No abstract

It was studied the state of the vascular wall at metabolic syndrome (MS) in children and adolescents. The study included two groups of children: 45 people with MS and 25 patients with simple obesity. With the help of the device Vasotens assessed indicators of vascular stiffness: pulse wave velocity in the aorta, the indices of stiffness and augmentation of the peripheral arteries and the aorta, the maximum rate of rise in blood pressure. The study showed that 70% of children with MS increased arterial stiffness and thickness of the vascular wall. The rigidity of the vascular wall in children with MS revealed a 3.5 times more likely than children with simple obesity. Pulse wave velocity is a predictor of hypertension and has a high correlation with SDS IMT.

Pubertal impairment in adolescents is an urgent pediatric problem conditioned by progressive worsening of the population’s reproductive health in general. One of the variants of teenagers’ puberty disturbances is the delayed puberty (DP) as no secondary sexual maturation by the age of 14 years in boys. There are discriminated hypogonadism and constitutional DP, the latter being the most frequent form of the disorder of pubertal development in boys, accounting for 60-80% of all forms of DP. At the base of pathogenesis of constitutional DP there are lied deteriorations in the system of the regulation of impulse secretion of gonadotrophin-releasing hormone (Gn-RH) and such non-hormonal factors as monamines of adrenergic and dophaminergic system, melatonin, neuropeptide Y, estragens, insulin-like growth factor-1 (IGF-1), leptine, interacting with Gn-RH-secreting neurons. Pathology of the development and maturation of structures of the diencephalon, as the cause of constitutional APD, is a rather frequent consequence of antenatal and perinatal pathology, traumatic injury of the brain, microcirculatory disorders. DP proves to be an unfavorable factor in the formation of bone density and final growth, in psychological personality achievement of the adolescent, in the maturation of the reproductive system, thus is the subject to correct. The problem of the therapy of constitutional puberty arrest remains to be controversial up to recent time. Some authors adhere to the observation tactics. They believe that children with constitutional DP may have a favorable prognosis of final growth and pubertal development. However, obvious negative psychosocial and medical consequences of the delayed growth and puberty have not yet been taken into consideration. Lately, hormonal therapy with puberty-stimulating steroids, anabolic agents has been suggested. But both a number of questions concerning the effect of therapy with androgens on activation of the hypophyseal-gonadal system in adolescents, and the impact of this treatment on the final growth of the patients remain to be unsolved. The necessity to produce an effect on central mechanisms of regulation by applying physiotherapeutic transcranial techniques, particularly transcranial magnetic therapy also exists. The presented review showed the diversity of factors influencing on the pubertal development of adolescents, various tactical methods of treatment and correction of disorders in patients with constitutional PD.

11β-hydroxylase deficiency is a rare autosomal recessive disorder due to impaired steroidogenesis in the adrenal cortex caused by pathogenic mutations in the CYP11B1 gene. The main clinical manifestations are determined by a deficiency of cortisol, ACTH hyperproduction, excessive androgens secretion and the accumulation of 11-deoxycorticosterone, which leads to the development of arterial hypertension. In the diagnostic search, it is important to take into account the ethnicity of the patient, since the frequency of the disease and the prevalence of mutations differ between ethnic groups. The article presents a clinical case of 11β-hydroxylase deficiency as the result of compound heterozygous mutations in the CYP11B1 gene in a patient of Turkic origin. This case shows the clinical manifestations and the development of complications of 11β-hydroxylase deficiency, the stages of differential diagnosis of patients with 21-hydroxylase deficiency.