles in the databases: PubMed, eLIBRARY.RU, The Cochrane Library, via Google Scholar and in reference lists of selected articles. We have selected articles published in Russian or English with no publication date limitation and corresponding to inclusion criteria. Results. 185 (8.5%) out of 2,184 identified literature sources were included in the study. The results of 25 works were included in the analysis of transition programs efficacy. There were no Russian-language articles matching the inclusion criteria. The majority of publications types were original scientific studies (retrospective observational studies, questionnaire surveys, qualitative and mixed methods studies with interviewing) and narrative reviews. It was shown that both complex programs and narrow-focus interventions have been implemented to improve the transition process. It has been noted that the efficacy of such initiatives still remains insufficiently studied, including among experimental studies. Conclusion. Formation of the scientific knowledge system on the transition of patients with chronic kidney disease to adult health service requires high-level evidence. Further researches are needed in this field in the Russian health system as well.

Background. Searching for new strategies for the rehabilitation of adolescents with obesity and comorbid arterial hypertension (AHT) before significant pathological changes development in the cardiovascular system remains the urgent challenge. Objective. The aim of the study was to examine the effect of interval hypoxic training (IHT) on blood pressure (BP) levels in adolescents with overweight / obesity and comorbid AHT. Methods. Adolescents aged 14-17 years with body mass index SDS ≥ 1 and grade I AHT (mean level of systolic and/or diastolic BP ≥ 95th percentile for population of corresponding age, sex and height) have been randomized to the group «aerobic training» (treadmill walking) and “aerobic training + IHT” (usage of hypoxicator in intermittent operation cycles). All patients were on subcaloric diet (10% reduction in caloric intake for given age). Primary outcome measure was the difference between groups on systolic/diastolic BP levels according to 24-hour BP monitoring after completion of training program (10 classes each). The effects of IHT on body composition (bioelectrical impedance analysis), heart rate variability and psychoemotional state (Spielberger scale, assessment of health, activity and mood) were further estimated. Results. 43 patients were assigned to the index group, and 42 — to the control group. 67 patients have completed the research program. 5 patients (12%) from the IHT group and 13 patients (31%; p = 0.029) from the control group prematurely discontinued participation in the trial due to poor exercise tolerance. The groups were comparable in baseline systolic and diastolic BP. Decrease in BP occurred in both groups after 10 workouts. There were no differences in decrease value: mean difference for SBP was 2.4 mm Hg (95% CI -6.6 ... 1.8), for DBP — 0.2 mm Hg (-3.6 ... 4.0). However, the incidence of reaching the targeted SBP (< 95th percentile for the corresponding age and sex) after completion of the treatment was recorded in 66% patients in the experimental group and in 42% patients in the control group (p = 0,047). Positive dynamics in several indicators of heart rate variability and psychoemotional state were mentioned in the IHT group. Conclusion. The IHT implementation in the complex of rehabilitation program for adolescents with overweight / obesity and AHT has no additional positive effect on BP levels. However, the incidence of reaching the targeted SBP (< 95th percentile for the corresponding age and sex) after completion of the treatment was recorded in 66% patients in the experimental group and in 42% patients in the control group (p = 0,054).

Background. Recently, there has been an increase in the incidence of pertussis in unvaccinated, as well as vaccinated children and adults. The major risk group for pertussis are infants and children over 4 years of age due to gradual decrease in basic immunity. Objective. The aim of the study was to analyze postvaccinal immunity against pertussis in individuals aged 3 to 25 years in Krasnodar. Methods. The study was carried out in the period between July 2018 and October 2019 and included patients aged 3 to 25 years with completed (according to National Immunization Schedule) immunization against pertussis with no history of pertussis in the past. Postvaccinal immunity against pertussis in this sample was determined by the ratio of study participants with minimum protective antibodies titer to Bordetella pertussis (≥ 1:160). Results. The minimum protective antibody titer was revealed in 24 out of 76 participants. Distribution to age groups was the following: children aged 3 to 7 years — 2/28 (7%), 8 to 17 years — 13/22 (59%; p < 0.001, compared with the younger age group), ≥ 18 years — 9/26 (35%; p = 0.017). There was no postvaccinal immunity (no antibodies detected) in 6 (21%), 1 (5%) and 4 (15%) participants, respectively. Conclusion. Insufficient postvaccinal (humoral) immunity against pertussis was revealed in majority of children under 7 years. The ratio of such children decreases with age. It suggests the presence of hidden circulation of Bordetella pertussis and, as a result, previous medical condition in erased or abortive form.

Background. KID syndrome (keratitis-ichthyosis-deafness) is an orphan genetic multisystem disease with autosomal recessive and dominant types of inheritance, it manifests in the neonatal period. The leading triad of symptoms is: skin lesions, ophthalmological diseases and hearing organ pathology. Clinical Case Description. Girl V., 17 years old, with KID syndrome applied to the hospital complaining on painful infiltrates of the parietal region. Multiple abscesses were lanced. Hyperkeratotic crusts were removed, unviable skin regions were excised, and abscesses' cavities were washed with antiseptic solution during daily dressings. Purulent discharge from wounds has been maintaining for 7 days. Conclusion. Where is no pathogenetic treatment for KID syndrome yet. Prevention of secondary surgical infections remains the crucial aspect in the management of such patients. Local wound treatment, symptomatic and antibacterial therapy are effective in case of skin infection.

Background. Mucopolysaccharidosis (MPS) type II is an X-linked hereditary disease from the lysosomal storage diseases group. The absence or malfunctioning of the iduronate-2-sulfatase enzyme leads to disruption in glycosaminoglycans (GAG) degradation. The GAG accumulation in lysosomes leads to dysfunction of various tissues and organs. There are neuropathic (severe) and non-neuropathic (relatively mild) forms of the disease. Early diagnostics of non-neuropathic form of MPS II is quite difficult due to slow disease progression and because early symptoms are hard to notice. Clinical Cases Description. This article describes three clinical cases of non-neuropathic form of MPS II. Patients had following symptoms: non-rough facial features changes (gargoylism), hepatomegaly, hernias, recurrent otitis, non-inflammatory joint contractures, and cardiac valves involvement. The children height conformed to the age or was little lower than reference values. The described form of MPS II is characterized with the absence of cognitive impairment. Conclusion. Enzyme replacement therapy efficacy has been shown via disease progression slowing down, significant improvement of clinical symptoms and quality of life.

Background. Defects in pyruvate dehydrogenase complex (PDC), involved in the glycolysis products integration into the cells' energy metabolism, are one of the reasons of mitochondrial pathology development. The diagnosis of this condition can be pretty complicated also due to the lack of description of such patients with encephalomyopathy associated with PDC deficiency in Russian population. Clinical Cases Description. We have performed the analysis of clinical manifestations polymorphism of progressive mitochondrial encephalomyopathy caused by pathogenic variants in nuclear X linked gene, PDHA1 (encodes alpha subunit of pyruvate dehydrogenase), in 8 boys aged from 1 to 8 years. The adverse perinatal period was mentioned in all cases. The major features of symptom complex by the time of hospital examination were psychomotor retardation, ataxy, myopathic manifestations. Dystonic attacks were observed in 2 sibs. All patients had changes on brain magnetic resonance imaging: in basal ganglia in 6 children and ventriculomegaly in 2 children. All children had lactic acidosis. Clinical examination has shown that 4 patients had severe damage of nervous system, other 4 patients had moderate damage. Missense mutations in the PDHA1 gene were revealed in 6 children, insertions and duplications including 6 and 16 base pairs — in 2 children. The moderate positive dynamics was noticed as a result of complex treatment of children: stabilization of the overall condition, no metabolic crises, decrease in frequency of dystonic attacks. Conclusion. The clinical polymorphism of mitochondrial encephalomyopathy associated with PDC deficiency is described. The differences in manifestations of severe and moderate forms of disease are shown. The presented description may be useful for medico-genetic counseling and providing medico-genetic care for families.