MODEL OF TEACHING MALAY LANGUAGE TO NON-NATIVE AND FOREIGN SPEAKER

Purpose – This study aims to develop a teaching model of the Malay language for non-native and foreign speakers in schools. Implementing this model can assist educators in teaching the Malay language to students who do not use it as their first language in everyday use. Methodology – This study used a qualitative approach to develop teaching models for non-native and foreign Malay speakers. Interviews were conducted with 27 teachers and 23 stakeholders who are experts in teaching the Malay language, especially in teaching non-native speakers. A prototype model was developed thematically as a result of interviews using ATLAS.ti 22. Further, the prototype model was used by ten teachers from primary, secondary, and international schools in their teaching. Findings – This study has succeeded in developing a model for teaching the Malay language to non-native speakers and foreigners that includes five elements: teaching strategies, individual differences, input, social, and motivation. This model strongly emphasises instruction involving interaction, communication, and bilateral relations as a medium for students’ language acquisition and meaningful input. Verbal interaction between teachers and students can stimulate and improve students’ language skills in learning Malay. Significance – This model can help teachers teach the Malay language to non-native speakers and foreigners with a greater focus on achieving learning objectives. This model is expected to expand the usage, cultivate interest, and facilitate teaching the Malay language in educational institutions

Loss-of-Function Mutations in SERPINB8 Linked to Exfoliative Ichthyosis with Impaired Mechanical Stability of Intercellular Adhesions

M.P. is supported by a Fellowship from the Deutsche Forschungsgemeinschaft. This work was supported in part by a generous donation of Israel and Ruthi Ram (E.S.) and from a British Heart Foundation Programme grant (D.P.K.)

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Application of Dudley-Evans & St. John Model in Malay Language: The Needs Analysis Phase

Researchers need to look at the current needs to ensure the relevance and validity of a study. The present study applies a model proposed by Dudley-Evans and St. John (1998) to investigate the current needs in the field of Malay language education; aspects of reading skills, particularly, reading comprehension. In addition, the researchers also intend to examine the suitability of this model in working with information in the current situation so that the research to be conducted is rich in information. This study uses a qualitative approach that involves semi-structured interviews and document analysis. Semi-structured interviews involved seven field experts and 321 documents of non-native Malay speakers. The results of the thematic analysis using ATLAS.ti 22 software found that this model is very suitable for working on the current needs to be used as a guideline by any researcher in obtaining information related to the research. Application of the Dudley-Evans and St. John (1998) can assist researchers in planning and developing models or modules, products, instruments, and so on to suit current issues or needs in the world of education

Content Validity of The Reading Skills Teaching Module Through a Foreign Language Teaching Approach

Reading skills are basic skills needed by students as early as primary school. However, there are still students who are able to read but do not understand the reading material read. Therefore, a teaching module through foreign language teaching approach was develop to help teachers to implement teaching and learning especially in the aspect of reading comprehension. This study aims to look at the aspects of content validity of the modules that had been develop through content validity index (CVI) and frequency and percentage involving three experts. The results of the study showed that the overall value of the content validity index of I-CVI and S-CVI was high with value of 1.0 and the overall percentage of module content was obtained as 94%. The findings of the study also found the whole construct; module usability (96.7%), module content (90.8%), teaching assignments and activities (93.2%), module suitability (95.6%) and teaching evaluation (93.8%) obtained values above 90% while I-CVI and S-CVI with a value of 1.0 for each construct. Overall, these findings indicate that the content of the modules meet the standards for the content and quality aspects of a module. This module is suitable for students and teacher and is easy to use in teaching and learning reading skills, especially aspects of reading comprehension

Cutaneous and Developmental Effects of CARD14 Overexpression in Zebrafish

Background: Gain-of-function mutations in CARD14 have recently been shown to be involved in the pathogenesis of psoriasis and pityriasis rubra pilaris (PRP). Those mutations were found to activate the NF-kB signaling pathway. Objective: Zebrafish is often used to model human diseases in general, and in skin disorders more particularly. In the present study, we aimed to examine the effect of CARD14 overexpression in zebrafish with the aim to validate this model for future translational applications. Methods: We used light microscopy, scanning electron microscopy, histological analysis and whole mount in situ hybridization as well as real-time PCR to ascertain the effect of CARD14 overexpression in the developing zebrafish. Results: Overexpression of human CARD14 had a marked morphological and developmental effect on the embryos. Light microscopy demonstrated a characteristic cutaneous pattern including a granular surface and a spiky pigment pattern. In situ hybridization revealed keratinocytes of uneven size and shape. Scanning electron microscopy showed aberrant production of actin microridges and a rugged keratinocyte cell surface, reminiscent of the human hyperkeratotic phenotype. Developmentally, overexpression of CARD14 had a variable effect on anterior-posterior axis symmetry. Similar to what has been observed in humans with psoriasis or PRP, NF-kB expression was higher in CARD14-overexpressing embryos compared to controls. Conclusions: Overexpression of CARD14 results in a distinct cutaneous pattern accompanied by hyperactivation of the NF-kB pathway, suggesting that the zebrafish represents a useful system to model CARD14-associated papulosquamous diseases

Dominant frontonasal dysplasia with ectodermal defects results from increased activity of ALX4

Abstract Frontonasal dysplasia (FND) refers to a group of rare developmental disorders characterized by abnormal morphology of the craniofacial region. We studied a family manifesting with clinical features typical for FND2 including neurobehavioral abnormalities, hypotrichosis, hypodontia, and facial dysmorphism. Whole‐exome sequencing analysis identified a novel heterozygous frameshift insertion in ALX4 (c.985_986insGTGC, p.Pro329Argfs*115), encoding aristaless homeobox 4. This and a previously reported dominant FND2‐causing variant are predicted to result in the formation of a similar abnormally elongated protein tail domain. Using a reporter assay, we showed that the elongated ALX4 displays increased activity. ALX4 negatively regulates the Wnt/β‐catenin pathway and accordingly, patient keratinocytes showed altered expression of genes associated with the WNT/β‐catenin pathway, which in turn may underlie ectodermal manifestations in FND2. In conclusion, dominant FND2 with ectodermal dysplasia results from frameshift variants in ALX4 exerting a gain‐of‐function effect

Loss-of-function variants in C3ORF52 result in localized autosomal recessive hypotrichosis

Purpose Localized autosomal recessive hypotrichosis (LAH) has been associated with pathogenic variants in DSG4, encoding a desmosomal protein as well as in LIPH and LPAR6, encoding respectively lipase H, which catalyzes the formation of 2-acyl-lysophosphatidic acid (LPA), and lysophosphatidic acid receptor 6, a receptor for LPA. LPA promotes hair growth and differentiation. In this study we aimed at delineating the genetic basis of LAH in patients without pathogenic variants in these three genes. Methods Variant analysis was conducted using exome and direct sequencing. We then performed quantitative reverse transcription polymerase chain reaction (RT-qPCR), immunofluorescence staining, immunoblotting, enzymatic, and coimmunoprecipitation assays to evaluate the consequences of potential etiologic variants. Results We identified homozygous variants in C3ORF52 in four individuals with LAH. C3ORF52 was found to be coexpressed with lipase H in the inner root sheath of the hair follicle and the two proteins were found to directly interact. The LAH-causing variants were associated with decreased C3ORF52 expression and resulted in markedly reduced lipase H-mediated LPA biosynthesis. Conclusion LAH can be caused by abnormal function of at least three proteins which are necessary for proper LPA biosynthesis