Modern information technologies in the presentation of the results of the competitive experience at the championship "young professionals"

Information technologies in presenting the results of the competitive assignment in the competence "Teaching Technology" at the "Young Professionals" Championship allow participants to more accurately represent the results of their developments, and experts - to more accurately assess them, guided by a large number of criteria, given the visibility and clarity of the presentation of resultsИнформационные технологии в представлении результатов конкурсного задания по компетенции «Преподавание технологии» на чемпионате «Молодые профессионалы» позволяют участникам более качественно представлять результаты своих разработок, а экспертам - более четко их оценивать, руководствуясь большим количеством критериев, учитывая наглядность и четкость представления результато

Про роль Державного бюро розслідувань в правоохоронній системі України

Ліскова А. К. Про роль Державного бюро розслідувань в правоохоронній системі України / А. К. Ліскова // Державне бюро розслідувань: на шляху розбудови : матер. Міжнар. наук.-практ. конф. (м. Одеса, 16 червня 2018 р.) / редкол.: Г. О. Ульянова (голова ред.), В. М. Дрьомін, Є. Л. Стрельцов [та ін.] ; НУ "ОЮА". - Одеса : Юридична література, 2018. - С. 167-170

ALG3-CDG: a patient with novel variants and review of the genetic and ophthalmic findings

BACKGROUND: ALG3-CDG is a rare autosomal recessive disease. It is characterized by deficiency of alpha-1,3-mannosyltransferase caused by pathogenic variants in the ALG3 gene. Patients manifest with severe neurologic, cardiac, musculoskeletal and ophthalmic phenotype in combination with dysmorphic features, and almost half of them die before or during the neonatal period. CASE PRESENTATION: A 23 months-old girl presented with severe developmental delay, epilepsy, cortical atrophy, cerebellar vermis hypoplasia and ocular impairment. Facial dysmorphism, clubfeet and multiple joint contractures were observed already at birth. Transferrin isoelectric focusing revealed a type 1 pattern. Funduscopy showed hypopigmentation and optic disc pallor. Profound retinal ganglion cell loss and inner retinal layer thinning was documented on spectral-domain optical coherence tomography imaging. The presence of optic nerve hypoplasia was also supported by magnetic resonance imaging. A gene panel based next-generation sequencing and subsequent Sanger sequencing identified compound heterozygosity for two novel variants c.116del p.(Pro39Argfs*40) and c.1060 C > T p.(Arg354Cys) in ALG3. CONCLUSIONS: Our study expands the spectrum of pathogenic variants identified in ALG3. Thirty-three variants in 43 subjects with ALG3-CDG have been reported. Literature review shows that visual impairment in ALG3-CDG is most commonly linked to optic nerve hypoplasia

Information technologies in the design approach to training teachers of professional training in the field of energy

In the design approach to the preparation of teachers of vocational training in any field, including in the energy field, the role of information technology increases dramatically. It becomes almost impossible to prepare a qualified teacher training without the use of Internet resources and information educational environment of high schoolПри проектном подходе к подготовке педагогов профессионального обучения в любой области, в том числе в области энергетики, роль информационных технологий резко возрастает. Становится практически невозможным подготовить квалифицированного педагога профессионального обучения без использования интернет-ресурсов и информационной образовательной среды вуз

Methodology for conducting an integrated linguocultural lesson in teaching Russian as a foreign language

The article discusses the linguocultural aspect of teaching Russian as a foreign language. Attention is drawn to the close interconnection of language and culture. The processes of studying national-specific vocabulary are investigated. The characteristics and classification of non-equivalent vocabulary are given. The methodology developed by the author for conducting an integrated linguoculturological lesson on the study of the national and regional studies component of the non-equivalent vocabulary is described. Tasks for the development of grammar skills on the topic "Ural cuisine" are presented. It is concluded that this technique contributes to the development of foreign students' linguistic, socio-cultural, and communicative competenceРассмотрен лингвокультурологический аспект преподавания русского языка как иностранного. Показана тесная взаимосвязь языка и культуры. Исследованы процессы изучения национально-специфической лексики. Даны характеристика и классификация безэквивалентной лексики. Описана разработанная авторами методика проведения комплексного лингвокультурологического урока по изучению национально-регионоведческого компонента безэквивалентной лексики на примере темы «Уральская кухня». Сделан вывод, что данная методика способствует развитию у иностранных студентов лингвокультурной, социокультурной и коммуникативной компетенци

Novel disease-causing variants and phenotypic features of X-linked megalocornea

Purpose: The aim of the study was to describe the phenotype and molecular genetic causes of X-linked megalocornea (MGC1). We recruited four British, one New Zealand, one Vietnamese and four Czech families. // Methods: All probands and three female carriers underwent ocular examination and Sanger sequencing of the CHRDL1 gene. Two of the probands also had magnetic resonance imaging (MRI) of the brain. // Results: We identified nine pathogenic or likely pathogenic and one variant of uncertain significance in CHRDL1, of which eight are novel. Three probands had ocular findings that have not previously been associated with MGC1, namely pigmentary glaucoma, unilateral posterior corneal vesicles, unilateral keratoconus and unilateral Fuchs heterochromic iridocyclitis. The corneal diameters of the three heterozygous carriers were normal, but two had abnormally thin corneas, and one of these was also diagnosed with unilateral keratoconus. Brain MRI identified arachnoid cysts in both probands, one also had a neuroepithelial cyst, while the second had a midsagittal neurodevelopmental abnormality (cavum septum pellucidum et vergae). // Conclusion: The study expands the spectrum of pathogenic variants and the ocular and brain abnormalities that have been identified in individuals with MGC1. Reduced corneal thickness may represent a mild phenotypic feature in some heterozygous female carriers of CHRDL1 pathogenic variants