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Characteristics of patients with Wilson disease in the United States: An insurance claims database study

World Journal of Hepatology 2024年第5期16卷 791-799页

作者： Thomas Daniel-robin Pradeep Kumar Bernard Benichou Jean-Philippe Combal Vivet Therapeutics Paris 75008France Clarivate Noida 201301Uttar PradeshIndia

BACKGROUND Wilson disease(WD)is a progressive,potentially fatal degenerative disease affecting the liver and central nervous *** its low prevalence,collecting data on large cohorts of patients with WD is *** insur-ance claims databases provide powerful tools to collect retrospective data on large numbers of patients with rare *** To describe patients with WD in the United States,their treatment and clinical outcome,using a large insurance claims *** This retrospective,longitudinal study was performed in the Clarivate Real-World Data Product *** patients with≥2 claims associated with an Interna-tional Classification of Diseases 10(ICD-10)diagnostic code for WD(e83.01)between 2016 and 2021 were included and followed until death or study *** were divided into two groups by whether or not they were documented to have received a specific treatment for *** manifestations,hospital-isations,liver transplantation and death were *** Overall,5376 patients with an ICD-10 diagnostic code for WD were *** mean age at inclusion was 41.2 years and 52.0%were men.A specific WD treatment was documented for 885 patients(15.1%),although the number of patients taking zinc salts may be underestimated due to over the counter *** inclusion,the mean age of patients with a documented treatment was 36.6±17.8 years vs 42.2±19.6 years in those without a documented *** follow-up,273 patients(5.1%)*** with the American general population,the standardised mortality ratio was *** proportion of patients with a documented WD-specific treatment who died during follow-up was 4.0%and the mean age at death 52.7 *** Patients treated for WD in the United States had an excess early mortality compared with the American *** findings indicate that there is a significant unmet need for effective treatment for WD in the United States.

关键词： Wilson disease Mortality Treatment Copper chelators Zinc acetate United States

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effect of the Dietary Substitution of Fish Meal with Achatina fulica Meat Meal on the Growth Performance and Production Cost of African Catfish (Clarias gariepinus) Fingerlings

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Open Journal of Animal Sciences 2024年第2期14卷 123-136页

作者： Divine ewane Akeson Akeh Andoh Fidelis Narika Ambeno Bertha Anyizi Nkemnyi Mbeng Ashu Arrey Benedicta Oshuware Oben Pius mbu Oben Department of Animal Science Faculty of Agriculture and Veterinary Medicine University of Buea Buea Cameroon Department of Fisheries and Aquatic Resources Management Faculty of Agriculture and Veterinary Medicine University of Buea Buea Cameroon Department of Veterinary Medicine Faculty of Agriculture and Veterinary Medicine University of Buea Buea Cameroon

Fishmeal is the most preferred source of protein in aquafeeds, but it is expensive and scarce. Hence, Achatina fulica meat meal (AFM), which is much less preferred for human consumption out of three species of African giant land snails, was tested as a fishmeal substitute for Clarias gariepinus growth. Five iso-nitrogenous and iso-calorific diets were formulated, in which AFM substituted fish meal at 0% (control or Diet A), 25% (Diet B), 50% (Diet C), 75% (Diet D) and 100% (Diet e). These dietary treatments were each replicated thrice in a completely randomized design experiment, using 36-L plastic tanks in which the fish were fed daily rations corresponding to 5% of their body weight, for 8 weeks. Water quality parameters in the tanks were monitored. Proximate analyses were conducted on the fish meal, snail meal and experimental diets before the feeding trials. Cost-benefit analysis of the different diets was performed. The crude protein content of AFM (69.18%) was significantly higher than that of fish meal (55.81%). There was no significant difference (P > 0.05) in the mean weight gain, specific growth rate, feed conversion ratio, protein efficiency ratio and survival rate in fish fed Diet A and Diet B. The best protein efficiency ratio (0.77) was recorded in fish fed Diet B. Furthermore, the survival rate of fish increased with increased levels of AFM substitution. Water quality parameters were within a suitable range for tropical fish culture, indicating that the AFM did not pollute the water. The fish fed 25% AFM diet significantly (P Clarias gariepinus diets at a 25% substitution level. The aquaculture industry can thus exploit the availability of this feed resource.

关键词： Fish Meal Achatina fulica Meat Meal Aquafeeds Clarias gariepinus

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Genome-wide Studies Reveal Genetic Risk Factors for Hepatic Fat Content

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Genomics, Proteomics & Bioinformatics 2024年第2期22卷 57-68页

作者： Yanni Li eline H.van den Berg Alexander Kurilshikov Dasha V.Zhernakova Ranko Gacesa Shixian Hu esteban A.Lopera-Maya Alexandra Zhernakova Lifelines Cohort Study Vincent e.de Meijer Serena Sanna robin P.F.Dullaart Hans Blokzijl eleonora A.M.Festen Jingyuan Fu Rinse K.Weersma Department of Gastroenterology and Hepatology University of GroningenUniversity Medical Center GroningenGroningen 9713 GZThe Netherlands Department of Genetics University of GroningenUniversity Medical Center GroningenGroningen 9713 GZThe Netherlands Department of Gastroenterology and Hepatology Tianjin Medical University General HospitalTianjin Medical UniversityTianjin 300052China Laboratory of Genomic Diversity Center for Computer TechnologiesITMO UniversitySaint Petersburg 199034Russia Institute of Precision Medicine the First Affiliated HospitalSun Yat-sen UniversityGuangzhou 510080China Department of Surgery Section of Hepatobiliary Surgery and Liver TransplantationUniversity of GroningenUniversity Medical Center GroningenGroningen 9713 GZThe Netherlands Department of endocrinology University of GroningenUniversity Medical Center GroningenGroningen 9713 GZThe Netherlands Department of Pediatrics University of GroningenUniversity Medical Center GroningenGroningen 9713 GZThe Netherlands 不详

Genetic susceptibility to metabolic associated fatty liver disease(MAFLD)is complex and poorly *** characterization of the genetic background of hepatic fat content would provide insights into disease etiology and causality of risk *** performed genome-wide association study(GWAS)on two noninvasive definitions of hepatic fat content:magnetic resonance imaging proton density fat fraction(MRI-PDFF)in 16,050 participants and fatty liver index(FLI)in 388,701 participants from the United Kingdom(UK)Biobank(UKBB).Heritability,genetic overlap,and similarity between hepatic fat content phenotypes were analyzed,and replicated in 10,398 participants from the University Medical Center Groningen(UMCG)Genetics Lifelines Initiative(UGLI).Meta-analysis of GWASs of MRI-PDFF in UKBB revealed five statistically significant loci,including two novel genomic loci harboring CReB3L1(rs72910057-T,P=5.40e−09)and GCM1(rs1491489378-T,P=3.16e−09),respectively,as well as three previously reported loci:PNPLA3,TM6SF2,and *** of FLI in UKBB identified 196 genome-wide significant loci,of which 49 were replicated in UGLI,with top signals in ZPR1(P=3.35e−13)and FTO(P=2.11e−09).Statistically significant genetic correlation(rg)between MRI-PDFF(UKBB)and FLI(UGLI)GWAS results was found(rg=0.5276,P=1.45e−03).Novel MRI-PDFF genetic signals(CReB3L1 and GCM1)were replicated in the FLI *** identified two novel genes for MRI-PDFF and 49 replicable loci for *** a difference in hepatic fat content assessment between MRI-PDFF and FLI,a substantial similar genetic architecture was *** is identified as an easy and reliable approach to study hepatic fat content at the population level.

关键词： Hepatic fat content MAFLD Genome-wide association study Fatty liver index Magnetic resonance imaging proton density fat fraction

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A study on wildfire impacts on greenhouse gas emissions and regional air quality in South of Orléans,France

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Journal of environmental Sciences 2024年第1期135卷 521-533页

作者： Chaoyang Xue Gisèle Krysztofiak Yangang Ren Min Cai Patrick Mercier Frédéric Le Fur Corinne robin Benoit Grosselin Véronique Daele Max RMcGillen Yujing Mu Valéry Catoire Abdelwahid Mellouki Laboratoire de Physique et Chimie de l’environnement et de l’espace(LPC2e) CNRS–UniversitéOrléans–CNES(UMR 7328)Orléans Cedex 245071France Institut de Combustion AérothermiqueRéactivitéEnvironnement(ICARE)CNRSOrléans Cedex 245071France Research Centre for eco-environmental Sciences Chinese Academy of SciencesBeijing 100085China Lig’Air-Association de surveillance de la qualitéde l’air en région Centre-Val de Loire Saint-Cyr-en-Val 45590France Mohammed V University RabatMorocco

Wildfire events are increasing globally which may be partly associated with climate change,resulting in significant adverse impacts on local,regional air quality and global *** September 2020,a small wildfire(burned area:36.3 ha)event occurred in Souesmes(Loiret-Cher,Sologne,France),and its plume spread out over 200 km on the following day as observed by the MODIS *** on measurements at a suburban site(~50 km northwest of the fire location)in Orléans and backward trajectory analysis,young wildfire plumes were *** increases in gaseous pollutants(CO,CH_(4),N_(2)O,VOCs,etc.)and particles(including black carbon)were found within the wildfire plumes,leading to a reduced air *** factors,defined as eF(X)=ΔX/ΔCO(where,X represents the target species),of various trace gases and black carbon within the young wildfire plumes were determined accordingly and compared with previous *** in the ambient ions(such as ammonium,sulfate,nitrate,chloride,and nitrite in the particle-and gasphase)and aerosol properties(e.g.,aerosol water content,aerosol p H)were also quantified and ***,we estimated the total carbon and climate-related species(e.g.,CO_(2),CH_(4),N_(2)O,and BC)emissions and compared them with fire emission *** biomass burning emission inventories have uncertainties in estimating small fire burned areas and *** instance,we found that the Global Fire Assimilation System(GFAS)may underestimate emissions(e.g.,CO)of this small wildfire while other inventories(GFeD and FINN)showed significant *** that it is the first time to record wildfire plumes in this region,related atmospheric implications are presented and discussed.

关键词： Wildfire Air quality emission factor emission inventory VOLTAIRe supersite

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2023: Weather and Climate extremes Hitting the Globe with emerging Features

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Advances in Atmospheric Sciences 2024年第6期41卷 1001-1016页

作者： Wenxia ZHANG robin CLARK Tianjun ZHOU Laurent LI Chao LI Juan RIVeRA Lixia ZHANG Kexin GUI Tingyu ZHANG Lan LI Rongyun PAN Yongjun CHeN Shijie TANG Xin HUANG Shuai HU State Key Laboratory of Numerical Modeling for Atmospheric Sciences and Geophysical Fluid Dynamics Institute of Atmospheric PhysicsChinese Academy of SciencesBeijing 100029China Met Office Hadley Centre Exeter EX13PBUK University of Chinese Academy of Sciences Beijing 100049China Laboratoire de Météorologie Dynamique CNRSSorbonne UniversitéEcole Normale SupérieureEcole PolytechniqueParis 75230France Max Planck Institute for Meteorology Hamburg 20146Germany Instituto Argentino de Nivología Glaciología Y Ciencias Ambientales(IANIGLA)CCT CONICETMendoza CP 5500Argentina Shanghai Typhoon Institute China Meteorological AdministrationShanghai 200030China Asia-Pacific Typhoon Collaborative Research Center Shanghai 201306China

Globally,2023 was the warmest observed year on record since at least 1850 and,according to proxy evidence,possibly of the past 100000 *** in recent years,the record warmth has again been accompanied with yet more extreme weather and climate events throughout the ***,we provide an overview of those of 2023,with details and key background causes to help build upon our understanding of the roles of internal climate variability and anthropogenic climate *** also highlight emerging features associated with some of these extreme *** extremes are occurring earlier in the year,and increasingly simultaneously in differing parts of the world(e.g.,the concurrent hot extremes in the Northern Hemisphere in July 2023).Intense cyclones are exacerbating precipitation extremes(e.g.,the North China flooding in July and the Libya flooding in September).Droughts in some regions(e.g.,California and the Horn of Africa)have transitioned into flood *** extremes also show increasing interactions with ecosystems via wildfires(e.g.,those in Hawaii in August and in Canada from spring to autumn 2023)and sandstorms(e.g.,those in Mongolia in April 2023).Finally,we also consider the challenges to research that these emerging characteristics present for the strategy and practice of adaptation.

关键词： weather and climate extremes temperature extremes extreme precipitation drought wildfires

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中国手斧技术演化:新进展和未来展望

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Science Bulletin 2024年第14期69卷 2161-2165页

作者：李浩王社江 Matt G.Lotter robin Dennell Kathleen Kuman 陈发虎 State Key Laboratory of Tibetan Plateau earth System Resources and Environment(TPESER)Institute of Tibetan Plateau ResearchChinese Academy of SciencesBeijing 100101China Key Laboratory of Vertebrate evolution and Human Origins Institute of Vertebrate Paleontology and PaleoanthropologyChinese Academy of SciencesBeijing 100044China Palaeo-Research Institute University of JohannesburgAuckland Park 2006South Africa Department of Archaeology University of ExeterExeter EX44QEUK School of Geography Archaeology and Environmental StudiesUniversity of the WitwatersrandJohannesburg WITS 2050South Africa

Handaxes are often regarded as the"tool-type-specimenoof the Acheulean techno-complex,not only because they are a very common artefact but because they contain rich technological,behavioral,and cognitive data that have been used to highlight trends in human evolution and lithic production *** this end,the handaxe is the earliest tool possessing consistent morphological features,in particular,the appearance of a symmetrical concept in handaxe shape when compared with other Acheulean large shaping tools(e.g.,cleavers),and their manufacture is widely regarded as a significant milestone in the evolution of lithic technology,indicating improvements in tool-making hominins’abilities for complex thought and cognition.

关键词：技术演化未来展望 earliest

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The emerging roles of transplanted radial glial cells in regenerating the central nervous system

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Neural Regeneration Research 2015年第10期10卷 1548-1551页

作者： robin e.White Denis S.Barry Biology Department Westfield State University Department of Anatomy Trinity Biomedical Sciences Institute Trinity College Dublin University of Dublin

Scientists conclude that a combination of treatments involving rehabilitation,drug delivery,surgery and cell transplantation are necessary to achieve significant progress in regenerating the injured central nervous sy... 详细信息

关键词： cell The emerging roles of transplanted radial glial cells in regenerating the central nervous system

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effect of Different Mulch Materials on Yield and Nutrition Profile of Common Capsicum (Capsicum annuum) Cultivars in Bangladesh

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Agricultural Sciences 2024年第2期15卷 246-255页

作者： Prince Biswas Md. Abubakar Siddik Md. Shariful Islam Mohammad Zahir Ullah Md. Shamsuzzoha Hasina Akter Akm Maksudul Alam Mominul Hauque robin Nutrition Analysis Division Bangladesh Institute of Research and Training on Applied Nutrition Araihazar Bangladesh Department of Crop Botany Faculty of Agriculture Habiganj Agricultural University Habiganj Bangladesh Field Crop Division Bangladesh Institute of Research and Training on Applied Nutrition Araihazar Bangladesh Medicine Crop Wing Bangladesh Institute of Research and Training on Applied Nutrition Araihazar Bangladesh Department of Statistics University of Dhaka Dhaka Bangladesh Department of Agricultural extension Upazila Agriculture Office Habiganj Bangladesh Department of Agricultural Chemistry Sher-e-Bangla Agricultural University Dhaka Bangladesh

Capsicum is a nutritious vegetable and its cultivation in farms is getting popular in Bangladesh. Although many efforts have lain to explore better yielding and nutritionally rich cultivars with suitable modern cultivation techniques but still have to find the desired outcome. Thus, it’s necessary to conduct further research to identify the high-yielding and nutritious capsicum cultivars in Bangladesh. An experiment was conducted from July 2021 to June 2022 at the Bangladesh Institute of Research and Training on Applied Nutrition (BIRTAN) research field with three cultivars of capsicum: B0 = California Wonder, B1 = BARI Misti Morich-1 and B2 = BARI Misti Morich-2 and three mulching: T0 = No mulching, T1 = Water hyacinth, T2 = Poly Mulching in randomized complete block design with three replications to identify better quality capsicum cultivar and suitable mulching material. Among cultivars the BARI Misti Morich-2 (B2) showed increased agronomic parameters like number of branches and effective branches per plant, leaves length and width, consequently yield and yield contributing traits were also enhanced like fruits per plant, fruit length, fruit diameter and yield per plant (25.97%, 4.54%, 3.64% and 21.43%, respectively). Poly Mulching (T2) increased agronomic traits, yield traits and yield (0.61 kg) than BARI Misti Morich-1 (T1). The combined effect of B2T2 increased the number of branches per plant, effective branches per plant, leaves length and breadth by 40%, 90%, 15.57% and 26.22%, respectively, hence resulting in an increased yield of 20%. BARI Misti Morich-2 cultivar showed an increase in Fe, Zn and Vitamin-C content of 26.24% and 23.10%, 8.82% and 5.14%, and 6.03% and 5.74% than B0 and B1 cultivars, respectively. Therefore, BARI Misti Morich-2 exhibited the improved agronomic, yield and nutritional traits of capsicum under poly mulching a

关键词： Capsicum Cultivars Mulch Materials Yield Yield Contributing Traits Nutritional Quality

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Host-environment mismatches associated with subalpine fir decline in Colorado

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Journal of Forestry Research 2016年第5期27卷 1177-1189页

作者： robin M. Reich John e. Lundquist Kristina Hughes Colorado State University Fort Collins CO USA U.S. Forest Service Anchorage Anchorage AK 99501 USA

Subalpine fir decline （SFD） has killed more trees in Colorado＇s high elevation forests than any other insect or disease problem. The widespread nature of this disorder suggests that the cause involves climatic factors. We examined the influence of varying combinations of average annual temperature and precipitation on the incidence and distribution of SFD. Climatic transition matrices generated in this study indicate that most healthy trees are found in climatic zones with moderate to low temperatures and high precipitation; whereas, SFD occurs mostly in zones of moderate temperatures and moderate precipitation. The contrasting distributions define an environmental mismatch. Forests matched with favorable climatic conditions thrive; those that are mismatched can become vulnerable to decline disease.

关键词： Abiotic disorder Climate stress High elevation forests Forest disease - Subalpine fir

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Prospective cohort study on facial profile changes in infants with robin sequence and healthy controls

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World Journal of Pediatrics 2024年第6期20卷 581-589页

作者： Cornelia Wiechers Julian Sowula Karen Kreutzer Christoph e.Schwarz Christina Weismann Michael Krimmel Christian F.Poets Bernd Koos Department of Neonatology Tuebingen University HospitalCalwerstrasse 772076 TuebingenGermany Center for Cleft Lip Palate and Craniofacial MalformationsTuebingen University HospitalTuebingenGermany Department of Orthodontics Tuebingen University HospitalTuebingenGermany Department of Oral and Maxillofacial Surgery Tuebingen University HospitalTuebingenGermany

Background Various conservative and surgical approaches exist to treat robin sequence(RS),but their effects on facial profile and mandibular catch-up growth are unclear.A functional treatment concept,used in our centre for 25 years,includes an individualized palatal plate with a velo-pharyngeal extension and intensive feeding *** We performed a prospective study to objectively describe facial profiles in infants with RS treated with the above *** with isolated RS were admitted to our tertiary perinatal and national referral centre for craniofacial malformations between May 2018 and Nov *** with RS received 3D-photographs during clinically indicated *** controls were recruited from Dec 2018 to Sep 2019 and received 3D-photographs every 3 *** digitally measured jaw index(JI),defined as alveolar overjet(O)x maxillary arch(U)/mandibular arch(L),and the soft tissue reference points A’-point,Nasion’,B’-point angle(ANB’),describing the relative position of maxilla to mandible,were *** mixed models were used to examine time trajectories in JI and ANB’.Results A total of 2073D images,obtained in 19 infants with RS and 32 controls,were *** and ANB’decreased over time in both groups[for JI−0.18(95%CI−0.25 to−0.10);for ANB’:−0.40°per month[(95%CI−0.48 to−0.32)]]but remained lower in controls[for JI−2.5(95%CI−3.2 to−1.8);for ANB’-1.7°(95%CI−2.4 to−1.0)].Also,the ANB’model showed a significant effect of the interaction term diagnosis x *** Based on longitudinal 3D images,we describe changes in objective parameters of facial profile in infants with and without RS during the first year of *** findings indicate catch-up growth in infants treated for RS.

关键词： Infant Mandibular catch-up growth Pre-epiglottic Baton Plate robin sequence Three-dimensional photography

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