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| 1 | Liver cell transplantation for Crigler-Najjar syndrome type Ⅰ: Update and perspectives显示文摘Liver cell transplantation is an attractive technique to treat liver-based inborn errors of metabolism. The feasibility and efficacy of the procedure has been demonstrated, leading to medium term partial metabolic control of various diseases. Crigler-Najjar is the paradigm of such diseases in that the host liver is lacking one function with an otherwise normal parenchyma. The patient is at permanent risk for irreversible brain damage. The goal of liver cell transplantation is to reduce serum bilirubin levels within safe limits and to alleviate phototherapy requirements to improve quality of life. Preliminary data on Gunn rats, the rodent model of the disease, were encouraging and have led to successful clinical trials. Herein we report on two additional patients and describe the current limits of the technique in terms of durability of the response as compared to alternative therapeutic procedures. We discuss the future developments of the technique and new emerging perspectives. | Philippe A Lysy Mustapha Najimi Xavier Stéphenne Annick Bourgois Franoise Smets Etienne M Sokal | 2008 | World Journal of Gastroenterology2008，14，22： | 9 |
| 2 | Atrial fibrillation: the current epidemic显示文摘 | Carlos A Morillo Amitava Banerjee Pablo Perel David Wood Xavier Jouven | 2017 | Journal of Geriatric Cardiology2017，14，3： | 7 |
| 3 | SIRT3-mediated inhibition of FOS through histone H3 deacetylation prevents cardiac fibrosis and inflammation显示文摘Sirtuin 3(SIRT3)is a deacetylase that modulates proteins that control metabolism and protects against oxidative stress.Modulation of SIRT3 activity has been proposed as a promising therapeutic target for ameliorating metabolic diseases and associated cardiac disturbances.In this study,we investigated the role of SIRT3 in inflammation and fibrosis in the heart using male mice with constitutive and systemic deletion of SIRT3 and human cardiac AC16 cells.SIRT3 knockout mice showed cardiac fibrosis and inflammation that was characterized by augmented transcriptional activity of AP-1.Consistent with this,SIRT3 overexpression in human and neonatal rat cardiomyocytes partially prevented the inflammatory and profibrotic response induced by TNF-α.Notably,these effects were associated with a decrease in the mRNA and protein levels of FOS and the DNA-binding activity of AP-1.Finally,we demonstrated that SIRT3 inhibits FOS transcription through specific histone H3 lysine K27 deacetylation at its promoter.These findings highlight an important function of SIRT3 in mediating the often intricate profibrotic and proinflammatory responses of cardiac cells through the modulation of the FOS/AP-1 pathway.Since fibrosis and inflammation are crucial in the progression of cardiac hypertrophy,heart failure,and diabetic cardiomyopathy,our results point to SIRT3 as a potential target for treating these diseases. | Xavier Palomer MSilvia Román-Azcona Javier Pizarro-Delgado Ana Planavila Francesc Villarroya Brenda Valenzuela-Alcaraz Fátima Crispi Álvaro Sepúlveda-Martínez Irene Miguel-Escalada Jorge Ferrer JFrancisco Nistal Raquel García Mercy MDavidson Emma Barroso Manuel Vázquez-Carrera | 2020 | Signal Transduction and Targeted Therapy2020，5，1： | 7 |
| 4 | Diagnosis and Epidemiology of H elicobacter pylori Infection显示文摘 | Xavier Calvet María‐José Ramírez Lázaro Philippe Lehours Francis Mégraud | 2013 | Helicobacter2013，，： | 5 |
| 5 | Mobilization of hematopoietic progenitor cells in patients with liver cirrhosis显示文摘AIM:To test the hypothesis that liver cirrhosis is associated with mobilization of hematopoietic progenitor cells. METHODS:Peripheral blood samples from 72 patients with liver cirrhosis of varying etiology were analyzed by flow cytometry.Identified progenitor cell subsets were immunoselected and used for functional assays in vitro. Plasma levels of stromal cell-derived factor-1(SDF-1) were measured using an enzyme linked immunosorbent assay.RESULTS:Progenitor cells with a CD133 + /CD45 + CD14 + phenotype were observed in 61%of th patients.Between 1%and 26%of the peripheral bloo mononuclear cells(MNCs)displayed this phenotype Furthermore,a distinct population of c-kit + progenito cells(between 1%and 38%of the MNCs)could b detected in 91%of the patients.Additionally,18% of the patients showed a population of progenito cells(between 1%and 68%of the MNCs)that wa characterized by expression of breast cancer resistanc protein-1.Further phenotypic analysis disclosed tha the circulating precursors expressed CXC chemokin receptor 4,the receptor for SDF-1.In line with thi finding,elevated plasma levels of SDF-1 were presen in all patients and were found to correlate with th number of mobilized CD133 + progenitor cells. | Ursula M Gehling Marc Willems Kathleen Schlagner Ralf A Benndorf Maura Dandri Jrg Petersen Martina Sterneck Joerg-Matthias Pollok Dieter K Hossfeld Xavier Rogiers | 2010 | World Journal of Gastroenterology2010，16，2： | 4 |
| 6 | Outcome in obscure gastrointestinal bleeding after capsule endoscopy显示文摘AIM: To investigate the clinical impact of capsule endoscopy(CE) after an obscure gastrointestinal bleeding(OGIB) episode, focusing on diagnostic work-up, followup and predictive factors of rebleeding. METHODS: Patients who were referred to Hospital del Mar(Barcelona, Spain) between 2007 and 2009 for OGIB who underwent a CE were retrospectively analyzed. Demographic data, current treatment with non-steroid antiinflammtory drugs or anticoagulant drugs, hemoglobin levels, transfusion requirements, previous diagnostic tests for the bleeding episode, as well as CE findings(significant or non-significant), work-up and patient out-comes were analyzed from electronic charts. Variables were compared by χ 2 analysis and Student t test. Risk factors of rebleeding were assessed by Log-rank test, Kaplan-Meier curves and Cox regression model. RESULTS: There were 105 patients [45.7% women, median age of 72 years old(interquartile range 56-79)] and a median follow-up of 326 d(interquartile range 123-641) included in this study. The overall diagnostic yield of CE was 58.1%(55.2% and 63.2%, for patients with occult OGIB and overt OGIB, respectively). In 73 patients(69.5%), OGIB was resolved. Multivariate analysis showed that hemoglobin levels lower than 8 g/dL at diagnosis [hazard ratios(HR) = 2.7, 95%CI: 1.9-6.3], patients aged 70 years and above(HR = 2.1, 95%CI: 1.2-6.1) and significant findings in CE(HR = 2.4, 95%CI: 1.1-5.8) were independent predictors of rebleeding. CONCLUSION: One third of the patients presented with rebleeding after CE; risk factors were hemoglobin levels < 8 g/dL, age ≥ 70 years or the presence of significant lesions. | Alex Caas-Ventura Lucia Márque Xavier Bessa Josep Maria DedeuDepartment of Gastroenterology Hospital del Mar Research Institute Pompeu Fabra University Marc Puigvehí Sílvia Delgado-Aros Ines Ana Ibáez Agustin Seoane Luis Barranco Felipe Bory Montserrat Andreu Begoa González-Suárez | 2013 | World Journal of Gastrointestinal Endoscopy2013，5，11： | 4 |
| 7 | Aplastic anemia and severe pancytopenia during treatment with peg-interferon,ribavirin and telaprevir for chronic hepatitis C显示文摘Telaprevir and Boceprevir are the first direct acting antivirals approved for chronic hepatitis C in combination with peg-interferon alfa and ribavirin.Pancytopenia due to myelotoxicity caused by these drugs may occur,but severe hematological abnormalities or aplastic anemia(AA) have not been described.We collected all cases of severe pancytopenia observed during triple therapy with telaprevir in four Spanish centers since approval of the drug in 2011.Among 142 cirrhotic patients receiving treatment,7 cases of severe pancytopenia(5%) were identified and three were consistent with the diagnosis of AA.Mean age was 59 years,five patients had compensated cirrhosis and two patients had severe hepatitis C recurrence after liver transplantation.Severe pancytopenia was diagnosed a median of 10 wk after the initiation of therapy.Three patients had pre-treatment hematological abnormalities related to splenomegaly.In six patients,antiviral treatment was interrupted at the onset of hematological abnormalities.Two patients died due to septic complications and one patient due to acute alveolar hemorrhage.The remaining patients recovered.Severe pancytopenia and especially AA,are not rare during triple therapy with telaprevir in patients with advanced liver disease.Close monitoring is imperative in this setting to promptly detect serious hematological disorders and to prevent further complications. | Sabela Lens Jose L Calleja Ana Campillo Jose A Carrion Teresa Broquetas Christie Perello Juan de la Revilla Zoe Marino Maria-Carlota Londono Jose M Sanchez-Tapias Alvaro Urbano-Ispizua Xavier Forns | 2015 | World Journal of Gastroenterology2015，21，17： | 4 |
| 8 | New genes emerging for colorectal cancer predisposition显示文摘Colorectal cancer(CRC)is one of the most frequent neoplasms and an important cause of mortality in the developed world.This cancer is caused by both genetic and environmental factors although 35%of the variation in CRC susceptibility involves inherited genetic differences.Mendelian syndromes account for about5%of the total burden of CRC,with Lynch syndrome and familial adenomatous polyposis the most common forms.Excluding hereditary forms,there is an important fraction of CRC cases that present familial aggregation for the disease with an unknown germline genetic cause.CRC can be also considered as a complex disease taking into account the common diseasecommom variant hypothesis with a polygenic model of inheritance where the genetic components of common complex diseases correspond mostly to variants of low/moderate effect.So far,30 common,low-penetrance susceptibility variants have been identified for CRC.Recently,new sequencing technologies including exomeand whole-genome sequencing have permitted to add a new approach to facilitate the identification of new genes responsible for human disease predisposition.By using whole-genome sequencing,germline mutations in the POLE and POLD1 genes have been found to be responsible for a new form of CRC genetic predisposition called polymerase proofreading-associated polyposis. | Clara Esteban-Jurado Pilar Garre Maria Vila Juan José Lozano Anna Pristoupilova Sergi Beltrán Anna Abulí Jenifer Muoz Francesc Balaguer Teresa Ocaa Antoni Castells Josep M Piqué Angel Carracedo Clara Ruiz-Ponte Xavier Bessa Montserrat Andreu Luis Bujanda Trinidad Caldés Sergi Castellví-Bel | 2014 | World Journal of Gastroenterology2014，20，8： | 3 |
| 9 | Liver fat deposition and mitochondrial dysfunction in morbid obesity:An approach combining metabolomics with liver imaging and histology显示文摘AIM: To explore the usefulness of magnetic resonance imaging(MRI) and spectroscopy(MRS) for assessment of non-alcoholic fat liver disease(NAFLD) as compared with liver histological and metabolomics findings. METHODS: Patients undergoing bariatric surgery following procedures involved in laparoscopic sleeve gastrectomy were recruited as a model of obesityinduced NAFLD in an observational, prospective, singlesite, cross-sectional study with a pre-set duration of 1 year. Relevant data were obtained prospectively and surrogates for inflammation, oxidative stress and lipid and glucose metabolism were obtained through standard laboratory measurements. To provide reliable data from MRI and MRS, novel procedures were designed to limit sampling variability and other sources of error using a 1.5T Signa HDx scanner and protocols acquired from the 3D or 2D Fat SAT FIESTA prescription manager. We used our previously described 1H NMRbased metabolomics assays. Data were obtained immediately before surgery and after a 12-mo period including histology of the liver and measurement of metabolites. Values from 1H NMR spectra obtained after surgery were omitted due to technical limitations.RESULTS: MRI data showed excellent correlation with the concentration of liver triglycerides, other hepatic lipid components and the histological assessment, w h i c h e xc l u d e d t h e p r e s e n c e o f n o n-a l c o h o l i c steatohepatitis(NASH). MRI was sufficient to follow up NAFLD in obese patients undergoing bariatric surgery and data suggest usefulness in other clinical situations. The information provided by MRS replicated that obtained by MRI using the-CH3 peak(0.9 ppm), the-CH2- peak(1.3 ppm, mostly triglyceride) and the-CH=CH- peak(2.2 ppm). No patient depicted NASH. After surgery all patients significantly decreased their body weight and steatosis was virtually absent even in patients with previous severe disease. Improvement was also observed in the serum concentrations of selected variables. The most relevant findings using metabolomics indicate increased levels of triglyceride and monounsaturated fatty acids in severe steatosis but those results were accompanied by a significant depletion of diglycerides, polyunsaturated fatty acids, glucose-6-phosphate and the ATP/AMP ratio. Combined data indicated the coordinated action on mitochondrial fat oxidation and glucose transport activity and may support the consideration of NAFLD as a likely mitochondrial disease. This concept may helpto explain the dissociation between excess lipid storage in adipose tissue and NAFLD and may direct the search for plasma biomarkers and novel therapeutic strategies. A limitation of our study is that data were obtained in a relatively low number of patients.CONCLUSION: MRI is sufficient to stage NAFLD in obese patients and to assess the improvement after bariatric surgery. Other data were superfluous for this purpose. | Nahum Calvo Raúl Beltrán-Debón Esther Rodríguez-Gallego Anna Hernández-Aguilera Maria Guirro Roger Mariné-Casadó Lidón Millá Josep M Alegret Fàtima Sabench Daniel del Castillo María Vinaixa Miguelàngel Rodríguez Xavier Correig Roberto García-álvarez Javier A Menendez Jordi Camps Jorge Joven | 2015 | World Journal of Gastroenterology2015，21，24： | 2 |
| 10 | Susceptibility Genetic Variants Associated With Colorectal Cancer Risk Correlate With Cancer Phenotype显示文摘 | Anna Abulí Xavier Bessa Juan Ramón González Clara Ruiz–Ponte Alejandro Cáceres Jenifer Mu?oz Victoria Gonzalo Francesc Balaguer Ceres Fernández–Rozadilla Dolors González Luisa de Castro Juan Clofent Luís Bujanda Joaquín Cubiella Josep M a Re?é Juan Diego | 2010 | Gastroenterology2010，，3： | 2 |
| 11 | ChREBP binding to fatty acid synthase and L-type pyruvate kinase genes is stimulated by glucose in pancreatic β-cells显示文摘 | Xavier G S Rutter G A Diraison F | 2006 | J Lip Res2006，11，47： | 1 |
| 12 | Chemical Coal Cleaning Using Selective Oxidation显示文摘 | Stephen R P Edwin J H Xavier A D | 1994 | Fuel1994，73，2： | 1 |
| 13 | Synthesis and characterization of silica/poly (methyl methacrylate)nanocomposite latex particles through emulsion polymerization using a cationic azo initiator 显示文摘 | Xavier J L Guyot A Lanai E B | 2002 | Journal of Colloid and Interface Science2002，250，1： | 1 |
| 14 | Synthesis and characterization of silica/poly(methyl methacrylate) nanocomposite latex particles through emulsion polyme-rization using a cationic azo initiator显示文摘 | LUIZ J XAVIER L GUYOT A | 2002 | Journal of Colloid and Interface Science2002，250，： | 1 |
| 15 | Identification of a novel Ras -regulated proapoptotic pathway显示文摘 | Khokhlatchev A Rabizadeh S Xavier R | 2002 | Curr Biol Curr Biol2002，12，4： | 1 |
| 16 | Microstructure,mechanical Properties and wearresistance of high speedsteel rolls for hot rolling mills 显示文摘 | Carvalho M A De Xavier R R Filho C S P | 2002 | I& SM2002，29，1： | 1 |
| 17 | Effect of fixed dose combinations of metoprolol and amlodipine in essential hyper- tension: MARS- -a randomized controlled trial 显示文摘 | Devi P Xavier D Sigarnani A | 2011 | Blood Press Suppl2011，2，： | 1 |
| 18 | In vitro antibiotic susceptibility of field isolates of Mycoplasma synoviae in Argentina显示文摘 | Cerda R O Giacoboni G I Xavier J A | 2002 | Avian Diseases2002，46，1： | 1 |
| 19 | Amelioration of radiation-induced fibrosis inhibition fo transforming growth factor-signing by halofuginone显示文摘 | Xavier S Russo A | 2004 | J Biol Chem2004，279，15： | 1 |
| 20 | New sex attractant composition for the click beetle Agriotcs proximus: Similarity to the pheromone of Agriotes lineatus 显示文摘 | Toth M Furlan L Xavier A | 2008 | J Chem Ecol2008，34，： | 1 |
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