The physical and neurological development of children born to mothers with gestation diabetes mellitus (GDM) is a matter of research interest. We examined 28 children whose mothers were diagnosed with GDM, in the newborn period and at 6 months. By the parameters of physical development at birth the number of children with outstripping development and the average body mass index (BMI) was predominant and by the end of the first semester of life most of the children had the values of the average parameters specified above, that is, normal for that age, range, but the proportion of the deficient BMI remained unchanged. Insulin therapy during pregnancy had no significant impact on the frequency characteristics of children with physical development disorders. The neurological disorders of the six-month-old children born of women with gestation diabetes mellitus and non-targeted metabolic control were more severely represented by sensory sphere disturbances and interaction with subjects; to a lesser extent - by emotional and voice development. The children of this group had the minimum disturbances in the development of the motor sphere.

Introduction. Hyperinsulinemic hypoglycemia in children is most commonly due to congenital hyperinsulinism. When hyperinsu-linemia is accompanied by fasting hypoglycemia and postprandial hyperglycemia, rare syndromes of severe insulin resistance, which include Rabson - Mendenhall syndrome, should be suspected. This article provides an analytical review of current data on this rare genetic pathology and presents a clinical case of a previously undescribed combination of Rabson-Mendenhall syndrome with mutations in the insulin receptor gene INSR in the compound heterozygous state with multiple congenital anomalies of other organs.Clinical case. Patient N, 5.5 months old boy, with suspected congenital hyperinsulinism due to episodes of frequent severe hypoglycemia from the first day of life. At the age of 5 months, an episode of hypoglycemia up to 2.2 mmol/L was registered at an appointment with a pediatric endocrinologist. An examination was ordered, which found that against a background decrease in blood glucose to 1.9 mmol/L, C-Peptide level >5000 ng/mL, insulin level >300 lU/mL, cortisol - 971 nmol/L, TSH -3.88 mlU/L, free T4 - 10.53 pmol/L (10-23.2).The importance of early diagnosis of severe insulin resistance to prevent developmental disorders in children is emphasized. The issue of organizing multiple effective monitoring of a patient’s glycemia required special attention in this clinical case. Due to the features of metabolism in young children, we abandoned flash glucose monitoring systems and used a modern glucose meter with an integration program with a mobile application and the ability to generate reports for subsequent analysis as a reliable means of glycemic control.Summary. Based on the results of the genetic study in association with the clinical phenotype, age of debut, the patient was clinically diagnosed with Rabson-Mendenhall syndrome.Discussion. The paradoxical nature of glycemic fluctuations (severe fasting hypoglycemia and postprandial diabetic hyperglycemia) is quite typical for syndromes of severe insulin resistance and should draw the attention of an informed primary care physician.Conclusion. Careful attention to the symptoms of hypoglycemia, especially with a debut in the neonatal period, recurrent episodes, and the severity of the decrease in blood glycemia. If normal or elevated levels of insulin and C-peptide are detected against the background of hypoglycemia, the first thing to think about is congenital hyperinsulinism.

Backgraund: Gestational diabetes mellitus (GDM) is one of the most common metabolic disorders found during pregnancy. Currently, it is relevant not only to search optimal target levels of glycemia during pregnancy, but also to study the ­effect of different glycemia levels on fetal development and further changes in glucose and lipid metabolism in children.Aims: To describe perinatal period, physical development and metabolic status of children born to women with GDM and different glucose levels during pregnancy.Materials and methods: The perinatal period features and anthropometric parameters at birth were evaluated in 300 children born to women with GDM and different levels of glycemia during pregnancy. Over the course two years, 141 children have been evaluated for physical development parameters and glucose and lipid metabolism. Fasting and postprandial glycemia was measured with glucometer for 14 days in 33 children aged 1 to 4 years.Results: The anthropometric parameters of children at birth did not differ from the parameters of the control group (p> 0.05) when during pregnancy fasting blood glucose was less than 5.1 mmol / l and 7.0 mmol / l 1 hour after a meal. The glycemia in women above this level was associated with an increase of frequency and risk of a body mass index, body mass / length ratio and head circumference “above average” in children at birth (p <0.05). With the dynamic control of anthropometric parameters up to 2 years, no differences between the comparison groups were obtained (p> 0.05). The change in metabolic parameters was represented by neonatal hypoglycemia in children of GDM group (GDM group — 23%, control group — 3.5%, p = 0.000002), the least risk of which occurred in group with the lowest fasting and postprandial glycemic values during pregnancy. Fasting glucose, and insulin levels, НOMA index, triglycerides and cholesterol, as well as monitoring fasting and postprandial glycemia for 14 days, were obtained no significant differences between the comparison groups of children (p> 0.05).Conclusions: The lowest risks of neonatal hypoglycemia and anthropometric deviations at birth were associated with the lowest glycemia levels during pregnancy, which correspond to the criteria of the Russian clinical guidelines.

Background. Due to the increasing prevalence of abnormal uterine bleeding (AUB) among obese adolescent girls, a factorial analysis of the probable causes of this pathology is highly relevant. Aim. To analyze and characterize clinical, hormonal, metabolic features and parameters relevant to the assessment of ovarian reserve in adolescent girls with obesity and AUB. Materials and methods. The study included 2 groups of obese adolescent girls who have passed at least 12 months from the start of menarche, who do not have endocrine and severe somatic diseases; main group: adolescent girls with obesity (SDS BMI more than 2) and abnormal uterine bleeding of the type of opso-oligomenorrhea and secondary amenorrhea; comparison group: adolescent girls with obesity (SDS BMI more than 2) and absence of menstrual cycle disorders. Results. Among adolescent girls without AUB, severe forms of obesity (34 art.) were more common than in the group with AUB. According to the metabolic status, plasma triglyceride and cholesterol levels were significantly higher in the AUB group compared with adolescent girls without menstrual disorders (p=0.035 and 0.043, respectively).When assessing carbohydrate metabolism, no significant differences between the groups were obtained (p=1.000). According to the results of the study of the hormonal status, no significant differences between the groups were obtained. The hirsute number had no significant differences in the groups. The correlation analysis revealed a positive correlation between the severity of obesity with the HOMA index (p=0.034), a positive correlation of the prolactin level with the hirsute number (p=0.021) and estradiol (p=0.04). The median level of anti-muller hormone in the group with AUB was significantly higher compared to the group without AUB (6.13 ng/ml vs 2.09 ng/ml, p0.05). When analyzing the follicular apparatus of the ovaries, it was found that the median volume of both the right and left ovaries in girls of group 1 with obesity and the AUB was larger than in the comparison group. The median number of follicles in the section in group 1 also indicated a greater number of them compared to the comparison group. Conclusion. In the studied groups, girls with a more severe degree of obesity were more often associated with the absence of AUB, but metabolic disorders were more common in the group of girls with AUB. When assessing the ovarian reserve, a direct correlation was obtained between the level of anti-muller hormone and the number of antral follicles in the ovary.

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