bluuebunny/crossref_metadata_2025_split huggingface dataset:
A rare and preventable aetiology of neurodevelopmental delay and epilepsy: familial glucocorticoid deficiency
Authors: Özbek Mehmet Nuri , Demiral Meliha , Unal Edip , Karaşin Nezahat Doğan , Baran Rıza Taner , Demirbilek Huseyin 
Published: July 2021

Abstract

Objectives
Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive disorder characterised by isolated glucocorticoid deficiency. Melanocortin receptor 2 (MC2R) mediates the functions of adrenocorticotropic hormone (ACTH) in the adrenal cortex. MC2R accessory protein (MRAP) is a transmembrane protein involved in the trafficking of MC2R to the cell surface. Mutations in MC2R and MRAP genes cause FGD type 1 and 2. In the present case series, we evaluate the clinical characteristics and long-term follow-up of six cases with FGD due to mutations in MC2R and MRAP.

Case presentation
Data of six cases with FGD (five with mutations in MC2R and one with a mutation in MRAP) who were being followed at our paediatric endocrine centre was evaluated. Diagnosis of FGD was considered in case of elevated ACTH and inappropriately low cortisol level, and exclusion of other aetiologies. The main presenting complaints were hyperpigmentation and hypoglycaemic convulsion in all cases. During a follow-up period of 26–115 months, one patient with homozygous 560delT mutation in MC2R, one female with G226R mutation in MC2R and one female with IVS3ds+1delG mutation in MRAP had a neurodevelopmental delay (NDD), while the other three patients had normal neurodevelopment.

Conclusions
FGD patients due to MC2R and MRAP mutations with early diagnosis and compliance to the hydrocortisone therapy had normal neurodevelopment, while delay in diagnosis and poor compliance was associated with severe hypoglycaemic convulsions and subsequent complications NDD.
The effect of sickle cell anemia on the linear growth of Nigerian children
Authors: Chikani Ugo Nnenna , Bisi-Onyemaechi Adaobi , Ohuche Ijeoma , Onu Justus , Ugege Shalewa , Ogugua Chinwe , Mbanefo Ngozi , Chime Paschal , Emodi Ifeoma 
Published: July 2021

Abstract

Objectives
Despite the high prevalence of children with sickle cell anaemia (SCA) in West Africa, there is paucity of data on the height velocity and prevalence of growth failure in SCA patients. With advances in clinical care of SCA patients, could there be a spatial and secular trend in the growth pattern of these children? Hence, the compelling needs to embark on this study. The objectives of the study were to determine the prevalence of growth failure among patients with SCA and its correlation with age, gender and age at diagnosis.

Methods
A Prospective longitudinal study of a cohort of sickle cell anaemic paediatric patients from Pediatrics SCA Clinic, University of Nigeria Teaching Hospital, Ituku Ozalla. Patients were enrolled over a period of two years using a non-parametric convenient sampling method. Their heights were measured at baseline, three months, six months and at 12 months intervals and subsequently plotted on a standard WHO growth chart. The height velocities at different monthly intervals were calculated and compared with the WHO standard normal linear growth rates) for children (used as control) to identify those with GF. (i.e. <10th percentile). The main outcome measures were the mean height velocities at different months' intervals calculated and compared using the repeated measurement analysis of variance (ANOVA) and the Wilcoxon signed test.

Results
A cohort of 316 children aged 1–18 years with SCA was evaluated with a male preponderance of 161 (57.4%). The mean age and age at diagnosis were 11.04 ± 5.56 and 4.2 ± 1.7 years, respectively. The prevalence of growth failure and short stature was 84.7%. The burden of GF was highest among post-pubertal participants (94.1%). The most important predictor of growth velocity deficit was age (R2=0.045, standard β coefficient = −0.22, t=−03.51, p=0.001).

Conclusions
The study demonstrated high prevalence of growth failure in children and adolescents with SCA which intensified with advancement in age and older age at diagnosis.
The use of PAMG-1 testing in patients with preterm labor, intact membranes and a short sonographic cervix reduces the rate of unnecessary antenatal glucocorticoid administration
Authors: Kehl Sven , Weiss Christel , Pretscher Jutta , Baier Friederike , Faschingbauer Florian , Beckmann Matthias W. , Stumpfe Florian M. 
Published: July 2021

Abstract

Objectives
To assess the frequency of antenatal corticosteroid (ACS) administration in cases with shortened cervical length by addition of placental alpha-microglobulin-1 (PAMG-1) testing to sonographic examination.

Methods
Single centre retrospective cohort study. Rate of ACS administration was compared between cases with cervical length between 15 and 25 mm and cases with positive PAMG-1 testing and cervical length between 15 and 25 mm. We evaluated the following outcome parameters: Rate of ACS administration, gestational age at delivery, time to delivery, delivery within seven days, delivery <34 and <37 weeks’ gestation, rate of admission to neonatal intensive care unit (NICU).

Results
In total, 130 cases were included. “PAMG-1 group” consisted of 68 women, 62 cases built the “historical control group”. ACS administration was performed less frequently in the “PAMG-1 cohort” (18 (26%) vs. 46 (74%); p<0.001). The rate of delivery within seven days did not differ (2 (3%) vs. 4 (6.5%); p=0.4239). The rates of delivery <34 weeks’ gestation (7 (10%) vs. 9 (15%); p=0.4643) and <37 weeks’ gestation (19 (28%) vs. 26 (42%); p=0.0939) did not differ. Time to delivery interval was longer in the PAMG-1 group (61.5 vs. 43 days, p=0.0117). NICU admission occurred more often in the “historical control group” (22 (38%) vs. 28 (60%); p=0.0272).

Conclusions
Addition of biomarker testing can help to avoid unnecessary ACS administrations in women with shortened cervical length.
Development and characterization of healthy gummy jellies containing natural fruits
Authors: Teixeira-Lemos Edite , Almeida Ana Rita , Vouga Beatriz , Morais Cátia , Correia Inês , Pereira Pedro , Guiné Raquel P. F. 
Published: July 2021

Abstract
The reduction of sugar and the addition of healthier ingredients in gummy jellies brings some improved health characteristics to a product that usually is devoid of nutrition. Therefore, the aim of this study is to develop gummy jellies using natural ingredients, without added sugars or additives, in two varieties: one including orange juice and slightly sweetened with honey (ORH) and the other including puree made from a mixture of berries (BEM). These were submitted to physicochemical, microbiological, and sensorial analyses. Results of microbiological analyses showed that both gummies were suitable for consumption accordingly to EU legislation. The physicochemical analyses allowed making a nutritional evaluation, so that ORH and BEM presented 73.8 kcal/100 g and 39.8 kcal/100 g, respectively, five and nine times lower than similar commercial products. The contribution of macronutrients of ORH and BEM was as follows: 78.0 and 67% from carbohydrates, 21.7 and 33% from proteins, respectively. Regarding the potential functional properties, the antioxidant capacity was 50.4  ±  4.5 mg/L TE for ORH and 83.7  ±  7.6 mg/L for BEM. Sensorial evaluation showed that although the developed gummy jellies were slightly less appreciated than a commercial counterpart, still they were appreciated by members of a panel and particularly the ORH, which was rated with scores almost equal to those of the commercial sample. In addition, the developed gummies showed lower caloric values and higher antioxidant capacity than similar commercial candies. Overall, ORH and BEM gummy jellies could represent an opportunity to provide consumers with a healthier alternative to the common jelly candies available in the market.
Die Grenzen des Fortschritts
Authors: Zirfas Jörg 
Published: July 2021

AbstractDie Geschichte Europas wird spätestens seit der Aufklärung dominiert vom „Mythos des Fortschreitens“ (Sting). Fortschreiten bedeutet zunächst in einem formalen Sinne, die Dinge in einer spezifischen Weise wahrzunehmen, d.h. eine bestimmte Interpretation von Veränderungen vorzunehmen, dabei eine spezifische Richtung einzuschlagen, eine spezifische Betonung der Zukunft herauszustellen und letztlich ein spezifisches Ziel in der Zukunft zu realisieren zu suchen, wobei der Fortschritt nicht identisch mit der Erreichung des Ziels ist (Spaemann). Mit der Idee des Fortschritts gelingt eine Sicherung der Unvergeblichkeit jedes Schrittes, wobei er jede seiner Phasen zur bloßen Vorstufe der ihr folgenden herunterstuft (Blumenberg). Mit ihm lässt sich die beste aller Welten noch vervollkommnen, weil das Ziel der Vollendung in die Optimierung integriert wird: Der Fortschritt kennt nur eigene Grenzen bzw. kennt er keine mehr, weil er die Bewegung der Grenzverschiebung ist. Daher kommt er auch nie ans Ziel. Und das gilt nicht nur für Wissenschaft und Technik, sondern auch für die Bildung und den Menschen: Dieser wird zu einem sich stetig selbst überbietenden und optimierbaren Wesen ernannt. Erst die Selbstermächtigung des Menschen als homo faber, eine nicht auf Transzendenz teleologisierte Geschichte und die Depotenzierung Gottes durch den Deismus machen den modernen Fortschritt möglich. Doch mittlerweile droht der Fortschritt „das Ziel zunichte zu machen, das er verwirklichen soll – die Idee des Menschen“ (Horkheimer).
Religion als Perfektion?
Authors: Benner Dietrich 
Published: July 2021

Abstract
Der Beitrag erkennt dem Religiösen eine bleibende Bedeutung auch in modernen Gesellschaften zu und legt diese auf die Dimensionierung religiöser Bildung und Kompetenz nach religiösen Grundkenntnissen sowie religiös-theologischer Deutungs- und Partizipationskompetenz aus. Untersucht werden Entwicklungsaufgaben und -probleme, vor denen die drei monotheistischen Weltreligionen in ihrer eigenen Konfession, in ihrem Verhältnis zu anderen Religionen und mit Blick auf die Kultivierung öffentlicher Religiosität stehen. Die Ausführungen plädieren dafür, die Möglichkeiten und Erschwernisse einer Perfektionierung der genannten Religionen in intra-, inter- und öffentlichen Religionsdiskursen zu thematisieren und zum Gegenstand des öffentlichen Religionsunterrichts an Schulen zu machen.
Das Lob des Imperfekten oder zur angemessenen Rede von Gott und Mensch
Authors: Oorschot Jürgen van 
Published: July 2021

Abstract
Die alttestamentliche Gottesrede kennt in ihren unterschiedlichen Spielarten ein wechselseitiges Verweisverhältnis zwischen Gott und Mensch. Darin begegnet die Perfektion Gottes als Grenzbestimmung und das Imperfekte als Modus der Darstellung Gottes. In der Konsequenz lassen sich weder der Mensch noch Gott in den Vorstellungen des Perfekten oder des Imperfekten allein angemessen erfassen.
Die Unfähigkeit des Menschen zur Vervollkommnung als anthropologische Grundkategorie von Nicht-P in Gen 6-8
Authors: Wagner Andreas 
Published: July 2021

Abstract
Es scheint mir fruchtbar, bestimmte theologisch-anthropologische Grundkonzeption des Alten Testaments im Rahmen des Imperfektibilitäts-Diskurses zu beschreiben. Ausgangspunkt für die besonders an Gen 6,5 (im Kontext von Gen 6-8) anknüpfende Position der „Unfähigkeit des Menschen zur Vervollkommnung“: Alle Reflexionen geschehen in Gen 6,5ff. nur von Gott her und auf das Verhältnis Mensch-Gott hin; es gibt keine individuell/kollektive Unterscheidung bei dieser Problematik, sowohl der einzelne Mensch wie auch die Menschheit sind imperfektibel. Das Konzept ist offen für einen Inklusionsansatz, der vom Differenzprinzip her denkt, verfolgt selbst aber eine andere, theologische, Zielrichtung und führt den Lesenden die Angewiesenheit des Menschen auf Gottes Gnade sowie ihre Zusage vor Augen.
„Und siehe – es ist sehr gut“ (Genesis 1,31)
Authors: Erbele-Küster Dorothea 
Published: July 2021

Abstract
Die Idee der Vervollkommnung des Menschen und der Welt knüpft in der europäischen Geistesgeschichte immer wieder implizit oder explizit an den biblischen Schöpfungsbericht in Genesis 1 an. Wie tragfähig ist diese Vision angesichts der Erfahrung des Imperfekten, der Fragmentarität und des Zusammenbruchs? Der Beitrag untersucht wie es dem literarischen Masternarrativ, das die Alttestamentliche Wissenschaft als Priesterschrift bezeichnet, gelingt, an der Perfektibilität des Menschen und des Kosmos festzuhalten. Die ästhetisch reflexive Haltung Gottes gegenüber der Welt „siehe – es ist sehr gut“, zu der die Rezipierenden in der Mimesis eingeladen sind, impliziert eine ethische Haltung der Anerkennung. Die Utopie der Vollkommenheit ist damit bereits am Anfang gleichsam jenseits der Menschheitsgeschichte verankert
Anleitungen zur Selbstperfektionierung
Authors: Wiedemann Inga 
Published: July 2021

Abstract

Anleitungen zur Selbstperfektionierung wurden geschrieben, um für eine radikale Lebensreform zu werben. Die Kommunikation unter den Reformbefürwortern erfolgte über Schreiben, Lesen und Diskutieren, um Praktiken, Kenntnisse und den Zusammenhalt zu fördern. Da es nur wenigen gelang, sich zeitlebens einem entsagungsvollen Lebensstil zu unterziehen, erwachten immer wieder Bewegungen, die aufs Neue Ängste und Hoffnungen der Menschen artikulierten und zeitgemäße Rezepte für ein glücklicheres Leben lieferten. Ältere Schriften wurden von späteren Generationen verarbeitet und neu interpretiert. Waren die Anleitungen überzeugend, förderten sie über längere Zeit soziokulturelle Prozesse innerhalb der Gesellschaft. Zunächst radikal scheinende Forderungen wurden schließlich allgemein anerkannt.
The stannides Ca1.692Pt2Sn3.308, SrPtSn2 and EuAuSn2
Authors: Klenner Steffen , Reimann Maximilian Kai , Seidel Stefan , Pöttgen Rainer 
Published: July 2021

Abstract
Polycrystalline samples of the stannides Ca1.692Pt2Sn3.308, SrPtSn2 and EuAuSn2 were synthesized directly from the elements, using sealed tantalum ampoules as crucible material. The reactions were performed in muffle or induction furnaces. The phase purity of the samples was studied by X-ray powder diffraction (Guinier technique). The structures of Ca1.692Pt2Sn3.308 and SrPtSn2 were refined from single-crystal X-ray diffractometer data: NdRh2Sn4 type, Pnma, a = 1887.22(13), b = 441.22(3), c = 742.89(4) pm, wR = 0.0626, 1325 F
2 values, 45 variables for Ca1.692(8) Pt2Sn3.308(8) and CeNiSi2 type, Cmcm, a = 462.59(5), b = 1932.8(2), c = 458.00(5) pm, wR = 0.0549, 481 F
2 values, 18 variables for SrPtSn2. The calcium compound shows a homogeneity range Ca1+x
Pt2Sn4−x
with substantial Sn4/Ca2 mixing on one of the 4c Wyckoff positions. The [PtSn2] network is characterized by Pt–Sn (269–281 pm) and Sn–Sn (306–336 pm) bonding interactions. SrPtSn2 contains two different tin substructures: (i) Sn1–Sn1 zig-zag chains (282 pm) and (ii) orthorhombically distorted Sn2 squares (326 pm) with stronger and weaker Sn–Sn bonding. Together, the platinum and tin atoms build up a three-dimensional [PtSn2] network in which the platinum atoms have a distorted square-pyramidal tin coordination with Pt–Sn distances ranging from 261–270 pm. EuAuSn2 also crystallizes with the CeNiSi2-type structure with the lattice parameters a = 453.9(1), b = 2018.9(5) and c = 456.8(1) pm. Temperature dependent magnetic susceptibility studies indicate europium(II) with an experimental magnetic moment of 8.28(2) µB per Eu atom. EuAuSn2 is ordered antiferromagnetically at T

N
= 14.8(2) K. 151Eu Mössbauer spectra confirm the oxidation state +2 for europium (isomer shift δ = −11.17(2) mm s−1) and the magnetic ordering at low temperature (21.8 T magnetic hyperfine field at 6 K).
Localizing Microemboli within the Rodent Brain through Block-Face Imaging and Atlas Registration
Authors: McDonald Matthew W. , Jeffers Matthew S. , Filadelfi Melissa , Vicencio Andrea , Heidenreich Gavin , Wu Junzheng , Silasi Gergely 
Published: July 2021

AbstractBrain microinfarcts are prevalent in humans, however because of the inherent difficulty of identifying and localizing individual microinfarcts, brain-wide quantification is impractical. In mice, microinfarcts have been created by surgically introducing microemboli into the brain, but a major limitation of this model is the absence of automated methods to identify and localize individual occlusions. We present a novel and semi-automated workflow to identify the anatomic location of fluorescent emboli (microspheres) within the mouse brain through histologic processing and atlas registration. By incorporating vibratome block-face imaging with the QuickNII brain registration tool, we show that the anatomic location of microspheres can be accurately registered to brain structures within the Allen mouse brain (AMB) atlas (e.g., somatomotor areas, hippocampal region, visual areas, etc.). Compared with registering images of slide mounted sections to the AMB atlas, microsphere location was more accurately determined when block-face images were used. As a proof of principle, using this workflow we compared the distribution of microspheres within the brains of mice that were either perfused or immersion fixed. No significant effect of perfusion on total microsphere number or location was detected. In general, microspheres were distributed brain-wide, with the largest density found in the thalamus. In sum, our block-face imaging workflow enables efficient characterization of the widespread distribution of fluorescent microemboli, facilitating future investigation into the impact of microinfarct load and location on brain health.
Digital phonocardiography of cardiac arrhythmias in dogs – Preliminary experiences
Authors: Balogh Márton , Koch Fruzsina , Siver László , Krima Attila , Vörös Károly 
Published: July 2021

AbstractElectronic stethoscopes and digital phonocardiograms (DPCGs) can be applied when diagnosing cardiac murmurs, but their use for cardiac arrhythmias is not described in veterinary medicine. Data of 10 dogs are presented in this preliminary study, demonstrating the applicability of these techniques. Although the number of artefacts and the amount of baseline noise produced by the two digitising systems used did not differ, the Welch Allyn Meditron system or similar ones capable of simultaneous recording of electrocardiograms (ECGs) and DPCGs provide a better option for clinical research and education, whilst the 3M Littmann 3200 system might be more suitable for everyday clinical settings. A combined system with simultaneous phonocardiogram and ECG, especially with wireless transmission, might be a solution in the future.
COMBINED NEWTON’S THIRD-ORDER CONVERGENCE METHOD FOR MINIMIZE ONE VARIABLE FUNCTIONS
Authors: Kodnyanko V. A. , Grigorieva O. A. , Strok L. V. 
Published: July 2021

Contex. The article deals with the actual problem of numerical optimization of slowly computed unimodal functions of one variable. The analysis of existing methods of minimization of the first and second orders of convergence, which showed that these methods can be used to quickly solve these problems for functions, the values of which can be obtained without difficulty. For slowly computed functions, these methods give slow algorithms; therefore, the problem of developing fast methods for minimizing such functions is urgent.
Objective. Development of a combined third-order Newtonian method of convergence to minimize predominantly slowly computed unimodal functions, as well as the development of a database, including smooth, monotonic and partially constant functions, to test the method and compare its effectiveness with other known methods.
Method. A technique and an algorithm for solving the problem of fast minimization of a unimodal function of one variable by a combined numerical Newtonian method of the third order of convergence presented. The method is capable of recognizing strictly unimodal, monotonic and constant functions, as well as functions with partial or complete sections of a flat minimum.
Results. The results of comparison of the proposed method with other methods, including the fast Brent method, presented. 6954 problems were solved using the combined Newtonian method, while the method turned out to be faster than other methods in 95.5% of problems, Brent’s method worked faster in only 4.5% of problems. In general, the analysis of the calculation results showed that the combined method worked 1.64 times faster than the Brent method.
Conclusions. A combined third-order Newtonian method of convergence proposed for minimizing predominantly slowly computed unimodal functions of one variable. A database of problems developed, including smooth, monotone and partially constant functions, to test the method and compare its effectiveness with other known methods. It is shown that the proposed method, in comparison with other methods, including the fast Brent method, has a higher performance.
ONLINE PROBABILISTIC FUZZY CLUSTERING METHOD BASED ON EVOLUTIONARY OPTIMIZATION OF CAT SWARM
Authors: Bodyanskiy Ye. V. , Shafronenko A. Yu. , Klymova I. N. 
Published: July 2021

Context. The problems of big data clustering today is a very relevant area of artificial intelligence. This task is often found in many applications related to data mining, deep learning, etc. To solve these problems, traditional approaches and methods require that the entire data sample be submitted in batch form.
Objective. The aim of the work is to propose a method of fuzzy probabilistic data clustering using evolutionary optimization of cat swarm, that would be devoid of the drawbacks of traditional data clustering approaches.
Method. The procedure of fuzzy probabilistic data clustering using evolutionary algorithms, for faster determination of sample extrema, cluster centroids and adaptive functions, allowing not to spend machine resources for storing intermediate calculations and do not require additional time to solve the problem of data clustering, regardless of the dimension and the method of presentation for processing.
Results. The proposed data clustering algorithm based on evolutionary optimization is simple in numerical implementation, is devoid of the drawbacks inherent in traditional fuzzy clustering methods and can work with a large size of input information processed online in real time.
Conclusions. The results of the experiment allow to recommend the developed method for solving the problems of automatic clustering and classification of big data, as quickly as possible to find the extrema of the sample, regardless of the method of submitting the data for processing. The proposed method of online probabilistic fuzzy data clustering based on evolutionary optimization of cat swarm is intended for use in hybrid computational intelligence systems, neuro-fuzzy systems, in training artificial neural networks, in clustering and classification problems.
Sexual Size Dimorphism, Ovipositioning, and Hatching in Leiocephalus macropus asbolomus (Squamata: Leiocephalidae) in Alexander von Humboldt National Park in Eastern Cuba
Authors: Alfonso Yasel U. , Fajardo Gabriel , Suarez Eric , Krysko Kenneth L. 
Published: July 2021

The endemic Antillean family Leiocephalidae includes 28 currently recognized extant species in the genus Leiocephalus. These are distributed across Hispaniola, Cuba, and various islands and cays in the Bahamas. Sexual size dimorphism (SSD) is a fundamental and widespread biological phenomenon generally attributed to sexual differences in relationships between body size, survival, fecunditty, and mating success. Six species with 40 subspecies are known from Cuba. Few data are available regarding SSD and reproduction. Herein we report new life-history data and the sexual size dimorphism index (SSDI) in Leiocephalus macropus asbolomus. Fieldwork was performed during mid-August 2009 iin La Melba (Alexander von Humboldt National Park), HolguinProvince, Cuba. All males were larger in size then females and the SSDI (1.44) is the highest reported ffor any species of Leiocephalus. Egg measurments averaged 18.2 x 12.7 mml mean clutch size was 3. Eggs took 61-72 days to hatch; mean incubation time was 66.5 days, and mean hatchling SVL was 30.1 mm.