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1
Куликов, А. Г., Ю. В. Писаревский, А. Е. Благов, Н. В. Марченков, В. А. Ломонов, А. А. Петренко, and М. В. Ковальчук. "Перестройка дефектной структуры тетрабората лития (Li-=SUB=-2-=/SUB=-B-=SUB=-4-=/SUB=-O-=SUB=-7-=/SUB=-) во внешнем электрическом поле." Физика твердого тела 61, no. 4 (2019): 671. http://dx.doi.org/10.21883/ftt.2019.04.47411.250.
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The process of the defect structure rearrangement in a lithium tetraborate single crystal under the influence of high voltage external electric field applied along the polar direction [001] is studied with use of X-ray diffractometry. The results are supplemented by measurements of the conductivity kinetics. Under conditions of electric field of 300-500 V/mm strength, a sharp broadening of the 004 reflection diffraction peak and its integral intensity increasing by several times are observed, however its position and shape practically do not change. Under the influence of DC field with a strength in range of 500 to 1500 V/mm, the broadening process slows down, but the rocking curve asymmetry appears as well as its sharp shift to the smaller angles associated with an increase in the lattice parameter along the c-axis. This process is quasi-reversible, since the distorted structure is partially restored at a very slow rate (for several months). Two types of the diffraction peak parameters variation dependencies on the external field are interpreted as the manifestation of two ionic conductivity mechanisms: mobile lithium ions (Li+) at low-intensity electric field and oxygen vacancies (VO2+) at stronger fields. The process of charge carriers’ migration causes the increase of defects concentration and structure changes in the near-surface region of the crystal.
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Otsuka, Yuki, Tetsuya Yumoto, Hiromi Ihoriya, Namiko Matsumoto, Kota Sato, Koji Abe, Hiromichi Naito, and Atsunori Nakao. "Acute Agitation as an Initial Manifestation of Neuro-Behçet’s Disease." Case Reports in Emergency Medicine 2018 (November 29, 2018): 1–3. http://dx.doi.org/10.1155/2018/5437027.
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Managing acutely agitated or violent patients in the emergency department (ED) represents a significant challenge. Acute agitation as an initial manifestation of neuro-Behcet’s disease (NBD) is an extremely rare clinical entity. A 44-year-old male, who had been complaining about a severe headache and fever for several days, was admitted to our ED due to acutely presented incontinence and agitation. On admission, physical restraint and sedation with sevoflurane and propofol were required for his combative and violent behavior. Cerebrospinal fluid examination revealed increased cell count. Fluid attenuated inversion recovery magnetic resonance imaging showed a high intensity signal in the left parietal lobe and bilateral occipital lobe. As infectious meningoencephalitis was suspected, empirical therapy was immediately started. He recovered uneventfully without neurological defect in seven days. Based on positive human leukocyte antigen B-51 and clinical manifestations, the diagnosis of NBD was made and remitted by steroid therapy. Although acute NBD commonly presents with focal neurological symptoms, psychiatric symptoms could be considered the first manifestation. A focused and thorough examination coupled with appropriate management strategies can assist emergency clinicians safely and effectively manage these patients.
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Bludov, M. A., I. V. Khyzhniy, E. V. Savchenko, V. I. Sugakov, and S. A. Uyutnov. "Self-oscillations in solid methane irradiated by electrons." Nuclear Physics and Atomic Energy 21, no. 4 (December 25, 2020): 312–22. http://dx.doi.org/10.15407/jnpae2020.04.312.
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The formation of self-oscillations of temperature and concentration of radicals in an electron-irradiated methane film at low temperatures has been investigated experimentally and theoretically. Self-oscillations arise due to the activation nature of diffusion and radical recombination processes. Self-oscillations were studied experimentally by measuring the desorption of particles from an irradiated sample and theoretically by solving the kinetic equations for defects in a methane sample. Concentration self-oscillations of two types of particles have been found and investigated; namely, hydrogen atoms and CH3 radicals formed during the irradiation of methane by electrons. It is shown that with an increase in the irradiation intensity, the oscillation periods decrease, and the calculation value are of the order of magnitude observed in the experiment. A model of a manifestation of the self-oscillation of hydrogen molecule concentration during desorption is presented.
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Mokina, E., E. Naumenko, D. Kumanyaeva, M. Rakhmatullina, and E. Surgaeva. "Antenatal period and clinical picture analysis of the newborns with interatrial communications." Bulletin of Science and Practice 5, no. 3 (March 15, 2019): 58–63. http://dx.doi.org/10.33619/2414-2948/40/06.
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The birth of children with congenital heart disease is a serious problem due to the risk of infant mortality. Clinically, the interatrial septum defect belongs to the low manifest defects. The severity of its symptoms depends on the size and location of the defect, as well as, to a greater extent, on the duration of the defect and the development of secondary complications. This situation dictates the need for early diagnosis of this congenital heart disease, dynamic observation, timely treatment, which determines the relevance of this study. In this article, we tried to analyze in detail the characteristics of the course of the antenatal period and the clinical picture of newborns with atrial communication. Summing up our study, it should be noted that the clinical manifestations in interatrial communications in newborns are not specific. In most infants with interatrial communications, systolic noise of varying intensity can be heard, however, this noise is not associated with the presence of interatrial communications, but due to the presence of diagonally located chords in the left ventricle.
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Kornilov, T. A., and V. S. Supletsov. "Forecast Intensity of Manifestations of Defects and Damage to Steel Structures Operating under Extreme Conditions of the External and Internal Environment." IOP Conference Series: Materials Science and Engineering 463 (December 31, 2018): 032058. http://dx.doi.org/10.1088/1757-899x/463/3/032058.
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Sepulveda, Fernando E., Franck Debeurme, Gaël Ménasché, Mathieu Kurowska, Marjorie Côte, Jana Pachlopnik Schmid, Alain Fischer, and Geneviève de Saint Basile. "Distinct severity of HLH in both human and murine mutants with complete loss of cytotoxic effector PRF1, RAB27A, and STX11." Blood 121, no. 4 (January 24, 2013): 595–603. http://dx.doi.org/10.1182/blood-2012-07-440339.
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Abstract Inherited defects of granule-dependent cytotoxicity led to the life-threatening immune disorder hemophagocytic lymphohistiocytosis (HLH), characterized by uncontrolled CD8 T-cell and macrophage activation. In a cohort of HLH patients with genetic abnormalities expected to result in the complete absence of perforin, Rab27a, or syntaxin-11, we found that disease severity as determined by age at HLH onset differed significantly, with a severity gradient from perforin (early onset) > Rab27a > syntaxin-11 (late onset). In parallel, we have generated a syntaxin-11–deficient (Stx11−/−) murine model that faithfully reproduced the manifestations of HLH after lymphocytic choriomeningitis virus (LCMV) infection. Stx11−/− murine lymphocytes exhibited a degranulation defect that could be rescued by expression of human syntaxin-11 but not expression of a C-terminal–truncated mutant. Comparison of the characteristics of LCMV infection-induced HLH in the murine counterparts of the 3 human conditions revealed a similar gradient in the phenotypic severity of HLH manifestations. Strikingly, the severity of HLH was not correlated with the LCMV load and not fully with differences in the intensity of cytotoxic activity. The capacity of antigen presentation differed in vivo between Rab27a- and Syntaxin-11–deficient mutants. Our data indicate that cytotoxic effectors may have other immune-regulatory roles in addition to their role in controlling viral replication.
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Tanboga, Ilknur, Betul Kargul, Semra Ergeneli, M. Yalcin Aydin, and Metin Atasu. "Clinical features of incontinentia pigmenti with emphasis on dermatoglyphic findings." Journal of Clinical Pediatric Dentistry 25, no. 2 (January 1, 2002): 161–65. http://dx.doi.org/10.17796/jcpd.25.2.u14797172m01w72v.
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Incontinentia pigmenti is a syndrome characterized by both systemic and oral manifestations. Presented here are the dental, clinical, radiological, genetic and dermatoglyphic findings of a 6 year old female case and her family members. The following features were apparent: oligodontia in maxillary and mandibular arches in both dentition, peg-shaped incisors and brown lessions on the body surface. No other problems were observed. The case had remarkable dermatoglyphic findings such as hypothenar loops associated with distally displaced axial triradii on both palms, reduced total finger and summed palmar a-b ridge-counts, decreasing plantar pattern intensity on the left sole. The other family members had similar dermatoglyphic characteristics. The paternal grand father, the father and the brother had eye defects.
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Bagnenko, E. S., E. R. Araviiskaia, A. I. Bogatenkov, and S. S. Bagnenko. "The correlation of clinical and psychological characteristics of women seeking cosmetic help." Vestnik dermatologii i venerologii 97, no. 5 (December 23, 2021): 66–75. http://dx.doi.org/10.25208/vdv1246.
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Background. Patients seeking cosmetic help, most commonly, haven`t only defects in appearance, in particular, facial skin, but also experience certain psychological problems in this regard. On the other hand, the reaction to the performed cosmetic correction of the mentioned defects depends, including on the patients psychological characteristics. The study of the correlation between clinical and psychological characteristics should contribute to the individualization of the cosmetological correction process and optimization of the patient life quality.
Aims. To establish the correlation between age, clinical and psychological characteristics of women with cosmetic problems of the facial skin.
Materials and methods. Using the methods of psychological diagnostics, we examined 110 women who received cosmetic assistance. Semi-quantitative method was used to assess the cosmetic problem intensity, the duration of its existence and the effect of the correction performed. Clinical and psychological research was carried out using the author's structured interview, which questions were aimed at fixing both the formal social positions of the patients and the characteristics of the system of significant relationships. Psychometric research was carried out using standardized questionnaires in order to identify a wide range of personality characteristics. In the course of statistical processing of the results, a frequency analysis of the gradations of nominative and rank signs was carried out using Pearson's 2, as well as a correlation analysis of clinical and psychological characteristics using the Spearman -test.
Results. The author traced the statistical correlation between the degree of manifestation of the cosmetic problem and its influence on the psychological state of patients, as well as between the degree of their satisfaction with its solution and a number of personal characteristics and attitudes.
Conclusions. The assumption was confirmed that the effect of treatment is determined not only by clinical factors and the possibilities of modern cosmetology, but also by psychological factors, among which the motivational orientation and activity of the individual, which form the basis of patient compliance, are of decisive importance.
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Pietrucha, Barbara, Hanna Gregorek, Edyta Heropolitańska-Pliszka, and Ewa Bernatowska. "Primary Immunodeficiency with double strain break DNA (DSBs) and radiosensitvity: clinical, diagnostic and therapeutic implications." Postępy Higieny i Medycyny Doświadczalnej 72 (May 17, 2018): 449–60. http://dx.doi.org/10.5604/01.3001.0012.0547.
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Primary Immunodeficiencies (PNO) are a group of about 300 genetic disorders which result from the absence or dysfunction of the major components of the immune system. Among them an important subgroup constitute deficiencies associated with defects in DNA double strand breaks (DSBs) recognition and repair. These are primarily radiation-sensitive severe combined immune deficiencies (SCIDs) and combined immune deficiencies (CIDs) associated with genetic defects in the DNA-repair genes, which encode proteins necessary for T-cell and B cell maturation/differentiation. Due to increased risk of developing malignant neoplasms, mainly from hematopoietic origin, and over-reaction to standard anticancer radiotherapy and chemotherapy, treatment of these patients is a real challenge for clinicians. Clinical and laboratory manifestations, which may indicate increased radiosensitivity include: microcephaly, telangiectasias, lymphopenia, and translocation of chromosomes 7 and 14 in karyotype. A basic test showing increased radiosensitivity of lymphoblastoid cells lines or skin fibroblasts is percentage evaluation of their survival after exposition to ionizing radiation. Treatment of patients with impaired DNA repair depends on the clinical picture, immunological findings and type of immunodeficiency. Patients with SCID require immediate hematopoietic stem cell transplantation (HSCT) using reduced intensity conditioning (RIC). In patients with CID, standard treatment regimens require modification and/or avoidance of radiotherapy and some radiomimetic agents.
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Shevchenko, S. "Dynamics of hematological parameters, macromorphological and radiological results of reparative osteogenesis in rabbits for the use of platelet concentrates and hydroxyapatite ceramics." Naukovij vìsnik veterinarnoï medicini, no. 1(154) (May 21, 2020): 153–64. http://dx.doi.org/10.33245/2310-4902-2020-154-1-153-164.
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The results of the dynamics of the morphological parameters of the blood of a rabbit with model defects of the opening of the spongy and compact bone tissue at diff erent periods of reparative osteogenesis are presented. Formed 4 groups of rabbits. Bone lesions in the fi rst experimental group were fi lled with injectable platelet-rich fi brin, in the second - platelet-rich fi brin, in the third - a combination of platelet-rich fi brin, and hydroxyapatite with β-tricalcium phosphate.In the control group, the defects remained healed under a blood clot. All animals were in the same conditions of feeding and keeping, had unlimited access to water. During the study, rabbits were clinically observed. Blood was taken for morphological examination before surgery and on the 3rd, 7th, 14th, 21st, 42nd day. Animals were taken from the experiment on the 14th, 21st and 42nd days, an X-ray examination was carried out, bone tissue samples were taken. It was established that trauma of bone tissue leads to a number of reactions of the body aimed at restoring the damaged area. Against the background of the general picture of the obtained morphological results, the level of platelets signifi cantly changes compared to the physiological norm, while the number of red blood cells and white blood cells does not go beyond it. There is a slight increase in hemoglobin levels, especially in the experimental groups on the twenty-fi rst and forty-second day. Signifi cant changes in the number of red blood cells, white blood cells, platelets and hemoglobin concentration in the experimental groups were noted compared with the control group at diff erent periods of reparative osteogenesis.The use of various types of platelet concentrates aff ects the overall reaction of the body. On radiographs of the radial bones (compact bone tissue) on the twenty-fi rst day in the second and third experimental groups, where fi brin enriched with platelets and its combination with hydroxyapatite materials with β-tricalcium phosphate were used to replace bone defects, the infl ammatory reaction was manifested to a lesser extent. There was no signifi cant formation of bone callus compared with the fi rst, in which model defects were fi lled with injectable platelet-rich fi brin and the control group. Each of the types of platelet concentrates and their combination with hydroxyapatite ceramics have a diff erent eff ect on the restoration of bone defects and is accompanied by the appearance of a number of reactions, both local and general. According to the degree of intensity of this process, they can be placed in the following sequence: i-PRF ˂ PRF ˂ PRF+GT. Macromorphologically and radiologically signifi cant diff erences were not detected for diff erent types of bone tissue due to the use of each of the substances that concentrates platelets. The combination of hydroxyapatite granules with β-tricalcium phosphate and platelet-rich fi brin proved to be the best option for repairing the damaged area. Its use provides a signifi cantly smaller manifestation of the local infl ammatory reaction and causes the formation of optimal bone marrow. Key words: PRF, i-PRF, granules, centrifuges, growth factors, bone marrow.
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Mentl, Vaclav, Frantisek Vlasic, Denisa Bartkova, and Pavel Mazal. "Application of Acoustic Emission for Identification of Differences in Fatigue Damage of Selected Materials for Power Plants." Key Engineering Materials 627 (September 2014): 313–16. http://dx.doi.org/10.4028/www.scientific.net/kem.627.313.
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An acoustic emission is remarkable source of information about the fatigue process and its intensity under cyclic loading. Specimens made of reactor steel and INCONEL 713LC were subjected to bending fatigue loading in the high-cycle range. This study presents results of acoustic emission signal analysis. The main aim of this study is to propose a methodology for evaluation of the early manifestations of fatigue damage and to identify material changes in both materials by AE parameters. Signal comparison material indicates differences of damage mechanism in observed. An examination of crack initiation sites and microstructure has been also performed.Experiments were realized in cooperation between laboratories of Brno University of Technology and University of West Bohemia in Pilsen and its related to solving of project of the Czech Ministry of Industry and Commerce: “A diagnostic complex for the detection of pressure media and material defects in pressure components of nuclear and classic power plants“ and project New Technologies for Mechanical Engineering (NETME +).
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Rodionova, Olga G., Vitaliy I. Voshedskiy, Pavel G. Sakun, Elena A. Sheiko, Marina A. Gusareva, Tatiana G. Chalabova, Alla V. Pustovalova, et al. "Experience of low-intensity LED radiation (LILEDR) in combination treatment of radiation-induced rectal injuries." Journal of Clinical Oncology 39, no. 15_suppl (May 20, 2021): e17514-e17514. http://dx.doi.org/10.1200/jco.2021.39.15_suppl.e17514.
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e17514 Background: Malignant pelvic tumors account for more than 25% of cancer incidence in Russia. Radiation therapy is the most common treatment for such patients; however, 10-15% of patients develop radiation-induced complications of the pelvic organs, and more effective treatments are required to manage these complications. Methods: The study included 30 patients with cervical cancer T3NхM0 after combination treatment. 7-10 months after combined radiation treatment (total radiation dose to the primary focus 80 Gy), patients developed erosive ulcerative radiation rectitis (RTOG grade 1 and 2). Patients were divided into 2 groups: main group (n = 15) – conservative treatment combined with LILEDR. Each course included 10 LILEDR sessions, the red spectrum λ = 640 nm on the cubital vein projection (exposure time 5 minutes, dose 6.86 J/cm2) and locally on the ulcerated zones (exposure time 3 minutes, dose 3.96 J/cm2). Patients received 2 LILEDR courses with a 1-month interval. The control group received only conservative therapy. Results: Main clinical manifestations of rectitis (tenesmus, bloody mucous discharge) disappeared in the main group already on the 3-4th day of the first course, epithelialization of ulcerative defects occurred in a shorter period of 7-10 days. Soft superficial scars not causing rectal stenosis formed at the site of the ulcer by the end of LILEDR courses. The control group showed long periods of the ulcer epithelialization up to 30 days, late remission and a lingering recurrent character of the disease. Conclusions: LILEDR in combination with the main conservative therapy allows rapid managing with the clinical symptoms of radiation rectitis and regression of disorders developed after the complex treatment, which improves the quality of life of patients and shortens the rehabilitation period.
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Meuleman, Nathalie, C. Rocha, Jalil Bennani, Tatiana Tondreau, Alain Delforge, Philippe Lewalle, Philippe Martiat, Laurence Lagneaux, and Dominique Bron. "Reduced Intensity Conditioning Hematopoietic Stem Cell Transplantation (HSCT) with Mesenchymal Stem Cells (MSC) Infusion for Treatment of Metachromatic Leukodystrophy (MLD): A Case Report." Blood 108, no. 11 (November 16, 2006): 5255. http://dx.doi.org/10.1182/blood.v108.11.5255.5255.
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Abstract Background: Patients (pts) with MLD have neurological and musculo-skeletal defects with a limited survival. HSCT has been reported as an effective treatment to stabilize or improve defects associated with this disease. Koç and colleagues reported that donor allogeneic MSC infusion is safe and may be associated with reversal of the disease pathophysiology in some tissues (Bone Marrow transplant, 2002). We decide thus to perform a non myeloablative familial HSCT in adult pt with a symptomatic MLD in order to evaluate the safety and the benefit allogeneic HSCT with MSC infusion in a patient with MLD. An informed consent was obtained from the pt. Patient: a 23 years old women who presented an adult form of MLD for three years was admitted in our department. The most important symptoms associated with the disease were dizziness, proximal weakness of the lower limbs, difficulty to walk, disorder of the memory and urinary incontinence. The reduced intensity conditioning was preferred to decrease the morbidity and mortality of the procedure. MSC were isolated and amplified from the BM-mononuclear cells of the HSC donor. MSC expansion was made in a commercial serum-free medium (UltraCulture, Cambrex, Walkersville, MD) supplemented with a serum substitute (Ultroser, Pall Biosepra, Cergy-Saint-Christophe, France) as previously reported by our group (Eur J Haematol, 2006). Ex vivo expanded allogeneic MSC were intravenously infused at the dose of 1×106 MSC/kg of recipient body weight. The conditioning regimen of the HSCT consisted in Fludarabine and ATG combination and 5× 106 CD34 cells were infused concomitantly with 1.106 MSC cells. We did not observed any immediate or delayed side effects after the MSC infusion. The patient did not presented any complications after the HSCT. At day 8 of the transplantation she had an normal hematological recovery. The platelet nadir was 72.000/mm3 and she did not need any transfusion. With regards of her neurological status, since 22 months the patient had no new deterioration and we observed a stabilisation of the clinical manifestations. Conclusions: This case report suggests the feasibility and the potential efficacy of reduced intensity conditioning allogeneic HSCT with MSC infusion for patient with MLD. A larger trial is required to confirm this observation.
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Roth, Batia, Galia Ronen, Dror Rosengarten, Alexander Brill, Shoshana Vilk, Tony Futerman, Swetlana Boldin-Adamsky, Ari Zimran, and David Varon. "Platelet Adhesion Defect Is Common in Gaucher Disease." Blood 108, no. 11 (November 16, 2006): 1105. http://dx.doi.org/10.1182/blood.v108.11.1105.1105.
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Abstract Background: Bleeding diathesis is a common manifestation of Gaucher disease, usually attributed to thrombocytopenia due to hypersplenism and bone marrow infiltration by Gaucher cells. Hemorrhagic manifestations are not restricted to thrombocytopenic patients, and other mechanisms, e.g. platelet function defect, manifested in abnormal aggregation, have also been implicated. Aim: to evaluate platelet adhesion properties in Gaucher patients. Methods: Platelet function was studied in 44 Gaucher patients with a platelet count of ≥130×109/L, using a novel method for quantitative analysis of whole blood platelet interaction with immobilized plasma ligands under flow conditions, the Impact-R [Cone and Plate(let) analyzer] test. The results were compared to platelet function of 51 obligatory Gaucher carriers, 20 healthy volunteers and 5 transgenic Gaucher mice. Platelet’s glucocerebrosidase function was tested in platelet’s extract by incubation with C6-NBD-glucosylceramide, and by testing the fluorescence intensity of the end product, C6-NBD-ceramide. Results: Adhesion defect, manifested by surface coverage (SC) < 7.0%, was found in 70.4% of the patients (SC 5.1%±3.1), in 41.2% of the Gaucher carriers (SC 7.7%±2.8) and only in 20% of the control group (SC 8.7%±3.2, P=0.0007 and 0.09, respectively). This defect was independent of platelet count, or hematocrit. Further support for this observation was found in transgenic Gaucher mice, who also had low platelet adhesion compared to wild type mice (SC of 2.7%±0.6 vs. 12.5%±2.9, respectively). Average size of platelet aggregates was lower in Gaucher patients with bleeding tendency, compared to those with no bleeding history (23.5 μm2±12 and 29.2±10, p=0.026). A trend for lower SC in bleeders vs. non bleeders was also found (4.3±3.7 vs. 6.4±3.9, p=0.069). No difference was found in platelet counts between bleeder and non-bleeders (p=0.13). Surface coverage was higher in splenectomized patients and in patients receiving enzyme replacement therapy. Highest SC was observed in patients under both treatment modalities, SC= 8±4.6, compared to those treated with one modality, 4.9±3.7, and untreated ones, 3.3±2.3 (p=0.01). These differences were independent of platelet count. In order to elucidate the potential role of glucoceramide in the adhesion defect, we tested platelet’s glucocerebrozidase function in patients and controls. The specific activity thus calculated in 5 mildly thrombocytopenic Gaucher patients was 0.02–0.08 pmol/min/μgr protein compared to specific activity of 0.14-0.15 pmol/min/μg protein in platelet homogenate of 3 healthy volunteers. Conclusions: platelet adhesion defect is common in Gaucher disease, which may account for bleeding events in some patients with normal platelet count. Accumulation of glucoceramide in Gaucher platelets may be responsible for this defect. Further studies into the potential role of the stored material in the adhesion defect and bleeding phenomena in Gaucher is warranted.
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Zhmailik, Ruslan, Vasiliy Bogdan, Yulia Korneva, and Alexandr Dorosevich. "Morphological Features of Wound Regeneration in the Local Application of Recombinant Interferon Α2b in the Experiment." Vestnik of Experimental and Clinical Surgery 10, no. 3 (November 19, 2017): 225–30. http://dx.doi.org/10.18499/2070-478x-2017-10-3-225-230.
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Summery. Introduction. The research devoted to the study of pathogenetic, immunological, and biophysical aspects of the tissue regeneration process with wound defects of various etiologies is gaining in popularity in the "world" of medical science, at present this problem remains topical [1, 2, 6, 8, 9]
Purpose of the study. A comparative analysis of the dynamics of morphological manifestations of tissue regeneration occurring when recombinant interferon α2b, is used in the healing of an acute experimental wound is carried out.
Materials and methods. Depending on the nature of the wound treatment, the experimental animals were divided into two groups: 1) a comparison group in which the edges of the wound were struck with a 0.9% NaCl solution; 2) the main group, where a similar edge of the wound defect was cut off with recombinant interferon α2b at a dose of 1000 ME / 1ml diluted with 0.9% NaCl solution. The rats were removed from the experiment on the 4th, 8th and 20th day of the study, while a full-thickness skin flap was taken in the area of the scar (defect), which was examined by light microscopy. At the same time, the intensity of formation of the granulation tissue, its "maturation", and also in the margins and on the bottom of the wound was estimated in the absolute values, the cellular populations realizing the process of inflammation were determined.
As a result of the study, the character of the complex effect of recombinant interferon α2b was established, which included reduction of the negative effect of secondary alteration, differentiation of the intravascular immune response, formation of conditions for natural regeneration with activation of angiogenesis and synthesis of a valuable scar, decrease in propensity to keloidosis.
Conclusions. The obtained results testify to the morphological validity of the use of recombinant leukocyte interferon α2β as a stimulant of the inflammation phase, as against the background of the use of this substance, its ability to enhance phagocytosis was noted.
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Magerus-Chatinet, Aude, Benedicte Neven, Marie-Claude Stolzenberg, Cecile Daussy, Peter Arkwright, Marina Cavazzana-Calvo, Capucine Picard, Alain Fischer, and Frederic Rieux-Laucat. "Onset of Autoimmunity In ALPS as a Consequence of Genetic Defects Accumulation." Blood 116, no. 21 (November 19, 2010): 278. http://dx.doi.org/10.1182/blood.v116.21.278.278.
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Abstract Abstract 278 Autoimmune diseases develop in about 5% of human beings and arise with a delayed stochastic penetrance when lymphocytes directed towards self components, such as chromatin in lupus or insulin-producing cells in diabetes, are generated. This occurs when self-tolerance checkpoints of the immune system are bypassed as a consequence of inherited mutations of key genes involved in activation, survival or death of lymphocytes. The autoimmune lymphoproliferative syndrome (ALPS) is a model of self-tolerance checkpoints defects as autoimmune manifestations occur in 2/3 of ALPS patients. Mutations in the TNFRSF6 gene (encoding FAS, also known as the CD95 receptor) is the most common cause of ALPS. Patients carrying a homozygous TNFRSF6 mutation have an early-onset, severe phenotype, whereas subjects with heterozygous TNFRSF6 mutations present ALPS of variable intensity. T cells from patients with homozygous or heterozygous TNFRSF6 mutations respectively exhibit a complete or partial functional impairment in an in vitro FAS-induced apoptosis assay. Moreover, we recently described somatic heterozygous mutations in TNFRSF6 in patients with a clinical ALPS phenotype in which activated T cells showed normal sensitivity to FAS-induced apoptosis in vitro. The inability to detect an apoptosis defect was due to spontaneous in vitro apoptosis of the mutant T cells. However, more than 80% of these patients' DN T cells were mutated. These patients displayed mosaic expression of a somatic TNFRSF6 mutation, which provided the affected cells a selective advantage and accounted for the observed lymphoproliferation and autoimmunity. Heterozygous germline TNFRSF6 mutations are not always associated with clinical expression (partial clinical penetrance) since some mutation-positive relatives (MPRs) remain asymptomatic despite an in vitro FAS-induced apoptosis impairment on their T cells. This observation led us to postulate that a second event is necessary for disease expression in patients with TNFRSF6 mutations when an incomplete penetrance is observed. We analyzed double-negative (DN) T lymphocytes from seven ALPS patients with low penetrance mutations for FAS expression and defects in the second TNFRSF6 allele and compared with data from healthy, mutation-positive relatives and controls. Missense mutations (in three patients) and loss of heterozygosity (LOH, in four patients) were found in the second TNFRSF6 allele. The LOH observed in 4 of our patients with germline TNFRSF6 ECD mutations resulted from loss of the wild-type allele and concomitant duplication of the mutant one, i.e. telomeric UPD. This type of mechanism is usually observed in genetic disorders of development, where it can be involved in trisomy or monosomy rescue during embryonic development. In 6 patients, alteration or loss of the second TNFRSF6 allele resulted in the absence of FAS cell surface expression on DN T lymphocytes. In these seven patients the disease develop as a consequence of somatic genetic events of TNFRSF6 in a subpopulation of lymphocytes combined with inherited heterozygous mutations of the same gene. This finding is the first demonstration that combined germline and somatic mutations of the same gene is leading to a non-malignant autoimmune disease in humans. Disclosures: No relevant conflicts of interest to declare.
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Avraamova, O. G., N. P. Kalashnikova, V. V. Goryacheva, and T. V. Kulazhenko. "Diagnosis of early forms of dental caries in а primary school children." Pediatric dentistry and dental profilaxis 20, no. 3 (October 10, 2020): 230–34. http://dx.doi.org/10.33925/1683-3031-2020-20-3-230-234.
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Relevance. At present the question of finding and applying effective methods and approaches for diagnosing early manifestations of dental caries in the form of foci of demineralization during eruption of permanent teeth in children remains an important and relevant issue. Timely diagnosis at the age of 6-7 years prevents the transition of the initial forms of caries into carious defects and further excludes the use of invasive methods of surgical recovery treatment. The aim is improving the approach of caries diagnostics approach by identifying foci of demineralization and hidden carious cavities in children during teething of permanent teeth.Materials and methods. An epidemiological examination of 380 children in Moscow aged 6-7 years was carried out. Of the total number of children examined by the method of randomization 150 people were selected, which are divided into 3 groups depending on the intensity of caries. Children of each group were diagnosed with caries using various diagnostic methods – visual inspection, vital staining, hardware method (Estus-LED-Alladin Multicolor (Geosoft, Russia).Results. In children 6-7 years of age in Moscow, the average prevalence and intensity of caries was established. However, the epidemiological examination does not take into account the number of foci of demineralization and hidden carious cavities, which can subsequently be transformed into destructive forms and cause an increase in caries. This indicates the need to improve the diagnostic approach using different methods for identifying early forms of caries. When using the hardware method, a greater number of foci of demineralization and hidden carious cavities were revealed on all surfaces of permanent teeth. There was a tendency to an increase in the number of foci of demineralization and hidden carious cavities depending on the intensity of caries.Conclusions. The effectiveness of the hardware method in the group of children DMF = 0 was 40,9% in comparison with the visual method and 36,4% in comparison with vital staining; with DMF = 1-2 – 35,4% in comparison with other methods, with DMF ≥ 3 – 43.3% in comparison with the visual and 40% in comparison with the vital. Diagnosis of early forms of caries made it possible to prescribe treatment and preventive measures in a timely manner and further reduce the growth of caries.
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Prabahran, Ashvind Anand, James Anton Kuzich, Andrew B. M. Lim, Eric Wong, and David Ritchie. "Significant EBV Reactivation Is Associated with Poorer Overall Survival Due to Increased NRM Post Myeloablative/Reduced Intensity, T-Cell Deplete Allogeneic Stem Cell Transplant for MDS or Acute Leukaemia." Blood 136, Supplement 1 (November 5, 2020): 27. http://dx.doi.org/10.1182/blood-2020-142053.
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Introduction EBV reactivation post allogeneic stem cell transplantation (alloSCT) occurs due to profound suppression of T-cell number or function and subsequent reactivation of latent host or recipient EBV infection through loss of immune-surveillance. The relationship of EBV reactivation to patient outcomes has not been reported in patients who have received T-cell depletion (TCD) with Thymoglobulin. This analysis assessed the outcomes in patients who had previously received an alloSCT with TCD using Thymoglobulin and who developed EBV reactivation that either required treatment (EBV Treatment) vs those who did not require treatment (EBV No Treatment) vs those who had no evidence of EBV reactivation (No EBV). Methods This was a retrospective review of all patients who received TCD with Thymoglobulin prior to alloSCT at the Royal Melbourne Hospital between 2006-2017. Eligible patients received reduced intensity conditioning (RIC) or myeloablative conditioning (MAC) for acute leukemia (AL) or myelodysplastic syndrome (MDS). EBV was monitored weekly via semi-quantitative PCR . Preemptive rituximab was given based on peak viral loads, rate of rise in viral loads or biopsy proven post-transplant lymphoproliferative disorder (PTLD). Categorical and continuous baseline characteristics of the cohorts were compared using chi-squared and Kruskal-Wallis tests respectively. EBV Treatment was treated as a time-dependent variable and evaluated for its relationship to overall survival (OS) using Cox proportional modelling, adjusted for age and high or very high Disease Risk Index (DRI) and grade III-IV acute GVHD. Survival estimates were performed by Kaplan-Meier product-limit method. Cumulative incidence (CI) of relapse and non-relapse mortality (NRM) were calculated by the Fine-Gray method. Results Of 165 eligible patients, 101 (61%) had an episode of EBV reactivation at a median of 46 days post-transplant (range 10-221 days). 24 received therapy for EBV. Of these, 4 had biopsy-proven PTLD, and 23 received rituximab alone with 1 receiving R-CHOP for PTLD. An undetectable EBV viral load was achieved in 20 out of 24. The baseline characteristics of the subgroups are presented in Table 1. The median survival of the whole cohort was 79 months with an estimated 2-year OS of 60% (95%CI 56-68) and a median follow up of time of 81 months in survivors. The median survival of the EBV Treatment group was 14 months, compared with 121 months and 81.1 months in the EBV No Treatment and No EBV cohorts respectively (Figure 1). Table 2 lists causes of death per group with relapse and complications of poor graft function (PGF) being the leading causes of death in the EBV Treatment group. The incidence of grade III-IV acute GVHD at 100 days was similar between groups as was the incidence of any chronic GVHD at 6 and 12 months. EBV Treatment was significantly associated with poorer OS even when adjusting for age, high/very high DRI and presence of acute GVHD (HR 2.47 CI 1.46-4.17; p=0.0007). The EBV Treatment group had a significantly higher 2 year CI of NRM compared to the EBV Treatment and No EBV groups (38% vs 11% vs 18% p=0.006) but similar incidence of relapse (Figure 2) . Conclusion In this cohort of AL/MDS patients undergoing TCD alloSCT, those who required treatment for EBV reactivation experienced poorer survival due to increased NRM. This increase in NRM may reflect impaired immune reconstitution of which significant EBV reactivation is a manifestation. Further studies to understand the underlying immune defects post alloSCT that predispose patients to significant EBV reactivation and associated complications is warranted to identify immune recovery strategies that may be employed in this poor prognosis group. Disclosures No relevant conflicts of interest to declare.
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Pugh, Christopher J. A., Victoria S. Sprung, Graham J. Kemp, Paul Richardson, Fariba Shojaee-Moradie, A. Margot Umpleby, Daniel J. Green, N. Timothy Cable, Helen Jones, and Daniel J. Cuthbertson. "Exercise training reverses endothelial dysfunction in nonalcoholic fatty liver disease." American Journal of Physiology-Heart and Circulatory Physiology 307, no. 9 (November 1, 2014): H1298—H1306. http://dx.doi.org/10.1152/ajpheart.00306.2014.
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Nonalcoholic fatty liver disease (NAFLD) is an independent risk factor for cardiovascular disease (CVD). Endothelial dysfunction is an early manifestation of atherosclerosis and an important prognostic marker for future cardiovascular events. The aim of this study was twofold: to examine 1) the association between liver fat, visceral adipose tissue (VAT), and endothelial dysfunction in obese NAFLD patients and 2) the impact of supervised exercise training on this vascular defect. Brachial artery endothelial function was assessed by flow-mediated dilatation (FMD) in 34 obese NAFLD patients and 20 obese controls of similar age and cardiorespiratory fitness [peak oxygen uptake (V̇o2 peak)] (48 ± 2 vs. 47 ± 2 yr; 27 ± 1 vs. 26 ± 2 ml·kg−1·min−1−1). Magnetic resonance imaging and spectroscopy quantified abdominal and liver fat, respectively. Twenty-one NAFLD patients completed either 16 wk of supervised moderate-intensity exercise training ( n = 13) or conventional care ( n = 8). Differences between NAFLD and controls were compared using independent t-tests and effects of interventions by analysis of covariance. NAFLD patients had higher liver fat [11.6% (95% CI = 7.4, 18.1), P < 0.0005] and VAT [1.6 liters (95% CI = 1.2, 2.0), P < 0.0001] than controls and exhibited impaired FMD compared with controls [−3.6% (95% CI = −4.9, −2.2), P < 0.0001]. FMD was inversely correlated with VAT ( r = −0.54, P = 0.001) in NAFLD, although the impairment in FMD remained following covariate adjustment for VAT [3.1% (95% CI = 1.8, 4.5), P < 0.001]. Exercise training, but not conventional care, significantly improved V̇o2 peak [9.1 ml·kg−1·min−1 (95% CI = 4.1, 14.1); P = 0.001] and FMD [3.6% (95% CI = 1.6, 5.7), P = 0.002]. Endothelial dysfunction in NAFLD cannot be fully explained by excess VAT but can be reversed with exercise training; this has potential implications for the primary prevention of CVD in NAFLD.
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Pazdur, Małgorzata. "Nursing care for patients with rare diseases on the example of patients with Alternating Hemiplegia of Childhood." Health Promotion & Physical Activity 5, no. 4 (February 14, 2019): 12–21. http://dx.doi.org/10.5604/01.3001.0013.0307.
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Introduction: Rare diseases are those which occur in 1 person in 2000. Usually they are genetically conditioned. They also comprise diseases which appear as a result of metabolic, immunological defects, infections and uncommon cancers. Development of science, especially genetics prompted discovery of new gene’s mutations. One of them is a mutation of ATP1A3 which alternating hemiplegia of childhood. It is a disease whose manifestations appear in early childhood and are unpredictable. Strokes can be hemiplegic or tetraplegic. Sometimes they are accompanied by problems with swallowing and breathing. The aim of the thesis was to compare 2 different cases of patients suffering from AHC, to show problems connected with taking care of them in bio-psycho-socio-spiritual dimensions and to present a model of care on the example of Dorothey Orem’s theory. Material and methods: The comparative method used in the work compares two cases of patients. It is a quality work. Techniques of interview, observation, measurement and documentation analysis were also applied. An interview questionnaire containing 13 questions and an observation conducted in everyday circumstances made it possible to show care problems of patients with AHC. The research was carried out from March 2018 to May 2018. Two cases were juxtaposed: a 10-year-old girl and a 34-year-old man. Results and conclusions: Patients who suffer from rare diseases have restricted access to diagnosis, cure, rehabilitation and social assistance. They often grapple with lack of understanding on the part of society and medical staff who do not have enough knowledge about this issue. By comparing 2 cases it was proved that care problems of these patients are very complicated and vary depending on age, type of stroke, intensity, duration of the disease and family’s and society’s support. The care system of Dorothey Orem seems to be the best one.
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Kamut, N. V., and M. M. Kiselova. "PECULIARITIES OF CLINICAL PASSING OF ATOPIC DERMATITIS AND VITAMIN D EXCHANGE IN EARLY CHILDREN." Likarska sprava, no. 3-4 (June 30, 2020): 27–35. http://dx.doi.org/10.31640/jvd.3-4.2020(5).
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The article is devoted to the study of features of clinical course of atopic dermatitis and vitamin D exchange in young children conducted by us by estimation of severity of atopic dermatitis depending on the value of SCORAD index, determination of 25(OH)D concentration in serum of children by immunochemical method with chemocentric analysis according to the classification approved by experts of the international endocrinological society. In the examination of 188 young children, two groups were formed on the basis of the Lviv City Children's Clinical Hospital. Children from 1 month to 1 year of age with manifestations of atopic dermatitis (n = 120), who were born full-term and had no birth defects, metabolic disorders, severe perinatal CNS lesions formed the main group. The comparison group consisted of 68 healthy young children without chronic diseases. All children had atopic dermatitis in the main group and were classified by severity (depending on the SCORAD index): children with mild severity – 31 (26 %), moderate 47 (39 %), and severe 42 (35 %). As a result of the study, we found a association between the severity of clinical manifestations of atopic dermatitis and the level of vitamin D in the serum. Comparative analysis of the severity of atopic dermatitis, depending on the level of vitamin D in the serum, showed that with severe atopic dermatitis in children of the main group, the level of 25(OH)D was significantly lower compared to its indicators in children with moderate to severe (P < 0.05). Serum vitamin D levels are significantly lower in all forms of atopic dermatitis severity and correlate significantly with the overall SCORAD severity score, erythema intensity, excoriation, itching, and sleep disturbances. The scientific data on the presence of feedback correlation (r = –0,48; P < 0,05) between the development of atopic dermatitis in children and their level of 25(OH)D in serum were added. Our findings suggest that there is a need for a differentiated approach to treating early-stage atopic dermatitis of varying severity, a personalized therapeutic approach to choosing a vitamin D supplementation, given the level of 25(OH)D in the serum.
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Arafah, Baha M., Juan Ybarra, Robert W. Tarr, Zuhayr T. Madhun, and Warren R. Selman. "Pituitary Tumor Apoplexy: Pathophysiology, Clinical Manifestations, and Management." Journal of Intensive Care Medicine 12, no. 3 (May 1997): 123–34. http://dx.doi.org/10.1177/088506669701200303.
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Pituitary tumor apoplexy represents a rare clinical syndrome caused by hemorrhagic infarction of an existing, often previously unrecognized, large adenoma. We present our approach and experience in the field, and we provide a summary of pertinent published literature addressing diagnosis, management, and pathophysiology of the clinical manifestations of pituitary tumor apoplexy. Although many precipitating factors are known, most episodes occur spontaneously and present clinically with relatively sudden onset of severe headache, visual field defects, and ophthalmoplegia. Either computed tomography (nonenhanced and enhanced) or magnetic resonance imaging in a symptomatic patient can define pertinent anatomical changes and aid in establishing diagnosis. Immediate treatment with corticosteroids is necessary. Patients with the mild form of the syndrome respond quickly to steroids and can be managed conservatively with continued glucocorticoid therapy. Most patients with persistent neural deficits require urgent transsphenoidal decompression. Unless surgery is delayed, results of transsphenoidal decompression are usually very good, and improvements in neurological symptoms and endocrine function are noted within hours to days of the procedure. Patients need to be closely followed up after the episode because a large number continue to have residual tumor requiring additional therapy, or they have pituitary hormone abnormalities that need treatment. Close interaction between neurosurgeons and endocrinologists in the management of patients with pituitary tumor apoplexy is essential for optimal outcome.
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Narivskyi, A. V., A. N. Smirnov, N. I. Tarasevich, and S. Ye Kondratyuk. "Chemical and physical heterogeneities and gases in large steel ingot." Metaloznavstvo ta obrobka metalìv 96, no. 4 (December 1, 2020): 3–9. http://dx.doi.org/10.15407/mom2020.04.003.
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For creation of the high-tech equipment that is used in energy, heavy engineering, chemistry and transport, the unique large-sized steel products are required. In the manufacture of such products, large forging ingots in the mass to 600 tons are used. However, an increase in the mass of the ingots leads to the formation of chemical and physical heterogeneity, enlargement and unfavorable distribution of non-metallic inclusions, of the development of segregation defects in them, which reduce the strength and exploitation characteristics of the metal. In this connection, the quality forgings and finished parts are not always meet the producing demands and the loss of metal, in the form of technological waste and rejects are reaching significant values. It is known that eccentric zonal segregation, especially it’s the most dangerous variety - cords, significantly reduce the quality and properties of products from large steel ingots. In connection with the continuous expansion of the production of large ingots, the problem of creating optimal technologies for their formation, which reduce or exclude the possibility of the formation of chemical heterogeneity and cords in steel during crystallization, it is currently important and relevant. In this paper it are presented the results of studies of the structure, gas distribution, physical and chemical heterogeneities in the cross section and height of an ingot in the mass of 140 tons, which was casted in vacuum from steel 25KHN3MFA. It is shown that depending on the temperature and time conditions of ingot hardening, among which the crystallization interval (due to the chemical composition of steels), cooling intensity in different volumes in height and cross section of ingot, temperature gradient before the crystallization front, solubility of alloying elements and gas content in the melt, etc. Based on this, when developing technology for large ingots to ensure their quality, optimal structure and properties should take into account not only their dimensions, but also the combination of these thermokinetic parameters on the crystallization process, dendritic structure formation, manifestations of liquation in different ingot volumes. Keywords: ingot, segregation strip and inclusions, dendrites, structure, oxygen, oxides, sulfides.
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Kang Kim, Hyun. "Intensity, Manifestation, and Performance in the Politics of the Real." International Journal of Philosophy 7, no. 1 (2019): 24. http://dx.doi.org/10.11648/j.ijp.20190701.14.
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Starukhin, A. S., L. L. Gladkov, and A. M. Shul'ga. "Manifestation of vibronic intensity borrowing in fluorescence spectra of metalloporphyrins." Journal of Applied Spectroscopy 66, no. 4 (July 1999): 570–74. http://dx.doi.org/10.1007/bf02675388.
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Zaalishvili, V. B. "Features of the earthquakes’ manifestation intensity formation in mountainous areas." IOP Conference Series: Materials Science and Engineering 1083, no. 1 (February 1, 2021): 012086. http://dx.doi.org/10.1088/1757-899x/1083/1/012086.
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Bokotey, O. V., V. V. Vakulchak, A. A. Bokotey, and I. I. Nebola. "Manifestation of Point Defects in the Electronic Structure of Hg3Te2Cl2 Crystals." Ukrainian Journal of Physics 61, no. 10 (October 2016): 901–8. http://dx.doi.org/10.15407/ujpe61.10.0901.
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Polit, J., E. M. Sheregii, J. Cebulski, M. Pociask, A. Kisiel, A. Mycielski, B. V. Robouch, et al. "Manifestation of defects in phonon spectra of binary zinc-blende compounds." European Physical Journal Applied Physics 27, no. 1-3 (July 2004): 321–24. http://dx.doi.org/10.1051/epjap:2004078.
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Sanchez-Dalmau, B., F. J. Goni, M. Guarro, C. Roig, and F. Duch-Bordas. "Bilateral homonymous visual field defects as initial manifestation of multiple sclerosis." British Journal of Ophthalmology 75, no. 3 (March 1, 1991): 185–87. http://dx.doi.org/10.1136/bjo.75.3.185.
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Moran, Mark B., R. H. Kuo, and C. D. Marrs. "Scatter intensity mapping of laser-illuminated coating defects." Applied Optics 27, no. 5 (March 1, 1988): 957. http://dx.doi.org/10.1364/ao.27.000957.
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Craik, Robert J. M., R. Wilson, and R. Ming. "The Location of Building Defects Using Structural Intensity." Building Acoustics 3, no. 4 (December 1996): 217–31. http://dx.doi.org/10.1177/1351010x9600300401.
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Many defects in the construction of buildings act as transmission paths across which unwanted sound can travel. The resulting increase in power flow can be measured using structural intensity techniques and the location of the source found from the intersection of two or more intensity vectors. This technique can be used to locate a source (or sometimes a sink) when the intensity being measured is above the residual or background intensity. Some applications of this technique are discussed and it is used to locate a point source on a concrete floor and a line source exciting a concrete block wall. The technique was found to work well for a point source but was less reliable for a line source. This was partly because a line source is less well spatially defined and partly because the damping of the wall was low, resulting in a high residual intensity.
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Stojsin, Ivana, and Duska Blagojevic. "Etiopathogenetic consideration and definition of the clinical manifestation of erosive dental defects." Zbornik Matice srpske za prirodne nauke, no. 112 (2007): 83–93. http://dx.doi.org/10.2298/zmspn0712083s.
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Dental defects of erosive nature are defined as irreversible losses of dental tissue, caused by long lasting and repeated action of acids that dissolve top layer of hydroxyapatite and fluorideapatites crystal structure, under assumption that aggressive factor is not of bacterial nature. Acids that cause changes on teeth according to their origin are gastric, dietetic, or they are of environmental origin. Current way of life, as well as nutritional habits create potentially dangerous conditions for the hard dental tissue, for prevention of mineralization process causes defects of oral system homeostasis. Defects occur on primary teeth, as well as on permanent teeth. However, this happens once and a half time more frequently on primary teeth due to the weaker primary maturation. In initial phases, changes are localized in enamel and by their development the bottom locates in dentine. Defects appear as smooth, shiny, round concavities on caries immune positions, or as cupping of occlusal surfaces. The depth of an eroded lesion consists of the depth of the crater plus the depth of tissue demineralization at the base of the lesion. Early verification of the etiological factor, together with good knowledge of the manifested shape change has influence to the prevention of the crown of tooth loss, complete occlusion, mastication and speech.
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Smith, Amanda Elizabeth, Amy Jnah, and Desi Newberry. "Chromosome 16p13.11 Microdeletion Syndrome in a Newborn: A Case Study." Neonatal Network 37, no. 5 (September 2018): 303–9. http://dx.doi.org/10.1891/0730-0832.37.5.303.
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Chromosome 16p13.11 microdeletion syndrome is a rare copy number variant that carries increased risks for complications in the neonatal period and throughout the life span. Clinical manifestations and associated defects known to present in the neonatal period include motor delay, facial dysmorphisms, microcephaly, gastroesophageal reflux disease (GERD), and congenital heart defects. Management in the neonatal period focuses on associated comorbidities, including motor delay with or without GERD, which commonly manifests as feeding difficulties. Life span implications of chromosome 16p13.11 microdeletion syndrome include developmental, speech, and language delay; psychiatric and behavioral problems; seizure disorders; and, less commonly, obesity. Nursing assessment is critical to the early identification of nonspecific abnormalities associated with de novo genetic disorders. Early identification and diagnosis of chromosome 16p13.11 microdeletion syndrome are critical to optimizing outcomes throughout infancy and across the life span. We present a case report of an infant diagnosed with chromosome 16p13.11 microdeletion. A discussion of genetic influences, associated clinical manifestations, diagnostics, management, and health promotion strategies are presented to establish core knowledge of chromosome 16p13.11 microdeletion.
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Andreev, Denis. "Procedural Defects of Administrative Acts." Journal of Russian Law 4, no. 4 (April 11, 2016): 0. http://dx.doi.org/10.12737/18691.
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The article addresses the problem of procedural defectiveness of administrative acts, which has not yet been examined extensively in Russian legal literature. The article provides a definition of procedural defects of administrative acts and offers a brief comparative overview of the issue in legal systems of France, Germany, the UK, Bulgaria and Poland. The paper demonstrates historical development of the theme in the Russian legal doctrine while special attention is paid to the analysis of the current Russian law and application thereof. By a number of examples the author substantiates the thesis that contemporary administrative law of Russia contains sufficient ground for theoretical conceptualization of the notion of substantial procedural defects of administrative acts. The author puts forward a set of criteria for classification of procedural defects into substantial and non-substantial and argues that such differentiation constitutes a particular manifestation of general legal trends.
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Tan, P. H., C. Y. Hu, F. Li, S. Bai, P. X. Hou, and H. M. Cheng. "Intensity and profile manifestation of resonant Raman behavior of carbon nanotubes." Carbon 40, no. 7 (June 2002): 1131–34. http://dx.doi.org/10.1016/s0008-6223(01)00261-5.
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Muzahar, Muzahar, Aminatul Zahra, and Rika Wulandari. "Manifestation And Endoparacitic Intensity On Gonggong Conch (Laevistrombus turturella)." E3S Web of Conferences 324 (2021): 03012. http://dx.doi.org/10.1051/e3sconf/202132403012.
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Habitat the gonggong conch (Laevistrombus turturella) also known as Dog conch is a species from the Gastropod class that can be found in shallow waters with muddy sand substrate. In Kepulauan Riau Province, these animals are hunted by fishermen to sell to consumers since gonggong conch is a favourite culinary in this province. The availability of conch in the market has only relied on natural catches. The high consumption and natural fluctuations have decreased the conch population. To maintain the sustainability of the dog conch, it is necessary to carry out the cultivation activities. The cultivation begins with the selection of quality broodstock. The gonggong conch of natural origin needs to be determined before cultivation, in order to prevent the entry of disease in the cultivation system i.e. parasites. This study includes the determination, prevalence and infection intensity of endoparasites in conch from Madong Bay, Tanjungpinang. The results showed that the total length of the conch shells ranged from 55.5 to 79.5 mm with an average length of 67.72 mm and a frequency mode of 64.6 mm. The sex ratio between male and female conch is 1:1.1 (not ideal) n=30. Two kinds of parasites were found in the digestive tract of gonggong conchs are conoidacid oocysts and trematode sporocysts. The number of endoparasites, prevalence and intensity in wild caught has high numbers involving conoidacid oocysts (prev=100%; I=48.23 ind/conch) and trematode sporocysts (prev=100%; I= 135.33 ind/conch). It was concluded that the level of endoparasites infection in the dog conch from the Madong Bay was categorized as moderate-to-severe.
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Refetoff, Samuel. "Resistance to thyroid hormone is a manifestation of thyroid hormone receptor defects." Current Opinion in Endocrinology and Diabetes 1, no. 1 (January 1994): 155–66. http://dx.doi.org/10.1097/00060793-199400010-00029.
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Komarov, I. V., D. A. Polyakov, and K. I. Nikitin. "Partial discharges characteristics study in XLPE cable insulation with multiple defects." Omsk Scientific Bulletin, no. 180 (2021): 33–40. http://dx.doi.org/10.25206/1813-8225-2021-180-33-40.
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The paper simulates the electric field intensity picture in the cable with one and several defects. Modeling has shown that the presence of several closely spaced defects does not lead to an increase in the electric field intensity in comparison with one defect. On the basis of experimental studies, an analysis of defects' number influence on various parameters of partial discharges (PD) is performed. Artificial defects are created in the area of the XLPE-insulated cable termination. The following parameters of PD are measured: the magnitude of the apparent charge, the power (intensity) of partial discharges, the phase-resolved partial discharge (PRPD) patterns, the shape of PD signals. The analysis shows an increase in the number of defects does not affect the characteristics of partial discharges, despite the fact the defects are located in different sections of the cable with distributed parameters. Thus, it can be difficult to quantify defects during cable partial discharge measurements.
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Vassilikou-Dova, A. B., and K. Eftaxias. "Paramagnetic Defects in Benitoite." Zeitschrift für Naturforschung A 46, no. 7 (July 1, 1991): 579–82. http://dx.doi.org/10.1515/zna-1991-0703.
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Abstract In clear, blue, transparent bipyramidal crystals of the rare mineral benitoite, BaTiSi3O9, para magnetic defects have been investigated by electron paramagnetic resonance at room temperature and 9.43 GHz. They are attributed to Sn3+ and Fe3+ . A pair of satellites recorded for a wide angular rage around B0 || c (~40°) and a relative intensity of ~ 13% to the central signal is most likely due to hyperfine interaction with 117Sn and 119Sn isotopes. Attempts to bleach the colour of the crystal were unsuccessful.
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Dlhý, Pavol, Jan Poduška, Luboš Náhlík, Michael Berer, A. Gosch, Gerald Pinter, and Pavel Hutař. "Compression-Loaded Cracked Cylinder - Stress Intensity Factor Evaluation." Key Engineering Materials 774 (August 2018): 331–36. http://dx.doi.org/10.4028/www.scientific.net/kem.774.331.
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As polymer parts are made by injection molding, most defects are usually caused by the shrinkage of material during solidification. These defects are then source of cracks in these parts. Present contribution is dealing with central cracked compression loaded polymer cylinder. In order to describe crack behavior under operational load, parametrical finite element model was developed. As results of this study stress intensity factors for central crack with different crack lengths are determined to describe crack behavior.
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Horowitz, H. S. "Fluoride and Enamel Defects." Advances in Dental Research 3, no. 2 (September 1989): 143–46. http://dx.doi.org/10.1177/08959374890030021201.
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The concentration of fluoride in drinking water is the major determinant of the prevalence and severity of dental fluorosis in a community. Fluorosis is more prevalent and discernible in permanent teeth than in primary teeth; the intensity can range from barely perceptible, whitish striations in enamel to confluent pitting and dark staining. The traditional belief is that fluorosis is produced only during the secretory stages of ameloblastic activity. Some recent reports suggest that the maturation stages of enamel development are as important as or even more important than the secretory stages as the time when fluorosis can be produced. The question of timing remains unresolved. Many questions also remain about general and individual physiologic variations in relation to susceptibility to dental fluorosis. Good criteria for differential diagnosis exist to distinguish dental fluorosis from non-fluoride enamel opacities. An increasing number of reports indicates that the prevalence of fluorosis may be increasing among children in fluoridated and non-fluoridated communities. Reasons for the increases may relate to misuse of dietary fluoride supplements, ingestion of fluoride toothpastes, or increasing amounts of fluoride in foods or the atmosphere. The intensity of the increased fluorosis is in the milder categories and is not generally unsightly. It should be recognized that a small amount of fluorosis may be an alternative to a greater prevalence of dental caries, a disease that may produce cosmetic problems and sequelae worse than those produced by fluorosis.
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Baybulatov, A. G., A. R. Gilmutdinov, A. F. Kunafin, B. R. Gilmutdinov, and F. F. Usmanov. "Dynamics of clinical and morphological parameters in patients with peptic ulcer in the combined laser therapy." Fizioterapevt (Physiotherapist), no. 5 (September 26, 2020): 43–49. http://dx.doi.org/10.33920/med-14-2010-04.
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The eff ectiveness of the use of a therapeutic complex based on the combined eff ects of intragastric low-intensity and transcutaneous infrared laser therapy in the antiulcer drug therapy of 60 patients with gastric ulcer and duodenal ulcer was studied. In all 30 patients of the main group, after a course of combined laser exposure, the regression of the morphological picture of the activity of the infl ammatory process with epithelialization of the ulcer defect and positive dynamics of the clinical manifestations of the disease within 10 days of treatment was noted. Meanwhile, in the patients of the control group (n = 30), the positive dynamics of similar parameters in the same period was achieved in 70 % of patients against the background of basic drug therapy.
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Koshkareva, N. B., and I. I. Bakaytis. "Prototypical characterization statements and their expressive synonyms." Vestnik Tomskogo gosudarstvennogo universiteta. Kul'turologiya i iskusstvovedenie, no. 43 (2021): 293–307. http://dx.doi.org/10.17223/18137083/74/22.
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The proposition of qualitative characterization is prototypically expressed by statements like “kto / chto (est’) kakov” (somebody / something is of some kind). Expressive synonyms of such sentences are non-prototypical statements based on existential structures of the type “v kom / chem (est’) chto-to kakoe” (there is something of some kind in somebody / something). They are a means of expressing the intensity of the manifestation of the trait, persuasiveness (the author’s uncertainty that he has made an objective idea of the properties and qualities of the described object), comparison, and the actualization of the trait. The transformation method was used to clarify the differences in the semantics of prototypical and nonprototypical sentences of characterization. The impossibility of direct transformation is revealed when the predicate is expressed by an adjective, the semantics of which does not allow the combination with components denoting a weakened or, conversely, high manifestation of the trait. In the construction “v kom / chem (est’) chto-to kakoe,” any variation of the semantics of the circumstance by the category of intensity is possible. The existential structure becomes an expressive means of characterization. At the same time, the narrator is not sure about the nature and causes of the intensity of the manifestation of the trait and does not give a categorical assessment to the subject. A high degree of manifestation of the trait, represented in the statements like “kto / chto (est’) kakov,” is much more frequent. When the adjectival position is filled with a relative or possessive adjective, a non-categorical comparison is expressed.
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Chen, Xueqiong, Xiaoyan Li, Ziyang Chen, Jixiong Pu, Guowen Zhang, and Jianqiang Zhu. "Propagation characteristics of a high-power broadband laser beam passing through a nonlinear optical medium with defects." High Power Laser Science and Engineering 1, no. 3-4 (December 20, 2013): 132–37. http://dx.doi.org/10.1017/hpl.2013.22.
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AbstractThe intensity distributions of a high-power broadband laser beam passing through a nonlinear optical medium with defects and then propagating in free space are investigated based on the general nonlinear Schrödinger equation and the split-step Fourier numerical method. The influences of the bandwidth of the laser beam, the thickness of the medium, and the defects on the light intensity distribution are revealed. We find that the nonlinear optical effect can be suppressed and that the uniformity of the beam can be improved for a high-power broadband laser beam with appropriate wide bandwidth. It is also found that, under the same incident light intensity, a thicker medium will lead to a stronger self-focusing intensity, and that the influence of defects in the optical elements on the intensity is stronger for a narrowband beam than for a broadband beam.
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A. Yasin, Shehla. "PREVALENCE, INTENSITY AND MANIFESTATION OF COVID-19 FEAR: A CROSS SECTIONAL ANALYSIS." Psychiatria Danubina 32, no. 3-4 (December 24, 2020): 499–504. http://dx.doi.org/10.24869/psyd.2020.499.
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Anderson, Kristin, and Michelle Marciniak. "PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY: ADVANCING VISUAL FIELD DEFECTS AS A PRESENTING MANIFESTATION OF HIV." Optometry and Vision Science 78, SUPPLEMENT (December 2001): 189. http://dx.doi.org/10.1097/00006324-200112001-00312.
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Youssefian, L., A. Touati, H. Vahidnezhad, A. H. Saeidian, S. Sotoudeh, S. Zeinali, and J. Uitto. "Erythrokeratoderma: a manifestation associated with multiple types of ichthyoses with different gene defects." British Journal of Dermatology 178, no. 3 (January 22, 2018): e219-e221. http://dx.doi.org/10.1111/bjd.16053.
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Vlasov, Yu A., V. N. Astratov, A. V. Baryshev, A. A. Kaplyanskii, O. Z. Karimov, and M. F. Limonov. "Manifestation of intrinsic defects in optical properties of self-organized opal photonic crystals." Physical Review E 61, no. 5 (May 1, 2000): 5784–93. http://dx.doi.org/10.1103/physreve.61.5784.
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Drabik, Joanna, Karolina Ledwa, and Łukasz Marciniak. "Implementing Defects for Ratiometric Luminescence Thermometry." Nanomaterials 10, no. 7 (July 8, 2020): 1333. http://dx.doi.org/10.3390/nano10071333.
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In luminescence thermometry enabling temperature reading at a distance, an important challenge is to propose new solutions that open measuring and material possibilities. Responding to these needs, in the nanocrystalline phosphors of yttrium oxide Y2O3 and lutetium oxide Lu2O3, temperature-dependent emission of trivalent terbium Tb3+ dopant ions was recorded at the excitation wavelength 266 nm. The signal of intensity decreasing with temperature was monitored in the range corresponding to the 5D4 → 7F6 emission band. On the other hand, defect emission intensity obtained upon 543 nm excitation increases significantly at elevated temperatures. The opposite thermal monotonicity of these two signals in the same spectral range enabled development of the single band ratiometric luminescent thermometer of as high a relative sensitivity as 4.92%/°C and 2%/°C for Y2O3:Tb3+ and Lu2O3:Tb3+ nanocrystals, respectively. This study presents the first report on luminescent thermometry using defect emission in inorganic phosphors.
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Sairyo, Koichi, Toshinori Sakai, Natsuo Yasui, and Akira Dezawa. "Conservative treatment for pediatric lumbar spondylolysis to achieve bone healing using a hard brace: what type and how long?" Journal of Neurosurgery: Spine 16, no. 6 (June 2012): 610–14. http://dx.doi.org/10.3171/2012.2.spine10914.
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Object Various kinds of trunk braces have been used to achieve bone healing in cases of pediatric lumbar spondylolysis. However, the optimal brace for achieving bone healing is unclear. The purpose of the present study was to determine in what types of spondylolysis bone healing can be achieved and how long it takes. Methods In this prospective study, 63 pars interarticularis defects (spondylolysis) among 37 patients who were younger than 18 years (mean 13.5 ± 2.7 years) were treated using a hard brace. The youngest patient was 8 years old. Based on the results of CT scanning, the lyses were classified into 3 categories: early, progressive, and terminal defects. Progressive defects were further divided into 2 types according to STIR MRI findings: those with high signal intensity at the adjacent pedicle and those with low signal intensity (that is, a normal appearance). A hard brace, such as a molded plastic thoracolumbosacral orthosis, was used to immobilize the trunk. Approximately every 3 months, CT scanning was performed to evaluate bone healing until approximately 6 months. Results The union rates were 94%, 64%, 27%, and 0% for the early, progressive with high signal intensity, progressive with low signal intensity, and terminal defects, respectively. It was noted that no terminal defect was healed using conservative treatment. The mean time to healing among the defects that showed bone healing was 3.2, 5.4, and 5.7 months for the early, progressive with high signal intensity, and progressive with low signal intensity groups, respectively. Conclusions Patients with early-stage defects are the best candidates for conservative treatment with a hard brace because more than 90% of such cases can be healed in 3 months.