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| 1 | A deep learning-enabled portable imaging flow cytometer for cost-effective, highthroughput, and label-free analysis of natural water samples显示文摘We report a deep learning-enabled field-portable and cost-effective imaging flow cytometer that automatically captures phase-contrast color images of the contents of a continuously flowing water sample at a throughput of 100 mL/h.The device is based on partially coherent lens-free holographic microscopy and acquires the diffraction patterns of flowing micro-objects inside a microfluidic channel.These holographic diffraction patterns are reconstructed in real time using a deep learning-based phase-recovery and image-reconstruction method to produce a color image of each micro-object without the use of external labeling.Motion blur is eliminated by simultaneously illuminating the sample with red,green,and blue light-emitting diodes that are pulsed.Operated by a laptop computer,this portable device measures 15.5 cm×15 cm×12.5 cm,weighs 1 kg,and compared to standard imaging flow cytometers,it provides extreme reductions of cost,size and weight while also providing a high volumetric throughput over a large object size range.We demonstrated the capabilities of this device by measuring ocean samples at the Los Angeles coastline and obtaining images of its micro-and nanoplankton composition.Furthermore,we measured the concentration of a potentially toxic alga(Pseudo-nitzschia)in six public beaches in Los Angeles and achieved good agreement with measurements conducted by the California Department of Public Health.The cost-effectiveness,compactness,and simplicity of this computational platform might lead to the creation of a network of imaging flow cytometers for largescale and continuous monitoring of the ocean microbiome,including its plankton composition. | Zoltán Gӧrӧcs Miu Tamamitsu Vittorio Bianco Patrick Wolf Shounak Roy Koyoshi Shindo Kyrollos Yanny Yichen Wu Hatice Ceylan Koydemir Yair Rivenson Aydogan Ozcan | 2018 | Light(Science & Applications)2018，7，1： | 14 |
| 2 | Genetics of inflammatory bowel disease from multifactorial to monogenic forms显示文摘Inflammatory bowel disease(IBD) is a group of chronic multifactorial disorders. According to a recent study,the number of IBD association loci is increased to 201,of which 37 and 27 loci contribute specifically to the development of Crohn's disease and ulcerative colitis respectively. Some IBD associated genes are involved in innate immunity,in the autophagy and in the inflammatory response such as NOD2,ATG16L1 and IL23 R,while other are implicated in immune mediated disease(STAT3) and in susceptibility to mycobacterium infection(IL12B). In case of early onset of IBD(VEO-IBD) within the 6th year of age,the disease may be caused by mutations in genes responsible for severe monogenic disorders such as the primary immunodeficiency diseases. In this review we discuss how these monogenic disorders through different immune mechanisms can similarly be responsible of VEO-IBD phenotype. Moreover we would highlight how the identification of pathogenic genes by Next Generation Sequencing technologies can allow to obtain a rapid diagnosis and to apply specific therapies. | Anna Monica Bianco Martina Girardelli Alberto Tommasini | 2015 | World Journal of Gastroenterology2015，21，43： | 13 |
| 3 | 澳大利亚的语言政策与中文教学生态环境显示文摘为了充分利用地理优势扩大与亚洲的贸易,澳大利亚于上个世纪80年代中叶制定并开始实施一项语言国策,目的在于鼓励和推动亚洲语言的教育。本文着重探讨这项国策所导致的澳大利亚中文教学生态状况,其中包括中文在国家语言政策中的地位以及这项语言政策的变化对中文教育产生的影响,并且分析大学、中学、小学各级教育和社区中文教育所面临的一些特定问题。 | Joseph Lo Bianco 刘国强 | 2007 | 世界汉语教学2007，21，3： | 8 |
| 4 | Strategies for reducing speckle noise in digital holography显示文摘Digital holography(DH)has emerged as one of the most effective coherent imaging technologies.The technological developments of digital sensors and optical elements have made DH the primary approach in several research fields,from quantitative phase imaging to optical metrology and 3D display technologies,to name a few.Like many other digital imaging techniques,DH must cope with the issue of speckle artifacts,due to the coherent nature of the required light sources.Despite the complexity of the recently proposed de-speckling methods,many have not yet attained the required level of effectiveness.That is,a universal denoising strategy for completely suppressing holographic noise has not yet been established.Thus the removal of speckle noise from holographic images represents a bottleneck for the entire optics and photonics scientific community.This review article provides a broad discussion about the noise issue in DH,with the aim of covering the best-performing noise reduction approaches that have been proposed so far.Quantitative comparisons among these approaches will be presented. | Vittorio Bianco Pasquale Memmolo Marco Leo Silvio Montresor Cosimo Distante Melania Paturzo Pascal Picart Bahram Javidi Pietro Ferraro | 2018 | Light(Science & Applications)2018，7，1： | 8 |
| 5 | Food allergy in gastroenterologic diseases:Review of literature显示文摘Food allergy is a common and increasing problem worldwide. The newly-found knowledge might provide novel experimental strategies, especially for laboratory diagnosis. Approximately 20% of the population alters their diet for a perceived adverse reaction to food, but the application of double-blind placebo-controlled oral food challenge, the “gold standard” for diagnosis of food allergy, shows that questionnaire-based studies over- estimate the prevalence of food allergies. The clinical disorders determined by adverse reactions to food can be classified on the basis of immunologic or nonimmu- nologic mechanisms and the organ system or systems affected. Diagnosis of food allergy is based on clinical history, skin prick tests, and laboratory tests to detect serum-food specific IgE, elimination diets and challeng- es. The primary therapy for food allergy is to avoid the responsible food. Antihistamines might partially relieve oral allergy syndrome and IgE-mediated skin symptoms, but they do not block systemic reactions. Systemic cor- ticosteroids are generally effective in treating chronic IgE-mediated disorders. Epinephrine is the mainstay of treatment for anaphylaxis. Experimental therapies for IgE-mediated food allergy have been evaluated, such as humanized IgG anti-IgE antibodies and allergen specific immunotherapy. | Pasquale Mansueto Giuseppe Montalto Maria Luisa Pacor Maria Esposito-Pellitteri Vito Ditta Claudia Lo Bianco Stefania Maria Leto-Barone Gabriele Di Lorenzo | 2006 | World Journal of Gastroenterology2006，12，48： | 7 |
| 6 | 非甾体抗炎药作为抗哮喘剂的作用显示文摘气道粘膜的嗜酸性炎症在哮喘发病中起着核心作用。非甾体抗炎药虽然可在一部分病人中诱发哮喘,但对大部分病人有抗哮喘或减少对皮质类固醇依赖的作用,其中以赖氨酸乙酸水杨酸最好。它们的作用机制不仅仅是抑制环加氧酶,而是相当复杂的。很多问题还应进一步研究。 | BiancoS 镡旭民 石艳玲 | 1995 | 国外医学(药学分册)1995，22，1： | 6 |
| 7 | Motility-based label-free detection of parasites in bodily fluids using holographic speckle analysis and deep learning显示文摘Parasitic infections constitute a major global public health issue.Existing screening methods that are based on manual microscopic examination often struggle to provide sufficient volumetric throughput and sensitivity to facilitate early diagnosis.Here,we demonstrate a motility-based label-free computational imaging platform to rapidly detect motile parasites in optically dense bodily fluids by utilizing the locomotion of the parasites as a specific biomarker and endogenous contrast mechanism.Based on this principle,a cost-effective and mobile instrument,which rapidly screens~3.2 mL of fluid sample in three dimensions,was built to automatically detect and count motile microorganisms using their holographic time-lapse speckle patterns.We demonstrate the capabilities of our platform by detecting trypanosomes,which are motile protozoan parasites,with various species that cause deadly diseases affecting millions of people worldwide.Using a holographic speckle analysis algorithm combined with deep learningbased classification,we demonstrate sensitive and label-free detection of trypanosomes within spiked whole blood and artificial cerebrospinal fluid(CSF)samples,achieving a limit of detection of ten trypanosomes per mL of whole blood(~five-fold better than the current state-of-the-art parasitological method)and three trypanosomes per mL of CSF.We further demonstrate that this platform can be applied to detect other motile parasites by imaging Trichomonas vaginalis,the causative agent of trichomoniasis,which affects 275 million people worldwide.With its costeffective,portable design and rapid screening time,this unique platform has the potential to be applied for sensitive and timely diagnosis of neglected tropical diseases caused by motile parasites and other parasitic infections in resource-limited regions. | Yibo Zhang Hatice Ceylan Koydemir Michelle M.Shimogawa Sener Yalcin Alexander Guziak Tairan Liu Ilker Oguz Yujia Huang Bijie Bai Yilin Luo Yi Luo Zhensong Wei Hongda Wang Vittorio Bianco Bohan Zhang Rohan Nadkarni Kent Hill Aydogan Ozcan | 2018 | Light(Science & Applications)2018，7，1： | 6 |
| 8 | Endowing a plain fluidic chip with micro-optics: a holographic microscope slide显示文摘Lab-on-a-Chip(LoC)devices are extremely promising in that they enable diagnostic functions at the point-of-care.Within this scope,an important goal is to design imaging schemes that can be used out of the laboratory.In this paper,we introduce and test a pocket holographic slide that allows digital holography microscopy to be performed without an interferometer setup.Instead,a commercial off-the-shelf plastic chip is engineered and functionalized with this aim.The microfluidic chip is endowed with micro-optics,that is,a diffraction grating and polymeric lenses,to build an interferometer directly on the chip,avoiding the need for a reference arm and external bulky optical components.Thanks to the single-beam scheme,the system is completely integrated and robust against vibrations,sharing the useful features of any common path interferometer.Hence,it becomes possible to bring holographic functionalities out of the lab,moving complexity from the external optical apparatus to the chip itself.Label-free imaging and quantitative phase contrast mapping of live samples are demonstrated,along with flexible refocusing capabilities.Thus,a liquid volume can be analyzed in one single shot with no need for mechanical scanning systems. | Vittorio Bianco Biagio Mandracchia Valentina Marchesano Vito Pagliarulo Federico Olivieri Sara Coppola Melania Paturzo Pietro Ferraro | 2017 | Light(Science & Applications)2017，6，1： | 6 |
| 9 | Focus on acute diarrhoeal disease显示文摘Diarrhoea is an alteration of normal bowel movement characterized by an increase in the water content, volume, or frequency of stools. Diarrhoea needs to be classif ied according to the trends over time (acute or chronic) and to the characteristics of the stools (watery, fatty, inflammatory). Secretory diarrhoeas, mostly acute and of viral aetiology in more than 70% of cases, are by far the most important subtype of diarrhoeas in terms of frequency, incidence and mortality (over 2.5 million deaths/year in developing countries). Natural and synthetic opiates such as morphine, codeine, and loperamide which react with endogenous opiates (enkephalins, beta-endorphins, dynorphins) mainly act on intestinal motility and slow down transit. An antidiarrhoeal drug developed in recent years, racecadotril, acts as an enkephalinase inhibitor. Clinical studies have shown that it is just as effective as loperamide in resolving acute diarrhoea but with greater reduction in pain and abdominal distension. Some studies have explored the prevalence of diarrhoea in old age. An epidemiological study carried out in Italy by 133 General Practitioners on 5515 elderly outpatients reported a prevalence of diarrhoea, def ined according to the Rome criteria, of 9.1%. Infectious diseases (19%) and drug use (16%) were the most common causes of diarrhoea in old age. Regardless of the cause, the treatment of elderly patients with diarrhoea must include rehydration and nutritional support. Every year, more than 50 million tourists travel from industrialized countries to places where hygiene levels are poor. At least 75% of those travelling for short periods mention health problems, and in particular traveller's diarrhoea. | Fabio Baldi Maria Antonia Bianco Gerardo Nardone Alberto Pilotto Emanuela Zamparo | 2009 | World Journal of Gastroenterology2009，15，27： | 6 |
| 10 | Botulinum toxin for chronic anal fissure after biliopancreatic diversion for morbid obesity显示文摘AIM: To study the effect of botulinum toxin in patients with chronic anal fissure after biliopancreatic diversion (BPD) for severe obesity. METHODS: Fifty-nine symptomatic adults with chronic anal fissure developed after BPD were enrolled in an open label study. The outcome was evaluated clinically and by comparing the pressure of the anal sphincters before and after treatment. All data were analyzed in univariate and multivariate analysis. RESULTS: Two months after treatment, 65.4% of the patients had a healing scar. Only one patient had mild incontinence to flatus that lasted 3 wk after treatment, but this disappeared spontaneously. In the multivariate analysis of the data, two registered months after the treatment, sex (P = 0.01), baseline resting anal pressure (P = 0.02) and resting anal pressure 2 mo after treatment (P < 0.0001) were significantly related to healing rate.CONCLUSION: Botulinum toxin, despite worse results than in non-obese individuals, appears the best alternative to surgery for this group of patients with a high risk of incontinence. | Serafino Vanella Giuseppe Brisinda Gaia Marniga Anna Crocco Giuseppe Bianco Giorgio Maria | 2012 | World Journal of Gastroenterology2012，18，10： | 4 |
| 11 | Hepatitis C virus infection and health-related quality of life显示文摘Hepatitis C virus(HCV) hepatitis and other diseases related to HCV,such as cryoglobulinemia,lymphoma and renal failure,impair health-related quality of life(HRQoL).In addition,HCV per se might directly influence HRQoL via colonization of microglia in the brain or,indirectly,via the effect of systemic inflammatory cytokines which,in turn,can trigger brain interleukin production.The treatment of HCV-related disorders with interferon(IFN) has an effect on HRQoL.Initially,IFN causes a transient deterioration of HRQoL,due to the induction of depression and other side effects of treatment.Subsequently,the subjects who obtain a sustained virologic response experience an improvement in HRQoL.Only rarely does interferon treatment causes permanent detrimental effects on HRQoL,due to residual psychiatric or neurologic side effects.Liver transplantation is the only treatment for end-stage HCV-related liver disease.HRQoL generally improves massively a few months after transplantation,except in the case of serious complications of the transplant procedure.Furthermore,high levels of anxiety and neuroticism pre-transplant are associated with lower HRQoL one year after transplant.Additionally,six months after transplant,patients with HCV who experience virologic recurrence show significantly greater depression,anxiety,phobic anxiety,and paranoid ideation than anti-HCV-negative patients.In conclusion,optimal care for the overall well-being of patients with HCV infection requires adequate knowledge of their neurological and psychological status. | Piero Amodio Laura Salari Sara Montagnese Sami Schiff Daniele Neri Tonino Bianco Lina Minazzato | 2012 | World Journal of Gastroenterology2012，18，19： | 3 |
| 12 | Robot-assisted laparoscopic pancreatic surgery: single-surgeon experience显示文摘 | Pier Cristoforo Giulianotti Fabio Sbrana Francesco Maria Bianco Enrique Fernando Elli Galaxy Shah Pietro Addeo Giuseppe Caravaglios Andrea Coratti | 2010 | Surgical Endoscopy2010，，7： | 3 |
| 13 | Apple whole genome sequences:recent advances and new prospects显示文摘In 2010,a major scientific milestone was achieved for tree fruit crops:publication of the first draft whole genome sequence(WGS)for apple(Malus domestica).This WGS,v1.0,was valuable as the initial reference for sequence information,fine mapping,gene discovery,variant discovery,and tool development.A new,high quality apple WGS,GDDH13 v1.1,was released in 2017 and now serves as the reference genome for apple.Over the past decade,these apple WGSs have had an enormous impact on our understanding of apple biological functioning,trait physiology and inheritance,leading to practical applications for improving this highly valued crop.Causal gene identities for phenotypes of fundamental and practical interest can today be discovered much more rapidly.Genome-wide polymorphisms at high genetic resolution are screened efficiently over hundreds to thousands of individuals with new insights into genetic relationships and pedigrees.High-density genetic maps are constructed efficiently and quantitative trait loci for valuable traits are readily associated with positional candidate genes and/or converted into diagnostic tests for breeders.We understand the species,geographical,and genomic origins of domesticated apple more precisely,as well as its relationship to wild relatives.The WGS has turbo-charged application of these classical research steps to crop improvement and drives innovative methods to achieve more durable,environmentally sound,productive,and consumer-desirable apple production.This review includes examples of basic and practical breakthroughs and challenges in using the apple WGSs.Recommendations for“what’s next”focus on necessary upgrades to the genome sequence data pool,as well as for use of the data,to reach new frontiers in genomics-based scientific understanding of apple. | Cameron P.Peace Luca Bianco Michela Troggio Eric van de Weg Nicholas P.Howard Amandine Cornille Charles-Eric Durel Sean Myles ZoëMigicovsky Robert J.Schaffer Evelyne Costes Gennaro Fazio Hisayo Yamane Steve van Nocker Chris Gottschalk Fabrizio Costa David Chagné Xinzhong Zhang Andrea Patocchi Susan E.Gardiner Craig Hardner Satish Kumar Francois Laurens Etienne Bucher Dorrie Main Sook Jung Stijn Vanderzande | 2019 | Horticulture Research2019，6，1： | 3 |
| 14 | Bariatric surgery and diabetes remission: Sleeve gastrectomy or mini-gastric bypass?显示文摘AIM:To investigate the weight loss and glycemic control status[blood glucose,hemoglobin A1c(HbA1c)and hypoglycaemic treatment].METHODS:The primary risk factor for type 2 diabetes is obesity,and 90%of all patients with type 2 diabetes are overweight or obese.Although a remarkable effect of bariatric surgery is the profound and durable resolution of type 2 diabetes clinical manifestations,little is known about the difference among various weight loss surgical procedures on diabetes remission.Data from patients referred during a 3-year period(from January2009 to December 2011)to the University of Naples'FedericoⅡ'diagnosed with obesity and diabetes were retrieved from a prospective database.The patients were split into two groups according to the surgical intervention performed[sleeve gastrectomy(SG)and mini-gastric bypass(MGB)].Weight loss and glycemic control status(blood glucose,HbA1c and hypoglycaemic treatment)were evaluated.RESULTS:A total of 53 subjects who underwent sleeve gastrectomy or mini-gastric bypass for obesity and diabetes were screened for the inclusion in this study.Of these,4 subjects were excluded because of surgical complications,7 subjects were omitted because young surgeons conducted the operations and 11 subjects were removed because of the lack of follow-up.Thirtyone obese patients were recruited for this study.A total of 15 subjects underwent SG(48.4%),and 16underwent MGB(51.6%).After adjusting for various clinical and demographic characteristics in a multivariate logistic regression analysis,high hemoglobin A1c was determined to be a negative predictor of diabetes remission at 12 mo(OR=0.366,95%CI:0.152-0.884).Using the same regression model,MGB showed a clear trend toward higher diabetes remission rates relative to SG(OR=3.780,95%CI:0.961-14.872).CONCLUSION:Although our results are encouraging regarding the effectiveness of mini-gastric bypass on diabetes remission,further studies are needed to provide definitive conclusions in selecting the ideal procedure for diabetes remission. | Marco Milone Matteo Nicola Dario Di Minno Maddalena Leongito Paola Maietta Paolo Bianco Caterina Taffuri Dario Gaudioso Roberta Lupoli Silvia Savastano Francesco Milone Mario Musella | 2013 | World Journal of Gastroenterology2013，19，39： | 3 |
| 15 | A high-density, multi-parental SNP genetic map on apple validates a new mapping approach for outcrossing species显示文摘Quantitative trait loci(QTL)mapping approaches rely on the correct ordering of molecular markers along the chromosomes,which can be obtained from genetic linkage maps or a reference genome sequence.For apple(Malus domestica Borkh),the genome sequence v1 and v2 could not meet this need;therefore,a novel approach was devised to develop a dense genetic linkage map,providing the most reliable marker-loci order for the highest possible number of markers.The approach was based on four strategies:(i)the use of multiple full-sib families,(ii)the reduction of missing information through the use of HaploBlocks and alternative calling procedures for single-nucleotide polymorphism(SNP)markers,(iii)the construction of a single backcross-type data set including all families,and(iv)a two-step map generation procedure based on the sequential inclusion of markers.The map comprises 15417 SNP markers,clustered in 3 K HaploBlock markers spanning 1267 cM,with an average distance between adjacent markers of 0.37 cM and a maximum distance of 3.29 cM.Moreover,chromosome 5 was oriented according to its homoeologous chromosome 10.This map was useful to improve the apple genome sequence,design the Axiom Apple 480 K SNP array and perform multifamily-based QTL studies.Its collinearity with the genome sequences v1 and v3 are reported.To our knowledge,this is the shortest published SNP map in apple,while including the largest number of markers,families and individuals.This result validates our methodology,proving its value for the construction of integrated linkage maps for any outbreeding species. | Erica A Di Pierro Luca Gianfranceschi Mario Di Guardo Herma JJ Koehorst-van Putten Johannes W Kruisselbrink Sara Longhi Michela Troggio Luca Bianco Hélène Muranty Giulia Pagliarani Stefano Tartarini Thomas Letschka Lidia Lozano Luis Larisa Garkava-Gustavsson Diego Micheletti Marco CAM Bink Roeland E Voorrips Ebrahimi Aziz Riccardo Velasco François Laurens W Eric van de Weg | 2016 | Horticulture Research2016，3，1： | 3 |
| 16 | Undernutrition, risk of malnutrition and obesity in gastroenterological patients: A multicenter study显示文摘AIM: To investigate the prevalence of undernutrition, risk of malnutrition and obesity in the Italian gastroenterological population. METHODS: The Italian Hospital Gastroenterology Association conducted an observational, cross-sectional multicenter study. Weight, weight loss, and body mass index were evaluated. Undernutrition was defined as unintentional weight loss > 10% in the last threesix months. Values of Malnutrition Universal Screening Tool(MUST) > 2, NRS-2002 > 3, and Mini Nutritional Assessment(MNA) from 17 to 25 identified risk of malnutrition in outpatients, inpatients and elderly patients, respectively. A body mass index ≥ 30 indicated obesity. Gastrointestinal pathologies were categorized into acute, chronic and neoplastic diseases. RESULTS: A total of 513 patients participated in the study. The prevalence of undernutrition was 4.6% in outpatients and 19.6% in inpatients. Moreover, undernutrition was present in 4.3% of the gastrointestinal patients with chronic disease, 11.0% of those with acute disease, and 17.6% of those with cancer. The risk of malnutrition increased progressively and significantly in chronic, acute and neoplastic gastrointestinal diseases in inpatients and the elderly population. Logistical regression analysis confirmed that cancer was a risk factor for undernutrition(OR = 2.7; 95%CI: 1.2-6.44, P = 0.02). Obesity and overweight were more frequent in outpatients. CONCLUSION: More than 63% of outpatients and 80% of inpatients in gastroenterological centers suffered from significant changes in body composition and required specific nutritional competence and treatment. | Massimiliano Rizzi Silvia Mazzuoli Nunzia Regano Rosa Inguaggiato Margherita Bianco Gioacchino Leandro Elisabetta Bugianesi Donatella Noè Nicoletta Orzes Paolo Pallini Maria Letizia Petroni Gianni Testino Francesco William Guglielmi | 2016 | World Journal of Gastrointestinal Oncology2016，8，7： | 2 |
| 17 | MicroRNA-181b通过调节金属蛋白酶组织抑制因子3和弹性蛋白影响动脉粥样硬化和动脉瘤显示文摘动脉粥样硬化和动脉瘤是全世界人口的主要致死原因。微小RNA(microRNAs,miR)是基因和蛋白表达的关键决定因素,miR异常表达与许多心血管病有关。目前对它们在动脉粥样硬化斑块和腹主动脉瘤(abdominal aortic aneurysm,AAA)稳定性中的作用了解甚少。 | 刘莉 叶鹏 Di Gregoli K Mohamad Anuar NN Bianco R White SJ Newby AC George SJ Johnson JL | 2016 | 中华高血压杂志2016，24，11： | 2 |
| 18 | Effects of prostaglandin E1α cyclodestrin treatment on endothelial dysfunction in patients with systemic sclerosis显示文摘 | Cristina Giannattasio MariaRosa Pozzi Marco Gradinali Elisabetta Montemerlo Francesca Citterio Silvia Maestroni Elena Fantini Monica Failla Maria Robuschi Salvatore Bianco Giuseppe Mancia | 2007 | Journal of Hypertension2007，，4： | 2 |
| 19 | 血型抗原在传染病中的作用显示文摘血型抗原可将感染因子作为受体与疾病直接关联,或间接被宿主的免疫反应干扰。只有少数感染因子,如:疟原虫、细小病毒B19影响红细胞及其前体。大多数感染因子附着在红细胞上,把它们作为靶器官的载体,或附着在粘膜表达的血型抗原上。 血型抗原与疾病之间的一些关系可起因于它们与补体系统的关系:KN抗原(Kn,McC和Yk)位于补体Ⅰ型受体分子(C R1)上,CR1存在于RBCs、淋巴细胞、巨噬细胞、树突状细胞及单核细胞上。RBCs上的CR1数量在免疫学疾病发生时减少,如获得性免疫缺乏症、系统性红斑狼疮及一些肿瘤。 获得性和先天性遗传疾病 正常RBC表面含有糖基磷脂酰肌醇GPI联结蛋白,GPI调节因子在阵发性睡眠性血红蛋白尿个体中缺失,使RBC对补体介导的溶解更敏感,受影响的RBC缺失所有Cromer、Yt、JMH、Do和Emm血型抗原。 | Maria Rios Celso Bianco 李芳 | 2001 | 国外医学(输血及血液学分册)2001，24，5： | 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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