Case Report: Novel SLC9A6 Splicing Variant in a Chinese Boy With Christianson Syndrome With Electrical Status Epilepticus During Sleep

We investigated the existence and potential pathogenicity of a SLC9A6 splicing variant in a Chinese boy with Christianson Syndrome (CS), which was reported for the first time in China. Trio whole-exome sequencing (WES) was performed in the proband and his parents. Multiple computer prediction tools were used to evaluate the pathogenicity of the variant, and reverse transcription-polymerase chain reaction (RT-PCR) analysis and cDNA sequencing were performed to verify the RNA splicing results. The patient presented with characteristic features of CS: global developmental delay, seizures, absent speech, truncal ataxia, microcephaly, ophthalmoplegia, smiling face and hyperkinesis with electrical status epilepticus during sleep (ESES) detected in an electroencephalogram (EEG). A SLC9A6 splicing variant was identified by WES and complete skipping of exon 10 was confirmed by RT-PCR. This resulted in altered gene function and was predicted to be pathogenic. ESES observed early in the disease course is considered to be a significant feature of CS with the SLC9A6 variant. Combined genetic analysis at both the DNA and RNA levels is necessary to confirm the pathogenicity of this variant and its role in the clinical diagnosis of CS.

The formation of a Kinh traditional village in Huế in early modern Vietnam

This article traces the transformation of Huế from an open migrant society to a closed community from the seventeenth through nineteenth centuries through an examination of the village documents of Thanh Phước in Thừa Thiên Huế province. In Thanh Phước, the expansion of cultivated land reached its limits around the end of the seventeenth century. Subsequently, continuous population pressure resulted in the emergence of social groups with closed and fixed membership called làng and dòng họ after the eighteenth century. A significant feature of this social development was that the patrilineal kinship favoured by Confucianism was used to protect the vested interests of the earliest inhabitants of the village and their descendants. This indicates that the penetration of Confucianism among the common people and the development and stagnation of agriculture in early modern Vietnam were mutual, complementary phenomena.

The Review on the Role of Ambiguity of Tolerance and Resilience on Students’ Engagement

Due to the arrival of positive psychology (PP) in the development of teaching, the construct of engagement has been thrived and got a notable function in the educational arena. Alternatively, numerous individual differences, containing ambiguity of tolerance, have been taken into consideration as a result of the key role they can play in the process of learning, and thus, on different facets of the learners’ engagement. Furthermore, resilience is recommended to be an alternate and effective way of engaging English as a foreign language (EFL) learners. Also, it is a significant feature of the human adaptation system in which students can efficaciously manage and tackle stressful involvements despite their troubles and disasters. Given the eminence of both ambiguity tolerance and resilience in educational settings and the fact that little attention has been given to these constructs in foreign language learning, the present review makes an effort to scrutinize the impact of ambiguity of tolerance and resilience on EFL learners’ engagement. Succinctly, the fundamental roles of ambiguity tolerance and resilience in learners’ engagement were confirmed, and consistent with the conclusions drawn from the present review, some suggestions are set forth concerning the implications of this paper.

Research on gearbox impact feature extraction method based on improved ESMD

A gearbox vibration signal contains non-linear impact characteristics and the significant feature information tends to be overwhelmed by other interference components, which make it difficult to extract the typical fault features fully and effectively. Aiming at the key issue of how to effectively extract the impact characteristics, a fault diagnosis method based on improved extreme symmetric mode decomposition (ESMD) and a support vector machine (SVM) is proposed in this paper. The vibration signal is adaptively decomposed into multiple intrinsic mode function (IMF) components by the improved ESMD and then a certain number of components are selected with the maximum kurtosis-envelope spectrum index. The singular spectral entropy, energy entropy and permutation entropy of each component are applied to construct the feature vector set, in which the dimensionality of the set is reduced with the distance separability criterion. Finally, the dimension-reduced feature vector set is input into the SVM for pattern recognition. Dynamic simulation and experimental gearbox research show that the improved ESMD method can extract and identify gearbox fault information effectively.

Factors Affecting the Effectiveness of Flood Resilience Plan for Urban Sustainability of Ratnapura Municipal Council Area

Natural disasters adversely affect urban built-up areas all over the world. Flood is the most destructive natural disaster that affects Ratnapura, the provincial capital of the Sabaragamuwa Province in Sri Lanka. This monsoonal flooding is a prolonged problem that arises mainly due to the overspill of River Kalu and other water bodies connected to the river during heavy rainy seasons. It causes short-term and long-term damages to human lives, properties, the economy, and the environment. Floods cause severe damages to private residential buildings. Hence, the availability of a flood resilience plan is a significant feature of the good governance of the Local Authorities. There are many factors to consider when preparing a flood resilience plan. This study aims to assess the factors affecting the effectiveness of the existing flood resilience plan in the Ratnapura Municipal Council Area. The main data instrument used is a questionnaire survey from residents in the flood-prone area. It assessed the effectiveness of actions taken by the government pre-disaster, during a disaster, and post-disaster. Various factors affecting the flood resilience plan were identified and categorized: community resilience, economic resilience, ecological resilience, emergency readiness and responsiveness, infrastructure resilience, and social and cultural resilience. Findings revealed that the respondents were more satisfied with emergency readiness and responsiveness out of the five main categories. In addition, respondents address the effectiveness of the existing flood resilience plan and its affected factors like community resilience, economic resilience, emergency readiness and responsiveness, infrastructure resilience and social, and cultural resilience. Findings will be helpful for town planners and the Municipal Council of Ratnapura to identify flood resilience strategies through community perspectives to mitigate the flood hazard and propose innovative strategies to achieve urban sustainability and build resilient communities.

Significant Applications of IoT in Covid Pandemic: A Systematic Review

Today the entire world is in the exigency state-COVID-19 quarantine days. We should stay home to live. To avoid a fight over the spread of corona, the governments of all the countries have enforced a nationwide lockdown. Though the lockdown may have helped to control the spread of COVID, it has had a devastating impact on numerous domains like health, agriculture, education, global supply chains, trade, and various industries like automotive, power – electronics, travel, aeronautical, tourism industry…etc, which are the basic roots of the growth of a nation. This censorious situation can be wielded with the eminent technology “IoT”. Anytime, Anything, Anywhere”- this is the most significant feature of IoT. Any real-world object can be transformed into an intelligent object by the technology “IoT”. Because of the affordability and availability of smart devices, the entire world is more connected with IoT than ever before. From this standpoint, the authors have chosen five real-time areas health, education, industry, agriculture, and society. This survey initiates from the impacts of COVID in the above-chosen areas, how it diminishes the day-to-day events of human life, the vitality of IoT, how it helps to tackle the COVID issues without any quality degradation in this quarantine period. This systematic review completely appraises the innovations and contributions of IoT used by various researchers to defend the impacts of COVID and concludes with the pros and cons. A detailed exploration has been done in this article particularly on “IoT in COVID pandemic”. This will be more useful to the researchers to acquire clear-cut knowledge about the power of IoT, in particular how IoT plays a significant role in the period of COVID and further assists them to travel towards an innovative and serviceable direction in their research.

Spatial-Temporal Responses of Ecosystem Services to Land Use Transformation Driven by Rapid Urbanization: A Case Study of Hubei Province, China

Exploring the changes of ecosystem services value caused by land use transformation driven by urbanization is crucial for ensuring the safety of the regional ecological environment and for enhancing the value of ecosystem services. Based on the land use remote sensing data during the rapid urbanization development period of Hubei Province from 1995 to 2015, this study analyzed the characteristics of land use/land cover change and land use transformation. The spatial–temporal response characteristics and evolution of ecosystem services value (ESV) to land use transformation driven by urbanization were measured by equivalent factor method, spatial autocorrelation analysis, hot spot analysis and gravity model. We found that: (1) Driven by urbanization, the most significant feature of land use transformation in Hubei Province was the expansion of the built-up land and the significant reduction of cropland and forest, among which 90% of the new built-up land was converted from cropland and forest. (2) This land use transformation became the main source of ESV losses. Especially, the sharp increase of the built-up land from 2010 to 2015, occupying cropland and forest, resulted in ESV losses of nearly USD 320 million. The service capacity of climate regulation, soil conservation, gas regulation and food production undertaken by cropland and forest decreased. (3) The ecosystem services value in the study area showed spatial distribution characteristics of high in the west and low in the middle and east regions. The center of gravity of ESV shifted from northwest to southeast. Due to the sharp increase of the built-up land from 2010 to 2015, the center of gravity shift rebounded. This study can help policymakers better understand the trade−offs between land use transformation and ecosystem services driven by urbanization.

Prediction of Alzheimer’s Disease Based on Coordinate-Dense Attention Network

Alzheimer’s disease (AD) is a degenerative disease of the nervous system. Mild cognitive impairment (MCI) is a condition between brain aging and dementia. The prediction will be divided into stable sMCI and progressive pMCI as a binary task. Structural magnetic resonance imaging (sMRI) can describe structural changes in the brain and provide a diagnostic method for the detection and early prevention of Alzheimer’s disease. In this paper, an automatic disease prediction scheme based on MRI was designed. A dense convolutional network was used as the basic model. By adding a channel attention mechanism to the model, significant feature information in MRI images was extracted, and the unimportant features were ignored or suppressed. The proposed framework is compared with the most advanced methods, and better results are obtained.

By Canoe and Speedboat: The Recent Expansion of the Ìlàjẹ Fishing Trade

Competition has remained a significant feature of trade, particularly in regions with diverse social groups like the Niger Delta region of Nigeria. This article examines the expansion of trade among the Ìlàjẹ communities of Ondo State in the coastal area of Yorùbáland, which to the best of our knowledge has not been documented. Historical method of data collection and analysis were employed in the study. These include primary and secondary sources. The primary sources are mainly comprised of interviews of key informants and participants’ observations. The secondary sources consist of journal articles and texts. The results were analyzed qualitatively. The growth in coastal trade in the Ìlàjẹ area from 2010 onwards was largely due to the construction of new roads along the coast, which linked Ìlàjẹ to the wider road network. Between 2009 and 2013, new trading routes emerged along the road from Igbokoda to Araromi via Atijere on the western side to Ògun and Lagos state, passing through towns like Ipárè, Oko-Ńlá, Obinehin, Etíkàn, Erékè, and Àbòtó. Another route linked Igbokoda with Òde-Ùgbò and Ùgbò-Ńlá coastal market in the east. As traders and goods came into Ìlàjẹ from various parts of Nigeria, new markets sprang up to facilitate the exchange of upland goods for fish products. The construction of new roads also encouraged social and economic development by bringing into the area goods and commodities hitherto not transportable on water, such as ‘modern’ building materials. However, far from reducing water-based activity, the overall increase in trade has also contributed to an increase of water-based traffic, especially for fishing.

“A Part of Speech” by Joseph Brodsky: Bilingualism as a Creative Method in Poetry

This article is an attempt of textological conceptualization of an interlingual transfer (doubling) of a literary text. This transfer, in case it has been done by the author him/herself, is traditionally called self-translation, yet it can also by qualified as an adaptation of the “old” text to the perceptive abilities of the “new” recipient, i.e. a collective reader representing a different national and cultural medium and bearing its values as a prevalent aesthetic waymark. Special importance in the above process is attached to such a significant feature of any literary text of high quality as its elasticity. The textological approach to the scientific understanding of the differentlanguage text binary is often complemented by the bilinguological categorization of the subject, i.e. a text of poetry and, at the same time, a poetic cycle. From this standpoint, the mode of primary/secondary character of the two interconnected texts might lose its substantial preciseness. Further on, the article scrutinizes a landmark (for the Russian culture) literary text and its self-translation: the poetic cycle “A Part of Speech” by Joseph Brodsky. Four conceptual dominants of the cycle, with regard to their axiological hierarchy, are distinguished: 1) space/distance – location – oblivion; 2) time (epoch) – history – event – oblivion; 3) love – breakup – oblivion; 4) speech – art – extension – immortality. Further, a comparative analysis of their different-language verbalization is carried out. It is stated that Brodsky’s creation of this bilingual cycle reflects his striving for a cultural adaptation of each version to the background knowledge, historical experience, principles, traditions, aesthetic attitudes and, generally, sentiments of different groups of readers. In some cases this is done by means of the national language only, in other cases by the general complication of the poems’ figurative fabric.