[Morphofunctional aspects of genetic analysis of osteogenesis imperfecta in cultured skin fibroblasts]. / Morfofunktsional'nye aspekty geneticheskogo analiza nesovershennogo osteogeneza v kul'ture kozhnykh fibroblastov.

Osteogenesis imperfecta (OI) is a disease with biochemical evidence of abnormality in collagen biosynthesis. We report here that expression of the OI phenotype extends to the level of dermal fibroblast morphology in vitro. Growth characteristics and morphology of control (n = 3) and 01 cell strains (n = 10) were compared. Our results show that (1) OI fibroblasts take longer time (16 days) than that with control cells (13 days) to reach stationary phase; (2) OI fibroblasts achieve a lower cell density (1.0 +/- 0.09) at stationary phase compared to control cells (1.47 +/- 0.1); p < 0.01; (3) cell shape (expressed as the width/length ratio) was also abnormal in OI cells: they have significantly increased ratios (p < 0.05) compared to control. These changes were associated with an increased level of fibronectin concentration and engorged cytoplasmic vesicles in dermal fibroblasts in vitro. We have reason to suspect that dysmorphology of fibroblasts may be related to aberrant collagen metabolism that leads to inadequacy of extracellular substratum, that in its turn hinders normal adhesion of cells as well as their spreading, morphology and fibronectin concentration.

[Pathogenetic aspects of studying clonal properties of bone marrow stromal cells in diseases of the osteoarticular system]. / Patogeneticheskie aspekty izucheniia klonal'nykh svoistv stromal'nykh kletok kostnogo mozga pri zabolevaniiakh kostno-sustavnogo apparata.

Properties of clonogenic stromal fibroblasts of bone marrow which are precursors of osteo- and chondrogenesis, of patients with different diseases of osteoarticular apparatus have been studied. Peculiarities of their reaction on growth-stimulating influence of nonstromal auto- and xenogeneic cells have been demonstrated. The possibilities of data application for studying of pathogenesis of hereditary osteochondrodysplasias as well as other groups of the skeleton affections are considered.

[Morphological characteristics of lesions of the cartilaginous growth plate in several forms of osteochondrodysplasias]. / Morfologicheskie osobennosti porazheniia khriashchevoi rostkovoi plastinki pri nekotorykh formakh osteokhondrodisplazii.

The authors have carried out a search for morphological markers for diagnosing, differential diagnosis and medico-genetic consulting of 4 forms of osteochondrodysplasias. The cells and the intercellular substance of the cartilaginous graft plate from the upper flaring portion of the ileum of the patients were investigated. A common feature of most conditions were changes on the part of the granular endoplasmatic net, which points at a disturbance in the function of the synthesis apparatus and/or of the transport of the synthesized biopolymers. No morphological changes were revealed in the cartilaginous graft plate which were pathognomonic for spondyloepiphyseal dysplasia, achondroplasia and multiple exostosis chondrodysplasia. In one of the forms of pseudoachondroplasia (its severe dominant heritable variation) there were specific inclusions in the chondrocytes of the patients, which apparently were a morphological manifestation of disturbed assembly of proteoglycan aggregates which is the basis of the pathogenesis of pseudoachondroplasia. The possibility of using this morphological marker in the clinico-genetic classification of pseudoachondroplasia, in differential diagnosis of the disease in unclear cases as well as in medico-genetic consulting of the patients' families is discussed.

[Study of clinical strains of microorganisms in a scanning electron microscope]. / Issledovanie klinicheskikh shtammov mikroorganizmov v rastvorom élektronnom mikroskope.

The authors present an available method for preparing bacterial cells for examination in a scanning electron microscope. The method has been tried with clinical strains of S. aureus, P. aeruginosa, Acinetobacter calcoaceticus v. anitratus. Morphologic features of various types of microorganisms are described.

[Cytochemical and electron microscopic research on the peripheral blood leukocytes and skin fibroblasts in mucopolysaccharidoses]. / Tsitokhimicheskie i élektronno-mikroskopicheskie issledovaniia leikotsitov perifericheskoi krovi i fibroblastov kozhi pri mukopolisakharidozakh.

Cytochemical studies of the peripheral blood leucocytes in patients suffering from mucopolysaccharidoses (MPS) have revealed metachromatic granules in the cell cytoplasm. Electron microscopy of these cells has shown multiple cytoplasmic vacuoles. It is supposed that metachromatic granules in blood leucocytes of patients with MPS observed in the photo-optical studies correspond to the vacuoles found under electron microscope. The obtained data have shown that peripheral blood leucocytes and skin fibroblasts have the common ultrastructure in MPS patients. The data of electron histochemical studies testify to that the vacuoles of skin fibroblasts are filled with glycosaminoglycans.

[Ultrastructure of skin fibroblasts in storage diseases (mucopolysaccharidosis types IV and VI)]. / Ul'trastruktura fibroblastov kozhi pri nekotorykh bolezniakh nakopleniia (mukopolisakharidozakh IV i VI tipov).

Ultrastructure of skin fibroblasts was studied in patients with mucopolysaccharidoses of types IV and VI and their relatives. In MPS VI and keratan-nonexcreting form of MPS IV the cytoplasm of fibroblasts contained numerous vacuoles with material of various electron density. The ultrastructure of cell did not differ from the norm in the keratan-excreting form of MPS IV. Skin fibroblasts from parents and siblings with MPS VI were found to contain a large number of residue bodies. The possible usage of ultrastructural data for early diagnosis of MPS and in medical genetic consultation of families of the patients with MPS is discussed.

[Characteristics of the structure and functional potentials of the metaepiphysis of tubular bones]. / K kharakteristike struktury i funktsional'nykh potentsii metaépifiza trubchatykh kostei.

During early postnatal ontogenesis in the metaepiphyseal plate of the developing rat tibial bone, the borderline zone, the columnar cartilage zone and the zone of macrovesiculous cells differ in their cell composition, structure of basic substance and in functional and reactive potencies, which manifest themselves most distinctly after injection of growth-promoting hormones to the animals. The organic specificity of the metaepiphyseal borderline zone depends, first of all, on its intermediate position between the cartilage, fulfilling further bone growth lengthwise as an organ, and a developing endochondral bone substituting cartilage in the epiphysis. The borderline zone retains these features under hormonal effect. In the zone of columnar cells, it is reasonable to distinguish a layer of cuboidal cells--mature chondrocytes posessing great reactive and reparative possibilities, which become especially distincitive after prednisolon and TCT injection.