bluuebunny/crossref_metadata_2025_split huggingface dataset:
Netherton syndrome—A therapeutic challenge in childhood
Authors: Kostova Polina , Petrova Guergana , Shahid Martin , Papochieva Vera , Miteva Dimitrinka , Yordanova Ivelina , Drenovska Kossara , Bradinova Irena , Janniger Camila K. , Schwartz Robert A. , Vassileva Snejina 
Published: April 2024

Key Clinical MessageHigh‐dose intravenous immunoglobulin exhibits great potential in the treatment of Netherton syndrome.AbstractNetherton syndrome (NS) is a rare autosomal recessive genodermatosis (OMIM #256500) characterized by superficial scaling, atopic manifestations, and multisystemic complications. It is caused by loss‐of‐function mutations in the SPINK5 gene, which encode a key kallikrein protease inhibitor. There are two subtypes of the syndrome that differ in clinical presentation and immune profile: ichthyosiform erythroderma and ichthyosis linearis circumflexa. NS is a multisystemic disease with numerous extracutaneous manifestations. Current therapy for patients with NS is mainly supportive, as there is no curative or specific treatment, especially for children with NS, but targeted therapies are being developed. We describe an 8‐year‐old boy with genetically proven NS treated with intravenous immunoglobulin for recurrent skin and systemic infections from infancy, growth retardation, and associated erythroderma. Under this therapy, his skin status, infectious exacerbations, and quality of life all improved. Knowledge of the cytokine‐mediated pathogenesis of NS and the development of new biologic drugs open new possibilities for NS patients. However, the different therapeutic options have been applied in a limited number of cases, and variable responses have been shown. Randomized controlled trials with a sufficient number of patients stratified and treated according to their specific immune profile and clinical phenotype are needed to evaluate the safety and efficacy of treatment options for patients with NS.
Waldmann's disease: Primary intestinal lymphangiectasia diagnosed by 99mTc‐labeled albumin macroaggregate scintigraphy—A case report in an adult patient
Authors: Castellón Méndez Alex José , Bodán Campbell Allan , Rosales Obregón Victor , Zahran Mohammed 
Published: April 2024

Key Clinical MessageRare yet significant, this case sheds light on the uncommon presentation of Waldmann's disease in adults, showcasing the diagnostic challenges it poses. A multidisciplinary approach, integrating clinical, endoscopic, histological, and radiological evaluations, is crucial for accurate diagnosis and management. Further research is needed to deepen our understanding of this complex disorder.AbstractWaldmann's disease, or primary intestinal lymphangiectasia, is a rare disorder characterized by protein‐losing enteropathy due to dilation and leakage of intestinal lymphatic vessels. Although typically diagnosed in early childhood, we present a case of a 55‐year‐old male with a complex medical history. The patient's history included intestinal obstruction, multidrug‐resistant pulmonary tuberculosis, and primary antiphospholipid syndrome. He presented with a 2‐year history of chronic diarrhea, weight loss, and lower limb edema. Endoscopic and histological examination revealed scattered white spots in the duodenum and terminal ileum, indicative of intestinal lymphangiectasia. Nuclear medicine studies confirmed abnormal protein loss. The rarity of Waldmann's disease in adulthood and its association with other significant medical conditions pose diagnostic challenges. The distinct endoscopic and histological findings, coupled with scintigraphy results, contribute to a comprehensive understanding of this complex case. Differential diagnoses and management considerations are discussed. This case highlights the atypical presentation of Waldmann's disease in adulthood, emphasizing the importance of a multidisciplinary approach for accurate diagnosis and management. Further research is warranted to enhance our understanding of this uncommon disorder and its potential implications for patients with complex medical histories.
Adjuvant therapy for rare rectal gastrointestinal stromal tumors: A case report
Authors: Chu Ting‐Yi , Pu Ta‐Wei , Chen Chao‐Yang 
Published: April 2024

Key Clinical MessageAnorectal gastrointestinal stromal tumors are extremely rare, constituting less than 0.1% of rectal tumors. Surgical resection using a transanal wide excision followed by adjuvant therapy with tyrosine kinase inhibitors can be a successful treatment combination to remove the mass and prevent recurrence while preserving the integrity of the anal sphincter.AbstractGastrointestinal stromal tumors (GISTs) are a rare subset of neoplasms, accounting for about 1%–2% of primary gastrointestinal malignancies. The stomach is the most common site for GISTs, with anorectal GISTs being exceptionally rare, representing only 0.1% of all rectal tumors. The standard approach for managing localized GIST involves complete surgical excision to achieve negative microscopic margins (R0) while preserving the tumor capsule and maintaining anal sphincter function. Surgical resection with transanal wide excision followed by adjuvant therapy using tyrosine kinase inhibitors can successfully remove the mass, prevent recurrence, and preserve the anal sphincter's integrity. Adjuvant therapy with imatinib is the recommended treatment for all localized GISTs assessed to have an intermediate or high risk of relapse. Here, we report a case of a 63‐year‐old male with a rectal GIST who underwent transanal wide excision followed by adjuvant therapy with tyrosine kinase inhibitors.
Marchiafava‐Bignami disease: Prompt diagnosis made by magnetic resonance brain imaging
Authors: Akita Satori , Takakuwa Takeshi , Kajinami Kouji 
Published: April 2024

Key Clinical MessageMarchiafava‐Bignami disease, a rare condition often associated with alcoholism, shows myelin degeneration with tissue necrosis specifically in the corpus callosum. Urgent application of magnetic resonance imaging could lead to prompt diagnosis.AbstractA 66‐year‐old male with habitual alcohol drink complained acute deterioration of left‐side muscle weakness as initial presentation. On the arrival, the patient was confused, with stable vital sign and unremarkable pyramidal sign. Although several potential diagnoses could be considered, brain computed tomography did not provide diagnostic information, and subsequently‐performed magnetic resonance imaging revealed hyperintense lesions on T2‐flair images in corpus callosum, suggesting MBD as clinical diagnosis. Prompt diagnosis enabled us to introduce thiamine administration with subsequent favorable neurological outcome.
Duodenal gastrointestinal stromal tumor presenting with life‐threatening upper GI bleeding in a young patient: A case report and literature review
Authors: Abera Samuel Addisu , Tadesse Amanuel Kassa , Abera Kirubel Addisu , Zegeye Kassa Berie , Ibrahim Mohammed Alemu , Feleke Ashenafi Amsalu , Gebrehiwet Cheru lilay , Mengistu Segenet Bizuneh , Alemu Hirut Tesfahun , Molla Yohannis Derbew 
Published: April 2024

Key Clinical MessageDuodenal GISTs are rare and challenging tumors. Acute life‐threatening upper GI bleeding is a possible presentation of duodenal GISTs. Surgery is the standard treatment for localized duodenal GISTs. Imatinib is an effective adjuvant therapy for duodenal GISTs.AbstractGIST is the most common mesenchymal neoplasm of the gastrointestinal tract, accounting for 1%–2% of gastrointestinal tumors. They originate from the interstitial cells of Cajal and are rare in patients younger than 30 years. The stomach is the most common site, followed by the small intestine and colon. GISTs are caused by a gain‐of‐function mutation in the proto‐oncogene receptor tyrosine kinase, with activating mutations in KIT being the most common. Most GISTs are asymptomatic. Even if gastrointestinal bleeding is the most common complication life‐threatening hemorrhage is extremely uncommon. We present a case of a 31‐year‐old male patient presented with massive active hematemesis and melena with hemorrhagic shock. The patient presented with massive hematemesis and melena of 1 h duration. Endoscopy showed pulsating active bleeding from the third part of the duodenum which was difficult to manage via endoscopy. Histopathologic evaluation showed spindle cell type GIST. Intraoperatively, there was a nodular mass with active bleeding on the third part of the duodenum. Duodenectomy with end‐to‐end anastomosis was done. Discharged with no postoperative complication and was put on imatinib. There are considerable challenges that arise in the diagnosis and treatment of duodenal gastrointestinal stromal tumors (GISTs) when they present with life‐threatening upper gastrointestinal hemorrhage. In order to achieve the best possible outcomes for patients, it is crucial to have a comprehensive understanding of the clinical presentation, diagnostic methods, and treatment approaches.
A review of electrospun separators for lithium‐based batteries: Progress and application prospects
Authors: Sun Xiangru , Zhou Ying , Li Dejun , Zhao Kai , Wang Liqun , Tan Peiran , Dong Hongyang , Wang Yueming , Liang Ji 
Published: April 2024

AbstractDue to the limitations of the raw materials and processes involved, polyolefin separators used in commercial lithium‐ion batteries (LIBs) have gradually failed to meet the increasing requirements of high‐end batteries in terms of energy density, power density, and safety. Hence, it is very important to develop next‐generation separators for advanced lithium (Li)‐based rechargeable batteries including LIBs and Li–S batteries. Nonwoven nanofiber membranes fabricated via electrospinning technology are highly attractive candidates for high‐end separators due to their simple processes, low‐cost equipment, controllable microporous structure, wide material applicability, and availability of multiple functions. In this review, the electrospinning technologies for separators are reviewed in terms of devices, process and environment, and polymer solution systems. Furthermore, strategies toward the improvement of electrospun separators in advanced LIBs and Li–S batteries are presented in terms of the compositions and the structure of nanofibers and separators. Finally, the challenges and prospects of electrospun separators in both academia and industry are proposed. We anticipate that these systematic discussions can provide information in terms of commercial applications of electrospun separators and offer new perspectives for the design of functional electrospun separators for advanced Li‐based batteries.
Enhancing potassium‐ion storage of Bi2S3 through external–internal dual synergism: Ti3C2Tx compositing and Cu2+ doping
Authors: Sha Dawei , You Yurong , Hu Rongxiang , Ding Jianxiang , Cao Xin , Zhang Yuan , Pan Long , Sun ZhengMing 
Published: April 2024

AbstractPotassium‐ion batteries (PIBs) offer a cost‐effective and resource‐abundant solution for large‐scale energy storage. However, the progress of PIBs is impeded by the lack of high‐capacity, long‐life, and fast‐kinetics anode electrode materials. Here, we propose a dual synergic optimization strategy to enhance the K+ storage stability and reaction kinetics of Bi2S3 through two‐dimensional compositing and cation doping. Externally, Bi2S3 nanoparticles are loaded onto the surface of three‐dimensional interconnected Ti3C2Tx nanosheets to stabilize the electrode structure. Internally, Cu2+ doping acts as active sites to accelerate K+ storage kinetics. Various theoretical simulations and ex situ techniques are used to elucidate the external–internal dual synergism. During discharge, Ti3C2Tx and Cu2+ collaboratively facilitate K+ intercalation. Subsequently, Cu2+ doping primarily promotes the fracture of Bi2S3 bonds, facilitating a conversion reaction. Throughout cycling, the Ti3C2Tx composite structure and Cu2+ doping sustain functionality. The resulting Cu2+‐doped Bi2S3 anchored on Ti3C2Tx (C‐BT) shows excellent rate capability (600 mAh g–1 at 0.1 A g–1; 105 mAh g–1 at 5.0 A g–1) and cycling performance (91 mAh g–1 at 5.0 A g–1 after 1000 cycles) in half cells and a high energy density (179 Wh kg–1) in full cells.
Identification and management of mental health distress in Moroccan patients with cancer: Strategies adopted by oncology nurses and barriers to practice
Authors: Aquil Amina , Mouallif Mustapha , Elgot Abdeljalil 
Published: April 2024

AbstractBackgroundAdressing mental distress among cancer patients presents a substantial challenge in the delivery of oncology care.AimsThis present study aims to explore the nursing strategies for identifying and managing distress in cancer patients as well as the concomitant barriers that prevent them from achieving this task.MethodsThis qualitative study is based on a semi‐structured interview with 25 practicing nurses in oncology.ResultsStrategies used by nurses to identify mental distress in their patients include: receiving information, mobilizing interpersonal skills, and identifying causes of distress. When asked about the barriers that hinder the practice of identifying and responding to patients' distress, nurses reported facing several barriers that can be classified into three categories: health care system‐related barriers, patient‐related barriers, and nurse‐related barriers.ConclusionOncology nurses should benefit from specific training on the systematic assessment of mental distress in cancer patients, in order to improve the overall management of oncology patients.
Safety, Tolerability, and Pharmacokinetics of Anaprazole, a Novel Proton Pump Inhibitor, in Healthy Chinese Subjects
Authors: Wang Fangfang , Niu Xiaoye , Liu Fei , Ma Xifeng , Cheng Fang , Xu Haiyan , Wang Li , Xu Yanjun , Li Haiyan 
Published: April 2024

AbstractAnaprazole, a newly developed oral proton pump inhibitor, was evaluated for safety, tolerability, and pharmacokinetics in healthy Chinese subjects. This study involved administering either anaprazole sodium enteric‐coated tablet or placebo, followed by monitoring the incidence and severity of any adverse events (AEs). The pharmacokinetic parameters of anaprazole, its isomer, and main metabolisms were determined. The results showed that both single‐dose (2.5‐120 mg) and multiple‐dose (20 mg once daily, 40 mg once daily, or 20 mg twice daily) oral administration of anaprazole sodium enteric‐coated tablet were safe and well tolerated. Following single‐dose administration, the median time to reach maximum plasma concentration of anaprazole was between 3.50 and 5.25 hours, with mean elimination half‐life of 1.22‐3.79 hours. The absorption and elimination of anaprazole in the human body appeared to basically follow linear kinetics. After repeated dosing, steady‐state concentrations of anaprazole, its isomer, and primary metabolites were achieved, with a median time to reach maximum plasma concentration of 3‐3.75 hours and a mean elimination half‐life of 1.61‐2.27 hours for anaprazole. There was no significant drug accumulation after multiple‐dose oral administration. In conclusion, anaprazole sodium enteric‐coated tablets were found to be safe and well tolerated in healthy Chinese individuals. Anaprazole is absorbed and metabolized consistently in the human body without any accumulation.
Targeting glutamate metabolism in chronic lung diseases
Authors: Guo Zhihou , Yan Furong 
Published: April 2024

AbstractAmino acids are necessary for all life forms, which play various roles. Disorder of amino acid metabolism is now considered an important driving mechanism in diverse pulmonary conditions, particularly chronic lung diseases like chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis and lung cancer. Glutamate actively participates in multiple vital biological processes, while the intricate glutamate metabolism, glutamate receptors and glutamate transporters assume crucial regulatory functions in the development of chronic lung diseases. This review aims to discuss the relationship between glutamate dysfunction and chronic lung diseases. By discussing the physiological and pathological function of glutamate, we probe the potential drug targets for chronic lung diseases in the glutamate pathway.
Computational repurposing of oncology drugs through off‐target drug binding interactions from pharmacological databases
Authors: Walpole Imogen R. , Zaman Farzana Y , Zhao Peinan , Marshall Vikki M. , Lin Frank P. , Thomas David M. , Shackleton Mark , Antolin Albert A. , Ameratunga Malaka 
Published: April 2024

AbstractPurposeSystematic repurposing of approved medicines for another indication may accelerate drug development in oncology. We present a strategy combining biomarker testing with drug repurposing to identify new treatments for patients with advanced cancer.MethodsTumours were sequenced with the Illumina TruSight Oncology 500 (TSO‐500) platform or the FoundationOne CDx panel. Mutations were screened by two medical oncologists and pathogenic mutations were categorised referencing literature. Variants of unknown significance were classified as potentially pathogenic using plausible mechanisms and computational prediction of pathogenicity. Gain of function (GOF) mutations were evaluated through repurposing databases Probe Miner (PM), Broad Institute Drug Repurposing Hub (Broad Institute DRH) and TOPOGRAPH. GOF mutations were repurposing events if identified in PM, not indexed in TOPOGRAPH and excluding mutations with a known Food and Drug Administration (FDA)‐approved biomarker. The computational repurposing approach was validated by evaluating its ability to identify FDA‐approved biomarkers. The total repurposable genome was identified by evaluating all possible gene‐FDA drug‐approved combinations in the PM dataset.ResultsThe computational repurposing approach was accurate at identifying FDA therapies with known biomarkers (94%). Using next‐generation sequencing molecular reports (n = 94), a meaningful percentage of patients (14%) could have an off‐label therapeutic identified. The frequency of theoretical drug repurposing events in The Cancer Genome Atlas pan‐cancer dataset was 73% of the samples in the cohort.ConclusionA computational drug repurposing approach may assist in identifying novel repurposing events in cancer patients with no access to standard therapies. Further validation is needed to confirm a precision oncology approach using drug repurposing.
Synthesis and investigation of the cholinesterase inhibitory and antioxidant capacities of some novel N'‐(quinolin‐4‐ylmethylene)propanehydrazides against Alzheimer's disease
Authors: Kilic Burcu , Dogruer Deniz S. 
Published: April 2024

AbstractOne of the worst long‐term health issues of the past few decades is Alzheimer's disease (AD). Unfortunately, there are currently insufficient choices for treating and caring for AD, which makes it a popular subject for drug development research. Studies on the development of drugs for AD have primarily concentrated on the use of multitarget directed ligands. Following this strategy, we designed new ChE inhibitors with additional antioxidant and metal chelator effects. In this research, eight novel N’‐(quinolin‐4‐ylmethylene)propanehydrazide derivatives were synthesized and characterized. We then evaluated the inhibition potency of all the final compounds for cholinesterase enzymes. Among them, 4e (IC50 acetylcholinesterase [AChE] = 0.69 µM and butyrylcholinesterase [BChE]= 26.00 µM) and 4h (IC50's AChE= 7.04 µM and BChE= 16.06 µM) were found to be the most potent AChE and BChE inhibitors, respectively.
Substituted Pyrrole‐based Schiff Bases: Effect On The Luminescence Of Neutral Heteroleptic Cu(I) Complexes
Authors: Ferraro Valentina , Fuhr Olaf , Bizzarri Claudia , Bräse Stefan 
Published: April 2024

AbstractNeutral Cu(I) complexes containing Schiff bases as chelating N‐donors and phosphines as P‐donors were efficiently isolated and characterized. The imines were obtained by straightforward condensation between pyrrole‐2‐carboxaldehyde and the corresponding aromatic amines. The structure of most of the Cu(I) species was elucidated through single‐crystal X‐ray diffraction. The Cu(I) complexes exhibited intense absorptions around 400–500 nm ascribed to MLCT transitions. For some Cu(I) derivatives, orange‐ and red‐orange emissions were detected upon excitation with near‐UV and blue irradiation in the solid state. The emission maxima were red‐shifted once the benzothiazole‐derived Schiff base or Xantphos were employed as chelating ligands. Microsecond‐long lifetimes, wide emissions, and large Stokes shifts suggest the involvement of triplet states in the luminescent process. Electrochemical measurements and DFT calculations were employed to rationalize the photoluminescence properties, which were ascribed to mixed 3LC/3MLCT transitions.
Scanner: Simultaneously temporal trend and spatial cluster detection for spatial‐temporal data
Authors: Wang Xin , Zhang Xin 
Published: April 2024

AbstractIdentifying the underlying trajectory pattern in the spatial‐temporal data analysis is a fundamental but challenging task. In this paper, we study the problem of simultaneously identifying temporal trends and spatial clusters of spatial‐temporal trajectories. To achieve this goal, we propose a novel method named spatial clustered and sparse nonparametric regression (). Our method leverages the B‐spline model to fit the temporal data and penalty terms on spline coefficients to reveal the underlying spatial‐temporal patterns. In particular, our method estimates the model by solving a doubly‐penalized least square problem, in which we use a group sparse penalty for trend detection and a spanning tree‐based fusion penalty for spatial cluster recovery. We also develop an algorithm based on the alternating direction method of multipliers (ADMM) algorithm to efficiently minimize the penalized least square loss. The statistical consistency properties of estimator are established in our work. In the end, we conduct thorough numerical experiments to verify our theoretical findings and validate that our method outperforms the existing competitive approaches.
A novel friction model for steel‐polymer interfaces in sliding seismic isolation bearings
Authors: Messina Armando , Miranda Eduardo 
Published: April 2024

AbstractExperiments have shown that the coefficient of friction in steel‐polymer interfaces is influenced by several interacting parameters, making it difficult to accurately model the instantaneously varying coefficient of friction in sliding seismic isolation bearings. A new model to characterize the variation of the coefficient of friction in sliding seismic isolation bearings in which a thermoplastic slides relative to a steel surface is presented. The model takes into account the variation in the coefficient of friction produced by changes in sliding velocity, normal pressure, and stick‐slip phenomena, as well as degradation due to frictional heating. In particular, the model takes into account the differences in behavior during reversing and nonreversing stick‐slip transitions. It is shown that the proposed model is able to reproduce very well experimental results of friction tests using various steel‐polymer interfaces.