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https://www.readbyqxmd.com/read/29454792/clinical-and-genomic-correlates-of-neutrophil-reactive-oxygen-species-production-in-pediatric-patients-with-crohn-s-disease
#1
Lee A Denson, Ingrid Jurickova, Rebekah Karns, Kelly A Shaw, David J Cutler, David Okou, Anne Dodd, Kathryn Quinn, Kajari Mondal, Bruce J Aronow, Yael Haberman, Aaron Linn, Adam Price, Ramona Bezold, Kathleen Lake, Kimberly Jackson, Thomas D Walters, Anne Griffiths, Robert N Baldassano, Joshua D Noe, Jeffrey S Hyams, Wallace V Crandall, Barbara S Kirschner, Melvin B Heyman, Scott Snapper, Stephen L Guthery, Marla C Dubinsky, Neal S Leleiko, Anthony R Otley, Ramnik J Xavier, Christine Stevens, Mark J Daly, Michael E Zwick, Subra Kugathasan
BACKGROUND & AIMS: Individuals with monogenic disorders of phagocyte function develop chronic colitis that resembles Crohn's Disease (CD). We tested for associations between mutations in genes encoding NADPH oxidases, neutrophil function, and phenotypes of CD in pediatric patients. METHODS: We performed whole-exome sequence analysis to identify mutations in genes encoding NADPH oxidases (such as CYBA, CYBB, NCF1, NCF2, NCF4, RAC1, and RAC2) using DNA from 543 pediatric patients with inflammatory bowel diseases...
February 15, 2018: Gastroenterology
https://www.readbyqxmd.com/read/29450466/diagnostic-criteria-of-ulcerative-pyoderma-gangrenosum-a-delphi-consensus-of-international-experts
#2
Emanual Maverakis, Chelsea Ma, Kanade Shinkai, David Fiorentino, Jeffrey P Callen, Uwe Wollina, Angelo Valerio Marzano, Daniel Wallach, Kyoungmi Kim, Courtney Schadt, Anthony Ormerod, Maxwell A Fung, Andrea Steel, Forum Patel, Rosie Qin, Fiona Craig, Hywel C Williams, Frank Powell, Alexander Merleev, Michelle Y Cheng
Importance: Pyoderma gangrenosum is a rare inflammatory skin condition that is difficult to diagnose. Currently, it is a "diagnosis of exclusion," a definition not compatible with clinical decision making or inclusion for clinical trials. Objective: To propose and validate diagnostic criteria for ulcerative pyoderma gangrenosum. Evidence Review: Diagnostic criteria were created following a Delphi consensus exercise using the RAND/UCLA Appropriateness Method...
February 14, 2018: JAMA Dermatology
https://www.readbyqxmd.com/read/29450453/the-association-of-age-with-clinical-presentation-and-comorbidities-of-pyoderma-gangrenosum
#3
Hovik J Ashchyan, Daniel C Butler, Caroline A Nelson, Megan H Noe, William G Tsiaras, Stephen J Lockwood, William D James, Robert G Micheletti, Misha Rosenbach, Arash Mostaghimi
Importance: Pyoderma gangrenosum is an inflammatory neutrophilic dermatosis. Current knowledge of this rare disease is limited owing to a lack of validated diagnostic criteria and large population studies. Objective: To evaluate the association of age with the clinical presentation and comorbidities of pyoderma gangrenosum. Design, Setting, and Participants: This was a multicenter retrospective cohort study performed at tertiary academic referral centers in urban settings...
February 14, 2018: JAMA Dermatology
https://www.readbyqxmd.com/read/29449577/neutralization-of-il-15-abrogates-experimental-immune-mediated-cholangitis-in-diet-induced-obese-mice
#4
José L Reyes, Danielle T Vannan, Tina Vo, Aliya Gulamhusein, Paul L Beck, Raylene A Reimer, Bertus Eksteen
Obesity is a global epidemic affecting chronic inflammatory diseases. Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that can occur as an extraintestinal manifestation of inflammatory bowel disease (IBD). Previously we reported that patients with PSC who are obese have a higher risk of advanced liver disease. Currently it is unknown how obesity accelerates or worsens PSC. We evaluated the progression of PSC in an antigen-driven cholangitis mouse model of diet-induced obesity. Obesity was induced in our murine model of immune-mediated cholangitis (OVAbil)...
February 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29447637/crohn-disease-associated-neutrophilic-urticarial-dermatosis-report-and-literature-review-of-neutrophilic-urticarial-dermatosis
#5
Zhe Hou, Brian R Hinds, Philip R Cohen
Neutrophilic urticarial dermatosis (NUD) is a useful diagnostic term for urticarial lesions that are less pruritic and more painful than conventional urticaria. The histopathologic features include neutrophilic infiltrates in the interstitial dermis with a higher density than idiopathic urticaria. NUD has been associated with several systemic conditions, which are predominantly autoimmune and autoinflammatory in nature. A woman with Crohn disease who developed NUD is described. Literature reports of other conditions in which neutrophilic urticarial dermatosis have been observed are also reviewed and summarized...
November 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29438165/histologic-features-of-gastrointestinal-tract-biopsies-in-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura
#6
Christine Y Louie, Adam J Gomez, Richard K Sibley, Dorsey Bass, Teri A Longacre
Immunoglobulin A (IgA) vasculitis or Henoch-Schönlein purpura (HSP) typically occurs in the pediatric population, although rare cases also occur in adults. Gastrointestinal (GI) involvement is common. The "classic" histologic finding in IgA vasculitis (HSP) is leukocytoclastic vasculitis (LCV); other histologic features in biopsies of IgA vasculitis (HSP) have only been rarely described. The pathology archival files at our institution were searched for GI biopsies from patients with IgA vasculitis (HSP)...
February 12, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29437913/comparison-of-different-histological-indexes-in-the-assessment-of-uc-activity-and-their-accuracy-regarding-endoscopic-outcomes-and-faecal-calprotectin-levels
#7
Fernando Magro, Joanne Lopes, Paula Borralho, Susana Lopes, Rosa Coelho, José Cotter, Francisca Dias de Castro, Helena Tavares de Sousa, Marta Salgado, Patrícia Andrade, Ana Isabel Vieira, Pedro Figueiredo, Paulo Caldeira, A Sousa, Maria A Duarte, Filipa Ávila, João Silva, Joana Moleiro, Sofia Mendes, Sílvia Giestas, Paula Ministro, Paula Sousa, Raquel Gonçalves, Bruno Gonçalves, Ana Oliveira, Isadora Rosa, Marta Rodrigues, Cristina Chagas, Cláudia Camila Dias, Joana Afonso, Karel Geboes, Fátima Carneiro
OBJECTIVE: Histological remission is being increasingly acknowledged as a therapeutic endpoint in patients with UC. The work hereafter described aimed to evaluate the concordance between three histological classification systems-Geboes Score (GS), Nancy Index (NI) and RobartsHistopathologyIndex (RHI), as well as to evaluate their association with the endoscopic outcomes and the faecal calprotectin (FC) levels. DESIGN: Biopsy samples from 377 patients with UC were blindly evaluated using GS, NI and RHI...
February 3, 2018: Gut
https://www.readbyqxmd.com/read/29421581/therapeutic-efficacy-of-osthole-against-dinitrobenzene-sulphonic-acid-induced-colitis-in-rats
#8
Hanan Khairy, Hanan Saleh, Abeer M Badr, Mohamed-Assem S Marie
Several mediators were associated with the pathogenesis of inflammatory bowel disease such as oxidative stress through the production of reactive oxygen metabolites, neutrophils infiltration and release of pro-inflammatory cytokines. This study was designed to investigate the therapeutic efficacy of osthole against dinitrobenzene sulfonic acid (DNBS) induced-colitis in rats through its anti-oxidant and anti-inflammatory properties. Colitis was induced in rats by single intracolonic instillation of (250 μl DNBS-25 mg/rat)...
February 5, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29406827/pyoderma-gangrenosum-a-review-of-pathogenesis-and-treatment
#9
Christine Ahn, Deborah Negus, William Huang
Pyoderma gangrenosum (PG) is a complex neutrophilic dermatosis that can occur as an idiopathic disease, in association with systemic conditions such as inflammatory bowel disease, as part of an inherited inflammatory syndrome. It can be challenging to treat, as it occurs in a wide variety of clinical settings and there is a lack of a standardized treatment approach. The main limitations to treatment have been an incomplete understanding of the pathogenesis. However, recent advances have been made in understanding the pathogenesis of this condition, and PG is now considered an autoinflammatory disease process...
February 6, 2018: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29386837/paraneoplastic-pyoderma-gangrenosum-associated-with-rectal-adenocarcinoma
#10
Hye Rin You, Jae-Kyun Ju, Sook Jung Yun, Jee-Bum Lee, Seong-Jin Kim, Young Ho Won, Seung-Chul Lee
Pyoderma gangrenosum (PG) is a rare chronic neutrophilic dermatosis characterized by painful necrotic ulceration. The most common diseases associated with PG are inflammatory bowel disease, certain rheumatologic and hematologic diseases, and malignancy. Here, we describe the case of a 60-year-old man who presented with pruritic and painful erythematous ulcerative macules and patches on both lower extremities, and was diagnosed with PG based on his clinical and histologic features. His PG became exacerbated despite standard therapy with a high-dose systemic steroid in combination with dapsone and cyclosporine...
February 2018: Annals of Dermatology
https://www.readbyqxmd.com/read/29386146/positivity-of-antineutrophil-cytoplasmic-antibodies-in-children-prevalence-and-etiologies
#11
Ameni Jerbi, Sawsan Feki, Salma Ben Ameur, Hend Hachicha, Lamia Gargouri, Thouraya Kamoun, Abdelmajid Mahfoudh, Mongia Hachicha, Hatem Masmoudi
In adults, anti-neutrophil cytoplasmic antibodies (ANCA) are considered as serological markers of several diseases, especially vasculitis and glomerulonephritis. Since ANCA are rarely positive in children, few data about the clinical relevance of these auto-antibodies in pediatric population have been reported. Therefore, our study aims to describe the spectrum of disorders associated with positive ANCA in Tunisian children. This study had been carried out over a period of 12 years and a half. All patients under the age of 15 for whom ANCA screening was performed in our laboratory were included...
January 1, 2018: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/29370679/fecal-calprotectin-beyond-intestinal-organic-diseases
#12
Gian P Caviglia, Davide G Ribaldone, Chiara Rosso, Giorgio M Saracco, Marco Astegiano, Rinaldo Pellicano
Fecal calprotectin (FC) is a calcium-binding protein with antimicrobic, imunomodulatory and antiproliferative properties that is mainly found in the cytoplasm of neutrophil granulocytes. During the last decades, FC became an increasingly useful tool both for gastroenterologists and for general practitioners for distinguishing inflammatory bowel disease (IBD) from irritable bowel syndrome. FC correlates with clinical scoring systems and endoscopic lesions in IBD and is considered a reliable biomarker for the prediction of clinical relapse or remission...
January 25, 2018: Panminerva Medica
https://www.readbyqxmd.com/read/29343654/intestinal-epithelial-cell-specific-deletion-of-%C3%AE-mannosidase-ii-ameliorates-experimental-colitis
#13
Koichiro Suzuki, Takahiro Yamada, Keiko Yamazaki, Masato Hirota, Narumi Ishihara, Mizuki Sakamoto, Daisuke Takahashi, Hideki Iijima, Koji Hase
Inflammatory bowel disease (IBD) is a refractory disease of the gastrointestinal tract that is believed to develop in genetically susceptible individuals. Glycosylation, a type of post-translational modification, is involved in the development of a wide range of diseases, including IBD, by modulating the function of various glycoproteins. To identify novel genes contributing to the development of IBD, we analyzed single nucleotide polymorphisms (SNPs) of glycosylation-related genes in IBD patients and identified MAN2A1, encoding alpha-mannosidase II (α-MII), as a candidate gene...
January 18, 2018: Cell Structure and Function
https://www.readbyqxmd.com/read/29335491/validation-and-optimization-of-the-systemic-inflammation-based-modified-glasgow-prognostic-score-in-predicting-postoperative-outcome-of-inflammatory-bowel-disease-preliminary-data
#14
Chenyan Zhao, Chao Ding, Tingbin Xie, Tenghui Zhang, Xujie Dai, Yao Wei, Yi Li, Jianfeng Gong, Weiming Zhu
Systemic Inflammation-Based modified Glasgow Prognostic Score (mGPS) was developed as an objective tool to grade state of inflammation. However, the association between mGPS and postoperative complications for inflammatory bowel disease (IBD) patients was still unknown. In our study, 270 IBD patients [Crohn's disease (CD), n = 186; Ulcerative colitis (UC), n = 84] from January 2013 and January 2016 who underwent elective bowel resection were retrospectively analyzed, and, the levels of preoperative C-reactive protein (CRP) and albumin were included as parameters of mGPS...
January 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29334018/pyoderma-gangrenosum-demographics-treatments-and-outcomes-an-analysis-of-2-273-cases
#15
Sarah E Sasor, Tahereh Soleimani, Michael W Chu, Julia A Cook, Peter J Nicksic, Sunil S Tholpady
OBJECTIVE: Pyoderma gangrenosum is a rare, neutrophil-mediated, auto-inflammatory dermatosis. This condition has clinical features analogous to infectious processes and must be quickly diagnosed to be properly treated. The purpose of this study was to characterise relevant clinical features associated with pyoderma gangrenosum based on a large inpatient cohort. METHOD: The National Inpatient Sample (US) was used to identify patients with the diagnosis of pyoderma gangrenosum using ICD-9 diagnosis code 686...
January 1, 2018: Journal of Wound Care
https://www.readbyqxmd.com/read/29324769/impaired-resolution-of-dss-induced-colitis-in-mice-lacking-the-glucocorticoid-receptor-in-myeloid-cells
#16
Garrit K Meers, Hanibal Bohnenberger, Holger M Reichardt, Fred Lühder, Sybille D Reichardt
Inflammatory bowel disease (IBD) is a highly prevalent intestinal disorder for which no cure exists. Currently, the standard first-line treatment of IBD consists of systemic glucocorticoid (GC) application, even though therapy can be complicated by unresponsiveness or adverse effects. In view of the importance of macrophages and neutrophils for the pathogenesis of IBD we set out to define the relevance of these cell types as targets of GC using the mouse model of DSS-induced colitis. We found that the disease did not resolve in GRlysM mice lacking the GC receptor (GR) in myeloid cells after removal of the chemical insult...
2018: PloS One
https://www.readbyqxmd.com/read/29259036/loss-of-acid-ceramidase-in-myeloid-cells-suppresses-intestinal-neutrophil-recruitment
#17
Mel Pilar Espaillat, Ashley J Snider, Zhijuan Qiu, Breana Channer, Nicolas Coant, Edward H Schuchman, Richard R Kew, Brian S Sheridan, Yusuf A Hannun, Lina M Obeid
Bioactive sphingolipids are modulators of immune processes and their metabolism is often dysregulated in ulcerative colitis, a major category of inflammatory bowel disease (IBD). While multiple axes of sphingolipid metabolism have been investigated to delineate mechanisms regulating ulcerative colitis, the role of acid ceramidase (AC) in intestinal inflammation is yet to be characterized. Here we demonstrate that AC expression is elevated selectively in the inflammatory infiltrate in human and murine colitis...
December 19, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29247242/myeloid-differentiation-primary-response-gene-myd-88-signalling-is-not-essential-for-intestinal-fibrosis-development
#18
C Lutz, B Weder, A Hünerwadel, S Fagagnini, B Lang, N Beerenwinkel, J B Rossel, G Rogler, B Misselwitz, M Hausmann
Dysregulation of the immune response to microbiota is associated with inflammatory bowel disease (IBD), which can trigger intestinal fibrosis. MyD88 is a key component of microbiota signalling but its influence on intestinal fibrosis has not been clarified. Small bowel resections from donor-mice were transplanted subcutaneously into the neck of recipients C57BL/6 B6-MyD88tm1 Aki (MyD88-/-) and C57BL/6-Tg(UBC-green fluorescence protein (GFP))30Scha/J (GFP-Tg). Grafts were explanted up to 21 days after transplantation...
December 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29212102/-conservative-treatment-of-an-aseptic-abscess-syndrome-with-splenic-abscesses-in-crohn-s-disease
#19
Thomas Göbel, Jutta Rauen-Vossloh, Hubert Georg Hotz, Andree Boldt, Andreas Erhardt
A 24-year old woman with a history of Crohn's disease developed bloody diarrhea and multiple abdominal abscesses, daily fever, leukocytosis, and elevated CRP several months after her immunosuppressive therapy with azathioprine was stopped. Recurrent abscess punctures did not detect any pathogenic germs and neither clinical nor serological response was achieved by administration of different antimicrobial therapies. Additionally, new splenic abscesses arose despite ongoing therapy. Under the suspicion of the rare aseptic abscess syndrome, representing an auto-inflammatory, extra-intestinal manifestation of Crohn's disease, the antimicrobial therapy was stopped and an intravenous therapy with prednisolone was initiated...
December 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29210335/predictive-role-of-nudt15-variants-on-thiopurine-induced-myelotoxicity-in-asian-inflammatory-bowel-disease-patients
#20
Natalia Sutiman, Sylvia Chen, Khoon Lin Ling, Sai Wei Chuah, Wai Fook Leong, Vinayak Nadiger, Madeline Tjai, Chris San Choon Kong, Brian John Schwender, Webber Chan, Hang Hock Shim, Wee Chian Lim, Chiea Chuen Khor, Yin Bun Cheung, Balram Chowbay
BACKGROUND: Genetic variants of TPMT and NUDT15 have been reported to predict the inter-patient variability in response and toxicity profiles of patients receiving thiopurine therapy. However, the clinical utility of TPMT genotyping in guiding thiopurine doses has been questionable, in part due to underlying differences in the prevalence of TPMT variants in both Caucasian and Asian populations. Several NUDT15 variants have been associated with thiopurine-induced leukopenia, particularly in Asian cohorts...
December 6, 2017: Pharmacogenomics
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