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| 1 | Induced pluripotent stem cells for modeling neurological disorders显示文摘Several diseases have been successfully modeled since the development of induced pluripotent stem cell(i PSC) technology in 2006. Since then, methods for increased reprogramming efficiency and cell culture maintenance have been optimized and many protocols for differentiating stem cell lines have been successfully developed, allowing the generation of several cellular subtypes in vitro. Gene editing technologies have also greatly advanced lately, enhancing disease-specific phenotypes by creating isogenic cell lines, allowing mutations to be corrected in affected samples or inserted in control lines. Neurological disorders have benefited the most from i PSC-disease modeling for its capability for generating disease-relevant cell types in vitro from the central nervous system, such as neurons and glial cells, otherwise only available from post-mortem samples. Patient-specific i PSC-derived neural cells can recapitulate the phenotypes of these diseases and therefore, considerably enrich our understanding of pathogenesis, disease mechanism and facilitate the development of drug screening platforms for novel therapeutic targets. Here, we review the accomplishments and the current progress in human neurological disorders by using i PSC modeling for Alzheimer's disease, Parkinson's disease, Huntington's disease, spinal muscular atrophy, amyotrophic lateral sclerosis, duchenne muscular dystrophy, schizophrenia and autism spectrum disorders, which include Timothy syndrome, Fragile X syndrome, Angelman syndrome, Prader-Willi syndrome, PhelanMc Dermid, Rett syndrome as well as Nonsyndromic Autism. | Fabiele B Russo Fernanda R Cugola Isabella R Fernandes Graciela C Pignatari Patricia C B Beltrão-Braga | 2015 | World Journal of Transplantation2015，5，4： | 4 |
| 2 | Quality of life, work productivity impairment and healthcare resources in inflammatory bowel diseases in Brazil显示文摘BACKGROUND Inflammatory bowel diseases(IBD)have been associated with a low quality of life(QoL)and a negative impact on work productivity compared to the general population.Information about disease control,patient-reported outcomes(PROs),treatment patterns and use of healthcare resources is relevant to optimizing IBD management.AIM To describe QoL and work productivity and activity impairment(WPAI),treatment patterns and use of healthcare resources among IBD patients in Brazil.METHODS A multicenter cross-sectional study included adult outpatients who were previously diagnosed with moderate to severe Crohn’s disease(CD)or ulcerative colitis(UC).At enrolment,active CD and UC were defined as having a Harvey Bradshaw Index≥8 or a CD Activity Index≥220 or calprotectin>200μg/g or previous colonoscopy results suggestive of inadequate control(per investigator criteria)and a 9-point partial Mayo score≥5,respectively.The PRO assessment included the QoL questionnaires SF-36 and EQ-5D-5L,the Inflammatory Bowel Disease Questionnaire(IBDQ),and the WPAI questionnaire.Information about healthcare resources and treatment during the previous 3 years was collected from medical records.Chi-square,Fisher’s exact and Student’s t-/Mann-Whitney U tests were used to compare PROs,treatment patterns and the use of healthcare resources by disease activity(α=0.05).RESULTS Of the 407 patients in this study(CD/UC:64.9%/35.1%,mean age 42.9/45.9 years,54.2%/56.6%female,38.3%/37.1%employed),44.7%/25.2%presented moderate-to-severe CD/UC activity,respectively,at baseline.Expressed in median values for CD/UC,respectively,the SF-36 physical component was 46.6/44.7 and the mental component was 45.2/44.2,the EQ-visual analog scale score was 80.0/70.0,and the IBDQ overall score was 164.0/165.0.Moderate to severe activity,female gender,being unemployed,a lower educational level and lower income were associated with lower QoL(P<0.05).Median work productivity impairment was 20%and 5%for CD and UC patients,respectively,and activity impairment was 30%,the latter being higher among patients with moderate to severe disease activity compared to patients with mild or no disease activity(75.0%vs 10.0%,P<0.001).For CD/UC patients,respectively,25.4%/2.8%had at least one surgery,38.3%/19.6%were hospitalized,and 70.7%/77.6%changed IBD treatment at least once during the last 3 years.The most common treatments at baseline were biologics(75.3%)and immunosuppressants(70.9%)for CD patients and 5-ASA compounds(77.5%)for UC patients.CONCLUSION Moderate to severe IBD activity,especially among CD patients,is associated with a substantial impact on QoL,work productivity impairment and an increased number of IBD surgeries and hospitalizations in Brazil. | Rogerio Serafim Parra Julio MF Chebli Heda MBS Amarante Cristina Flores Jose ML Parente Odery Ramos Milene Fernandes Jose JR Rocha Marley R Feitosa Omar Feres Antonio S Scotton Rodrigo B Nones Murilo M Lima Cyrla Zaltman Carolina D Goncalves Isabella M Guimaraes Genoile O Santana Ligia Y Sassaki Rogerio S Hossne Mauro Bafutto Roberto LK Junior Mikaell AG Faria Sender J Miszputen Tarcia NF Gomes Wilson R Catapani Anderson A Faria Stella CS Souza Rosana F Caratin Juliana T Senra Maria LA Ferrari | 2019 | World Journal of Gastroenterology2019，25，38： | 2 |
| 3 | Alliance management: a view from the past and a look to the future显示文摘 | Spckman R E Forbes T M Isabella L A Macavoy T C | 1998 | Journal of Management Studies1998，，35： | 1 |
| 4 | Identification and quantification of phenolic compounds in grapes by HPLC-PDA- ESI-MS on a semimicro separation scale显示文摘 | ISABELLA N CRISTIANO B ANTONELLA R | 2008 | J Agric Food Chern2008，56，19： | 1 |
| 5 | Creating strategic alliances which endure 显示文摘 | Spekman R E Isabella L A Thomas C | 1996 | Long Range Plan- ning1996，29，3： | 1 |
| 6 | Mutation of toxB and a truncated version of the efa-1 gene in Escherichia coli O157 : H7 influences the expression and secretion of locus of en- terocyte effacement-encoded proteins but not intestinal colonization in calves or sheep 显示文摘 | MARK P S ANDREW J R ISABELLA V | 2006 | Infection and Immunity2006，72，9： | 1 |
| 7 | Self-repairing systems based on ionomers and epoxidized natural rubber blends显示文摘 | Arifur R Maurizio P Isabella P | 2011 | Appl Mater In- terfaces2011，3，12： | 1 |
| 8 | Alliances Management: a View from Past and a Look to the Future显示文摘 | Spekman R E Isabella L A Macavor T C | 1998 | Journal of Management Studies1998，，6： | 1 |
| 9 | Alliance management: A view from the past and a look to the future 显示文摘 | SPEKMAN R E FORBES T M ISABELLA L A MACAVOR T C | 1998 | Journal of Management Studies1998，35，6： | 1 |
| 10 | Creating Strategic Alliances which Edure显示文摘 | Spekman R E Isabella L A MacAvoy T C | 1996 | Long Range Planning1996，，29： | 1 |
| 11 | Controlled insulin release from chitosan microparticles显示文摘 | FRANCESCA B MARIA A R ISABELLA O | 1998 | Arch Pharm Pharm Med Chem1998，331，： | 1 |
| 12 | Alliance management: A view from the past and a look to the future 显示文摘 | Spekman R E Forbes T M Isabella L A | 1998 | Journal of Management Studies1998，35，6： | 1 |
| 13 | Maternal Personal Exposure to Airborne Benzene and Intrauterine Growth显示文摘 | Slama Rémy Thiebaugeorges Olivier Goua Valérie Aussel Lucette Sacco Paolo Bohet Aline Forhan Anne Ducot Béatrice Annesi-Maesano Isabella Heinrich Joachim Magnin Guillaume Schweitzer Michel Kaminski Monique Charles Marie-Aline | 2009 | Environmental Health Perspectives2009，，8： | 1 |
| 14 | Alliance Management: A View from the Past and a Look to the Future显示文摘 | Spekman R E Forbes T M Isabella L A Macavor T C | 1998 | Journal of Management Studies1998，，35： | 1 |
| 15 | Alliance management:a view from the past and a look to the future显示文摘 | SPEKMAN R E FORBES T M ISABELLA L | 1998 | Journal of Management Studies1998，35，6： | 1 |
| 16 | Alliance management : a view from the past and a look to the future 显示文摘 | SPEKMAN R E FORBES Ⅲ T M ISABELLA L A MACAVOY T C | | Journal Of Management Studies0，35，6： | 1 |
| 17 | Protein carbonyl groups as biomarkers of oxidative stress显示文摘 | ISABELLA D R_ANIERI R DANIELA G | 2003 | Clin Chim Acta2003，，329： | 1 |
| 18 | Contribtution of SLN investigation with 99mWc nanocolloid in clifiical staging of thyroid cancer: technical feasibility显示文摘 | Maria R P Domenico R Isabella M | 2007 | Eur J Nucl Med Mol Imaging2007，34，6： | 1 |
| 19 | Association of dopamine D4 receptor (DRD4) exon Ⅲ repeat polymorphism with temperament in 3-year-old infants显示文摘 | Alessandro DL Mario R Isabella T | 2003 | Neurogenetics2003，4，4： | 1 |
| 20 | Alliance management: a view from the past and a look to the future显示文摘 | Spekman R E Forbes T M Isabella L A Macavoy T C | 1998 | Journal of Management Studies1998，35，6： | 1 |
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