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https://www.readbyqxmd.com/read/29620681/-immune-thrombocytopenia-as-key-feature-of-a-novel-ada2-deficiency-variant-implication-on-differential-diagnostics-of-itp-in-children
#1
Mikael Sundin, Per Marits, Stefan Nierkens, Antonios G A Kolios, Jakob Nilsson
Thrombocytopenia presenting during early childhood is most commonly diagnosed as immune/idiopathic thrombocytopenic purpura (ITP), where the antibody-mediated destruction of thrombocytes is often transient. If treatment is indicated, the majority of patients respond to immune-modulation by intravenous immunoglobulin G infusion or systemic corticosteroids. Differential diagnoses to childhood ITP includes thrombocytopenia due to infections, drugs, rheumatologic conditions, immune dysregulation, and inherited bone marrow failures, for example, congenital amegakaryocytic thrombocytopenia...
April 3, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29535105/filgrastim-enhances-t-cell-clearance-by-antithymocyte-globulin-exposure-after-unrelated-cord-blood-transplantation
#2
Coco de Koning, Julie-Anne Gabelich, Jurgen Langenhorst, Rick Admiraal, Jurgen Kuball, Jaap Jan Boelens, Stefan Nierkens
Residual antithymocyte globulin (ATG; Thymoglobulin) exposure after allogeneic hematopoietic (stem) cell transplantation (HCT) delays CD4+ T-cell immune reconstitution (CD4+ IR), subsequently increasing morbidity and mortality. This effect seems particularly present after cord blood transplantation (CBT) compared to bone marrow transplantation (BMT). The reason for this is currently unknown. We investigated the effect of active-ATG exposure on CD4+ IR after BMT and CBT in 275 patients (CBT n = 155, BMT n = 120; median age, 7...
March 13, 2018: Blood Advances
https://www.readbyqxmd.com/read/29487057/human-herpesvirus-6-viremia-affects-t-cell-reconstitution-after-allogeneic-hematopoietic-stem-cell-transplantation
#3
Coco de Koning, Rick Admiraal, Stefan Nierkens, Jaap Jan Boelens
Human herpesvirus 6 (HHV6) viremia is a common cause of morbidity following allogeneic hematopoietic cell transplantation (HCT). We previously associated T-cell reconstitution with HHV6 viremia. Here, we investigated whether HHV6 viremia affects T-cell reconstitution after HCT in a time-dependent retrospective analysis. We included 273 pediatric patients (0.1-22.7 years; median follow-up, 58 months) receiving a first HCT between 2004 and 2014. HHV6 was screened weekly in plasma via polymerase chain reaction and occurred in 79 patients (29%) at a median time of 19 days after transplant...
February 27, 2018: Blood Advances
https://www.readbyqxmd.com/read/29412785/fine-tuning-antithymocyte-globulin-dosing-and-harmonizing-clinical-trial-design
#4
Jaap Jan Boelens, Rick Admiraal, Jurgen Kuball, Stefan Nierkens
No abstract text is available yet for this article.
February 7, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29410314/serum-biomarker-profiles-suggest-that-atopic-dermatitis-is-a-systemic-disease
#5
Judith L Thijs, Ian Strickland, Carla A F M Bruijnzeel-Koomen, Stefan Nierkens, Barbara Giovannone, Edward F Knol, Eszter Csomor, Bret R Sellman, Tomas Mustelin, Matthew A Sleeman, Marjolein S de Bruin-Weller, Athula Herath, Julia Drylewicz, Richard D May, DirkJan Hijnen
No abstract text is available yet for this article.
February 2, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29363167/endocytosed-soluble-cowpox-virus-protein-cpxv012-inhibits-antigen-cross-presentation-in-human-monocyte-derived-dendritic-cells
#6
Lotte Spel, Rutger D Luteijn, Jan W Drijfhout, Stefan Nierkens, Marianne Boes, Emmanuel J H Wiertz
Viruses may interfere with the MHC class I antigen presentation pathway in order to avoid CD8+ T cell-mediated immunity. A key target within this pathway is the peptide transporter TAP. This transporter plays a central role in MHC class I-mediated peptide presentation of endogenous antigens. In addition, TAP plays a role in antigen cross-presentation of exogenously derived antigens by dendritic cells (DCs). In this study, a soluble form of the cowpox virus TAP inhibitor CPXV012 is synthesized for exogenous delivery into the antigen cross-presentation route of human monocyte-derived (mo)DCs...
February 2018: Immunology and Cell Biology
https://www.readbyqxmd.com/read/29359983/use-of-cord-blood-derived-t-cells-in-cancer-immunotherapy-milestones-achieved-and-future-perspectives
#7
Vania Lo Presti, Stefan Nierkens, Jaap Jan Boelens, Niek P van Til
Hematopoietic cell transplantation is a potentially lifesaving procedure for patients with hematological malignancies who are refractory to conventional chemotherapy and/or irradiation treatment. Umbilical cord blood (CB) transplantation, as a hematopoietic stem and progenitor cell (HSPC) source, has several advantages over bone marrow transplantation with respect to matching and prompt availability for transplantation. Additionally, CB has some inherent features, such as rapid expansion of T cells, lower prevalence of graft-versus-host disease and higher graft versus tumor efficacy that make this HSPC cell source more favorable over other HSPC sources...
March 2018: Expert Review of Hematology
https://www.readbyqxmd.com/read/29334817/quantiferon-plus-does-not-discriminate-between-active-and-latent-tuberculosis
#8
Regina W Hofland, Aik W J Bossink, Stefan Nierkens, Sanne P A Paardekooper, Brigitte T A van den Broek, Jan-Willem J Lammers, Ineke van Haeften, Steven F T Thijsen
No abstract text is available yet for this article.
January 15, 2018: Infectious Diseases
https://www.readbyqxmd.com/read/28751481/recruitment-and-retention-in-a-10-month-social-network-based-intervention-promoting-diabetes-self-management-in-socioeconomically-deprived-patients-a-qualitative-process-evaluation
#9
Charlotte Vissenberg, Vera Nierkens, Paul J M Uitewaal, Barend J C Middelkoop, Karien Stronks
OBJECTIVES: Socioeconomically deprived patients with type 2 diabetes often face challenges with self-management, resulting in more diabetes-related complications. However, these groups are often under-represented in self-management interventions. Evidence on effective recruitment and retention strategies is growing, but lacking for intensive self-management interventions. This study aims to explore recruitment, retention and effective intervention strategies in a 10-month group-based intervention among Dutch, Moroccan, Turkish and Surinamese patients from socioeconomically deprived neighbourhoods...
July 26, 2017: BMJ Open
https://www.readbyqxmd.com/read/28707567/the-impact-of-a-social-network-based-intervention-on-self-management-behaviours-among-patients-with-type-2-diabetes-living-in-socioeconomically-deprived-neighbourhoods-a-mixed-methods-approach
#10
EDITORIAL
Charlotte Vissenberg, Vera Nierkens, Irene van Valkengoed, Giel Nijpels, Paul Uitewaal, Barend Middelkoop, Karien Stronks
AIMS: This paper aims to explore the effect of the social network based intervention Powerful Together with Diabetes on diabetes self-management among socioeconomically deprived patients. This 10-month group intervention targeting patients and significant others aimed to improve self-management by stimulating social support and diminishing social influences that hinder self-management. METHODS: This intervention was evaluated in a quasi-experimental study using a mixed methods approach...
August 2017: Scandinavian Journal of Public Health
https://www.readbyqxmd.com/read/28691750/human-neonatal-thymectomy-induces-altered-b-cell-responses-and-autoreactivity
#11
Theo van den Broek, Asaf Madi, Eveline M Delemarre, Alvin W L Schadenberg, Kiki Tesselaar, José A M Borghans, Stefan Nierkens, Frank A Redegeld, Henny G Otten, Maura Rossetti, Salvatore Albani, Rachel Sorek, Irun R Cohen, Nicolaas J G Jansen, Femke van Wijk
An association between T-cell lymphopenia and autoimmunity has long been proposed, but it remains to be elucidated whether T-cell lymphopenia affects B-cell responses to autoantigens. Human neonatal thymectomy (Tx) results in a decrease in T-cell numbers and we used this model to study the development of autoreactivity. Two cohorts of neonatally thymectomized individuals were examined, a cohort of young (1-5 years post-Tx, n = 10-27) and older children (>10 years, n = 26), and compared to healthy age-matched controls...
November 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28674122/effectiveness-of-a-targeted-lifestyle-intervention-in-primary-care-on-diet-and-physical-activity-among-south-asians-at-risk-for-diabetes-2-year-results-of-a-randomised-controlled-trial-in-the-netherlands
#12
Everlina M A Vlaar, Vera Nierkens, Mary Nicolaou, Barend J C Middelkoop, Wim B Busschers, Karien Stronks, Irene G M van Valkengoed
OBJECTIVES: In South Asian populations, little is known about the effects of intensive interventions to reduce the risk of type 2 diabetes on health behaviour. We examined the effectiveness at 2 years of a culturally targeted lifestyle intervention on diet, physical activity and determinants of behaviour change among South Asians at risk for diabetes. DESIGN: Randomised controlled trial with de facto masking. SETTING: Primary care. PARTICIPANTS: A total of 536 18- to 60-year-old South Asians at risk for diabetes (ie, with impaired glucose tolerance, impaired fasting glucose or relatively high insulin resistance) were randomised to the intervention (n=283) or a control (n=253) group...
July 2, 2017: BMJ Open
https://www.readbyqxmd.com/read/28607912/immune-reconstitution-and-outcomes-after-conditioning-with-anti-thymocyte-globulin-in-unrelated-cord-blood-transplantation-the-good-the-bad-and-the-ugly
#13
REVIEW
Coco de Koning, Rick Admiraal, Stefan Nierkens, Jaap Jan Boelens
Unrelated umbilical cord blood transplantation (UCBT) exhibits a low risk of graft-versus-host-disease (GvHD) and has unique potent anti-virus and anti-leukemia effects. Anti-thymocyte globulin (ATG) in the conditioning regimen for UCBT is successful in reducing graft rejection and GvHD. Nevertheless, this beneficial effect of ATG coincides with its detrimental effect on immune reconstitution. The latter directly relates to a high incidence of viral infections and leukemia relapses. ATG has been used in transplant patients for over 30 years...
2017: Stem Cell Investigation
https://www.readbyqxmd.com/read/28412391/moving-toward-endotypes-in-atopic-dermatitis-identification-of-patient-clusters-based-on-serum-biomarker-analysis
#14
Judith L Thijs, Ian Strickland, Carla A F M Bruijnzeel-Koomen, Stefan Nierkens, Barbara Giovannone, Eszter Csomor, Bret R Sellman, Tomas Mustelin, Matthew A Sleeman, Marjolein S de Bruin-Weller, Athula Herath, Julia Drylewicz, Richard D May, DirkJan Hijnen
BACKGROUND: Atopic dermatitis (AD) is a complex, chronic, inflammatory skin disease with a diverse clinical presentation. However, it is unclear whether this diversity exists at a biological level. OBJECTIVE: We sought to test the hypothesis that AD is heterogeneous at the biological level of individual inflammatory mediators. METHODS: Sera from 193 adult patients with moderate-to-severe AD (six area, six sign atopic dermatitis [SASSAD] score: geometric mean, 22...
September 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28392330/viral-reactivations-and-associated-outcomes-in-the-context-of-immune-reconstitution-after-pediatric-hematopoietic-cell-transplantation
#15
Rick Admiraal, Coco C H de Koning, Caroline A Lindemans, Marc B Bierings, Annemarie M J Wensing, A Birgitta Versluys, Tom F W Wolfs, Stefan Nierkens, Jaap Jan Boelens
BACKGROUND: Viral reactivations (VRs) after hematopoietic cell transplantation (HCT) contribute to significant morbidity and mortality. Timely immune reconstitution (IR) is suggested to prevent VR. OBJECTIVES: We studied the relation between IR (as a continuous predictor over time) and VR (as a time-varying predictor) and the relation between VR and other clinical outcomes. METHODS: In this retrospective analysis all patients receiving a first HCT between January 2004 and September 2014 were included...
December 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28369318/candidate-serum-markers-in-early-crohn-s-disease-predictors-of-disease-course
#16
Carolijn Smids, Carmen S Horjus Talabur Horje, Stefan Nierkens, Julia Drylewicz, Marcel J M Groenen, Peter J Wahab, Ellen G van Lochem
Background and Aims: More than half of patients with Crohn's disease [CD] develop disease complications requiring aggressive medical therapy or surgery over time. However, predicting disease course and treatment response remains difficult. We therefore identified distinctive serum analytes associated with disease activity and course in newly diagnosed, untreated patients at presentation and during their follow-up. Methods: In a pilot study, a multiplex immunoassay analysis on 36 markers was performed on serum from 20 CD patients at the time of primary diagnosis following endoscopic evaluation...
September 1, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/28330607/association-between-anti-thymocyte-globulin-exposure-and-survival-outcomes-in-adult-unrelated-haemopoietic-cell-transplantation-a-multicentre-retrospective-pharmacodynamic-cohort-analysis
#17
Rick Admiraal, Stefan Nierkens, Moniek A de Witte, Eefke J Petersen, Ger-Jan Fleurke, Luka Verrest, Svetlana V Belitser, Robbert G M Bredius, Reinier A P Raymakers, Catherijne A J Knibbe, Monique C Minnema, Charlotte van Kesteren, Jurgen Kuball, Jaap J Boelens
BACKGROUND: Anti-thymocyte globulin (ATG) is used to prevent graft-versus-host disease (GvHD) after allogeneic haemopoietic cell transplantation (HCT). However, ATG can also cause delayed immune reconstitution of T cells, negatively affecting survival. We studied the relation between exposure to ATG and clinical outcomes in adult patients with acute leukaemia and myelodysplastic syndrome. METHODS: We did a retrospective, pharmacokinetic-pharmacodynamic analysis of data from patients with acute lymphoid leukaemia, acute myeloid leukaemia, or myelodysplastic syndrome receiving their first T-cell repleted allogeneic peripheral blood stem cell HCT with ATG (thymoglobulin) as part of non-myeloablative conditioning from March 1, 2004, to June 1, 2015...
April 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28175052/p267-candidate-serum-markers-in-newly-diagnosed-crohn-s-disease-patients
#18
C Smids, C Horjus Talabur Horje, S Nierkens, J Drylewicz, M Groenen, P Wahab, E van Lochem
No abstract text is available yet for this article.
February 1, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/28128370/an-ocular-protein-triad-can-classify-four-complex-retinal-diseases
#19
J J W Kuiper, L Beretta, S Nierkens, R van Leeuwen, N H Ten Dam-van Loon, J Ossewaarde-van Norel, M C Bartels, J D F de Groot-Mijnes, P Schellekens, J H de Boer, T R D J Radstake
Retinal diseases generally are vision-threatening conditions that warrant appropriate clinical decision-making which currently solely dependents upon extensive clinical screening by specialized ophthalmologists. In the era where molecular assessment has improved dramatically, we aimed at the identification of biomarkers in 175 ocular fluids to classify four archetypical ocular conditions affecting the retina (age-related macular degeneration, idiopathic non-infectious uveitis, primary vitreoretinal lymphoma, and rhegmatogenous retinal detachment) with one single test...
January 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28017520/microbial-dysbiosis-in-common-variable-immune-deficiencies-evidence-causes-and-consequences
#20
REVIEW
Roos-Marijn Berbers, Stefan Nierkens, Jacob M van Laar, Debby Bogaert, Helen L Leavis
Common variable immunodeficiency (CVID) is an immune disorder that not only causes increased susceptibility to infection, but also to inflammatory complications such as autoimmunity, lymphoid proliferation, malignancy, and granulomatous disease. Recent findings implicate the microbiome as a driver of this systemic immune dysregulation. Here, we critically review the current evidence for a role of the microbiome in the pathogenesis of CVID immune dysregulation, and describe the possible immunologic mechanisms behind causes and consequences of microbial dysbiosis in CVID...
March 2017: Trends in Immunology
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