[Results of a clinical and genealogical analysis of pedigrees of children with cerebral palsy in the population of Rostov region]. / Rezul'taty kliniko-genealogicheskogo analiza rodoslovnykh detei s detskim tserebral'nym paralichom v populiatsii Rostovskoi oblasti.

AIM: To perform clinical and genealogical pedigree analysis and determine the proportion of hereditary factors in the etiopathogenesis of cerebral palsy (CP) in children in the Rostov Region. MATERIAL AND METHODS: Pedigrees and the prevalence of CP, congenital malformations and other related diseases among relatives of I, II and III degrees of kinship of 229 probands with CP were studied. RESULTS AND CONCLUSION: The family concentration of the disease was detected in 15 (6.6%) cases. There were 96 (41.9%) pedigrees with probands with a family history and 118 (51.5%) without family history of CP. The frequency of CP was by 19.2 times higher in the relatives of probands in the families with a history of disease and 2.2 times higher in the siblings compared to the population frequency. The frequency of congenital malformations in the relatives of probands in this group was 23.8 times higher, while in the group of probands with a family history 14.9 times higher than the expected value. Miscarriages and stillbirths preceded the birth of children with CP in 29.2-33.3% of the mothers, and percentage of unrealized pregnancies ranged from 16.7 to 18.7%. CP in 51.5% of cases can be considered as a sporadic disease, and in 48.5% of cases it was genetically determined and can be attributed to a group of multifactorial diseases with polygenic determination of genetic predisposition components.

[Ultrastructural changes of child rib cartilage in different deformations of chest cells]. / Osobennosti ul'trastrukturnykh izmenennii rebernogo khriashcha detei pri razlichnykh deformatsiiakh grudnoi kletki.

Different chest deformations are accompanied by appearance of asbestos-like fibrills in pericellular matrix, development of significant lipid and carbohydrate chondrocyte dystrophy, irreversible changes in their majority. Anomalous accumulation of intermediate filaments, lipid and glycogen, may substitute the cytoplasm organells in Marfan's syndrome. Pathologic disturbances of costal cartilage, connected with ultrastructural changes of chondrocytes, intercellular substance and extracellular matrix are morphologic substrate of different chest deformations.