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A Case of Fahr’s Disease with Epilepsy as the First Symptom in Infancy

Case Reports in Clinical Medicine 2024年第4期13卷 101-107页

作者： Qicheng Qiao Xinlu Tan Qiubo Li Clinical Medicine College Jining Medical University Jining China departments of Pediatrics Affiliated Hospital of Jining Medical University Jining China

Background: Fahr’s disease, also recognized as Idiopathic Basal Ganglia Calcification (IBGC) or Primary Familial Brain Calcification (PFBC), was first identified by the German neurologist Karl Theodor Fahr in 1930. This rare condition, which involves the calcification of the basal ganglia and presents significant treatment challenges, is most commonly diagnosed in middle-aged adults and is notably uncommon in children. Purpose: We report a case of a younger patient and review this disease as an aid to early detection and diagnosis of the disease. Case Introduction: In this report, we present a unique case of Fahr’s disease in a child, where epilepsy manifested as the initial symptom during infancy. In this report, we present a case of Fahr’s disease in a child who presented with epilepsy as the first symptom in infancy. The child had no imaging abnormalities at the onset of the seizure, and subsequent antiepileptic drugs were reduced and discontinued, and when the seizure recurred 3 years later, a perfect cranial CT revealed symmetrical calcifications in the brain, which gradually worsened, and subsequently the child was unable to take care of himself and had regression of his psychomotor development, and the family requested discharge from the hospital, and then the child died during the follow-up visit. Conclusion: The disease is currently associated with a number of disorders for which there is no specific treatment, and half of all patients currently have a well-defined gene, emphasizing more importantly the importance of genetic counseling for parents known to be at risk prior to conception.

关键词： Fahr Primary Familial Brain Calcification Epilepsy

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Multimodal cardiac imaging assisted tumor characterization and surgical planning of a patient with rare primary cardiac paraganglioma

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Journal of Geriatric Cardiology 2024年第2期21卷 246-250页

作者： Shu-Yu MENG Li-Qun WANG Hao-Dan DANG Lin ZHANG Sheng-Li JIANG Bo-Han LIU Department of Ultrasound Diagnosis the First Medical CenterChinese PLA General HospitalBeijingChina Department of Nuclear Medicine the First Medical CenterChinese PLA General HospitalBeijingChina Department of Pathology the First Medical CenterChinese PLA General HospitalBeijingChina Department of Cardiovascular Surgery the First Medical CenterChinese PLA General HospitalBeijingChina

Paragangliomas,also known as pheochromocytomas(1–9 cases per million),arise in the paraganglia.[1]Pheochromocytomas occur in the adrenal glands,while paragangliomas occur elsewhere.[2]Paragangliomas originate from paraganglion cells,which are derived from the neural ectoderm of the nerves and migrate along both sides of the median axis from the base of the skull to the pelvis during embryonic development.

关键词： glioma cardiac nerves

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Glial response in the midcingulate cortex in Huntington’s disease

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Neural Regeneration Research 2025年第1期20卷 207-208页

作者： Thulani H.Palpagama Andrea Kwakowsky Center for Brain Research and Department of Anatomy and medical imaging Faculty of Medical and Health SciencesUniversity of AucklandAucklandNew Zealand Pharmacology and Therapeutics School of MedicineGalway Neuroscience CenterUniversity of GalwayGalwayIreland

Huntington’s disease(HD)is a genetic disease characterized by the progressive degeneration of the striatum and *** can present with a variety of symptoms that can broadly be classified into motor symptoms,inclusive of choreatic movements and rigidity,mood and psychiatric symptoms,such as depression and apathy,and cognitive symptoms,such as cognitive *** causal mutation underlying HD results from an expansion of a CAG repeat sequence on the IT15 gene,resulting in the formation and accumulation of a mutant huntingtin protein.

关键词： Huntington degeneration cortex

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Two Monte Carlo-based simulators for imaging-system modeling and projection simulation of flat-panel X-ray source

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Nuclear Science and Techniques 2024年第7期35卷 31-46页

作者： Meng-Ke Qi Ting He Yi-Wen Zhou Jing Kang Zeng-Xiang Pan Song Kang Wang-Jiang Wu Jun Chen Ling-Hong Zhou Yuan Xu School of Biomedical Engineering Southern Medical UniversityGuangzhou510515China State Key Laboratory of Optoelectronic Materials and Technologies Guangdong Province Key Laboratory of Display Material and TechnologySchool of Electronics and Information TechnologySun Yat-sen UniversityGuangzhou510275China Department of Radiotherapy Guangdong Provincial People’s Hospital(Guangdong Academy of Medical Sciences)Southern Medical UniversityGuangzhou510515China

The advantages of a flat-panel X-ray source(FPXS)make it a promising candidate for imaging *** imaging-system modeling and projection simulation are critical for analyzing imaging performance and resolving overlapping projection issues in *** conventional analytical ray-tracing approach is limited by the number of patterns and is not applicable to FPXS-projection ***,the computation time of Monte Carlo(MC)simulation is independent of the size of the patterned arrays in *** study proposes two high-efficiency MC projection simulators for FPXS:a graphics processing unit(GPU)-based phase-space sampling MC(gPSMC)simulator and GPU-based fluence sampling MC(gFSMC)*** two simulators comprise three components:imaging-system modeling,photon initialization,and physical-interaction simulations in the ***-system modeling was performed by modeling the FPXS,imaging geometry,and *** gPSMC simulator samples the initial photons from the phase space,whereas the gFSMC simulator performs photon initialization from the calculated energy spectrum and fluence *** entire process of photon interaction with the geometry and arrival at the detector was simulated in parallel using multiple GPU kernels,and projections based on the two simulators were *** accuracies of the two simulators were evaluated by comparing them with the conventional analytical ray-tracing approach and acquired projections,and the efficiencies were evaluated by comparing the computation *** results of simulated and realistic experiments illustrate the accuracy and efficiency of the proposed gPSMC and gFSMC simulators in the projection calculation of various phantoms.

关键词： Flat-panel X-ray source(FPXS) imaging-system modeling Projection simulation Monte Carlo-based simulators

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Enhanced optical imaging and fluorescent labeling for visualizing drug molecules within living organisms

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Acta Pharmaceutica Sinica B 2024年第6期14卷 2428-2446页

作者： Ting Sun Huanxin Zhao Luyao Hu Xintian Shao Zhiyuan Lu Yuli Wang Peixue Ling Yubo Li Kewu Zeng Qixin Chen School of Pharmaceutical Sciences National Key Laboratory of Advanced Drug Delivery SystemMedical Science and Technology Innovation CenterShandong First Medical University&Shandong Academy of Medical SciencesJinan 250062China departments of Diagnostic Radiology SurgeryChemical and Biomolecular Engineeringand Biomedical EngineeringYong Loo Lin School of Medicine and College of Design and EngineeringNational University of SingaporeSingapore 119074Singapore Institute of Biochemical and Biotechnological Drugs School of Pharmaceutical SciencesCheeloo College of MedicineShandong UniversityJinan 250012China Key Laboratory of Biopharmaceuticals Postdoctoral Scientific Research WorkstationShandong Academy of Pharmaceutical ScienceJinan 250098China State Key Laboratory of Natural and Biomimetic Drugs School of Pharmaceutical SciencesPeking UniversityBeijing 100191China School of Life Sciences Science and Technology Innovation CenterShandong First Medical University&Shandong Academy of Medical SciencesJinan 250062China School of Chinese Materia Medica Tianjin University of Traditional Chinese MedicineTianjin 301617China Tianjin Pharmaceutical DA REN TANG Group Corporation Limited Traditional Chinese Pharmacy Research Institute Tianjin 300457China Key Laboratory of Systems Bioengineering(Ministry of Education) School of Chemistry Engineering and TechnologyTianjin UniversityTianjin 300072China

The visualization of drugs in living systems has become key techniques in modern *** advancements in optical imaging technologies and molecular design strategies have revolutionized drug *** the subcellular level,super-resolution microscopy has allowed exploration of the molecular landscape within individual cells and the cellular response to *** beyond subcellular imaging,researchers have integrated multiple modes,like optical near-infrared II imaging,to study the complex spatiotemporal interactions between drugs and their *** combining these visualization approaches,researchers gain supplementary information on physiological parameters,metabolic activity,and tissue composition,leading to a comprehensive understanding of drug *** review focuses on cutting-edge technologies in drug visualization,particularly fluorescence imaging,and the main types of fluorescent molecules ***,we discuss current challenges and prospects in targeted drug research,emphasizing the importance of multidisciplinary cooperation in advancing drug *** the integration of advanced imaging technology and molecular design,drug visualization has the potential to redefine our understanding of pharmacology,enabling the analysis of drug micro-dynamics in subcellular environments from new perspectives and deepening pharmacological research to the levels of the cell and organelles.

关键词： Optical imaging Drug visualization Fluorophore labeling Therapeutics

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Dual neovascular targets of vascular endothelial growth factor receptors and platelet-derived growth factor receptor ameliorate thioacetamide induced liver fibrosis in rats

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Portal Hypertension & Cirrhosis 2024年第1期3卷 1-13页

作者： Bin Xiong Yaowei Bai Jiacheng Liu Tongqiang Li Yingliang Wang Chen Zhou Department of Interventional Radiology The First Affiliated Hospital of Guangzhou Medical UniversityGuangzhouGuangdongChina Department of Radiology Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhanHubeiChina Hubei Province Key Laboratory of Molecular imaging WuhanHubeiChina

Aims:Neovascularization plays a crucial role in liver fibrosis(LF),and blocking vascular endothelial growth factor receptors(VEGFR)has been shown to improve *** aim of our study was to investigate the role of dual neovascularization targets,VEGFR,and platelet-derived growth factor receptor(PDGFR),in ameliorating ***:In vitro,we observed the effects of apatinib(APA)(a VEGFR inhibitor)and donafenib(DON)(a VEGFR and PDGFR inhibitor)on the activation,proliferation,and apoptosis of hepatic stellate cells(HSCs)from rats and *** vivo,we established a thioacetamide(TAA)-induced liver fibrosis rat model to explore the antifibrosis effect of APA and *** used the method of random table to randomly divide the rats into 4 *** detected the expression of angiogenesis-related proteins using Western blot and ***:APA and DON inhibited the proliferation and activation of HSCs,promoted apoptosis of HSCs,and arrested the S phase of the cell cycle in *** also found that DON had a stronger inhibitory effect on *** vivo,APA and DON ameliorated liver fibrosis,reduced collagen deposition andα-SMA expression in rats,and DON had a stronger improvement *** and DON downregulated the expression of VEGFR2 while inhibiting the phosphorylation of Akt and ERK1/*** can act through both VEGF and PDGF pathways,whereas APA can only act through the VEGF ***:Antiangiogenesis is a promising approach for the treatment of *** with a single-target drug(APA),the dual-target drug(DON)can achieve better therapeutic effects.

关键词： antiangiogenesis apatinib donafenib fibrosis

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Neuroimaging features in a patient with non-ketotic hyperglycaemic seizures: A case report

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World Journal of Clinical Cases 2024年第5期12卷 1004-1009页

作者： Jing Wu Huijie Feng Yaxiong Zhao Junfeng Li Ting Li Kefeng Li Department of Radiology Heping Hospital Affiliated to Changzhi Medical CollegeChangzhi 046000Shanxi ProvinceChina Changzhi Key Lab of Functional imaging for Brain Diseases Heping Hospital Affiliated to Changzhi Medical CollegeChangzhi 046000Shanxi ProvinceChina Faculty of Applied Sciences Macao Polytechnic UniversityMacao 999078China

BACKGROUND Non-ketotic hyperglycaemic(NKH)seizures are a rare neurological complication of diabetes caused by hyperglycaemia in non-ketotic and non-hyperosmotic *** clinical characteristics of NKH seizures are atypical and lack unified diagnostic criteria,leading to potential misdiagnoses in the early stages of the *** SUMMARY This report presents a rare case of NKH seizures in a 52-year-old male patient with a history of type 2 diabetes *** performed comprehensive magnetic resonance imaging(MRI)studies at admission,12 d post-admission,and 20 d *** imaging techniques included contrast-enhanced head MRI,T2-weighted imaging(T2WI),fluid-attenuated inversion recovery(FLAIR),diffusion-weighted imaging,susceptibility-weighted imaging,magnetic reso-nance spectroscopy(MRS),and magnetic resonance *** the time of admission,T2WI and FLAIR of the cranial MRI showed that the left parieto-occipital cortex had gyrus-like swelling and high signal,and subcortical stripes had low *** showed a reduced N-acetylaspartate peak and increased creatine and choline peaks in the affected areas.A follow-up MRI 20 d later showed that the swelling and high signal of the left parieto-occipital cortex had disappeared,and the low signal of the subcortex had *** This case study provides valuable insights into the potential pathogenesis,diagnosis,and treatment of NKH *** comprehensive MRI findings highlight the potential utility of various MRI sequences in diagnosing and characterizing NKH seizures.

关键词： Non-ketotic hyperglycaemia seizures Magnetic resonance imaging Magnetic resonance spectroscopy Diabetes Case report

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Orientation Bias of Self-Assembled Alkanes and Their Derivatives Under Dynamic Lateral Nanoconfinement Conditions

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CCS Chemistry 2024年第4期6卷 1024-1033页

作者： Conditions Lihua Yu Yuanzhi Xia Tianze Hu Brent Daelemans Steven De Feyter Department of Chemistry Division of Molecular Imaging and PhotonicsKU LeuvenB-3001 Leuven

Self-assembled molecular networks(SAMNs)formed on crystalline solid substrates result from the spontaneous arrangement of individual molecules into structured *** interaction between molecules and the substrate leads to a nonrandom orientation of SAMNs with respect to the substrate’s *** the case of graphite,the sixfold rotational symmetry of the substrate results in an equivalent rotational degeneracy in the orientation of *** this study,we explored how nanoconfinement,that is,the restriction of SAMN formation to nanometersized areas,known as nanocorrals,could impact and potentially alleviate the orientational degeneracy of SAMNs,with emphasis on the influence of molecule symmetry,size,and intermolecular *** this end,we investigated the orientation distribution of n-alkanes and functionalized alkanes within nanocorrals,which were fabricated using scanning tunneling microscopy lithography(nanoshaving).We observed lifting of orientational degeneracy for long alkanes,contrary to shorter alkanes,which did not exhibit this orientation ***,functionalized alkanes with hydrogen bonding groups demonstrated preferential alignment regardless of length,highlighting the significance of stabilizing intermolecular interactions in the alignment *** study discussed the underlying principles of the molecular packing process and shed light on the complexity of molecular self-assembly in nanoconfined spaces.

关键词： self-assembly confinement condition nanocorral orientation bias scanning tunneling microscopy

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Contribution of Computed Tomography to the Diagnosis of Urinary Lithiasis in a West African University Hospital

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Open Journal of Radiology 2024年第2期14卷 25-32页

作者： Some Milckisédek Judicaël Marouruana Tankoano Aïda Ida Ouedraogo Pakisba Ali Tonde Kouka François Dassis Kindo Bassirou Zanga Soré Moussa Ouedraogo Nina-Astrid Bamouni Yomboé Abel Department of medical imaging Ouahigouya Regional University Hospital University of Ouahigouya Ouahigouya Burkina Faso Department of medical imaging Souro Sanou University Hospital Nazi Boni University Bobo Dioulasso Burkina Faso Department of medical imaging Charles de Gaulle University Paediatric Hospital Joseph Ki Zerbo University Ouagadougou Burkina Faso Department of medical imaging Bogodogo University Hospital Joseph Ki Zerbo University Ouagadougou Burkina Faso Workers Health Office Joseph Ki Zerbo University Ouagadougou Burkina Faso

Introduction: Urinary lithiasis is defining as a condition characterized by the formation of concretions in the kidneys or urinary excretory tract. We aimed to study professional practice in CT urinary lithiasis at CHUYO. Materials and method: This was a descriptive cross-sectional study with retrospective data collection covering 3 years from January 2017 to December 2019. Results: In general, it was noted that CT scans in our environment provide all the elements needed by urologists to diagnose urinary lithiasis and its impact on the urinary system. However, there are shortcomings, particularly in terms of the accuracy of the shape and density of the stones in the CT scan reports, which does not make it easier for urologists to decide on treatment. Conclusion: Computed tomography plays a major role in the diagnosis and therapeutic management of urinary lithiasis, and its use needs to be improved in our context.

关键词： Computed Tomography Urinary Lithiasis Diagnosis CHUYO

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Development and validation of a nomogram for predicting in-hospital mortality of intensive care unit patients with liver cirrhosis

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World Journal of Hepatology 2024年第4期16卷 625-639页

作者： Xiao-Wei Tang Wen-Sen Ren Shu Huang Kang Zou Huan Xu Xiao-Min Shi Wei Zhang Lei Shi Mu-Han Lü Department of Gastroenterology The Affiliated Hospital of Southwest Medical UniversityLuzhou 646099Sichuan ProvinceChina Nuclear Medicine and Molecular imaging Key Laboratory The Affiliated Hospital of Southwest Medical UniversityLuzhou 646099Sichuan ProvinceChina Department of Gastroenterology Lianshui People’Hospital of Kangda College Affiliated to Nanjing Medical UniversityHuaian 223499Jiangsu ProvinceChina

BACKGROUND Liver cirrhosis patients admitted to intensive care unit(ICU)have a high mortality *** To establish and validate a nomogram for predicting in-hospital mortality of ICU patients with liver *** We extracted demographic,etiological,vital sign,laboratory test,comorbidity,complication,treatment,and severity score data of liver cirrhosis patients from the medical Information Mart for Intensive Care IV(MIMIC-IV)and electronic ICU(eICU)collaborative research database(eICU-CRD).Predictor selection and model building were based on the MIMIC-IV *** variables selected through least absolute shrinkage and selection operator analysis were further screened through multivariate regression analysis to obtain final *** final predictors were included in the multivariate logistic regression model,which was used to construct a ***,we conducted external validation using the *** area under the receiver operating characteristic curve(AUC),decision curve,and calibration curve were used to assess the efficacy of the *** Risk factors,including the mean respiratory rate,mean systolic blood pressure,mean heart rate,white blood cells,international normalized ratio,total bilirubin,age,invasive ventilation,vasopressor use,maximum stage of acute kidney injury,and sequential organ failure assessment score,were included in the multivariate logistic *** model achieved AUCs of 0.864 and 0.808 in the MIMIC-IV and eICU-CRD databases,*** calibration curve also confirmed the predictive ability of the model,while the decision curve confirmed its clinical *** The nomogram has high accuracy in predicting in-hospital *** the included predictors may help improve the prognosis of patients.

关键词： Liver cirrhosis Intensive care unit Nomogram Predicting model Mortality

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