bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effects of Primary Kidney Disease Etiology on Renal Osteodystrophy in Pediatric Dialysis Patients
Authors: Sirimongkolchaiyakul Ornatcha , Wesseling‐Perry Katherine , Gales Barbara , Markovic Daniela , Elashoff David , Ramos Georgina , Pereira Renata C , Hanudel Mark R , Salusky Isidro B 
Published: January 2022

ABSTRACTCongenital diseases of the kidney and urinary tract (CAKUT) and glomerulonephritis are the main causes of chronic kidney disease (CKD) in children. Although renal osteodystrophy (ROD) and indices of mineral metabolism have been characterized in dialyzed children, the impact of primary kidney disease on ROD is unknown. We performed a cross‐sectional study of bone biopsies performed in 189 pediatric dialysis patients aged 12.6 ± 5.4 years. Patients were classified into three groups according to primary kidney disease: CAKUT (n = 82), hereditary (n = 22), or glomerular disease (n = 85). Serum concentrations of calcium, phosphate, alkaline phosphatase (ALP), parathyroid hormone (PTH), and 25(OH) vitamin D were measured at the time of biopsy. Fibroblast growth factor 23 (FGF23) levels were measured in a subset of 59 patients. Levels of calcium, phosphate, PTH, and 25(OH) vitamin D were similar across groups. CAKUT patients had higher serum ALP and lower C‐terminal FGF23 levels. Bone turnover and bone volume parameters did not differ across groups. However, osteoid volume (OV/BV), osteoid surface (OS/BS), and osteoid maturation time (OMT) were highest in the CAKUT group and lowest in the hereditary group. Multiple regression analysis revealed that calcium, phosphate, ALP, and PTH were independently associated with OV/BV and osteoid thickness (O.Th). PTH was an independent factor affecting bone formation rate. The relationship between CKD etiology and bone histomorphometric variables was abrogated after adjustment for biochemical parameters in the multivariable models. Overall, bone histology differed according to CKD etiology in the unadjusted analysis; however, this association could not be confirmed independently of biochemical parameters. Although CAKUT patients had a greater mineralization defect with elevated serum ALP levels, longitudinal studies will be needed to elucidate mediation pathways that might be involved in the complex interplay of CKD‐mineral bone disease (MBD). © 2022 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.
Measurement of sulfatides in the amniotic fluid supernatant: A useful tool in the prenatal diagnosis of metachromatic leukodystrophy
Authors: Kubaski Francyne , Herbst Zackary M. , Burin Maira Graeff , Michelin‐Tirelli Kristiane , Trapp Franciele B. , Gus Rejane , Netto Alice B. O. , Brusius‐Facchin Ana Carolina , Leistner‐Segal Sandra , Sanseverino Maria Teresa , Souza Carolina Moura Fischinger de , Wilke Matheus V. M. B. , Oliveira Thiago , Magalhães Jose A. A. , Giugliani Roberto 
Published: January 2022

AbstractMetachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal disorder caused by deficiency of arylsulfatase A (ARSA), leading to an accumulation of sulfatides. Sulfatides have been quantified in urine, dried blood spots (DBS), and tissues of patients with MLD. Newborn screening (NBS) for MLD has already been proposed based on a two‐tier approach with the quantification of sulfatides in DBS followed by the quantification of ARSA by liquid chromatography–tandem mass spectrometry (LC–MS/MS). Prenatal screening for MLD is also crucial, and sulfatide quantification in amniotic fluid (AF) can aid diagnosis. The prenatal study was initiated due to a family history of MLD at 19 weeks of gestation. ARSA was quantified in cultured amniocytes. C16:0 sulfatide was quantified by LC‐MS/MS in the supernatant of AF. Molecular analysis of the ARSA gene was performed in cultured amniocytes. ARSA was deficient in fetal cells, and C16:0 sulfatides were significantly elevated in comparison to age‐matched controls (3‐fold higher). Genetic studies identified the c.465+1G>A variant in homozygosis in the ARSA gene. Our study shows that sulfatides can be quantified in the supernatant of AF of MLD fetuses, and it could potentially aid in a faster and more accurate diagnosis of MLD patients.
Inflammation in Remote Myocardium and Left Ventricular Remodeling After Acute Myocardial Infarction: A Pilot Study Using T2 Mapping
Authors: Yang Meng‐xi , Shi Ke , Xu Hua‐yan , He Yong , Ma Min , Zhang Lu , Wang Jun‐long , Li Xue‐sheng , Fu Chuan , Li Hong , Zhou Bin , Zhou Xiao‐yue , Yang Zhi , Guo Ying‐kun , Yang Zhi‐gang 
Published: January 2022

LGE and T2 Mapping Images of Representative Cases. On LGE Images (A for Animals and C for Human), Infarcted Myocardium Demonstrated the Area with High Intensity; On T2 Maps (B for Animals and D for Human), The High Ntensity of Infarcted Myocardium Decreases from 3 Days to 3 Months Following MI. The Measurement of Remote T2 is Performed within the Defined White Contour Area. LGE: Late Gadolinium Enhancement; MR = Magnetic Resonance; MI = Myocardial Infarction; STEMI = ST†Elevated Myocardial Infarction. BY Yang et al. (555‐564)
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Role of melatonin in autism spectrum disorders in a male murine transgenic model: Study in the prefrontal cortex
Authors: Borsani Elisa , Bonomini Francesca , Bonini Sara Anna , Premoli Marika , Maccarinelli Giuseppina , Giugno Lorena , Mastinu Andrea , Aria Francesca , Memo Maurizio , Rezzani Rita 
Published: January 2022

AbstractAutism spectrum disorders (ASDs) are a group of clinically heterogeneous neurodevelopmental disorders sharing common features related to impaired social and communication abilities in addition to stereotyped behaviors. ASD patients present encephalic morphological, physiological, and biomolecular alterations with low levels of melatonin due to alterations in its pathways. Therefore, even if ASDs have traditionally been framed as behavioral disorders, several lines of evidence are accumulating that ASDs are characterized by certain anatomical and physiological abnormalities, including oxidative stress and inflammation in peripheral biomarkers, but likewise present in human brain tissue also characterized by alterations in synaptic remodeling and neuromodulation. Melatonin has also protective and antioxidant properties, so we can therefore hypothesize that alterations in melatonin's pathways may be one of the causes of the symptomatology of autism. The aim of the present study was to analyze the beneficial effect induced by melatonin administration and its possible mechanism of action in a transgenic mouse model of autism, immediately after weaning. The male mice were daily treated per os with melatonin (10 mg/Kg/day) or vehicle for 8 weeks starting from the sixth week of life. The antioxidant modulation, the GABAergic/glutamatergic impairment, and the synaptic remodeling in the prefrontal cortex have been evaluated. Social and repetitive behaviors were also evaluated. The behavioral results showed no statistical evidences, instead the immunohistochemical results indicated the ability of melatonin to promote the activity of antioxidant system, the GABAergic/glutamatergic equilibrium, and the synaptic remodeling. The results show that melatonin may be a possible adjuvant therapeutic strategy in ASDs.
Elucidation of pathological mechanism caused by human disease mutation in CaMKIIβ
Authors: Mutoh Hiroki , Aoto Kazushi , Miyazaki Takehiro , Fukuda Atsuo , Saitsu Hirotomo 
Published: January 2022

AbstractRecently, we have identified CaMKIIα and CaMKIIβ mutations in patients with neurodevelopmental disorders by whole exome sequencing study. Most CaMKII mutants have increased phosphorylation of Thr286/287, which induces autonomous activity of CaMKII, using cell culture experiments. In this study, we explored the pathological mechanism of motor dysfunction observed exclusively in a patient with Pro213Leu mutation in CaMKIIβ using a mouse model of the human disease. The homozygous CaMKIIβ Pro213Leu knockin mice showed age‐dependent motor dysfunction and growth failure from 2 weeks after birth. In the cerebellum, the mutation did not alter the mRNA transcript level, but the CaMKIIβ protein level was dramatically decreased. Furthermore, in contrast to previous result from cell culture, Thr287 phosphorylation of CaMKIIβ was also reduced. CaMKIIβ Pro213Leu knockin mice showed similar motor dysfunction as CaMKIIβ knockout mice, newly providing evidence for a loss of function rather than a gain of function. Our disease model mouse showed similar phenotypes of the patient, except for epileptic seizures. We clearly demonstrated that the pathological mechanism is a reduction of mutant CaMKIIβ in the brain, and the physiological aspects of mutation were greatly different between in vivo and cell culture.
Joint kinematics alone can distinguish hip or knee osteoarthritis patients from asymptomatic controls with high accuracy
Authors: Emmerzaal Jill , Van Rossom Sam , van der Straaten Rob , De Brabandere Arne , Corten Kristoff , De Baets Liesbet , Davis Jesse , Jonkers Ilse , Timmermans Annick , Vanwanseele Benedicte 
Published: January 2022

AbstractOsteoarthritis (OA) is one of the leading musculoskeletal disabilities worldwide, and several interventions intend to change the gait pattern in OA patients to more healthy patterns. However, an accessible way to follow up the biomechanical changes in a clinical setting is still missing. Therefore, this study aims to evaluate whether we can use biomechanical data collected from a specific activity of daily living to help distinguish hip OA patients from controls and knee OA patients from controls using features that potentially could be measured in a clinical setting. To achieve this goal, we considered three different classes of statistical models with different levels of data complexity. Class 1 is kinematics based only (clinically applicable), class 2 includes joint kinetics (semi‐applicable under the condition of access to a force plate or prediction models), and class 3 uses data from advanced musculoskeletal modeling (not clinically applicable). We used a machine learning pipeline to determine which classification model was best. We found 100% classification accuracy for KneeOA‐vs‐Asymptomatic and 93.9% for HipOA‐vs‐Asymptomatic using seven features derived from the lumbar spine and hip kinematics collected during ascending stairs. These results indicate that kinematical data alone can distinguish hip or knee OA patients from asymptomatic controls. However, to enable clinical use, we need to validate if the classifier also works with sensor‐based kinematical data and whether the probabilistic outcome of the logistic regression model can be used in the follow‐up of patients with OA.
Value co‐creation on technology‐enabled platforms for business model responsiveness and position enhancement in global value chains
Authors: Siaw Christopher Agyapong , Okorie Chukwuneke 
Published: January 2022

AbstractGlobal value chains (GVCs) enable multinational enterprises (MNEs) to engage supplier and partner firms in developing/emerging economies for lower production costs and sell products in advanced economies for maximum profits. However, digital technologies are shifting the basis of value offering in GVC from products and services to platforms. Considering that these platforms facilitate business exchanges in multi‐actor networks, the partner or supplier firms of MNEs can easily become competitors to MNEs if these firms participate in different networks. Nevertheless, relatively little research addresses how MNEs can develop structural flexibility for competitive association with and differentiation from their partner firms while developing effective strategies to optimize value creation and capture from their relationship with these partner firms. This study develops a conceptual framework to explain how value co‐creation on technology‐enabled platforms facilitates structural flexibility and strategic management of firm relationships and activities in GVC. The framework has significant implications for responsive business models and enhanced positions for firms to participate and influence value creation and appropriation in GVC.
Anti‐diabetic and gut microbiota modulation effects of sacha inchi (Plukenetia volubilis L.) leaf extract in streptozotocin‐induced type 1 diabetic mice
Authors: Lin Jinming , Wen Jiamin , Xiao Nan , Cai Yu‐Tong , Xiao Jie , Dai Wenhao , Chen Jian‐Ping , Zeng Ke‐Wu , Liu Fengsong , Du Bing , Li Pan 
Published: January 2022

AbstractBACKGROUNDSacha inchi (Plukenetia volubilis L.) tea has been used as an adjuvant treatment for diabetes in Pu’er, in the Yunnan province of China. The effects of sacha inchi tea on diabetes and the underlying mechanisms remain unknown. This study was conducted to investigate the influence of a water extract of sacha inchi (P. volubilis L.) leaves (PWE) on hypoglycemic activity and gut microbiota composition in mice with streptozotocin (STZ)‐induced type 1 diabetes mellitus (T1DM). During the 6 weeks of the study, T1DM mice were administered PWE intragastrically at 400 mg kg−1 body weight (BW) per day.RESULTSTreatment with PWE reduced excessive loss of BW and excessive intake of food. It significantly decreased blood glucose levels and improved oral glucose tolerance. The treatment caused protective histopathological transformations in sections of the pancreas, leading to decreased insulin resistance and improved insulin sensitivity. Treatment with PWE also significantly ameliorated disorders of the gut microbiota structure and increased the richness and diversity of intestinal microbial species in T1DM mice. At the genus level, the populations of several crucial bacteria, such as Akkermansia, Parabacteroides, and Muribaculum increased in the PWE treatment group but the abundance of Ruminiclostridium and Oscillibacter decreased.CONCLUSIONSTreatment with PWE can ameliorate hyperglycemic symptoms in STZ‐induced T1DM mice, and the anti‐diabetic effect of PWE was related to the amelioration of gut microbial structural disorder and the enrichment of functional bacteria. © 2022 Society of Chemical Industry.
Hydrophilic interaction LC–MS/MS method to avoid endogenous interference in the analysis of 4‐hydroxy isoleucine from dietary supplementation of fenugreek
Authors: Gowtham Lakshminarayanan , Ujjalkumar Das , Moksha Laxmi , Roy Tapas Kumar , Halder Nabanita , Velpandian Thirumurthy 
Published: January 2022

Abstract4‐Hydroxy isoleucine is one of the potent hypoglycemic active constituents of fenugreek seeds. A method capable of reducing biological interferences is required for bioavailability studies. An isocratic separation of 4‐hydroxy isoleucine from endogenous interferences was achieved in ZIC‐cHILIC column using 0.1% formic acid in water and acetonitrile (20:80, % v/v) pumped at 0.5 ml/min. Quantification was performed in multiple reaction monitoring mode using the transitions of m/z 148.1→102.1 and m/z 276.1→142.2 for 4‐hydroxy isoleucine and homatropine (as internal standard), respectively. After full method validation, 4‐hydroxy isoleucine levels in human plasma and commercial fenugreek formulations were determined. This method showed good linearity in the range of 50–2000 ng/mL. Intra‐ and interday accuracies were in the range of 90.64–109.0% and precision was <4.82% CV. The mean (SD) plasma concentration of 4‐hydroxy isoleucine in healthy individuals at 2 h after oral administration of fenugreek tablet was found to be 1590 (260) ng/mL. Half of marketed formulations were found to contain <0.05% of 4‐hydroxy isoleucine content. We developed a rapid hydrophilic interaction liquid chromatography–tandem mass spectrometry method for analysis of 4‐hydroxy isoleucine in human plasma. This method can be applied directly to conduct the clinical pharmacokinetics studies of 4‐hydroxy isoleucine in human population.
Systemic Bevacizumab Treatment for Recurrent Respiratory Papillomatosis: Long‐Term Follow‐Up
Authors: Ruiz Ryan , Balamuth Naomi , Javia Luv R. , Zur Karen B. 
Published: January 2022

Objectives/HypothesisSystemic bevacizumab is a new adjuvant therapy for recurrent respiratory papillomatosis (RRP) that has shown promising preliminary results in children. The objective of this study was to report the largest series to date that includes long‐term follow‐up data on bevacizumab treatment.Study DesignRetrospective chart review.MethodsRetrospective review of seven pediatric patients treated within the past 6 years with systemic bevacizumab for RRP refractory to traditional debridement.ResultsAll seven patients had a significant reduction in disease burden after initiation of systemic bevacizumab. There have been no major complications associated with systemic therapy so far. Median duration of bevacizumab treatment was 2.13 years. Three of the seven patients have been on treatment for over 3 years with the longest duration of treatment in our first patient now at 5.5 years. One patient experienced significant disease recurrence on two occasions when therapy was temporarily discontinued and was recently diagnosed with squamous cell carcinoma of the lung.ConclusionSystemic bevacizumab is an effective therapy for cases of severe RRP with promising results both in short‐term and long‐term follow‐up. Side effects are minimal. Patients must be followed closely to determine appropriate dosing intervals to control disease and to screen for disease progression.Level of Evidence4 Laryngoscope, 132:2071–2075, 2022
Featured Front Cover
Authors: Liu Yuhuai , Shahbaz Muhammad , Fang Yunying , Li Baozhen , Wei Xiaomeng , Zhu Zhenke , Lynn Tin Mar , Lu Shunbao , Shibistova Olga , Wu Jinshui , Guggenberger Georg , Ge Tida 
Published: January 2022

The cover image is based on the Original Article Stoichiometric theory shapes enzyme kinetics in paddy bulk soil but not in rhizosphere soil by Ge Tida et al., https://doi.org/10.1002/ldr.4141.
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Tracing the footsteps of open research data in China
Authors: Li Chengzan , Zhou Yuanchun , Zheng Xiaohuan , Zhang Zeyu , Jiang Lulu , Li Zongwen , Wang Pengyao , Li Jianhui , Xu Songyuan , Wang Zhanjie 
Published: January 2022

AbstractWhile the scientific research value, economic value and social value of research data have become increasingly apparent, the significance of open research data has reached a consensus. This article gives an introduction to open research data policies and measures in China, and reports on the status of constructing necessary infrastructure, specifically open data repositories. We compare open data repositories in China and Western countries in terms of scale, subject distribution, data policies, service and content operations. In addition, this article summarizes methods and motivations for data sharing among researchers in China. Finally, the paper discusses the characteristics, potential problems and challenges of China's open research data practices. We conclude with some suggestions for the future development of open research data in China from data policy, infrastructure construction, compliance with international standards and norms, credibility and influence improvement, incentives for data sharing and encouraging data sharing research practices.
Hybrid Nonisocyanate Polyurethanes (H‐NIPUs): A Pathway towards a Broad Range of Novel Materials
Authors: Bizet Boris , Grau Etienne , Asua José M. , Cramail Henri 
Published: January 2022

AbstractThe increasing demand for using less toxic and dangerous products has fostered the development of nonisocyanate polyurethanes (NIPUs). Despite several years of progress within the search for these novel materials, they are still facing difficulties to reach the market. This may be due to the low mechanical properties obtained, which can be explained by the difficult access to high molar masses polymers. The combination of NIPUs with other types of polymers, thus forming polymer–polymer hybrid‐NIPUs (so called H‐NIPUs) can be an interesting alternative for the valorization of NIPUs though. This review describes the latest advancements in the developments of H‐NIPUs, with a specific focus on the combination with epoxy, polyacrylic, and silicium‐containing polymers. Finally, the emerging field of combining NIPUs with biopolymers is discussed. A specific focus on the synthetic challenges is provided, as well as their resulting influence onto the final material properties, thus showing how the synergistic combination of NIPUs with other polymers can be a promising pathway toward the formation of novel materials.
Eco‐Friendly Needleless Electrospinning and Tannic Acid Functionalization of Polyurethane Nanofibers with Tunable Wettability and Mechanical Performances
Authors: Dodero Andrea , Castellano Maila , Vicini Silvia , Hébraud Anne , Lobry Emeline , Nhut Jean‐Mario , Schlatter Guy 
Published: January 2022

AbstractElectrospinning is the leading process allowing the fabrication of polymeric nanofibrous membranes and mats. Unfortunately, it generally requires the solubilization of the polymer in a toxic solvent causing health and environmental issues during the production. Here, it is demonstrated that polyurethane (PU) fibrous membranes can be produced by electrospinning and then, moreover, functionalized using water as the only solvent for each processing step. Waterborne polyurethane in the form of a dispersion is firstly electrospun using poly(ethylene oxide) (PEO) as template polymer. After removing PEO via a washing cycle in water, the membranes show physico‐chemical properties influenced by the initial polymeric component ratio with greater mechanical performances and hydrophobicity observed at a higher PU amount. Aqueous solutions of bio‐based tannic acid (TA) are then employed to modify the surface properties of the membranes. The high amount of galloyl units per TA molecule allows its efficient anchoring on the fiber surface through supramolecular hydrogen bonding. By varying the TA amount and the order of washing cycle, the wettability can be tuned expanding the application range of the membranes. Finally, their efficient electrospinning at high flow‐rate using a home‐made needleless emitter paves the way for industrial production via full environmentally green processing.
Synthesis of high‐performance bio‐based benzoxazine for flame retardant application
Authors: Naiker Vidhukrishnan E. , Patil Dhananjay A. , More Aarti P. , Mhaske Shashank T. 
Published: January 2022

AbstractIn this study, a phosphorous‐containing benzoxazine was synthesized using a bio‐based primary amine, vanillin diamine true sample 2 (VDTS2,IUPAC name: 4‐(([3‐aminopropyl] imino) methyl)‐2‐methoxyphenyl(4‐(([3‐aminopropyl] imino) methyl)‐2‐methoxyphenyl) phenylphosphonate), paraformaldehyde and vanillin and tested for its use as a flame‐retardant. The molecular structure of the synthesized benzoxazines was characterized by FTIR spectroscopy and1H NMR spectroscopy. Polybenzoxazine was obtained through the ring‐opening polymerization of the benzoxazine monomer, and the formation of the same was confirmed through FTIR spectroscopy. The polymerization mechanism of the benzoxazine was investigated using thermogravimetric analysis (TGA). The thermal properties of the benzoxazine were studied using differential scanning calorimetry and TGA. The flame‐retardant properties of the polybenzoxazine films obtained were investigated by the LOI. Due to the highly brittle nature of the polybenzoxazine, the synthesized polybenzoxazine was further crushed and incorporated into an epoxy system to investigate its use as a flame‐retardant additive. The thermal property and flame‐retardant property of the epoxy films were then studied. The flame‐retardant and thermal stability test results of the epoxy‐pbz films showed that the epoxy‐pbz films showed improved thermal stability and achieved a UL‐94 V‐0 rating and an LOI of 28% at 15% by weight of the polybenzoxazine concentration. SEM analysis was also conducted to study the surface morphology of the polybenzoxazine and epoxy‐pbz films pre and post the flame test. FTIR analysis was carried out to study the flame retardant mechanism of the polybenzoxazine and the epoxy‐pbz film. The respective films' mechanical properties were also investigated to study the film‐forming capability of the films.