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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 | Molecular alterations in gastric cancer with special reference to the early-onset subtype显示文摘Currently, gastric cancer(GC) is one of the most frequently diagnosed neoplasms, with a global burden of 723000 deaths in 2012. It is the third leading cause of cancer-related death worldwide. There are numerous possible factors that stimulate the procarcinogenic activity of important genes. These factors include genetic susceptibility expressed in a singlenucleotide polymorphism, various acquired mutations(chromosomal instability, microsatellite instability, somatic gene mutations, epigenetic alterations) and environmental circumstances(e.g., helicobcter pylori infection, EBV infection, diet, and smoking). Most of the aforementioned pathways overlap, and authors agree that a clear-cut pathway for GC may not exist. Thus, the categorization of carcinogenic events is complicated. Lately, it has been claimed that research on early-onset gastric carcinoma(EOGC) and hereditary GC may contribute towards unravelling some part of the mystery of the GC molecular pattern because young patients are less exposed to environmental carcinogens and because carcinogenesis in this setting may be more dependent on genetic factors. The comparison of various aspects that differ and coexist in EOGCs and conventional GCs might enable scientists to: distinguish which features in the pathway of gastric carcinogenesisare modifiable, discover specific GC markers and identify a specific target. This review provides a summary of the data published thus far concerning the molecular characteristics of GC and highlights the outstanding features of EOGC. | Malgorzata Skierucha Anya NA Milne G Johan A Offerhaus Wojciech P Polkowski Ryszard Maciejewski Robert Sitarz | 2016 | World Journal of Gastroenterology2016，22，8： | 6 |
| 3 | Gastroenterologist perceptions of faecal microbiota transplantation显示文摘AIM: To explore gastroenterologist perceptions towards and experience with faecal microbiota transplantation(FMT).METHODS: A questionnaire survey consisting of 17 questions was created to assess gastroenterologists' attitude towards and experience with FMT. This was anonymously distributed in hard copy format amongst attendees at gastroenterology meetings in Australia between October 2013 and April 2014. Basic descriptive statistical analyses were performed.RESULTS: Fifty-two clinicians participated. Twenty one percent had previously referred patients for FMT,8% more than once. Ninety percent would refer patients with Clostridium difficile infection(CDI) for FMT if easily available,37% for ulcerative colitis,13% for Crohn's disease and 6% for irritable bowel syndrome. Six percent would not refer any indication,including recurrent CDI. Eighty-six percent would enroll patients in FMT clinical trials. Thirty-seven percent considered the optimal mode of FMT administration transcolonoscopic,17% nasoduodenal,13% enema and 8% oral capsule. The greatest concerns regarding FMT were: 42% lack of evidence,12% infection risk,10% non infectious adverse effects/lack of safety data,10% aesthetic,10% lack of efficacy,4% disease exacerbation,and 2% inappropriate use; 6% had no concerns. Seventy seven percent believed there is a lack of accessibility while 52% had an interest in learning how to provide FMT. Only 6% offered FMT at their institution.CONCLUSION: Despite general enthusiasm,most gastroenterologists have limited experience with,or access to,FMT. The greatest concerns were lack of supportive evidence and safety issues. However a significant proportion would refer indications other than CDI for FMT despite insufficient evidence. These data provide guidance on where education and training are required. | Sudarshan Paramsothy Alissa J Walsh Thomas Borody Douglas Samuel Johan van den Bogaerde Rupert WL Leong Susan Connor Watson Ng Hazel M Mitchell Nadeem O Kaakoush Michael A Kamm | 2015 | World Journal of Gastroenterology2015，21，38： | 5 |
| 4 | Gastroenterostoma after Billroth antrectomy as a premalignant condition显示文摘Gastric stump carcinoma(GSC) following remote gastric surgery is widely recognized as a separate entity within the group of various types of gastric cancer.Gastrectomy is a well established risk factor for the development of GSC at a long time after the initial surgery.Both exoas well as endogenous factors appear to be involved in the etiopathogenesis of GSC,such as achlorhydria,hypergastrinemia and biliary reflux,Epstein-Barr virus and Helicobacter pylori infection,atrophic gastritis,and also some polymorphisms in interleukin-1 and maybe cyclo-oxygenase-2.This review summarizes the literature of GSC,with special reference to reliable early diagnostics.In particular,dysplasia can be considered as a dependable morphological marker.Therefore,close endoscopic surveillance with multiple biopsies of the gastroenterostomy is recommended.Screening starting at 15 years after the initial ulcer surgery can detect tumors at a curable stage.This approach can be ofspecial interest in Eastern European countries,where surgery for benign gastroduodenal ulcers has remained a practice for a much longer time than in Western Europe,and therefore GSC is found with higher frequency. | Robert Sitarz Ryszard Maciejewski Wojciech P Polkowski G Johan A Offerhaus | 2012 | World Journal of Gastroenterology2012，18，25： | 5 |
| 5 | Early-onset gastric cancer:Learning lessons from the young显示文摘There is by no means a clear-cut pattern of mutations contributing to gastric cancers,and gastric cancer research can be hampered by the diversity of factors that can induce gastric cancer,such as Helicobacter pylori infection,diet,ageing and other environmental factors.Tumours are unquestionably riddled with genetic changes yet we are faced with an unsolvable puzzle with respect to a temporal relationship.It is postulated that inherited genetic factors may be more important in early-onset gastric cancer (EOGC) than in gastric cancers found in older patients as they have less exposure to environmental carcinogens.EOGC,therefore,could provide a key to unravelling the genetic changes in gastric carcinogenesis.Gastric cancers occurring in young patients provide an ideal background on which to try and uncover the initiating stages of gastric carcinogenesis.This review summarizes the literature regarding EOGC and also presents evidence that these cancers have a unique molecular-genetic phenotype,distinct from conventional gastric cancer. | Anya N Milne G Johan A Offerhaus | 2010 | World Journal of Gastrointestinal Oncology2010，2，2： | 3 |
| 6 | 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 |
| 7 | Heterogeneous Modeling and Design of Control Systems显示文摘 | Xiao jun Liu Jie Liu Johan Eker Edward A Lee | 2001 | IEEE Control Systems2001，35，5： | 2 |
| 8 | 显示文摘 | Kapteijn Freek Heiszwolf Johan J Nijhuis T A (Xander) | 1999 | CATTECH1999，3，： | 2 |
| 9 | Analysis of orthopedic surgical procedures in children with cerebral palsy显示文摘BACKGROUND Orthopedic surgery in children with cerebral palsy(CP)aims to improve function and prevent deformities.Each child’s condition in CP is unique and many covariables influence surgical decision-making including a patient's age and their functional level.Little is known about the frequency of different types of orthopedic surgery in children with CP who have varied functional levels,particularly in countries from Latin America.AIM To assess the type of orthopedic surgical procedures in relation to age and gross motor function in children with CP.METHODS This retrospective study included all children with CP(n=245)treated with elective orthopedic surgery at a Uruguayan university hospital between October 2010 and May 2016 identified from a surgical database.Eighteen children(7%)were lost to follow-up due to missing medical charts.Demographics,gross motor function classification(GMFCS),and orthopedic surgeries were obtained from the medical records of 227 children.Chi-squared tests and analysis of variance were used to assess the frequency of surgery,accounting for GMFCS levels.Mean age for soft tissue vs bone surgery was compared with the independent samples t-test.RESULTS A total of 711 surgical procedures were performed between 1998 and 2016.On average,children had 3.1 surgical procedures and the mean age at first surgery was 8.0 years.There were no significant differences in age at first surgery among GMFCS levels(P=0.47).The most common procedures were lower leg soft tissue surgery(n=189,27%),hip tenotomy(n=135,19%),and hamstring tenotomy(n=104,14%).For children with GMFCS level Ⅰ,the mean number of surgeries per child[1.8(range 1-9)]differed significantly at P<0.05 in children with GMFCS levels Ⅱ[3.2(1-12)],Ⅲ[3.2(1-8)],Ⅳ[3.3(1-13)],and Ⅴ[3.6(1-11)].Within Ⅱ,Ⅲ,Ⅳ,and Ⅴ,there was no significant difference in mean number of surgeries per child when comparing across the groups.The proportion of soft tissue surgery vs bone surgery was higher in GMFCS levels Ⅰ-Ⅲ(80%-85%)compared to levels IV(68%)and V(55%)(P<0.05).CONCLUSION The frequency of surgical procedures per child did not increase with higher GMFCS level after level Ⅰ.However,the proportion of bone surgery was higher in GMFCS levels Ⅳ-Ⅴ compared to Ⅰ-Ⅲ. | Ignacio Rehbein Viviana Teske Ignacio Pagano Alejandro Cúneo María Elena Pérez Johan von Heideken | 2020 | World Journal of Orthopedics2020，11，4： | 2 |
| 10 | Regulation of adipocyte differentiation显示文摘 | Hana K Johan A | 2001 | Ann Med2001，33，8： | 1 |
| 11 | Separation mechanism in dehydration of water/organic binary liquids bypcrvaporation through microporous silica 显示文摘 | Sekulic J Johan E Elshof T Dave H A | 2005 | Journal of Membrane Science2005，254，12： | 1 |
| 12 | The SAVE approach to component-based development of vehicular systems显示文摘 | Mikael A kerholm Jan Carlson Johan Fredriksson | 2007 | Journal of Systems and Software2007，80，5： | 1 |
| 13 | A shapley decomposition of carbon emissionswithout residuals 显示文摘 | Johan A Delphine F Koen S | 2002 | Energy Policy2002，30，： | 1 |
| 14 | Lensed fiber-array assembly with individu- al fiber fine positioning in the submicrometer range 显示文摘 | Johan H C van Zantvoort Simon G L Plukker Erwin C A Dekkers | 2006 | IEEE Selected Topics in Quantum Electronics2006，12，05： | 1 |
| 15 | The role of market orientation and entrepreneurial orientation for new production development performance in manufacturing firms显示文摘 | JOHAN F SVEN A H | 2007 | Technology Analysis&Strategic Management2007，，11： | 1 |
| 16 | Rapid measurement of urinary trypsinogen-2 as a screening test for acute pancreatitis 显示文摘 | Esko A Kemppaimen MD Johan I | 1997 | N EngJ Med1997，336，： | 1 |
| 17 | Journal status显示文摘 | Johan B Marko A R Herbert V S | 2006 | Scientometrics2006，69，3： | 1 |
| 18 | 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 |
| 19 | 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 |
| 20 | Insulin:a wonder drug in the critically ill? 显示文摘 | Johan Groeneveld AB Beishuizen A Visser FC | 2002 | Crit Care2002，6，2： | 1 |
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