Okhapkina O. Risk factors and conditions for the formation of dysplasia-dependent pathology in case of fibrillogenesis disorder in children.

The thesis systematized results of the study of peculiarities of undifferentiated connective tissue dysplasia (UCTD) and formation of dysplasia-dependent pathology (DDP) with regard for adaptive-compensatory mechanisms, autonomic nervous control, haemodynamic responses of the child's organism, revealed compensatory-adaptive reactions which are typical for children with disturbances of fibrillogenesis, grounded and worked out algorithms for diagnosing and prognosticating the course of UCTD, as well as a scheme for prophylactic medical examinations of this group of patients. While the prevalence rate of fibrillogenesis disturbance equivalents ranges from 34.5±1.2) % to (29.4±1.1) %, the general prevalence rate of UCTD syndrome proper with regard for biochemical markers of collagen formation makes up (25.58±2.1) %. The most diagnostically significant phenotypical signs of UCTD for children are as follows: hypognathia, skin hyperelastosis, scoliotic posture, epicanthus, joint hypermobility syndrome with arachnodactylia, and pigeon chest. Biochemical indices of collagen formation processes (oxyproline, GAGIII), hair calcium level within 1,100-1,600 ?g/g of hair, equivalent haemodynamic responses in the autonomic nervous system, critical age periods of progression of dysplastic disorders, and peculiarities in the physical development rates with the formation of the asthenic constitution play the most significant part in the formation of multisystem DDP in childhood. The relation, reliably established between the degree of disorders in the collagen formation processes and exacerbation of somatic DDP, predetermines that a therapeutic complex for such patients should include the drugs, aimed at collagen stabilization. A scheme for clinical monitoring of children with DDP was developed.