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| 1 | Current and future therapies for inherited cholestatic liver diseases显示文摘Familial intrahepatic cholestasis(FIC) comprises a group of rare cholestatic liver diseases associated with canalicular transport defects resulting predominantly from mutations in ATP8B1, ABCB11 and ABCB4. Phe-notypes range from benign recurrent intrahepatic cholestasis(BRIC), associated with recurrent cholestatic attacks, to progressive FIC(PFIC). Patients often suffer from severe pruritus and eventually progressive cholestasis results in liver failure. Currently, first-line treatment includes ursodeoxycholic acid in patients with ABCB4 deficiency(PFIC3) and partial biliary diversion in patients with ATP8B1 or ABCB11 deficiency(PFIC1 and PFIC2). When treatment fails, liver transplantation is needed which is associated with complications like rejection, post-transplant hepatic steatosis and recurrence of disease. Therefore, the need for more and better therapies for this group of chronic diseases remains. Here, we discuss new symptomatic treatment options like total biliary diversion, pharmacological diversion of bile acids and hepatocyte transplantation. Furthermore, we focus on emerging mutation-targeted therapeutic strategies, providing an outlook for future personalized treatment for inherited cholestatic liver diseases. | Wendy L van der Woerd Roderick HJ Houwen Stan FJ van de Graaf | 2017 | World Journal of Gastroenterology2017,23,5: | 11 |
| 2 | 单一端元岩浆流体的减压沸腾作用是钨-锡矿形成的一种重要机制显示文摘全球范围内的锡(Sn)和钨(W)矿化普遍与高演化花岗岩有关,但是达到经济品位的矿床较少,且矿化的类型也各有不同,其矿床成因机制一直存在较大争议。近日,《地质学》杂志报道了德国地球科学研究中心的Maximilian Korges等人关于上述问题的研究成果。该团队以位于德国和捷克的Erzgebirge山脉的ZinnwaldS n-W矿床为研究对象,进行了系统的流体包裹体地球化学研究。 | Maximilian Korges Philipp Weis Volker Lüders Oscar Laurent 袁超 | 2018 | 矿物岩石地球化学通报2018,37,2: | 10 |
| 3 | 白喉-破伤风-无细胞百日咳-b型流行性感冒嗜血杆菌联合疫苗对中国婴儿安全性和免疫原性的研究显示文摘目的评价白喉-破伤风-无细胞百日咳-b型流行性感冒嗜血杆菌联合疫苗(Diphtheria,Tetanus,Acellular Pertussis and Haemophilus Influenzae Type b Combined Vaccine;DTaP-Hib),对中国婴儿的安全性和免疫原性。方法通过一项安全性研究(研究A)和一项安全性及免疫原性研究(研究B),评价DTaP-Hib联合疫苗接种于3、4、5月龄婴儿的安全性及免疫原性,并与DTaP联合疫苗及Hib疫苗(DTaP+Hib)同时分别接种进行比较。结果共690名健康婴儿接受了DTaP-Hib联合疫苗或DTaP联合疫苗与Hib疫苗分别同时接种的基础免疫。DTaP-Hib联合疫苗的耐受性与DTaP联合疫苗+Hib疫苗分别接种一致,两组局部及全身不良反应发生率差异无统计学意义。在接种DTaP-Hib联合疫苗的受试者中,至少有97.5%在基础免疫后一个月产生了针对白喉、破伤风、Hib的血清学保护性抗体,以及对无细胞百日咳抗原的免疫应答。按照预先制订的标准,DTaP-Hib联合疫苗的免疫应答与DTaP联合疫苗+Hib疫苗分别接种免疫应答应的差异无统计学意义。结论DTaP-Hib联合疫苗接种后可获得预期的安全性和免疫原性。临床试验注册国家食品药品监督管理局《药物临床批件》2003L03331。 | 李艳萍 张庶民 叶强 侯启明 李亚男 李红 徐颖华 马霄 刘幼平 陈晓玲 黄莉荣 Gunasekaran Ramakrishnan 李荣成 赵雷 唐海文 Olivier Van Der Meeren Hans L Bock | 2010 | 中国疫苗和免疫2010,16,2: | 7 |
| 4 | Near-infrared fluorescence imaging of a solitary fibrous tumor of the pancreas using methylene blue显示文摘A 67-year-old female presented with unexplained abdominal pain. A contrast-enhanced computed tomography scan of the abdomen incidentally revealed a mass in the uncinate process of the pancreas. This mass was resected and based on histopathological findings, diagnosed as a solitary fibrous tumor (SFT) of the pancreas. A SFT is an extremely rare benign mesenchymal tumor that in 65% of cases affects the visceral pleura but can also affect extra-pleural sites. The intraoperative demarcation of pancreatic tumors, such as SFTs, can bechallenging. In this report, the first clear intraoperative identification of a SFT of the pancreas in a human was shown using near-infrared fluorescence and methylene blue. | Joost R van der Vorst Alexander L Vahrmeijer Merlijn Hutteman Tjalling Bosse Vincent THBM Smit Cornelis JH van de Velde John V Frangioni Bert A Bonsing | 2012 | World Journal of Gastrointestinal Surgery2012,4,7: | 7 |
| 5 | Optimizing stem cells for cardiac repair:Current status and new frontiers in regenerative cardiology显示文摘Cell therapy has the potential to improve healing of ischemic heart, repopulate injured myocardium and restore cardiac function. The tremendous hope and potential of stem cell therapy is well understood, yet recent trials involving cell therapy for cardiovascular diseases have yielded mixed results with inconsistent data thereby readdressing controversies and unresolved questions regarding stem cell efficacy for ischemic cardiac disease treatment. These controversies are believed to arise by the lack of uniformity of the clinical trial methodologies, uncertainty regarding the underlying reparative mechanisms of stem cells, questions concerning the most appropriate cell population to use, the proper delivery method and timing in relation to the moment of infarction, as well as the poor stem cell survival and engraftment especially in a diseased microenvironment which is collectively acknowledged as a major hindrance to any form of cell therapy. Indeed, the microenvironment of the failing heart exhibits pathological hypoxic, oxidative and inflammatory stressors impairing the survival of transplanted cells. Therefore, in order to observe any significant therapeutic benefit there is a need to increase resilience of stem cells to death in the transplant microenvironment while preserving or better yet improving their reparative functionality. Although stem cell differentiation into cardiomyocytes has been observed in some instance, the prevailing reparative benefits are afforded through paracrine mechanisms that promote angiogenesis, cell survival, transdifferentiate host cells and modulate immune responses. Therefore, to maximize their reparative functionality, ex vivo manipulation of stem cells through physical, genetic and pharmacological means have shown promise to enable cells to thrive in the postischemic transplant microenvironment. In the present work, we will overview the current status of stem cell therapy for ischemic heart disease, discuss the most recurring cell populations employed, the mechanisms by which stem cells deliver a therapeutic benefit andstrategies that have been used to optimize and increase survival and functionality of stem cells including ex vivo preconditioning with drugs and a novel 'pharmacooptimizer' as well as genetic modifications. | Shant Der Sarkissian Thierry Lévesque Nicolas Noiseux | 2017 | World Journal of Stem Cells2017,9,1: | 6 |
| 6 | General competencies of problem‐based learning (PBL) and non‐PBL graduates显示文摘 | Katinka J A HPrince Patrick W L JVan Eijs Henny P ABoshuizen Cees P MVan Der Vleuten Albert J J AScherpbier | 2005 | Medical Education2005,,4: | 5 |
| 7 | Chronic intestinal failure and short bowel syndrome in Crohn’s disease显示文摘Chronic intestinal failure(CIF)is a rare but feared complication of Crohn’s disease.Depending on the remaining length of the small intestine,the affected intestinal segment,and the residual bowel function,CIF can result in a wide spectrum of symptoms,from single micronutrient malabsorption to complete intestinal failure.Management of CIF has improved significantly in recent years.Advances in home-based parenteral nutrition,in particular,have translated into increased survival and improved quality of life.Nevertheless,60%of patients are permanently reliant on parenteral nutrition.Encouraging results with new drugs such as teduglutide have added a new dimension to CIF therapy.The outcomes of patients with CIF could be greatly improved by more effective prevention,understanding,and treatment.In complex cases,the care of patients with CIF requires a multidisciplinary approach involving not only physicians but also dietitians and nurses to provide optimal intestinal rehabilitation,nutritional support,and an improved quality of life.Here,we summarize current literature on CIF and short bowel syndrome,encompassing epidemiology,pathophysiology,and advances in surgical and medical management,and elucidate advances in the understanding and therapy of CIF-related complications such as catheter-related bloodstream infections and intestinal failure-associated liver disease. | Aysegül Aksan Karima Farrag Irina Blumenstein Oliver Schröder Axel U Dignass Jürgen Stein | 2021 | World Journal of Gastroenterology2021,27,24: | 4 |
| 8 | Minimally invasive versus open oesophagectomy for patients with oesophageal cancer: a multicentre, open-label, randomised controlled trial显示文摘 | Surya SAY Biere Mark I van Berge Henegouwen Kirsten W Maas Luigi Bonavina Camiel Rosman Josep Roig Garcia Suzanne S Gisbertz Jean HG Klinkenbijl Markus W Hollmann Elly SM de Lange H Jaap Bonjer Donald L van der Peet Miguel A Cuesta | 2012 | The Lancet . 2012 (9829)2012,,: | 3 |
| 9 | Scintigraphy in laryngopharyngeal and gastroesophageal reflux disease:A definitive diagnostic test?显示文摘AIM: To investigate the utility of scintigraphic studies in predicting response to laparoscopic fundoplication(LF) for chronic laryngopharyngeal reflux symptoms.METHODS: Patients with upper aero-digestive symptoms that remained undiagnosed after a period of 2 mo were studied with conventional p H and manometric studies. Patients mainly complained of cough, sore throat, dysphonia and globus. These patients were imaged after ingestion of 99m-technetium diethylene triamine pentaacetic acid. Studies were quantified with time activity curves over the pharynx, upper and lower oesophagus and background. Late studies of the lungs were obtained for aspiration. Patients underwent LF with post-operative review at 3 mo after surgery.RESULTS: Thirty four patients(20 F, 14 M) with an average age of 57 years and average duration of symptoms of 4.8 years were studied. Twenty four hour p H and manometry studies were abnormal in all patients. On scintigraphy, 27/34 patients demonstrated pharyngeal contamination and a rising or flat pharyngeal curve. Lung aspiration was evident in 50% of patients. There was evidence of pulmonary aspiration in 17 of 34 patients in the delayed study(50%). Pharyngeal contamination was found in 27 patients. All patients with aspiration showed pharyngeal contamination. In the 17 patients with aspiration, graphical time activity curve showed rising activity in the pharynx in 9 patients and a flat curve in 8 patients. In those 17 patients without pulmonary aspiration, 29%(5 patients) had either a rising or flat pharyngeal graph. A rising or flat curve predicted aspiration with a positive predictive value of 77% and a negative predictive valueof 100%. Over 90% of patients reported a satisfactory symptomatic response to LF with an acceptable sideeffect profile. CONCLUSION: Scintigraphic reflux studies offer a good screening tool for pharyngeal contamination and aspiration in patients with gastroesophageal reflux disease. | Gregory L Falk John Beattie Alvin Ing SE Falk Michael Magee Leticia Burton Hans Van der Wall | 2015 | World Journal of Gastroenterology2015,21,12: | 2 |
| 10 | Possibilities and limits of infrared light microthermometry applied to studies of pyrite-hosted fluid inclusions显示文摘 | Volker Lüders Martin Ziemann | 1999 | Chemical Geology1999,,1: | 2 |
| 11 | Endometrial receptivity in an in vitro fertilization program as assessed by spiral artery blood flow, endometrial thickness, endometrial volume, and uterine artery blood flow显示文摘 | Ralf L Schild Christine Knobloch Christoph Dorn Rolf Fimmers Hans van der Ven Manfred Hansmann | 2001 | Fertility and Sterility2001,,2: | 2 |
| 12 | Reverse Structural and Gap-Junctional Remodeling After Prolonged Atrial Fibrillation in the Goat显示文摘 | Jannie Ausma Huub M.W. van der Velden Marie-Hélène Lenders Erwin P. van Ankeren Habo J. Jongsma Frans C.S. Ramaekers Marcel Borgers Maurits A. Allessie | 2003 | Circulation: Journal of the American Heart Association2003,,15: | 2 |
| 13 | Itraconazole oral solution and intravenous formulations:a review of pharmacokinetics and pharmacodynamics显示文摘 | Willems L van der Geest R de Beule K | | 0,,: | 2 |
| 14 | Flow-Injection Chemiluminescence Determination of Chlorpromazine Hydrochloride Applying the Luminol-Hydrogen Peroxide-Chromium ( Ⅲ ) System 显示文摘 | Wang L Baeyens W R G Van Der Weken G | 2000 | Luminescence2000,15,: | 2 |
| 15 | Efficient photon coupling from a diamond nitrogen vacancy center by integration with silica fiber显示文摘A central goal in quantum information science is to efficiently interface photons with single optical modes for quantum networking and distributed quantum computing.Here,we introduce and experimentally demonstrate a compact and efficient method for the low-loss coupling of a solid-state qubit,the nitrogen vacancy(NV)center in diamond,with a single-mode optical fiber.In this approach,single-mode tapered diamond waveguides containing exactly one high quality NV memory are selected and integrated on tapered silica fibers.Numerical optimization of an adiabatic coupler indicates that near-unity-efficiency photon transfer is possible between the two modes.Experimentally,we find an overall collection efficiency between 16%and 37%and estimate a single photon count rate at saturation above 700 kHz.This integrated system enables robust,alignment-free,and efficient interfacing of single-mode optical fibers with single photon emitters and quantum memories in solids. | Rishi N Patel Tim Schröder Noel Wan Luozhou Li Sara L Mouradian Edward H Chen Dirk R Englund | 2016 | Light(Science & Applications)2016,5,1: | 2 |
| 16 | Accuracy assessment of satellite derived land-cover data: a review显示文摘 | Janssen L F F J M van der Wel | 1994 | Photogrammetric Engineering and Remote Sensing1994,60,4: | 2 |
| 17 | Anticipation and Response: Emergency Services in Severe Weather Situations in Germany显示文摘Communicating meteorological uncertainty allows earlier provision of information on possible future events. The desired benefit is to enable the end-user to start with preparatory protective actions at an earlier time based on the end-user's own risk assessment and decision threshold. The presented results of an interview study,conducted with 27 members of German civil protection authorities, show that developments in meteorology and weather forecasting do not necessarily fit the current practices of German emergency services. These practices are mostly carried out based on alarms and ground truth in a superficial reactive manner, rather than on anticipation based on prognoses or forecasts. Emergency managers cope with uncertainty by collecting, comparing, and blending different information about an uncertain event and its uncertain outcomes within the situation assessment to validate the information. Emergency managers struggle most with an increase of emergency calls and missions due to the impacts of severe weather. Because of the additional expenditures, the weather event makes it even harder for them to fulfill their core duties. These findings support the need for impact-based warnings. | Thomas Kox Catharina Lüder Lars Gerhold | 2018 | International Journal of Disaster Risk Science2018,9,1: | 2 |
| 18 | A new class of tobacco chitinase homologous to bacterial exochitinases displays antifungal activity显示文摘 | Melchers L S Apothekerde Groot M van der Knaap J A | 1994 | Plant Journal1994,,5: | 1 |
| 19 | Optimization algorithms for structural reliability 显示文摘 | Liu P L Der Kiureghian A | 1991 | Structural Safety1991,9,3: | 1 |
| 20 | ZNF265--a novel spliceosomal protein able to induce alternative splicing显示文摘 | Adams D J Van der Weyden L Mayeda A | 2001 | J Cell Biol2001,154,1: | 1 |