bluuebunny/crossref_metadata_2025_split huggingface dataset:
Changes in the clinical management of 5α-reductase type 2 and 17β-hydroxysteroid dehydrogenase type 3 deficiencies in France
Authors: Bonnet Estelle , Winter Mathias , Mallet Delphine , Plotton Ingrid , Bouvattier Claire , Cartigny Maryse , Martinerie Laetiti , Polak Michel , Bachelot Anne , Huet Frédéric , Baron Sabine , Houang Muriel , Soskin Sylvie , Lienhardt Anne , Bertherat Jérôme , Amouroux Cyril , Bouty Aurore , Duranteau Lise , Besson Rémi , El Ghoneimi Alaa , Samara-Boustani Dinane , Becmeur François , Kalfa Nicolas , Paris Françoise , Medjkane François , Brac de la Perrière Aude , Bretones Patricia , Lejeune Hervé , Nicolino Marc , Mouriquand Pierre , Gorduza Daniela-Brindusa , Gay Claire-Lise 
Published: January 2023

Objectives
To examine the changes in diagnostic practices and clinical management of patients with 5α-reductase type 2 (SRD5A2) or 17β-hydroxysteroid dehydrogenase type 3 (HSD17B3) deficiency since molecular diagnoses became available.

Methods
Clinical, laboratory, and therapeutic data were retrieved from the medical records of 52 patients with a molecular diagnosis of SRD5A2 (n = 31) or HSD17B3 (n = 21) deficiency. Temporal trends regarding age at assessment and initial sex assignment over 1994–2020 were qualitatively analyzed. Age at molecular diagnosis was compared between two subgroups of patients according to their year of birth.

Results
Fifty-eight percent (n = 30) patients were diagnosed during the perinatal period, 33% (n = 17) during infancy, and 9% (n = 5) during adolescence or adulthood. Over the studied period, the patients’ age at initial assessment and diagnosis frankly decreased. The median (range) age at diagnostic confirmation was 10.5 (0–53.2) years for patients born before 2007 and 0.4 (0–9.3) years for those born in 2007 or later (P = 0.029). Genetic testing identified 27 different variants for the SRD5A2 gene (30% novel, n = 8) and 18 for the HSD17B3 gene (44% novel, n = 8). Before 2002, most patients were initially assigned as females (95%, n = 19), but this proportion dropped for those born later (44%, n = 14; P < 0.001). The influence of initial genital appearance on these decisions seemingly decreased in the most recent years. Therapeutic interventions differed according to the sex of rearing. Ten percent (n = 2) patients requested female-to-male reassignment during adulthood.

Conclusion
This study showed, over the past two decades, a clear trend toward earlier diagnosis and assignment of affected newborns as males.
Sonlu elemanlar yöntemi ile lomber spine implant tasarımı ve biyomekanik etkilerin belirlenmesi
Authors: TAHERZADEH Paniz , KELLECİ Kübra , ÖZER Sevil 
Published: January 2023

Bu çalışmada omurganın normal şekilde stabilize edilmesine yardımcı olabilecek yeni bir pedikül- vida tabanlı posterior dinamik stabilizasyon implantı, sonlu elemanlar metotu kullanılarak tasarlanmış ve sonlu elemanlar metotu ile biyomekanik etkileri tespit edilerek karşılaştırılmıştır.
Standart sınırlarda hareket aralığını koruyan iki farklı implant ve cihaz bileşenleri SOLİDWORKS programıyla oluşturulup, hareket aralığını hesaplamak için ABAQUS CAD simülasyon programı ve MATLAB programı birlikte kullanılmıştır. Tüm cihazlarda, L4-L5 omurlarını birbirine bağlayan çubuk, vida, pim ve somun bileşenler yüksek spesifik dayanım, kırılma tokluğu, gibi avantajlı özellikleri nedeniyle titanium malzemesi seçilerek tasarlanmıştır. Lomber omurganın sağlam ve farklı implantlarla tedavi edilen modelleri, Bilgisayarlı tomografi (BT) verilerine göre fizyolojik yükleme koşulları altında simüle edilmiştir. Tasarlanan tüm cihazlar altı farklı yönde (sağ-sol eksenel dönüş, sağ-sol yanal bükme ve fleksiyon-uzama) hareket aralığı, bitişik seviye etkisi ve restorasyon yüzdeleri hesaplanmıştır. İmplant parçalarının tasarımında omurganın implant ile birlikte hareketinde kabul edilebilir değer olan %70 restorasyon yüzdesine, her yönde ulaşılmaya çalışılmıştır.
Optimum verilerin elde edildiği cihaz ile Z ve Y yönünde daha fazla restorasyon sağladığı tespit edilmiştir. Restorasyon değerleri uzama için %33, fleksiyon için %53, yanal bükülme ve eksenel rotasyonlarda ise sırasıyla %68 ve %55 'dir. Bu simülasyon çalışması ile tasarlanan pedikül-vida implantların deneysel doğrulama ve klinik denemelerden sonra uygulanabilir olacağı söylenebilir.
Handwritten Arabic Digit Recognition Using Convolutional Neural Network
Authors: Alkhateeb Jawad Hasan 
Published: January 2023

In Computer vision systems, computer vision works by imitating humans in their vision way which is known as the human vision system (HVS). In HVS, humans use their eyes and brains in order to see and classify any object around them. Hence, computer vision systems imitate HSV by developing several algorithms for classifying images and objects. The main goal of this paper is to propose a model for identifying and classifying the Arabic handwritten digits with high accuracy. The concept of deep learning via the convolutional neural network (CNN) with the ADBase database is used to achieve the goal. The training is done by having a 3*3 and 5*5 filters. Basically, while the classification phase distinct learning rates are used to train the network. The obtained results are encouraging and promising.
Analysis of Cybersecurity Standard and Framework Components
Authors: Syafrizal Melwin , Selamat Siti Rahayu , Zakaria Nurul Azma 
Published: January 2023

Satisfactory cybersecurity protection, encompassing all data security solutions, can only be achieved by adopting a cybersecurity framework that provides a structure and methodology for protecting critical digital assets. In addition, security experts recommend using cybersecurity standards which consist of a collection of best practices to protect organizations from cyber threats. However, many organizations, companies and governments lack experienced personnel in the cybersecurity domain, so they have difficulty adopting a standard approach or cybersecurity framework. Protecting organizations from cyber threats while demonstrating compliance with laws and standards is seen as extremely complex due to the difficulty on choosing the appropriate standard to be used. Moreover, lack of knowledge on the elements needed that offered by the standard is lead to the problem on identifying the started point where the protection will be began. Therefore, in this paper, a literature and the analysis is presented in identifying the elements of cybersecurity standard and framework that can be facilitate the organization or government on choosing the appropriate standard and framework to be used and utilized. The literature review was carried out to understand the various types of cybersecurity standards and frameworks and the analysis is conducted to identify the elements in each of them. In this paper, eight steps are presented and include the types of international standards, which are general, local regulation, as well as specific standards used in the industrial sector, to conclude the findings of the analysis. Furthermore, a relation map is presented using Writing a Literature Review release 2.0 approach to show the relationship between the literature review and future research.
Cost Effective Cloud Storage Interoperability Between Public Cloud Platforms
Authors: Serhane Yassine , Sekkaki Abderrahim , Benzidane Karim , Abid Mehdi 
Published: January 2023

With recent advancement in technology, cloud storage became cheaper enabling organizations around the world to store more data on the cloud (texts, images, videos, databases etc.), whereas it’s for a backup, archiving or just storing data streams. New digital laws and regulation (eg. General Data Protection Regulation) require these organizations to change their way of processing or handling data, which results usually in a change of cloud providers or adoption of hybrid architecture or multi-cloud one. With the amount of data stored increasing year after year, it becomes difficult for these organizations to change cloud platforms or cloud provider and migrate their data without thinking about the technical complexity, the time and the huge cost it may incur. This article discusses the data migration and interoperability issues between cloud platforms; the proposed approach provides a simple cost-effective migration that would help organizations save time and money in this process based on a hybrid ontology approach for the brokerage of data transfers.. Keywords-Cloud Computing; Storage; Security; data; migration; cost optimization;
A Low Power Architectural Framework for Automated Surveillance System with Low Bit Rate Transmission
Authors: Mahmoud Hanan A. Hosni 
Published: January 2023

Abstract The changed security scenario of the modern time has necessitated increased and sophisticated vigilance of the countries' borders. The technological challenges involved in accomplishing such feat of automated security system are many and require research at the components-and-algorithms as well as the architectural levels. This paper proposes an architectural framework for automated video surveillance comprising a network of sensors and closed circuit television cameras as well as proposing algorithmic/component research of software and hardware for the core functioning of the framework, such as: communication protocols, object detection, data-integration, object identification, object tracking, video compression, threat identification, and alarm generation. In this paper, we are addressing some general topological and routing features that would be adopted in our system. There are two types of data with regard to data communication – video stream and object detection. The network is broken down into several disjoint, almost equal zones. A zone have one or more one cluster. A zone manager is chosen among the cluster heads depending on their relative residual energies. There are several levels of control that could be implemented with this arrangement with localized decision made, to get distributed effect at all levels. A cell tracks each target in its zone. If the target moves out of the range of a cell, the cell manager will send the target description to estimated next cell. The next cell starts tracking the target. If the estimated cell is wrongly chosen, corrections will be made by the cluster heads to get the new target-tracking. We also propose bitrate reduction algorithms to accommodate the limited bandwidth. One of the main feature of this paper is introducing a Low-Power Low-Bit rate video compression algorithm to accommodate the low power requirements at sensor nodes, and the low bit rate requirement for the communication protocol. We proposed two algorithms the ALBR and LPHSME. ALBR is addressing low bit rate required for sensors network with limited bandwidth which achieves a reduction in Average number of bits per Iframe by approximately 60% in case of low motion video sequences and 53% in case of fast motion video sequences . LPHSME addresses low power requirements of multi sensor network that has limited power batteries. The performance of the proposed LPHSME algorithm versus full search and three step search indicates a reduction in motion estimation time by approximately 89% in case of low motion video sequences (e.g., Claire ) and 84% in case of fast motion video sequences. The reduced complexity of LPHSME results in low power requirements.
Clinical and laboratory characteristics of acne tarda in women of Perm Krai
Authors: Elkin V. D. , Kobernik M. Yu. , Sedova T. G. , Kuznetsov I. D. 
Published: January 2023

Objective. To evaluate clinical and laboratory changes in patients with acne tarda. Acne is a common chronic inflammatory disease that develops on the seborrheic areas of the skin in the form of comedones, follicular papules and pustules, as well as nodes. Acne is mainly formed at puberty, but can also be observed at an older age, later acne (acne tarda), as a rule, occurs in women. At the age of 25, 45 % of women suffer from acne, at the age of 3140 acne, is observed in 26 % of women, premenstrual acne periodically occurs in 78 % of women of different ages.
Materials and methods. The study involved 62 women aged 3045 years, who formed two groups: group I included 30 patients with acne tarda, group II consisted of 32 women without acne acne tarda. The study included the investigation of complaints and anamnesis of disease and life, assessment of general and dermatological status, general blood and urine tests, biochemical blood analysis with indicators of lipid spectrum, carbohydrate metabolism, liver enzymes, pituitary hormones (LH, FSH, prolactin, TSH) and sex hormones. The methods of parametric and nonparametric statistics were used to analyze the data obtained, standard error (m) was used in the calculations, the level of statistical significance was noted at p 0.05.
Results. Most of the patients with acne tarda had a history of pubertal acne and acne in relatives. Among them changes in lipid metabolism were detected significantly more often, primarily an increase in the level of leptin (46.7 9.1 %) (p 0.05), as well as hyperlipidemia and elevated atherogenicity index. Ovarian pathology was also significantly more often observed in patients with acne tarda, mainly in the form of functional cysts (53.3 9.1 %) (p 0.05), and an increase in progesterone levels (80.0 7.3 %) (p 0.05). There were no significant differences in other sex hormones and pituitary hormones, indicators of carbohydrate metabolism and the function of hepatobiliary system in patients with acne tarda and without it.
Conclusions. Acne tarda develops mainly in women with a pubertal acne in anamnesis and a burdened heredity of acne. The disease is accompanied by a change in lipid metabolism, especially an increase in the blood leptin level, and proceeds against the background of hormonal homeostasis disorders in the form of hyperprogesteronemia with the formation of functional ovarian cysts.
Scientific Substantiation, Development, Evaluation of the Safety and Clinical Efficacy of Vitrectomy Technology in Patients with Severe Forms of Proliferative Diabetic Retinopathy Who are on Hemodialysis
Authors: Golovin A. S. , Belikova E. I. 
Published: January 2023

Purpose. Scientific substantiation, development, evaluation of the safety and clinical efficacy of vitrectomy technology in patients with severe forms of proliferative diabetic retinopathy (PDR) who are on hemodialysis (HD).Methods. We observed 100 patients (69 % women; 31 % men, mean age 63.2 ± 2.4 years) with PDR and chronic renal failure (CRF) requiring regular HD sessions (for at least 3 months). Patients were divided into two equal age, gender and number of patients (50 patients, 50 eyes) groups — the main group (MG), who underwent vitrectomy according to the developed technology, and the control group (CG), who underwent surgical intervention according to the traditional method. A comprehensive examination of the vision’s state was performed before and 14 days after surgery according to clinical and subjective indicators.Results. The data obtained indicate a more pronounced dynamics of the studied parameters in the MG compared to the CG, manifested by a significant (by 3–18 %) decrease in the incidence of intra and postoperative complications, a more pronounced (by 16.6 %, p < 0.01) decrease total score according to two developed specific subjective qualitative criteria for the condition of the fundus, a significantly higher (by 20 %) probability of an increase in BCVA, as well as a more significant increase in the level of the patient’s “quality of life” (by 10.4–14.9 %, p < 0.05 depending on the questionnaire used).Conclusion. The developed technology for performing vitrectomy in patients with PDR and end-stage chronic renal failure requiring continuous hemodialysis provides (compared to traditional) a higher level of safety and clinical efficacy due to an integrated approach to surgical intervention based on improving the stages of the operation (the maximum possible removal of the basal vitreum, performing peripheral endolaser coagulation, bimanual vitrectomy technique, and a number of others), as well as the developed medical and anesthetic support, which is confirmed (compared to the traditional technique) by a decrease in the incidence of intra and postoperative complications, as well as a more pronounced positive dynamics of BCVA, the state of the fundus and patient’s quality of life.
The Incidence and the Clinical Course of Retinal Complications of Intracameral Cefuroxime in Cataract Surgery
Authors: Svetozarskiy S. N. , Andreev A. N. , Shvaikin A. V. 
Published: January 2023

Aim. To determine the incidence and the clinical course of retinal complications of intracameral administration of cefuroxime in minimally invasive cataract surgery.Methods. From 2016 to 2021, a prospective clinical study was conducted at the Volga District Medical Center of the Federal Medical and Biological Agency of Russia, which included patients with cataract in one or two eyes to be surgically treated. All patients received 1 mg of cefuroxime injected into the anterior chamber at the end of surgery. All patients underwent ophthalmological examination including evaluation of best corrected visual acuity with determination of logarithm of minimum angle of resolution (logMAR), tonometry, biomicroscopy, and ophthalmoscopy. Each patient before and the day after surgery underwent optical coherence tomography of the retina on a spectral optical tomograph RTVue-100 (Optovue). The dynamics of the condition was evaluated on days 3 and 7 after surgery.Results. Five cases of retinal complications of intracameral cefuroxime injection were identified during the 5-year study period. According to data of optical coherence tomography, retinal edema was localized in the internal nuclear layer of the retina, and the height of serous retinal detachment in the fovea area on the next day after surgery was 315.0 ± 11.0 µm and reduced statistically significantly to 59.8 ± 42.3 µm on the 3rd day after the intervention (p = 0.043). One week later, all patients had complete retinal adherence and recovery of visual function. Retinal edema did not recur in the long term after surgery.Conclusion. Serous retinal detachment with macular edema is the most common clinically relevant complication of intracameral antibiotic prophylaxis, which develops both in compliance with the dilution protocol and in overdose, and has a favourable prognosis.
Assessment of the Effectiveness of Different Variants of Frequency Doubling Technology Perimetry in Monitoring the Glaucoma Process
Authors: Simakova I. L. , Kulikov A. N. , Tikhonovskaya I. A. 
Published: January 2023

Purpose: To compare the diagnostic effectiveness of non-standard perimetry and standard automated perimetry (SAP) during earlystage monitoring of primary open-angle glaucoma (POAG).Patients and methods. The study involved 30 patients (46 eyes) with early-stage of POAG, aged 30 to 65 years (54.9 ± 1.3), who were under ongoing monitoring for 3 to 4 years (3.50 ± 0.68 years). All subjects, in addition to the standard ophthalmological examination, performed the threshold program “24-2” perimetry using the Humphrey II, the own modification of Frequency Doubling Technology (FDT) Perimetry, in the form of 2 threshold strategies: the wellknown “FDT-16” and the new “FDT-64”. The condition of the optic nerve head (ONH) was assessed using HRT 3 (Heidelberg Retina Tomograph, Germany) and OCT — Topcon 3D OCT- 2000 (TOPCON, Japan). During monitoring, RTVue FD-OCT (Optovue, USA) was additionally performed, evaluating the parameters of the retinal ganglion cell complex (GCC).Results. Regular follow-up, the implementation of the prescribed treatment contributed to the stabilization of glaucoma, as evidenced by the stability of the average MD index values according to all 3 threshold strategies, but reliably (p = 0.000) only according to SAP data. According to the criterion of the number of scotomas, there was a weakly expressed, but statistically significant negative dynamics for all the compared strategies. According to the results of the structural assessment of the ONH (the size of the excavation), a weakly pronounced negative dynamics was obtained in dynamics according to both HRT and OCT data. The average MD index values, the number of scotomas according to the three perimeter strategies and the excavation size based on HRT and OCT in patients with early-stage of POAG turned out to be stronger and more significant using the “FDT-16”, and the correlation characteristics with the results of “24-2” HFA II, “FDT-64” largely coincided.Conclusion. A reliable correlation between structural and functional parameters indicates the feasibility of using “FDT-64” in combination with SAP not only in for the diagnosis of the early-stage of glaucoma, but also in assessing its course. The sensitivity level of the results of “FDT-64” (100 %) compared to “FDT-16” (88 %) in patients with POAG was higher, therefore, “FDT-64” is advisable to use in complex diagnostic cases and in monitoring glaucoma.
The Effect of Product Quality, WOM (Word Of Mouth), Price on Interest to Buy at Nyamleng Rice Stop in Village
Authors: Khusna Annafik Asmaul , Hariasih Misti 
Published: January 2023

This study aims to determine the effect of Product Quality, WOM, and Price on Interest in Buying Warung Nasi Pecel Nyamleng. This research is descriptive research using quantitative methods. The population used in this study were visitors who had bought at Warung Nasi Pecel Nyamleng, totaling 96 respondents using the accidental sampling technique.
This study uses data collection techniques used as a questionnaire. The data analysis technique used in this research is Multiple Linear Regression analysis using SPSS version 25 for Windows.
The results of this study indicate that product quality has a significant effect on buying interest, the promotion has a significant effect on purchase interest, and the price has a significant impact on buying interest.
Where is my footprint located? Estimating the geographical variance of Hybrid-LCA footprints
Authors: Jakobs Arthur , Schulte Simon , Pauliuk Stefan 
Published: January 2023

Abstract

Hybrid- Life Cycle Assessments (LCA) mostly fails to fully exploit valuable information from Multi-Regional Input-Output (MRIO) models by aggregating regional supply chains to the lower geographical resolution of process LCA databases. We propose a method for sampling the various individual regions within the aggregated regional scope of LCA processes. This sampling not only maximises the information content of the hybrid LCA footprint results by preserving the regional variance, it also allows for using regional price distributions form BACI/UN-COMTRADE international trade statistics to simultaneously improve the accuracy of the hybrid model. In this work we analyse the impact of regional variance and the use of regional price distributions on the uncertainty of the hybrid footprint results for both the carbon (GWP100) and land use footprint and compare this to the variance resulting from price variance only and regional variance only. We find that the median process footprint intensity increases by 7 +18 − 3 % for the GWP100 due to hybridisation, and 90 +143 − 23 % for the Land Use footprint. Our results also show that the magnitude of the footprint uncertainty strongly depends on the product sector of the LCA process and environmental impact considered. Additionally, we illustrate the effect of regional variance and price distributions on a consumption footprint using a case study of Swiss household consumption. Here the truncation error estimates are 8 . 4 +9 . 2 − 2 . 7 % for the GWP100 and 36 +64-14 % for the land use footprint. The relative uncertainties of the truncation error correction are very high (110-178% for the upper limits) moreover the have a strong positive skew. Our results highlight the importance of regionalisation of process LCA databases, as it has the potential to significantly improve both the precision and accuracy of hybrid LCA models.