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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

Feather content of porphyrins in Eurasian eagle owl(Bubo bubo)fledglings depends on body condition and breeding site quality

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Integrative Zoology 2018年第5期13卷 569-578页

作者： Ismael GALVÁN maría del mar DELGADO Pablo RCAmarERO Rafael MATEO RuiLOURENÇO Vincenzo PENTERIANI Department of Evolutionary Ecology Doñana Biological Station-CSICSevillaSpain Research Unit of Biodiversity-UOCSIC-PA Oviedo UniversityMieresSpain Institute for Game and Wildlife Research IREC-CSIC-UCLM-JCCMCiudad RealSpain Labor-Laboratório de Ornitologia Instituto de Ciências Agrárias e Ambientais MediterrânicasUniversity of EvoraÉvoraPortugal Pyrenean Institute of Ecology(IPE)-CSIC ZaragozaSpain

Porphyrins are pigments produced in most animal cells during the synthesis of heme,but their importance for external coloration is ***(Order Strigiformes)are among the few animals that accumulate porphyrins in the integument,where it could serve as a means of *** we hypothesized that the porphyrin content of feathers may depend on body condition and breeding site quality in Eurasian eagle owl(Bubo bubo)fledglings and,thus,constitute amplifiers of the quality of the area where they are *** high-performance liquid chromatography,we found 2 porphyrins(protoporphyrin IX and coproporphyrin III)in the body feathers of 19 eagle owl fledglings from 7 breeding *** III,but not protoporphyrin IX feather concentration,was positively associated with the body mass of fledglings and with the quality of the breeding sites where they were reared with respect to food quality and *** coproporphyrin III is produced under oxidative stress,we suggest that good breeding sites may lead to fledglings in good ***,in turn,may make fledglings induce a certain level of free radical and coproporphyrin III production to signal to conspecifics their site-mediated capacity to cope with oxidative *** is the first time that porphyrin content in the integument has been found to be related to individual quality,opening a new scenario for studying evolution of animal coloration.

关键词： amplifiers condition-dependence eagle owl fluorescence porphyrins

Tumor microenvironment involvement in colorectal cancer progression via Wnt/β-catenin pathway:Providing understanding of the complex mechanisms of chemoresistance

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World Journal of Gastroenterology 2022年第26期28卷 3027-3046页

作者： maría Belén Novoa Díaz maría Julia martín Claudia Gentili Departamento de Biología Bioquímica y FarmaciaUniversidad Nacional del Sur(UNS)-INBIOSUR(CONICET-UNS)Bahía Blanca 8000Argentina Departamento de Química Universidad Nacional del Sur(UNS)-INQUISUR(CONICET-UNS)Bahía Blanca 8000Argentina

Colorectal cancer(CRC)continues to be one of the main causes of death from cancer because patients progress unfavorably due to resistance to current *** of the Wnt/β-catenin pathway plays a fundamental role in the genesis and progression of several types of cancer,including *** many subtypes of CRC,hyperactivation of theβ-catenin pathway is associated with mutations of the adenomatous polyposis coli ***,it can also be associated with other *** recent years,studies of the tumor microenvironment(TME)have demonstrated its importance in the development and progression of *** this tumor nest,several cell types,structures,and biomolecules interact with neoplastic cells to pave the way for the spread of the ***-communications between tumor cells and the TME are then established primarily through paracrine factors,which trigger the activation of numerous signaling *** advances in the field of oncology have been made in the last *** Minireview aims to actualize what is known about the central role of the Wnt/β-catenin pathway in CRC chemoresistance and aggressiveness,focusing on crosscommunication between CRC cells and the *** this analysis,our main objective was to increase the understanding of this complex disease considering a more global *** many treatments for advanced CRC fail due to mechanisms involving chemoresistance,the data here exposed and analyzed are of great interest for the development of novel and effective therapies.

关键词： Colorectal cancer β-catenin pathway Tumor stroma Tumor microenvironment factors Cancer progression Drug resistance

Deflection analysis of reinforced concrete beams strengthened with carbon fibre reinforced polymer under long-term load action

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Journal of Zhejiang University-Science A(Applied Physics & Engineering) 2012年第8期13卷 571-583页

作者： Mykolas DAUGEVIIUS Juozas VALIVONIS Gediminas mar IUKAITIS Department of Reinforced Concrete and Masonry Structures Vilnius Gediminas Technical University

This paper presents the results of an experimental research on reinforced concrete beams strengthened with an external carbon fibre reinforced polymer(CFRP) layer under long-term load action that lasted for 330 *** describe the characteristics of deflection development of the beams strengthened with different additional anchorages of the external carbon fibre composite layer during the period of *** conducted experiments showed that the additional anchorage influences the slip of the external layer with respect to the strengthened ***,concrete and carbon fibre composite interface stiffness decreases with a long-term load ***,the proposed method of analysis based on the built-up-bars theory can be used to estimate concrete and carbon fibre composite interface stiffness in the case of long-term load.

关键词： Carbon fibre composite Beam strengthening Beam deflection Long-term load Bending stiffness Concrete and carbon fibre composite interface stiffness Effective inertia moment

Long-term outcome of chronic hepatitis C patients with sustained virological response to peginterferon plus ribavirin

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World Journal of Gastroenterology 2011年第4期17卷 493-498页

作者： maría Trapero-marugán Jorge Mendoza maría Chaparro Leticia González-Moreno José Andrés Moreno-Monteagudo maría Jesús Borque Ricardo Moreno-Otero Gastroenterology-Hepatology Department University Hospital La Princesa and Princesa Research Institute Autonomous University of Madrid and CIBERehd (Centro de Investigación Biomédica en Red en Enfermedades Hepáticas y Digestivas)

AIM: To assess the clinical, biochemical and virological long-term outcome in chronic hepatitis C (CHC) patients with a sustained virological response (SVR) after peginterferon (PEG-IFN) plus ribavirin combination therapy. METHODS: One hundred and fifty three patients with a SVR after treatment with PEG-IFN plus ribavirin were included in a 5-year follow-up study in a single Spanish center, based on standard clinical practice. Clinical anamnesis, biochemical analysis, hepatitis C virus RNA and alpha-fetoprotein measurement, ultrasonography and transient elastography were performed annually. RESULTS: The mean follow-up period of the 153 patients was 76 ± 13 mo after they obtained a SVR. Five patients (3.26%) presented with cirrhosis before treatment and 116 (75.8%) had genotype 1. No patient showed evidence of hepatic decompensation. One patient (0.65%) developed a hepatocellular carcinoma at month 30 after achieving SVR. There were no virological relapses during this follow-up period. Persistently elevated alanine aminotransferase was found in only one patient (0.65%). At the end of the 5-year follow-up, the mean value of transient elastography was 7 ± 4.3 kPa (F1). There were no deaths and no other tumors. CONCLUSION: The long-term outcome of 153 CHC patients with SVR to PEG-IFN plus ribavirin was good. No evidence of a virological relapse was seen. One patient (0.65%) developed a hepatocellular carcinoma.

关键词： Chronic hepatitis C Peginterferon Ribavirin Sustained virological response Long-term effects

Risk Transfer for Populations in Precarious Urban Environments

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International Journal of Disaster Risk Science 2020年第1期11卷 74-86页

作者： Juan Pablo Sarmiento Ana maría Torres-Munoz Extreme Events Institute/Disaster Risk and Resilience in the Americas(DRRA) Florida International UniversityMiamiFL 33199USA

This study explores risk transfer options that precarious and mar.inal urban communities could use to protect themselves from future damages and losses generated by socio-natural hazards and disasters at the individual and community levels. The design is framed within an evidence-based disaster risk reduction(DRR) strategy and follows the case study research approach. We analyze the2018 Neighborhood Approach for DRR programming evaluation carried out in four Latin American cities’ informal settlements and review relevant risk transfer experiences aimed at vulnerable populations. We calculate the pure risk premium for the four cases selected, using a previous catastrophe risk assessment for earthquakes and landslides. We propose three risk transfer options based on our analysis:(1) voluntary collective insurance;(2) structural reinforcement with a comprehensive housing insurance;and(3) hybrid parametric insurance. Risk transfer mechanisms conventionally focus on residual risk management. Here, due to the precariousness of the analyzed urban settings, the proposed alternatives go beyond the management of just residual risk to positively impact the beneficiaries’ quality of life and the reduction of the built environment’s physical vulnerability in the short and medium terms. Our study proposes a prospective estimation of future risk despite the limitations of data availability. This study opens a window to new approaches and proposes a systematic process to design DRR policy aimed at the poor and vulnerable strata of society.

关键词： Case study research Catastrophe risk assessment Informal settlements Risk transfer Urban precariousness Urban risk

Exosomes as mediators of neuron-glia communication in neuroinflammation

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Neural Regeneration Research 2020年第5期15卷 796-801页

作者： maría Pascual Francesc Ibánez Consuelo Guerri Department of Molecular and Cellular Pathology of Alcohol Príncipe Felipe Research CenterValenciaSpain Department of Physiology School of Medicine and DentistryUniversity of ValenciaValenciaSpain

In recent years,a type of extracellular vesicles named exosomes has emerged that play an important role in intercellular communication under physiological and pathological *** nanovesicles (30–150 nm) contain proteins,RNAs and lipids,and their internalization by bystander cells could alter their normal *** review focuses on recent knowledge about exosomes as messengers of neuron-glia communication and their participation in the physiological and pathological functions in the central nervous *** emphasis is placed on the role of exosomes under toxic or pathological stimuli within the brain,in which the glial exosomes containing inflammatory molecules are able to communicate with neurons and contribute to the pathogenesis of neuroinflammation and neurodegenerative *** the small size and characteristics of exosomes,they can cross the blood-brain barrier and be used as biomarkers and diagnosis for brain disorders and ***,although the application potential of exosome is still limited,current studies indicate that exosomes represent a promising strategy to gain pathogenic information to identify therapeutically targets and biomarkers for neurological disorders and neuroinflammation.

关键词： biomarkers exosomes glial cells neuroinflammation neuron-glia commuication neurons neuropathology therapy

Evaluation of trace element mobilization and control under different fluidized bed combustion scenarios

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Clean Energy 2018年第2期2卷 167-174页

作者： maría Luisa Contreras Alberto Bahillo CIEMAT Department of EnergyAvdaComplutense 4028040 MadridSpain

As consequence of its relatively abundant reserves compared to other energy sources,coal will continue to be widely employed in power *** reduce coal’s negative impact on the environment,new approaches have been evaluated and adopted by various countries in recent years to minimize CO_(2) *** developments focused on the selection of new fuels(such as biomass fuels)to combine with coal to reduce emissions from coal combustion,whereas subsequent approaches aimed at implementing new processes for CO_(2) capture and storage,such as oxy-fuel *** addition,coal combustion is one of the main anthropogenic sources of trace elements(TE)emitted to the *** increasing knowledge acquired as to the impact of these pollutants in the environment has led to more restricted emission standards for some of these elements and to increased research on the technologies and pathways to control these *** article reviews the main factors involved in TE capture by comparing results obtained for Hg and other TEs under different control *** the effect of novel combustion processes and the potential of different pollution control techniques toward these pollutants are discussed.

关键词： trace elements mercury oxy-fuel combustion cofiring biomass

Stem cell-based approaches: Possible route to hearing restoration?

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World Journal of Stem Cells 2020年第6期12卷 422-437页

作者： maría Beatriz Durán-Alonso Institute of Biology and Molecular Genetics University of ValladolidValladolid 47003Spain

Disabling hearing loss is the most common sensorineural disability *** affects around 466 million people and its incidence is expected to rise to around 900 million people by 2050,according to World Health Organization *** cases of hearing impairment are due to the degeneration of hair cells(HCs)in the cochlea,mechano-receptors that transduce incoming sound information into electrical signals that are sent to the *** to these cells is mainly caused by exposure to aminoglycoside antibiotics and to some anti-cancer drugs such as cisplatin,loud sounds,age,infections and genetic *** deficits may also result from damage to the spiral ganglion neurons that innervate cochlear *** from what is observed in avian and nonmammalian species,there is no regeneration of missing sensory cell types in the adult mammalian cochlea,what makes hearing loss an irreversible *** review summarizes the research that has been conducted with the aim of developing cell-based strategies that lead to sensory cell replacement in the adult cochlea and,ultimately,to hearing *** main lines of research are discussed,one directed toward the transplantation of exogenous replacement cells into the damaged tissue,and another that aims at reactivating the regenerative potential of putative progenitor cells in the adult inner *** from some of the studies that have been conducted are presented and the advantages and drawbacks of the various approaches discussed.

关键词： Hearing loss Cochlear hair cells Spiral ganglion neurons Cell regeneration Adult stem cells Cell transplantation