Glycogen storage disease is the hereditary carbohydrate metabolism pathology which is caused by mutations in various genes encoding enzymes responsible for glycogenesis and glycogenolysis. Excessive glycogen deposition in various tissues cells (mostly in liver and muscles) occurs due to enzyme defects. The authors present recent epidemiological data and features of glycogen storage disease etiology and pathogenesis. Clinical characteristics of different types of this disease are also presented. The data on laboratory-instrumental and morphological signs of glycogen storage disease in children, as well as data on its treatment methods is provided in accordance with the developed clinical guidelines. The article provides relevant information on disease types with predominant liver involvement, besides the variety of clinical forms of glycogenosis.

The article summarizes the materials of modern publications on cognitive development of premature infants in connection with perinatal factors and parenting conditions. Leading risk predictors of cognitive defects in premature infants are severe dysmaturity by the time of birth (gestational age <27 weeks) and need for intensive care during the first weeks of life. The data of longitudinal researches of the premature infants’ development until reaching their adulthood is presented. The structure of cognitive defects in this population is studied. The most common problems were revealed in learning mathematics, operational memory and purposeful behavior and activity. Frequency of these cognitive defects is associated with both: stage of prematurity social problems of the family. Modern neurovisualization methods (diffusion weighted imaging and functional magnetic resonance imaging (MRI) of the brain) allows to identify the defects in child nervous system (Connectome) development already at the age of 18 months. It can be the substrate of cognitive defects, and it will allow to predict individual development pathway and implement direct corrections and interventions.

The review of the literature is mostly based on its own research and is devoted to the influence of natural (bottomland, 28 m below sea level, climate conditions with severe changes in temperature during the year, biogeochemical province with moderate iodine deficiency) and anthropogenic (increased air, water and soil pollution) environmental factors on children’s health in the Astrakhan region. The impact of the Astrakhan gas condensate field on the health condition is shown: growing number of children with physical and psychomotor disorders, imbalance in cellular and humoral immunity factors, growing number of frequently ill children with problems in adaptation to kindergartens and schools. The influence of vehicles on environmental pollution, frequency of allergic diseases among children (asthma, allergic rhinitis, atopic dermatitis), and degraded quality of life was noted. There is the negative situation with water sources: samples from main Volga riverbed show both non-organic and organic contamination. Contamination of drinking water is considered as serious factor increasing the incidence of atopic pathology. The correlation between the prevalence of atopic dermatitis and soil contamination with benzapyrene on the territory of these children residence was revealed. Complex influence of iodine deficiency and adverse anthropogenic factors leads to abnormal children’s blood composition: development of erythrocytosis, leukocytosis, inhibition of hemoglobin synthesis function, lymphopenia. The incidence of diffusenodular endemic goiter, subclinical hypothyroidism and thyroiditis can increase as well. It is necessary to develop prevention and rehabilitation measures for children diseases associated with combined adverse effect of natural and anthropogenic factors in the Astrakhan region.

Atopic dermatitis (AD) is one of the common multifactorial inflammatory diseases manifesting predominantly in childhood. There is significant number of cases of self-regression of the disease with aging. On the other hand, there is also another scenario ending with AD persistent course and/or development of comorbid allergic pathologies that can significantly worsen patient’s quality of life and finally lead to social maladjustment. The pathogenesis of such way includes epidermal barrier disturbance, transcutaneous sensibilisation and aberrant allergic (Th2) immune systemic response development. Main role in preventing of this pathological pathway is lying on the new class of moisturizers containing active components "emollients plus". They are considered as foundation for the therapy and prevention of the development of AD and other allergic diseases. This literature review provides relevant data on AD pathogenesis and development of comorbid allergic pathologies. This paper also covers data on the effect of emollients in restoration of the epidermal barrier and their use as preventive measures.

Unilateral laterothoracic exanthem (ULE) is relatively rare self-limited pediatric skin inflammatory disease; its etiology still remains unknown. The diagnosis can be established due to specific clinical findings: presence of spotted and papular rash on one side of the body, more often in the axillary or inguinal area with possible generalization. This article presents the clinical case of ULE in the girl (4 years 8 months), it shows the dynamics of exanthematous syndrome at this pathology on different stages of the disease course.

Background. The incidence of sepsis among newborns ranges from 1–12 to 38 per 1,000 live births in the world according to scientific literature [1, 2]. The clinical case demonstrates the features of the newborn organism sensitivity and the therapeutic and diagnostic process difficulties.

Clinical case description. The boy was born on the 37th week of gestation, 1st of twins, with body weight 3330 g, height 51 cm, APGAR score 8/9 points. He was on breastfeeding. His condition has deteriorated rapidly on the 9th day of life. The condition was severe due to intoxication syndrome, necrotising enterocolitis (NEC) manifestation, further development of systemic inflammatory response syndrome and multiple organ dysfunction syndrome. Severe pain syndrome and movement restraint in limbs have appeared on the 20th day of life. X-ray imaging: NEC signs, multiple osteomyelitis foci in the limbs.

Conclusion. Modern adequate diagnosis and justified treatment tactics have led to positive outcome: child’s condition has improved, body weight has increased, pain syndrome has been managed, the volume of movements in the limbs has increased, inflammatory markers have stabilised. The child was discharged from hospital in satisfactory condition at the age of 2 months.