Quality of Life in Wilson’s Disease: A Systematic Literature Review

**Background:** Wilson’s disease (WD) is a rare inherited genetic disorder characterized by the progressive accumulation of copper in the brain, liver, and other major organ systems. To date, there have been no comprehensive studies synthesizing evidence pertaining to the quality of life (QOL) in WD. **Objective:** We conducted a systematic literature review to identify and synthesize the evidence on QOL in patients with WD. **Methods:** To address this gap in the literature, we conducted a systematic literature review in MEDLINE and EMBASE to identify observational studies and clinical trials reporting QOL outcomes among people living with WD. **Results:** A total of 442 publications were identified, 41 publications were eligible for full-text screening, and 7 articles, representing 7 studies, met all inclusion criteria. QOL questionnaires used across studies included the 12-Item Short Form Health Survey Questionnaire (version 1) (SF-12) (n=2), the 36-Item Short Form Health Survey Questionnaire (version 1) (SF-36) (n=3), Global Assessment Scale (GAS) (n=1), and World Health Organization QOL brief questionnaire (WHO-QOL-BREF) (n=1). Overall, the pattern in QOL from most studies demonstrated a worse QOL in WD patients compared with the general population, a deterioration in QOL for patients presenting with neurologic symptoms, and more frequent psychiatric symptoms compared with the ones with hepatic symptoms. **Discussion:** Although our understanding of the underlying pathophysiology of WD has advanced, and novel therapeutics are on the horizon, our understanding of how WD affects overall QOL remains limited. Evidence from this review demonstrates the substantial heterogeneity in reporting outcomes pertaining to the QOL associated with WD. These differences may be attributable to the fact that QOL is not typically assessed and the lack of a standardized method for assessing QOL in WD. **Conclusion:** This review demonstrates a need for more up-to-date studies with larger sample sizes to further evaluate QOL in patients with WD. The study also demonstrates the need for a WD-specific instrument to measure the QOL in WD patients.

Textbook Evaluation: A Case Study of Punjab, Pakistan

The textbook is one of the most substantial elements of classroom learning. As a result, it is critical to conduct a textbook evaluation to improve the quality of the teaching and learning environment in the classroom. Keeping in mind the very importance of the textbook evaluation, the current study evaluated a 10th-grade English textbook published by Punjab Curriculum and Textbook Board Lahore, Pakistan. The present study aimed to evaluate the book’s overall pedagogical worth according to teachers’ and students’ needs and demands. For this purpose, 10 teachers and 60 students were selected from different public and private schools. Two different questionnaires were prepared by Litz (2001) with slight variations. The teachers’ questionnaire version had 20 items, while the students’ version had 15 items. The statistical analysis was carried out using SPSS after the data had been collected. The study’s findings revealed that the textbook as a whole is well-designed and offers an array of useful extra materials. The book is also visually pleasing, well-organized, consistent, and orderly. Overall, the positive characteristics outnumbered the negative ones by a wide margin. Despite a few flaws in the book, such as the lack of a glossary and grammar, the results showed that the textbook was adequate for language learners. Overall, it was considered that, despite some flaws, the textbook fulfilled the needs of students and might be a practical book in the hands of competent teachers.Keywords: textbook; evaluation; English language

Exploring the use of the Psychological Characteristics of Developing Excellence (PCDEs) in younger age groups: First steps in the validation process of the PCDE Questionnaire for Children (PCDEQ-C)

Athletes who want to benefit most optimally and efficient from the Talent Development (TD) pathway, should start developing their psychological characteristics at a young age. The Psychological Characteristics Developing Excellence Questionnaire–Version 2 (PCDEQ2) can provide a full assessment of the mental characteristics athletes need. However, the PCDEQ2 has only been validated in adolescent athletes and as a consequence its does not contribute to the understanding of these psychological skills in a younger age group. The main purpose of this study was to take a first step in examining the factor structure and reliability of the Psychological Characteristics of Developing Excellence Questionnaire for Children (PCDEQ-C), a questionnaire based on the PCDEQ– 2. Firstly, the original questionnaire was translated to Dutch, age-adapted and redesigned for implementation in young athletes. Secondly, 774 participants (400 girls) from Flanders, Belgium, between 7 and 13 years (mean age of 10.61 ± 1.58) old filled out the questionnaire. After exploratory factor analysis, a new factor structure for the PCDEQ-C deemed an acceptable fit with 51 items in 5 factors. In the third stage, the reliability showed a good overall and internal consistency, with adequate relations between factors. The first steps in the validation process of the PCDEQ-C, suggest that this questionnaire could be a useful and reliable tool to assess the developmental psychological characteristics of 7-to-13-year-old athletes. The questionnaire is one of the first formative assessment tools to monitor and develop the psychological characteristics needed during the earliest talent development stages of a young athlete.

Effect of preferred learning styles on academic achievements: A cross sectional descriptive study.

Objective: This study aims to find out the preferred mode of study for undergraduate Physiology students and effect of learning style preferences on academic achievements. Study Design: Cross Sectional, Descriptive study. Setting: Fatima Jinnah Medical University, Lahore. Period: December 2018 to June 19. Material & Methods: Total 170 undergraduate’s 2nd year MBBS Physiology students were included. The VARK questionnaire (Version 7.1) designed by Fleming was administered to 170 students after taking consent. This questionnaire is a valid tool to assess the learning style preferences of students. It consists of 16 questions having four options each and a scoring chart at the end, for the students to calculate their own scores. The aggregate percentage of periodic assessments throughout the academic year was defined as academic achievement. It was matched with their learning style preferences. A relationship between the learning style preferences and academic achievement was studied. Data was analyzed by using SPSS version 23.0. Results: Out of a total of 170 students, 73 (43%) preferred kinesthetic while 51(30%) preferred aural mode of learning. However, no association between learning style preferences and academic achievements could be found. Conclusion: The study concluded that the most preferred learning style among the M.B.B.S 2nd year Physiology students was kinesthetic. No significant association was found between the learning style preferences and academic achievement. Teaching modalities can be improved and incorporated according to students’ preferences.

Pembangunan Aplikasi Pengelolaan Data pada Rumah Sakit Berbasis Web

Hospital has a variety of facilities and services. Every day there are transactions recorded by the hospital in the form of documentation such as excel. Over time, if the transactions increase, the files that will be stored will be more and more large. Therefore, a website was created that can make it easier to manage data from these files. The web application is created using PHP and integrates with the MySQL database. The main module of the web application created is to import data from an excel file. The expected results from making this website are to make it easier for employees to manage data and view information in the form of tables or graphs. Data collection methods using observation, through interviews with the person in charge from hospital by giving a questionnaire about the interface of the application. Assessment of the results of interface improvements using a questionnaire from Post-Study System Usability Questionnaire version three, totalling 16 questions. The purpose of the User Interface and User Experience analysis is to assess and serve as a guide for improving the display that has been made whether it is in accordance with the standards requested by the hospital.

The Associations between Self-Determined Motivation, Multidimensional Self-Efficacy, and Device-Measured Physical Activity

Self-determined motivation (SDT) and self-efficacy theory have been widely used for understanding individuals’ physical activity motivation and self-efficacy. However, there is a gap of evidence on the relations between SDT and multidimensional self-efficacy with device-measured physical activity in healthy adults. Questionnaires including the behavior regulation in exercise questionnaire version 2 (BREQ-2) and the multidimensional self-efficacy for exercise scale (MSES) were completed by the participants at baseline. All participants wore an accelerometer for seven days to record their physical activities at baseline and eight-week follow up. In total, thirty healthy adults completed the study (12 men, 18 women). The results showed that intrinsic motivation and scheduling self-efficacy had significantly positive associations with moderate-to-vigorous physical activity energy expenditure and duration. Multiple regression analysis showed that the relative autonomy index, task and scheduling efficacy could predict physical activity at baseline, but no SDT or self-efficacy variable could predict physical activity behavior after eight weeks. These results showed that the associations between motivation and self-efficacy with physical activity might change within a short period of time, which suggests that the regular assessment of motivation and self-efficacy might be needed in interventional programs to promote continued physical activity participation in healthy adults.

Biological Risk Factors and Early Developmental Delay Assessment in Infants Using Ages and Stages Questionnaire, Version 3 (ASQ-3)

Background: Development during early years of life is very crucial in the context of overall development of an individual. This is especially true for infants who have biological risk factors for maldevelopment. This study aimed to find the association of biological risk factors and developmental delay in infants aged 3-18 months. Materials and Methods: For this study, enrolment of 460 infants was done in the age group of 3-18 months who were attending Immunization Clinic in district level Civil Hospital, Faridkot, Punjab. Developmental assessment in the five areas including Gross motor, Fine motor, Communication, Problem solving and Personal-social development was done using a standardized tool called Ages and Stages Questionnaire, version 3 (ASQ-3). Results: Most of the study subjects viz., 426 (92.6%) were born full term whereas 34 (7.3%) infants were born preterm. Mean birth weight of infants was 2.9 ± 0.387 kgs. Out of the total, 259 (56.3%) study subjects were males and 201(43.7%) were females. Biological risk factors like gestation age and birth weight had statistically significant association with all domains of developmental delay except in the area of personal social development. On the other hand, gender had no association with any domain of developmental delay. Conclusion: Gestation age and birth weight are the typical risk factors having significant impact on all domains of developmental delay except for the personal social delay. However, gender of infant had no association with developmental delay. Keywords: Infant, Gestation age, birth weight, Developmental delay, ASQ.

Oxford ankle foot questionnaire: Localization in Russia

BACKGROUND: According to literature data, the Oxford Foot Questionnaire for children is a valid instrument for the assessment of complaints and therefore requires adaptation in Russian. AIM: Russian localization of the Oxford Ankle Foot Questionnaire. MATERIALS AND METHODS: Localization of the questionnaire was gradually carried out in accordance with international standards. To specify the accuracy of anatomical comprehension of the lower extremity, 35 children aged 516 years old and their parents were interviewed preliminary. For final validation of the Russian version, the pilot testing was performed in 20 children aged 516 years and their parents. RESULTS: As all the interviewed children correctly anatomically specified the leg, 91.4% of them correctly pointed out the foot, and only 20.0% of children, and 57.0% of the parents were able to find the ankle joint, we translated the phrase ankle and foot as стопа. This was represented in the title and text of the questionnaire items. The final questionnaire version survey illustrated that, generally, children and their parents answered all questions without any difficulties, and additions and clarifications were not essential. CONCLUSIONS: This Russian version of the Oxford Ankle Foot Questionnaire is the only instrument used for the assessment of different foot complaints in children aged 516 years and parents opinion on how much the existing pathology affects the physical, social, and emotional components of childrens complaints.

Physical, emotional and social pain communication by patients diagnosed and living with multiple myeloma

Aim: To describe physical, social and emotional aspects of pain self-reported by patients with multiple myeloma (MM), and patient–physician communication of physical pain. Materials & methods: We analyzed self-reported data from 330 adults receiving anti-MM therapy in Germany and Italy on health-related quality of life (European Organisation for Research and Treatment of Cancer Quality of Life Core-30 Questionnaire version 3, -MY20) and bone pain symptoms. Results: Patients experienced clinically important physical (69%), emotional (58%) and social (22%) pain. Less than three-quarters of physicians’ records matched patients’ perception of bone pain (71.5%), with bone pain not recorded in 19.7% of patients experiencing it. Nearly half of physicians underestimated bone pain severity. Conclusion: Patients with MM experience physical, social and emotional pain. Discordance regarding bone pain symptoms and severity was observed, suggesting the need for improved communication.