Репозиторий Университета

© 2019 Elsevier B.V. In the present study, porous three-dimensional (3D) printed titanium (Ti) implants of complex shape and predefined architecture were produced by selective laser sintering (SLS) technique. Electrochemical deposition combined with biomimetic approach was applied to low-temperature coating of these implants with metastable octacalcium phosphate (OCP) achieved via chemical transformation of dicalcium phosphate dehydrate (DCPD). X-ray diffraction (XRD), Scanning electron microscopy (SEM), Fourier transform infrared spectroscopy (FTIR) and compressive strength analyses were applied to study the chemical composition, morphology and mechanical properties of the final OCP coating on the titanium surface. In vivo comparative study of the porous 3D printed Ti and OCP coated Ti implants has been performed using critical-size crania model, porous 3D printed Ti and coated implants were compared. A statistically significant difference in the newly formed bone thickness for OCP coated Ti implants was detected already at 6 weeks after implantation. Our results provide an experimental proof of a new concept of OCP coating for cranioplasty clinical applications.

© 2019 Elsevier B.V. A massive-scale production of engineered nanoparticles (ENPs) becomes one of the most important environmental issues. The mechanisms of ENPs' (eco)toxic action are not fully understood, and the estimation of those mechanisms is a complicated task because even slight changes in particle characteristics could dramatically change their toxicity. As a result of continuous manufacturing of ENPs with specific functionality and different physicochemical properties, conventional methods of in vivo and in vitro testing would not be able to fill the existing knowledge gap in nanotoxicology. The objectives of this review are to overlook the current achievements based on the new approaches of ENPs' risk assessment, such as bioinformatics approaches and machine learning tools. These methods confirmed their ability to reliable prediction and evaluation of ENPs' behavior and their toxic endpoints. Databases and projects based on these methods and approaches would be highly useful in addressing the problem of ENPs’ regulation.

© 2018, © 2018 Informa UK Limited, trading as Taylor  &  Francis Group. Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study. Results of study showed high risk of life-threatening complications during pregnancy and delivery, especially the risk of hemorrhage and cardiovascular complications. In all the patients, we observed the progression of bleeding or development of bleeding in new localizations (epistaxis in 27 patients, easy brushing in 22, skin and mucosa telangiectasia in 20, gastrointestinal bleedings in 4, hemoptysis in 4, hematomas for minor traumas in 14, conjunctivas hemorrhages in 5). Conclusion: The pathogenesis of bleeding in such patients has mixed pattern: besides vascular wall pathology coagulation deficiency plays some role. The preferred delivery method for such patients is caesarean section. Deep vaginal ruptures and serious hemorrhage accompany vaginal delivery.

© 2019 Most researchers associate the increase in the permeability of lipid bilayers of artificial and biological membranes observed in various experiments with the formation of hypothetical hydrophobic and hydrophilic pores. Although the existence of hydrophobic defects, as the first stage of the formation of a hydrophilic pore, was hypothesized decades ago from electroporation experiments, the difficulty of describing this stage is determined by the lack of experimental data confirming the existence or at least associated with hydrophobic pores. We explored the increase in the current variance through the lipid membrane, observed when approaching the phase transition from the side of high temperatures, and have associated it with capacitive currents arising in response to the formation of hydrophobic pores. Assuming that the number of hydrophobic pores in a membrane follows a Poisson distribution, and thus, the mean number of hydrophobic pores is equal to the variance of that number, we used the measurements of the membrane current variance to evaluate the number of hydrophobic pores. Analysis of experimental data within this model allows us to estimate the number of hydrophobic pores at or above the phase transition and shows that the number of hydrophobic pores in a membrane close to the phase transition increased 20 times compared to the number of hydrophobic pores existing in the membrane far from the melting transition.

© 2019 Elsevier B.V. Fabry disease (FD [MIM:301500]) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene. Deficient activity of its product, lysosomal enzyme α-galactosidase A (α-Gal A), leads to excessive accumulation of glycosphingolipids in cells of multiple organs. The establishing of the diagnosis is challenge in female patients because of milder clinical manifestation and normal α-Gal A activity. The globotriaosylsphingosine (lysoGb3) is described as a more sensitive diagnostic biomarker for females with pathogenic mutation in the GLA gene. Thus, the aim of this study is to improve the biochemical diagnostic efficiency for FD in females. Here we report the α-Gal A/lysoGb3 ratio as the novel biochemical criteria for diagnosis of female patients with FD, using dried blood spots (DBS) as test samples. It showed 100% sensitivity in distinguishing our group of 35 female patients from control (n = 140). Whereas measurement of α-Gal A and lysoGb3 alone showed 8.6% and 74.4% respectively. A new approach of using the ratio of α-Gal A activity to lysoGb3 concentration in DBS may provide a more accurate screening tool for identification of FD females.

© 2018, © 2018 Informa UK Limited, trading as Taylor  &  Francis Group. Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study. Results of study showed high risk of life-threatening complications during pregnancy and delivery, especially the risk of hemorrhage and cardiovascular complications. In all the patients, we observed the progression of bleeding or development of bleeding in new localizations (epistaxis in 27 patients, easy brushing in 22, skin and mucosa telangiectasia in 20, gastrointestinal bleedings in 4, hemoptysis in 4, hematomas for minor traumas in 14, conjunctivas hemorrhages in 5). Conclusion: The pathogenesis of bleeding in such patients has mixed pattern: besides vascular wall pathology coagulation deficiency plays some role. The preferred delivery method for such patients is caesarean section. Deep vaginal ruptures and serious hemorrhage accompany vaginal delivery.

© 2019 Most researchers associate the increase in the permeability of lipid bilayers of artificial and biological membranes observed in various experiments with the formation of hypothetical hydrophobic and hydrophilic pores. Although the existence of hydrophobic defects, as the first stage of the formation of a hydrophilic pore, was hypothesized decades ago from electroporation experiments, the difficulty of describing this stage is determined by the lack of experimental data confirming the existence or at least associated with hydrophobic pores. We explored the increase in the current variance through the lipid membrane, observed when approaching the phase transition from the side of high temperatures, and have associated it with capacitive currents arising in response to the formation of hydrophobic pores. Assuming that the number of hydrophobic pores in a membrane follows a Poisson distribution, and thus, the mean number of hydrophobic pores is equal to the variance of that number, we used the measurements of the membrane current variance to evaluate the number of hydrophobic pores. Analysis of experimental data within this model allows us to estimate the number of hydrophobic pores at or above the phase transition and shows that the number of hydrophobic pores in a membrane close to the phase transition increased 20 times compared to the number of hydrophobic pores existing in the membrane far from the melting transition.

© 2019 Elsevier B.V. Fabry disease (FD [MIM:301500]) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene. Deficient activity of its product, lysosomal enzyme α-galactosidase A (α-Gal A), leads to excessive accumulation of glycosphingolipids in cells of multiple organs. The establishing of the diagnosis is challenge in female patients because of milder clinical manifestation and normal α-Gal A activity. The globotriaosylsphingosine (lysoGb3) is described as a more sensitive diagnostic biomarker for females with pathogenic mutation in the GLA gene. Thus, the aim of this study is to improve the biochemical diagnostic efficiency for FD in females. Here we report the α-Gal A/lysoGb3 ratio as the novel biochemical criteria for diagnosis of female patients with FD, using dried blood spots (DBS) as test samples. It showed 100% sensitivity in distinguishing our group of 35 female patients from control (n = 140). Whereas measurement of α-Gal A and lysoGb3 alone showed 8.6% and 74.4% respectively. A new approach of using the ratio of α-Gal A activity to lysoGb3 concentration in DBS may provide a more accurate screening tool for identification of FD females.

© 2018, © 2018 Informa UK Limited, trading as Taylor  &  Francis Group. Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study. Results of study showed high risk of life-threatening complications during pregnancy and delivery, especially the risk of hemorrhage and cardiovascular complications. In all the patients, we observed the progression of bleeding or development of bleeding in new localizations (epistaxis in 27 patients, easy brushing in 22, skin and mucosa telangiectasia in 20, gastrointestinal bleedings in 4, hemoptysis in 4, hematomas for minor traumas in 14, conjunctivas hemorrhages in 5). Conclusion: The pathogenesis of bleeding in such patients has mixed pattern: besides vascular wall pathology coagulation deficiency plays some role. The preferred delivery method for such patients is caesarean section. Deep vaginal ruptures and serious hemorrhage accompany vaginal delivery.

© 2019 Most researchers associate the increase in the permeability of lipid bilayers of artificial and biological membranes observed in various experiments with the formation of hypothetical hydrophobic and hydrophilic pores. Although the existence of hydrophobic defects, as the first stage of the formation of a hydrophilic pore, was hypothesized decades ago from electroporation experiments, the difficulty of describing this stage is determined by the lack of experimental data confirming the existence or at least associated with hydrophobic pores. We explored the increase in the current variance through the lipid membrane, observed when approaching the phase transition from the side of high temperatures, and have associated it with capacitive currents arising in response to the formation of hydrophobic pores. Assuming that the number of hydrophobic pores in a membrane follows a Poisson distribution, and thus, the mean number of hydrophobic pores is equal to the variance of that number, we used the measurements of the membrane current variance to evaluate the number of hydrophobic pores. Analysis of experimental data within this model allows us to estimate the number of hydrophobic pores at or above the phase transition and shows that the number of hydrophobic pores in a membrane close to the phase transition increased 20 times compared to the number of hydrophobic pores existing in the membrane far from the melting transition.

© 2019 Elsevier B.V. Fabry disease (FD [MIM:301500]) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene. Deficient activity of its product, lysosomal enzyme α-galactosidase A (α-Gal A), leads to excessive accumulation of glycosphingolipids in cells of multiple organs. The establishing of the diagnosis is challenge in female patients because of milder clinical manifestation and normal α-Gal A activity. The globotriaosylsphingosine (lysoGb3) is described as a more sensitive diagnostic biomarker for females with pathogenic mutation in the GLA gene. Thus, the aim of this study is to improve the biochemical diagnostic efficiency for FD in females. Here we report the α-Gal A/lysoGb3 ratio as the novel biochemical criteria for diagnosis of female patients with FD, using dried blood spots (DBS) as test samples. It showed 100% sensitivity in distinguishing our group of 35 female patients from control (n = 140). Whereas measurement of α-Gal A and lysoGb3 alone showed 8.6% and 74.4% respectively. A new approach of using the ratio of α-Gal A activity to lysoGb3 concentration in DBS may provide a more accurate screening tool for identification of FD females.

© 2018, © 2018 Informa UK Limited, trading as Taylor  &  Francis Group. Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study. Results of study showed high risk of life-threatening complications during pregnancy and delivery, especially the risk of hemorrhage and cardiovascular complications. In all the patients, we observed the progression of bleeding or development of bleeding in new localizations (epistaxis in 27 patients, easy brushing in 22, skin and mucosa telangiectasia in 20, gastrointestinal bleedings in 4, hemoptysis in 4, hematomas for minor traumas in 14, conjunctivas hemorrhages in 5). Conclusion: The pathogenesis of bleeding in such patients has mixed pattern: besides vascular wall pathology coagulation deficiency plays some role. The preferred delivery method for such patients is caesarean section. Deep vaginal ruptures and serious hemorrhage accompany vaginal delivery.

© 2019 Most researchers associate the increase in the permeability of lipid bilayers of artificial and biological membranes observed in various experiments with the formation of hypothetical hydrophobic and hydrophilic pores. Although the existence of hydrophobic defects, as the first stage of the formation of a hydrophilic pore, was hypothesized decades ago from electroporation experiments, the difficulty of describing this stage is determined by the lack of experimental data confirming the existence or at least associated with hydrophobic pores. We explored the increase in the current variance through the lipid membrane, observed when approaching the phase transition from the side of high temperatures, and have associated it with capacitive currents arising in response to the formation of hydrophobic pores. Assuming that the number of hydrophobic pores in a membrane follows a Poisson distribution, and thus, the mean number of hydrophobic pores is equal to the variance of that number, we used the measurements of the membrane current variance to evaluate the number of hydrophobic pores. Analysis of experimental data within this model allows us to estimate the number of hydrophobic pores at or above the phase transition and shows that the number of hydrophobic pores in a membrane close to the phase transition increased 20 times compared to the number of hydrophobic pores existing in the membrane far from the melting transition.

© 2019 Elsevier B.V. Fabry disease (FD [MIM:301500]) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene. Deficient activity of its product, lysosomal enzyme α-galactosidase A (α-Gal A), leads to excessive accumulation of glycosphingolipids in cells of multiple organs. The establishing of the diagnosis is challenge in female patients because of milder clinical manifestation and normal α-Gal A activity. The globotriaosylsphingosine (lysoGb3) is described as a more sensitive diagnostic biomarker for females with pathogenic mutation in the GLA gene. Thus, the aim of this study is to improve the biochemical diagnostic efficiency for FD in females. Here we report the α-Gal A/lysoGb3 ratio as the novel biochemical criteria for diagnosis of female patients with FD, using dried blood spots (DBS) as test samples. It showed 100% sensitivity in distinguishing our group of 35 female patients from control (n = 140). Whereas measurement of α-Gal A and lysoGb3 alone showed 8.6% and 74.4% respectively. A new approach of using the ratio of α-Gal A activity to lysoGb3 concentration in DBS may provide a more accurate screening tool for identification of FD females.

© 2018, © 2018 Informa UK Limited, trading as Taylor  &  Francis Group. Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study. Results of study showed high risk of life-threatening complications during pregnancy and delivery, especially the risk of hemorrhage and cardiovascular complications. In all the patients, we observed the progression of bleeding or development of bleeding in new localizations (epistaxis in 27 patients, easy brushing in 22, skin and mucosa telangiectasia in 20, gastrointestinal bleedings in 4, hemoptysis in 4, hematomas for minor traumas in 14, conjunctivas hemorrhages in 5). Conclusion: The pathogenesis of bleeding in such patients has mixed pattern: besides vascular wall pathology coagulation deficiency plays some role. The preferred delivery method for such patients is caesarean section. Deep vaginal ruptures and serious hemorrhage accompany vaginal delivery.

© 2019 Most researchers associate the increase in the permeability of lipid bilayers of artificial and biological membranes observed in various experiments with the formation of hypothetical hydrophobic and hydrophilic pores. Although the existence of hydrophobic defects, as the first stage of the formation of a hydrophilic pore, was hypothesized decades ago from electroporation experiments, the difficulty of describing this stage is determined by the lack of experimental data confirming the existence or at least associated with hydrophobic pores. We explored the increase in the current variance through the lipid membrane, observed when approaching the phase transition from the side of high temperatures, and have associated it with capacitive currents arising in response to the formation of hydrophobic pores. Assuming that the number of hydrophobic pores in a membrane follows a Poisson distribution, and thus, the mean number of hydrophobic pores is equal to the variance of that number, we used the measurements of the membrane current variance to evaluate the number of hydrophobic pores. Analysis of experimental data within this model allows us to estimate the number of hydrophobic pores at or above the phase transition and shows that the number of hydrophobic pores in a membrane close to the phase transition increased 20 times compared to the number of hydrophobic pores existing in the membrane far from the melting transition.

© 2019 Elsevier B.V. Fabry disease (FD [MIM:301500]) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene. Deficient activity of its product, lysosomal enzyme α-galactosidase A (α-Gal A), leads to excessive accumulation of glycosphingolipids in cells of multiple organs. The establishing of the diagnosis is challenge in female patients because of milder clinical manifestation and normal α-Gal A activity. The globotriaosylsphingosine (lysoGb3) is described as a more sensitive diagnostic biomarker for females with pathogenic mutation in the GLA gene. Thus, the aim of this study is to improve the biochemical diagnostic efficiency for FD in females. Here we report the α-Gal A/lysoGb3 ratio as the novel biochemical criteria for diagnosis of female patients with FD, using dried blood spots (DBS) as test samples. It showed 100% sensitivity in distinguishing our group of 35 female patients from control (n = 140). Whereas measurement of α-Gal A and lysoGb3 alone showed 8.6% and 74.4% respectively. A new approach of using the ratio of α-Gal A activity to lysoGb3 concentration in DBS may provide a more accurate screening tool for identification of FD females.

© 2018, © 2018 Informa UK Limited, trading as Taylor  &  Francis Group. Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study. Results of study showed high risk of life-threatening complications during pregnancy and delivery, especially the risk of hemorrhage and cardiovascular complications. In all the patients, we observed the progression of bleeding or development of bleeding in new localizations (epistaxis in 27 patients, easy brushing in 22, skin and mucosa telangiectasia in 20, gastrointestinal bleedings in 4, hemoptysis in 4, hematomas for minor traumas in 14, conjunctivas hemorrhages in 5). Conclusion: The pathogenesis of bleeding in such patients has mixed pattern: besides vascular wall pathology coagulation deficiency plays some role. The preferred delivery method for such patients is caesarean section. Deep vaginal ruptures and serious hemorrhage accompany vaginal delivery.

© 2019 Most researchers associate the increase in the permeability of lipid bilayers of artificial and biological membranes observed in various experiments with the formation of hypothetical hydrophobic and hydrophilic pores. Although the existence of hydrophobic defects, as the first stage of the formation of a hydrophilic pore, was hypothesized decades ago from electroporation experiments, the difficulty of describing this stage is determined by the lack of experimental data confirming the existence or at least associated with hydrophobic pores. We explored the increase in the current variance through the lipid membrane, observed when approaching the phase transition from the side of high temperatures, and have associated it with capacitive currents arising in response to the formation of hydrophobic pores. Assuming that the number of hydrophobic pores in a membrane follows a Poisson distribution, and thus, the mean number of hydrophobic pores is equal to the variance of that number, we used the measurements of the membrane current variance to evaluate the number of hydrophobic pores. Analysis of experimental data within this model allows us to estimate the number of hydrophobic pores at or above the phase transition and shows that the number of hydrophobic pores in a membrane close to the phase transition increased 20 times compared to the number of hydrophobic pores existing in the membrane far from the melting transition.

© 2019 Elsevier B.V. Fabry disease (FD [MIM:301500]) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene. Deficient activity of its product, lysosomal enzyme α-galactosidase A (α-Gal A), leads to excessive accumulation of glycosphingolipids in cells of multiple organs. The establishing of the diagnosis is challenge in female patients because of milder clinical manifestation and normal α-Gal A activity. The globotriaosylsphingosine (lysoGb3) is described as a more sensitive diagnostic biomarker for females with pathogenic mutation in the GLA gene. Thus, the aim of this study is to improve the biochemical diagnostic efficiency for FD in females. Here we report the α-Gal A/lysoGb3 ratio as the novel biochemical criteria for diagnosis of female patients with FD, using dried blood spots (DBS) as test samples. It showed 100% sensitivity in distinguishing our group of 35 female patients from control (n = 140). Whereas measurement of α-Gal A and lysoGb3 alone showed 8.6% and 74.4% respectively. A new approach of using the ratio of α-Gal A activity to lysoGb3 concentration in DBS may provide a more accurate screening tool for identification of FD females.