bluuebunny/crossref_metadata_2025_split huggingface dataset:
Phenotypic Pleiotropy in Arginase Deficiency
Authors: Bharathi Narmadham K. , Thomas Maya Mary , Yoganathan Sangeetha , Chandran Mahalakshmi , Aaron Rekha , Danda Sumita 
Published: November 2022

Background:
Arginase deficiency is considered a masquerader of diplegic cerebral palsy. The rarity of hyperammonemic crisis and the slowly progressive course has made it a unique entity among the urea cycle defects.

Objectives:
The aim of our study is to describe the varied phenotypic spectrum of children with arginase deficiency.

Methodology:
This retrospective study included children and adolescents aged <18 years with a biochemical or genetic diagnosis of arginase deficiency from May 2011 to May 2022. Data were collected from the hospital's electronic database. The clinical presentation, laboratory parameters at baseline and during metabolic decompensation, neuroimaging, electroencephalography findings, and molecular studies were analyzed.

Results:
About 11 children from nine families with biochemically or genetically proven arginase deficiency were analyzed. The male: female ratio was 2.7:1. Consanguineous parentage was observed in all children. The median age at presentation was 36 months (Range: 5 months-18 years). All children with onset of symptoms in early childhood had a predominant delay in motor milestones of varying severity. Metabolic decompensation with encephalopathy occurred in all except two children (n = 9, 81.8%). Pyramidal signs were present in all patients and additional extrapyramidal signs in two children. Positive family history was present in four probands. Seizures occurred in all children. Epilepsy with electrical status in slow wave sleep and West syndrome was noted in three children. All children had elevated ammonia and arginine at the time of metabolic crisis. The spectrum of neuroimaging findings includes periventricular, subcortical, and deep white matter signal changes and diffusion restriction. The mean duration of follow-up was 38.6 ± 34.08 months. All patients were managed with an arginine-restricted diet and sodium benzoate with or without ornithine supplementation.

Conclusion:
Spastic diparesis, recurrent encephalopathy, presence of family history, and elevated serum arginine levels must alert the clinician to suspect arginase deficiency. Atypical presentations in our cohort include frequent metabolic crises and epileptic encephalopathy. Early identification and management will ensure a better neurodevelopmental outcome.
Prevalence and Risk Factors of Peripheral Neuropathy in Parkinson's Disease
Authors: Ramachandran Aparna , Jose James , Gafoor Abdul V , Das Smita , Balaram Neetha 
Published: November 2022

Introduction:
A lesser studied aspect of Parkinson's disease (PD) is its associated peripheral sensory-motor neuropathy. Peripheral neuropathy is an intriguing aspect of PD, a problem not given sufficient attention and which if tackled properly could make a difference to the multifaceted sufferings of the PD patient. Studies regarding the prevalence of peripheral neuropathy and its risk factors in patients with PD are scarce from the Indian subcontinent.

Methods:
This prospective observational study was conducted in a tertiary care teaching hospital in South India. Patients diagnosed with idiopathic Parkinson's disease (IPD) were screened and enrolled. All the patients underwent detailed evaluation of symptoms, signs, and electrophysiology (Nerve conduction study, Sympathetic skin response), stimulated skin wrinkling with Eutectic Mixture of Local Anesthetics. Patients found to have large/small fiber neuropathy underwent additional tests to exclude other causes of neuropathy.

Results:
A total of 154 patients with IPD were enrolled in the study (mean age: 61.96 ± 9.15 years, mean duration of disease was 4.08 ± 3.16 years). The mean Hoehn and Yahr (H and Y) score was 2.3 ± 0.825 and the mean Unified Parkinsons Disease Rating Scale (UPDRS)-3 score in the ON state was 23.07 ± 11.14. The mean cumulative levodopa dose was 482.68 ± 651.76 (median: 292; range: 4728.57) grams. Peripheral neuropathy was found in 49 patients (31.8%), large fiber in 28 (18.2%) and small fiber in 47 (30.5%); an overlap of large and small fiber neuropathy was seen in 26 patients (16.9%). Around 34% of patients had serum homocysteine levels >20 mg/dl. In univariate analysis, duration of disease, levodopa cumulative dose, serum homocysteine level, H and Y score, UPDRS-3 ON score, Toronto Clinical Neuropathy Score (P < 0.001 for all), age at presentation, and rigidity predominant presentation (P = 0.02 for both) were associated with large fiber neuropathy. All of these variables were also associated with the presence of small fiber neuropathy (P = 0.004 for age at presentation and P < 0.001 for rest), except the type of PD presentation. However, in multivariate logistic regression analysis, only duration of disease, levodopa cumulative dose, and H and Y score were associated with the presence of large and small fiber neuropathy.

Conclusions:
In our cohort, majority of the patients were in early-stage PD and around one-fifth and one-third of patients suffer from large and small fiber polyneuropathy, respectively. Large and small fiber neuropathy in PD is mainly associated with duration of disease, levodopa cumulative dose, and H and Y score.
Analysis of reasons of blood donor deferral at a tertiary care institute in India and its reflections on community health status
Authors: Malhotra Sheetal , Negi Gita 
Published: November 2022

INTRODUCTION:
Safe blood donors form the backbone of safe blood transfusion services.[1] Donor eligibility policies are a critical layer of blood safety designed to ensure selection of healthy donors and to protect recipients from any harm. This study was planned to analyze the pattern of whole blood donor deferrals and its characteristics and reasons at a tertiary care institute in northern India, as the pattern varies according to epidemiology of diseases in different demographic areas.

MATERIALS AND METHODS:
It was a cross-sectional study of 2 years’ duration from December 2015 to November 2017. The data of the potential donors who were deferred were recorded on a separate pro forma which included their demographic details, type of donation - voluntary donor and replacement donor; first time and repeat donor; type of deferrals (permanent and temporary); and the reasons of deferrals.

RESULTS:
A total of 3133 donors (voluntary - 1446 and replacement - 1687) donated and 597 donors were deferred (deferral rate - 16%) during this period. Majority of the deferrals, i.e., 525 (88%) were temporary, while 72 (12%) were permanent. The most common reason of temporary deferral was anemia. The most common reason of permanent deferrals was a medical history of jaundice.

CONCLUSIONS:
Our study results indicate that the blood donor deferral can have subtle variations based on regional aspects that should be considered when national policies are developed as pattern of deferral varies according to the epidemiology of diseases in different demographic areas.
Utilização de anticorpos monoclonais na terapia contra a COVID-19
Authors: Santana Diogo Campos , Maciel Mayara de Castro , Nakao Lilian Hikaru , Dos Santos Deny Anderson 
Published: November 2022

O SARS-Cov-2 é um vírus envelopado de fita simples e RNA positivo do gênero Betacoronavírus. Com o surgimento da covid-19 no final de 2019 e seu rápido avanço dando início a uma pandemia, pesquisadores do mundo todo empenharam-se no desenvolvimento de métodos profiláticos e terapêuticos para combater a doença. Até agosto de 2022 já havia mais de 500 milhões casos positivos e mais de 6 milhões de óbitos registrados no mundo. As vacinas, como principal método de contenção de sintomas moderados a graves da doença, tiveram um papel fundamental para o controle da pandemia, diminuindo os casos de hospitalizações e óbitos. Apesar de inúmeros estudos desenvolvidos nos últimos dois anos e utilização de medicamentos depois comprovados ineficazes (como a hidroxicloroquina e ivermectina), não há a existência de uma terapia eficaz e exata contra o vírus, fazendo com que o desenvolvimento de ferramentas biotecnológicas para um tratamento específico e eficiente seja muito aguardado. Dentre as principais moléculas terapêuticas estão os anticorpos monoclonais (MAbs) que são produtos biofarmacêuticos provenientes de hibridomas cuja função é desempenhar um efeito específico e ponderado sobre um determinado alvo. Atualmente há estudos para MAbs que possuem como alvo a proteína spike, o receptor da enzima conversora de angiotensina 2 (ACE2), IL-6, CD6 IgG1, GM-CSF, IL-1, CD147, TNF-alfa e para uso profilático. Apesar de promissora, é uma terapia que possui alguns obstáculos por ser trabalhosa e cara.
Konsep Ta’awun dalam Al-Qur’an Sebagai Penguat Tauhid dan Solidaritas Sosial
Authors: Saputra Teguh 
Published: November 2022

Penelitian ini bertujuan untuk menumbuhkan pribadi yang taat kepada Allah dan pribadi yang peduli kepada sesama manusia. Penelitian ini menggunakan metode kualitatif, jenis studi pustaka, sumber data yang berasal dari ayat al-Qur’an tentang ta’awun, teknik pengumpulan data berupa dokumen dan teknis analisis data dengan menggunakan tujuh langkah metodologi kajian tafsir mawdlu’iy al-farmawi. Hasil dan pembahasan penelitian ini adalah kata ta’awun beserta derivasinya disebutkan sebanyak 12 kali dalam al-Qur’an, yaitu dalam surah al-Fatihah: 5, surah al-Baqarah: 45, 68 dan 153, surah Maidah: 2 disebutkan sebanyak 2 kali, surah al-A’raf: 128, surah Yusuf: 18, surah al-Kahfi: 95, surah al-Anbiya: 112, surah al-Furqan: 4, dan surah al-Ma’un: 7 serta konsep ta’awun dalam al-Qur’an terbagi menjadi dua, yaitu meminta pertolongan kepada Allah Swt dan tolong menolong sesama manusia di jalan Allah Swt. Penelitian ini menyimpulkan cara terbaik meminta pertolongan adalah meminta pertolongan kepada Allah Swt (tauhid) dengan shalat dan sabar kemudian Allah Swt juga memerintahkan untuk tolong-menolong dalam kebaikan dan takwa (solidaritas sosial).
Pengaruh Faktor Makroekonomi terhadap Profitabilitas BUS: Studi pada Bank Umum Syariah di Indonesia yang Terdaftar Dalam OJK Periode 2018-2020
Authors: Maharani Emylia Dwi , Budiman Ahmad 
Published: November 2022

The purpose of this study is to determine the impact of inflation, exchange rates, and Gross Domestic Product (GDP) on the profitability of Islamic banking which in this study uses the ratio of Return on Assets (ROA) and Return on Equity (ROE). The object of research is 11 Islamic Commercial Banks that publish their financial reports to the Otoritas Jasa Keuangan for the period 2018-2020. The data used include quarterly statistics compiled from the websites of Bank Indonesia, the Badan Pusat Statistik, and Otoritas Jasa Keuangan from 2018 to 2020. The research was conducted using a quantitative methodology. After testing, it was found that inflation, exchange rate, and GDP simultaneously did not have a significant effect on ROA and ROE. Partially the exchange rate and GDP have a significant impact on ROA and ROE, but inflation does not.
Keywords: Inflation, Exchange Rate, GDP, ROA, ROE
A AGROECOLOGIA E OS DESAFIOS DAS TRANSIÇÕES AGROALIMENTARES NO CENÁRIO DE CRISE AMBIENTAL. UM ESTUDO DE CASO NO ASSENTAMENTO TRÊS NA ZONA RURAL DE ARARAS-SP
Authors: Figueiredo Eduardo 
Published: November 2022

Uma das consequências da pandemia de Covid-19 foi a quebra da cadeia de suprimentos que se deterioraram devido aos problemas logísticos, mercado mundial de fertilizantes, e os eventos climáticos extremos desempenham um papel importante na seca e desertificação, devido à erosão e contaminação dos procedimentos agricultáveis convencionais. A partir dessa premissa, a agroecologia que trabalha com métodos de aproveitamento do espaço, com produção diversificada e utilização de insumos naturais que mitigam o impacto ambiental, além de incluir trabalhadores familiares e uma logística de cadeias curtas de comercialização, surge como um projeto viável que alimenta a pequena propriedade. A discussão teórico-metodológica utilizará bibliografia dos principais debates sobre essa questão e a metodologia do ator-rede de Latour. Nesse contexto, o presente artigo pretende um estudo de caso sobre a realidade de uma família envolvendo suas produções agrícolas familiares, manejos transitórios agroecológicos e o desenvolvimento socioeconômico no Assentamento Três na zona rural do município de Araras, estado de São Paulo.
Influence of Sanitation on Employee Performance at National Social and Security Fund, Kenya
Authors: On’gonge Hezra O. , Ng’eno Weldon K. 
Published: November 2022

Purpose: Organizations exist to produce goods and services to meet the customers' demands and to give favorable working environment for workers as outlined in the Occupational Health and safety standards Act (2007). Since organizations depend on the employees as the main working force to help them meet their objectives, the provision of safe and healthy program will depend on the size of the organizations. The purpose of this study was to examine the extent to which sanitation influences employee performance at NSSF.
Methodology: In the research methodology, Descriptive research design was used where 4750 staff at the National Social Security Fund (NSSF), headquarters in Nairobi, were considered as the population. Stratified and purposive samplings were used in this study where a sample of 369 from the population was considered. The study used structured questionnaires and observation methods as instruments of data collection. Descriptive statistics was used to analyse mean, percentages and Standard deviation. This was aided by using Statistical package for social sciences (SPSS) software. Qualitative data was examined using inferential statistics. The link between sanitation as an independent variable and employee performance as a dependent variable was examined using a linear regression model.
Findings: Research’s findings suggested that a number of illnesses and disorders were brought on by poor sanitation. The research validated the 1959 Two-Factor Theory put out by Frederick Herzberg. According to the theory, two factors—motivators, or things that will gratify an employee, and hygiene factors, or items that are related to employees' mental health—have an impact on how well employees perform inside an organization.
Unique contribution to theory, policy and practice: This study is distinctive since it emphasizes how sanitation would help to boost performance while an unfavorable work environment would make the employees unhappy, which would lower their performance. According to the report, firms should create Sanitation policies and regulations that comply with the Occupational Safety and Health Act of 2007, as employee performance is a major factor in organizational advancement. Offices are becoming more often filthy as a result of the company's growing customer base, necessitating stronger waste control. Additional cleaners should be engaged, and restrooms should stock extra paper towels and hand soaps, among other necessities, in order to undertake cleaning more frequently.
Relationship between partnership and the sustainability of institutionalized child care in Lira City, northern Uganda
Authors: Otto David Ekwang , Mwesigwa David Mwesigwa 
Published: November 2022

Purpose: This study assessed the relationship between partnership and the sustainability of institutionalized child care in Lira City, northern Uganda. Specifically, three objectives guided the study, namely; to examine the relationship between networks and sustainability of institutionalized child care, to examine the relationship between coordination and sustainability of institutionalized child care, and to determine the relationship between collaboration and sustainability of institutionalized child care. As a consequence, the paper reverts to three selected variables of partnership, that is, networks, coordination and collaboration vis-à-vis sustainability of institutionalized child care.
Methodology: A mixed approach was used and data were collected using a structured questionnaire with a five-point Likert scale and an interview guide. The study was based on Forebelian principles and attachment theory. Statistical data were analyzed using descriptive statistics and inferential statistics (linear regressions). Thematic analysis was used to analyse qualitative data.
Results: The study established a positive and significant relationship between networking and sustainability of institutionalized children's care (R2 =.362, Coef. =.425) as it increases the sustainability of institutionalized child care by 36.2%. Further, the findings revealed that coordination increased sustainability of institutionalized children's care by 47.7% since it produced an adjusted R2 of.477. The regression coefficient of.477 suggested a strong and positive correlation. Finally, it was established that a significant correlation exists between collaboration and sustainability.
Unique contribution to theory, policy and practice: The study recommended that the government should partner with institutionalized children's homes since partnership is one of the fundamental mechanisms in promoting sustainability. Also, there is need for capacity building through trainings on partnerships organized by either the government or NGOs, and further studies may be conducted to examine other factors that affect the sustainability of institutionalized children’s care apart from partnerships. Consequently, the results contribute to the national policy on child care centers seeing that they are becoming more instrumental in caring for the vulnerable children.
CİNUÇEN TANRIKORUR’UN OLGUNLUK DÖNEMİ BESTECİLİĞİNDE KIYMETLİ ŞAİR MEHMET TURAN YARAR’IN YERİ VE ÖNEMİ
Authors: UYMAZ Türkan 
Published: November 2022

Cinuçen Tnrıkorur ve Mehmet Turan Yarar, Türk mûsikîsine, edebiyatına, diline ve kültürüne gönül vererek, bu yola ömrünü adamış ve hizmet etmiş, bilim, kültür ve sanat insanları olarak saygın bir yer edinerek ve önemli bir manevi miras bırakarak bu dünyadan göçmüş iki önemli değerdir. Eserleri, fikirleri, nezaketli, münevver kimlikleriyle bu iki örnek san’atkârın bıraktığı miras, medeniyetimiz var oldukça 20.yüzyıldan bütün devirlere ışık tutacak, kültürümüzün eskimeyen değerleri olarak hizmetleri ilelebet dâim olacaktır.
Bu çalışma, bu iki değerin yol arkadaşlıkları, hatıraları, mektupları, müşterek eserleri, kültür ve sanata olan hizmetleri, sanatçı kişilikleri ve dostluklarına dair edinilen bilgilerin yanı sıra, Hacı Arif Bey’e ithâfen bestelenen Kürdilihicazkar Faslı’nın sözel, makamsal ve sanatsal açıdan değerlendirilmesi konularını içermektedir. Cinuçen Tnarıkorur ve Mehmet Turan Yarar’ın müşterek eserlerinin sayısı 67’dir. Çalışmamızın hazırlanmasında destek ve birikimini esirgemeyen Barihüda Tanrıkorur Hanımefendi’ye ve Bora Uymaz’a hürmet ve teşekkürlerimizi sunarız.
BEST PRACTISE PENERAPAN MODEL PEMBELAJARAN MAKE A MATCH UNTUK MENINGKATKAN HASIL BELAJAR PPKn
Authors: IDAYATI WAHYU 
Published: November 2022

The use of techniques that are more concerned with memorization makes student achievement in Civics subjects not very good. Therefore, a new learning model is needed that can encourage students to be active in teaching and learning activities. One of the interactive learning models that can be used is the Make a Match type of Cooperative learning model. In this learning model the teacher does not dominate the lecture material, but students are active. This model requires the teacher to make two cards containing questions or problems and answer cards, then the student's task is to find a pair of cards. The use of this learning model has proven to be the best experience in overcoming problems that occur during learning. Before using the Make a Match model, students who could be categorized as complete were 58.82%, whereas after using the Make a Match model it increased to 94%. From this study it can be concluded that the use of the Make a Match model can improve student achievement in class.
ABSTRAKPenggunaan teknik yang lebih mementingkan hafalan membuat prestasi siswa pada mata pelajaran PPKn tidak terlalu baik. Oleh karena itu, diperlukan model pembelajaran baru yang dapat mendorong siswa aktif dalam kegiatan belajar-mengajar. Salah satu model pembelajaran interaktif yang dapat dipakai adalah model pembelajaran Kooperatif tipe Make a Match. Pada model pembelajaran ini guru tidak mendominasi dengan ceramah materi, tetapi siswalah yang aktif. Model ini mengharuskan guru untuk membuat dua kartu yang berisi soal atau permasalahan dan kartu jawaban, kemudian tugas siswa mencari pasangan kartunya. Penggunaan model pembelajaran ini terbukti menjadi pengalaman terbaik dalam mengatasi permasalahan yang terjadi pada saat pembelajaran. Sebelum menggunakan model Make a Match siswa yang dapat dikateogrikan tuntas adalah sebesar 58,82%, sedangkan setelah menggunakan model Make a Match naik menjadi 94%. Dari penelitian ini dapat disimpulkan bahwa penggunaan model Make a Match dapat meningkatkan prestasi siswa di kelas.
PENERAPAN MODEL PEMBELAJARAN EXPERIENTIAL LEARNING DALAM MENINGKATKAN HASIL BELAJAR PADA POKOK BAHASAN SUGGESTIONS AND OFFERS
Authors: SAJIATMOJO AAN 
Published: November 2022

Based on the description of the research results that have been presented, the researcher draws conclusions from the results of research conducted at SMA Negeri 2 Tanjung Selor, namely that there is an increase in the learning outcomes of class XI IPA students at SMA Negeri 2 Tanjung Selor in the Subject of Suggestions and offers. In the pre-cycle 7 students who studied thoroughly, then increased in cycle 1 there were 21 students who completed learning and in cycle 2 reached 29 students. The percentage of student completeness shows in the pre-cycle 22.2%, then increases to 66.6% in cycle 1 and becomes 94.4% in cycle 2. Then research with the problem "Application of Experiential Learning Models in Improving Student Learning Outcomes on the Subject Suggestions and offers in Class XI IPA SMA Negeri 2 Tanjung Selor Academic Year 2019/2020", in a process and a short period of time, the Experiential Learning Learning Model has a positive impact on students, namely students in class XI Science can improve their learning outcomes on the subject matter of Suggestions and offers.
ABSTRAKBerdasarkan uraian hasil penelitian yang telah dipaparkan, maka peneliti membuat kesimpulan dari hasil penelitian yang dilakukan di SMA Negeri 2 Tanjung Selor yaitu terdapat peningkatan Hasil Belajar siswa kelas XI IPA SMA Negeri 2 Tanjung Selor pada Pokok Bahasan Suggestions and offers. Pada pra siklus 7 siswa yang tuntas belajar, kemudian meningkat pada siklus 1 ada 21 siswa yang tuntas belajar dan pada siklus 2 mencapai 29 siswa. Presentase ketuntasan siswa menunjukkan pada pra siklus 22,2 %, kemudian meningkat menjadi 66,6 % pada siklus 1 dan menjadi 94,4 % pada siklus 2. Maka penelitian dengan permasalahan "Penerapan Model Pembelajaran Experiential Learning Dalam Meningkatkan Hasil Belajar Siswa Pada Pokok Bahasan Suggestions and offers Di Kelas XI IPA SMA Negeri 2 Tanjung Selor Tahun Ajaran 2019/2020", dalam proses dan jangka waktu yang tidak pendek, Model Pembelajaran Experiential Learning tersebut berdampak positif bagi siswa yaitu siswa kelas XI IPA bisa meningkatkan Hasil Belajarnya Pada Pokok Bahasan Suggestions and offers.