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| 1 | 关于孤立特发性中枢性性腺功能低下(ICH)遗传病因的新发现显示文摘特发性低促性腺激素性性功能减退症(IHH)是一种罕见的疾病,它的病症表现为青春期的推迟或者缺失,以及(或者)不育症。这些病症是由于促性腺激素释放激素(GnRH)的作用未能对正常的垂体.性腺轴产生足够的刺激。由于罹患该病的患者体内的促卯泡激素(FSH)和黄体生成素(LH)的水平偏低或正常,所以采用孤立特发性中枢性性腺功能低下(ICH)这一术语定义该疾病可能更为合适。该疾病应区别于伴有其它垂体缺陷的中枢性性腺功能减退。孤立特发性中枢性性腺功能低下的发病机制比较复杂,且在男性中的发病率比中枢性性腺功能减退多5倍。大部分情况下,该病的患病人群比较分散,但也曾经报道过几例家族病例。这一发现加上其它很多致病基因突变的调查,以及一些基因敲除模型的出现,证明了该疾病的发病可能有一个强大的遗传学因素存在。孤立特发性中枢性性腺功能低下可能与一些包含锇缺乏在内的形态遗传学异常有关联,而锇缺乏与孤立特发性中枢性性腺功能低下构成了卡尔曼氏综合症(KS)的主要病征。在全部的孤立特发性中枢性性腺功能低下病例中,卡尔曼氏综合症约占40%,也被视作一个特殊的患者子群体。然而,经过对患有孤立的锇缺陷或者卡尔曼氏综合症(KS)亦或孤立特发性中枢性性腺功能低下的包含亲戚在内的几个家族谱系内部的病患的调查,证明孤立特发性中枢性性腺功能低下是一种复杂的遗传性疾病,它具有复杂多样的表征以及显性。由此得出,不管是多个基因变异还是环境因素或者核外遗传的变化都有可能导致复杂多样的病征。本文综述了己知的、与孤立特发性中枢性性腺功能低下的发病机制相关的遗传机制,并且对由意大利孤立特发性中枢性性腺功能低下网络合作中心所收集的227例病例作出临床概述。 | Marco Bonomi Domenico Vladimiro Libri Fabiana Guizzardi Elena Guarducci Elisabetta Maiolo Elisa Pignatti Roberta Asci Luca Persani | 2012 | Asian Journal of Andrology2012，14，1： | 10 |
| 2 | Successful Large-scale Renewables Integration in Portugal:Technology and Intelligent Tools显示文摘Portugal is seen worldwide as a case of success in the large-scale integration of renewables in its power system,especially for wind power.Consistent policies and sound management decisions are fundamental,but a sustainable process is not possible without the development of endogenous knowledge.This paper summarizes a set of models,both applied by the industry and representing actual technologic advancement,denoting the context of research and innovation in the country that helps to explain such success.Novelties arise in reliability assessment for systems with renewables,active and reactive power control,integration of wind farms,storage,electric vehicle integration,wind and solar power forecasting and distribution operation and state estimation taking advantage of smart grid structures.In all cases,one relevant trait is evident:the pervasive use of computational intelligence tools. | Vladimiro Miranda | 2017 | CSEE Journal of Power and Energy Systems2017，3，1： | 7 |
| 3 | Fiber Laser Sensor Based on a Phase-Shifted Chirped Grating for Acoustic Sensing of Partial Discharges显示文摘 | Sanderson E. U. LIMA Rubem G FARIAS Francisco M. ARAUJO Luis A. FERREIRA José L. SANTOS Vladimiro MIRANDA1 Orlando FRAZAO | 2013 | Photonic Sensors2013，3，1： | 5 |
| 4 | Recovery of rare earth elements adsorbed on clay minerals: I. Desorption mechanism显示文摘 | Georgiana A. Moldoveanu Vladimiros G. Papangelakis | 2012 | Hydrometallurgy2012，，： | 2 |
| 5 | Genetic algorithms in optimal multistage distribution network planning显示文摘 | Vladimiro Miranda Ranito J V Proenca L M | 2000 | IEEE Transaction on Power Systems2000，9，4： | 1 |
| 6 | Genetic algorithms in optimalmultistage distribution network planning显示文摘 | Vladimiro M Ranito J V Pmenca L M | 1994 | IEEE Transactions onPower Systems1994，9，4： | 1 |
| 7 | Comparison of selected methods of assessing freshness quality and remaining storage life of iced gilthead sea bream ( Sparus aurata )显示文摘 | Vladimiros P. Lougovois Eustratios R. Kyranas Vasiliki R. Kyrana | 2002 | Food Research International2002，，6： | 1 |
| 8 | Gearshift control for automated manual transmissions 显示文摘 | Luigi Glielmo Luigi lannelli Vladimiro Vacca | 2006 | IEEE/ASME Transactions on Mechatronic2006，11，1： | 1 |
| 9 | Economic dispatch model with fuzzy wind constraints and attitudes of dispatchers显示文摘 | Vladimiro Miranda Pun Sio Hang | 2005 | IEEE Trans on Power Systems2005，20，4： | 1 |
| 10 | CHF5074, a Novel Γ-Secretase Modulator, Restores Hippocampal Neurogenesis Potential and Reverses Contextual Memory Deficit in a Transgenic Mouse Model of Alzheimer’s Disease显示文摘 | Bruno P. Imbimbo Luciana Giardino Sandra Sivilia Alessandro Giuliani Marco Gusciglio Vladimiro Pietrini Elda Del Giudice Antonello D'Arrigo Alberta Leon Gino Villetti Laura Calzà | 2010 | Journal of Alzheimer’s Disease2010，，1： | 1 |
| 11 | Behaviour of cobalt during sulphuric acid pressure leaching of a limoniic laterite显示文摘 | Dimitri Georgiou Vladimiros G Papangelakis | 2009 | Hydrometallurgy2009，，100： | 1 |
| 12 | Knowledge discovery in neural networks with application to transformer failure diagnosis显示文摘 | Adriana Rosa Garcez Castro Vladimiro Miranda | 2005 | Power Systems2005，20，2： | 1 |
| 13 | Oxidative stability ofpork meat lipids as related to high-loeic sunflower oil andvitamin E diet supplementation and storage conditions 显示文摘 | Vladimiro C Maria T R Fabio C | 2011 | Meat Science2011，88，2： | 1 |
| 14 | Cleaning of waste smelter slags and recovery of valuable metals by pressure oxidative leaching显示文摘 | YunJiao Li Perederiy llya Vladimiros G | 2008 | Journal of Hazardous Materials2008，152，： | 1 |
| 15 | Knowledge discovery in neural networks with application to transformer failure diagnosis显示文摘 | Gardez C A R Vladimiro M | 2005 | IEEE Trans on Power Systems2005，20，2： | 1 |
| 16 | Economic dis-patch model with fuzzy wind constraints and attitudes of dispatchers 显示文摘 | Vladimiro Miranda Pun Sio Hang | 2005 | IEEE Transactions on power systems2005，20，4： | 1 |
| 17 | Knowledge discovery in neural networks with application to trans- former failure diagnosis 显示文摘 | Adriana Rosa Garcez Castro Vladimiro Miranda | 2005 | Power Systems2005，20，2： | 1 |
| 18 | Economic dis- patch model with fuzzy wind constraints and atti- tudes of dispatchers 显示文摘 | Vladimiro Miranda Pun Sio Hang | 2005 | IEEE Trans on Power Systems2005，20，4： | 1 |
| 19 | Late Electrical and Mechanical Remodeling After Atrial Septal Defect Closure in Children: Surgical Versus Percutaneous Approach显示文摘 | Biagio Castaldi Vladimiro L. Vida Anna Argiolas Nicola Maschietto Alessia Cerutti Dario Gregori Giovanni Stellin Ornella Milanesi | 2015 | The Annals of Thoracic Surgery2015，，1： | 1 |
| 20 | Comparison of selected methods of assessing freshness quality and remaining storage life of iced gilthead sea bream(sparus aurata)显示文摘 | Vladimiros P L Eustratios R K Vasiliki R K | 2003 | Food Research Internatinal2003，36，： | 1 |
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