Maternal diabetes mellitus (DM) is a chronic disease that carries significant risks for the fetus and newborn. Hyperglycemia during pregnancy contributes to the development of structural heart defects, myocardial hypertrophy, and impaired cardiac function in newborns. The objective of the study was to evaluate the echocardiographic parameters of the cardiac function in children born to mothers with type 1 diabetes mellitus (DM1).A prospective study of 163 newborns was conducted on the basis of the Republican Scientific and Practical Center “Mother and Child”. The children were divided into 2 groups. The main group consisted of 107 children born to mothers with DM1 (Gr1), the control group – 56 healthy infants born to women with normoglycemia during pregnancy (Gr2).It was found that a newborn in Gr1 has a larger thickness of the interventricular septum (IVS) (p < 0.001), the posterior wall of the left ventricle (p < 0.001), and the size of the right ventricle (p < 0.001). It was revealed that in Gr1 the frequency of myocardial hypertrophy with an interventricular septum thickness of 5 mm or more is 30.8 %, which is statistically significantly higher in comparison with Gr2 (1.8 %, F = 0.115, p < 0.001). When assessing the blood flow using a pulse-wave Doppler study, the manifestations of the diastolic dysfunction in Gr1 were revealed. The E/A ratio for the   flow through the mitral valve in Gr1 was 0.9 (0.8–1.1), in Gr2 – 1.2 (1.0–1.4), p = 0.043. The study confirmed a lower frequency of registration of myocardial hypertrophy with good glycemic control during pregnancy (p < 0.05). Direct correlations between the average level of glycated hemoglobin in mothers and the IVS thickness (r = 0.374, p < 0.05) and the posterior wall of the left ventricle (r = 0.293, p < 0.05) of their newborns were observed in infants in Gr1.The use of pump insulin therapy in pregnant women with type 1 diabetes allowed a statistically significant reduction in the frequency of hypertrophy of the left ventricle in infants in the neonatal period.Diabetes mellitus type 1 in the mother determines not only the risk of congenital heart defects, but also hypertrophic changes in the myocardium, contributes to the diastolic dysfunction in this group of children. The achievement of target levels of glycated hemoglobin during pregnancy, using pump insulin therapy, provides a lower frequency of registration of ventricular septal hypertrophy. The revealed statistically significant differences in the IVS thickness and the posterior wall of the left ventricle in infants born to mothers with type 1 diabetes should be taken into account by doctors in the system of organizing medical care in inpatient settings and at the stage of dispensary observation.

Congenital deficiency of biotinidase is a rare congenital metabolic disorder that most often occurs in newborns and young children. Since the activity of the enzyme in the nervous tissue is low, it is the most sensitive and vulnerable, which is manifested primarily by muscle hypotension, convulsions, ataxia, and dermal disorders. Clinically, there are two forms of biotinidase deficiency, which are determined by the residual activity of the enzyme: early (deep deficiency), late (partial). The leading laboratory tests are the detection of metabolic acidosis in the patient, the study of urine for organic acids, low enzyme activity, hyperammonemia, and the detection of mutations in the sequencing of the BTD gene. The features of neurological disorders in biotinidase deficiency are analyzed on the example of a clinical case and a review of the literature. Early diagnosis and administration of biotin therapy play an important role in preventing disease progression and clinical signs.

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The purpose — to identify the key predictors of neonatal hypoglycemia in children born to mothers with type 1 diabetes mellitus (DM1) and to develop predictive models to determine the likelihood of early hypoglycemia. Material and methods. We examined 130 children of mothers with DM1, who were born and treated in the «Mother and Child” Republican Scientific and Practical Center in Minsk. The main group consisted of 101 infants born to mothers with pregravid DM1. The patients were divided into group 1 (Gr1, n = 56) with diagnosed early hypoglycemia and group 2 (Gr2, n = 45) without hypoglycemia. Results. The most significant clinical and biochemical predictors associated with the risk of hypoglycemia in the first hours of life of infants are insufficient compensation for DM in women (the average level of HbA1c in the 2–3rd trimesters is more than 6.5%), levels of cholesterol, triglycerides and LDL-cholesterol in the mother’s serum on the eve of childbirth, the value of the z-score of the child’s body weight at birth, the level of cord blood leptin, the presence of signs of a perinatal infectious disease in the child. Mathematical models of the probability of early hypoglycemia in newborns of mothers with DM1 were developed. The control of the models’ correct operation was carried out on a sample of 29 children of women with DM. The high reproducibility of the developed prognostic models was established. Conclusion. The predictive accuracy of models for determining the likelihood of early hypoglycemia in newborns of mothers with DM1 was 83.1% (95% CI 74.1–92.0) and 83.2% (95% CI 74.1–92.3), which justifies the expediency of their use in healthcare organizations offering perinatal care of various levels.

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