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Variable outcome in infantile-onset inflammatory bowel disease in an Asian cohort

World Journal of gastroenterology 2016年第48期22卷 10653-10662页

作者： Way Seah Lee Ruey Terng Ng Koon-Wing Chan Yu-Lung Lau Department of paediatrics University Malaya Medical Center University Malaya paediatric and Child Health Research Group University Malaya Department of paediatrics and Adolescent Medicine Queen Mary Hospital LKS Faculty of Medicine The University of Hong Kong

AIM Infantile-onset inflammatory bowel disease(IO-IBD) with the onset of disease before 12 mo of age, is a different disease entity from childhood IBD. We aimed to describe the clinical features, outcome and role of mutation in interleukin-10(IL-10) and interleukin-10 receptors(IL-10R) in Asian children with IO-IBD. METHODS All cases of IO-IBD, defined as onset of disease before 12 mo of age, seen at University Malaya Medical Center, Malaysia were reviewed. We performed mutational analysis for IL10 and IL10 R genes in patients with presenting clinical features of Crohn's disease(CD).RESULTS Six [13%; CD = 3, ulcerative colitis(UC) = 2, IBDunclassified(IBD-U) = 1] of the 48 children(CD = 25; UC = 23) with IBD have IO-IBD. At final review [median(range) duration of follow-up: 6.5(3.0-20) years], three patients were in remission without immunosuppression [one each for post-colostomy(IBD-U), after standard immunosuppression(CD), and after total colectomy(UC)]. Three patients were on immunosuppression:one(UC) was in remission while two(both CD) had persistent disease. As compared with later-onset disease, IO-IBD were more likely to present with bloody diarrhea(100% vs 55%, P = 0.039) but were similar in terms of an associated autoimmune liver disease(0% vs 19%, P = 0.31), requiring biologics therapy(50% vs 36%, P = 0.40), surgery(50% vs 29%, P = 0.27), or achieving remission(50% vs 64%, P = 0.40). No mutations in either IL10 or IL10 R in the three patients with CD and the only patient with IBD-U were *** The clinical features of IO-IBD in this Asian cohort of children who were negative for IL-10 or IL-10 R mutations were variable. As compared to childhood IBD with onset of disease after 12 mo of age, IO-IBD achieved remission at a similar rate.

关键词： Infantile-onset inflammatory bowel disease Pediatric

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Apolipoprotein E variants correlate with the clinical presentation of paediatric inflammatory bowel disease:A cross-sectional study

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World Journal of gastroenterology 2021年第14期27卷 1483-1496页

作者： Aleksandra Glapa-Nowak Mariusz Szczepanik Barbara Iwańczak Jarosław Kwiecień Anna Barbara Szaflarska-Popławska Urszula Grzybowska-Chlebowczyk Marcin Osiecki Marcin Dziekiewicz Andrzej Stawarski Jarosław Kierkuś Tomasz Banasiewicz Aleksandra Banaszkiewicz Jarosław Walkowiak Department of Pediatric gastroenterology and Metabolic Diseases PoznańUniversity of Medical SciencesPoznań60-572Poland Department of Pediatrics Medical University of WroclawWroclaw 50-369Poland Department of Pediatrics Faculty of Medical Sciences in ZabrzeMedical University of SilesiaZabrze 41-800Poland Department of Pediatrics Faculty of Medical SciencesMedical University of Silesia in KatowiceKatowice 40-752Poland Department of gastroenterology HepatologyFeeding Disorders and PaediatricsThe Children’s Memorial Health InstituteWarsaw 04-730Poland Department of Pediatric gastroenterology and Nutrition Medical University of WarsawWarsaw 02-091Poland Department and Clinic of Pediatrics Gastroenterology and NutritionWroclaw Medical UniversityWroclaw 50-369Poland Chair and Department of General Surgery Gastroenterological Surgical Oncology and Plastic SurgeryPoznańUniversity of Medical SciencesPoznań60-355Poland

BACKGROUND It has been suggested that apolipoprotein E(APOE)polymorphisms are associated with the risk of developing inflammatory bowel disease(IBD)and the early age of disease ***,there are no reports regarding the relationship with clinical characteristics and disease *** To summarise that APOE polymorphisms are associated with the risk of developing IBD and the early age of disease *** In total,406 patients aged 3-18 with IBD(192 had ulcerative colitis and 214 had Crohn’s disease)were genotyped using the TaqMan hydrolysis probe *** expression was described at diagnosis and the worst flare by disease activity scales,albumin and C-reactive protein levels,localisation and behaviour(Paris classification).Systemic steroid intake with the total number of courses,immunosuppressive,biological,and surgical treatment with the time and age of the first intervention were *** total number of exacerbation-caused hospitalisations,the number of days spent in hospital due to exacerbation,the number of relapses,and severe relapses were also *** Ulcerative colitis patients with the APOEε4 allele had lower C-reactive protein values at diagnosis(P=0.0435)and the worst flare(P=0.0013)compared to patients with the APOEε2 allele and genotype APOEε3/ε***’s disease patients with the APOEε2 allele scored lower on the Pediatric Crohn’s Disease Activity Index at diagnosis(P=0.0204).IBD patients with APOEε2 allele spent fewer days in the hospital due to relapse(P=0.0440).CONCLUSION APOE polymorphisms are associated with the risk of developing IBD and the clinical expression of ***,the clinical relevance of the differences identified is rather modest.

关键词： Apolipoprotein E polymorphism Crohn’s disease Ulcerative colitis Immunosuppression Surgery Disease severity

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Detection and characterization of murine colitis and carcinogenesis by molecularly targeted contrast-enhanced ultrasound

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World Journal of gastroenterology 2017年第16期23卷 2899-2911页

作者： Markus Brückner Jan Heidemann Tobias M Nowacki Friederike Cordes Jorg Stypmann Philipp Lenz Faekah Gohar Andreas Lügering Dominik Bettenworth Department of Medicine B University Hospital of MünsterD-48149 MünsterGermany Department of gastroenterology Klinikum Bielefeld MitteAcademic Teaching HospitalUniversity of MünsterD-33604 BielefeldGermany Department of Cardiology and Angiology University Hospital of MünsterD-48149 MünsterGermany Department of Palliative Care University Hospital of MünsterD-48149 MünsterGermany Department of paediatric Rheumatology and Immunology University Hospital of MünsterD-48149 MünsterGermany Medical Care Center Portal 10 D-48155 MünsterGermany

AIM To study mucosal addressin cellular adhesion molecule-1(MAd CAM-1) and vascular endothelial growth factor(VEGF)-targeted contrast enhanced ultrasound(CEUS) for the assessment of murine colitis and carcinogenesis. METHODS C57BL/6 mice were challenged with 3% dextran sodium-sulfate(DSS) for three, six or nine days to study the development of acute colitis. Ultrasound was performed with and without the addition of unspecific contrast agents. MAd CAM-1-targeted contrast agent was used to detect and quantify MAd CAM-1 expression. Inflammatory driven colorectal azoxymethane(AOM)/DSS-induced carcinogenesis was examined on day 42 and 84 using VEGF-targeted contrast agent. Highly specific tissue echogenicity was quantified using specialized software. Sonographic findings were correlated to tissue staining, western blot analysis and immunohistochemistry to quantify the degree of inflammation and stage of carcinogenesis. RESULTS Native ultrasound detected increased general bowel wall thickening that correlated with more progressed and more severe DSS-colitis(healthy mice: 0.3 mm ± 0.03 vs six days DSS: 0.5 mm ± 0.2 vs nine days DSS: 0.6 mm ± 0.2, P < 0.05). Moreover, these sonographic findings correlated well with clinical parameters such as weight loss(r2 = 0.74) and histological damage(r2 = 0.86)(P < 0.01). In acute DSS-induced murine colitis, CEUS targeted against MAd CAM-1 detected and differentiated stages of mild, moderate and severe colitis via calculation of mean pixel contrast intensity in decibel(9.6 d B ± 1.6 vs 12.9 d B ± 1.4 vs 18 d B ± 3.33, P < 0.05). Employing the AOM/DSSinduced carcinogenesis model, tumor development was monitored by CEUS targeted against VEGF and detected a significantly increased echogenicity in tumors as compared to adjacent healthy mucosa(healthy mucosa, 1.6 d B ± 1.4 vs 42 d, 18.2 d B ± 3.3 vs 84 d, 18.6 d B ± 4.9, P < 0.01). Tissue echogenicity strongly correlated with histological analysis and immunohistochemistry findings(VEGF-posi

关键词： Colitis Dextran sodium-sulfate AOMDSS Carcinogenesis Ultrasound Contrast-enhanced ultrasound Vascular endothelial growth factor Mucosal addressin cellular adhesion molecule-1

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Magnets, children and the bowel: A dangerous attraction?

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World Journal of gastroenterology 2012年第38期18卷 5324-5328页

作者： Anil Thomas George Sandeep Motiwale Department of Physiology St Mark's Hospital London HA1 3UJ United Kingdom Department of paediatric Surgery Queen's Medical Center University Hospitals Nottingham NG7 2UH United Kingdom

Reports of magnet ingestion are increasing rapidly globally. However, multiple magnet ingestion, the subsequent potential complications and the importance of the early identification and proper management remain both under-recognized and underestimated. Published literature on such cases could possibly represent only the tip of an iceberg with press reports, web blogs and government documents highlighting further occurrence of many more such incidents. The increasing number of complications worldwide being reported secondary to magnet ingestion point not only to an acute lack of awareness about this condition among the medical profession but also among parents and carers who will be in most cases the first to pick up on magnet ingestion. There still seems to be no consensus on the management of magnet ingestion with several algorithms being proposed for management. Prevention of this condition remains a much better option than cure. Proper education and improved awareness among parents and carers and frontline medical staff is key in addressing this rapidly emerging problem. The goal of managing such cases of suspected magnet ingestion should be aimed at reducing delays between ingestion time, diagnosis time and intervention time.

关键词： Multiple magnet ingestion Children Bowel injury Fistulation Necrosis

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应该在开放获取期刊上发表文章吗?

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英国医学杂志中文版 2020年第11期23卷 639-641页

作者： Matthew Kurien David S Sanders James J Ashton R Mark Beattie 赵巍(译) Academic unit of gastroenterology Departments of Infection and Immunity and Cardiovascular ScienceUniversity of Sheffield Medical SchoolSheffieldUK Department of gastroenterology Royal Hallamshire HospitalSheffield Department of paediatric gastroenterology Southampton Children's HospitalUK Department of Human Genetics and Genomic Medicine University of Southampton 中华医学会杂志社总编室

Matthew Kurien和David S Sanders说,"作者付费"出版模式是让所有人都能获得生物医学研究成果的唯一公平途径,但James J Ashton和R Mark Beattie担心,这会导致证据基础因商业驱动造成偏倚。

关键词：医学研究成果出版模式开放获取期刊证据基础

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欧洲儿科胃肠病学、肝病学和营养协会儿童及青少年炎症性肠病诊断的改良波尔图标准(2014年版)

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中华儿科杂志 2016年第10期54卷 728-732页

作者： Arie Levine Sibylle Koletzko Dan Turner Johanna C.Escher Salvatore Cucchiara Lissy de Ridder Kaija-Leena Kolho Gabor Veres Richard K.Russell Anders Paerregaard Stephan Buderus Mary-Louise C.Greer Jorge A.Dias Gigi Veereman-Wauters Paolo Lionetti Malgorzata Sladek Javier Martin de Carpi Annamaria Staiano Frank M.Ruemmele David C.Wilson 罗优优陈洁 Pediatric gastroenterology and Nutrition unit Wolfson Medical CenterSackler School of MedicineTel-Aviv University the Dr von Hauner Children's Hospital Ludwig Maximilians University the Pediatric gastroenterology unit Shaare Zedek Medical CenterThe Hebrew University of Jerusalem the Pediatric gastroenterology Department of PediatricsErasmus MC-Sophia Children's Hospital 5. the Pediatric Gastroenterology and Liver UnitSapienza University of Rome 6. the Children's HospitalUniversity of Helsinki 7. the Semmelweis University 8. the Department of Paediatric Gastroenterology and NutritionYorkhill Children's Hospital 9. the Department of PaediatricsHvidovre University Hospital 10. the St.Marien-HospitalDepartment of Pediatrics 11. the Department of Diagnostic ImagingThe Hospital for Sick Childrenthe Department of Medical ImagingUniversity of Toronto 12. the Hospital S.Joao 13. the-Pediatric Gastroenterology and Nutrition 14. the Departement NeurofarbaUniversity of FlorenceMeyer Children Hospital 15. the Department of PediatricsGastroenterology and NutritionJagiellonian University Medical College 16. the Department of Pediatric GastroenterologyHepatology and NutritionHospital Sant Joan de De'u 17. the Department of Translational Medical SciencesSection of PediatricsUniversit

炎症性肠病(IBD)包括克罗恩病(CD)、溃疡性结肠炎(UC)和未分型IBD(IBDU)。IBD的确切诊断需综合病史、体检、实验室检查、内镜以及影像学的检查,并严格排除肠道感染、过敏性疾病或原发性免疫缺陷病(PID)。近年对儿童期起病的IBD(PIBD)的... 详细信息

炎症性肠病(IBD)包括克罗恩病(CD)、溃疡性结肠炎(UC)和未分型IBD(IBDU)。IBD的确切诊断需综合病史、体检、实验室检查、内镜以及影像学的检查,并严格排除肠道感染、过敏性疾病或原发性免疫缺陷病(PID)。近年对儿童期起病的IBD(PIBD)的研究高度提示3种PIBD的亚型均存在不典型的表型。本标准对2005年波尔图标准进行改良,利用循证方法,整合最新证据,包括诊断方法、以巴黎分型为基础的PIBD亚型的定义以及诊断误区,为新一代的PIBD提供优质的个体化管理以及可靠的诊断、评估和预后。

关键词：体检实验室检查内镜

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Homologous recombination mediates stable Fah gene integration and phenotypic correction in tyrosinaemia mouse-model

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World Journal of Hepatology 2018年第2期10卷 277-286页

作者： Norman Junge Qinggong Yuan Thu Huong Vu Simon Krooss Christien Bednarski Asha Balakrishnan Toni Cathomen Michael P Manns Ulrich Baumann Amar Deep Sharma Michael Ott Department of Pediatric gastroenterology and Hepatology Hannover Medical School Department of gastroenterology Hepatology and Endocrinology Hannover Medical School TWINCORE Centre for Experimental and Clinical Infection Research Medical Center University of Freiburg Institute for Cell and Gene Therapy Research Group Micro RNA in Liver Regeneration Cluster of Excellence REBIRTH Hannover Medical School

AIM To stably correct tyrosinaemia in proliferating livers of fumarylacetoacetate-hydrolase knockout(Fah-/-) mice by homologous-recombination-mediated targeted addition of the Fah *** C57 BL/6 Fah?exon5 mice served as an animal model for human tyrosinaemia type 1 in our study. The vector was created by amplifying human Fah c DNA including the TTR promoter from a lentivirus plasmid as described. The Fah expression cassette was flanked by homologous arms(620 bp and 749 bp long) of the Rosa26 gene locus. Mice were injected with 2.1 × 108 VP of this vector(r ***) via the tail vein. Mice in the control group were injected with 2.1 × 108 VP of a similar vector but missing the homologous arms(r ***). Primary hepatocytes from Fah-/-recipient mice, treated with our vectors, were isolated and 1 × 106 hepatocytes were transplanted into secondary Fah-/-recipient mice by injection into the spleen. Upon either vector application or hepatocyte transplantation NTBC treatment was stopped in recipient mice. RESULTS Here, we report successful HR-mediated genome editing by integration of a Fah gene expression cassette into the "safe harbour locus" Rosa26 by recombinant AAV8. Both groups of mice showed long-term survival, weight gain and FAH positive clusters as determined by immunohistochemistry analysis of liver sections in the absence of NTBC treatment. In the group of C57 BL/6 Fah?exon5 mice, which have been transplanted with hepatocytes from a mouse injected with r *** 156 d before, 6 out of 6 mice showed long-term survival, weight gain and FAH positive clusters without need for NTBC treatment. In contrast only 1 out 5 mice, who received hepatocytes from r *** treated mice, survived and showed few and smaller FAH positive clusters. These results demonstrate that homologous recombinationmediated Fah gene transfer corrects the phenotype in a mouse model of human tyrosinaemia type 1(Fah-/-mice) and

关键词： Gene therapy AAV8 Liver based metabolic disease Targeted integration ROSA26 paediatric liver disease

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Diagnosis in bile acid-CoA:Amino acid N-acyltransferase deficiency

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World Journal of gastroenterology 2012年第25期18卷 3322-3326页

作者： Nedim Hadzi Laura N Bull Peter T Clayton AS Knisely paediatric Liver Service and Institute of Liver Studies King's College HospitalLondon SE5 9RSUnited Kingdom Liver Center Laboratory University of California San FranciscoSan FranciscoCA 94110United States Biochemistry Research Group Clinical and Molecular Genetics UnitUniversity College London Institute of Child HealthLondon WC1 N1EHUnited Kingdom Institute of Liver Studies King's College HospitalLondon SE5 9RSUnited Kingdom

Cholate-CoA ligase （,EEL） and bile acid-CoA： amino acid N-acyltransferase （BAAT） sequentially mediate bile-acid amidation. Defects can cause intrahepatic cholestasis. Distinction has required gene sequencing. We assessed potential clinical utility of immunostaining of liver for CCL and BAAT. Using commercially available antibodies against BAAT and CCL, we immunostained liver from an infant with jaundice, deficiency of amidated bile acids, and transcription-terminating mutation in BAAT. CCL was normally expressed. BAAT expression was not de- tected. Immunostaining may facilitate diagnosis in bile- acid amidation defects.

关键词： Amidation Bile acid-CoA Amino acid N-ac- yltransferase Cholate-CoA ligase, Cholestasis Conjuga-tion Electrospray ionisation-mass spectroscopy Immu-nohistochemistry Liver Neonatal hepatitis SIC27A5 Transmission electron microscopy

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Hepatocellular adenoma in the paediatric population:Molecular classification and clinical associations

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World Journal of gastroenterology 2020年第19期26卷 2294-2304页

作者： Elan Hahn Juan Putra Division of Pathology Department of Paediatric Laboratory Medicinethe Hospital for Sick ChildrenToronto M5G 1X8ONCanada Department of Laboratory Medicine and Pathobiology University of TorontoToronto M5S 1A8ONCanada

Hepatocellular adenomas(HCAs)represent rare,benign liver tumours occurring predominantly in females taking oral *** children,HCAs comprise less than 5%of hepatic tumours and demonstrate association with various *** contemporary classification of HCAs,based on their distinctive genotypes and clinical phenotypes,includes hepatocyte nuclear factor 1 homeobox alpha-inactivated HCAs,beta-catenin-mutated HCAs,inflammatory HCAs,combined beta-catenin-mutated and inflammatory HCAs,sonic hedgehogactivated HCAs,and unclassified *** children,there is a lack of literature on the characteristics and distribution of HCA *** this review,we summarized different HCA subtypes and the clinicopathologic spectrum of HCAs in the paediatric population.

关键词： paediatric Hepatocellular adenoma Malignant transformation Beta-catenin HNF1A Glycogen storage disorders

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Consumption of gluten with gluten-degrading enzyme by celiac patients: A pilot-study

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World Journal of gastroenterology 2013年第35期19卷 5837-5847页

作者： Greetje J Tack Jolanda MW van de Water Maaike J Bruins Engelina MC Kooy-Winkelaar Jeroen van Bergen Petra Bonnet Anita CE Vreugdenhil Ilma Korponay-Szabo Luppo Edens B Mary E von Blomberg Marco WJ Schreurs Chris J Mulder Frits Koning Department of gastroenterology and Hepatology VU University Medical Centre DSM Biotechnology Centre Department of Immunohematology and Blood Transfusion Leiden University Medical Centre Department of Pathology VU University Medical Centre Department of paediatrics University Hospital Maastricht Department of paediatrics University of Debrecen paediatric Research Centre University of Tampere

AIM:To assesses the safety and efficacy of Aspergillus niger prolyl endoprotease(AN-PEP)to mitigate the im-munogenic effects of gluten in celiac ***:Patients with initial diagnosis of celiac disease as confirmed by positive serology with subtotal or total villous atrophy on duodenal biopsies who adhere to a strict gluten-free diet(GFD)resulting in normalised antibodies and mucosal healing classified as Marsh 0 orⅠwere *** a randomised double-blind placebo-controlled pilot study,patients consumed toast(approximately 7 g/d gluten)with AN-PEP for 2 wk(safety phase).After a 2-wk washout period with adherence of the usual GFD,14 patients were randomised to gluten intake with either AN-PEP or placebo for 2 wk(efficacy phase).Measurements at baseline included complaints,quality-of-life,serum antibodies,immunophenotyping of T-cells and duodenal mucosa ***,serum and quality of life questionnaires were collected during and after the safety,washout and efficacy *** biopsies were collected after the safety phase and after the efficacy phase.A change in histological evaluation according to the modified Marsh classification was the primary ***:In total,16 adults were enrolled in the *** serious adverse events occurred during the trial and no patients withdrew during the *** mean score for the gastrointestinal subcategory of the celiac disease quality(CDQ)was relatively high throughout the study,indicating that AN-PEP was well *** the efficacy phase,the CDQ scores of patients consuming gluten with placebo or gluten with AN-PEP did not significantly deteriorate and moreover no differences between the groups were *** the efficacy phase,neither the placebo nor the AN-PEP group developed significant antibody *** IgA-EM concentrations remained negative in both *** patients were excluded from entering the efficacy phase as their mucosa showed an increase oftwo Marsh steps a

关键词： Celiac disease Gluten Enzyme Prolyl endoprotease Aspergillus niger prolyl endoprotease Treatment Adverse events efficacy IgA-tTG intestinal deposits

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