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| 1 | Molecular overview of progressive familial intrahepatic cholestasis显示文摘Cholestasis is a clinical condition resulting from the imapairment of bile flow.This condition could be caused by defects of the hepatocytes,which are responsible for the complex process of bile formation and secretion,and/or caused by defects in the secretory machinery of cholangiocytes.Several mutations and pathways that lead to cholestasis have been described.Progressive familial intrahepatic cholestasis(PFIC)is a group of rare diseases caused by autosomal recessive mutations in the genes that encode proteins expressed mainly in the apical membrane of the hepatocytes.PFIC 1,also known as Byler’s disease,is caused by mutations of the ATP8B1 gene,which encodes the familial intrahepatic cholestasis 1 protein.PFIC 2 is characterized by the downregulation or absence of functional bile salt export pump(BSEP)expression via variations in the ABCB11 gene.Mutations of the ABCB4 gene result in lower expression of the multidrug resistance class 3 glycoprotein,leading to the third type of PFIC.Newer variations of this disease have been described.Loss of function of the tight junction protein 2 protein results in PFIC 4,while mutations of the NR1H4 gene,which encodes farnesoid X receptor,an important transcription factor for bile formation,cause PFIC 5.A recently described type of PFIC is associated with a mutation in the MYO5B gene,important for the trafficking of BSEP and hepatocyte membrane polarization.In this review,we provide a brief overview of the molecular mechanisms and clinical features associated with each type of PFIC based on peer reviewed journals published between 1993 and 2020. | Sriram Amirneni Nils Haep Mohammad A Gad Alejandro Soto-Gutierrez James E Squires Rodrigo MFlorentino | 2020 | World Journal of Gastroenterology2020，26，47： | 17 |
| 2 | 硬膜外低压麻醉在髋关节置换中预防血栓性疾病的作用显示文摘目的研究全髋关节置换术中使用硬膜外低压麻醉的有效性和安全性。方法对993例全髋关节置换术采用硬膜外低压麻醉(平均动脉压维持在45~55 mm Hg)。处理股骨侧髓腔之前2~3 min,静脉给予肝素10~15 U/kg。结果本组平均手术时间83 min(35~268 min),平均失血量183 ml(50~1 400 ml)。术后随访3个月,共有13例发生血栓性疾病,占总数的1.3%,其中确诊为下肢深静脉血栓(DVT)的有10例,发生率为1.0%。无心血管疾病、硬膜外血肿发生。随访期间无死亡病例。结论全髋关节置换术中使用硬膜外低压麻醉安全,术中出血少,术后血栓发生率低,术后无相关的并发症。 | 鲁宁 杨阳 思永玉 Nigel E Sharrock Alejandro González Della Valle Eduardo A.Salvati | 2012 | 中国骨与关节损伤杂志2012，27，12： | 5 |
| 3 | Predicting survival after liver transplantation in patients with hepatocellular carcinoma beyond the Milan criteria: a retrospective, exploratory analysis显示文摘 | Vincenzo Mazzaferro Josep M Llovet Rosalba Miceli Sherrie Bhoori Marcello Schiavo Luigi Mariani Tiziana Camerini Sasan Roayaie Myron E Schwartz Gian Luca Grazi René Adam Peter Neuhaus Mauro Salizzoni Jordi Bruix Alejandro Forner Luciano De Carlis Umberto | 2009 | Lancet Oncology2009，，1： | 4 |
| 4 | Delayed reconstruction of lateral complex structures of the ankle显示文摘Lateral ankle instability is one of the most common and well-recognized conditions presenting to foot and ankle surgeons.It may exist as an isolated entity or in conjunction with other concomitant pathology,making it important to appropriately diagnose and identify other conditions that may need to be addressed as part of treatment.These associated conditions may be a source of chronic pain,even when the instability has been appropriately treated,or may lead to failure of treatment by predisposing the patient to ankle inversion injuries.The primary goal of this editorial is to provide a brief summary of the common techniques used in the delayed reconstruction of lateral ankle ligamentous injuries and present a method we have successfully employed for over 15 years.We will also briefly discuss the diagnosis and treatment of the more common associated conditions,which are important to identify to achieve satisfactory results for the patient.We present the outcomes of 250 consecutive reconstructions performed over the last 10 years and describe our operative technique for addressing lateral ankle ligamentous injuries. | Gordon L Slater Alejandro E Pino Martin O’Malley | 2011 | World Journal of Orthopedics2011，2，4： | 2 |
| 5 | An alterna- tive methodology to predict aging effects on the mechanical properties of glass fiber reinforced cements (GRC)显示文摘 | ALEJANDRO E LAURA S P VICENTE S G | 2012 | Con- struction and Building Materials2012，27，1： | 1 |
| 6 | Lysophosphatidic acid and endothelin-induced proliferation of ovarian cancer cell lines is mitigated by neutralization of granulin epithelin precursor (GEP), a prosurvival factor for ovarian cancer 显示文摘 | KAMRAVA M SIMPKINS F ALEJANDRO E | 2005 | Oncogene2005，24，47： | 1 |
| 7 | Blood pressure and body water distribution in chronic renal failure patients显示文摘 | Maria A Alejandro M Mario E | 2001 | Nephrol Dial Transplant2001，16，1： | 1 |
| 8 | Impact of Hillslopescale Organization of Topography, Soil Moisture, Soil Temperature,and Vegetation on Modeling Surface Microwave Radiation Emission显示文摘 | Alejandro N F Valeriy Y I Dara E | 2009 | IEEE Transactions on Geoscience Remote Sensing2009，47，8： | 1 |
| 9 | OPC Based Distributed Real Time Simulation of Complex Continuous Processes 显示文摘 | Raul Alves Santos Julio E Normey-Rico Alejandro Merino Gomez | 2005 | Simulation Modelling Practice and Theory2005，13，7： | 1 |
| 10 | Insulin protects islets from apoptosis via Pdx1 and specific changes in the human islet proteome显示文摘 | Johnson J D Bernal-Mizrachi E Alejandro E U | 2006 | Proc Natl Acad Sci USA2006，103，19： | 1 |
| 11 | A novel methodology for the monitoring of the agricultural production process based on wireless sensor networks 显示文摘 | Soledad E D Jesu C P Alejandro C M | 2011 | Computers and Electronics in Agriculture2011，76，2： | 1 |
| 12 | Performance evaluation of MAC transmission power control in wireless sensor networks 显示文摘 | Javier V A Esteban E L Alejandro M S | 2007 | Computer Networks2007，51，6： | 1 |
| 13 | Predicting survival after liver transplantation in patients with hepatocellular carcinoma beyond the Milan criteria: a retrospective, exploratory analysis显示文摘 | Vincenzo Mazzaferro Josep M Llovet Rosalba Miceli Sherrie Bhoori Marcello Schiavo Luigi Mariani Tiziana Camerini Sasan Roayaie Myron E Schwartz Gian Luca Grazi René Adam Peter Neuhaus Mauro Salizzoni Jordi Bruix Alejandro Forner Luciano De Carlis Umberto | 2009 | Lancet Oncology2009，，1： | 1 |
| 14 | Mesenchymal Stem Cell显示文摘 | JOSEJ M ALEJANDRO E PAULETTE C | 2001 | SEBM2001，226，： | 1 |
| 15 | Tracking of Returnable Packaging and Transport Units with Active RFID in the Grocery Supply Chain 显示文摘 | Alejandro S Esteban E L Felipe G S | 2009 | Computers in Industry2009，60，3： | 1 |
| 16 | Predicting survival after liver transplantation in patients with hepatocellular carcinoma beyond the Milan criteria: a retrospective, exploratory analysis显示文摘 | Vincenzo Mazzaferro Josep M Llovet Rosalba Miceli Sherrie Bhoori Marcello Schiavo Luigi Mariani Tiziana Camerini Sasan Roayaie Myron E Schwartz Gian Luca Grazi René Adam Peter Neuhaus Mauro Salizzoni Jordi Bruix Alejandro Forner Luciano De Carlis Umberto | 2009 | Lancet Oncology2009，，1： | 1 |
| 17 | Primiceri, Andrea Tambalotti, 2010, Investment Shocks andBusiness Cycles 显示文摘 | Justiniano Alejandro Giorgio E | 2010 | Journal of Monetary Economics2010，，： | 1 |
| 18 | Category-based induction显示文摘 | Daniel N Osherson Smith E E Ormond Wilkie Alejandro Lopez Eldar Shafir | 1990 | Psycho- logical Review1990，，97： | 1 |
| 19 | Frequency regulation contribution through variable- speed wind energy conversion systems 显示文摘 | JUAN M ALEJANDRO M ANTONIO G E | 2009 | IEEE Transactions on Power Systems2009，24，1： | 1 |
| 20 | Maternal diet-induced micrornas and mtor underlie beta cell dysfunction in offspring 显示文摘 | Alejandro E U Gregg B Wallen T | 2014 | J Clin Invest2014，124，10： | 1 |
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