bluuebunny/crossref_metadata_2025_split huggingface dataset:
Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease
Authors: Rodinova Marie , Krizova Jana , Stufkova Hana , Bohuslavova Bozena , Askeland Georgina , Dosoudilova Zaneta , Juhas Stefan , Juhasova Jana , Ellederova Zdenka , Zeman Jiri , Eide Lars , Motlik Jan , Hansikova Hana 
Published: July 2019

ABSTRACT
Skeletal muscle wasting and atrophy is one of the more severe clinical impairments resulting from the progression of Huntington's disease (HD). Mitochondrial dysfunction may play a significant role in the etiology of HD, but the specific condition of mitochondria in muscle has not been widely studied during the development of HD. To determine the role of mitochondria in skeletal muscle during the early stages of HD, we analyzed quadriceps femoris muscle from 24-, 36-, 48- and 66-month-old transgenic minipigs that expressed the N-terminal portion of mutated human huntingtin protein (TgHD) and age-matched wild-type (WT) siblings. We found altered ultrastructure of TgHD muscle tissue and mitochondria. There was also significant reduction of activity of citrate synthase and respiratory chain complexes (RCCs) I, II and IV, decreased quantity of oligomycin-sensitivity conferring protein (OSCP) and the E2 subunit of pyruvate dehydrogenase (PDHE2), and differential expression of optic atrophy 1 protein (OPA1) and dynamin-related protein 1 (DRP1) in the skeletal muscle of TgHD minipigs. Statistical analysis identified several parameters that were dependent only on HD status and could therefore be used as potential biomarkers of disease progression. In particular, the reduction of biomarker RCCII subunit SDH30 quantity suggests that similar pathogenic mechanisms underlie disease progression in TgHD minipigs and HD patients. The perturbed biochemical phenotype was detectable in TgHD minipigs prior to the development of ultrastructural changes and locomotor impairment, which become evident at the age of 48 months. Mitochondrial disturbances may contribute to energetic depression in skeletal muscle in HD, which is in concordance with the mobility problems observed in this model.
This article has an associated First Person interview with the first author of the paper.
Tumor lysis syndrome in pediatric acute lymphoblastic leukemia at tertiary care center
Authors: Memon Bilquis Naeem , Moorani Khemchand N , Anjum Misbah , Imam Uzma 
Published: July 2019

Objectives: Tumor lysis syndrome (TLS) is common complication of acute lymphoblastic leukemia (ALL). It is characterized by presence of two or more of hyperkalemia, hyperuricemia, hyperphosphatemia and hypocalcemia. TLS may cause acute kidney injury (AKI), arrhythmias and seizures. Our objective was to determine the frequency of TLS and its biochemical abnormalities in children with ALL.
Methods: A retrospective study on 91 children, aged 2-13 years with ALL was carried out in Nephrology and Oncology departments of National Institute of Child Health, Karachi from January 2016 to December 2017. Patients already received chemotherapy were excluded. Data including risk categories, immunophenotyping, laboratory parameters like complete blood picture, serum creatinine (SCr), potassium (K), calcium (Ca), phosphorus (P) and uric acid (UA) on day 0,3 and7 after chemotherapy were collected. Data analyzed on SPSS using descriptive statistics. Independent t- test was applied to compare means and P- value<0.05 was taken as significant.
Results: Ninety-one children with mean age of 6.39±3.08 years were studied. Male were 57% and 43% female. High risk ALL were 61.5%. Pre –BALL were 82.4% and 17.5% had T-cell ALL. All patients had anemia (hemoglobin7.69±2.66 g/dl) and thrombocytopenia (43.61± 18.6 x109) where as hyperleukocytosis and blast cells were observed in 20.87% and 73.6% respectively. Comparing the biochemical parameters of ALL, the difference in SCr from D0 vs D3 (0.46±0.16 vs0.54± 0.35 and D7, 0.44±0.22) was significant (p=0.001). Similarly, difference in UA (D0, 4.12±2.40 vs D3, 3.82±1.73 and D7, 3.56±1.42), SP (D0, 4.24±1.34 vs D3, 4.61±1.76 and D7,4.13±1.07mg/dl)and for K (p=0.038) was significant. There was no difference in Ca from D0 vs D3 (0.092) and D7 (0.277). TLS was found in 62.6% children, it was chemotherapy induced in 72% and spontaneous in 28%. Clinical-TLS was observed in 14% and all CTLS had AKI. Hyperuricemia and hyperphosphatemia were the most common biochemical abnormalities in laboratory-TLS and CTLS.
Conclusion: TLS was found in 62.6% despite preventive measures. Early recognition and treatment is essential to avoid morbidity and mortality.
doi: https://doi.org/10.12669/pjms.35.4.715
How to cite this:Naeem B, Moorani KN, Anjum M, Imam U. Tumor lysis syndrome in pediatric acute lymphoblastic leukemia at tertiary care center. Pak J Med Sci. 2019;35(4):899-904. doi: https://doi.org/10.12669/pjms.35.4.715
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Biogenetic study of the emissions of species: Pinus radiata, Eucalyptus globulus Labill and Alnus acuminata in Riobamba canton, Ecuador
Authors: Mendoza Benito , Cruz Marco , Carrera Lucero , Jimenez Mauro , Caicedo Juan , Osorio Miguel , Santillán Luis , Arias Fabian 
Published: July 2019

Background: Air pollution is one of the biggest problems in the world, and it is generated by industrial production, vehicular flow and use of fossil fuels, leaving aside other important emission sources such as vegetation. The aim of this research is to quantify the emissions of natural volatile organic compounds produced by the forest species: Eucalyptus globulus L., Pinus radiata and Alnus acuminata in Riobamba, Ecuador. Methods: Identification of plant coverings in the years 2014 and 2017was performed using geographic information systems tools, complemented with the application of the Guenther model for the calculation of monoterpenes and other organic volatile compounds; thus, to analyze the relationship between meteorological variables and concentrations of volatile organic compounds and nitrogen dioxide per species. Results: Mathematical calculation of emissions in Riobamba showed that Eucalyptus globulus L. registered higher emissions in the years 2014-2017, followed by Pinus radiata and Alnus acuminata. These emissions are due to the vegetation cover covering each species. The analysis of volatile organic compounds in forest plantations in air is directly related to the emissions represented in the environment and correlated with the meteorological variables of temperature, global solar radiation and wind velocity. The proposed method manages to estimate concentrations of monoterpenes and volatile organic compounds for the two examined seasons, presenting the influence of the species introduced in this study such as Eucalyptus globulus L. and Pinus radiata, with a reduction in their emissions (less area found in the year 2017, with respect to 2014). However, the emission of Alnus acuminata can be quantified only in 2017, since in 2014 no records of this species were found. Conclusions: Volatile organic compound concentrations in the air are directly related to the emissions represented spatially and correlated with the meteorological variables of temperature, global solar radiation and wind velocity.
Contentious Issues in Surrogacy: Legal and Ethical Perspectives in India
Authors: Challa Kaumudhi 
Published: July 2019

In the past few decades, the problem of infertility among
married couples has been on the rise. The advancements
in medical science particularly in the field of artificial human reproductive technologies (ART) has benefited childless couples and provided them with various options to beget a genetically related child. Among all the options
available in ART, surrogacy is becoming very popular in India. This method of human reproduction raises conflicting human rights issues such as renting of womb, selection of surrogate, welfare and safety of child, commercialisation of reproduction, paternity and custody issues. Hence it is very essential to identify the various contentious issues involved in surrogacy and examine the
adequacy of existing legal framework in India for the regulation of practices of surrogacy. Accordingly this paper examines the contentious issues raised by the practice of surrogacy. Further it attempts to provide some practical solution for resolving those issues.
Victims of Involuntary Disappearance: An Overview of International Law
Authors: Arfat Shabina , Banday Beauty 
Published: July 2019

Victims of involuntary disappearance suffer from
physical/mental injury, emotional suffering, economic
loss resulting in substantial impairment of fundamental
rights, through acts or omissions that constitute gross
violations of international human rights law, or
humanitarian law. It also reveals that even the rights of
immediate family of a victim of involuntary
disappearance are violated. Unlike olden times, the rights
of the sufferers of involuntary disappearance are now
well protected under various international instruments.
This paper throws light on acts of involuntary
disappearance, as one of the worst case of violation of
human rights.
Coexistence of the features of perfectionism and anorexia readiness in school youth
Authors: Świerczyńska Justyna 
Published: July 2019

Cel pracyCelem przeprowadzonych badań była próba określenia struktury i nasilenia perfekcjonizmu wśród młodzieży gimnazjalnej, które mogą znacząco wpływać na wystąpienie Syndromu Gotowości Anorektycznej (SGA).MetodaBadania empiryczne przeprowadzono za pomocą Skali do Badania Indywidualnego Stosunku do Jedzenia autorstwa Ziółkowskiej (2001) i Wielowymiarowego Kwestionariusza do Badania Perfekcjonizmu autorstwa Frosta i in. (1990).Wyniki1. Wysoki poziom gotowości anorektycznej współwystępuje z wysoką krytyką rodziców i wysokimi oczekiwaniami rodziców.
2. Wysoki poziom gotowości anorektycznej współwystępuje z nadmierną koncentracją na własnych błędach i z wątpliwościami w działaniu.
3. Spostrzeganie własnej atrakcyjności współwystępuje z nadmierną koncentracją na własnych błędach i nadmierną krytyką ze strony rodziców.
4. Im wyższa tendencja do zbijania wagi tym wyższa koncentracja na własnych błędach i wątpliwości w działaniu.
5. Osoby o wysokim poziomie gotowości anorektycznej w porównaniu z osobami o niskiej gotowości anorektycznej mają wyższą koncentrację na błędach i wyższe standardy osobiste.WnioskiW badaniach grupa z podwyższonym poziomem zachowań predysponujących do powstania zaburzeń jedzenia stanowiła 25,2%. Uczniowie z wysokim poziomem gotowości anorektycznej znacznie dotkliwiej odczuwają presję związaną z wymaganiami, oczekiwaniami i krytyką ze strony rodziców niż ich koledzy z niskim poziomem zachowań anorektycznych. Stała presja w kierunku wysokich osiągnięć powoduje frustrację i brak poczucia spełnienia. W konsekwencji może zrodzić się obawa przed porażką i krytyką, co prowadzi do unikania nowych wyzwań.
A planning of sustainable development and 2018 Winter Olympic Games
Authors: Białkowski Cezary 
Published: July 2019

A postulated dynamic of the economic growth does not guarantee a protect natural environment in the level satisfed for next generations. It should minimalise a implementation of sustainable development. The aim of this paper is a presentation of a sustainable development planning propose. In the text it is compared the sustainable development planning concept with a solution prepared by an organizing committee of Winter Olympic Games 2018. To describe this issue it used a document analyse method. Organizers of PyeongChang 2018 created very similar propose to the presented concept, which was documented by much examples of the implementation of sustainable development rules. It used mainly environmental and strategic management tools.
STUDI KUAT TEKAN BETON DENGAN AGREGAT KASAR DARI BATU KAPUR
Authors: Hartono Hartono 
Published: July 2019

Hartono, in this paper explain that to obtain the allowed characteristic compressive strength of concrete from a concrete construction is quite difficult , because it is influenced by the mix of materials used for the manufacture of the construction, in which the require material of the concrete mix had to be in accordance with Reinforced Concrete Indonesia Rule Year 1991. The main factor of mix material that affect permitted the compressive strength of concrete is aggregate characteristics, namely the coarse aggregate or crushed stone. Therefore this study is intended to determine the compressive strength of concrete with the characteristics of coarse aggregate material of crushed stone that comes from limestone. This research use Gresik PC mixture concrete, muntilan sand, and kricak of limestone. To determine concrete compressive characteristics strength of concrete, concrete specimen as many as 20 pieces, with mixed-use PC weight ratio of 1 : 2 Ps : 3, cube molded kricak with the size of 15 cm X 15 cm X 15 cm was made. From these results, it can be obtained that concrete compressive characteristic strength σ 1 bk = 215.41 kg / cm2. Keyword: Concrete construction
Spatial Analysis of New Testament Textual Emendations Utilizing Confusion Distances
Authors: van Altena Vincent , Krans Jan , Bakker Henk , Dukai Balázs , Stoter Jantien 
Published: July 2019

Abstract
Before the interpretation of any text can start, the original wording of the text itself must be critically established. Conventionally, this is done following qualitative criteria. This article, however, explores the application of spatial analyses to New Testament textual criticism by demonstrating how the Levenshtein edit distance could be adapted to calculate confusion distances for variant readings in New Testament manuscripts, i.e. the possibility that a (combination of) letter(s) is confused by another (combination of) letter(s). Subsequently the outcomes are translated to Euclidian space using classical Multi-Dimensional Scaling, which enables visualisation and spatial analyses (in this case not related to geographical space). The article focuses on the data preparation and algorithm to make the data suitable for spatial analyses, thus providing the New Testament textual critic with new analytical tools.
Embedded, not Plugged-In: Digital Humanities and Fair Participation in Systematic Theological Research
Authors: Robinson Matthew Ryan 
Published: July 2019

Abstract
The article examines the disparity in use of digital humanities tools and resources among the theological disciplines, highlighting the question of why systematic theology has struggled to follow the digital turn. The author argues that issues of fairness in access and use of digital resources in knowledge production constitute an important set of concerns for systematic theologians in considering appropriate uses of the digital in their research. The article suggests that there are indeed reasons for methodological innovation in systematic theology in reaction to the digital revolution in humanities research – not, however, toward more plugged-in methods but toward methods embedded in life with the poor, underrepresented, and excluded. Three principles for a methodological “reboot” in systematic theology are given, which offer directions for further research as well as material for debate.
Living in the Existential Margins: Reflections on the Relationship Between Philosophy and Theology
Authors: Simmons J. Aaron 
Published: July 2019

Abstract
In this essay, I outline my view of an epistemic distinction between philosophy and theology along the lines of different evidential authorities operating in the two disciplines. I then go on to suggest that this epistemic view allows for a recognition of different discursive loyalties. In distinction from what I term “Plantinga-type” views, I contend that my broadly postmodern Thomistic account is preferable because of the way in which it better reflects the existential reality of a plurality of loyalties in our lived existence. In this way, I offer a defense of an existential relationship of philosophy to theology that serves as increased warrant for the epistemic account.
Model of evaluation of enterprise risk management
Authors: Mackevičius Jonas , Giriūnas Lukas 
Published: July 2019

It should be noted that in this article the enterprise‘s activity risk management and evaluation problem is defined. The risk classification is defined in these criteries: coming way, effect, activity‘s area, time, probability of result, probability of management, importance and misuses.
The model of evaluation of enterprise risk management is recommended. The model consists of these stages of risk management and evaluation: 1) identification of enterprise activity internals, 2) identification of risk determinants, 3) risk identification, 4) evaluation of risk level, 5) evaluation of risk consequence, 6) system of risk management tools, 7) evaluation of costs of risk management, 8) choice of strategy of risk management, 9) evaluation of results.
Reposisi Strukturalisme, Semantik, Semiotik, dan Hermeneutik Pada Metodologi Tafsir Alquran Dalam Dimensi Filsafat Ilmu
Authors: Tamrin Abu 
Published: July 2019

Abstract:The Koran is the revelation of Allah SWT given to the Prophet Muhammad as a miracle. The study of interpretive methodology is said to be born in Islamic intellectual thought. It is proven after the interpretation has developed, while the new methodology gets the position as the object of study. In the discussion of the style of interpretation and its schools there are eleven groups that interpret the Qur'an. While the interpretation style can be explained in the field: Fiqhy Interpretation (legal style), Philosophical Interpretation (philosophical interpretation), Ilmy Interpretation (scientific interpretation), Tarbawy Interpretation (educational interpretation), Akhlaqi Interpretation (moral interpretation), Tafsir I'tiqady (interpretation of theology) , and interpretation of Sufy (tasawuf interpretation). As for the development of interpretation methodology it can look like; The period of the Prophet Muhammad, the mutaqaddimin period, the final period, and contemporary methods. Some methods of interpretation and style of interpretation: Tafsir al-Tahlili (analytical descriptive), Ijmali interpretation (global interpretation), Muqaran interpretation (comparative interpretation), and interpretation of Maudhu'i. In this paper the Qur'anic interpretation methodology discusses structuralism, semantics, semiotics and hermeneutics. In addition to the final discussion on the methodological study of several commentators, namely: Imam Sufyan al-Tsauri, Imam al-Sulami and Syankani Imam. The paper ends with a closing.Keywords: Method of interpretation, history of development, methodology of interpretation AbstrakAlquran merupakan wahyu Allah Swt yang diberikan kepada Nabi Muhammad sebagai mukjizat. Kajian tentang metodologi tafsir dikatakan baru lahir dalam pemikiran intelektual Islam. Terbukti setelah tafsir mengalami perkembangan, sementara metodologi baru mendapat posisi sebagai objek kajian. Dalam pembahasan corak tafsir dan aliran-alirannya terdapat sebelas golongan yang menafsirkan Alquran. Sedang corak tafsir dapat dijelaskan perbidang: Tafsir Fiqhy (corak hukum), Tafsir Falsafi (tafsir filsafat), Tafsir Ilmy (tafsir ilmiah), Tafsir Tarbawy (tafsir pendidikan), Tafsir Akhlaqi (tafsir akhlak), Tafsir I’tiqady (tafsir teologi), dan Tafsir Sufy (tafsir tasawuf). Akan halnya perkembangan metodologi tafsir dapat terlihat seperti; Periode Nabi Muhammad, periode mutaqaddimin, periode mutaakhirin, dan metode kontemporer. Beberapa metode tafsir dan corak penafsiran: Tafsir al-Tahlili (deskriptif analitis), tafsir Ijmali (tafsir global), tafsir Muqaran (tafsir perbandingan), dan tafsir Maudhu’i. Dalam makalah ini dibahas metodologi tafsir Alquran tentang strukturalisme, semantik, semiotik, dan hermeneutik. Selain pembahasan akhir pada kajian metodologi beberapa ahli tafsir yaitu: Imam Sufyan al-Tsauri, Imam al-Sulami dan Imam Syankani. Makalah diakhiri dengan penutup.Kata kunci: Metode tafsir, sejarah perkembangan, metodologi tafsir
Erythrocyte Adenosine A2B Receptor-Mediated AMPK Activation: A Missing Component Counteracting CKD by Promoting Oxygen Delivery
Authors: Peng Zhangzhe , Luo Renna , Xie Tingting , Zhang Weiru , Liu Hong , Wang Wei , Tao Lijian , Kellems Rodney E. , Xia Yang 
Published: July 2019

Significance Statement
Oxygen deprivation or hypoxia drives CKD and contributes to end organ damage. The erythrocyte’s role in delivery of oxygen (O2) is regulated by hypoxia, but the effects of CKD are unknown. The authors use untargeted metabolomics to show that 2,3-BPG, an erythrocyte-specific metabolite that triggers O2 release, increases in a mouse model of CKD. Mouse genetic and human studies revealed that increased erythrocyte 2,3-BPG production and O2 release mediated by the ADORA2B-AMPK signaling cascade counteracts CKD. Enhancing AMPK activation in mice promotes 2,3-BPG production and O2 release, reducing kidney hypoxia and CKD progression. More study is needed to determine if therapies boosting 2,3-BPG production and O2 delivery slow CKD progression.

Background
Oxygen deprivation or hypoxia in the kidney drives CKD and contributes to end organ damage. The erythrocyte’s role in delivery of oxygen (O2) is regulated by hypoxia, but the effects of CKD are unknown.

Methods
We screened all of the metabolites in the whole blood of mice infused with angiotensin II (Ang II) at 140 ng/kg per minute up to 14 days to simulate CKD and compared their metabolites with those from untreated mice. Mice lacking a receptor on their erythrocytes called ADORA2B, which increases O2 delivery, and patients with CKD were studied to assess the role of ADORA2B-mediated O2 delivery in CKD.

Results
Untargeted metabolomics showed increased production of 2,3-biphosphoglycerate (2,3-BPG), an erythrocyte-specific metabolite promoting O2 delivery, in mice given Ang II to induce CKD. Genetic studies in mice revealed that erythrocyte ADORA2B signaling leads to AMPK-stimulated activation of BPG mutase, promoting 2,3-BPG production and O2 delivery to counteract kidney hypoxia, tissue damage, and disease progression in Ang II–induced CKD. Enhancing AMPK activation in mice offset kidney hypoxia by triggering 2,3-BPG production and O2 delivery. Patients with CKD had higher 2,3-BPG levels, AMPK activity, and O2 delivery in their erythrocytes compared with controls. Changes were proportional to disease severity, suggesting a protective effect.

Conclusions
Mouse and human evidence reveals that ADORA2B-AMPK signaling cascade–induced 2,3-BPG production promotes O2 delivery by erythrocytes to counteract kidney hypoxia and progression of CKD. These findings pave a way to novel therapeutic avenues in CKD targeting this pathway.
Kiğı Piltan (Pilten) Bey Camisi.
Authors: Uzun Celalettin , UĞURLU Yaşar 
Published: July 2019

Bugün
Bingöl ilinin Kiğı ilçesinde bulunan camisi Akkoyunlu döneminde Pilten Bey
tarafından inşa ettirilm­iştir. Adını banisinden alan yapı Balaban Camisi
olarak da bilinmektedir. Osmanlı döneminde şehir halkına hizmet veren cami,
kaza merkezinde bulunan en büyük ibadethane olma özelliğini taşımaktadır. Bu
sebep­ten adı arşiv belgelerinde Câmi-i Kebîr olarak zikredilmektedir.

Bu
çalışmada bu kadim yapının Osmanlı dönemindeki işleyişi hakkında bilgi
verilmiştir. Bu çerçevede mimari özelliklerine değinilmiş ve doğal afetler
sebebiyle tarihi süreç içerinde geçirdiği onarımlardan bahsedilmiştir. Ayrıca
çalışmada camideki görevliler ve bunların aldıkları ücretler ile cami vakfına
tahsis edilen gelirler hakkında bilgi verilmiştir. Böylece Osmanlı döneminde
Piltan Bey Camisi ve vakfının tarihsel süreci her yönüyle incelenmiş ve
müessesenin sosyal hayattaki rolü üzerinde durulmuştur.

Bugün
varlığını sürdürerek bölgedeki halka hizmet veren ibadethane 2017 yılında
aslına uygun bir şekil­de restore edilmek istenmiştir. Fakat yapılan bu
yenileme çalışmasının caminin tarihi dokusuna uygun olmadığı görülmektedir.