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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 | Coxsackievirus mutants that can bypass host factor PI4KIIIβ and the need for high levels of PI4P lipids for replication显示文摘 | Hilde M van der Schaar Lonneke van der Linden Kjerstin H W Lanke Jeroen R P M Strating Gerhard Purstinger Erik de Vries Cornelis A M de Haan Johan Neyts Frank J M van Kuppeveld | 2012 | Cell Research2012，22，11： | 3 |
| 3 | A PPARγ-FGF1 axis is required for adaptive adipose remodelling and metabolic homeostasis显示文摘 | JONKER Johan W MYOUNG SUH Jae ATKINS Annette R | | 0，，7398： | 1 |
| 4 | Niches in evolutionary theories of technical change: a critical survey of the literature 显示文摘 | Johan Schot Frank W Geels | 2007 | Evol Econ2007，17，： | 1 |
| 5 | Strategic niche manage- ment and sustainable innovation journeys: theory, findings, re- search agenda, and policy显示文摘 | JOHAN SCHOT FRANK W GEELS | 2008 | Technology Analysis & Strategic Management2008，20，5： | 1 |
| 6 | A century of gestalt psychology in visual perception: perceptual groupingand figure ground organization显示文摘 | JOHAN W JAMES H E MICHAEL K | 2012 | Psychological Bulletin2012，138，6： | 1 |
| 7 | The endoxylanases from family 11: computer analysis of protein sequences reveals important structural and phylogenetic relationships显示文摘 | Amalia S Johan W Christophe L | 2002 | Journal of Biotechnology2002，95，2： | 1 |
| 8 | Mammalian drug efflux transporters of the ATP binding cassette (ABC) family: an overview显示文摘 | Alfred H Schinkel Johan W Jonker | 2003 | Advanced Drug Delivery Reviews2003，，1： | 1 |
| 9 | Side effect of endotra- cheal suction in pressure-and volume controlled ventilation显示文摘 | Birgitta A Carl Johan W Erkki H | 2004 | Chest2004，125，3： | 1 |
| 10 | Antitumor effect radioactive cisplatin on nude mice显示文摘 | Johan A Johan W Anders J | 2001 | Int J Radiat Oncol Biol Phy2001，49，3： | 1 |
| 11 | Mineral absorption and excretion as affected by microbial phytase,and their effect on energy metabolism in young piglets 显示文摘 | Arie K K Walter J J G Johan W S | 2005 | J Nutr2005，135，： | 1 |
| 12 | Neo-adjuvant chemotherapy followed by surgery and chemotherapy or by surgery and chemoradiotherapy for patients with resectable gastric cancer (CRITICS)显示文摘 | Johan L Dikken Johanna W van Sandick HA Maurits Swellengrebel | | 0，，： | 1 |
| 13 | Short circuit current of wind turbines with doubly fed induction generator显示文摘 | Johan M Sjoerd W H | 2007 | IEEE Transactions on Energy Conversion2007，22，1： | 1 |
| 14 | Behavior of a moored ship in unsteady current, wind and waves显示文摘 | Jan O de Kat Johan E W Wiehers | 1991 | Marine Technology1991，，： | 1 |
| 15 | Ridethrough of wind turbines with doubly-fed induction generator during a voltage dip显示文摘 | Johan Morren Haan S W H de | 2005 | IEEE Trans on Energy Conversion2005，20，2： | 1 |
| 16 | Quality of life and metabolic status in mildly depressed women with type 2 diabetes treated with paroxetine:A single-blind randomized placebo controlled trial 显示文摘 | Maria PH Kristian W Johan G | 2003 | BMC family practice2003，14，： | 1 |
| 17 | Antitumer effect radioactive eisplatin on mude mice显示文摘 | Johan A Johan W Anders J | 2001 | Int J Radial Oncol Biol Phy2001，49，3： | 1 |
| 18 | Ride through of wind turbines with doubly-fed induction generator during a voltage dip显示文摘 | Johan M Sjoerd W H | 2005 | IEEE Transaction on Energy Conversion2005，20，2： | 1 |
| 19 | Strategic niche management and sustainable innovation journeys : theory, findings, re- search agenda, and policy显示文摘 | Johan Scbot Frank W Geels | 2008 | Technology Analysis & stra- tegic management2008，20，5： | 1 |
| 20 | Collaborative trial validation study of two methods, one based on high performance liquid chromatography tandem mass spectrometry and on gas chromatography - mass spectrometry for the determination of acrylarnide in bakery and potato products 显示文摘 | Thomas W Lubomir K Johan R | 2006 | Journal of Chromatography A2006，1132，： | 1 |
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