106 results on '"Rus R"'
Search Results
2. Data on the Timing of Spawning and Embryonic Development of the Alaska Plaice Pleuronectes quadrituberculatus (Pleuronectidae) of the Northern Part of the Sea of Okhotsk
- Author
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Yusupov, R. R. and Yusupov, Rus. R.
- Published
- 2023
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3. On the problem of identification for eggs of alaska place Pleuronectes quadrituberculatus (Pleuronectidae) from ichthyoplankton collections in the northern Okhotsk Sea
- Author
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R. R. Yusupov and Rus. R. Yusupov
- Subjects
alaska place ,pleuronectes quadrituberculatus ,fish egg ,pigmentation ,northern okhotsk sea ,Aquaculture. Fisheries. Angling ,SH1-691 - Abstract
Species composition in catches of the ichthyoplankton survey conducted on June 4, 2021 in the periphery of the Tauiskaya Guba Bay (northern Okhotsk Sea) is considered. Eggs of 3 fish species were collected, the portion of alaska place was 1.3 %. Specific pigmentation of the alaska place embryos was defined that allowed to identify reliably the eggs of this species, in combination with other signs.
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- 2022
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4. Visualization skills and learning style of household economics students who take engineering drawing courses.
- Author
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Hooi, C. K., Hanapi, Z., Wafi, A. A., Mohamed, S., and Rus, R. C.
- Abstract
Visualization skill is an important skill that must be practiced by the students so that they can master the subject of Engineering Drawing. Students who face difficulties in solving Engineering Drawing problems due to weakness in visualization skills. Effective learning styles can influence the acquisition of information and knowledge as well as mastery of an individual's skills in the process of acquiring knowledge. Failure to understand engineering drawings well may be influences by inaccurate learning styles. The purpose of this study is to identify the visualization skills and learning style of students as well as the relationship between visualization skills and learning style practiced by Engineering Drawing students. The population of this study consists of household economics student who take engineering drawing course. This study is designing in quantitative form. The instrument that has been used in this study is questionnaire. The level visualization skills of student was tested using the Purdue Spatial Visualization Test of Rotation (PSVT:R) and Minnesota Paper Form Board Test (MPFBT). Both of these test can examine the level of visualization skills in two aspects which are mental rotation skills and mental merging skills. The Learning Style Questionnaire by Neil Flemming was used to determine learning style of students. T-test has been used to differentiate the level of visualization skills between the genders of students. Spearman correlation analysis was used to determine the relationship between visualization skills and the learning style of students. The findings show that most of the students have a good level of visualization skills and do not have a significant difference between visualization skills and genders. Mean 7.91 has shown the most respondents are practicing visual learning style. The result also show that no significant relationship between the level of visualization skills and learning style. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
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5. Implementation of the 21st century learning approach among technical and vocational education trainee teachers
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Zulkifli, R.M., primary, Hussain, M.A.Mohd, additional, Wahid, N.H.Abd, additional, Suhairom, N., additional, and Rus, R. Che, additional
- Published
- 2019
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6. Эмбриональное и раннее личиночное развитие бурого терпуга Hexagrammos octogrammus (Scorpeniformes: Hexagrammidae) северной части Охотского моря
- Author
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Rus. R. Yusupov and R. R. Yusupov
- Subjects
General Materials Science - Abstract
The roe of Hexagrammos octogrammus from of the Northern part of the Sea of Okhotsk was incubated in an experiment. A detailed description of the morphology of the species development, from fertilization of eggs to the fry transition to external nutrition, accompanied by microphotographs of eggs and larvae fry in vivo, is given. It was established that the fertilized eggs of H. octogrammus from the northern part of the Sea of Okhotsk are 1.75 mm in diameter with fluctuations of 1.61-1.98 mm. Hatching of embryos occurs after 33.5 days of incubation at an average temperature of 11.5°C. Hatched yolk fry is 7.78 mm (7.15-8.06 mm) of TL (Total length) and 7.39 mm (6.83-7.68 mm) of SL (Standard length). Upon reaching TL of 8.74 mm (8.14-9.26 mm) and SL of 8.21 mm (7.63-8.86 mm), the fry switch to external feeding. The regional features of the masked greenling pigmentation in early ontogenesis are revealed.
- Published
- 2021
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7. On the problem of identification for eggs of alaska place Pleuronectes quadrituberculatus (Pleuronectidae) from ichthyoplankton collections in the northern Okhotsk Sea
- Author
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Yusupov, R. R., primary and Yusupov, Rus. R., additional
- Published
- 2022
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8. Hybrid de novo Whole-Genome Assembly and Annotation of SARS-CoV-2 Virus from Nosocomial Infection in Pahang, Malaysia
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Zainulabid, U.A., primary, Kamarudin, N., additional, Rus, R. Mohd, additional, Soffian, S.N., additional, Yassim, A.S. Mat, additional, How, S.H., additional, and Ahmad, H.F., additional
- Published
- 2022
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9. Visualization skills and learning style of household economics students who take engineering drawing courses
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Hooi, C. K., Hanapi, Z., Wafi, A. A., Mohamed, S., and Rus, R. C.
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- 2023
- Full Text
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10. ANALYSIS OF EXPERT VALIDATION ON DEVELOPING FACTORS INFLUENCING CAREER CHOICE MALAYSIAN VOCATIONAL COLLEGE TEACHERS’ MODEL
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Che Rus R, M I Mahmud, M R Abdul Mutalib, H. Mohd Affandi, and I Irawan
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Medical education ,General Arts and Humanities ,Vocational education ,05 social sciences ,050301 education ,General Social Sciences ,050109 social psychology ,0501 psychology and cognitive sciences ,Psychology ,0503 education ,Career choice - Abstract
Purpose of study: The teaching profession is often seen as a non-rewarding career due to high burden duties that the teachers must endure. Consequently, many teachers lose interest in teaching, which resulted in a lack of job satisfaction. However, this profession has been chosen due to intrinsic, extrinsic and altruistic motivation. In view of this, there are some factors that are not explored in depth in a teaching career at Vocational Colleges.The purpose of this study is to explore Malaysian Vocational College teachers’ career choice. The goals of this research are to discover factors that lead Malaysian Vocational College teachers to pursue teaching and to identify the sub-factors in preparing them to be vocational teachers. Methodology: The development of the constructs Malaysian Vocational College teachers’ career choice is based on 114 literature review articles. This goal is accomplished through 5 panels of experts consensus through the online questionnaires. Online questionnaires are chosen to conduct the survey because of its good review and user-friendliness. All levels of the agreement will be elevated by using the Fliess Kappa (K ) because more than 2 respondents. Fleiss Kappa is then used to evaluate the level of agreement between the raters. Result: There are six major key factors found: family influence, socio-cultural influence, individual, situational and spiritual. There are 130 items developed and the overall finding K value is 0.55. Nevertheless, the K value within six major key factors is 0.4 to 0.86. The expert panels agree that this questionnaire might be acceptable with some improvement based on their additional comments in the online form. Based on this validation process, this questionnaire can be used in selecting the quality of Malaysian Vocational College teachers or TVET instructors. For the next step, this questionnaire will be conducted on 52 excellent teachers at Malaysian Vocational Colleges to understand in depth the key factors that significantly influence the decision of an individual to enter the vocational teaching profession in Malaysian Vocational Colleges. Implications/Applications: In the future, the findings on this research will expose rich sources on the field of teaching and will be helpful to identify some high-quality TVET teachers in Malaysian Vocational College.
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- 2019
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11. Validity and Reliability of Malay Language Breast Cancer Awareness Scale for Male-to-Female Transgender.
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Draman S., Md Zin H., Shalihin S. E., Mohd Rus R., and Ahmad Alwi A. A.
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TRANS women ,MALAY language ,BREAST cancer ,EXPLORATORY factor analysis ,CRONBACH'S alpha ,COMPUTER adaptive testing - Abstract
INTRODUCTION: Risks of breast cancer occurring among female transgender using hormonal therapy is emerging. However, most transgenders are also engaged in breast implant procedures resembling female sexual characteristics and satisfaction without realizing the risk of cancer development. Thus, breast cancer risk assessment of female transgenders in Malaysia should be evaluated further. This study aims to develop and validate an assessment tool in the Malay language version to measure breast cancer awareness among female transgenders in Malaysia. MATERIALS AND METHODS: A crosssectional study with a purposive sampling method was conducted among 300 respondents in districts in the states of Pahang, Kedah, and Kelantan. The minimum number of respondents required was 300 persons based on the Nunnally method, with an item ratio of 1:10. All the subjects were given questionnaires on sociodemographic data and breast cancer-related items, consisting of five domains; knowledge of symptoms and clinical features, breast cancer risk factors, breast screening programmes, barriers in seeking medical help, and lastly, the confidence, skills and behaviour concerning breast selfexamination. Each domain consisted of five to seven items. All the responses were recorded as 'Yes,' 'No,' or 'Not Sure'. Reliability was determined using Cronbach's alpha for internal consistency, while construct validity was assessed using exploratory factor analysis. RESULTS: The reliability of the 28 items for Malay Version of Breast Cancer Awareness scale by Cronbach Alpha was 0.864, suggesting that the constructs were reliable and acceptable. In addition, the construct validity was assessed using exploratory factor analysis highlights five meaningful domain. DISCUSSION AND CONCLUSION: This Malay language breast cancer awareness scale is a convenient self-assessment method, inexpensive and understandable which highlights common issues faced by male-to-female transgender with targeted domains. This scale is valid and reliable by achievable internal consistency value of 0.864. [ABSTRACT FROM AUTHOR]
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- 2023
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12. Open-Source Software Tools for Measuring Resources Consumption and DASH Metrics
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M. Montagud, J. Antonio De Rus, R. Fayos-Jordán, M. Garcia-Pineda J. Segura-Garcia
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Open-source software tools for measuring resources consumption and DASH metrics ,GeneralLiterature_MISCELLANEOUS - Abstract
When designing and deploying multimedia systems, it is essentialto accurately know about the necessary requirements and the Quality of Service (QoS) offered to the customers. This paperpresents two open-source software tools that contribute to these key needs. The first tool is able to measure and register resourcesconsumption metrics for any Windows program (i.e. process id), like the CPU, GPU and RAM usage. Unlike the Task Manager,which requires manual visual inspection for just a subset of these metrics, the developed tool runs on top of the Powershell toperiodically measure these metrics, calculate statistics, andregister them in log files. The second tool is able to measure QoSmetrics from DASH streaming sessions by running on top ofTShark, if a non-secure HTTP connection is used. For eachDASH chunk, the tool registers: the round-trip time from requestto download, the number of TCP segments and bytes, the effectivebandwidth, the selectedDASH representation, and the associatedparameters in the MPD (e.g., resolution, bitrate). It also registersthe MPD and the total amount of downloaded frames and bytes.The advantage of this second tool is that these metrics can beregistered regardless of the player used, even from a deviceconnected to the same network than the DASH player.
- Published
- 2020
13. Ichthyofauna of the Islands of the Taui Bay coastal waters and recreational fishing
- Author
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Yusupov, R R, primary, Radchenko, O A, additional, Moreva, I N, additional, and Yusupov, Rus R, additional
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- 2021
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14. Activating Mutations of the Calcium-Sensing Receptor: The Calcilytic NPS-2143 Mitigates Excessive Signaling of Novel Mutants Causing Autosomal Dominant Hypocalcemia.
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Letz, S, primary, Haag, C, additional, Rus, R, additional, Schulze, E, additional, Frank-Raue, K, additional, Dorr, HG, additional, Schnabel, D, additional, Mayr, B, additional, Raue, F, additional, and Schofl, C, additional
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- 2010
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15. Arteriovenous Fistula in Pediatric Patients with End-Stage Renal Disease: The Role of Preoperative Ultrasonography Mapping: P113 (EI0215)
- Author
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Buturovic-Ponikvar, J., Persic, V., Rus, R., Novljan, G., and Ponikvar, R.
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- 2011
16. Outcome of Core Out Fistulectomy and the Ligation of Anal Fistula Tract: A Single Center Experience in Malaysia
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C.Z, M Salehudin, primary, M. Nor, Azmi, additional, and Mohd Rus, R, additional
- Published
- 2020
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17. Development of mobile application upon mechanical engineering students’ learning styles
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Kannapiran, S, primary, Kob, C G C, additional, Rus, R C, additional, Shah, A, additional, and Dewi, N R, additional
- Published
- 2020
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18. Acute pyelonephritis with renal abscesses and acute renal failure after salmonella infection
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Rus, R R and Kersnik Levart, T
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- 2010
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19. Ichthyofauna of the Islands of the Taui Bay coastal waters and recreational fishing
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Rus R Yusupov, R. R. Yusupov, I N Moreva, and O A Radchenko
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Fishery ,Geography ,Boreal ,biology ,Benthic zone ,Range (biology) ,Littoral zone ,Blue rockfish ,biology.organism_classification ,Bay ,Diversity of fish ,Demersal zone - Abstract
The material for research was a collected fish in the littoral and sublittoral zones of the Islands of Nedorazumeniya and Zavyalov coasts (Northern part of the sea of Okhotsk) in June 2018 and August 2019.The authors’ research identified 22 and 25 species of fish that live in the summer in the littoral and sublittoral zones of the sea coast of Zavyalov and Nedorazumeniya Islands, respectively. Of these the most numerous are the Blue rockfish Sebastes glaucus (24.5-49.0%), and White spotted greenling Hexagrammos stelleri (13.5-35.7%). Most species are demersal and benthic ichthyocoen. 41% of the species are characterized by the broad-boreal near-Asian type of range, 28% - mainly boreal, 13% - Arctic-boreal, 9% - high-boreal near-Asian and broad-boreal Pacific. For the development of recreational fishing, 15 species of fish can be promising, for which size and weight indicators are given.
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- 2021
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20. Predictors of anemia without active bleeding signs in patients referred for endoscopy
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Munteanu Sabrina Nicoleta, Cozac-Szőke Andreea Raluca, Mocan Simona, Zait Tania Mihaela, Rus Răzvan Iacob, Petri Răzvan Emil, and Negovan Anca
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anemia ,chronic gastritis ,dyspepsia ,helicobacter pylori ,premalignant gastric lesions ,Medicine - Abstract
Objective: The objective of the present study is to clarify the value of clinic, endoscopic, and histologic variables that may predict anemia in patients performing esophagogastroduodenoscopy for gastrointestinal complaints in the absence of bleeding signs or lower digestive disease.
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- 2023
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21. ANALYSIS OF EXPERT VALIDATION ON DEVELOPING FACTORS INFLUENCING CAREER CHOICE MALAYSIAN VOCATIONAL COLLEGE TEACHERS’ MODEL
- Author
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Mutalib M. R., Abdul, primary, M. I., Mahmud, additional, Affandi, H., Mohd, additional, I., Irawan, additional, and Rus R, Che, additional
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- 2019
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22. The Importance of Generic Skills for Technical and Vocational Students Employability
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Haron, M. A., primary, Mohammad Hussain, M. A., additional, Ali, E., additional, Che Rus, R., additional, and Mohammad Zulkifli, R., additional
- Published
- 2019
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23. Systematic Review on the Relevancy of Paracetamol and Breastfeeding Post Infants Vaccination
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Mohd Rus R, Shamsuddin Sh, Drahman S, and Suleiman N
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Vaccination ,medicine.medical_specialty ,Immunization ,business.industry ,Family medicine ,other ,Breastfeeding ,Medicine ,business - Abstract
Background: Paracetamol may be use as antipyretic agent for the treatment of fever, as well as an analgesic in the treatment of mild to moderate pain on post vaccination in infants. The use of Paracetamol during fever may be or may not be recommended since it may alter natural human body immune response although it may reduce pain. Objectives: This study described the relevancy of Paracetamol use post infants vaccination based on data collection systematic review analyses. This study aims to describe the effectiveness of breastfeeding in reducing pain and Paracetamol in reducing fever and pain post infants vaccination. Data Sources and Study Selection: Electronic literature search by hand searching six (6) databases which include Ovid LWW Total Access Collection and Medline, CINAHL (Cumulative Index to Nursing and Alled Health Literature) Plus with Fulltext, Science Direct, Proquest Dissertations and Theses, Proquest Education Journal and Proquest Health and Medical Complete. Additionally, manual reference checks of all articles on Paracetamol and breastfeeding post infants vaccination published in English Language between 1978 and 2017. Two level of screening were used on 9614 citations which include screening of abstracts and titles followed by full text screening. Data Synthesis: Data synthesis were tabulated into study characteristics, quality and effects. Authors of trials were not contacted for further details or provision of original data if the published report contained insufficient information. The study findings, as reported by the authors, were included in this review. The data in this research cannot be pool due to not enough data regarding odd ratio or relative risk as well as confidence interval in each study. Results: Systematic review of breastfeeding included three (3) studies from 9614 of database searching. The reviews of all these three (3) studies found significant benefit from breastfed in pain score and duration of crying as well as behavioral changes. None study stated the unbeneficial of breastfeeding before, during and after immunization. Meanwhile, systematic review of Paracetamol effectiveness included four (4) studies from 1177 of database searching. The reviews of two (2) studies found significant benefit from prophylaxis Paracetamol in fever and only one (1) study found significant benefit from prophylaxis Paracetamol in fussiness. On the other hand, there was one (1) study found not signifiant benefit from prophylaxis Paracetamol in fever. Other than that, there were two (2) studies evaluate the safety of prophylactic Paracetamol which revealed different outcomes, in which study by Prymula et. al. in 2009 found that antibody responses to several antigens were reduced significantly, and the other study by Uhari et. al. in 1988 found that antibody titres to DTP bacteria of placebo and PCM not differ significantly. Thus, Paracetamol seems to be not relevant post infants vaccination and breastfeeding was found to be beneficial post infants vaccination. Conclusions: The relevancy of giving Paracetamol post all types of vaccination may be questionable since the safety issue of this intervention may be arised. Breastfeeding before, during and after immunization are recommended for pain reduction as it was proved effectively. Finally, in deciding Paracetamol to be of rational use following infants immunization, it may need for further research which include in depth quantitative and qualitative studies to identify specific problem and causes regarding this issue.
- Published
- 2018
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24. Neonatal Clavicle Fracture: A Review of Fourteen Cases in East Coast Peninsular Malaysia
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Awang Ms, Mohd Rus R, Che Ahmad A, and Abdul Razak Ah
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medicine.medical_specialty ,Obstetrics ,business.industry ,Vaginal delivery ,Birth weight ,Incidence (epidemiology) ,Gestational age ,General Medicine ,medicine.disease ,Shoulder dystocia ,medicine.anatomical_structure ,Clavicle ,medicine ,Prospective cohort study ,business ,Brachial plexus - Abstract
Introduction: The purpose of this study is to identify the incidence of clavicle fractures in newborn associated with fetal, maternal and process of deliveries in Kuantan General Hospital from June 2012 until January 2014. This study is to determine epidemiological data of clavicle fractures, maternal and baby risk factors associated with clavicle fractures of newborn and its’ outcome. Methods: This is a prospective study. 13 patients were identified to fulfill the inclusion criteria of the study. The data of sociodemographic, associated fetal and maternal risk factors and the outcomes were recorded using proforma. The statistical data analysis was done using SPSS 12.0. Results: Out of 20,257 live births at our centre during the study period, 13 infants were diagnosed to have clavicle fractures, giving an incidence of 0.64 per 1000 live births. There were 5 (38.5%) left, 7 (53.8%) right and one (7.7%) bilateral fracture. All fractures located at the mid shaft of the clavicle and none have associated brachial plexus injuries. All infants were delivered through vaginal delivery (61.5%); five through assisted delivery (instrumental); 2 (15.4%) forcep and 3 (23.1%) vacuum. Two of the babies developed shoulder dystocia. The average birth weight was 3371 grams (SD 0.269) and mean gestational age was 38.7 weeks (SD 1.16). Five of the mothers (38.5%) were primigravida and eight (61.5%) were multigravida in which,7 (53.8%)were healthy without other co-morbidty, 5 (38.5%) having gestational diabetis and one (7.7%) hypertension. The average maternal weight was 62.0 kg and height 1.58 metres with average BMI of 24.16 (3.29SD). All eventually had a complete recovery at 6 weeks with clinical and radiological evident of fracture union. Conclusions: In conclusion, all patients with clavicle fractures were found following vaginal delivery. There were no associations between neonatal clavicle fractures with maternal or baby risk factors. All fractures healed without any complications. KEYWORDS: Brachial plexus, shoulder dystocia, clavicle fractures
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- 2017
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25. [Testing of the gas vortex culture stirrer under conditions of short-term microgravity]
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V K, Ul'in, D V, Korshunov, A I, Borodulin, R R, Kaspranskiĭ, A M, Khomenchuk, M V, Gernet, Rus R, Kaspranskiĭ, and S Iu, Firsov
- Subjects
Microbiological Phenomena ,Bioreactors ,Aircraft ,Research Design ,Cell Culture Techniques ,Gases ,Space Flight ,Weightlessness Simulation - Abstract
Functional testing of the bioreactor for stirring culture in microgravity was performed during the microgravity episodes aboard research airplane Il-76 following the Kepler parabolic flight path. The attempt to produce the gas vortex stirring effect in a given liquid volume was a failure. Nonetheless, the stirrer ventilator is capable to agitate liquid acting as a mechanical stirrer.
- Published
- 2011
26. A Pilot Study on Spectators’ Motivations and Their Socio-Economic Perceptions of a Film Festival
- Author
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Yolal, M., Rus, R., Cosma, S., Gursoy, Dogan, Yolal, M., Rus, R., Cosma, S., and Gursoy, Dogan
- Abstract
The main objective of this pilot study is to examine spectators’ motives for attending a film festival, their perceptions of socio-economic impacts of the festival to the host community, and their satisfaction with the festival utilizing data collected from the attendees of the Transylvania International Film Festival, which takes place on a regular basis in Cluj-Napoca, Romania. The results indicate that there are significant variations in motivations, satisfaction, and perceptions of impacts across gender, age, level of education, and occupation of attendees. Theoretical contributions, managerial implications, and limitations are addressed.
- Published
- 2015
27. Identification and functional characterization of a homozygous, inactivating CaSR mutation in a patient with rickets
- Author
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Szczawinska, D, primary, Mayr, B, additional, Letz, S, additional, Rus, R, additional, Schnabel, D, additional, and Schöfl, C, additional
- Published
- 2013
- Full Text
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28. Functional analysis of six novel mutations of the calcium-sensing receptor in familial hypocalciuric hypercalcemia and autosomal dominant hypocalcemia
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Rus, R, primary, Bumke-Vogt, C, additional, Schnell, S, additional, Andreas, J, additional, Bähr, V, additional, Haag, C, additional, Schulze, E, additional, Frank-Raue, K, additional, Raue, F, additional, and Schöfl, C, additional
- Published
- 2007
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29. 9 The Effect of Handgrip Training and Intermittent Compression of Upper Arm Veins on Forearm Vessels in Patients With End-Stage Renal Failure
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Rus, R, primary, Ponikvar, R, additional, Kenda, R B, additional, and Buturovic-Ponikvar, J, additional
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- 2005
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30. 19 Effect of Intermittent Compression of Upper Arm Veins on Forearm Vessels in Patients with End‐Stage Renal Disease
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Rus, R R, primary, Ponikvar, R, additional, Kenda, R B, additional, Urbančič, A, additional, and Buturović‐Ponikvar, J, additional
- Published
- 2005
- Full Text
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31. CHRONIC SUBDURAL HEMATOMAS AND HYDROCEPHALUS IN A DOG
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Nykamp, Stephanie, primary, Scrivani, Peter, additional, Delahunta, Alexander, additional, Yu-Speight, Audrey, additional, and Rus, R., additional
- Published
- 2001
- Full Text
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32. RELIEFUL ANTROPIC DIN REGIUNEA MINIERĂ ABRUD-ROŞIA MONTANĂ.
- Author
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MUNTEANU, O. L., RUS, R., and SURDEANU, V.
- Published
- 1998
33. Some considerations regarding motivations and overall perceptions on film festivals
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Cosma, S., Bota, M., Fleşeriu, C., Negruşa, A., Valentin Toader, and Rus, R. V.
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perception on festivals ,lcsh:HB71-74 ,differences ,M31 ,events’ industry ,lcsh:Economics as a science ,lcsh:Business ,motivation ,film festival ,ddc:330 ,events' industry ,L83 ,sociodemographic characteristics ,lcsh:HF5001-6182 - Abstract
At the global level the events industry is developing and it is directly connected with sustainable development of communities in each country. Festivals are perceived nowadays as an important part of cultural and leisure attractiveness of destinations. Festivals as major events have the power to mobilize large numbers of people and create a significant impact on their lives by creating a positive experience for spectators and by changing humans’ behaviour on long term in a positive way. The main purpose of the research is to study participants’ motivations for attending a film festival, their perceptions and overall impression from different segments point of view. An exploratory and descriptive research was conducted. Data were collected from the attendees of the Transilvania International Film Festival, which takes place on a regular basis in Cluj-Napoca, Romania using a faceto- face interview based on a short questionnaire. The obtained results reveal significant variations in motivations and overall impression across main demographic features of the participants. The study brings some theoretical contributions, but also managerial implications.
34. Knowledge, attitude and practice of sawmill workers towards noise-induced hearing loss in Kota Bharu, Kelantan
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Rus, R. M., Aziah Daud, Musa, K. I., and Naing, L.
35. Phosphorus Loading from Fish Farming Activities to Wadaslintang Reservoir Waters
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Sih Piranti Agatha, RUS Rahayu Diana, and Waluyo Gentur
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Environmental sciences ,GE1-350 - Abstract
Wadaslintang Reservoir located in Wonosobo Regency of Central Java provides to fish farming area using floating net cage. There are 2 feeding system applied that are semi intensively system managed by local community and super intensively managed by PT. Aquafarm. Both different feeding systems resulted in the amount of pollutant load coming into the reservoirs in the form of total P derived from the remaining feed and feces of fish entering into the reservoir. This can trigger the eutrophication condition of the reservoir waters. The purposes of this study were 1) to calculate the capacity of Wadaslintang reservoir to phosphorus load based on its morphometry and hydrology, 2) to evaluate the capacity of wadaslintang reservoir to Phosphorus load from floating net cage activities in wadaslintang reservoir, and 3) to design the program on the reducing load of Total Phosphorus from floating net activities in wadaslintang reservoir. The research was conducted by survey method in the Wadaslintang Reservoir during January 2016 in both locations of floating net cage fish farming. Parameters measured were P content in fish and P content of feed used in both cultivation systems, and P concentration of reservoir water. The amount of pollutant load coming into the reservoir and its impact on the P concentration of water was calculated using the formula. The results showed that Wadaslintang Reservoir to phosphorus load capacity was 106,56 ton P/year. The Phosphorus load from floating net cage was 216,2 ton P/year. The P load from PT. Aquafarm have exceeded the capacity as much as 70,74 ton P/year. In general the existence of fish farming system using floating net cage in Wadaslintang Reservoir has contributed to increase the concentration of P equal to 43,9% or in other words as much as 50% of P load that goes into reservoir of wadaslintang. Programs designed to reduce the phosphorus load could be done by reducing the number of floating net and decreasing all the activities in the catchment area up to 50% to fulfill phosphorus standard of 100 mg/m3.
- Published
- 2018
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36. Technocracy Carries On.
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Rus, R. W.
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LETTERS to the editor ,TECHNOCRACY - Abstract
A letter to the editor is presented in response to the article about technocracy in the February 8, 1933 issue.
- Published
- 1933
37. Surgical Management of Distal Tibia Fracture: Towards An Outcome-based Treatment Algorithm
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Rushdi I, Che-Ahmad A, Abdul-Ghani KAH, and Mohd-Rus R
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distal tibia fracture ,tibia pilon fracture ,tibial plafond fracture ,ilizarov external fixator ,internal fixation ,Orthopedic surgery ,RD701-811 - Abstract
INTRODUCTION: Distal tibia fractures are frequently associated with an extensive soft tissue injury which then leads to a higher risk of complications such as infection, non-union and eventually poor overall outcome. The purpose of this study is to measure the outcome of distal tibia fractures treated with internal fixation, external fixator or Ilizarov external fixator(IEF). We aim to propose an algorithm for management of distal tibia fractures by evaluating the treatment options, outcomes and risk factors present. MATERIALS AND METHODS: This study is a cross-sectional study of all distal tibia fractures treated surgically in Tengku Ampuan Rahimah Hospital, Klang from 1st January 2016 till 30th June 2018. Patient records were reviewed to analyse the outcomes of surgical treatment and risk factors associated with it. RESULTS: Ninety-one patients were included with a mean age of 41.5 years (SD = 16.4). Thirty-nine cases (42.9%) were open fractures. Thirty-eight patients (41.8%) were treated with internal fixation, 27 patients (29.7%) were treated with IEF and 26 patients (28.6%) were treated with an external fixator. Among open fractures cases, no significant finding can be concluded when comparing each surgical option and its outcome, although one option was seen better than the other in a particular outcome. Initial skeletal traction or temporary spanning external fixator in close fractures reduced the risk of mal-alignment (p value=0.001). Internal fixation is seen superior to IEF and external fixator in close fractures in term of articular surface reduction (p value =0.043) and risk of mal-alignment (p value = 0.007). CONCLUSION: There is no single method of fixation that is ideal for all pilon fractures and suitable for all patients. This proposed algorithm can help surgeons in deciding treatment strategies in the challenging management of distal tibia fractures to reduce associated complications.
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- 2020
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38. Community-Supported Shared Infrastructure in Support of Speech Accessibility.
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Hasegawa-Johnson M, Zheng X, Kim H, Mendes C, Dickinson M, Hege E, Zwilling C, Channell MM, Mattie L, Hodges H, Ramig L, Bellard M, Shebanek M, Sarι L, Kalgaonkar K, Frerichs D, Bigham JP, Findlater L, Lea C, Herrlinger S, Korn P, Abou-Zahra S, Heywood R, Tomanek K, and MacDonald B
- Subjects
- Humans, Speech, Female, Male, Speech Disorders rehabilitation, Machine Learning, Parkinson Disease complications, Adult, Middle Aged, Aged, Speech Recognition Software, Dysarthria diagnosis
- Abstract
Purpose: The Speech Accessibility Project (SAP) intends to facilitate research and development in automatic speech recognition (ASR) and other machine learning tasks for people with speech disabilities. The purpose of this article is to introduce this project as a resource for researchers, including baseline analysis of the first released data package., Method: The project aims to facilitate ASR research by collecting, curating, and distributing transcribed U.S. English speech from people with speech and/or language disabilities. Participants record speech from their place of residence by connecting their personal computer, cell phone, and assistive devices, if needed, to the SAP web portal. All samples are manually transcribed, and 30 per participant are annotated using differential diagnostic pattern dimensions. For purposes of ASR experiments, the participants have been randomly assigned to a training set, a development set for controlled testing of a trained ASR, and a test set to evaluate ASR error rate., Results: The SAP 2023-10-05 Data Package contains the speech of 211 people with dysarthria as a correlate of Parkinson's disease, and the associated test set contains 42 additional speakers. A baseline ASR, with a word error rate of 3.4% for typical speakers, transcribes test speech with a word error rate of 36.3%. Fine-tuning reduces the word error rate to 23.7%., Conclusions: Preliminary findings suggest that a large corpus of dysarthric and dysphonic speech has the potential to significantly improve speech technology for people with disabilities. By providing these data to researchers, the SAP intends to significantly accelerate research into accessible speech technology., Supplemental Material: https://doi.org/10.23641/asha.27078079.
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- 2024
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39. Automatic Speech Recognition of Conversational Speech in Individuals With Disordered Speech.
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Tobin J, Nelson P, MacDonald B, Heywood R, Cave R, Seaver K, Desjardins A, Jiang PP, and Green JR
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- Humans, Male, Female, Adult, Middle Aged, Aged, Young Adult, Reading, Speech Recognition Software, Speech Disorders rehabilitation, Speech
- Abstract
Purpose: This study examines the effectiveness of automatic speech recognition (ASR) for individuals with speech disorders, addressing the gap in performance between read and conversational ASR. We analyze the factors influencing this disparity and the effect of speech mode-specific training on ASR accuracy., Method: Recordings of read and conversational speech from 27 individuals with various speech disorders were analyzed using both (a) one speaker-independent ASR system trained and optimized for typical speech and (b) multiple ASR models that were personalized to the speech of the participants with disordered speech. Word error rates were calculated for each speech model, read versus conversational, and subject. Linear mixed-effects models were used to assess the impact of speech mode and disorder severity on ASR accuracy. We investigated nine variables, classified as technical, linguistic, or speech impairment factors, for their potential influence on the performance gap., Results: We found a significant performance gap between read and conversational speech in both personalized and unadapted ASR models. Speech impairment severity notably impacted recognition accuracy in unadapted models for both speech modes and in personalized models for read speech. Linguistic attributes of utterances were the most influential on accuracy, though atypical speech characteristics also played a role. Including conversational speech samples in model training notably improved recognition accuracy., Conclusions: We observed a significant performance gap in ASR accuracy between read and conversational speech for individuals with speech disorders. This gap was largely due to the linguistic complexity and unique characteristics of speech disorders in conversational speech. Training personalized ASR models using conversational speech significantly improved recognition accuracy, demonstrating the importance of domain-specific training and highlighting the need for further research into ASR systems capable of handling disordered conversational speech effectively.
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- 2024
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40. The Diagnostic Value of qPCR Quantification of Paenibacillus larvae in Hive Debris and Adult Bees for Predicting the Onset of American Foulbrood.
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Papić B, Žvokelj L, Pislak Ocepek M, Hočevar B, Kozar M, Rus R, Zajc U, and Kušar D
- Abstract
American foulbrood (AFB) is a serious infectious disease of honeybees ( Apis mellifera ) caused by Paenibacillus larvae . Increased P. larvae count in hive-related material is associated with an increased risk of AFB. Here, we quantified P. larvae cells in 106 adult bee and 97 hive debris samples using quantitative PCR (qPCR); 66/106 adult bee and 66/97 hive debris samples were collected simultaneously from the same bee colony (paired-sample design). The corresponding bee colonies were also examined for the presence of AFB clinical signs. A binary logistic regression model to distinguish between AFB-affected and unaffected honeybee colonies showed a strong diagnostic accuracy of both sample types for predicting the onset of AFB based on P. larvae counts determined by qPCR. The colonies with a P. larvae count greater than 4.5 log cells/adult bee or 7.3 log cells/mL hive debris had a 50% probability of being clinically affected and were categorized as high-risk. The AFB-unaffected colonies had significantly lower P. larvae counts than the AFB-affected colonies, but the latter did not differ significantly in P. larvae counts in relation to the severity of clinical signs. Both bee-related sample types had a high diagnostic value for predicting disease outcome based on P. larvae counts. These results improve the understanding of the relationship between P. larvae counts and AFB occurrence, which is essential for early detection of high-risk colonies.
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- 2024
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41. Microanatomical, histochemical and morphometric features of the major and selected minor salivary glands in laboratory Wistar rat.
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Adrian Florin G, Maria-Catalina ML, Viorel M, Vasile R, Calin L, and Adela RI
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- Rats, Humans, Animals, Rats, Wistar, Parotid Gland, Salivary Glands, Minor anatomy & histology, Salivary Glands
- Abstract
The major and minor salivary glands are responsible for saliva production, a fluid drained in the oral cavity that will be involved in several functions at this level. The present study aimed to compare the microanatomy of the major (parotid, mandibular, sublingual) and minor salivary glands (von Ebner's and Weber's) of the Wistar rat, from a histological, histochemical and morphometrically point of view. Predominantly serous glands (parotid, mandibular and von Ebner's) showed differences in the secretion composition on both PAS and alcian blue reactions. The same observations were identified also for the mostly mucous salivary glands (sublingual and Weber). In terms of surface dimensions, the measurements suggested that the mucous acini have a greater surface compared with the serous ones, and, at the same time, a better-represented polymorphism. In conclusion, the major and minor salivary glands in Wistar rats, a commonly used species in experimental procedures, showed similarities with other species including humans, but some structural traits are breed-specific, this finding is very important for an extensive understanding of the morphophysiology of the salivary glands., (© 2023 Wiley-VCH GmbH. Published by John Wiley & Sons Ltd.)
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- 2024
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42. Zero-Fluoroscopy Catheter Ablation of Supraventricular Tachycardias in the Pediatric Population.
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Topalović M, Jan M, Kalinšek TP, Žižek D, Štublar J, Rus R, and Kuhelj D
- Abstract
Catheter ablation (CA) of supraventricular tachycardias (SVTs) is conventionally performed with the aid of X-ray fluoroscopy. Usage of a three-dimensional (3D) electro-anatomical mapping (EAM) system and intracardiac echocardiography (ICE) enables zero-fluoroscopy ablation, eliminating the harmful effects of radiation. We retrospectively analyzed the feasibility, effectiveness and safety of zero-fluoroscopy radiofrequency and cryoablation of various types of SVTs in pediatric patients. Overall, in 171 consecutive patients (12.5 ± 3.9 years), 175 SVTs were diagnosed and 201 procedures were performed. The procedural success rate was 98% (193/197), or more precisely, 100% (86/86) for AVNRT, 95.8% (91/95) for AVRT, 94.1% (16/17) for AT and 100% (2/2) for AFL. No complications were recorded. Follow-up was complete in 100% (171/171) of patients. During the mean follow-up period of 488.4 ± 409.5 days, 98.2% of patients were arrhythmia-free with long-term success rates of 98.7% (78/79), 97.5% (78/80), 100% (13/13) and 100% (2/2) for AVNRT, AVRT, AT and AFL, respectively. Zero-fluoroscopy CA of various types of SVTs in the pediatric population is a feasible, effective and safe treatment option.
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- 2023
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43. Clinical and genetic characteristics of a patient with phosphoribosyl pyrophosphate synthetase 1 deficiency and a systematic literature review.
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Štajer K, Kovač N, Šikonja J, Mlinarič M, Bertok S, Brecelj J, Debeljak M, Kovač J, Markelj G, Neubauer D, Rus R, Žerjav Tanšek M, Drole Torkar A, Zver A, Battelino T, Jiménez Torres R, and Grošelj U
- Abstract
Phosphoribosylpyrophosphate synthetase 1 (PRS-I) is an enzyme involved in nucleotide metabolism. Pathogenic variants in the PRPS1 are rare and PRS-I deficiency can manifest as three clinical syndromes: X-linked non-syndromic sensorineural deafness (DFN2), X-linked Charcot-Marie-Tooth neuropathy type 5 (CMTX5) and Arts syndrome. We present a Slovenian patient with PRS-I enzyme deficiency due to a novel pathogenic variant - c.424G > A (p.Val142Ile) in the PRPS1 gene, who presented with gross motor impairment, severe sensorineural deafness, balance issues, ataxia, and frequent respiratory infections. In addition, we report the findings of a systemic literature review of all described male cases of Arts syndrome and CMTX5 as well as intermediate phenotypes. As already proposed by other authors, our results confirm PRS-I deficiency should be viewed as a phenotypic continuum rather than three separate syndromes because there are multiple reports of patients with an intermediary clinical presentation., Competing Interests: Authors declare no conflict of interest., (© 2023 The Authors.)
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- 2023
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44. Early Distal Radius Remodeling after Radius Dome Osteotomy in Madelung's Deformity.
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Rus R, Scheider P, and Farr S
- Abstract
Background Distal radius realignment procedures such as the dome osteotomy have been shown to reliably correct the geometry of this three-dimensional deformity. However, it has not been clarified whether the correction is durable over time. We thus reviewed a prospective Madelung's database and evaluated all patients who received a radius dome osteotomy to see whether there was any loss of correction over time. Patients and Methods A total of 16 patients with 19 operated wrists were included. Mean age at surgery was 14 years; the most recent radiograph that was considered for analysis was obtained at a mean of 20 months after the index surgery. Three established radiographic deformity parameters (ulnar tilt, lunate subsidence, palmar carpal displacement) were repetitively measured by an independent observer over the course of the postoperative follow-up and compared using the analysis of variance statistics. Results A mild but nonsignificant worsening of deformity parameters was observed in six patients (32%). Overall, ulnar tilt improved from 50 to 40 degrees after surgery and was 43 degrees at latest follow-up. The values for lunate subsidence were -5.5, 0, and -1.6 mm, respectively. Palmar carpal displacement did not recur after correction (21, 17, and 16 mm, respectively). Conclusions Frontal plane parameters remodeled to a certain degree, although not in a statistically significant manner, soon after corrective osteotomies in Madelung's deformity. Although mild surgical overcorrection may be warranted in younger patients, it remains unclear if a concomitant distal radial epiphysiodesis would generally be beneficial. Level of Evidence Level IV., Competing Interests: Conflict of Interest None declared., (Thieme. All rights reserved.)
- Published
- 2023
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45. Knowledge gaps and future directions in cognitive functions in children and adolescents with primary arterial hypertension: A systematic review.
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Lucas I, Puteikis K, Sinha MD, Litwin M, Merkevicius K, Azukaitis K, Rus R, Pac M, Obrycki L, Bårdsen T, Śladowska-Kozłowska J, Sagsak E, Lurbe E, Jiménez-Murcia S, Jankauskiene A, and Fernández-Aranda F
- Abstract
Arterial hypertension (AH) among adults is known to be associated with worse cognitive outcomes. Similarly, children and adolescents with AH could be expected to underperform during neuropsychological evaluations when compared with healthy peers. Our aims were to review the existing literature on cognitive functioning among children and adolescents with primary AH and to identify what additional evidence may be needed to substantiate the impact of hypertension on poor cognitive outcomes in this population. We conducted a systematic review of articles in PubMed and Web of Science published before 17 January 2022, reporting on cognitive testing among children and adolescents with primary AH. From 1,316 records, 13 were included in the review-7 used battery-testing while other employed indirect measures of cognitive functions. Most of the studies reported worse results among individuals with AH. Results of two prospective trials suggested that cognitive functioning may improve after starting antihypertensive treatment. Ambulatory blood pressure monitoring was shown to be more strongly related to cognitive testing results than office measures of blood pressure. Significant confounders, namely obesity and sleep apnea, were identified throughout the studies. Our review indicates that evidence relating AH with poor cognitive functioning among youth is usually based on indirect measures of executive functions (e.g., questionnaires) rather than objective neuropsychological tests. Future prospective trials set to test different cognitive domains in children and adolescents undergoing treatment for AH are endorsed and should consider using standardized neuropsychological batteries as well as adjust the assessing results for obesity and sleep disorders., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Lucas, Puteikis, Sinha, Litwin, Merkevicius, Azukaitis, Rus, Pac, Obrycki, Bårdsen, Śladowska-Kozłowska, Sagsak, Lurbe, Jiménez-Murcia, Jankauskiene and Fernández-Aranda.)
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- 2022
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46. Remoteness, models of primary care and inequity: Medicare under-expenditure in the Northern Territory.
- Author
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Zhao Y, Wakerman J, Zhang X, Wright J, VanBruggen M, Nasir R, Duckett S, and Burgess P
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- Aged, Delivery of Health Care, Humans, National Health Programs, Northern Territory, Primary Health Care methods, Health Expenditures, Health Services, Indigenous
- Abstract
Objective To analyse Medicare expenditure by State/Territory, remoteness, and Indigenous demography to assess funding equality in meeting the health needs of remote Indigenous populations in the Northern Territory. Methods Analytic descriptions of Medicare online reports on services and benefits by key demographic variables linked with Australian Bureau of Statistics data on remoteness and Indigenous population proportion. The Northern Territory Indigenous and non-Indigenous populations were compared with the Australian average between the 2010/2011 and 2019/2020 fiscal years in terms of standardised rates of Medicare services and benefits. These were further analysed using ordinary least squares, simultaneous equations and multilevel models. Results In per capita terms, the Northern Territory receives around 30% less Medicare funds than the national average, even when additional Commonwealth funding for Aboriginal medical services is included. This funding shortfall amounts to approximately AU$80 million annually across both the Medicare Benefits Schedule and Pharmaceutical Benefits Scheme. The multilevel models indicate that providing healthcare for an Aboriginal and Torres Strait Islander person in a remote area involves a Medicare shortfall of AU$531-AU$1041 less Medicare Benefits Schedule benefits per annum compared with a non-Indigenous person in an urban area. Indigenous population proportion, together with remoteness, explained 51% of the funding variation. An age-sex based capitation funding model would correct about 87% of the Northern Territory primary care funding inequality. Conclusions The current Medicare funding scheme systematically disadvantages the Northern Territory. A needs-based funding model is required that does not penalise the Northern Territory population based on the remote primary health care service model.
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- 2022
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47. Phenotypic Variability in Siblings With Autosomal Recessive Polycystic Kidney Disease.
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Ajiri R, Burgmaier K, Akinci N, Broekaert I, Büscher A, Dursun I, Duzova A, Eid LA, Fila M, Gessner M, Gokce I, Massella L, Mastrangelo A, Miklaszewska M, Prikhodina L, Ranchin B, Ranguelov N, Rus R, Sever L, Thumfart J, Weber LT, Wühl E, Yilmaz A, Dötsch J, Schaefer F, and Liebau MC
- Abstract
Introduction: Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disorder characterized by early onset fibrocystic hepatorenal changes. Previous reports have documented pronounced phenotypic variability even among siblings in terms of patient survival. The underlying causes for this clinical variability are incompletely understood., Methods: We present the longitudinal clinical courses of 35 sibling pairs included in the ARPKD registry study ARegPKD, encompassing data on primary manifestation, prenatal and perinatal findings, genetic testing, and family history, including kidney function, liver involvement, and radiological findings., Results: We identified 70 siblings from 35 families with a median age of 0.7 (interquartile range 0.1-6.0) years at initial diagnosis and a median follow-up time of 3.5 (0.2-6.2) years. Data on PKHD1 variants were available for 37 patients from 21 families. There were 8 patients from 7 families who required kidney replacement therapy (KRT) during follow-up. For 44 patients from 26 families, antihypertensive therapy was documented. Furthermore, 37 patients from 24 families had signs of portal hypertension with 9 patients from 6 families having substantial hepatic complications. Interestingly, pronounced variability in the clinical course of functional kidney disease was documented in only 3 sibling pairs. In 17 of 20 families of our cohort of neonatal survivors, siblings had only minor differences of kidney function at a comparable age., Conclusion: In patients surviving the neonatal period, our longitudinal follow-up of 70 ARPKD siblings from 35 families revealed comparable clinical courses of kidney and liver diseases in most families. The data suggest a strong impact of the underlying genotype., (© 2022 International Society of Nephrology. Published by Elsevier Inc.)
- Published
- 2022
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48. Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD.
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Burgmaier K, Kilian S, Arbeiter K, Atmis B, Büscher A, Derichs U, Dursun I, Duzova A, Eid LA, Galiano M, Gessner M, Gokce I, Haeffner K, Hooman N, Jankauskiene A, Körber F, Longo G, Massella L, Mekahli D, Miloševski-Lomić G, Nalcacioglu H, Rus R, Shroff R, Stabouli S, Weber LT, Wygoda S, Yilmaz A, Zachwieja K, Zagozdzon I, Dötsch J, Schaefer F, and Liebau MC
- Subjects
- Adolescent, Biomarkers, Child, Child, Preschool, Cohort Studies, Disease Progression, Female, Glomerular Filtration Rate physiology, Humans, Infant, Liver Cirrhosis physiopathology, Longitudinal Studies, Male, Organ Size genetics, Organ Size physiology, Polycystic Kidney, Autosomal Recessive metabolism, Prognosis, Receptors, Cell Surface genetics, Renal Insufficiency, Chronic physiopathology, Ultrasonography, Kidney physiopathology, Polycystic Kidney, Autosomal Recessive mortality, Polycystic Kidney, Autosomal Recessive physiopathology
- Abstract
Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational study we analyzed the longitudinal development of sonographic kidney measurements in a cohort of 456 ARPKD patients from the international registry study ARegPKD. We furthermore evaluated correlations of sonomorphometric findings and functional kidney disease with the aim to describe the natural disease course and to identify potential prognostic markers. Kidney pole-to-pole (PTP) length and estimated total kidney volume (eTKV) increase with growth throughout childhood and adolescence despite individual variability. Height-adjusted PTP length decreases over time, but such a trend cannot be seen for height-adjusted eTKV (haeTKV) where we even observed a slight mean linear increase of 4.5 ml/m per year during childhood and adolescence for the overall cohort. Patients with two null PKHD1 variants had larger first documented haeTKV values than children with missense variants (median (IQR) haeTKV 793 (450-1098) ml/m in Null/null, 403 (260-538) ml/m in Null/mis, 230 (169-357) ml/m in Mis/mis). In the overall cohort, estimated glomerular filtration rate decreases with increasing haeTKV (median (IQR) haeTKV 210 (150-267) ml/m in CKD stage 1, 472 (266-880) ml/m in stage 5 without kidney replacement therapy). Strikingly, there is a clear correlation between haeTKV in the first eighteen months of life and kidney survival in childhood and adolescence with ten-year kidney survival rates ranging from 20% in patients of the highest to 94% in the lowest quartile. Early childhood haeTKV may become an easily obtainable prognostic marker of kidney disease in ARPKD, e.g. for the identification of patients for clinical studies., (© 2021. The Author(s).)
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- 2021
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49. FHR-5 Serum Levels and CFHR5 Genetic Variations in Patients With Immune Complex-Mediated Membranoproliferative Glomerulonephritis and C3-Glomerulopathy.
- Author
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Garam N, Cserhalmi M, Prohászka Z, Szilágyi Á, Veszeli N, Szabó E, Uzonyi B, Iliás A, Aigner C, Schmidt A, Gaggl M, Sunder-Plassmann G, Bajcsi D, Brunner J, Dumfarth A, Cejka D, Flaschberger S, Flögelova H, Haris Á, Hartmann Á, Heilos A, Mueller T, Rusai K, Arbeiter K, Hofer J, Jakab D, Sinkó M, Szigeti E, Bereczki C, Janko V, Kelen K, Reusz GS, Szabó AJ, Klenk N, Kóbor K, Kojc N, Knechtelsdorfer M, Laganovic M, Lungu AC, Meglic A, Rus R, Kersnik Levart T, Macioniene E, Miglinas M, Pawłowska A, Stompór T, Podracka L, Rudnicki M, Mayer G, Rysava R, Reiterova J, Saraga M, Seeman T, Zieg J, Sládková E, Stajic N, Szabó T, Capitanescu A, Stancu S, Tisljar M, Galesic K, Tislér A, Vainumäe I, Windpessl M, Zaoral T, Zlatanova G, Józsi M, and Csuka D
- Subjects
- Adolescent, Adult, Alleles, Case-Control Studies, Complement Activation, Disease Management, Disease Susceptibility, Enzyme-Linked Immunosorbent Assay, Female, Genetic Predisposition to Disease, Glomerulonephritis, Membranoproliferative diagnosis, Glomerulonephritis, Membranoproliferative mortality, Humans, Kidney Function Tests, Male, Polymorphism, Single Nucleotide, Prognosis, ROC Curve, Symptom Assessment, Young Adult, Antigen-Antibody Complex immunology, Biomarkers, Complement C3 immunology, Complement System Proteins genetics, Complement System Proteins metabolism, Genetic Variation, Glomerulonephritis, Membranoproliferative blood, Glomerulonephritis, Membranoproliferative etiology
- Abstract
Background: Factor H-related protein 5 (FHR-5) is a member of the complement Factor H protein family. Due to the homology to Factor H, the main complement regulator of the alternative pathway, it may also be implicated in the pathomechanism of kidney diseases where Factor H and alternative pathway dysregulation play a role. Here, we report the first observational study on CFHR5 variations along with serum FHR-5 levels in immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) patients together with the clinical, genetic, complement, and follow-up data., Methods: A total of 120 patients with a histologically proven diagnosis of IC-MPGN/C3G were enrolled in the study. FHR-5 serum levels were measured in ELISA, the CFHR5 gene was analyzed by Sanger sequencing, and selected variants were studied as recombinant proteins in ELISA and surface plasmon resonance (SPR)., Results: Eight exonic CFHR5 variations in 14 patients (12.6%) were observed. Serum FHR-5 levels were lower in patients compared to controls. Low serum FHR-5 concentration at presentation associated with better renal survival during the follow-up period; furthermore, it showed clear association with signs of complement overactivation and clinically meaningful clusters., Conclusions: Our observations raise the possibility that the FHR-5 protein plays a fine-tuning role in the pathogenesis of IC-MPGN/C3G., Competing Interests: Author VJ was employed by company Medimpax. The remaining author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Garam, Cserhalmi, Prohászka, Szilágyi, Veszeli, Szabó, Uzonyi, Iliás, Aigner, Schmidt, Gaggl, Sunder-Plassmann, Bajcsi, Brunner, Dumfarth, Cejka, Flaschberger, Flögelova, Haris, Hartmann, Heilos, Mueller, Rusai, Arbeiter, Hofer, Jakab, Sinkó, Szigeti, Bereczki, Janko, Kelen, Reusz, Szabó, Klenk, Kóbor, Kojc, Knechtelsdorfer, Laganovic, Lungu, Meglic, Rus, Kersnik Levart, Macioniene, Miglinas, Pawłowska, Stompór, Podracka, Rudnicki, Mayer, Rysava, Reiterova, Saraga, Seeman, Zieg, Sládková, Stajic, Szabó, Capitanescu, Stancu, Tisljar, Galesic, Tislér, Vainumäe, Windpessl, Zaoral, Zlatanova, Józsi and Csuka.)
- Published
- 2021
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50. A novel mutation of congenital nephrotic syndrome in a Slovenian child eventually receiving a renal transplant.
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Golob V, Nosan G, Bertok S, Frelih M, Boštjanči E, and Rus R
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- Child, Humans, Infant, Membrane Proteins genetics, Mutation, Slovenia, Kidney Transplantation, Nephrotic Syndrome drug therapy, Nephrotic Syndrome genetics
- Abstract
Congenital nephrotic syndrome (CNS) is a rare disease defined as heavy proteinuria, hypoalbuminemia, hyperlipidemia, and edema presenting in the first three months of life. It is most commonly caused by mutations in the NPHS1 gene associated with nephrotic syndrome type 1, also known as Finnish-type CNS, which is inherited in an autosomal recessive manner. Symptomatic treatment with intravenous albumins, vitamins, minerals, nutritional, and hormonal supplementation and treatment of complications are mandatory. Children refractory to the symptomatic treatment are recommended to undergo nephrectomy and renal replacement therapy, preferably renal transplantation. We report on a child with Finnish type CNS with a NPHS1 mutation, which is the first case confirmed by genetic study in Slovenia. We showed for the first time that homozygous mutation c.2928-3del in the NPHS1 gene caused exon 22 skipping, leading to a truncated protein and Fin-minor phenotype.
- Published
- 2021
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