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| 1 | Juvenile polyposis syndrome显示文摘Juvenile polyposis syndrome is a rare autosomal dominant syndrome characterized by multiple distinct juvenile polyps in the gastrointestinal tract and an increased risk of colorectal cancer.The cumulative life-time risk of colorectal cancer is 39% and the relative risk is 34.Juvenile polyps have a distinctive histology characterized by an abundance of edematous lamina propria with inflammatory cells and cystically dilated glands lined by cuboidal to columnar epithelium with reactive changes.Clinically,juvenile polyposis syndrome is defined by the presence of 5 or more juvenile polyps in the colorectum,juvenile polyps throughout the gastrointestinal tract or any number of juvenile polyps and a positive family history of juvenile polyposis.In about 50%-60% of patients diagnosed with juvenile polyposis syndrome a germline mutation in the SMAD4 or BMPR1A gene is found.Both genes play a role in the BMP/TGF-beta signalling pathway.It has been suggested that cancer in juvenile polyposis may develop through the socalled 'landscaper mechanism' where an abnormal stromal environment leads to neoplastic transformation of the adjacent epithelium and in the end invasive carcinoma.Recognition of this rare disorder is important for patients and their families with regard to treatment,follow-up and screening of at risk individuals.Each clinician confronted with the diagnosis of a juvenile polyp should therefore consider the possibility of juvenile polyposis syndrome.In addition,juvenile polyposis syndrome provides a unique model to study colorectal cancer pathogenesis in general and gives insight in the molecular genetic basis of cancer.This review discusses clinical manifestations,genetics,pathogenesis and management of juvenile polyposis syndrome. | Lodewijk AA Brosens Danielle Langeveld W Arnout van Hattem Francis M Giardiello G Johan A Offerhaus | 2011 | World Journal of Gastroenterology2011，17，44： | 7 |
| 2 | Central line-associated bloodstream infection among children with biliary atresia listed for liver transplantation显示文摘BACKGROUND Pre-transplant nutrition is a key driver of outcomes following liver transplantation in children.Patients with biliary atresia(BA) may have difficulty achieving satisfactory weight gain with enteral nutrition alone,and parenteral nutrition(PN) may be indicated.While PN has been shown to improve anthropometric parameters of children with BA listed for liver transplantation,less is known about the risks,particularly infectious,associated with this therapy among this specific group of patients.AIM To describe the incidence,microbiology,and risk factors of central line-associated bloodstream infection(CLABSI) among children with BA listed for liver transplantation.METHODS Retrospective review of children aged ≤ 2-years of age with BA who were listed for primary liver transplantation at Texas Children's Hospital from 2008 through2015(n = 96).Patients with a central line for administration of PN(n = 63) were identified and details of each CLABSI event were abstracted.We compared the group of patients who experienced CLABSI to the group who did not,to determine whether demographic,clinical,or laboratory factors correlated with development of CLABSI.RESULTS Nineteen of 63 patients(30%,95%CI:19,43) experienced 29 episodes of CLABSI during 4800 line days(6.04 CLABSI per 1000 line days).CLABSI was predominantly associated with Gram-negative organisms(14/29 episodes,48%)including Klebsiella spp.,Enterobacter spp.,and Escherichia coli.The sole polymicrobial infection grew Enterobacter cloacae and Klebsiella pneumoniae.Grampositive organisms(all Staphylococcus spp.) and fungus(all Candida spp.)comprised 9/29(31%) and 6/29(21%) episodes,respectively.No demographic,clinical,or laboratory factors were significantly associated with an increased risk for the first CLABSI event in Cox proportional hazards regression analysis CONCLUSION There is substantial risk for CLABSI among children with BA listed for liver transplantation.No clinical,demographic,or laboratory factor we tested emerged as an independent predictor of CLABSI.While our data did not show an impact of CLABSI on the short-term clinical outcome,it would seem prudent to implement CLABSI reduction strategies in this population to the extent that each CLABSI event represents potentially preventable hospitalization,unnecessary healthcare dollar expenditures,and may exact an opportunity cost,in terms of missed allograft offers. | Nicole D Triggs Stacey Beer Sonam Mokha Kat Hosek Danielle Guffey Charles G Minard Flor M Munoz Ryan W Himes | 2019 | World Journal of Hepatology2019，11，2： | 2 |
| 3 | 2009年南极冰穹A的双色天光背景统计和分析显示文摘天光背景是天文台址的一个重要指标。利用2009年位于南极冰穹A的CSTAR望远镜两个波段的观测数据,对南极冰穹A的天光背景进行了统计和分析。结果表明约74%的g波段天光背景的ADU值小于每像素100/s;90.5%的r波段的天光背景的ADU值小于每像素100/s。在不考虑大气消光的情况下,定标后的结果为2009年冰穹A的观测数据中天光背景亮度的中值:g波段为19.9 mag/arcsec2,r波段为20.1 mag/arcsec2。 | 宗伟凯 付建宁 牛家树 Ashley Michael C B 宫雪非 Lawrence J S 刘强 Luong-Van Daniel Pennypacker C R Storey John W V 王灵芝 王力帆 杨惠根 袁祥岩 York Donald G 朱镇熹 | 2014 | 天文研究与技术2014，11，1： | 2 |
| 4 | Activitybased costing accounting for a market orientation 显示文摘 | Daniel J G Greg W M William B C | 1998 | Industrial Marketing Management1998，，127： | 1 |
| 5 | Topology Discovery for Public IPv6 Networks 显示文摘 | Daniel G W Fangzhe C Ramesh V | 2003 | Computer Communications Review2003，33，3： | 1 |
| 6 | Evaluation of efficacy of an ultrasonic scalpel for pulmonary vascular ligation in an animal model显示文摘 | Daniel G Maoxin W Yun J | | 0，，01： | 1 |
| 7 | Regulation of the human catalytic subunit of telomerase (hTERT) 显示文摘 | Daniel M Peek G W Tollefsbol T O | 2012 | Gene2012，498，2： | 1 |
| 8 | Assessing Relationships Among Strategic Types,Distinctive Marketing Competencies and Organizational Performance显示文摘 | Arch G W Daniel P S Randolph J T | 1999 | Journal of Business research1999，，45： | 1 |
| 9 | Topology dis- covery for public IPv6 networks 显示文摘 | Daniel G W Fangzhe C Ramesh V | 2003 | Computer Communi- cations Review2003，33，3： | 1 |
| 10 | Solid-state modification of poly (butylene terephthalate)with a bio-based fatty acid dimer diol furnishing copolyesters with unique morphologies显示文摘 | Erik G Lidia J W Daniel H M | 2013 | Macromolecules2013，46，： | 1 |
| 11 | Bt toxin resistance from loss of a putative carbohydrate2 modifying enzyme 显示文摘 | Joel S G Johanna L W Daniel E S | 2001 | Science2001，293，3： | 1 |
| 12 | Generalized quadratic stability for perturbated singular systems显示文摘 | Lu G P Daniel W C Ho Yeung L F | 2006 | IEEE Transactions on Automatic Control2006，51，： | 1 |
| 13 | The early diagnosis of transient osteoporosis by magnetic resonance imaging: A case report显示文摘 | DANIEL W W SANDERS P C ALARCcóN G S | 1992 | J Bone Joint Surg1992，74，8： | 1 |
| 14 | The VS and V blood group polymorphisms in Africans: a serological and molecular analysis显示文摘 | Daniels G L Faas B W Green C A | 1998 | Transfusion1998，38，9： | 1 |
| 15 | Time on the market: The impact of residential brokerage显示文摘 | Donald J G Terry S G Daniel W T | 1996 | Journal of Real Estate Research1996，12，： | 1 |
| 16 | Xenon purity analysis for exo-200 via mass spectrometry 显示文摘 | Dobi A Hall C Slutsky S Yen Y R Aharmin B Auger M Barbeau P S Benitez-Medina C Breidenbach M Cleveland B Conley R Cook J Cook S Counts I Craddock W Daniels T Davis C G Davis J Devoe R Dixit M Dolinski M J Donato K Fairbank Jr W Farine J Fierlinger P Franco D Giroux G Gornea R Graham K Gratta G Green C Hagemann C Hall K Hallman D Hargrove C Herrin S Hughes M Hodgson J Juget F Karelin A Kaufman L J Kuchenkov A Kumar K Leonard D S Lutter G Mackay D Maclellan R Marino M Mong B Montero Diez M Morgan P MUller A R Neilson R Odian A Oullivan K Piepke A Pocar A Prescott C Y Pushkin K Rivas A Rollin E Rowson P C Sabourov A Sinclair D Skarpaas K Stekhanov V Strickland V Swift M Twelker K Vuilleumier J L Vuilleumier J M Weber M Wichoski U Wodin J Wright J D Yang L | 2012 | Nuclear Instruments and Methods in Physics Research Section A: Accelerators Spectrometers Detectors and Associated Equipment2012，675，： | 1 |
| 17 | Variation of modal parameters of a cable-stayed bridge identified from ambient vibration measurements and FE modelling显示文摘 | MACDONALD J H G DANIELL W E | | 0，，13： | 1 |
| 18 | The mammary gland development regulation and unetion显示文摘 | Daniel C W Silberstein G B | 1987 | New York:Plenum Press1987，，： | 1 |
| 19 | Modifiable Factors Associated With Improved Cardiac Arrest Survival in a Multicenter Basic Life Support/Defibrillation System: OPALS Study Phase I Results显示文摘 | Ian G Stiell George A Wells Valerie J DeMaio Daniel W Spaite Brian J Field Douglas P Munkley Marion B Lyver Lorraine G Luinstra Roxanne Ward | 1999 | Annals of Emergency Medicine1999，，： | 1 |
| 20 | Synthesis and properties of biocompatible water-soluble silica-coated CdSe/ZnS semiconductor quantum dots显示文摘 | Daniele G Shimon W Alivisatos A P | 2001 | J Phys Chem B2001，105，37： | 1 |
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