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| 1 | Inflammation and immunosuppression in severe acute pancreatitis显示文摘Acute pancreatitis(AP) is a common disease,which usually exists in its mild form.However,in a fifth of cases,the disease is severe,with local pancreatic complications or systemic organ dysfunction or both.Because the development of organ failure is the major cause of death in AP,early identification of patients likely to develop organ failure is important.AP is initiated by intracellular activation of pancreatic proenzymes and autodigestion of the pancreas.Destruction of the pancreatic parenchyma first induces an inflammatory reaction locally,but may lead to overwhelming systemic production of inflammatory mediators and early organ failure.Concomitantly,anti-inflammatory cytokines and specific cytokine inhibitors are produced.This anti-inflammatory reaction may overcompensate and inhibit the immune response,rendering the host at risk of systemic infection.At present,there is no specific treatment for AP.Increased understanding of the pathogenesis of systemic inflammation and development of organ dysfunction may provide us with drugs to ameliorate physiological disturbances. | Marja-Leena Kylnp Heikki Repo Pauli Antero Puolakkainen | 2010 | World Journal of Gastroenterology2010，16，23： | 97 |
| 2 | 基于原子力显微镜的沥青微观结构研究显示文摘采用原子力显微镜和均方根粗糙度参数研究了沥青种类、短期老化以及制样过程中冷却速率对沥青微观结构的影响。结果表明,不同沥青的蜂状结构尺寸、数量不尽相同,对短期老化的敏感性也不相同,而制样过程中冷却速率对蜂状结构的影响十分显著;同时,在沥青表面应力松弛过程中,不同区域的蜡链排列倾角存在差异是蜂状结构呈正弦波形式(波长约为500nm)的原因。根据相分离理论分析实验现象后得出,沥青质及胶质与蜡之间的相互作用是影响沥青相分离行为的重要因素。 | 杨军 龚明辉 PAULI Troy 魏建明 王潇婷 | 2015 | 石油学报(石油加工)2015，31，4： | 36 |
| 3 | Nature:骨髓微环境发生突变可导致造血干细胞异常显示文摘白血病骨髓微环境中支持血液发育的某些DNA突变可以驱动周围造血干细胞形成白血病,来自艾默里大学温希普癌症研究所和亚特兰大儿童健康中心的研究人员在国际学术期刊Nature上公布了他们的最新研究进展。许多致癌突变都作用于细胞自身。让细胞自身生长更快。相比之下,努南综合征小鼠模型上可以观察到间接的邻近细胞效应,努南综合征是一种能够增加白血病患病风险的遗传紊乱。 | Lei Dong, Wen-Mei Yu, Hong Zheng, Melinda Pauly, Muxiang Zhou Cheng-Kui Qu Mignon L. Loh Silvia T. Bunting Gang Huang Hal E. Broxmeyer David T. Scadden | 2016 | 现代生物医学进展2016，16，35： | 18 |
| 4 | Reactive Oxygen Species during Plant-microorganism Early Interactions显示文摘Reactive Oxygen Species (ROS) are continuously produced as a result of aerobic metabolism or in response to biotic and abiotic stresses. ROS are not only toxic by-products of aerobic metabolism, but are also signalling molecules involved in several developmental processes in all organisms. Previous studies have clearly shown that an oxidative burst often takes place at the site of attempted invasion during the early stages of most plant-pathogen interactions. Moreover, a second ROS production can be observed during certain types of plant-pathogen interactions, which triggers hypersensitive cell death (HR). This second ROS wave seems absent during symbiotic interactions. This difference between these two responses is thought to play an important signalling role leading to the establishment of plant defense. In order to cope with the deleterious effects of ROS, plants are fitted with a large panel of enzymatic and non-enzymatic antioxidant mechanisms. Thus,increasing numbers of publications report the characterisation of ROS producing and scavenging systems from plants and from microorganisms during interactions. In this review, we present the current knowledge on the ROS signals and their role during plant-microorganism interactions. | Amrit K.Nanda Emilie Andrio Daniel Marino Nicolas Pauly Christophe Dunand | 2010 | Journal of Integrative Plant Biology2010，52，2： | 15 |
| 5 | Comprehensive management of full-thickness luminal defects: The next frontier of gastrointestinal endoscopy显示文摘Full thickness gastrointestinal defects such as perforations, leaks, and fistulae are a relatively common result of many of the endoscopic and surgical procedures performed in modern health care. As the number of these procedures increases, so too will the number of resultant defects. Historically, these were all treated by open surgical means with the associated morbidity and mortality. With the recent advent of advanced endoscopic techniques, these defects can be treated definitively while avoiding an open surgical procedure. Here we explore the various techniques and tools that are currently available for the treatment of gastrointestinal defects including through the scope clips, endoscopic suturing devices, over the scope clips, sealants, endoluminal stents, endoscopic suction devices, and fistula plugs. As fistulae represent the most recalcitrant of defects, we focus this editorial on a multimodal approach of treatment. This includes optimization of nutrition, treatment of infection, ablation of tracts, removal of foreign bodies, and treatment of distal obstructions. We believe that by addressing all of these factors at the time of attempted closure, the patient is optimized and has the best chance at longterm closure. However, even with all of these factors addressed, failure does occur and in those cases, endoscopic therapies may still play a role in that they allow the patient to avoid a definitive surgical therapy for a time while nutrition is optimized, and infections are addressed. | Joshua S Winder Eric M Pauli | 2015 | World Journal of Gastrointestinal Endoscopy2015，7，8： | 13 |
| 6 | QSAR Studies on the Toxicity of Nitrobenzenes to Population Growth of Tetrahymena Pyriformis显示文摘The IGC 50 (50% inhibitory growth concentration) values of 27 nitrobenzenes were determined for the population growth endpoint of Tetrahymena pyriformis . The toxicity order of the observed compounds was found to be as follows: dinitro compounds>mononitro compounds, dichloronitrobenzenes>monochloronitrobenzenes, meta substituted nitrobenzenes>ortho /para substituted nitrobenzenes(NT, NPh, NAnis) except for the dinitrobenzenes and nitroanilines(DNB, NAn). Quantitative structure activity relationships(QSARs) were developed with the logarithm of the reciprocal of IGC 50 [lg(IGC 50 ) -1 ] in mole liter as dependent variable and six molecular descriptors lg P , 1 X Ⅴ, I, 1K a, ∑σ - and E LUMO as independent variables. Via multiple regression analysis, one best equation was obtained: lg(IGC 50 ) -1 =3.029+0.860∑ σ -+0.341I n=27, r=0.924, r 2=0.854, s=0.265, f=70.44 , Pr> f =0.000 1 The equation was used to estimate IGC 50 for five analogues. | XU Jing bo 1** , JING Ti song 2, Pauli W. 3 and Berger S. 31. Department of Environmental Science, Northeast Normal University, Changchun 130024, P.R.China 2. College of Environment and Resource, Jilin University, Changchun 130026, P.R. China | 2002 | Chemical Research in Chinese Universities2002，18，3： | 9 |
| 7 | Comparison of operative link for gastritis assessment, operative link on gastric intestinal metaplasia assessment, and TAIM stagings among men with atrophic gastritis显示文摘BACKGROUND Gastric cancer is the world’s third most lethal malignancy. Most gastric cancers develop through precancerous states of atrophic gastritis and intestinal metaplasia. Two staging systems, operative link for gastritis assessment(OLGA)and operative link on gastric intestinal metaplasia assessment(OLGIM), have been developed to detect high gastric cancer risk. European guidelines recommend surveillance for high-risk OLGA/OLGIM patients(stages Ⅲ–Ⅳ),and for those with advanced stage of atrophic gastritis in the whole stomach mucosa. We hypothesize, that by combining atrophy and intestinal metaplasia into one staging named TAIM, more patients with increased gastric cancer risk could be detected.AIM To evaluate the clinical value of the OLGA, OLGIM, and novel TAIM stagings as prognostic indicators for gastric cancer.METHODS In the Helsinki Gastritis Study, 22346 elderly male smokers from southwestern Finland were screened for serum pepsinogen I(PGI). Between the years 1989 and1993, men with low PGI values(PGI < 25 μg/L), were invited to undergo an oesophagogastroduodenoscopy. In this retrospective cohort study, 1147 men that underwent gastroscopy were followed for gastric cancer for a median of 13.7 years, and a maximum of 27.3 years. We developed a new staging system, TAIM,by combining the topography with the severity of atrophy or intestinal metaplasia in gastric biopsies. In TAIM staging, the gastric cancer risk is classified as low or high.RESULTS Twenty-eight gastric cancers were diagnosed during the follow-up, and the incidence rate was 1.72 per 1000 patient-years. The cancer risk associated positively with TAIM [Hazard ratio(HR) 2.70, 95%CI: 1.09–6.69, P = 0.03]. The risk increased through OLGIM stages 0-Ⅳ(0 vs Ⅳ: HR 5.72, 95%CI: 1.03–31.77, P for trend = 0.004), but not through OLGA stages 0–Ⅳ(0 vs Ⅳ: HR 5.77, 95%CI:0.67–49.77, P for trend = 0.10). The sensitivities of OLGA and OLGIM stages Ⅲ–Ⅳ were low, 21% and 32%, respectively, whereas that of TAIM high-risk was good, 79%. On the contrary, OLGA and OLGIM had high specificity, 85% and81%, respectively, but TAIM showed low specificity, 42%. In all three staging systems, the high-risk men had three-to four-times higher gastric cancer risk compared to the general male population of the same age.CONCLUSION OLGIM and TAIM stagings show prognostic value in assessing gastric cancer risk in elderly male smokers with atrophic gastritis. | Anna A Nieminen Jukka Kontto Pauli Puolakkainen Jarmo Virtamo Arto Kokkola | 2020 | World Journal of Gastroenterology2020，26，24： | 7 |
| 8 | Helicobacter pylori infection and low serum pepsinogen I level as risk factors for gastric carcinoma显示文摘AIM: To study whether examination of CagA antibodies could increase the odds ratio for gastric cancer in a casecontrol study, and how often other serum markers of gastric cancer risk could be found in Helicobacter pylorinegative patients.METHODS: H pylori CagA and parietal cell antibodies(PCAs), and serum pepsinogen Ⅰ (SPGI) levels were compared between patients with gastric cancer and controls who received endoscopic examination due to reasons other than gastrointestinal malignancy.RESULTS: The odds ratio (OR) for gastric cancer was2.9 (95% CI 1.4-5.8) in H pylori+ patients, and 2.4 (95%CI 1.2-4.9) in CagA+ patients. When results of H pylori and CagA antibodies were combined, OR increased to5.0 (95% CI 2.5-10.0). Furthermore, if cardia cancer patients were excluded, the OR increased to 6.8 (95% CI3.1-14.8). Among patients with a low SPGI level, the OR was 12.0 (95% CI 4.1-35.3). However, the risk was significant only in the older age group. The number of patients with low SPGI was significantly higher in H pylori-/CagA+ patients as compared to other cancer patients.CONCLUSION: Examination of both H pylori and CagA antibodies increases the OR for gastric cancer in our casecontrol study. CagA antibodies are important in detecting previous H pylori infection in advanced atrophic gastritis or cancer when spontaneous decline of H pylori antibodies occurs. SPGI may be helpful in screening elderly gastric cancer patients. | Arto Kokkola Johanna Louhimo Pauli Puolakkainen Henrik Alfthan Caj Haglund Hilpi Rautelin | 2005 | World Journal of Gastroenterology2005，11，7： | 6 |
| 9 | 桦树花粉相关的食物过敏综合征一例显示文摘 | 邵洁 罗海燕 APurohit G Pauli | 2005 | 中华儿科杂志2005，43，2： | 6 |
| 10 | RNA-Seq Analysis of Developing Nasturtium Seeds (Tropaeolum majus): Identification and Characterization of an Additional Galactosyltransferase Involved in Xyloglucan Biosynthesis显示文摘一条深定序的途径被追求利用 454,定序方法发现新基因的 Illumina 在 xyloglucan 包含了生合成。cDNA 序列从开发旱金莲属植物(金莲花属之植物 majus ) 被产生作为种子存储聚合物播种 xyloglucan,它生产大量 non-fucosylated。除了已知的 xyloglucan biosynthetic 基因,一以前 uncharacterized 通常认为的 xyloglucan galactosyltransferase 被识别。在相应 ortholog (AT5G62220 ) 的一个 Arabidopsis thaliana 异种线有缺陷者的分析表明这基因不与以前描绘的 xyloglucan galactosyltransferase 显示出冗余性, MUR3,但是在一个不同位置为 xyloglucan 的 galactosyl 替换被要求。基因为 Xyloglucan L 方面链 galactosylTransferase 位置 2 被称为 XLT2。它在作为 MUR3 的 glycosyltransferase 家庭 47 的一样的 subclade 代表酶。在 MUR3 (mur3.1 ) 和 XLT2 的一个双变异的有缺陷者与由仅仅 xylosylated glucosyl 单位实质上组成的 xyloglucan 导致了 Arabidopsis 植物,没有进一步的替换。 | Jacob K. Jensen Alex Schultink Kenneth Keegstra Curtis G. Wilkerson Markus Pauly | 2012 | Molecular Plant2012，5，5： | 6 |
| 11 | Event Specific Transgene Detection in Bt11 Corn by Quantitative PCR at the Integration Site显示文摘 | Andreas Zimmermann Jürg Lüthy Urs Pauli | 2000 | LWT - Food Science and Technology2000，，3： | 4 |
| 12 | Blood glucose response after oral intake of lactulose in healthy volunteers:A randomized,controlled,cross-over study显示文摘AIM To investigate possible changes of blood glucose levels after oral intake of lactulose in healthy subjects.METHODS The study was performed as prospective, randomized, two-part study with 4-way cross-over design with n = 12 in each study arm. Capillary blood glucose levels were determined over a time period of 180 min after intake of a single dose of 10 g or 20 g lactulose provided as crystal or liquid formulation. During the manufacturing process of lactulose, impurities with sugars(e.g., lactose, fructose, galactose) occur. Water and 20 g glucose were used as control and reference. Because lactulose is used as a functional food ingredient, it may also be consumed by people with impaired glucose tolerance, including diabetics. Therefore, it is of interest to determine whether the described carbohydrate impurities may increase blood glucose levels after ingestion. RESULTS The blood glucose concentration-time curves after intake of 10 g lactulose, 20 g lactulose, and water were almost identical. None of the three applications showed any changes in blood glucose levels. After intake of 20 g glucose, blood glucose concentration increased by approximately 3 mmol/L(mean Cmax = 8.3 mmol/L), reaching maximum levels after approximately 30 min and returning to baseline within approximately 90 min, which was significantly different to the corresponding 20 g lactulose formulations(P < 0.0001). Comparing the two lactulose formulations, crystals and liquid, in the dosage of 10 g and 20 g, there was no difference in the blood glucose profile and calculated pharmacokinetic parameters despite the different amounts of carbohydrate impurities(1.5% for crystals and 26.45% for liquid). Anyhow, the absolute amount of single sugars was low with 0.3 g in crystals and 5.29 g in liquid formulation in the 20 g dosages. Lactulose was well tolerated by most volunteers, and only some reported mild to moderate mainly gastrointestinal side effects. CONCLUSION The unchanged blood glucose levels after lactulose intake in healthy subjects suggest its safe use in subjects with impaired glucose tolerance. | Jasmin Steudle Christiane Schon Manfred Wargenau Lioba Pauly Susann Schwejda-Güttes Barbara Gaigg Angelika Kuchinka-Koch John F Stover | 2018 | World Journal of Gastrointestinal Pharmacology and Therapeutics2018，9，3： | 4 |
| 13 | 法国工程师教育中的口语测试方式研究显示文摘口语测试是法国预科教育的重要特色之一。在对法国预科教育中口语测试的含义,对工程师教育模式下的口语测试方式的历史以及口语测试的试题形式、组织方式、评价标准、效度和信度进行了研究的基础上,以北京航空航天大学中法工程师学院的教学实践为例进行数据统计分析,研究发现,口语测试在工程师预科教育,尤其是中国的工程师教育中非常重要,从而为中国的工程师教育提供了借鉴。 | 张心婷 王乐梅 Marc Pauly | 2013 | 北京航空航天大学学报(社会科学版)2013，26，6： | 4 |
| 14 | 全球捕捞船队的燃料问题显示文摘20世纪,化石燃料逐渐成为世界大多数渔场的主要能源输入。尽管各种分析报告确定了对个别渔场的燃料输入数量,但迄今为止,还没有对全球范围的燃料输入进行量化,及绘制渔业所消耗燃料分布图的尝试。通过用2000年空间分解渔获量统计数据综合了代表世界各地250多个渔场的数据,我们认为,在全球范围内,在8000万t海洋鱼类和无脊椎动物上市的过程中,渔船烧掉了近500亿L燃料,平均为620L/t。因此,渔船约占全球油耗的1.2%,相当于全球第18位油耗国——荷兰的数量,并直接向大气排入1.3亿tCO_2。从效能角度看,全球渔船所烧掉的燃料的能量含量是所产生的渔获量可食用蛋白质能量含量的12.5倍。 | Peter H.Tyedmers Reg Watson Daniel Pauly 林宝法(译) 胡恩和(校) | 2005 | AMBIO-人类环境杂志2005，34，B12： | 3 |
| 15 | Reassessing the environmental Kuznets curve for CO 2 emissions: A robustness exercise显示文摘 | Marzio Galeotti Alessandro Lanza Francesco Pauli | 2005 | Ecological Economics2005，，1： | 3 |
| 16 | Pectin May Hinder the Unfolding of Xyloglucan Chains during Cell Deformation: Implications of the Mechanical Performance of Arabidopsis Hypocotyls with Pectin Alterations显示文摘种房间墙,象大量的其它一样生物材料,是自然增强纤维的合成材料。他们的机械性质自己高度依赖于生硬含纤维的阶段和软矩阵阶段的相互影响并且在矩阵变丑上。用特定的 Arabidopsis thaliana 异种,我们与改变的 xyloglucan 和果胶作文在胚轴的主要房间墙中学习了矩阵集会的机械角色。标准 microtensile 测试和周期的装载协议象少些展出 50% 的 qua2 一样与影响 RGII 硼酸盐 diester 交叉结合和妨碍的 xyloglucan fucosylation 在 mur1 胚轴上被执行 homogalacturonan 与相比野类型。作为控制,野类型的植物(Col-0 ) 和在果胶网络展出特定的 xyloglucan fucosylation 和没有差别的 mur2 被利用。在标准张力的测试,有果胶改变的异种的胚轴的最终的压力层次(张力的力量)(mur1, qua2 ) 是相当未受影响的,而他们的张力的僵硬显著地与 Col-0 相比被减少。周期的装载测试显示了一在首先拉紧然而,其度为 mur1 和 qua2 胚轴是高得多的期间在第一个周期和塑料变丑以后所有胚轴使硬。基于机械数据和当前的房间墙模型,在纤维素纤丝之间的合拢的 xyloglucan 链可以趋于在胚轴拉紧期间展开,这被假定。这回答被几何限制可能由于果胶刚硬妨碍。 | Willie Abasolo Michaela Eder Kazuchika Yamauchi Nicolai Obel Antje Reinecke Lutz Neumetzler John W.C. Dunlop Gregory Mouille Markus Pauly Herman Hofte Ingo Burgert | 2009 | Molecular Plant2009，2，5： | 2 |
| 17 | Monitoring Greenhouses Gases over China Using Space-Based Observations Monitoring Greenhouses Gases over China Using Space-Based Observations显示文摘The atmospheric carbon dioxide(CO 2)concentration has increased to more than 405 parts per million(ppm.1 ppm=10-6 m/s 2)in 2017 due to human activities such as deforestation,land-use change and burning of fossil fuels.Although there is broad scientific consensus on the damaging consequences of the change in climate associated with increasing concentrations of greenhouse gases,fossil CO 2 emissions have continued to increase in recent years mainly from rapidly developing economies and China is now the largest emitter of CO 2 generating about 30%of all emissions globally.To allow more reliable forecast of the future state of the carbon cycle and to support the efforts for mitigation greenhouse gas emissions,a better understanding of the global and regional carbon budget is needed.Space-based measurements of CO 2 can provide the necessary observations with dense coverage and sampling to provide improved constrains on of carbon fluxes and emissions.The Chinese Global Carbon Dioxide Monitoring Scientific Experimental Satellite(TanSat)was established by the National High Technology Research and Development Program of China with the main objective of monitoring atmospheric CO 2 and CO 2 fluxes at the regional and global scale.TanSat has been successfully launched in December 2016 and as part of the Dragon programme of ESA and the Ministry of Science and Technology(MOST),a team of researchers from Europe(UK and Finland)and China has evaluated early TanSat data and contrast it against data from the GOSAT mission and models.In this manuscript,we report on retrieval intercomparisons of TanSat data using two different retrieval algorithms,on validation efforts for the Eastern Asia region using GOSAT CO 2 data and first assessments of TanSat and GOSAT CO 2 data against model calculations using the GEOS-Chem model. | Hartmut BOESCH Yi LIU Paul I PALMER Johanna TAMMINEN Jasdeep S ANAND Zhaonan CAI Ke CHE Huilin CHEN Xi CHEN Liang FENG Janne HAKKARAINEN Pauli HEIKKINEN Nikoleta KALAITZI Rigel KIVI Robert PARKER Peter SOMKUTI Jing WANG Alex WEBB Dongxu YANG Lu YAO You YI | 2020 | Journal of Geodesy and Geoinformation Science2020，3，4： | 2 |
| 18 | Arabidopsis thaliana AtUTr7 Encodes a Golgi-Localized UDP-Glucose/UDP-Galactose Transporter that Affects Lateral Root Emergence显示文摘核苷酸糖 transporters (NST ) 是包括在植物在复杂房间墙多糖和 glycoproteins 的生合成起一个基本作用的一个基因家庭的 antiporters。由于有看得见的显型的相关异种的有限数字,然而,在房间墙生合成和汇编的大多数 NST 的生物功能留下了逃犯。这里,我们从 Arabidopsis (Arabidopsis thaliana (L.) Heynh ) 报导 AtUTr7 的描述,它对从果蝇 melanogaster,人,和 Caenorhabditis elegans 的多特定的 UDPsugar transporters 相应。我们证明 AtUTr7 拥有在 NST 之中保存的普通结构的特征。用一块草地荧光灯蛋白质(GFP ) 标注了版本,我们证明 AtUTr7 在 Golgi 仪器是局部性的。我们也证明 AtUTr7 广泛地被表示,特别在根并且在特定的花的机关。另外,结果一在里面与一棵烟草执行的 vitro 核苷酸糖运输试金和一个酵母表达式系统建议 AtUTr7 能够转 UDPGal 和 UDPGlc,然而并非另外的 UDP 和 GDPsugars 的一个范围,进 Golgi 腔。当在高蔗糖集中栽培了时, AtUTr7 的异种缺乏表示象弄歪的根头发一样展出了侧面的根的早增长。而且,有甲基酯化作用的低度的 homogalacturonan 的分发与野类型的植物相比在异种的侧面的根尖端不同,尽管另外的分析过程没在根房间墙的作文揭示进一步的差别。这条证据建议进在高根生物资源生产的条件下面的 Golgi 的 UDPGal 和 UDPGlc 的运输在侧面的根和根头发开发起一个作用。 | Michael Handford Cecilia Rodriguez-Furlan Lorena Marchan Marcelo Segura Daniela Gomez Elena Alvarez-Buyllal Guang-Yan Xiong Markus Pauly Ariel Orellana | 2012 | Molecular Plant2012，5，6： | 2 |
| 19 | Vasohibin-1 and vasohibin-2 expression in gastric cancer cells and TAMs显示文摘 | Zhanlong Shen Tuuli Kauttu Hanna Sepp?nen Sanna Vainionp?? Yingjiang Ye Shan Wang Harri Mustonen Pauli Puolakkainen | 2012 | Medical Oncology2012，，4： | 2 |
| 20 | Neither hereditary periodic fever nor periodic fever, aphthae, pharingitis, adenitis: Undifferentiated periodic fever in a tertiary pediatric center显示文摘AIM To describe the frequency and clinical characteristics of patients with undifferentiated periodic fever(UPF) and to investigate whether a clinical classification of UPF based on the PRINTO-Eurofever score can help predicting the response to treatment and the outcome at follow-up.METHODS Clinical and therapeutic information of patients with recurrent fever who presented at a single pediatric rheumatology center from January 2006 through April 2016 were retrospectively collected. Patients with a clinical suspicion of hereditary periodic fever(HPF) syndrome and patients with clinical picture of periodic fever, aphthae, pharingitis, adenitis(PFAPA) who were refractory to tonsillectomy underwent molecular analysis of five HPF-related genes: MEFV(NM_000243.2), MVK(NM_000431.3), TNFRSF1 A(NM_001065.3), NLRP3(NM_001079821.2), NLRP12(NM_001277126.1). All patients who had a negative genetic result were defined as UPF and further investigated. PRINTO-Eurofever score for clinical diagnosis of HPF was calculated in all cases. RESULTS Of the 221 patients evaluated for periodic fever, twelve subjects with a clinical picture of PFAPA who were refractory to tonsillectomy and 22 subjects with a clinical suspicion of HPF underwent genetic analysis. Twenty-three patients(10.4%) resulted negative and were classified as UPF. The median age at presentation of patients with UPF was 9.5 mo(IQR 4-24). Patients with UPF had a higher frequency of aphthae(52.2% vs 0%, P = 0.0026) and musculoskeletal pain(65.2% vs 18.2%, P = 0.0255) than patients with genetic confirmed HPF. Also, patients with UPF had a higher frequency of aphthous stomatitis(52.2% vs 10.7%, P < 0.0001), musculoskeletal pain(65.2% vs 8,0%, P < 0.0001), and abdominal pain(52.2% vs 4.8%, P < 0.0001) and a lower frequency of pharyngitis(56.6% vs 81.3%, P = 0.0127) compared with typical PFAPA in the same cohort. Twenty-one of 23 patients with UPF(91.3%) received steroids, being effective in 16; 13(56.2%) were given colchicine, which was effective in 6. Symptoms resolution occurred in 2 patients with UPF at last follow-up. Classification according to the PRINTOEurofever score did not correlate with treatment response and prognosis. CONCLUSION UPF is not a rare diagnosis among patients with periodic fever. Clinical presentation place UPF half way on a clinical spectrum between PFAPA and HPF. The PRINTOEurofever score is not useful to predict clinical outcome and treatment response in these patients. | Silvia De Pauli Sara Lega Serena Pastore Domenico Leonardo Grasso Anna Monica Rosaria Bianco Giovanni Maria Severini Alberto Tommasini Andrea Taddio | 2018 | World Journal of Clinical Pediatrics2018，7，1： | 2 |
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