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| 1 | Wilson's disease: Prospective developments towards new therapies显示文摘Wilson's disease(WD) is an autosomal recessive disorder of copper metabolism, caused by mutations in the ATP7 B gene. A clear demand for novel WD treatment strategies has emerged. Although therapies using zinc salts and copper chelators can effectively cure WD, these drugs exhibit limitations in a substantial pool of WD patients who develop intolerance and/or severe side effects. Several lines of research have indicated intriguing potential for novel strategies and targets for development of new therapies. Here, we review these new approaches, which comprise correction of ATP7 B mutants and discovery of new compounds that circumvent ATP7B-deficiency, as well as cell and gene therapies. We also discuss whether and when these new therapeutic strategies will be translated into clinical use, according to the key requirements for clinical trials that remain to be met. Finally, we discuss the hope for the current rapidly developing research on molecular mechanisms underlying WD pathogenesis and for the related potential therapeutic targets to provide a solid foundation for the next generation of WD therapies that may lead to an effective, tolerable and safe cure. | Giusy Ranucci Roman Polishchuck Raffaele Iorio | 2017 | World Journal of Gastroenterology2017，23，30： | 6 |
| 2 | 左心室成形术临床应用评价显示文摘目的 总结左心室成形术治疗缺血性心肌病的临床应用经验。方法 1998至 2 0 0 0年意大利米兰SanDonato医院收治合并室壁瘤的缺血性心肌病病人 4 6例。在低温体外循环下完成冠状动脉旁路移植远端吻合后切开心尖部室壁瘤 ,沿室壁瘤边缘行心室内环缩成形术 (Dor手术 ) ,之后用补片或直接闭合室壁瘤壁 ,重塑左室形态。结果 围术期死亡 7例 ,死亡率为 15 2 % ;术后左室收缩末期及舒张末期容积均显著降低 (P <0 0 0 1) ;肺动脉压亦显著下降 (P <0 0 5 )。中期随访生存率为 71 8%。 | 李温斌 Menicanti L Di Donato M Santambrogio C Ranucci M San Donato | 2004 | 中华胸心血管外科杂志2004，20，3： | 2 |
| 3 | Multiple electrode whole-blood aggregometry and bleeding in cardiac surgery patients receiving thienopyridines 显示文摘 | RANUCCI M BARYSHNIKOVA E SORO G | 2011 | Ann Thorac Surg2011，91，1： | 1 |
| 4 | Transcatheter aortic valve implan- tation versus surgical aortic valve replacement for severe aortic stenosis: results from an intermediate risk propensity-matched population of the Italian OBSERVANT study显示文摘 | D' Errigo P Barhanti M Ranucci M | 2013 | Int J Cardiol2013，167，5： | 1 |
| 5 | Biodegradable polymers from renewable sources: rheological characterization of hemicellulose-based hydrogels 显示文摘 | Lindblad M S Albertsson A C Ranucci E | 2005 | Biomacromolecules2005，6，2： | 1 |
| 6 | A Sampling Board Optimized for Pulse Shape Discrimination in Liquid Scintillator Applications显示文摘 | Ranucci G | 2004 | IEEE Trans Nucl Sci2004，51，4： | 1 |
| 7 | Biodegradable polymers from renewable sources: New hemicellulose-based hydrogels显示文摘 | Lindblad M S Ranucci E Albertsson A C | 2001 | Macromolecular Rapid Communications2001，22，12： | 1 |
| 8 | Risk factors for renal dysfunction after coronary surgery:the role of cardiopulmonary bypass technique显示文摘 | Ranucci M Pavesi M Mazza E | 1994 | Perfusion1994，9，5： | 1 |
| 9 | Hyperlactatemia during cardiopulmonary bypass:determinants and impact on postoperative outcome显示文摘 | Ranucci M De Toffol B Isgrò G | 2006 | Crit Care2006，10，6： | 1 |
| 10 | Preparation and in vitro evaluation of the antiviral activity of the Acyclovir complex of a β - cyclodextrin/poly (amidoamine) copolymer显示文摘 | BENCINI M RANUCCI E FERRUTI P | 2004 | Journal of Controlled Release2004，126，1： | 1 |
| 11 | Determinants of early dis- charge from the intensive care unit after cardiac operations 显示文摘 | Ranucci M Bellucci C Conti D | 2007 | Ann Thorac Surg2007，83，3： | 1 |
| 12 | Transcatheter aortic valve implantation versus surgical aortic valve re- placement for severe aortic stenosis : results from an inter- mediate risk propensity- matched population of the Italian OBSERVANT study显示文摘 | DErrigo P Barbanti M Ranucci M | 2013 | Int J Cardiol2013，167，5： | 1 |
| 13 | Risk of assessing mortality risk in elective cardiac operations age, creatinine, ejection fraction, and the law of parsimony显示文摘 | Ranucci M Castelvecchio S Menicanti L | 2009 | Circulation2009，119，： | 1 |
| 14 | Fenoldopam prophylaxis of postoperative acute renal failure in high-risk cardiac surgery patients 显示文摘 | RANUCCI M SORO G BARAZGHI N | 2004 | Ann Thorac Surg2004，78，4： | 1 |
| 15 | Normaothermic perfusion andlung function after cardiopulmonary bypass : effects in pulmonary riskpatients 显示文摘 | Ranucci M Soro G Frigiola A | 1997 | Per fusion1997，12，5： | 1 |
| 16 | Influence of the timing of cardiac catheterization and the amount of contrast media on acute renal failure after cardiac surgery显示文摘 | Ranucci M Ballotta A Kunkl A | 2008 | Am J Cardiol2008，101，8： | 1 |
| 17 | Total and differential cell count in milk of primiparous Comisana ewes without clinical signs of masdtis 显示文摘 | Morgante M Ranucci S Pauselli M | 1996 | Small Ruminant Research1996，21，： | 1 |
| 18 | Bioerodible hydrogels based on 2-hydroxyethyl methacrylate:synthesis and characterization 显示文摘 | Chiellini F Petrucci F Ranucci E | 2002 | J Appl Polym Sci2002，85，： | 1 |
| 19 | Surgical therapy for ischemic heart failure: single-center experience with surgical anterior ventricular restoration显示文摘 | Menicanti L Castelvecchio S Ranucci M | 2007 | J Thorac Cardiovasc Surg2007，134，2： | 1 |
| 20 | Hyperlactatemia during cardiopulmonary bypass:determinants and impact on postoperative outcome显示文摘 | RANUCCI M DE TOFFOL B ISGROG | 2006 | Crit Care2006，10，： | 1 |
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