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Senning operation for correction of the transposition of the great arteries, results, long-term outcome and quality of life

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World Journal of Cardiovascular Diseases 2012年第3期2卷 213-219页

作者： miguel angel Maluf Disciplina de Cirurgia Cardiovascular Universidade Federal de Sao PauloSao PauloBrazil

Objective: Long-term results after the Senning operation for transposition of the great arteries are little known. Sinus node dysfunction and systemic ventricular dysfunction are crucial in patient survival. We evaluated the results, long term outcome and quality of life in a group of 39 patients. Methods: The study was a retrospective analysis, of 39 (39/40 = 97.5%) surgical surviving patients, submitted to Senning operation, with a mean follow-up time of 14.7 +/– 3.1 years. Electrocardiogram, echocardiogram, and chest radiograph series were performed every 6 months. Thirty six patients of this series underwent Holter study and ergometric test, to evaluate the physical capacity. Three patients living overseas were excluded. Results: There was only one late death (1/39) (late mortality = 2.5%): a 16 year-old patient had a no cardiac death. The actuarial survival was 95.0% (38/40) (simple or with little VSD, TGA). The probability of staying in sinus rhythm, in 39 surviving patients was 77.1% (30) or normal right ventricular function was 76.5% (29), 10 to 20 years after operation. The incidence of sinus node and right ventricular dysfunction increased gradually over time. No re-operations and pacemaker implantation, was performed. Functional class: I = 30 (85.7%) cases and functional class II = 5 (14.3%) cases. Conclusions: Patients with simple TGA submitted to Senning procedure in our experience, presented during late follow-up: 1—Low incidence of right ventricular dysfunction and active arrhythmias;2—Low mortality and no sudden death recorded;3—Good quality of life and 4—Satisfactory surgical results (free of re-operation or definitive pacemaker implantation).

关键词： Congenital Heart Disease Surgery Treatment Outcome

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Dysregulation of the PD-1/PD-L1 pathway contributes to the pathogenesis of celiac disease

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Cellular & Molecular Immunology 2019年第9期16卷 777-779页

作者： Candelaria Ponce de Leon miguel angel Lopez-Casado Pedro Lorite Teresa Palomeque Maria Isabel Torres Department of Experimental Biology University of JaenJaenSpain Department of Pediatrics Gastroenterology Hospital Virgen de las NievesGranadaSpain

In this study,we focus on the alteration of the programmed cell death 1(PD-1)/PD-L1 pathway in celiac *** discuss the diverse roles of the PD-1 pathway in regulating immune responses and how this knowledge can improve celiac disease(CD)*** PD-1 and PD-L1 levels in the serum and in intestinal biopsies of CD patients may be relevant to the determination of a possible correlation between markers of the autoimmune response,inflammation,and disease activity.

关键词： celiac pathogenesis inflammation

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Role of the Wnt/β-catenin pathway in gastric cancer: An indepth literature review

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World Journal of Experimental Medicine 2015年第2期5卷 84-102页

作者： miguel angel Chiurillo Laboratorio de Genética Molecular "Dr. Yunis-Turbay" Decanato de Ciencias de la Salud Universidad Centroccidental Lisandro Alvarado Departamento de Patologia Clínica Universidade Estadual de Campinas

Gastric cancer remains one of the most common cancers worldwide and one of the leading cause for cancerrelated deaths. Gastric adenocarcinoma is a multifactorial disease that is genetically, cytologically and architecturally more heterogeneous than other gastrointestinal *** aberrant activation of the Wnt/β-catenin signaling pathway is involved in the development and progression of a significant proportion of gastric cancer cases. This review focuses on the participation of the Wnt/b-catenin pathway in gastric cancer by offering an analysis of the relevant literature published in this field. Indeed, it is discussed the role of key factors in Wnt/β-catenin signaling and their downstream effectors regulating processes involved in tumor initiation, tumor growth, metastasis and resistance to therapy. Available data indicate that constitutive Wnt signalling resulting from Helicobacter pylori infection and inactivation of Wnt inhibitors(mainly by inactivating mutations and promoter hypermethylation) play an important role in gastric cancer. Moreover, a number of recent studies confirmed CTNNB1 and APC as driver genes in gastric cancer. The identification of specific membrane, intracellular, and extracellular components of the Wnt pathway has revealed potential targets for gastric cancer therapy. High-throughput "omics" approaches will help in the search for Wnt pathway antagonist in the near future.

关键词： Gastric cancer Wnt β-catenin Oncogene Tumor suppressor Epigenetics Helicobacter pylori Adenomatous polyposis coli

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Flat Belt Separation System under Slat in Fattening Pig Housing: Effect of Belt Slope Angle

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Agricultural Sciences 2018年第6期9卷 692-702页

作者： Francisco Alonso Aranzazu mateos Jesús Vázquez miguel angel Garcimartín Elvira Sánchez Ismael Ovejero Escuela Técnica Superior de Ingeniería Agronómica Alimentaria y de Biosistemas Technical University of Madrid Ciudad Universitaria Madrid Spain

In this study, the separation and drying efficiency of the excreta, separated into solid and liquid fraction by a flat conveyor belt under a partial slatted floor in a facility for fattening pigs, was determined. Two transverse slope angles (4° and 6°) for the flat belts were used, in a trial with pigs from 20.61 to 117.83 kg of live weight (LW), and the sample size was 7 pigs per pen, 42 pigs per room. The flat belt was more efficient with 6° than with 4° slope angle (32.65% vs. 29.91% dry matter content of solid fraction during the whole fattening period, respectively;P ° than for the 4° (1.4137 vs. 1.3030, during the whole fattening period;P °, this efficiency was not improved in finishing period -83.40 to 117.83 kg LW (two extractions per day) with regard to the growth period -20.61 to 83.40 kg LW (one extraction per day). Meanwhile, with 4° it did improved. The ventilation system underneath slat seems to play a key role in the obtained results.

关键词： Flat Belt Manure Separation and Drying Efficiency Separation System Swine

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Innovation in pathogenesis and management of aortic aneurysm

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World Journal of Experimental Medicine 2024年第2期14卷 35-45页

作者： Maryam Barkhordarian Hadrian Hoang-Vu Tran Aiswarya Menon Sai Priyanka Pulipaka Izage Kianifar Aguilar Axel Fuertes Shraboni Dey angel Ann Chacko Tanni Sethi Ayrton Bangolo Simcha Weissman Department of Internal Medicine Palisades Medical CenterNorth BergenNJ 07047United States

Aortic aneurysm(AA)refers to the persistent dilatation of the aorta,exceeding three *** the pathophysiology of this condition is important for its prevention and management,given its responsibility for more than 25000 deaths in the United *** are classified based on their location or *** pathophysiologic pathways including inflammation,the immune system and atherosclerosis have been implicated in its *** markers such as transforming growth factorβ,interleukin-1β,tumor necrosis factor-α,matrix metalloproteinase-2 and many more may contribute to this *** genetic disorders such as Marfan syndrome,Ehler-Danlos syndrome and Loeys-Dietz syndrome have also been associated with this *** years has seen the investigation of novel management of AA,exploring the implication of different immune suppressors,the role of radiation in shrinkage and prevention,as well as minimally invasive and newly hypothesized surgical *** this narrative review,we aim to present the new contributing factors involved in pathophysiology of *** also highlighted the novel management methods that have demonstrated promising benefits in clinical outcomes of the AA.

关键词： Aortic aneurysm Abdominal aneurysm Thoracic aneurysm Immunotherapy Surgical management Pathophysiology Inflammation and molecular pathways

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利用采光井进行建筑通风的新型应用模型:基于案例研究评估的建议（英文）

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Journal of Zhejiang University-Science A(Applied Physics & Engineering) 2018年第10期19卷 796-810页

作者： miguel angel PADILLA-MARCOS Alberto MEISS Jesus FEIJO-MUNOZ Research Group of Architecture&Energy Universidad de ValladolidValladolid 47014Spain

目的:1.探究如何在有限的室外空间下简便地计算换气质量;2.建立利用采光井进行建筑通风的应用模型。创新点:1.案例研究评估的成熟方法论应用;2.得到采光井对室内空气通风促进度的速算图表。方法:1.采用风洞分析的两步确认法;2.对实际采... 详细信息

目的:1.探究如何在有限的室外空间下简便地计算换气质量;2.建立利用采光井进行建筑通风的应用模型。创新点:1.案例研究评估的成熟方法论应用;2.得到采光井对室内空气通风促进度的速算图表。方法:1.采用风洞分析的两步确认法;2.对实际采光井模型进行计算流体动力学应用。结论:1.高长比小于3的采光井表现出了不错的换气能力;2.对空气质量要求更高的居住空间(如卧室和起居室)而言,室内通风口必须被设在高度低于24米的采光井的上半部分或者其他采光井中距离顶部12米以内的位置。

关键词：室外空气质量采光井自然通风计算流体动力学换气效率

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Whole-genome amplification/preimplantation genetic testing for propionic acidemia of successful pregnancy in an obligate carrier Mexican couple:A case report

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World Journal of Clinical Cases 2021年第29期9卷 8797-8803页

作者： Adina Neumann miguel angel Alcantara-Ortigoza Ariadna González-del angel Nestor Alejandro Zarate Díaz Javier Sam Santana Leonardo M Porchia Esther López-Bayghen Laboratorio de Investigación y Diagnóstico Molecular IngenesMexico City 05320Mexico Laboratorio de Biología Molecular Subdirección de Investigación MédicaInstituto Nacional de PediatríaMexico City 04530Mexico DNA-GEN S.C.Centro de Alta Especialidad en Genética Humana Mexico City 14070Mexico Investigación Clínica IngenesPuebla 72820Mexico Departamento de Toxicología Centro de Investigación y de Estudios Avanzados del Instituto Politécnico NacionalMexico City 07360Mexico

BACKGROUND Identifying a potential single monogenetic disorder in healthy couples is costly due to the Assisted Reproduction facilities'current methodology for screening,which focuses on the detecting multiple genetic disorders at ***,we report the successful application of a low-cost and fast preimplantation genetic testing for monogenic/single gene defects(PGT-M)approach for detecting propionic acidemia(PA)in embryos obtained from a confirmed heterozygous propionyl-CoA carboxylase alpha subunit(PCCA)*** SUMMARY A fertile 32-years old Mexican couple with denied consanguinity sought antenatal genetic *** were suspected obligate PA carriers due to a previous deceased PA male newborn with an unknown PCCA/propionyl-CoA carboxylase beta subunit(PCCB)***-Generation Sequencing revealed a heterozygous genotype for a pathogenic PCCA variant(c.2041-1G>T,ClinVar:RCV-000802701.1;dbSNP:rs1367867218)in both *** couple requested in vitro fertilization(IVF)and PGT-M for *** IVF,12 oocytes were collected and fertilized,of which two resulted in high-quality *** biopsies and Whole Genome Amplification by a fragmentation/amplification-based method were performed and revealed that the two embryos were *** polymerase chain reaction and further Sanger sequencing of the exon-intron borders revealed a wild-type PCCA male embryo and a heterozygous c.2041-1G>T female *** embryos were transferred,resulting in a clinical pregnancy and the delivery of a healthy male newborn(38 wk,weight:4080 g,length:49 cm,APGAR 9/9).The absence of PA was confirmed by expanded newborn *** We show that using PGT-M with Whole Genome Amplification templates,coupled with IVF,can reduce the transmission of a pathogenic variant of the PCCA gene.

关键词： Propionic acidemia Autosomal recessive Propionyl-CoA carboxylase alpha subunit(PCCA)gene Preimplantation genetic testing Next-generation sequencing Embryo transfer

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Poly-contextural Logic： New Resource for Transdisciplinary Research in Sociology

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Sociology Study 2011年第2期1卷 143-152页

作者： miguel angel Briceflo Gil aCentral University of Venezuela Caracas Venezuela

A couple of decades ago natural phenomena began to be approached from a comprehensive and transdisciplinary point of view, as it was understood that living beings and their environments are not linear but complex. There is no doubt that this perspective of visualizing complexity and working inter- and trans-disciplinarily has to be applied. The reflection on the theoretical observation （i.e., meta-observation） involved in the concept of poly-contexturality is the framework in which a theory of complex systems is possible, which in turn enables an observation that oscillates （a concept of chaos theory） between models structured in a hierarchical order （normally linked to a logical-deductive formalization） and models structured in hetero-hierarchy. And this would allow this reflection to be done in a formalized language that does not follow either the principles of the Aristotelian logic or the postulates of the Kantian transcendental reflection. It is precisely this liberation from the dictates of mono-contextural logic what paves the way to an observation of complexity, in which one or the other language is used to model the states of things, such as the epistemological problems of molecular biology or the social systems. And-what is gaining relevance-it also paves the way to a true transdisciplinary meta-observation, since each discipline chooses its own contexture and only the use of poly-contexturality makes it possible to formulate transdisciplinary relationships within the framework of such meta-logic.

关键词： Poly-contextural logic transdisciplinary non-linear complex systems

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Does an association exist between chronic pancreatitis and liver cirrhosis in alcoholic subjects?

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World Journal of Gastroenterology 2008年第40期14卷 6171-6179页

作者： Luis Aparisi Luis Sabater Juan Del-Olmo Juan Sastre miguel-angel Serra Ricardo Campello Daniel Bautista Abdalla Wassel José-Manuel Rodrigo Liver Unit Universitary Clinic Hospital Department of Surgery Universitary Clinic Hospital Department of Physiology University of Valencia Unit of Psiquiatry Servicio Valenciano de SaludValencia 46010Spain Department of Preventive Medicine Hospital Dr.Peset

AIM: To study the possible association between chronic pancreatitis (CP) and liver cirrhosis (LC) of alcoholic etiology,after excluding any other ***: One hundred and forty consecutive alcoholic patients were subdivided into three groups: CP (n = 53),LC (n = 57),and asymptomatic alcoholic (n = 30).Clinical,biochemical and morphological characteristics,Child-Pugh index,indocyanine green test,and fecal pancreatic elastase-1 test were ***: In patients with cirrhosis,major clinical manifestations of CP such as pancreatic pain and steatorrhea,as well as imaging alterations of CP such as calcifications,duct dilation and pseudocysts were absent; insulin-dependent diabetes was present in 5.3% of cases,and elastase-1 test was altered in only 7%,and severely altered in *** patients with CP,clinical characteristics of cirrhosis such as ascites,encephalopathy and gastrointestinal hemorrhage were present in one case,Child-Pugh grade > A in 5.7%,and altered indocyanine green test in 1.9% *** asymptomatic alcoholism,there was only a non-coincident alteration of elastase-1 test and indocyanine test in 14.8% and 10%,respectively,but other characteristics of cirrhosis or CP were *** inverse correlation (r = -0.746) between elastase-1 test and indocyanine test was found in alcoholic ***: There is a scarce coincidence in clinical and morphological alterations among patients with CP or LC of alcoholic etiology,but an inverse correlation between pancreatic and liver function *** findings support that these alcoholic diseases evolve in a different manner and have different etiopathogenesis.

关键词： Alcoholic chronic pancreatitis Alcoholicliver cirrhosis Alcoholism Pancreatic function Hepaticfunction

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Arabidopsis Squalene Epoxidase 3 （SQE3） Complements SQE1 and Is Important for Embryo Development and Bulk Squalene Epoxidase Activity

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Molecular Plant 2015年第7期8卷 1090-1102页

作者： Sara Laranjeira Vitor Amorim-Silva Alicia Esteban Monserrat Arro Albert Ferrer Rui Manuel Tavares miguel angel Botella Abel Rosado Herlander Azevedo Center for Biodiversity Functional & Integrative Genomics (BioFIG) Plant Functional Biology Center University of Minho Campus de Gualtar 4710-057 BragaPortugal Instituto de Hortofruticultura Subtropical y Mediterranea 'La Mayora' Universidad de Malaga-Consejo Superior de Investigaciones Cientificas (IHSM-UMA-CSIC)Departmento de Biologia Molecular y Bioquimica Universidad de Malaga Campus Teatinos 29071 Malaga Spain Department of Molecular Genetics Centre for Research in Agricultural Genomics (CSIC-IRTA-UAB-UB) Bellaterra-Cerdanyola del Valles 08193 Spain Department of Biochemistry and Molecular Biology Faculty of Pharmacy University of Barcelona 08028 Barcelona Spain Canada Research Chair in Plant Physiology and Cellular Dynamics Department of Botany Faculty of Sciences University of British Columbia VancouverBC V6T 1Z4 Canada CIBIO InBIO - Research Network in Biodiversity and Evolutionary Biology Universidade do Porto Campus Agrario de Vairao 4485-661 Vairao Portugal

The existence of multigenic families in the mevalonate pathway suggests divergent functional roles for pathway components involved in the biosynthesis of plant sterols. Squalene epoxidases （SQEs） are key components of this pathway, and Squalene Epoxidase 1 （SQE1） has been identified as a fundamental enzyme in this biosynthetic step. In the present work, we extended the characterization of the remaining SQE family members, phylogenetically resolving between true SQEs and a subfamily of SQE-like proteins that is exclusive to Brassicaceae. Functional characterization of true SQE family members, Squalene Epox- idase 2 （SQE2） and Squalene Epoxidase 3 （SQE3）, indicates that SQE3, but not SQE2, contributes to the bulk SQE activity in Arabidopsis, with sqe3-1 mutants accumulating squalene and displaying sensitivity to ter- binafine. We genetically demonstrated that SQE3 seems to play a particularly significant role in embryo development. Also, SQE1 and SQE3 both localize in the endoplasmic reticulum, and SQE3 can functionally complement SQEI. Thus, SQE1 and SQE3 seem to be two functionally unequal redundant genes in the pro- motion of plant SQE activity in Arabidopsis.

关键词： Arabidopsis embryo development MVA pathway squalene epoxidase sterol biosynthesis

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