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Liver disease and erythropoietic protoporphyria:A concise review

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World Journal of Gastroenterology 2010年第36期16卷 4526-4531页

作者： María José Casanova-González María Trapero-Marugán e anthony jones Ricardo Moreno-Otero Department of the Gastroenterology and Hepatology Hospital Universitario de la Princesa and Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas(CIBEREHD)Madrid 28006Spain

The porphyries are a group of metabolic disorders characterized by deficiencies in the activity of enzymes involved in the biosynthesis of *** erythropoietic protoporphyria(ePP),in the majority of cases an autosomal dominant disease,there is a mutation of the gene that encodes ferrochelatase(FeCH).FeCH deficiency is associated with increased concentrations of protoporphyrin in erythrocytes,plasma,skin and *** prevalence of this inherited disorder oscillates between 1:75 000 and 1:200 *** manifestations of ePP appear in early infancy upon first exposure to the ***,approximately 5%-20% of patients with ePP develop liver *** of protoporphyrin in the liver is associated with cholestatic phenomena and oxidative stress that predisposes to hepatobiliary disease of varying degrees of severity,such as cholelithiasis,mild parenchymal liver disease,progressive hepatocellular disease with end-stage liver disease and acute liver *** damage is the major risk in ePP patients,so surveillance and frequent clinical and biochemical liver follow-up is *** diagnostic approach consists in detecting increased levels of protoporphyrin,decreased activity of FeCH and genetic analysis of the FeCH gene.A variety of nonsurgical therapeutic approaches have been adopted for the management of ePP associated with liver disease,but none of these has been shown to be unequivocally ***,some may have a place in preparing patients for liver *** transplantation does not correct the constitutional deficiency of ***,there is a risk of recurrence of liver disease after liver transplantation as a result of continuing overproduction of *** authors recommend that bone marrow transplantation should be considered in liver allograft recipients to prevent recurrence of hepatic disease.

关键词： erythropoietic protoporphyria Protoporphyrin Liver Ferrochelatase

Assessment of the Visual Analogue Score in the evaluation of the Pruritus of Cholestasis

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Journal of Clinical and Translational Hepatology 2017年第3期5卷 203-207页

作者： Nora V.Bergasa e.anthony jones NYC H + H Metropolitan New York NY USA New York Medical College Valhalla NY USA Department of Gastroenterology and Liver Diseases Academic Medical Center Amsterdam The Netherlands

Background and Aims:A visual analogue score (VAS),based on application of a visual analogue scale,has been widely used to assess pruritus in clinical studies of patients with cholestatic liver disease.A VAS is a numerical score of the severity of the perception of pruritus,and,hence,is inherently *** objective of this study was to assessthe reliability of a VAS as an index of pruritus in cholestatic ***:In 8 patients with chronic pruritus due to primary biliary cholangitis,values for a VAS of pruritus were compared with corresponding measurements of scratching activity,which were generated by a monitoring system specifically designed to quantitate this *** relationship between individual values for the VAS and corresponding values for scratching activity during a specific interval immediately preceding the recording of the VAS was examined by determining the Spearman's rank correlation ***:The mean Spearman's rank correlation coefficient between individual values for the VAS and corresponding mean values for scratching activity was 0.072;the range of these coefficients was-0.04 to 0.26.A VAS of pruritus is an unreliable index of scratching activity,and,hence,of the pathophysiological process responsible for the pruritus of ***:It is concluded that the use of a VAS as a primary quantitative endpoint in trials of the efficacy of potential therapies for the pruritus of cholestasis may be inappropriate.

关键词： Pruritus Cholestasis Visual analogue score Scratching activity Primary biliary cholangitis Quantitative efficacy endpoint

Music as medicine for traumatic brain injury:a perspective on future research directions

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Neural Regeneration Research 2024年第10期19卷 2105-2106页

作者： anthony e.Kline eleni H.Moschonas Corina O.Bondi Department of Physical Medicine&Rehabilitation University of PittsburghPittsburghPAUSA Safar Center for Resuscitation Research University of PittsburghPittsburghPAUSA Center for Neuroscience University of PittsburghPittsburghPAUSA Department of Critical Care Medicine University of PittsburghPittsburghPAUSA Center for the Neural Basis of Cognition University of PittsburghPittsburghPAUSA Department of Psychology University of PittsburghPittsburghPAUSA Department of Neurobiology University of PittsburghPittsburghPAUSA

Traumatic brain injury(TBI) impacts 69 million individuals globally each year and is a leading cause of death and disability(Dewan et al.,2019).The majority of moderate-to-severe TBI survivors endure long-lasting disturbances in motor,cognitive,and affect that negatively impacts their *** a plethora of research on pharmacological interventions for TBI has been conducted,none has translated to the clinic,thus advocating for the evaluation of nonpharmacological therapeutic approaches that may increase translational success.

关键词： injury traumatic

Dysregulated bidirectional epithelial-mesenchymal crosstalk:A core determinant of lung fibrosis progression

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Chinese Medical Journal Pulmonary and Critical Care Medicine 2024年第1期2卷 27-33页

作者： Liudi Yao Zijian Xu Donna e.Davies Mark G.jones Yihua Wang Biological Sciences Faculty of Environmental and Life SciencesUniversity of SouthamptonSouthampton SO171BJUK Clinical and experimental Sciences Faculty of MedicineUniversity of SouthamptonSouthampton SO166YDUK Institute for Life Sciences University of SouthamptonSouthampton SO171BJUK NIHR Southampton Biomedical Research Centre University Hospital SouthamptonSouthampton SO166YDUK

Progressive lung fibrosis is characterized by dysregulated extracellular matrix(eCM)***-ing of disease pathogenesis remains limited and has prevented the development of effective *** an abnormal wound-healing response is strongly implicated in lung fibrosis initiation,factors that determine why fi-brosis progresses rather than regular tissue repair occur are not fully *** human lung fibrosis,there is evidence of altered epithelial and mesenchymal populations as well as cells undergoing epithelial-mesenchymal transition(eMT),a dynamic and reversible biological process by which epithelial cells lose their cell polarity and down-regulate cadherin-mediated cell-cell adhesion to gain migratory *** review will focus on the role of eMT and dysregulated epithelial-mesenchymal crosstalk in progressive lung fibrosis.

关键词： Lung fibrosis epithelial-mesenchymal transition(eMT) Crosstalk

Lateral ring compression test applied to a small caliber steel jacket:Identification of a constitutive model

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Defence Technology（防务技术） 2024年第6期36卷 133-148页

作者： Yann Coget Yael Demarty Christophe Czarnota anthony Bracq Jean-Sebastien Brest Alexis Rusinek French-German Research Institute of Saint-Louis 5 Rue Du General Cassagnou68300 Saint-LouisFrance Laboratoire d'etude des Microstructures et de Mecanique des Materiaux(LeM3) CNRSUniversite de LorraineArts&Metiers ParisTechF-57000 MetzFrance

The evolution of threats and scenarios requires continuous performance improvements of ballistic protections for armed *** a modeling point of view,it is necessary to use sufficiently precise material behavior models to accurately describe the phenomena observed during the impact of a projectile on a protective *** this context,the goal of this paper is to characterize the behavior of a small caliber steel jacket by combining experimental and numerical *** experimental method is based on the lateral compression of ring specimens directly machined from the thin and small *** speeds and temperatures are considered in a quasi-static regime in order to reveal the strain rate and temperature dependencies of the tested *** Finite element Updating Method(FeMU)is *** results are coupled with an inverse optimization method and a finite element numerical model in order to determine the parameters of a constitutive model representative of the jacket *** of the present model are verified against experimental results and a parametric study as well as a discussion on the identified material parameters are *** results indicate that the strain hardening parameter can be neglected and the behavior of the thin steel jacket can be described by a modeling without strain hardening sensitivity.

关键词： Full metal jacket ammunition Lateral ring compression Inverse identification Numerical simulation

LKB1 depletion-mediated epithelial-mesenchymal transition induces fibroblast activation in lung fibrosis

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Genes & Diseases 2024年第3期11卷 417-426页

作者： Zijian Xu elizabeth R.Davies Liudi Yao Yilu Zhou Juanjuan Li Aiman Alzetani Ben G.Marshall David Hancock Tim Wallis Julian Downward Rob M.ewing Donna e.Davies Mark G.jones Yihua Wang Biological Sciences Faculty of Environmental and Life SciencesUniversity of SouthamptonSouthampton SO171BJUK clinical and experimental Sciences Faculty of MedicineUniversity of SouthamptonSouthampton SO166YDUK Institute for Life Sciences University of SouthamptonSouthampton SO171BJUK NIHR Southampton Biomedical Research Centre University Hospital SouthamptonSouthampton SO166YDUK University Hospital Southampton Southampton SO166YDUK Oncogene Biology The Francis Crick InstituteLondon NW11ATUK

The factors that determine fibrosis progression or normal tissue repair are largely *** previously demonstrated that autophagy inhibition-mediated epithelial-mesenchymal transition(eMT)in human alveolar epithelial type Il(ATIl)cells augments local myofibroblast differentiation in pulmonary fibrosis by paracrine ***,we report that liver kinase B1(LKB1)inactivation in ATIl cells inhibits autophagy and induces eMT as a *** IPF lungs,this is caused by the down-regulation of CAB39L,a key subunit within the LKB1 complex.3D co-cultures of ATIl cells and MRC5 lung fibroblasts coupled with RNA sequencing(RNA-seq)confirmed that paracrine signaling between LKB1-depleted ATIl cells and fibroblasts augmented myofibroblast ***,these data suggest that reduced autophagy caused by LKB1 inhibition can induce eMT in ATIl cells and contribute to fibrosis via aberrant epithelial-fibroblast crosstalk.

关键词： CAB39L Crosstalk eMT LKB1 Pulmonary fibrosis

Anti-N-methyl-D-aspartate receptor-associated encephalitis: A review of clinicopathologic hallmarks and multimodal imaging manifestations

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World Journal of Radiology 2024年第1期16卷 1-8页

作者： Bryce David Beutler Alastair e Moody Jerry Mathew Thomas Benjamin Phillip Sugar Mark B Ulanja Daniel Antwi-Amoabeng Lucas anthony Tsikitas Department of Radiology University of Southern CaliforniaKeck School of MedicineLos AngelesCA 90033United States Department of Anesthesiology University of UtahSalt Lake CityUT 84132United States Department of Internal Medicine Christus Ochsner St.Patrick HospitalLake CharlesLA 70601United States

Anti-N-methyl-D-aspartate receptor-associated encephalitis(NMDARe)is a rare immune-mediated neuroinflammatory condition characterized by the rapid onset of neuropsychiatric symptoms and autonomic *** mechanism of pathogenesis remains incompletely understood,but is thought to be related to antibodies targeting the GluN1 subunit of the NMDA receptor with resultant downstream dysregulation of dopaminergic *** adults are most frequently affected;the median age at diagnosis is 21 *** is a strong female predilection with a female sex predominance of 4:*** often develops as a paraneoplastic process and is most commonly associated with ovarian ***,NMDARe has also been described in patients with small cell lung cancer,clear cell renal carcinoma,and other benign and malignant *** is based on correlation of the clinical presentation,electro-encephalography,laboratory studies,and *** tomography,positron emission tomography,and magnetic resonance imaging are essential to identify an underlying tumor,exclude clinicopathologic mimics,and predict the likelihood of long-term functional *** imaging may be of value for prognostication and to assess the response to *** may involve high-dose corticosteroids,intravenous immunoglobulin,and plasma ***,we review the hallmark clinicopathologic features and imaging findings of this rare but potentially devastating condition and summarize diagnostic criteria,treatment regimens,and proposed pathogenetic mechanisms.

关键词： Anti-N-methyl-D-aspartate receptor-associated encephalitis Autoimmune encephalitis encephalitis Ovarian teratoma Paraneoplastic syndrome Teratoma

An alfalfa MYB-like transcriptional factor MsMYBH positively regulates alfalfa seedling drought resistance and undergoes MsWAV3-mediated degradation

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Journal of Integrative Plant Biology 2024年第4期66卷 683-699页

作者： Kun Shi Jia Liu Huan Liang Hongbin Dong Jinli Zhang Yuanhong Wei Le Zhou Shaopeng Wang Jiahao Zhu Mingshu Cao Chris S.jones Dongmei Ma Zan Wang College of Grassland Science and Technology China Agricultural UniversityBeijing 100193China Institute of Animal Sciences Chinese Academy of Agricultural SciencesBeijing 100193China AgResearch Grasslands Research Centre Palmerston North 4442New Zealand International Livestock Research Institute Nairobi 00100Kenya School of ecology and environment Ningxia UniversityYinchuan 750021China

Drought is a major threat to alfalfa(Medicago sativa L.)*** discovery of important alfalfa genes regulating drought response will facilitate breeding for drought-resistant alfalfa ***,we report a genome-wide association study of drought resistance in *** identified and functionally characterized an MYB-like transcription factor gene(MsMYBH),which increases the drought resistance in *** with the wild-types,the biomass and forage quality were enhanced in MsMYBH overexpressed *** RNA-seq,proteomics and chromatin immunoprecipitation analysis showed that MsMYBH can directly bind to the promoters of MsMCP1,MsMCP2,MsPRX1A and MsCARCAB to improve their *** outcomes of such interactions include better water balance,high photosynthetic efficiency and scavenge excess H_(2)O_(2)in response to ***,an e3 ubiquitin ligase(MsWAV3)was found to induce MsMYBH degradation under long-term drought,via the 26S proteasome ***,variable-number tandem repeats in MsMYBH promoter were characterized among a collection of germplasms,and the variation is associated with promoter ***,our findings shed light on the functions of MsMYBH and provide a pivotal gene that could be leveraged for breeding drought-resistant *** discovery also offers new insights into the mechanisms of drought resistance in alfalfa.

关键词： alfalfa drought resistance e3 ubiquitin ligase MsMYBH MsWAV3 MYB-like transcriptional factor

There is more to the knee joint than just the quadriceps:A systematic review with meta-analysis and evidence gap map of hamstring strength,flexibility,and morphology in individuals with gradual-onset knee disorders

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Journal of Sport and Health Science 2024年第4期13卷 521-536页

作者： Helder S.Lopes Marina C.Waiteman Liliam B.Priore Neal R.Glaviano David M.Bazett-jones Ronaldo V.Briani Fabio M.Azevedo School of Science and Technology Physical Therapy DepartmentSao Paulo State University(UNESP)Presidente Prudente 19060-900Brazil Department of Kinesiology College of AgricultureHealth and Natural ResourcesUniversity of ConnecticutStorrsCT 06269USA School of exercise and Rehabilitation Sciences College of Health and Human ServicesUniversity of ToledoToledoOH 43606USA

Background:Impairments in hamstring strength,flexibility,and morphology have been associated with altered knee biomechanics,pain,and *** the presence of these impairments in individuals with gradual-onset knee disorders is important and may indicate targets for assessment and *** systematic review aimed to synthesize the literature to determine the presence of impairments in hamstring strength,flexibility,and morphology in individuals with gradual-onset knee ***:Five databases(MeDLINe,embase,CINAHL,SPORTDiscus,and Web of Science)were searched from inception to September *** studies comparing hamstring outcomes(e.g.,strength,flexibility,and/or morphology)between individuals with gradual-onset knee disorders and their unaffected limbs or pain-free controls were ***-analyses for each knee disorder were ***-level certainty was assessed using the Grading of Recommendations Assessment,Development,and evaluation,and evidence gap maps were ***:Seventy-nine studies across 4 different gradual-onset knee disorders(i.e.,knee osteoarthritis(OA),patellofemoral pain(PFP),chondromalacia patellae,and patellar tendinopathy)were *** with knee OA presented with reduced hamstring strength compared to pain-free controls during isometric(standard mean difference(SMD)=-0.76,95%confidence interval(95%CI):-1.32 to-0.21)and concentric contractions(SMD=-0.97,95%CI:-1.49 to-0.45).Individuals with PFP presented with reduced hamstring strength compared to painfree controls during isometric(SMD=-0.48,95%CI:-0.82 to-0.14),concentric(SMD=-1.07,95%CI:-2.08 to-0.06),and eccentric contractions(SMD=-0.59,95%CI:-0.97 to-0.21).No differences were observed in individuals with patellar *** with PFP presented with reduced hamstring flexibility when compared to pain-free controls(SMD=-0.76,95%CI:-1.15 to-0.36).evidence gap maps identified insufficient evidence for chondromalacia patel

关键词： Chondromalacia patellae Knee flexors Knee osteoarthritis Patellar tendinopathy Patellofemoral pain