Konsensuální doporučení ČNHP (Českého národního hemofilického programu) pro diagnostiku a léčbu pacientů s vrozenou hemofilií a s inhibitorem FVIII/FIX

Cílem standardu je stanovit základní diagnostické a především terapeutické postupy v péči o pacienty s vrozenou hemofilií a s inhibitorem koagulačního faktoru VIII, resp. faktoru IX.The aim of the guidelines is to set-up and standardize diagnostic as well as therapeutic approaches to care about patients with congenital haemophilia and inhibitors to coagulation factor VIII or factor I

Fine structure of S-layers

S-layers are now considered a common cell wall structure in Bacteria and Archaea as well as in some algae. Morphological and chemical studies have revealed that S-layers consist of crystalline arrays of protein or glycoprotein subunits forming oblique, square or hexagonal lattices on the cell surface. Electron microscopy and computer image enhancement techniques have been applied to obtain structural information down to the nanometer range. This chapter deals with the wide distribution of S-layers among cyanobacteria, and their morphological and chemical characterization, and the potential of high resolution electron microscopic studies applied to the cell envelope of Pyrodictium. The occurrence of S-layers in cyanobacteria was investigated by cryomethods and ultrathin sectioning. These investigations indicate that the ultrastructure of S-layers may be exploited as an auxiliary taxonomic criterion in the classification of cyanobacteria. Pyrodictium is the first organism which has shown an optimum growth temperature above 100°C. The highly irregularly shaped, flagellated cells are interconnected by extracellular tubules. The three-dimensional structure of this network was visualized with high resolution scanning electron microscopy while the fine structure of the cell wall architecture was studied in detail with various electron microscopic techniques. Both contributions demonstrate that the investigation of the fine structure of S-layers is fundamental for establishing structure-function relationships for these two-dimensional crystalline arrays