What is known about deferasirox chelation therapy in pediatric HSCT recipients: two case reports of metabolic acidosis

To date, in pediatric field, various hematological malignancies are increasingly treated with allogeneic hematopoietic stem cell transplantation (allo-HSCT). Iron overload and systemic siderosis often occur in this particular cohort of patients and are associated with poor prognosis. We describe herein the case of two allo-HSCT patients, on treatment with deferasirox; they showed histopathological elements compatible with venoocclusive disease or vanishing bile duct syndrome in ductopenic evolution before deferasirox started. The first patient developed drug-induced liver damage with metabolic acidosis and the second one a liver impairment with Fanconi syndrome. After withdrawing deferasirox treatment, both patients showed improvement. Measurements of drug plasma concentrations were performed by HPLC assay. The reduction and consequent disappearance of symptoms after the suspension of deferasirox substantiate its role in inducing hepatic damage, probably enabling the diagnosis of drug-induced liver damage. But the difficulties in diagnosing drug-related toxicity must be underlined, especially in compromised subjects. For these reasons, in patients requiring iron-chelating therapy, close and careful drug therapeutic monitoring is strongly recommende

Genetic variability and structure of SNP haplotypes in the DMPK gene in Yakuts and other ethnic groups of northern Eurasia in relation to myotonic dystrophy

The genetic variability of the DMPK locus has been studied in relation to six SNP markers (rs2070736, rs572634, rs1799894, rs527221, rs915915, and rs10415988) in Yakuts with myotonic dystrophy (MD) in the Yakut population and in populations of northern Eurasia. Significant differences were observed in the allele frequencies between patients and a population sample of Yakuts for three SNP loci (rs915915, rs1799894, and rs0415988) associated with a high chance of disease manifestation. The odds ratios (OR) of MD development in representatives of the Yakut population for these three loci were 2.59 (95% CI, p = 0,004), 4.99 (95% CI, p = 0.000), and 3.15 (95% CI, p = 0.01), respectively. Haplotype TTTCTC, which is associated with MD, and haplotype GTCCTT, which was observed only in Yakut MD patients (never in MD patients of non-Yakut origin), were revealed. A low level of variability in the locus of DMRK gene in Yakuts (He = 0.283) compared with other examined populations was noted. An analysis of pairwise genetic relationships between populations revealed their significant differentiation for all the examined loci. In addition, a low level of differentiation in territorial groups of Yakut populations (FST = 0.79%), which was related to the high subdivision of the northern Eurasian population (FST = 11.83%), was observed

Relationship between oral health status and cognitive functions

With global increases in the aging population, the number of patients with cognitive decline is greatly increasing, which has become a big social problem. The studies conducted previously suggest the possible relationship between oral health and cognitive status. It is well-known, that the oral cavity is an important component not only the maxillofacial region, but also acontributor of general health. In this article, we highlight the relationship among dentoalveolar system and the cognitive functions. Studies using functional magnetic resonance imaging revealed that during mastication there is activation of somatosensory cortical areas, the supplementary motor area, insula, and other areas including the striatum, thalamus and cerebellum. According to the results of the сlinical studies, patients with dementia have demonstrated poor oral hygiene, greater incidence of periodontal diseases and tooth loss. Accumulating evidence suggests that cognitive decline may be associated with masticatory dysfunction. Masticatory dysfunction, as an umbrella term, refers to a debilitating condition in which normal masticatory function is compromised due to structural factors (e.g., tooth loss) or functional factors (e.g., weaker biting force or poorer masticatory performance). The presented data support the hypothesis that there is a possible relationship between mastication, oral health status and cognitive functions, therefore maintenance and adequate restoration of the whole masticatory system are important for the prevention of cognitive decline. This problem requires further clinical interdisciplinary studies, assessing cognitive abilities and oral health

Термоэлектрические явления и устройства в концепции Ландауэра–Датты–Лундстрома

С позиций концепции «снизу–вверх» транспортной модели Ландауэра–Датты–Лундстрома современной наноэлектроники рассматриваются термоэлектрические явления Зеебека и Пельтье и качественно обсуждаются закон Видемана–Франца, числа Лоренца и основные уравнения термоэлектричества с четырьмя транспортными коэффициентами (удельное сопротивление, коэффициенты Зеебека и Пельтье, электронная теплопроводность). С тех же позиций на примере 3D-резистора в диффузионном режиме анализируется работа термоэлектрических охладителя и генератора энергии с учётом лишь электронов, как реальных носителей тока, так и в рамках умозрительной, но удобной «дырочной» модели, вводятся и определяются понятия эффективности работы (КПД, фактора мощности и добротности) термоэлектрических устройств и рассматривается, каким образом транспортные коэффициенты зависят от свойств термиков.З позицій концепції «знизу–вгору» транспортної моделі Ландауера–Датти–Лундстрома сучасної наноелектроніки розглядаються термоелектричні явища Зеєбека і Пельтьє і якісно обговорюються закон Відеманна–Франца, Лоренцові числа й основні рівняння термоелектрики з чотирма транспортними коефіцієнтами (питомий опір, коефіцієнти Зеєбека і Пельтьє, електронна теплопровідність). З тих же позицій на прикладі 3D-резистора в дифузійному режимі аналізується робота термоелектричних охолоджувача і ґенератора енергії з урахуванням лише електронів, як реальних носіїв струму, так і в межах умоглядної, але зручної «діркової» моделі, вводяться і визначаються поняття ефективности роботи (ККД, фактора потужности і добротности) термоелектричних пристроїв і розглядається, яким чином транспортні коефіцієнти залежать від властивостей терміків.Based on the ‘bottom-to-top’ approach of the Landauer–Datta–Lundstrom transport model of state-of-the-art nanoelectronics, the thermoelectric Seebeck and Peltier phenomena are considered; the Wiedemann–Franz law and Lorenz numbers as well as the four transport coefficients (specific resistivity, Seebeck and Peltier coefficients, and electronic thermal conductivity) are qualitatively discussed. From the same positions for 3D-resistor within the diffusion regime, thermoelectric cooler and energy power generator are analysed with accounting only electrons as real current carriers as well as within the scope of the artificial but useful ‘hole’ model. Conceptions of operating performances (coefficient of efficiency, power factor, and figure of merit) for thermoelectric devices are introduced and defined, and dependences of transport coefficients on the properties of termics are discussed

Heterogeneity of population of microorganisms grown in presence of iron oxide maghemite nanoparticles

In this work γ-Fe2O3 MNPs were obtained by laser target evaporation and water based suspensions were prepared. Maximum permissive dose of iron in water (MPD) is 0.3 mg/L. It was found that 100 MPD dose of iron induces formation of non-typical colonies after 72 or 96 hours exposition: against a background of small black colonies large white colonies appeared due to a disruption in tyrosine synthesis. Multiple re-cloning of the white colonies grown with MNPs showed that they retained their properties both under standard conditions (temperature of 24 °C) and at the temperatures up to 37o C. E.nigrum grown with MNPs demonstrated very scant extension of small colonies at the cultivation temperature of 24o C, their growth was completely blocked at 37°C. Significant changes in the structure of the population were noted. First of all, large cells with pronounced aggregation were observed among the black colonies. These aggregates consisted of large cells connected to each other by matrix. In the white colonies the appearance of very long threadlike cells connecting different groups of the cells establishing an intercellular communication was evident. Fe2O3 MNPs induce an increase in the heterogeneity of the population, expressed as a change in morpho-physiological states. © 2018 The Authors, published by EDP Sciences.The work was supported by the Ministry of Education and Science of the Russian Federation (project No. 3.6121.210 )7 and R FFI №16-34-015 92 grant s. e tW hank R ndrA ade, Iu. Novoselova and I.V. Beketov for special support. Selected measurements were made at ISG KER services UPV/EHU

Substantiation of conditions for biomodification of starfish for production of fodder additives

Biotechnological stage in technology of fodder products of starfish is substantiated. The starfishes Evasterias echinosoma and Patiria pectinifera caught in the Severnaya Bay (Primorye coast of the Japan Sea) are used in the experiments on enzymic hydrolysis for production of the fodder additive with high biological value. The hydrolysis provides the more accessible forms of nutrients for animals and birds. Impacts of the enzyme preparation concentration, temperature, pH, time of hydrolysis, and water duty on efficiency of enzymic hydrolysis are tested in the experiments; these parameters influence on the amount of accumulated amino nitrogen and on the hydrolysis degree is evaluated. The following rational values are determined for the parameters in the process of protein-mineral fodder additive production: concentration of enzyme collagenase preparation - 0.25-1.00 %; time of treatment - 2-4 hours; temperature - 45-55oC; pH - 6.0; water duty - 0.2

Towards the understanding of the cocoa transcriptome: Production and analysis of an exhaustive dataset of ESTs of Theobroma cacao L. generated from various tissues and under various conditions

Theobroma cacao L., is a tree originated from the tropical rainforest of South America. It is one of the major cash crops for many tropical countries. T. cacao is mainly produced on smallholdings, providing resources for 14 million farmers. Disease resistance and T. cacao quality improvement are two important challenges for all actors of cocoa and chocolate production. T. cacao is seriously affected by pests and fungal diseases, responsible for more than 40% yield losses and quality improvement, nutritional and organoleptic, is also important for consumers. An international collaboration was formed to develop an EST genomic resource database for cacao. Fifty-six cDNA libraries were constructed from different organs, different genotypes and different environmental conditions. A total of 149,650 valid EST sequences were generated corresponding to 48,594 unigenes, 12,692 contigs and 35,902 singletons. A total of 29,849 unigenes shared significant homology with public sequences from other species. Gene Ontology (GO) annotation was applied to distribute the ESTs among the main GO categories. A specific information system (ESTtik) was constructed to process, store and manage this EST collection allowing the user to query a database. To check the representativeness of our EST collection, we looked for the genes known to be involved in two different metabolic pathways extensively studied in other plant species and important for T. cacao qualities: the flavonoid and the terpene pathways. Most of the enzymes described in other crops for these two metabolic pathways were found in our EST collection. A large collection of new genetic markers was provided by this ESTs collection. This EST collection displays a good representation of the T. cacao transcriptome, suitable for analysis of biochemical pathways based on oligonucleotide microarrays derived from these ESTs. It will provide numerous genetic markers that will allow the construction of a high density gene map of T. cacao. This EST collection represents a unique and important molecular resource for T. cacao study and improvement, facilitating the discovery of candidate genes for important T. cacao trait variation. (Résumé d'auteur

Autologous hematopoietic stem cell transplantation for Behçet’s disease: a retrospective survey of patients treated in Europe, on behalf of the autoimmune diseases working party of the European Society for Blood and Marrow Transplantation

Background: Behçet’s Disease (BD) is an autoimmune disease mostly presenting with recurrent oral and genital aphthosis, and uveitis. Patients are rarely refractory to immunosuppressive treatments. Autologous hematopoietic stem cell transplantation (aHSCT) is a standard of care in other autoimmune diseases. Some patients with BD have been treated with aHSCT based on compassionate use. Objectives: Evaluate the outcome of aHSCT in adult patients with BD treated in member centers of the European Society for Blood and Marrow Transplantation (EBMT). Methods: Adults who received aHSCT primarily for BD were identified retrospectively in the EBMT registry and/or in published literature. Data were extracted from either medical records of the patient or from publications. Results: Eight out of 9 cases reported to the registry and extracted data of 2 further patients from literature were analyzed. Four were female, median age at onset of BD was 24y (range 9-50). Median age at aHSCT was 32y (27-51). Patients had received median 4 (2-11) previous lines of therapy (89% corticosteroids, 50% methotrexate, anti-TNFα therapy or cyclophosphamide). All patients had active disease before mobilization. Conditioning regimen was heterogeneous. Median follow-up was 48 months (range 6-240). No treatment-related mortality was reported. This procedure induced complete remission (CR) in 80%, partial remission in 10% and lack of response in 10% of the patients. Relapse rate was 30% (2 relapses in patients in CR and 1 relapse in the patient in PR) with panuveitis (n=1), aphthosis (n=2) and arthralgia (n=1). Six patients were in CR. No late complications were reported. Conclusion: aHSCT has an acceptable safety profile and represents a feasible and relatively effective procedure in severe and conventional treatment-resistant cases of BD and has the potential to stabilize BD in patients with life-threatening involvements